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2. Ultra-Hypofractionated Whole Breast Radiotherapy with Automated Hybrid-VMAT Technique: A Pilot Study on Safety, Skin Toxicity and Aesthetic Outcomes

Author

Boccardi M, Cilla S, Fanelli M, Romano C, Bonome P, Ferro M, Pezzulla D, Di Marco R, Deodato F, and Macchia G

Subjects
breast cancer, ultra-hypofractionated radiotherapy, cutometer, hybrid-vmat technique., Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Mariangela Boccardi,1 Savino Cilla,2 Mara Fanelli,3 Carmela Romano,2 Paolo Bonome,1 Milena Ferro,1 Donato Pezzulla,1 Roberto Di Marco,4 Francesco Deodato,1,5,* Gabriella Macchia1,* 1Radiation Oncology Unit, Responsible Research Hospital, Campobasso, Italy; 2Medical Physics Unit, Responsible Research Hospital, Campobasso, Italy; 3Research Laboratories, Responsible Research Hospital, Campobasso, Italy; 4Department of Medicina e Scienze della Salute “V. Tiberio”, Università degli Studi del Molise, Campobasso, Italy; 5Istituto di Radiologia, Università Cattolica del Sacro Cuore, Roma, Italy*These authors contributed equally to this workCorrespondence: Milena Ferro, Responsible Research Hospital, L.go Gemelli, 1, Campobasso, 86100, Italy, Email milena.ferro@responsible.hospitalPurpose: The most prevalent treatment-related side effect related to adjuvant radiotherapy (RT) for breast cancer is acute skin toxicity in the irradiated area. The purpose of this single-institution pilot study is to provide preliminary clinical results on the feasibility and safety of a breast ultra-hypofractionated radiation treatment delivered using an automated hybrid-VMAT technique. Skin damage was assessed both with clinical examination and objectively using a Cutometer equipment.Patients and Methods: Patients received 26 Gy to the whole breast and 30 Gy to the tumoral bed in 5 fractions using an automated hybrid-VMAT approach with the option for the breath hold technique if necessary. Acute and late toxicities were clinically evaluated at baseline, 1- and 6-months after treatment using the CTC-AE v.5.0 scale. An instrumental evaluation of the skin elasticity was performed using a Cutometer® Dual MP580. Two parameters per patient, R0 (the total skin firmness) and Q1 (the elastic recovery), were registered at the different timelines.Results: From June 2022 to January 2024, 30 patients, stage T1-T2, N0 were enrolled in the study. Four out of 30 (13.3%) patients reported G2 acute skin toxicities. At 6 months, G2 late toxicity was registered in 3 patients (10%). A total of 2160 measures of R0 and Q1 were recorded. At 1 month after treatment, no correlation was found between measured values of R0 and Q1 and clinical evaluation. At 6 months after treatment, clinical late toxicity ≥ 1 was strongly associated with decreased R0 and Q1 values ≥ 24% (p = 0.003) and ≥ 18% (p = 0.022), respectively.Conclusion: Ultra-hypofractionated whole-breast radiotherapy, when supported by advanced treatment techniques, is both feasible and safe. No severe adverse effects were observed at any of the different timeframes. Acute and late skin toxicities were shown to be lower in contrast to data presented in the literature.Keywords: breast cancer, ultra-hypofractionated radiotherapy, Cutometer, hybrid-VMAT technique

Published
2024

8. Intensity-Modulated Radiotherapy with Concomitant Boost After Breast Conserving Surgery: A Phase I–II Trial

Author

Macchia G, Cilla S, Buwenge M, Zamagni A, Ammendolia I, Zamagni C, Frezza GP, Valentini V, Deodato F, and Morganti AG

Subjects
breast cancer, adjuvant radiotherapy, concomitant boost, imrt, toxicity., Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Gabriella Macchia,1 Savino Cilla,2 Milly Buwenge,3 Alice Zamagni,3 Ilario Ammendolia,3 Claudio Zamagni,4 Giovanni P Frezza,5 Vincenzo Valentini,6,7 Francesco Deodato,1,* Alessio G Morganti3,* 1Radiotherapy Unit, Gemelli Molise Hospital, Università Cattolica Del Sacro Cuore, Campobasso, Italy; 2Medical Physics Unit, Gemelli Molise Hospital, Università Cattolica Del Sacro Cuore, Campobasso, Italy; 3Radiation Oncology Center, Department of Experimental, Diagnostic and Specialty Medicine ‑ DIMES, University of Bologna, S. Orsola-Malpighi Hospital, Bologna, Italy; 4Addarii Medical Oncology Unit, S. Orsola-Malpighi Hospital, Bologna, Italy; 5Radiation Oncology Unit, Bellaria Hospital, Bologna, Italy; 6Dipartimento di Scienze Radiologiche, Radioterapiche ed Ematologiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, UOC di Radioterapia, Rome, Italy; 7Istituto di Radiologia, Università Cattolica Del Sacro Cuore, Rome, Italy*These authors contributed equally to this workCorrespondence: Milly BuwengeRadiation Oncology Unit, Department of Experimental, Diagnostic and Specialty Medicine – DIMES, University of Bologna, S. Orsola-Malpighi Hospital, Via Giuseppe Massarenti 9, 40138, BolognaTel +39 0512143564Fax +39 0516364336Email mbuwenge@gmail.comPurpose: A concomitant boost (CB) in patients treated with postoperative radiotherapy after conservative surgery of invasive breast cancer (BC) has been suggested for treatment time reduction and therapy intensification. The aim of this analysis was to assess long-term tolerability of a CB in patients treated with postoperative intensity Modulated Accelerated RAdiotherapy (MARA).Patients and Methods: In this phase I–II trial, 321 patients with intermediate-high risk BC (pT1-4 with at least one of the following characteristics: pre or perimenopausal status, pN2-3, positive or close margins) were enrolled. Patients were treated with forward-planned intensity modulated radiotherapy (IMRT) and CB. A total dose of 50 Gy (2 Gy/fraction) and 60 Gy (2.4 Gy/fraction) was prescribed to the whole breast and the tumor bed, respectively. The potential impact of hypertension, diabetes, smoking habit, alcohol consumption, chemotherapy, and hormone therapy on both skin and subcutaneous late toxicity-free survival (LTFS) was evaluated. Survival curves were calculated using the Kaplan–Meier method.Results: Median follow-up was 52 months (range: 3– 115). Regional node irradiation, adjuvant chemotherapy and hormonal therapy were prescribed to 29.3%, 65.4% and 81.0% of patients, respectively. Five-year G2 and G3 skin LTFS were 95.6% and 100.0%, respectively. Five-year G2 and G3 subcutaneous LTFS were 80.0% and 98.6%, respectively. Only diabetes showed a significant correlation with worse G3 subcutaneous LTFS (p: 0.024). Five-year loco-regional control, metastasis-free survival, disease-free survival, and overall survival were 98.0%, 91.8%, 89.7% and 96.3%, respectively.Conclusion: IMRT combined with CB was associated with a low risk of > G2 late toxicities (0.0% and 1.4% for skin and subcutaneous tissue, respectively). The cumulative actuarial incidence of local recurrences was 2.0% despite the exclusion of low-risk patients. Our results suggest that CB is safe and effective in patients with intermediate-high risk BC.Trial Registration: ClinicalTrials.gov: NCT03471741.Keywords: breast cancer, adjuvant radiotherapy, concomitant boost, IMRT, toxicity

Published
2020

11. Hypofractionated radiotherapy after conservative surgery may increase low–intermediate grade late fibrosis in breast cancer patients

Author

Digesù C, Deodato F, Macchia G, Cilla S, Pieri M, Zamagni A, Farioli A, Buwenge M, Ferrandina G, and Morganti AG

Subjects
breast cancer, radiotherapy, hypofractionation, retrospective study., Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Cinzia Digesù,1 Francesco Deodato,1 Gabriella Macchia,1 Savino Cilla,2 Martina Pieri,3 Alice Zamagni,4 Andrea Farioli,5 Milly Buwenge,4 Gabriella Ferrandina,6,* Alessio G Morganti4,* 1Radiotherapy Unit, General Oncology Unit, Fondazione Giovanni Paolo II, Campobasso, Italy; 2Medical Physics Unit, Fondazione Giovanni Paolo II, Campobasso, Italy; 3Radiotherapy Unit, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, Meldola, Italy; 4Radiation Oncology Center, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy; 5Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy; 6Department of Woman and Child Health, Gynecologic Oncology Unit, Fondazione “Policlinico Universitario A. Gemelli”, IRCSS, Universita’ Cattolica Sacro Cuore, Rome, Italy *These authors contributed equally to this work Aim: To compare late toxicity after postoperative hypofractionated radiotherapy (RT) and standard fractionated RT in patients with early-stage breast carcinoma.Methods: This retrospective study included 447 patients (Modulated Accelerated Radiotherapy [MARA-1]: 317 patients, and control group [CG]: 130 patients). In the CG, the whole breast received 50.4 Gy in 28 fractions (fx) using 3D-radiotherapy, plus a sequential electron boost (10 Gy in 4 fx) to tumor bed. In MARA-1 group, a forward-planned intensity-modulated radiotherapy technique with 40 Gy in 16 fx with a concomitant boost of 4 Gy to breast was used. The primary endpoint was to evaluate late toxicity, and secondary endpoints were acute toxicity, local control, and survival. ClinicalTrials.gov: NCT03461224.Results: Median follow-up was 52 months (range: 3–115 months). Late skin and subcutaneous toxicity were acceptable: 5-year actuarial cumulative incidence of Grade (G) 3 late skin toxicity was 1.5% in CG and 0.0% in MARA-1. Five-year actuarial cumulative incidence of G3 late subcutaneous toxicity was 0.8% in CG and 0.3% in MARA-1. On multivariate analysis, tobacco smoking and planning target volume were associated with an increased risk of late G1 skin toxicity (HR: 2.15, 95% CI: 1.38–3.34 and HR: 1.12, 95% CI: 1.07–1.18, respectively), whereas patients with a larger planning target volume also showed an increased risk of G1 and G2 late subcutaneous toxicity (HR: 1.14, CI 95%: 1.08–1.20 and HR: 1.14, 95% CI: 1.01–1.28, respectively). MARA-1 patients also showed an increased risk of late G1 and G2 subcutaneous toxicity (HR: 2.35, 95% CI: 1.61–3.41 and HR: 3.07, 95% CI: 1.11–8.53, respectively) compared to CG.Conclusion: In this retrospective analysis, postoperative accelerated-hypofractionated RT for early-stage-breast carcinoma was associated with higher incidence of subcutaneous side effects. However, this increase was limited to G1–G2 toxicity. In the future, development of predictive models could help in tailoring dose and fractionation based on the risk of toxicity. Keywords: breast cancer, radiotherapy, hypofractionation, retrospective study

