Radical cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in the treatment of peritoneal sarcomatosis: Results from a reference center and considerations based on current evidence.

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.

2023 GEIS Guidelines for gastrointestinal stromal tumors.

Gastrointestinal stromal tumor (GIST) is the most common malignant neoplasm of mesenchymal origin. GIST spans a wide clinical spectrum that ranges from tumors with essentially no metastatic potential to malignant and life-threatening spread diseases. Gain-of-function mutations in KIT or PDGFRA receptor tyrosine kinases are the crucial drivers of most GISTs, responsible for tumor initiation and evolution throughout the entire course of the disease. The introduction of tyrosine kinase inhibitors targeting these receptors has substantially improved the outcomes in this formerly chemoresistant cancer. As of today, five agents hold regulatory approval for the treatment of GIST: imatinib, sunitinib, regorafenib, ripretinib, and avapritinib. This, in turn, represents a success for a rare neoplasm. During the past two decades, GIST has become a paradigmatic model in cancer for multidisciplinary work, given the disease-specific particularities regarding tumor biology and tumor evolution. Herein, we review currently available evidence for the management of GIST. This clinical practice guideline has been developed by a multidisciplinary expert panel (oncologist, pathologist, surgeon, molecular biologist, radiologist, and representative of patients' advocacy groups) from the Spanish Group for Sarcoma Research, and it is conceived to provide, from a critical perspective, the standard approach for diagnosis, treatment, and follow-up.

Biomarkers of Early Liver Graft Damage in Circulatory Death and Brain Death Donors: A Propensity Score Matching Analysis.

BACKGROUND: Donation after circulatory death (DCD) is related to a warm ischemia time and more complications compared with traditional donors (donation after brain death [DBD]). METHODS: This study included biopsy samples retrospectively collected from November 2014 to December 2018 to compare histologic and biological markers of DCD and DBD liver grafts. The analysis includes marker of early apoptosis (p21), senescence (telomerase reverse transcriptase [TERT]), cell damage (caspase-3 active), endothelial damage (vascular endothelial growth factor), stem cell (CD90), hypoxia (HIF1A), inflammatory activation (COX-2), and cross-organ allograft rejection (CD44). A propensity score matching (PSM) was used to match patients receiving DCD livers to those receiving DBD livers. We analyzed the immunohistochemical initial liver damage-related warm ischemia time. RESULTS: Positive staining expression of liver damage biomarkers (COX-2, CD44, TERT, HIF1A, and CD90) was found, but no significant differences were found between DCD and DBD and with ischemic cholangiopathy. After PSM, there was a significant relationship between CD90 and male donors (odds ratio [OR], 0.26; 95% confidence interval [CI], 0.07-0.91), TERT with donor sodium (OR, 1.11; 95% CI, 1.02-1.2), HIF1A with steatosis (OR, 0.33; 95% CI, 0.13-0.83), and CD44 with donor vasoactive drugs (OR, 0.36; 95% CI, 0.13-1) and glutamic oxaloacetic transaminase 1 week increase (OR, 1.01; 95% CI, 1-1.03). CONCLUSIONS: DCD immunohistochemical initial liver damage was found to behave similarly to DBD. The increase in complications and cholangiopathy associated with warm ischemia could be related to a different later phenomenon.

[Giant lipomas or retroperitoneal liposarcomas? Controversies in their diagnosis and treatment]. / ¿Lipoma gigante o liposarcoma retroperitoneal? Controversias en su diagnóstico y tratamiento.

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.

Update in pelvic and retroperitoneal sarcoma management: The role of compartment surgery. / Actualización en el manejo de sarcomas retroperitoneales y pélvicos; el papel de la cirugía compartimental.

Surgery for retroperitoneal sarcomas should be "en bloc" compartmental, which involves resection of unaffected organs. Its upfront use is key, providing a high percentage of resections with negative margins, resulting in a better local control and increased survival in many patients. Preservation of organs should be done in an individualized manner, especially in the pelvic location, and adapted to the histological aggressiveness of the tumor. Preoperative biopsy is able to establish the diagnosis of sarcoma subtype and consequently an adequate perioperative strategy. These patients should be managed by expert surgeons at referral centers with multidisciplinary units and oncology committees. The use of chemotherapy and radiotherapy is not yet well defined, so it is only recommended at referral centers with clinical trials. Currently, this is the only option to offer the best morbidity and mortality rates, as well as possible improvements in the survival of these patients.

An ulcerated gastric ulcer and pseudotumour with pancreatic affectation associated with immunoglobulin G4-related disease: a case report and literature review.

