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Background: Port-wine stains (PWS) are congenital low-flow vascular malformations of the skin. PWS tend to become thicker and darker with time. Laser therapy is the gold standard and the first-line therapy for treating PWS. However, some resistant PWS, or PWS that have tissue hypertrophy, do not respond to this therapy. Our aim is to evaluate the role of surgery in the treatment of PWS birthmarks. Methods: A literature search was performed in PubMed, Scopus, Web of Science (WOS) and Google Scholar for all papers dealing with surgery for port-wine stains, from January 2010 to December 2020 using the search strings: (capillary vascular malformation OR port-wine stains OR Sturge Weber Syndrome OR sws OR pws) AND (surgical OR surgery). Results: Ten articles were identified and used for analysis. They were almost all case series with a short follow up period and lacked an objective–systematic score of evaluation. Conclusions: Delay in treatment of port wine stains may result in soft tissue and bone hypertrophy or nodules with disfiguring or destructive characteristics. The correction of PWS-related facial asymmetry often requires bone surgery followed by soft tissue corrections to achieve a more harmonious, predictable result. [ABSTRACT FROM AUTHOR]

Bone xanthoma is a rare benign primary bone lesion histologically characterized by sheets of foamy cells which are macrophages with a cytoplasm filled with droplets of fat. It is usually associated with endocrine or metabolic diseases, in the absence of which the lesion is called primary xanthoma. Because of the lack of pathognomonic radiologic and clinical features, they require a differential diagnosis with a broad spectrum of lesions with a varying degree of malignant potential. We describe a case of primary mandibular xanthoma of a 16-year-old girl without typical cutaneous manifestations and alterations in lipid values. The temporomandibular joint involvement at the mandibular condyle is peculiar in the case described here. We present even a qualitative systematic review of the literature on primary xanthoma of the jaws in others to draw up treatment guidelines. [ABSTRACT FROM AUTHOR]

A solitary fibrous tumor (SFT) is a benign neoplasm, firstly described as a mesenchymal tumor of the pleura. Its incidence range in the head and neck region is about 5--27%, but only rarely does it affect paranasal sinuses. The differential diagnosis is challenging, owing to its erosive growth pattern and immuno-histochemical features. SFTs have an aggressive behavior and an important recurrence potential. Therefore, a radical surgical excision is the gold standard therapeutic procedure. A rare SFT originating from the right maxillary sinus is reported here. The 37-yearold patient presented to the outpatient clinic with a painful expansive lesion in the whole right maxillary region. The overlying skin was inflamed and the patient had no epistaxis episodes. The 1.5 dentary element tested negative for vitality; however, a puncture of the lesion led to a hematic spill and no purulent discharge. An endoscopic-guided biopsy was suggestive either of SFT or hemangioperictoma, excluding a malignant neoplasm. A multi-equipe surgical team was activated. The lesion was embolized in order to achieve a good hemostatic control and, after 48 h, the neoplasm was radically excised with a combined open and endoscopic approach. The patient was disease-free at 12-month radiological and clinical follow-up. Given the rarity of this lesion and the delicacy required in addressing head and neck neoplasms, we believe that the present case report might be of help in further understanding how to approach cranio-facial SFTs. [ABSTRACT FROM AUTHOR]

Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare and aggressive malignancy arising exclusively within the jaws, without any initial connection with the oral mucosa. The etiology and the epidemiology are unclear due to the rarity of the disease, and there is no current universally accepted staging or treatment protocol. Clinically, the posterior mandible is the most affected site, and common symptoms are swelling and pain. The diagnosis is often difficult either because it requires stringent criteria to be satisfied or because of the absence of a pathognomonic histological pattern. Aggressive surgery is the first-choice treatment, often followed by radiotherapy. The lymph nodal status seems to be the most important factor influencing the prognosis, which is usually poor, with a 5-year survival rate ranging from 30% to 40%. In the present article, we report an unusual case of cystogenic PIOSCC interesting the anterior mandible of a young 34-year-old male, which came to our attention after complaint about recurrent infective episodes affecting a dentigerous cyst (impacted lower canine) discovered ten years before. The age, site, and extension are uncommon. Extensive surgical treatment with fibula free flap reconstruction, adjuvant therapy, and salvage surgery was carried out. The patient was disease-free at a 31-month follow-up. [ABSTRACT FROM AUTHOR]