Published
2018

12. Intensity modulated radiation therapy for breast cancer: current perspectives

Author

Buwenge M, Cammelli S, Ammendolia I, Tolento G, Zamagni A, Arcelli A, Macchia G, Deodato F, Cilla S, and Morganti AG

Subjects
intensity modulated radiotherapy, breast cancer, literature review., Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Milly Buwenge,1 Silvia Cammelli,1 Ilario Ammendolia,1 Giorgio Tolento,1 Alice Zamagni,1 Alessandra Arcelli,2 Gabriella Macchia,3 Francesco Deodato,3 Savino Cilla,4 Alessio G Morganti1 1Department of Experimental, Diagnostic and Specialty Medicine – DIMES, Radiation Oncology Center, University of Bologna, S.Orsola-Malpighi Hospital, 2Radiation Oncology Unit, Bellaria Hospital, Bologna, 3Radiation Oncology Unit, 4Medical Physics Unit, Research and Care Foundation ‘Giovanni Paolo II,’ Catholic University of Sacred Heart, Campobasso, Italy Background: Owing to highly conformed dose distribution, intensity modulated radiation therapy (IMRT) has the potential to improve treatment results of radiotherapy (RT). Postoperative RT is a standard adjuvant treatment in conservative treatment of breast cancer (BC). The aim of this review is to analyze available evidence from randomized controlled trials (RCTs) on IMRT in BC, particularly in terms of reduction of side effects. Methods: A literature search of the bibliographic database PubMed, from January 1990 through November 2016, was performed. Only RCTs published in English were included. Results: Ten articles reporting data from 5 RCTs fulfilled the selection criteria and were included in our review. Three out of 5 studies enrolled only selected patients in terms of increased risk of toxicity. Three studies compared IMRT with standard tangential RT. One study compared the results of IMRT in the supine versus the prone position, and one study compared standard treatment with accelerated partial breast IMRT. Three studies reported reduced acute and/or late toxicity using IMRT compared with standard RT. No study reported improved quality of life. Conclusion: IMRT seems able to reduce toxicity in selected patients treated with postoperative RT for BC. Further analyses are needed to better define patients who are candidates for this treatment modality. Keywords: intensity modulated radiation therapy, breast cancer, literature review

Published
2017

14. PD-0063 Final results of a randomized trial on accelerated radiotherapy in bone metastases (NCT03503682)

Author

Zamagni, A., primary, Siepe, G., additional, Bisello, S., additional, Scirocco, E., additional, Candoli, F., additional, Deodato, F., additional, Macchia, G., additional, Fiorica, F., additional, Farina, E., additional, Cilla, S., additional, Ammendolia, I., additional, Caravatta, L., additional, Malizia, C., additional, Cammelli, S., additional, Cellini, F., additional, and Morganti, A.G., additional

Published
2023
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15. PO-1474 Adjuvant radiotherapy of prostate cancer: a comprehensive analysis of toxicity predictors

Author

Cavallini, L., primary, Malizia, C., additional, Macchia, G., additional, Ntreta, M., additional, Arcelli, A., additional, Mammini, F., additional, Galietta, E., additional, Paolinelli, S., additional, Laghi, V., additional, Vallerossa, D., additional, Natoli, E., additional, Cilla, S., additional, Bisello, S., additional, Cortesi, A., additional, Cammelli, S., additional, Frezza, G.P., additional, Morganti, A.G., additional, Deodato, F., additional, and Buwenge, M., additional

Published
2023
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16. PD-0072 Reluctance to escalate drug therapy in cancer patients increases with the class of analgesics

Author

Donati, C.M., primary, Nardi, E., additional, Zamagni, A., additional, Siepe, G., additional, Cellini, F., additional, Di Rito, A., additional, Portaluri, M., additional, De Tommaso, C., additional, Santacaterina, A., additional, Tamburella, C., additional, Di Franco, R., additional, Parisi, S., additional, Cossa, S., additional, Fusco, V., additional, Bianculli, A., additional, Ziccarelli, P., additional, Ziccarelli, L., additional, Genovesi, D., additional, Caravatta, L., additional, Deodato, F., additional, Macchia, G., additional, Fiorica, F., additional, Napoli, G., additional, Buwenge, M., additional, Rossi, R., additional, Cammelli, S., additional, Maltoni, M., additional, and Morganti, A.G., additional

Published
2023
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17. OC-0919 Stereotactic reirradiation:toxicity profile results from a phase I dose escalation study (Destroy-1)

Author

Pezzulla, D., primary, Macchia, G., additional, Cilla, S., additional, Ferra, M., additional, Buwenge, M., additional, Romano, C., additional, Cammelli, S., additional, Bonome, P., additional, Picardi, V., additional, Boccardi, M., additional, Ferro, M., additional, Gambacorta, M.A., additional, Morganti, A.G., additional, and Deodato, F., additional

Published
2023
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18. MO-0051 SBRT in oligometastatic uterine cancer: a large, multicenter, retrospective study.

Author

Macchia, G., primary, Pezzulla, D., additional, Campitelli, M., additional, Laliscia, C., additional, Fodor, A., additional, Bonome, P., additional, Draghini, L., additional, Ippolito, E., additional, De Sanctis, V., additional, Ferioli, M., additional, Titone, F., additional, Balcet, V., additional, Di Cataldo, V., additional, Russo, D., additional, Vicenzi, L., additional, Cossa, S., additional, Lucci, S., additional, Deodato, F., additional, Gambacorta, M.A., additional, and Ferrandina, G., additional

Published
2023
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20. PD-0065 Short course palliative radiotherapy in advanced solid tumors: a pooled analysis (SHARON PROJECT)

Author

Donati, C.M., primary, Macchia, G., additional, Malizia, C., additional, Siepe, G., additional, Zamagni, A., additional, Cellini, F., additional, Buwenge, M., additional, Cilla, S., additional, Cammelli, S., additional, Rizzo, S., additional, Caravatta, L., additional, Wondemagegnhu, T., additional, Uddin, A.F.M.K., additional, Deressa, B.T., additional, Sumon, M.A., additional, Strigari, L., additional, Lodi Rizzini, E., additional, Bazzocchi, A., additional, Morganti, A.G., additional, Deodato, F., additional, and Farina, E., additional

Published
2023
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22. PO-1432 The role of inflammation markers in cervical cancers undergoing definitive chemoradiation

Author

Forlani, L., primary, Benini, A., additional, Laghi, V., additional, Medici, F., additional, Ma, J., additional, Ferioli, M., additional, Malizia, C., additional, Galuppi, A., additional, Macchia, G., additional, Deodato, F., additional, Cilla, S., additional, Buwenge, M., additional, Cammelli, S., additional, Morganti, A.G., additional, and Arcelli, A., additional

Published
2023
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24. PO-1360 Chemoradiation of locally advanced biliary cancer. A systematic review.

Author

Bisello, S., primary, Malizia, C., additional, Benini, A., additional, Mammini, F., additional, Laghi, V., additional, Paolinelli, S., additional, Guido, A., additional, Galuppi, A., additional, Arcelli, A., additional, Ferioli, M., additional, Buwenge, M., additional, Macchia, G., additional, Deodato, F., additional, Cilla, S., additional, Cammelli, S., additional, and Morganti, A.G., additional

Published
2023
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25. PO-2097 Acute toxicity prediction after breast radiotherapy using machine-learning and spectrophotometry

Author

Cilla, S., primary, Romano, C., additional, Macchia, G., additional, Boccardi, M., additional, Pezzulla, D., additional, Buwenge, M., additional, Di Castelnuovo, A., additional, Bracone, F., additional, De Curtis, A., additional, Cerletti, C., additional, Iacoviello, L., additional, Donati, M.B., additional, Deodato, F., additional, and Morganti, A.G., additional

Published
2023
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26. PD-0566 MRI before salvage radiotherapy after radical prostatectomy in prostate cancer: a case-control study

Author

Cavallini, L., primary, Gaudiano, C., additional, Galietta, E., additional, Mammini, F., additional, Paolinelli, S., additional, Laghi, V., additional, Vallerossa, D., additional, Natoli, E., additional, Morganti, A.G., additional, Arcelli, A., additional, Cammelli, S., additional, Macchia, G., additional, Deodato, F., additional, Golfieri, R., additional, Frezza, G.P., additional, and Ntreta, M., additional

Published
2023
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30. 19 A large, multicenter, retrospective study on efficacy and safety of stereotactic body radiotherapy (SBRT) in oligometastatic ovarian cancer (MITO RT1 study)

Author

Macchia, G, Lazzari, R, Colombo, N, Laliscia, C, Capelli, G, D’Agostino, GR, Deodato, F, Trippa, F, Ippolito, E, Ronchi, S, Pajar, F, Scorsetti, M, Cilla, S, Ingargiola, R, Huscher, A, Cerrotta, AM, Andrei, F, Vicenzi, L, Russo, D, Borghesi, S, Perrucci, E, Pignata, S, Aristei, C, Morganti, AG, Scambia, G, Valentini, V, Jereczek-Fossa, BA, and Ferrandina, G

