Bleeding and malignant pancreatic neuroendocrine neoplasms: A Malaysian case series

Abstract Casestudy: Pancreatic neuroendocrine neoplasms (PanNEN) are rare accounting for 2-5% of pancreatic tumors. Although mostly sporadic, 10-20% are associated with inherited syndromes, notably MEN-1, Von Hippel- Lindau disease, neurofibromatosis type 1, and tuberous sclerosis (TS). When compared to sporadic cases, PanNEN in hereditary syndromes occur at a younger age, are often multifocal, cystic, and may show characteristic microscopic patterns. TS is an autosomal dominant multi-system disorder with mutations involving TSC1 or TSC2 genes which function as tumor suppressors by inhibiting mTORC1 kinase. PanNEN is observed in 1.5-1.8% of patients with TS and no surveillance guidelines for the assessment of pancreatic lesions are established. Compared to other syndromes, PanNEN associated with TS are solitary. To our knowledge, only two cases of multifocal PanNEN in TS patients have been reported. We present a case of a 67-year-old gentleman with a history of TS also affecting two daughters. He presented to the emergency department with severe abdominal pain. Abdominal ultrasound suggested acute appendicitis and an incidental 2.0 cm solid lesion was noted in the head of the pancreas. Follow-up MRI revealed two additional non-cystic masses in the pancreatic tail. Endoscopic ultrasound-guided biopsy of a tail lesion revealed monomorphic tumor cells with stippled chromatin without cytologic atypia. Immunohistochemical staining was positive for synaptophysin and chromogranin. Ki-67 labelling index was under 1%. Diagnosis of a well-differentiated neuroendocrine tumor (G1) was made. The patient denied symptoms of the carcinoid syndrome and no biologically active hormones were detected. Gallium PET scan revealed multiple foci of radiotracer uptake throughout the pancreas in addition to those described on MRI. Although PanNEN are rare in TS, malignant behavior has been reported. This case reinforces the importance of early detection through active surveillance, especially as surgical options may be limited in multifocal disease.

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The Role of Visceral Transplantation for Neoplastic Disease

Digestive Disease Interventions ◽

10.1055/s-0040-1715810 ◽

2020 ◽

Vol 04 (03) ◽

pp. 273-281

Author(s):

Masato Fujiki ◽

Amit Nair ◽

Giuseppe D'amico ◽

Mohammed Osman

Keyword(s):

Patient Survival ◽

Ex Vivo ◽

Tumor Resection ◽

Case Series ◽

National Database ◽

Neuroendocrine Neoplasms ◽

Neoplastic Disease ◽

Desmoid Tumors ◽

Post Transplant ◽

Recipient Tissue

AbstractVisceral transplantation has been utilized as the most radical surgical treatment for neoplasms not amenable to conventional resection. The main indications for this procedure include mesenteric desmoid tumors threatening the root of mesentery and metastatic neuroendocrine neoplasms. Published case-series of visceral transplantation for such indications are reviewed in this article. Patients with desmoid tumors associated with familial adenomatous polyposis are transplanted with intestinal or multivisceral allografts. With surgical modification of technique, the native spleen is preserved while duodenopancreatic complex is removed to obviate the risk of malignant transformation of duodenal polyposis after transplantation. Preservation of spleen decreased incidence of post-transplant lymphoproliferative disorder, conferring therapeutic advantage. Patient survival is comparable to that of other indications, and desmoid tumor recurrence has been observed in the recipient tissue but not in the donor allograft. For visceral transplantation of metastatic neuroendocrine neoplasms, the majority of these patients have diffuse liver involvement, thus requiring full multivisceral transplantation. Post-transplant patient survival is acceptable with limited data available on recurrence. Autotransplantation following ex vivo tumor resection using visceral allografts has been also performed in a limited, select cohort of patients with various pathologies. Adenocarcinomas are associated with a prohibitive recurrence rate following the procedure, and its use for this indication is therefore not recommended. A national database of visceral transplantation undertaken for neoplastic disease should be developed to better understand predictors of outcomes and to help produce and standardize selection criteria.

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Gastric Mixed Neuroendocrine Non-Neuroendocrine Neoplasms: A Western Center Case Series

Medical Sciences ◽

10.3390/medsci9030047 ◽

2021 ◽

Vol 9 (3) ◽

pp. 47

Author(s):

Marcus Fernando Kodama Pertille Ramos ◽

Marina Alessandra Pereira ◽

Arthur Youssif Mota Arabi ◽

Melissa Mello Mazepa ◽

Andre Roncon Dias ◽

...

Keyword(s):

Gastric Adenocarcinoma ◽

Tumor Size ◽

Lymphovascular Invasion ◽

Comparison Group ◽

Case Series ◽

Clinicopathological Characteristics ◽

Neuroendocrine Neoplasms ◽

Rare Tumor ◽

Term Survival

Background: Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) represent a rare tumor composed of adenocarcinoma and neuroendocrine carcinoma components. This study reports a case series of gastric MiNEN and discusses issues related to its diagnosis, management, and outcomes. Methods: We retrospectively analyzed data from patients with gastric MiNEN who underwent surgical resection at our service from 2009 to 2020. Patients with gastric adenocarcinoma served as a comparison group. Clinical, pathologic, and surgical characteristics were compared. Results: During the selected period, 5 gastric MiNEN patients and 597 patients with gastric adenocarcinoma were included. Among the clinical variables, age, sex, BMI, and laboratory exams were similar between the two groups. Only ASA classification was different (p = 0.015). Pathological variables such as tumor size, lymphovascular invasion, number of retrieved lymph nodes, and pTNM staging were also similar between both groups. Lastly, early surgical outcomes and long-term survival did not differ between gastric MiNEN and adenocarcinoma patients. Conclusion: A MiNEN is a rare tumor that represents less than 1% of GC patients undergoing curative treatment, and demonstrated clinicopathological characteristics and outcomes similar to gastric adenocarcinoma.

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