To report a case of acinic cell carcinoma occurred in the lacrimal gland. A 59-year-old man was admitted because of sudden blurring of vision, progressive proptosis of the left eye, and mild double vision in left and down directions of the gaze (Hess-Lancaster test). His medical history detailed controlled bilateral keratoconus and open angle glaucoma. On examination, the best corrected visual acuity decreased from 8/20 till 1/50 in one week. There was a swelling of the left upper eyelid. A hard and tender mass was palpated in the superior temporal left orbit. Ultrasound scan showed an extraconal solid mass, situated in the superior lateral corner of the orbit. Computed tomography and magnetic resonance imaging (MRI) revealed a mass of two centimeters in diameter, with round well-defined outline, within the lacrimal gland. We performed an enucleoresection of the mass, via a coronal approach and a lateral orbitotomy by a piezosurgical device. The lesion appeared nodular, brownish, measuring about 2 × 1.5 cm. Histopathological findings were consistent with acinic cell carcinoma with a microcystic, focally papillary-cystic growth of pattern. Follow-up MRI outcomes led to removal of the residual lacrimal gland for suspicion of recurrence. No tumor recurrences where detected at 7-year follow-up. [ABSTRACT FROM AUTHOR]

The effect of multiple general anesthesia (mGA) procedures administered in early life is a critical theme and has led the Food and Drug Administration (FDA) to issue an alert. This systematic review seeks to explore the potential effects on neurodevelopment of mGA on patients under 4 years. The Medline, Embase and Web of Science databases were searched for publications up to 31 March 2021. The databases were searched for publications regarding "children multiple general anesthesia OR pediatric multiple general anesthesia". Case reports, animal studies and expert opinions were excluded. Systematic reviews were not included, but they were screened to identify any possible additional information. A total of 3156 studies were identified. After removing the duplicates, screening the remaining records and analyzing the systematic reviews' bibliography, 10 studies were considered suitable for inclusion. Comprehensively, a total cohort of 264.759 unexposed children and 11.027 exposed children were assessed for neurodevelopmental outcomes. Only one paper did not find any statistically significant difference between exposed and unexposed children in terms of neurodevelopmental alterations. Controlled studies on mGA administered before 4 years of age support that there might be a greater risk of neurodevelopmental delay in children receiving mGA, warranting the need for careful risk/benefit considerations. [ABSTRACT FROM AUTHOR]

Pediatric sarcomas are rare malignancies accounting for about 10% of solid tumors in childhood. Sarcomas represent a heterogeneous group of malignancies, especially they include Rhabdomyosarcoma (RMS) and Non-Rhabdomyosarcoma Type Sarcomas (STSNR). Therapeutic management of pediatric sarcomas is challenging. Multidisciplinary approach including chemotherapy, surgery and radiotherapy is the treatment of choice. The correct management of affected patients can be demanding, especially in terms of preserving a good quality of life (QoL). In the present study we described our experience with a series of patients diagnosed with head and neck non-metastatic sarcoma between January 1995 and December 2020, referred to Pediatric Oncology or to Maxillo-Facial Surgery of University Hospital of Modena and Reggio Emilia.The survivors were asked to complete questionnaires on health-related quality of life (HRQoL; PedsQL and EORTC QLQ-H&N 43). We found satisfactory results in terms of global HRQoL; although outstanding issues persist, such as deterioration in masticatory function, and speech. Long-term sequelae of multimodality treatment may lead to both significant aesthetic-functional and psychosocial implications that need to be further investigate during the whole the care pathway. [ABSTRACT FROM AUTHOR]

Capillary malformations (MCs), also referred to as port-wine stains (PWSs), or port-wine birthmarks (PWBs) represent one of the most common congenital vascular birthmarks. They consist of ectatic postcapillary venules within the papillary and superficial reticular dermis. The pathogenesis is due to a somatic activating mutation in guanine nucleotide–binding protein G(q) subunit alpha which mosaically occurs in endothelial cells (ECs) during embryogenesis. As a true congenital vascular malformation, PWSs are always present at birth, appearing as a flat pink to red macula mostly arising in the head and neck unilaterally. Although lightening during the first few months of life is possible, these lesions generally stay stable or thicken and darken over time. Consequent functional impairment, tissue thickening, development of blebs, and psychological burden may be relevant, so early laser therapy should often be considered to deter progression. What we present here is a unique cohort of 10 children presented to the Vascular Birthmarks Foundation (VBF) in Latham, NY, between 2016 and 2021, who were followed over time for cutaneous vascular anomalies present at birth. The clinical features were strongly suggestive of PWSs. However, all 10 of these children showed a significant spontaneous regression of the lesions during the observational period. In four cases, there was complete resolution. According to the international literature, capillary malformations persist throughout life and typically grow and darken if early laser treatment is not initiated. Such spontaneous improvement is extremely rare after the first 6 months of life. In the present case series, we photographically documented this previously unreported circumstance. Level of evidence: Level V, risk/prognostic study. [ABSTRACT FROM AUTHOR]