Published
2019
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31. Dabrafenib-Trametinib and Radiotherapy for Oligoprogressive BRAF Mutant Advanced Melanoma

Author

Rossi, Ernesto, Schinzari, Giovanni, Cellini, Francesco, Balducci, Mario, Pasqualoni, Mariangela, Maiorano, Brigida Anna, Fionda, Bruno, Longo, Silvia, Deodato, Francesco, Di Stefani, Alessandro, Peris, Ketty, Gambacorta, Maria Antonietta, Tortora, Giampaolo, Rossi E., Schinzari G. (ORCID:0000-0001-6105-7252), Cellini F. (ORCID:0000-0002-2145-2300), Balducci M. (ORCID:0000-0003-0398-9726), Pasqualoni M., Maiorano B. A., Fionda B., Longo S., Deodato F. (ORCID:0000-0003-1276-5070), Di Stefani A., Peris K. (ORCID:0000-0002-5237-0463), Gambacorta M. A. (ORCID:0000-0001-5455-8737), Tortora G. (ORCID:0000-0002-1378-4962), Rossi, Ernesto, Schinzari, Giovanni, Cellini, Francesco, Balducci, Mario, Pasqualoni, Mariangela, Maiorano, Brigida Anna, Fionda, Bruno, Longo, Silvia, Deodato, Francesco, Di Stefani, Alessandro, Peris, Ketty, Gambacorta, Maria Antonietta, Tortora, Giampaolo, Rossi E., Schinzari G. (ORCID:0000-0001-6105-7252), Cellini F. (ORCID:0000-0002-2145-2300), Balducci M. (ORCID:0000-0003-0398-9726), Pasqualoni M., Maiorano B. A., Fionda B., Longo S., Deodato F. (ORCID:0000-0003-1276-5070), Di Stefani A., Peris K. (ORCID:0000-0002-5237-0463), Gambacorta M. A. (ORCID:0000-0001-5455-8737), and Tortora G. (ORCID:0000-0002-1378-4962)

Abstract

The clinical management of metastatic melanoma has been changed by BRAF (BRAFi) and MEK inhibitors (MEKi), which represent a standard treatment for BRAF-mutant melanoma. In oligoprogressive melanoma patients with BRAF mutations, target therapy can be combined with loco-regional radiotherapy (RT). However, the association of BRAF/MEK inhibitors and RT needs to be carefully monitored for potential increased toxicity. Despite the availability of some reports regarding the tolerability of RT + target therapy, data on simultaneous RT and BRAFi/MEKi are limited and mostly focused on the BRAFi vemurafenib. Here, we report a series of metastatic melanoma patients who received fractioned RT regimens for oligoprogressive disease in combination with the BRAFi dabrafenib and the MEKi trametinib, which have continued beyond progression. None of the cases developed relevant adverse events while receiving RT or interrupted dabrafenib and trametinib administration. These cases suggest that a long period of dabrafenib/trametinib interruption during radiotherapy for oligoprogressive disease can be avoided. Prospective trials are warranted to assess the efficacy and safety of the contemporary administration of BRAF/MEK inhibitors and radiotherapy for oligoprogressive disease.

Published
2023

32. Acid sphingomyelinase deficiency (ASMD): addressing knowledge gaps in unmet needs and patient journey in Italy-a Delphi consensus

Author

Scarpa, M, Barbato, A, Bisconti, A, Burlina, A, Concolino, D, Deodato, F, Di Rocco, M, Dionisi-Vici, C, Donati, M, Fecarotta, S, Fiumara, A, Galeone, C, Giona, F, Giuffrida, G, Manna, R, Mariani, P, Pession, A, Scopinaro, A, Spada, M, Spandonaro, F, Trifirò, G, Carubbi, F, Cappellini, M, Scarpa, Maurizio, Barbato, Antonio, Bisconti, Annalisa, Burlina, Alberto, Concolino, Daniela, Deodato, Federica, Di Rocco, Maja, Dionisi-Vici, Carlo, Donati, Maria Alice, Fecarotta, Simona, Fiumara, Agata, Galeone, Carlotta, Giona, Fiorina, Giuffrida, Gaetano, Manna, Raffaele, Mariani, Paolo, Pession, Andrea, Scopinaro, Annalisa, Spada, Marco, Spandonaro, Federico, Trifirò, Gianluca, Carubbi, Francesca, Cappellini, Maria Domenica, Scarpa, M, Barbato, A, Bisconti, A, Burlina, A, Concolino, D, Deodato, F, Di Rocco, M, Dionisi-Vici, C, Donati, M, Fecarotta, S, Fiumara, A, Galeone, C, Giona, F, Giuffrida, G, Manna, R, Mariani, P, Pession, A, Scopinaro, A, Spada, M, Spandonaro, F, Trifirò, G, Carubbi, F, Cappellini, M, Scarpa, Maurizio, Barbato, Antonio, Bisconti, Annalisa, Burlina, Alberto, Concolino, Daniela, Deodato, Federica, Di Rocco, Maja, Dionisi-Vici, Carlo, Donati, Maria Alice, Fecarotta, Simona, Fiumara, Agata, Galeone, Carlotta, Giona, Fiorina, Giuffrida, Gaetano, Manna, Raffaele, Mariani, Paolo, Pession, Andrea, Scopinaro, Annalisa, Spada, Marco, Spandonaro, Federico, Trifirò, Gianluca, Carubbi, Francesca, and Cappellini, Maria Domenica

Abstract

Acid sphingomyelinase deficiency (ASMD) is an ultra-rare disease, and several gaps of knowledge on various issues remain, particularly at a regional/national level. Expert opinions collected through well-defined consensus methodologies are increasingly used to make available reliable information in the context of rare/ultra-rare diseases. With the aim to provide indications on infantile neurovisceral ASMD (also formerly known as Niemann–Pick disease type A), chronic neurovisceral ASMD (formerly known as Niemann–Pick disease type A/B) and chronic visceral ASMD (formerly known as Niemann–Pick disease type B) in Italy, we conducted a Delphi consensus of experts focused on five main areas: (i) patients and disease characteristics; (ii) unmet needs and quality of life; (iii) diagnostic issues; (iv) treatment-related aspects; and (v) patient journey. Pre-specified, objective criteria were used to outline the multidisciplinary panel, based on 19 Italian experts in ASMD in paediatric and adult patients from different Italian Regions, including both clinicians (n = 16) and ASMD patients’ advocacy or payors with expertise in rare diseases (n = 3). During two Delphi rounds, a high ratio of agreement was found on several topics related to ASMD characteristics, diagnosis, management and disease burden. Our findings may provide valuable indications for management of ASMD at a public health level in Italy.

Published
2023

36. Management of intracranial hypertension following traumatic brain injury: a best clinical practice adoption proposal for intracranial pressure monitoring and decompressive craniectomy. Joint statements by the Traumatic Brain Injury Section of the Italian Society of Neurosurgery (SINch) and the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI)

