1,721,110 research outputs found
Primary leiomyosarcoma of the pancreas: report of a case treated by local excision and review of the literature
Full text linkFirst described by Ross in 1951, primary pancreatic leiomyosarcoma is a rare mesenchymal tumour of the pancreas, with nonspecific clinical and radiological features and a poor prognosis, if unresectable
Lymphoepithelial Cyst of the Pancreas: A Challenging Differential Diagnosis among Cystic Pancreatic Tumors.
Full text linkContext First described by Luchtrath and Schriefers in 1985 [1], lymphoepithelial cysts (LECs) of the pancreas are rare true benign cystic tumors of uncertain etiology (0.5% of all pancreatic cysts). They are found mainly in middle-aged males in the tail of the pancreas (size range: 2-10 cm). The challenging preoperative differential diagnosis of pancreatic LECs is among pseudocysts, cystic neoplasms and intraductal carcinomas. Case report During follow up for prostatic cancer, a 66-year-old man presented as an incidental finding at abdomen CT scan, a multiloculated cystic lesion (8x6 cm), located between duodenum and pancreatic head, with a solid component in its lower side, without contrast enhancement. MRI confirmed the lesion, suspected to be a mucinous tumor non communicating with the Wirsung duct. A pancreatic EUS showed a inhomogeneous cystic mass of the head of the pancreas, which had internal septa and a solid component. The amylase level in the cystic fluid content was 84 U/L, and CEA and CA 19-9 levels were 301 μg/L and 76,579 kU/L, respectively. Histology of the solid component was inconclusive. A 18FDG-PET was negative for pancreatic malignancy. Blood tests showed a severe increase of creatinine and urea levels, because the patient had an acute renal failure due to the prostatic cancer, and serum CEA and CA 19-9 levels were 2.7 μg/L and 81 kU/L, respectively. After renal function normalization, with the suspicion of a mucinous cystic neoplasm (MCN), the patient underwent surgery. The mass had a tight-elastic thickness and seemed not to involve the pancreatic parenchyma, so a resection of the lesion was performed. The post-operative course was uneventful. Histology revealed a cystic lesion (8x4 cm) containing yellowish fluid, lined by a stratified squamous epithelium with focal sebaceous differentiation, and surrounded by lymphoid tissue. The patient is well and asymptomatic three months after surgery. Conclusion LECs should be considered in the differential diagnosis of cystic pancreatic tumors, whenever a large, well-defined solid or cystic peripheral pancreatic lesion is found. Imaging findings of LECs are non-specific, so surgical resection with pathological examination of the cyst is the gold standard for diagnosis. Cytology from EUS-FNA can help to distinguish LECs from cystic neoplasms
Pancreatic Neuroendocrine Neoplasms and Gastrointestinal Stromal Tumors: A Single-Institution Experience of a Rare Association and Review of the Literature
No full textPancreatic neuroendocrine neoplasms (pNENs) and gastrointestinal stromal tumors (GISTs) represent rare neoplasms. Nonsyndromic cases of pNENs associated with a synchronous GIST were evaluated, and a review of the literature was performed
Serotonin-Secreting Neuroendocrine Tumours of the Pancreas
Full text linkBackground: Serotonin-secreting pancreatic neuroendocrine tumours (5-HT-secreting pNETs) are very rare, and characterised by high urinary 5-hydroxyindole-acetic acid (5-HIAA) levels (or high serum 5-HT levels).
Methods: Patients with 5-HT-secreting pancreatic neoplasms observed in our unit (1986-2015) were included. Diagnosis was based on urinary 5-HIAA or serum 5-HT levels.
Results: Seven patients were enrolled (4 M/3 F), with a median age of 64 (range 38-69) years. Two patients had a carcinoid syndrome. Serum 5-HT was elevated in four patients. Urinary 5-HIAA levels were positive in six patients. The median tumour size was 4.0 (range 2.5-10) cm. All patients showed liver metastases at diagnosis. None underwent resective surgery; lymph node/liver biopsies were taken. Six lesions were well-differentiated tumours and one a poorly differentiated carcinoma (Ki67 range 3.4-70%). All but one patient received chemotherapy. Four patients received somatostatin analogues; three patients underwent ablation of liver metastases. One patient is alive with disease 117 months after observation. All the others died from disease progression after a follow-up within 158 months.
Conclusions: Primary 5-HT-secreting pNETs are mostly metastatic to the liver; patients are not amenable to resective surgery. Despite high 5-HIAA urinary levels, few patients present with carcinoid syndrome. A five-year survival rate of 42.9% may be achieved with multimodal treatment
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