We report the case of a 67 year old male who presented with a nine year history of a gastric ulcer with symptoms of hematemesis and melena. Histological analysis identified fibrotic lesions and the accumulation of immunoglobulin G4-positive plasma cells with no evidence of malignancy. The lesion extended into the pancreas, where histological lesions and gastric lesions were also observed. This is a case of an ulcerated gastric ulcer and pseudo-tumor with pancreatic affection that is associated with immunoglobulin G4-related disease.

The assessment of the Newborn Hearing Screening Program in the Region of Murcia from 2004 to 2012.

OBJECTIVES: Newborn (NB) auditory deficit has a prevalence of 1-2% in the world. Since the 1990s different screening programs have been put into practice. The Newborn Hearing Screening Program has been in operation since 2002 in our hospital (HCUVA) in Murcia (Spain) and two years later it was introduced into the whole of the Autonomous Community of the Region of Murcia as part of universal healthcare. The objective of this study was to analyze and assess its results. METHOD: The newborn (NB) population is divided into two groups: not-at-risk NBs and at-risk NBs. In the first case we carry out acoustic otoemissions (AOEs) 48 h after birth and depending on the result the child is either discharged or, in negative cases, the infant undergoes a series of tests in a period of 30-45 days to confirm or rule out the existence of hearing anomalies. In the at-risk group we combine AOEs with brain trunk potentials (BERA) following the stages in a decision tree diagram similar to the ones for not-at-risk children in order to provide a clinical diagnosis in the first three months of life. RESULTS: The screening performance was assessed for the 156,122 children studied, of which 151,258 belonged to the group of not-at-risk children; and 4864 to the group at risk of hypoacusia. As a result of the screening only 410 (0.26%) were sent to consultation, 213 in the not-at-risk group (0.14%) and 197 (24.7%) in the at-risk group. A total of 7452 false positives were identified (4.7%), 6951 (4.5%) in the not-at-risk group; and 501 (10.3%) in the group with risk factors; and there were 53 false negatives (0.03%). Sensitivity in the screening program was 88.5%, with a specificity of 95%. CONCLUSIONS: The Region of Murcia has a Newborn Hearing Screening Program with tests that provide a high level of sensitivity and specificity in accordance with the findings of the literature. Our results endorse the program and the patients were treated in a way that met the objective of providing a correct diagnosis and the appropriate therapeutic action.

[Additional primary malignancies in patients with gastrointestinal stromal tumors. Proposal for a new classification]. / Asociación de tumores del estroma gastrointestinal con otros tumores primarios. Propuesta de una nueva clasificación.

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.

[Primary, non-metastatic gastrointestinal stromal tumour surgery: results of the GRISK study]. / Cirugía de los tumores del estroma gastrointestinal primarios no metastásicos. Resultados del estudio GRISK.

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 risk¼ 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.

[Delayed right hepatic artery haemorrhage after iatrogenic gallbladder by laparoscopic cholecystectomy that required a liver transplant due to acute liver failure: clinical case and review of the literature]. / Hemorragia diferida de la arteria hepática derecha tras iatrogenia biliar por colecistectomía laparoscópica que precisó trasplante hepático por insuficiencia hepática aguda: caso clínico y revisión de la literatura.

INTRODUCTION: Right hepatic artery (RHA) injury after laparoscopic cholecystectomy (LC) may go unnoticed clinically, but can sometimes cause necrosis of the right lobe. Exceptionally, when the necrosis spreads to segment IV, fulminant liver failure (FLF) may occur, and an urgent liver transplantation (LT) may be required. PATIENTS AND METHOD: We provide a review of the literature on patients with indication for an LT due to vascular damage caused by bile duct injury following LC. The case reported herein is the fourth described in the specialized literature of LT due to RHA injury after LC and the second of FLF after RHA injury. RESULTS: LT due to RHA injury was performed in 3 of 13 patients reported in the literature: one LT was performed at 3 months due to FLF, after an extended right hepatectomy was performed, and the remaining two were performed due to secondary biliary cirrhosis. Our patient was transplanted due to FLF 15 days after the injury. CONCLUSIONS: RHA injury after LC may require LT due to FLF. Although exceptional, this possibility should be considered when there are RHA complications that may require occlusion.

[Unilocular macrocystic serous cystoadenoma of the pancreas]. / Cistoadenoma seroso unilocular macroquístico de páncreas.

The differential diagnosis between macrocystic serous cystoadenoma and mucinous cystoadenoma is very difficult but is highly important due to the latter's potential for malignant transformation. We present a case of unilocular macrocystic serous cystoadenoma. Due to the impossibility of obtaining an accurate preoperative diagnosis, all macrocystic unilocular lesions, in an appropriate clinical context, must be resected.