Author

Iaccarino C., Lippa L., Munari M., Castioni C. A., Robba C., Caricato A., Pompucci A., Signoretti S., Zona G., Rasulo F. A., Aimar E., Amato S., Angileri F. F., Anile C., Assietti R., Baratto V., Barbanera A., Basile L., Battaglia R., Bellocchi S., Bertuccio A., Blanco S., Bolognini A., Boniferro B., Bordi L., Bortolotti C., Brandini V., Broger M., Brollo M., Caffarella D. D., Caggiano C., Cantisani P. L., Capone C., Cappelletto B., Capuano C., Carangelo B., Caruselli G., Chessa M. A., Chiara M., Chibbaro S., Cioffi V., Ciprianocecchi P., Colistra D., Conti C., Contratti F., Costella G. B., Cuoci A., D'Avella D., D'Ercole M., Deangelis M., Defalco R., de Luca G., de Marinis P., Del Vecchio C., Delfinis C., Denaro L., Deodato F., Desogus N., Disomma A., Domenicucci M., Dones F., Fina M., Fiori L., Fricia M., Gaetani P., Gazzeri R., Gentile M., Germano A., Ghadirpour R., Gianfreda C. D., Gigante N., Gigli R., Giorgetti J., Giusa M., Gravina U. G., Grippi L., Guida F., Guizzardi G., Iannuzzo G., Kropp M., Lattanzi L., Lucantoni D., Maffei L., Magliulo M., Marconi F., Marruzzo D., Martellotta N., Marton E., Maugeri R., Mauro G., Meli F., Menniti A., Merciadri P., Milanese L., Nardacci B., Nasi D., Orvieto P., Pacca P., Pansini G., Panzarasa G., Passanisi M., Pavesi G., Pizzoni C., Pulera F., Rapana A., Ricci A., Rispoli R., Rotondo M., Russo N., Santilli S., Scarano E., Schwarz A., Servadei F., Simonetti G., Stefini R., Talamonti G., Turrisi A., Valente V. M., Villa A., Vindigni M., Visocchi M., Vitali M., Wierzbicki V., Zambon G., Zanotti B., Zenga F., Alampi D., Alessandri F., Aloj F., Amigoni A., Aspide R., Bertuetti R., Betti V., Bilotta F., Bonato V., Bosco E., Brita M., Buscema G., Cafiero T., Cappuccio D., Caradonna M., Caria C. G., Casartelliliviero M., Ciritella P., Cirrincione S., Citerio G., Colelli S., Coletta F., Concordia L., Congedo E., Covotta M., Crimella F., Dall'Acqua G., De Cassai A., Defulviis S., Deperi E., Deana C., Delgaudio A., Denittis N., Dicolandrea S., Divezza F., Ferri F., Flocco R., Fontana C., Forastierimolinari A., Frangiosa A., Fumagalli P., Fuselli E., Garbarino M. M., Gelormini D., Geraci C., Geraldini F., Giacomucci A., Giampaoli V., Giorgetti D., Gritti P., Gualdani S., Iacovazzo C., Iermano C., Latronico N., Lugari S., Lusenti F., Maglione C., Magnoni S., Maiarota F., Malla M., Marchesi M., Martino C., Matteotti I., Mazzeo A. T., Morello G., Nardiello I., Paticchio F., Pegoli M., Perotti V., Piazzolla M., Picciafuochi F., Rachedi N., Radolovich D. K., Recchia A., Riccardi S., Romagnoli S., Sala S., Scafuro M. A., Sgarlata P., Soragni A., Stefani F., Stival E., Stofella G., Terranova F., Tinturini R., Togni T., Toto R., Trapani D., Tringali E., Tullo L., Valente A., Valeo T., Varelli G., Villani R., Zamacavicchi F., Zanello M., Zarrillo N., Zugni N., Iaccarino, C, Lippa, L, Munari, M, Castioni, C, Robba, C, Caricato, A, Pompucci, A, Signoretti, S, Zona, G, Rasulo, F, Aimar, E, Amato, S, Angileri, F, Anile, C, Assietti, R, Baratto, V, Barbanera, A, Basile, L, Battaglia, R, Bellocchi, S, Bertuccio, A, Blanco, S, Bolognini, A, Boniferro, B, Bordi, L, Bortolotti, C, Brandini, V, Broger, M, Brollo, M, Caffarella, D, Caggiano, C, Cantisani, P, Capone, C, Cappelletto, B, Capuano, C, Carangelo, B, Caruselli, G, Chessa, M, Chiara, M, Chibbaro, S, Cioffi, V, Ciprianocecchi, P, Colistra, D, Conti, C, Contratti, F, Costella, G, Cuoci, A, D'Avella, D, D'Ercole, M, Deangelis, M, Defalco, R, de Luca, G, de Marinis, P, Del Vecchio, C, Delfinis, C, Denaro, L, Deodato, F, Desogus, N, Disomma, A, Domenicucci, M, Dones, F, Fina, M, Fiori, L, Fricia, M, Gaetani, P, Gazzeri, R, Gentile, M, Germano, A, Ghadirpour, R, Gianfreda, C, Gigante, N, Gigli, R, Giorgetti, J, Giusa, M, Gravina, U, Grippi, L, Guida, F, Guizzardi, G, Iannuzzo, G, Kropp, M, Lattanzi, L, Lucantoni, D, Maffei, L, Magliulo, M, Marconi, F, Marruzzo, D, Martellotta, N, Marton, E, Maugeri, R, Mauro, G, Meli, F, Menniti, A, Merciadri, P, Milanese, L, Nardacci, B, Nasi, D, Orvieto, P, Pacca, P, Pansini, G, Panzarasa, G, Passanisi, M, Pavesi, G, Pizzoni, C, Pulera, F, Rapana, A, Ricci, A, Rispoli, R, Rotondo, M, Russo, N, Santilli, S, Scarano, E, Schwarz, A, Servadei, F, Simonetti, G, Stefini, R, Talamonti, G, Turrisi, A, Valente, V, Villa, A, Vindigni, M, Visocchi, M, Vitali, M, Wierzbicki, V, Zambon, G, Zanotti, B, Zenga, F, Alampi, D, Alessandri, F, Aloj, F, Amigoni, A, Aspide, R, Bertuetti, R, Betti, V, Bilotta, F, Bonato, V, Bosco, E, Brita, M, Buscema, G, Cafiero, T, Cappuccio, D, Caradonna, M, Caria, C, Casartelliliviero, M, Ciritella, P, Cirrincione, S, Citerio, G, Colelli, S, Coletta, F, Concordia, L, Congedo, E, Covotta, M, Crimella, F, Dall'Acqua, G, De Cassai, A, Defulviis, S, Deperi, E, Deana, C, Delgaudio, A, Denittis, N, Dicolandrea, S, Divezza, F, Ferri, F, Flocco, R, Fontana, C, Forastierimolinari, A, Frangiosa, A, Fumagalli, P, Fuselli, E, Garbarino, M, Gelormini, D, Geraci, C, Geraldini, F, Giacomucci, A, Giampaoli, V, Giorgetti, D, Gritti, P, Gualdani, S, Iacovazzo, C, Iermano, C, Latronico, N, Lugari, S, Lusenti, F, Maglione, C, Magnoni, S, Maiarota, F, Malla, M, Marchesi, M, Martino, C, Matteotti, I, Mazzeo, A, Morello, G, Nardiello, I, Paticchio, F, Pegoli, M, Perotti, V, Piazzolla, M, Picciafuochi, F, Rachedi, N, Radolovich, D, Recchia, A, Riccardi, S, Romagnoli, S, Sala, S, Scafuro, M, Sgarlata, P, Soragni, A, Stefani, F, Stival, E, Stofella, G, Terranova, F, Tinturini, R, Togni, T, Toto, R, Trapani, D, Tringali, E, Tullo, L, Valente, A, Valeo, T, Varelli, G, Villani, R, Zamacavicchi, F, Zanello, M, Zarrillo, N, Zugni, N, Iaccarino, C., Lippa, L., Munari, M., Castioni, C. A., Robba, C., Caricato, A., Pompucci, A., Signoretti, S., Zona, G., Rasulo, F. A., Aimar, E., Amato, S., Angileri, F. F., Anile, C., Assietti, R., Baratto, V., Barbanera, A., Basile, L., Battaglia, R., Bellocchi, S., Bertuccio, A., Blanco, S., Bolognini, A., Boniferro, B., Bordi, L., Bortolotti, C., Brandini, V., Broger, M., Brollo, M., Caffarella, D. D., Caggiano, C., Cantisani, P. L., Capone, C., Cappelletto, B., Capuano, C., Carangelo, B., Caruselli, G., Chessa, M. A., Chiara, M., Chibbaro, S., Cioffi, V., Ciprianocecchi, P., Colistra, D., Conti, C., Contratti, F., Costella, G. B., Cuoci, A., D'Avella, D., D'Ercole, M., Deangelis, M., Defalco, R., de Luca, G., de Marinis, P., Del Vecchio, C., Delfinis, C., Denaro, L., Deodato, F., Desogus, N., Disomma, A., Domenicucci, M., Dones, F., Fina, M., Fiori, L., Fricia, M., Gaetani, P., Gazzeri, R., Gentile, M., Germano, A., Ghadirpour, R., Gianfreda, C. D., Gigante, N., Gigli, R., Giorgetti, J., Giusa, M., Gravina, U. G., Grippi, L., Guida, F., Guizzardi, G., Iannuzzo, G., Kropp, M., Lattanzi, L., Lucantoni, D., Maffei, L., Magliulo, M., Marconi, F., Marruzzo, D., Martellotta, N., Marton, E., Maugeri, R., Mauro, G., Meli, F., Menniti, A., Merciadri, P., Milanese, L., Nardacci, B., Nasi, D., Orvieto, P., Pacca, P., Pansini, G., Panzarasa, G., Passanisi, M., Pavesi, G., Pizzoni, C., Pulera, F., Rapana, A., Ricci, A., Rispoli, R., Rotondo, M., Russo, N., Santilli, S., Scarano, E., Schwarz, A., Servadei, F., Simonetti, G., Stefini, R., Talamonti, G., Turrisi, A., Valente, V. M., Villa, A., Vindigni, M., Visocchi, M., Vitali, M., Wierzbicki, V., Zambon, G., Zanotti, B., Zenga, F., Alampi, D., Alessandri, F., Aloj, F., Amigoni, A., Aspide, R., Bertuetti, R., Betti, V., Bilotta, F., Bonato, V., Bosco, E., Brita, M., Buscema, G., Cafiero, T., Cappuccio, D., Caradonna, M., Caria, C. G., Casartelliliviero, M., Ciritella, P., Cirrincione, S., Citerio, G., Colelli, S., Coletta, F., Concordia, L., Congedo, E., Covotta, M., Crimella, F., Dall'Acqua, G., De Cassai, A., Defulviis, S., Deperi, E., Deana, C., Delgaudio, A., Denittis, N., Dicolandrea, S., Divezza, F., Ferri, F., Flocco, R., Fontana, C., Forastierimolinari, A., Frangiosa, A., Fumagalli, P., Fuselli, E., Garbarino, M. M., Gelormini, D., Geraci, C., Geraldini, F., Giacomucci, A., Giampaoli, V., Giorgetti, D., Gritti, P., Gualdani, S., Iacovazzo, C., Iermano, C., Latronico, N., Lugari, S., Lusenti, F., Maglione, C., Magnoni, S., Maiarota, F., Malla, M., Marchesi, M., Martino, C., Matteotti, I., Mazzeo, A. T., Morello, G., Nardiello, I., Paticchio, F., Pegoli, M., Perotti, V., Piazzolla, M., Picciafuochi, F., Rachedi, N., Radolovich, D. K., Recchia, A., Riccardi, S., Romagnoli, S., Sala, S., Scafuro, M. A., Sgarlata, P., Soragni, A., Stefani, F., Stival, E., Stofella, G., Terranova, F., Tinturini, R., Togni, T., Toto, R., Trapani, D., Tringali, E., Tullo, L., Valente, A., Valeo, T., Varelli, G., Villani, R., Zamacavicchi, F., Zanello, M., Zarrillo, N., and Zugni, N.

