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Introduction: Peritoneal sarcomatosis is a rare disease, with multiple histological origins and poor overall prognosis. The option of radical cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is controversial. The results of a surgical team experienced in these procedures are analyzed and discussed based on the available evidence., Methods: Study on a prospective database of patients with peritoneal sarcomatosis who underwent CRS and HIPEC, from 2016 to 2022, in a national reference center for sarcomas and peritoneal oncological surgery, who met the established inclusion/exclusion criteria., Results: 23 patients were included in the study, with a median age of 53 years (6-68). Recurrent/persistent clinical presentation predominated (78.3%). Visceral origin (including GIST and non-GIST peritoneal) accounted for 47.8% of patients, compared to 43.5% uterine and 8.7% retroperitoneal. The median PCI was 17 (3-36), with CC0 cytoreduction of 87%. Postoperative morbidity (Dindo Clavien III-IV) of 13%, with no postoperative mortality in the series. Overall survival and disease-free survival at 5 years were 64% and 34%, respectively. Histological grade was the most influential prognostic factor for survival., Conclusions: The results of the series, with low morbidity, support the benefit of radical peritoneal oncological surgery in patients with peritoneal sarcomatosis after adequate selection, as long as it is performed in high-volume centers, experienced surgeons and expert multidisciplinary teams. However, the role of HIPEC remains to be demonstrated and pending future studies., (Copyright © 2024 AEC. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Retroperitoneal lipomas are extremely rare tumors that must be differentiated from well-differentiated liposarcomas (WD-LPS)., Objectives: To summarize the evidence about giant retroperitoneal lipomas or liposarcomas; and to elaborate recommendations for their management., Data Sources: A systematic literature search from January 1985 to December 2019 and a review of our own cases was performed., Results: Our series comprises four patients, two females and two males. The diagnosis was incidental in two cases. The medium size was 26 cm, being two cases located exclusively in the retroperitoneum, one in the inguinal region and one in the buttock via pelvic space. All cases were surgically removed being confirmed the initial diagnosis of retroperitoneal lipomas in two cases, as the rest two cases were classified as WD_LPS after MDM2/CDK4 genetic analysis. The review of the available literature plus our own cases revealed 30 cases, of which 58% were woman. Only two cases were asymptomatic. The main symptom was abdominal mass (53%) followed by abdominal pain (40,6%). The median size of the lesions was 24,9 cm with a median weight of 4.576,3 g. All cases were surgically removed, being necessary to remove contiguous organs in only four cases (12,5%)., Conclusions: Retroperitoneal lipoma is a rare tumor which must be differentiated from WD-LPS. This is a very difficult task, being necessary to determinate MDM2 status (by FISH or MLPA), present in liposarcoma but not in lipomas, for its correct diagnosis. The treatment must be based on a complete surgical resection with negative margins., (Copyright © 2020 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)

Additional primary malignancies in patients with gastrointestinal stromal tumor (GIST) is not only common but of growing interest in the scientific literature. This association is of great importance in terms of clinical challenge, diagnosis and therapy as well as for the prognosis impact it implies. In the published series there is a tendency to group these patients to determine the specific and distinguishable characteristics of GIST associated with other malignancies. On the other hand, there is no general consensus or unified classification. This classification would be of great interest, as it would unify criteria, agree groups to compare different series and demonstrate whether the aetiology underlying both tumours and the GIST's own characteristics really vary according to the type in question. We undertook a medical literature review and proposed a new classification for patients with GIST associated with other tumours., (Copyright © 2016 Elsevier España, S.L.U. All rights reserved.)

Introduction and Objectives: Radical surgery is the standard treatment for localised gastrointestinal stromal tumours (GIST). A series of primary GIST, their treatment and pre-established risk of recurrence after their follow-up is evaluated., Material and Methods: A retrospective, descriptive and multicentre study was conducted on primary, non-metastatic GIST operated on between June 2007 and December 2008. The variables of greater relevance were analysed, including, location, size, mitotic index, and NHI and AFIP recurrence prognostic criteria, and their correlation with the disease-free survival (DFS) of the patients., Results: The series included 141 patients with a mean age of 65 years. The most frequent GIST location was in the stomach (70.8%) and small intestine (22.9%), and with a mean tumour size of 6.7 cm (0.5-35 cm). The surgery was R0 in 97.2% of cases (laparoscopic approach, 21.5%). The distribution according to NHI/Flescher criteria was, high (31.95%), and intermediate (26.4%), and according to AFIP/Miettinen criteria it was, high (22.9%) and intermediate (12.5%). After a mean follow-up of 20.3 months, there was a 7.1% (10 cases) recurrence, with only 2 cases belonging to the group with a «low risk» using the NHI and AFIP prognostic criteria. The DFS at one year was 95.5% and 91.5% at 2 years., Conclusions: The series showed a high DFS and a good correlation with both the Flescher and the Miettinen criteria. However, the risk of recurrence varied according to the AFIP criteria (intermediate/high, 58.3%), or the AFIP criteria (intermediate/high, 35.4%) which included the tumour location. For this reason, we consider these latter criteria as the most adequate for assessing the prognostic risk of GIST recurrence., (Copyright © 2011 AEC. Published by Elsevier Espana. All rights reserved.)