Mucopolysaccharidoses (MPS) are a family of genetic diseases associated with a deficiency of alpha-L iduronidase, which causes a lack of catabolism of glycosaminoglycans (GAGs). Therefore, the accumulation of GAGs determines a wide spectrum of symptoms, typically found in a few syndromes like Hurler syndrome (HS). Among other specific manifestations, craniofacial abnormalities are crucial for the characterization of this syndrome. Ameloblastoma is a rare, benign, slow-growing, odontogenic tumor usually located in the mandible. Clear risk factors for the development of ameloblastoma remain unknown, but black patients have a fivefold increased risk. Clinically, it is characterized by a painless, variable-sized jaw swelling. Although classified as a benign tumor, ameloblastoma often has a severe clinical outcome. The most common type of ameloblastoma is the solid/multicystic/conventional one. A computed tomography scan (CT) with and without contrast is the gold standard for evaluating this kind of neoplasia. Conservative or radical surgery is the mainstay of treatment. In this case report, we described an unusual clinical assessment of conventional ameloblastoma interesting the posterior left mandible of a 35-month-old child affected by HS. This case represented a suggestive challenge both from a diagnostic and a therapeutic point of view. The patient was disease-free at 2 years' follow-up. [ABSTRACT FROM AUTHOR]

Reconstruction of defects of the jaws is mainly performed via free fibula flap. An incidence of 2–21% of overall flap failure is still described. We investigated the roles of volume, length and number of fibula flap segments on flap survival using novel three-dimensional segmentation tools. We also analyzed the role of other possible risk factors. Seventy-one consecutive patients with a follow up of at least three months and who underwent free fibula flap reconstruction in a single center between 2002 and 2022 have been evaluated. A total of 166 fibula segments were analyzed. Malignancies were the main reason of resection (45.1%). In 69% of the cases a reconstruction of the mandible was performed. The flaps were mainly divided in two segments (39%) (range 1–4), with a mean length of 2.52 cm and a mean volume was 3.37 cm3. Total flap failure (TFF) occurred in 12 cases, (16.9%), while partial flap failure (PFF) appeared in 3 patients (4.2%). Volume, length and number of fibula flap segments did not seem to influence flap failure incidence in uni- and multivariate analysis. Reconstruction of the maxilla and use of a recipient vessel different from the facial artery seemed to significantly impact on flap failure. Smoking and previous surgeries showed a higher trend to flap failure, but they did not reach statistical significance. Prospective and multicentric analysis on a wider population should be assessed. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this retrospective study was to determine the incidence and the clinical outcome of tongue cancer (TC) in patients affected by Fanconi anemia (FA) who received an allogeneic hematopoietic cell transplantation (HCT). Materials and methods: The patient database from the Bone Marrow Transplant Center of Pescara was reviewed to enroll FA patients. Patients', donors', HCT's, and screening's data were collected as well to look for the incidence and the treatment of TC. Results: Twelve patients affected by FA were identified. Three patients died for transplant-related causes. Five of nine surviving patients were diagnosed with TC at a median of 21.7 years since transplantation and at a median age of 32.10 years. Interestingly, no patient manifested graft-versus-host-disease (GvHD). The 28-year cumulative incidence function of TC was 46.9% (95% CI, 36.9–56.9%). Two patients were treated with chemotherapy alone, two patients were treated with surgery alone, and one with surgery followed by chemotherapy. Overall, 4 patients with TC showed a clinical course characterized by a marked aggressiveness of the tumor disease which led to death due to cancer progression between 2 and 13 months. One patient is surviving 8 months after diagnosis of TC. Conclusions: Our study confirms the high incidence of tumors and in particular tongue tumors in allotransplanted FA patients. A careful screening has to be life-long maintained. Clinical relevance: Considering the rarity of FA and the frailty of FA patients, this study may add important information for the cancer management of these patients. [ABSTRACT FROM AUTHOR]