Subjects
medicine.medical_specialty, Decompressive Craniectomy, Consensus development conference, Decompressive craniectomy, Guideline, Traumatic brain injuries, Critical Care, Intracranial Pressure, medicine.medical_treatment, MEDLINE, Neurosurgery, Traumatic brain injurie, law.invention, 03 medical and health sciences, 0302 clinical medicine, Randomized controlled trial, law, Intensive care, Settore MED/41 - ANESTESIOLOGIA, Brain Injuries, Traumatic, medicine, Humans, Anesthesia, Intensive care medicine, Randomized Controlled Trials as Topic, business.industry, Neurointensive care, Evidence-based medicine, Italy, 030220 oncology & carcinogenesis, Intracranial pressure monitoring, Surgery, Neurology (clinical), Analgesia, Intracranial Hypertension, business, 030217 neurology & neurosurgery, Human
Abstract

No robust evidence is provided by literature regarding the management of intracranial hypertension following severe traumatic brain injury (TBI). This is mostly due to the lack of prospective randomized controlled trials (RCTs), the presence of studies containing extreme heterogeneously collected populations and controversial considerations about chosen outcome. A scientific society should provide guidelines for care management and scientific support for those areas for which evidence-based medicine has not been identified. However, RCTs in severe TBI have failed to establish intervention effectiveness, arising the need to make greater use of tools such as Consensus Conferences between experts, which have the advantage of providing recommendations based on experience, on the analysis of updated literature data and on the direct comparison of different logistic realities. The Italian scientific societies should provide guidelines following the national laws ruling the best medical practice. However, many limitations do not allow the collection of data supporting high levels of evidence for intracranial pressure (ICP) monitoring and decompressive craniectomy (DC) in patients with severe TBI. This intersociety document proposes best practice guidelines for this subsetting of patients to be adopted on a national Italian level, along with joint statements from "TBI Section" of the Italian Society of Neurosurgery (SINch) endorsed by the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI). Presented here is a recap of recommendations on management of ICP and DC supported a high level of available evidence and rate of agreement expressed by the assemblies during the more recent consensus conferences, where members of both groups have had a role of active participants and supporters. The listed recommendations have been sent to a panel of experts consisting of the 107 members of the "TBI Section" of the SINch and the 111 members of the Neuroanesthesia and Neurocritical Care Study Group of the SIAARTI. The aim of the survey was to test a preliminary evaluation of the grade of predictable future adherence of the recommendations following this intersociety proposal. The following recommendations are suggested as representing best clinical practice, nevertheless, adoption of local multidisciplinary protocols regarding thresholds of ICP values, drug therapies, hemostasis management and perioperative care of decompressed patients is strongly recommended to improve treatment efficiency, to increase the quality of data collection and to provide more powerful evidence with future studies. Thus, for this future perspective a rapid overview of the role of the multimodal neuromonitoring in the optimal severe TBI management is also provided in this document. It is reasonable to assume that the recommendations reported in this paper will in future be updated by new observations arising from future trials. They are not binding, and this document should be offered as a guidance for clinical practice through an intersociety agreement, taking in consideration the low level of evidence.

Published
2021

37. Administration of palmitoylethanolamide in combination with topiramate in the preventive treatment of nummular headache

Author

Chirchiglia D, Della Torre A, Signorelli F, Volpentesta G, Guzzi G, Stroscio CA, Deodato F, Gabriele D, and Lavano A

Subjects
Algometry, Migraine, Nummular headache, palmitoylethanolamide, topiramate, Medicine (General), R5-920
Abstract

Domenico Chirchiglia,1 Attilio Della Torre,2 Francesco Signorelli,2 Giorgio Volpentesta,2 Giusy Guzzi,2 Carmelino Angelo Stroscio,2 Federica Deodato,2 Donatella Gabriele,2 Angelo Lavano,2 1Department of Neurosurgery, Neurophysiopathology Unit, 2Department of Neurosurgery, University of Catanzaro “Magna Graecia”, Catanzaro, Italy Abstract: Nummular headache has been recently described as a primary disorder characterized by head pain exclusively felt in a small rounded area typically 2–6 cm in diameter, not attributed to another disorder. Both size and shape of the painful area remain constant since the onset of symptoms. A 57-year-old woman presented with a history of focal episodic pain in a circumscribed area on the right parietal region. The administration of standard oral doses of palmitoylethanolamide and topiramate in combination showed an improvement in pain symptoms and on pain measuring scales. Keywords: algometry, migraine, nummular headache, palmitoylethanolamide, topiramate

Published
2016

38. Correction to: Hypofractionated sequential radiotherapy boost: a promising strategy in inoperable locally advanced pancreatic cancer patients (Journal of Cancer Research and Clinical Oncology, (2021), 147, 3, (661-667), 10.1007/s00432-020-03411-7)

Author

Mattiucci, G. C., Boldrini, L., Nardangeli, A., D'Aviero, A., Buwenge, M., Cellini, F., Deodato, F., Dinapoli, N., Frascino, V., Macchia, G., Morganti, A. G., Valentini, V., Mattiucci G. C. (ORCID:0000-0001-6500-0413), Boldrini L., Cellini F. (ORCID:0000-0002-2145-2300), Deodato F. (ORCID:0000-0003-1276-5070), Dinapoli N., Frascino V., Macchia G., Morganti A. G., Valentini V. (ORCID:0000-0003-4637-6487), Mattiucci, G. C., Boldrini, L., Nardangeli, A., D'Aviero, A., Buwenge, M., Cellini, F., Deodato, F., Dinapoli, N., Frascino, V., Macchia, G., Morganti, A. G., Valentini, V., Mattiucci G. C. (ORCID:0000-0001-6500-0413), Boldrini L., Cellini F. (ORCID:0000-0002-2145-2300), Deodato F. (ORCID:0000-0003-1276-5070), Dinapoli N., Frascino V., Macchia G., Morganti A. G., and Valentini V. (ORCID:0000-0003-4637-6487)

Abstract

In the original article published, the last sentence in the sixth paragraph of the introduction section is incorrect. The correct sentence is “This data, were confirmed also in an Italian phase II study, in which it has been demonstrated that gemcitabine-based chemoradiotherapy was correlated with improved overall survival, especially in pts who are clinically more fit to complete the foreseen treatment schedule (CRT) (Mattiucci et al. 2010)”. The original article has been corrected.

Published
2021

39. Systematic review of stereotactic body radiotherapy for nodal metastases

Author

Deodato, F., Macchia, G., Buwenge, M., Bonetti, M., Cilla, S., Zamagni, A., Re, A., Pezzulla, D., Cellini, F., Strigari, L., Valentini, V., Morganti, A. G., Deodato F. (ORCID:0000-0003-1276-5070), Macchia G., Cilla S., Cellini F. (ORCID:0000-0002-2145-2300), Valentini V. (ORCID:0000-0003-4637-6487), Morganti A. G., Deodato, F., Macchia, G., Buwenge, M., Bonetti, M., Cilla, S., Zamagni, A., Re, A., Pezzulla, D., Cellini, F., Strigari, L., Valentini, V., Morganti, A. G., Deodato F. (ORCID:0000-0003-1276-5070), Macchia G., Cilla S., Cellini F. (ORCID:0000-0002-2145-2300), Valentini V. (ORCID:0000-0003-4637-6487), and Morganti A. G.

Abstract

The aim of this analysis was to assess the efficacy of stereotactic body radiotherapy (SBRT) in terms of local control (LC) and progression-free survival (PFS) in patients with lymph node metastases (NMs) from solid tumors. A systematic literature search from the earliest date to July 25th, 2019 was performed following PRISMA guidelines. Papers reporting LC and/or PFS of NMs using SBRT (< 10 fractions) were selected. The clinical outcomes rates were pooled by means of a random or fixed-effect model. Twenty-nine studies were eligible (969 patients: 938 (LC) and 698 (PFS)). LC and PFS results were reported in 28 and 18 papers, respectively. Heterogeneity was observed in terms of patient and treatment characteristics. Pooled 2-year LC reported in 11 studies was 79.3% (95%CI, 72.8%–85.7%) with substantial heterogeneity between studies (Q2 test: p = 0.0083; I2 = 57.9%), while pooled 2-year PFS reported in 8 studies was 35.9% (95%CI, 22.1%–49.7%) with very high heterogeneity between studies (Q2 test: p < 0.0001; I2 = 86.1%). Grade ≥ 3 and Grade 5 toxicity rates were 2.0% and 0.2%, respectively. SBRT of NMs seems to be safe and effective in terms of LC. However, due to the marked heterogeneity of the included series, prospective studies are required.

Published
2021

40. Management of intracranial hypertension following traumatic brain injury: A best clinical practice adoption proposal for intracranial pressure monitoring and decompressive craniectomy: Joint statements by the Traumatic Brain Injury Section of the Italian Society of Neurosurgery (SINch) and the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI)

Author

Iaccarino, C, Lippa, L, Munari, M, Castioni, C, Robba, C, Caricato, A, Pompucci, A, Signoretti, S, Zona, G, Rasulo, F, Aimar, E, Amato, S, Angileri, F, Anile, C, Assietti, R, Baratto, V, Barbanera, A, Basile, L, Battaglia, R, Bellocchi, S, Bertuccio, A, Blanco, S, Bolognini, A, Boniferro, B, Bordi, L, Bortolotti, C, Brandini, V, Broger, M, Brollo, M, Caffarella, D, Caggiano, C, Cantisani, P, Capone, C, Cappelletto, B, Capuano, C, Carangelo, B, Caruselli, G, Chessa, M, Chiara, M, Chibbaro, S, Cioffi, V, Ciprianocecchi, P, Colistra, D, Conti, C, Contratti, F, Costella, G, Cuoci, A, D'Avella, D, D'Ercole, M, Deangelis, M, Defalco, R, de Luca, G, de Marinis, P, Del Vecchio, C, Delfinis, C, Denaro, L, Deodato, F, Desogus, N, Disomma, A, Domenicucci, M, Dones, F, Fina, M, Fiori, L, Fricia, M, Gaetani, P, Gazzeri, R, Gentile, M, Germano, A, Ghadirpour, R, Gianfreda, C, Gigante, N, Gigli, R, Giorgetti, J, Giusa, M, Gravina, U, Grippi, L, Guida, F, Guizzardi, G, Iannuzzo, G, Kropp, M, Lattanzi, L, Lucantoni, D, Maffei, L, Magliulo, M, Marconi, F, Marruzzo, D, Martellotta, N, Marton, E, Maugeri, R, Mauro, G, Meli, F, Menniti, A, Merciadri, P, Milanese, L, Nardacci, B, Nasi, D, Orvieto, P, Pacca, P, Pansini, G, Panzarasa, G, Passanisi, M, Pavesi, G, Pizzoni, C, Pulera, F, Rapana, A, Ricci, A, Rispoli, R, Rotondo, M, Russo, N, Santilli, S, Scarano, E, Schwarz, A, Servadei, F, Simonetti, G, Stefini, R, Talamonti, G, Turrisi, A, Valente, V, Villa, A, Vindigni, M, Visocchi, M, Vitali, M, Wierzbicki, V, Zambon, G, Zanotti, B, Zenga, F, Alampi, D, Alessandri, F, Aloj, F, Amigoni, A, Aspide, R, Bertuetti, R, Betti, V, Bilotta, F, Bonato, V, Bosco, E, Brita, M, Buscema, G, Cafiero, T, Cappuccio, D, Caradonna, M, Caria, C, Casartelliliviero, M, Ciritella, P, Cirrincione, S, Citerio, G, Colelli, S, Coletta, F, Concordia, L, Congedo, E, Covotta, M, Crimella, F, Dall'Acqua, G, De Cassai, A, Defulviis, S, Deperi, E, Deana, C, Delgaudio, A, Denittis, N, Dicolandrea, S, Divezza, F, Ferri, F, Flocco, R, Fontana, C, Forastierimolinari, A, Frangiosa, A, Fumagalli, P, Fuselli, E, Garbarino, M, Gelormini, D, Geraci, C, Geraldini, F, Giacomucci, A, Giampaoli, V, Giorgetti, D, Gritti, P, Gualdani, S, Iacovazzo, C, Iermano, C, Latronico, N, Lugari, S, Lusenti, F, Maglione, C, Magnoni, S, Maiarota, F, Malla, M, Marchesi, M, Martino, C, Matteotti, I, Mazzeo, A, Morello, G, Nardiello, I, Paticchio, F, Pegoli, M, Perotti, V, Piazzolla, M, Picciafuochi, F, Rachedi, N, Radolovich, D, Recchia, A, Riccardi, S, Romagnoli, S, Sala, S, Scafuro, M, Sgarlata, P, Soragni, A, Stefani, F, Stival, E, Stofella, G, Terranova, F, Tinturini, R, Togni, T, Toto, R, Trapani, D, Tringali, E, Tullo, L, Valente, A, Valeo, T, Varelli, G, Villani, R, Zamacavicchi, F, Zanello, M, Zarrillo, N, Zugni, N, Iaccarino C., Lippa L., Munari M., Castioni C. A., Robba C., Caricato A., Pompucci A., Signoretti S., Zona G., Rasulo F. A., Aimar E., Amato S., Angileri F. F., Anile C., Assietti R., Baratto V., Barbanera A., Basile L., Battaglia R., Bellocchi S., Bertuccio A., Blanco S., Bolognini A., Boniferro B., Bordi L., Bortolotti C., Brandini V., Broger M., Brollo M., Caffarella D. D., Caggiano C., Cantisani P. L., Capone C., Cappelletto B., Capuano C., Carangelo B., Caruselli G., Chessa M. A., Chiara M., Chibbaro S., Cioffi V., Ciprianocecchi P., Colistra D., Conti C., Contratti F., Costella G. B., Cuoci A., D'Avella D., D'Ercole M., Deangelis M., Defalco R., de Luca G., de Marinis P., Del Vecchio C., Delfinis C., Denaro L., Deodato F., Desogus N., Disomma A., Domenicucci M., Dones F., Fina M., Fiori L., Fricia M., Gaetani P., Gazzeri R., Gentile M., Germano A., Ghadirpour R., Gianfreda C. D., Gigante N., Gigli R., Giorgetti J., Giusa M., Gravina U. G., Grippi L., Guida F., Guizzardi G., Iannuzzo G., Kropp M., Lattanzi L., Lucantoni D., Maffei L., Magliulo M., Marconi F., Marruzzo D., Martellotta N., Marton E., Maugeri R., Mauro G., Meli F., Menniti A., Merciadri P., Milanese L., Nardacci B., Nasi D., Orvieto P., Pacca P., Pansini G., Panzarasa G., Passanisi M., Pavesi G., Pizzoni C., Pulera F., Rapana A., Ricci A., Rispoli R., Rotondo M., Russo N., Santilli S., Scarano E., Schwarz A., Servadei F., Simonetti G., Stefini R., Talamonti G., Turrisi A., Valente V. M., Villa A., Vindigni M., Visocchi M., Vitali M., Wierzbicki V., Zambon G., Zanotti B., Zenga F., Alampi D., Alessandri F., Aloj F., Amigoni A., Aspide R., Bertuetti R., Betti V., Bilotta F., Bonato V., Bosco E., Brita M., Buscema G., Cafiero T., Cappuccio D., Caradonna M., Caria C. G., Casartelliliviero M., Ciritella P., Cirrincione S., Citerio G., Colelli S., Coletta F., Concordia L., Congedo E., Covotta M., Crimella F., Dall'Acqua G., De Cassai A., Defulviis S., Deperi E., Deana C., Delgaudio A., Denittis N., Dicolandrea S., Divezza F., Ferri F., Flocco R., Fontana C., Forastierimolinari A., Frangiosa A., Fumagalli P., Fuselli E., Garbarino M. M., Gelormini D., Geraci C., Geraldini F., Giacomucci A., Giampaoli V., Giorgetti D., Gritti P., Gualdani S., Iacovazzo C., Iermano C., Latronico N., Lugari S., Lusenti F., Maglione C., Magnoni S., Maiarota F., Malla M., Marchesi M., Martino C., Matteotti I., Mazzeo A. T., Morello G., Nardiello I., Paticchio F., Pegoli M., Perotti V., Piazzolla M., Picciafuochi F., Rachedi N., Radolovich D. K., Recchia A., Riccardi S., Romagnoli S., Sala S., Scafuro M. A., Sgarlata P., Soragni A., Stefani F., Stival E., Stofella G., Terranova F., Tinturini R., Togni T., Toto R., Trapani D., Tringali E., Tullo L., Valente A., Valeo T., Varelli G., Villani R., Zamacavicchi F., Zanello M., Zarrillo N., Zugni N., Iaccarino, C, Lippa, L, Munari, M, Castioni, C, Robba, C, Caricato, A, Pompucci, A, Signoretti, S, Zona, G, Rasulo, F, Aimar, E, Amato, S, Angileri, F, Anile, C, Assietti, R, Baratto, V, Barbanera, A, Basile, L, Battaglia, R, Bellocchi, S, Bertuccio, A, Blanco, S, Bolognini, A, Boniferro, B, Bordi, L, Bortolotti, C, Brandini, V, Broger, M, Brollo, M, Caffarella, D, Caggiano, C, Cantisani, P, Capone, C, Cappelletto, B, Capuano, C, Carangelo, B, Caruselli, G, Chessa, M, Chiara, M, Chibbaro, S, Cioffi, V, Ciprianocecchi, P, Colistra, D, Conti, C, Contratti, F, Costella, G, Cuoci, A, D'Avella, D, D'Ercole, M, Deangelis, M, Defalco, R, de Luca, G, de Marinis, P, Del Vecchio, C, Delfinis, C, Denaro, L, Deodato, F, Desogus, N, Disomma, A, Domenicucci, M, Dones, F, Fina, M, Fiori, L, Fricia, M, Gaetani, P, Gazzeri, R, Gentile, M, Germano, A, Ghadirpour, R, Gianfreda, C, Gigante, N, Gigli, R, Giorgetti, J, Giusa, M, Gravina, U, Grippi, L, Guida, F, Guizzardi, G, Iannuzzo, G, Kropp, M, Lattanzi, L, Lucantoni, D, Maffei, L, Magliulo, M, Marconi, F, Marruzzo, D, Martellotta, N, Marton, E, Maugeri, R, Mauro, G, Meli, F, Menniti, A, Merciadri, P, Milanese, L, Nardacci, B, Nasi, D, Orvieto, P, Pacca, P, Pansini, G, Panzarasa, G, Passanisi, M, Pavesi, G, Pizzoni, C, Pulera, F, Rapana, A, Ricci, A, Rispoli, R, Rotondo, M, Russo, N, Santilli, S, Scarano, E, Schwarz, A, Servadei, F, Simonetti, G, Stefini, R, Talamonti, G, Turrisi, A, Valente, V, Villa, A, Vindigni, M, Visocchi, M, Vitali, M, Wierzbicki, V, Zambon, G, Zanotti, B, Zenga, F, Alampi, D, Alessandri, F, Aloj, F, Amigoni, A, Aspide, R, Bertuetti, R, Betti, V, Bilotta, F, Bonato, V, Bosco, E, Brita, M, Buscema, G, Cafiero, T, Cappuccio, D, Caradonna, M, Caria, C, Casartelliliviero, M, Ciritella, P, Cirrincione, S, Citerio, G, Colelli, S, Coletta, F, Concordia, L, Congedo, E, Covotta, M, Crimella, F, Dall'Acqua, G, De Cassai, A, Defulviis, S, Deperi, E, Deana, C, Delgaudio, A, Denittis, N, Dicolandrea, S, Divezza, F, Ferri, F, Flocco, R, Fontana, C, Forastierimolinari, A, Frangiosa, A, Fumagalli, P, Fuselli, E, Garbarino, M, Gelormini, D, Geraci, C, Geraldini, F, Giacomucci, A, Giampaoli, V, Giorgetti, D, Gritti, P, Gualdani, S, Iacovazzo, C, Iermano, C, Latronico, N, Lugari, S, Lusenti, F, Maglione, C, Magnoni, S, Maiarota, F, Malla, M, Marchesi, M, Martino, C, Matteotti, I, Mazzeo, A, Morello, G, Nardiello, I, Paticchio, F, Pegoli, M, Perotti, V, Piazzolla, M, Picciafuochi, F, Rachedi, N, Radolovich, D, Recchia, A, Riccardi, S, Romagnoli, S, Sala, S, Scafuro, M, Sgarlata, P, Soragni, A, Stefani, F, Stival, E, Stofella, G, Terranova, F, Tinturini, R, Togni, T, Toto, R, Trapani, D, Tringali, E, Tullo, L, Valente, A, Valeo, T, Varelli, G, Villani, R, Zamacavicchi, F, Zanello, M, Zarrillo, N, Zugni, N, Iaccarino C., Lippa L., Munari M., Castioni C. A., Robba C., Caricato A., Pompucci A., Signoretti S., Zona G., Rasulo F. A., Aimar E., Amato S., Angileri F. F., Anile C., Assietti R., Baratto V., Barbanera A., Basile L., Battaglia R., Bellocchi S., Bertuccio A., Blanco S., Bolognini A., Boniferro B., Bordi L., Bortolotti C., Brandini V., Broger M., Brollo M., Caffarella D. D., Caggiano C., Cantisani P. L., Capone C., Cappelletto B., Capuano C., Carangelo B., Caruselli G., Chessa M. A., Chiara M., Chibbaro S., Cioffi V., Ciprianocecchi P., Colistra D., Conti C., Contratti F., Costella G. B., Cuoci A., D'Avella D., D'Ercole M., Deangelis M., Defalco R., de Luca G., de Marinis P., Del Vecchio C., Delfinis C., Denaro L., Deodato F., Desogus N., Disomma A., Domenicucci M., Dones F., Fina M., Fiori L., Fricia M., Gaetani P., Gazzeri R., Gentile M., Germano A., Ghadirpour R., Gianfreda C. D., Gigante N., Gigli R., Giorgetti J., Giusa M., Gravina U. G., Grippi L., Guida F., Guizzardi G., Iannuzzo G., Kropp M., Lattanzi L., Lucantoni D., Maffei L., Magliulo M., Marconi F., Marruzzo D., Martellotta N., Marton E., Maugeri R., Mauro G., Meli F., Menniti A., Merciadri P., Milanese L., Nardacci B., Nasi D., Orvieto P., Pacca P., Pansini G., Panzarasa G., Passanisi M., Pavesi G., Pizzoni C., Pulera F., Rapana A., Ricci A., Rispoli R., Rotondo M., Russo N., Santilli S., Scarano E., Schwarz A., Servadei F., Simonetti G., Stefini R., Talamonti G., Turrisi A., Valente V. M., Villa A., Vindigni M., Visocchi M., Vitali M., Wierzbicki V., Zambon G., Zanotti B., Zenga F., Alampi D., Alessandri F., Aloj F., Amigoni A., Aspide R., Bertuetti R., Betti V., Bilotta F., Bonato V., Bosco E., Brita M., Buscema G., Cafiero T., Cappuccio D., Caradonna M., Caria C. G., Casartelliliviero M., Ciritella P., Cirrincione S., Citerio G., Colelli S., Coletta F., Concordia L., Congedo E., Covotta M., Crimella F., Dall'Acqua G., De Cassai A., Defulviis S., Deperi E., Deana C., Delgaudio A., Denittis N., Dicolandrea S., Divezza F., Ferri F., Flocco R., Fontana C., Forastierimolinari A., Frangiosa A., Fumagalli P., Fuselli E., Garbarino M. M., Gelormini D., Geraci C., Geraldini F., Giacomucci A., Giampaoli V., Giorgetti D., Gritti P., Gualdani S., Iacovazzo C., Iermano C., Latronico N., Lugari S., Lusenti F., Maglione C., Magnoni S., Maiarota F., Malla M., Marchesi M., Martino C., Matteotti I., Mazzeo A. T., Morello G., Nardiello I., Paticchio F., Pegoli M., Perotti V., Piazzolla M., Picciafuochi F., Rachedi N., Radolovich D. K., Recchia A., Riccardi S., Romagnoli S., Sala S., Scafuro M. A., Sgarlata P., Soragni A., Stefani F., Stival E., Stofella G., Terranova F., Tinturini R., Togni T., Toto R., Trapani D., Tringali E., Tullo L., Valente A., Valeo T., Varelli G., Villani R., Zamacavicchi F., Zanello M., Zarrillo N., and Zugni N.

Abstract

No robust evidence is provided by literature regarding the management of intracranial hypertension following severe traumatic brain injury (TBI). This is mostly due to the lack of prospective randomized controlled trials (RCTs), the presence of studies containing extreme heterogeneously collected populations and controversial considerations about chosen outcome. A scientific society should provide guidelines for care management and scientific support for those areas for which evidence-based medicine has not been identified. However, RCTs in severe TBI have failed to establish intervention effectiveness, arising the need to make greater use of tools such as Consensus Conferences between experts, which have the advantage of providing recommendations based on experience, on the analysis of updated literature data and on the direct comparison of different logistic realities. The Italian scientific societies should provide guidelines following the national laws ruling the best medical practice. However, many limitations do not allow the collection of data supporting high levels of evidence for intracranial pressure (ICP) monitoring and decompressive craniectomy (DC) in patients with severe TBI. This intersociety document proposes best practice guidelines for this subsetting of patients to be adopted on a national Italian level, along with joint statements from “TBI Section” of the Italian Society of Neurosurgery (SINch) endorsed by the Neuroanesthesia and Neurocritical Care Study Group of the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care (SIAARTI). Presented here is a recap of recommendations on management of ICP and DC supported a high level of available evidence and rate of agreement expressed by the assemblies during the more recent consensus conferences, where members of both groups have had a role of active participants and supporters. The listed recommendations have been sent to a panel of experts consisting of the 107 members of the “T

Published
2021

41. The Role of Radiotherapy in Orbital Pseudotumor: A Systematic Review of Literature

Author

Fionda, B., Pagliara, M. M., Lancellotta, V., Sammarco, M. G., Casa, C., Savino, G., Deodato, F., Morganti, A. G., Gambacorta, M. A., Tagliaferri, L., Blasi, M. A., Pagliara M. M., Savino G. (ORCID:0000-0002-9993-5986), Deodato F. (ORCID:0000-0003-1276-5070), Morganti A. G., Gambacorta M. A. (ORCID:0000-0001-5455-8737), Tagliaferri L. (ORCID:0000-0003-2308-0982), Blasi M. A. (ORCID:0000-0001-7393-7644), Fionda, B., Pagliara, M. M., Lancellotta, V., Sammarco, M. G., Casa, C., Savino, G., Deodato, F., Morganti, A. G., Gambacorta, M. A., Tagliaferri, L., Blasi, M. A., Pagliara M. M., Savino G. (ORCID:0000-0002-9993-5986), Deodato F. (ORCID:0000-0003-1276-5070), Morganti A. G., Gambacorta M. A. (ORCID:0000-0001-5455-8737), Tagliaferri L. (ORCID:0000-0003-2308-0982), and Blasi M. A. (ORCID:0000-0001-7393-7644)

Abstract

Purpose: Orbital pseudotumor (or pseudotumor orbitae) is a benign entity, also known as idiopathic orbital inflammatory syndrome (IOIS), which encompasses a wide range of non-tumoral, space occupying lesions of the orbit. In selected cases of refractory disease or presence of side effects or even comorbidities that limit systemic therapies, radiotherapy could play a role in the management because it has been demonstrated that irradiation is effective in suppressing the inflammatory process. Methods: A systematic review of the literature about the main scientific databases was launched and the time interval included all published articles present in the databases from their inception until September 2020. Results: We were able to identify 19 studies eligible for inclusion in this review from 1978 to 2018. Overall the data of 241 patients were collected and are presented in this systematic review. The response rate varied between 74% and 100% with a median recurrence rate of 10%. The median total dose was 20 Gy whereas the mean total dose was 21 Gy with a range from 4 to 36 Gy. Regarding the fractionation, 2 Gy/fraction daily was the most widely used. Conclusions: Radiotherapy seemed to achieve good response rates however, in most of the studies, inclusion criteria and outcome parameters are not uniform and therefore the results are difficult to compare. Often important parameters such as chronic pain and permanent functional deficits are not assessed in the outcome. Therefore, prospective studies, with good cohort characteristics and a clear definition of the outcome, are required.

Published
2021

43. PO-1703 Reproducibility and stability of spirometer-guided DIBH in left-breast radiotherapy

Author

Viola, P., primary, Craus, M., additional, Romano, C., additional, Macchia, G., additional, Boccardi, M., additional, Ferro, M., additional, Picardi, V., additional, Pezzulla, D., additional, Indovina, L., additional, Buwenge, M., additional, Cammelli, S., additional, Valentini, V., additional, Morganti, A.G., additional, Deodato, F., additional, and Cilla, S., additional

Published
2022
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46. A “SHort course Accelerated RadiatiON therapy” (SHARON) During and Beyond the COVID-19 Pandemic

Author

Morganti, Alessio Giuseppe, Macchia, Gabriella, Cellini, Francesco, Deodato, Francesco, Zamagni, A., Siepe, G., Buwenge, M., Morganti A. G., Macchia G., Cellini F. (ORCID:0000-0002-2145-2300), Deodato F. (ORCID:0000-0003-1276-5070), Morganti, Alessio Giuseppe, Macchia, Gabriella, Cellini, Francesco, Deodato, Francesco, Zamagni, A., Siepe, G., Buwenge, M., Morganti A. G., Macchia G., Cellini F. (ORCID:0000-0002-2145-2300), and Deodato F. (ORCID:0000-0003-1276-5070)

Abstract

N/A

Published
2022

47. Personalized Automation of Treatment Planning for Linac-Based Stereotactic Body Radiotherapy of Spine Cancer

Author

Cilla, Savino, Cellini, Francesco, Romano, C., Macchia, Gabriella, Pezzulla, D., Viola, P., Buwenge, M., Indovina, Luca, Valentini, Vincenzo, Morganti, Alessio Giuseppe, Deodato, Francesco, Cilla S., Cellini F. (ORCID:0000-0002-2145-2300), Macchia G., Indovina L., Valentini V. (ORCID:0000-0003-4637-6487), Morganti A. G., Deodato F. (ORCID:0000-0003-1276-5070), Cilla, Savino, Cellini, Francesco, Romano, C., Macchia, Gabriella, Pezzulla, D., Viola, P., Buwenge, M., Indovina, Luca, Valentini, Vincenzo, Morganti, Alessio Giuseppe, Deodato, Francesco, Cilla S., Cellini F. (ORCID:0000-0002-2145-2300), Macchia G., Indovina L., Valentini V. (ORCID:0000-0003-4637-6487), Morganti A. G., and Deodato F. (ORCID:0000-0003-1276-5070)

Abstract

Purpose/Objective(s): Stereotactic ablative body radiotherapy (SBRT) for vertebral metastases is a challenging treatment process. Planning automation has recently reported the potential to improve plan quality and increase planning efficiency. We performed a dosimetric evaluation of the new Personalized engine implemented in Pinnacle3 for full planning automation of SBRT spine treatments in terms of plan quality, treatment efficiency, and delivery accuracy. Materials/Methods: The Pinnacle3 treatment planning system was used to reoptimize six patients with spinal metastases, employing two separate automated engines. These two automated engines, the existing Autoplanning and the new Personalized, are both template-based algorithms that employ a wishlist to construct planning goals and an iterative technique to replicate the planning procedure performed by skilled planners. The boost tumor volume (BTV) was defined as the macroscopically visible lesion on RM examination, and the planning target volume (PTV) corresponds with the entire vertebra. Dose was prescribed according to simultaneous integrated boost strategy with BTV and PTV irradiated simultaneously over 3 fractions with a dose of 30 and 21 Gy, respectively. Dose-volume histogram (DVH) metrics and conformance indices were used to compare clinically accepted manual plans (MP) with automated plans developed using both Autoplanning (AP) and Personalized engines (Pers). All plans were evaluated for planning efficiency and dose delivery accuracy. Results: For similar spinal cord sparing, automated plans reported a significant improvement of target coverage and dose conformity. On average, Pers plans increased near-minimal dose D98% by 10.4% and 8.9% and target coverage D95% by 8.0% and by 4.6% for BTV and PTV, respectively. Automated plans provided significantly superior dose conformity and dose contrast by 37%–47% and by 4.6%–5.7% compared with manual plans. Overall planning times were dramatically reduced to about

Published
2022

48. Proposal of an Algorithm to Early Detect Attenuated Type I Mucopolysaccharidosis (MPS Ia) among Children with Growth Abnormalities

Author

Baronio, F, Zucchini, S, Zulian, F, Salerno, M, Parini, R, Cattoni, A, Deodato, F, Gaeta, A, Bizzarri, C, Gasperini, S, Pession, A, Baronio, Federico, Zucchini, Stefano, Zulian, Francesco, Salerno, Mariacarolina, Parini, Rossella, Cattoni, Alessandro, Deodato, Federica, Gaeta, Alberto, Bizzarri, Carla, Gasperini, Serena, Pession, Andrea, Baronio, F, Zucchini, S, Zulian, F, Salerno, M, Parini, R, Cattoni, A, Deodato, F, Gaeta, A, Bizzarri, C, Gasperini, S, Pession, A, Baronio, Federico, Zucchini, Stefano, Zulian, Francesco, Salerno, Mariacarolina, Parini, Rossella, Cattoni, Alessandro, Deodato, Federica, Gaeta, Alberto, Bizzarri, Carla, Gasperini, Serena, and Pession, Andrea

Abstract

Background and Objectives: Diagnostic delay is common in attenuated Mucopolysaccharidosis Type I (MPS Ia) due to the rarity of the disease and the variability of clinical presentation. Short stature and impaired growth velocity are frequent findings in MPS Ia, but they rarely raise suspicion as paediatric endocrinologists are generally poorly trained to detect earlier and milder clinical signs of this condition. Materials and Methods: Following a consensus-based methodology, a multidisciplinary panel including paediatric endocrinologists, paediatricians with expertise in metabolic disorders, radiologists, and rheumatologists shared their experience on a possible clinical approach to the diagnosis of MPS Ia in children with short stature or stunted growth. Results: The result was the formation of an algorithm that illustrates how to raise the suspicion of MPS Ia in a patient older than 5 years with short stature and suggestive clinical signs. Conclusion: The proposed algorithm may represent a useful tool to improve the awareness of paediatric endocrinologists and reduce the diagnostic delay for patients with MPS Ia.

Published
2022

49. Molecular genetics of niemann–pick type c disease in italy: An update on 105 patients and description of 18 NPC1 novel variants

Author

Dardis, A, Zampieri, S, Gellera, C, Carrozzo, R, Cattarossi, S, Peruzzo, P, Dariol, R, Sechi, A, Deodato, F, Caccia, C, Verrigni, D, Gasperini, S, Fiumara, A, Fecarotta, S, Carecchio, M, Filosto, M, Santoro, L, Borroni, B, Bordugo, A, Brancati, F, Russo, C, Di Rocco, M, Toscano, A, Scarpa, M, Bembi, B, Dardis A., Zampieri S., Gellera C., Carrozzo R., Cattarossi S., Peruzzo P., Dariol R., Sechi A., Deodato F., Caccia C., Verrigni D., Gasperini S., Fiumara A., Fecarotta S., Carecchio M., Filosto M., Santoro L., Borroni B., Bordugo A., Brancati F., Russo C. V., Di Rocco M., Toscano A., Scarpa M., Bembi B., Dardis, A, Zampieri, S, Gellera, C, Carrozzo, R, Cattarossi, S, Peruzzo, P, Dariol, R, Sechi, A, Deodato, F, Caccia, C, Verrigni, D, Gasperini, S, Fiumara, A, Fecarotta, S, Carecchio, M, Filosto, M, Santoro, L, Borroni, B, Bordugo, A, Brancati, F, Russo, C, Di Rocco, M, Toscano, A, Scarpa, M, Bembi, B, Dardis A., Zampieri S., Gellera C., Carrozzo R., Cattarossi S., Peruzzo P., Dariol R., Sechi A., Deodato F., Caccia C., Verrigni D., Gasperini S., Fiumara A., Fecarotta S., Carecchio M., Filosto M., Santoro L., Borroni B., Bordugo A., Brancati F., Russo C. V., Di Rocco M., Toscano A., Scarpa M., and Bembi B.

Abstract

Niemann-Pick type C (NPC) disease is an autosomal recessive lysosomal storage disorder caused by mutations in NPC1 or NPC2 genes. In 2009, the molecular characterization of 44 NPC Italian patients has been published. Here, we present an update of the genetic findings in 105 Italian NPC patients belonging to 83 unrelated families (77 NPC1 and 6 NPC2). NPC1 and NPC2 genes were studied following an algorithm recently published. Eighty-four different NPC1 and five NPC2 alleles were identified. Only two NPC1 alleles remained non detected. Sixty-two percent of NPC1 alleles were due to missense variants. The most frequent NPC1 mutation was the p.F284Lfs*26 (5.8% of the alleles). All NPC2 mutations were found in the homozygous state, and all but one was severe. Among newly diagnosed patients, 18 novel NPC1 mutations were identified. The pathogenic nature of 7/9 missense alleles and 3/4 intronic variants was confirmed by filipin staining and NPC1 protein analysis or mRNA expression in patient’s fibroblasts. Taken together, our previous published data and new results provide an overall picture of the molecular characteristics of NPC patients diagnosed so far in Italy.

Published
2020

50. Automated treatment planning as a dose escalation strategy for stereotactic radiation therapy in pancreatic cancer

Author

Cilla, S., Ianiro, A., Romano, C., Deodato, F., Macchia, G., Viola, P., Buwenge, M., Cammelli, S., Pierro, A., Valentini, V., Morganti, A. G., Cilla S., Romano C., Deodato F. (ORCID:0000-0003-1276-5070), Macchia G., Valentini V. (ORCID:0000-0003-4637-6487), Morganti A. G., Cilla, S., Ianiro, A., Romano, C., Deodato, F., Macchia, G., Viola, P., Buwenge, M., Cammelli, S., Pierro, A., Valentini, V., Morganti, A. G., Cilla S., Romano C., Deodato F. (ORCID:0000-0003-1276-5070), Macchia G., Valentini V. (ORCID:0000-0003-4637-6487), and Morganti A. G.

Abstract

Purpose: To assess the feasibility of automated stereotactic volumetric modulated arc therapy (SBRT-VMAT) planning using a simultaneous integrated boost (SIB) approach as a dose escalation strategy for SBRT in pancreatic cancer. Methods: Twelve patients with pancreatic cancer were retrospectively replanned. Dose prescription was 30 Gy to the planning target volume (PTV) and was escalated up to 50 Gy to the boost target volume (BTV) using a SIB technique in 5 fractions. All plans were generated by Pinnacle3 Autoplanning using 6MV dual-arc VMAT technique for flattened (FF) and flattening filter-free beams (FFF). An overlap volume (OLV) between the PRV duodenum and the PTV was defined to correlate with the ability to boost the BTV. Dosimetric metrics for BTV and PTV coverage, maximal doses for serial OARs, integral dose, conformation numbers, and dose contrast indexes were used to analyze the dosimetric results. Dose accuracy was validated using the PTW Octavius-4D phantom together with the 1500 2D-array. Differences between FF and FFF plans were quantified using the Wilcoxon matched-pair signed rank. Results: Full prescription doses to the 95% of PTV and BTV can be delivered to patients with no OLV. BTV mean dose was >90% of the prescribed doses for all patients at all dose levels. Compared to FF plans, FFF plans showed significant reduced integral doses, larger number of MUs, and reduced beam-on-times up to 51% for the highest dose level. Despite plan complexity, pre-treatment verification reported a gamma pass-rate greater than the acceptance threshold of 95% for all FF and FFF plans for 3%-2 mm criteria. Conclusions: The SIB-SBRT strategy with Autoplanning was dosimetrically feasible. Ablative doses up to 50 Gy in 5 fractions can be delivered to the BTV for almost all patients respecting all the normal tissue constraints. A prospective clinical trial based on SBRT strategy using SIB-VMAT technique with FFF beams seems to be justified.

Published
2020

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