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Ductal, acinar and islet cell components of pancreas show a common

embryogenesis from the foregut endoderm.

But the carcinomas with mixed exocrine and endocrine tissue are rare.

Among these, the rare "mixed" tumors reported are:

1. Ductal - islet cell carcinoma

2. Acinar - islet cell carcinoma

3. Ductal - acinar - islet cell carcinoma.

Visit: Pancreatic Pathology Online ; Exocrine Pancreatic Tumours.

                   

Mixed exocrine-endocrine tumor of the pancreas. JOP. 2005 Sep 10;6(5): 449-54.

CONTEXT: Neoplasms of the pancreas usually show ductal, acinar or endocrine differentiation. Tumors with mixed exocrine and endocrine components are unusual. We herein describe a case of a mixed ductal-endocrine tumor. CASE REPORT: A 65-year-old woman was referred to our department with a diagnosis of carcinoma of the tail of the pancreas. The patient had a short history of upper abdominal pain, nausea and melena. Upper gastrointestinal endoscopy revealed gastric fundus varices and CT scan demonstrated an inhomogeneous tumor located in the tail of the pancreas infiltrating the spleen and the splenic vein. The patient underwent distal pancreatectomy and splenectomy, and had an uneventful recovery. Pathological examination revealed a mixed ductal-endocrine tumor. The endocrine component was immunoreactive for glucagon, gastrin and somatostatin, and non-reactive for insulin. CONCLUSIONS: Because of the rarity and unpredictable biologic behavior of these tumors, the need for adjuvant therapy has not yet been well-defined. The patient has had a follow-up CT scan every six months, and one and a half years later remains disease free.

A case of mixed acinar-endocrine carcinoma of the pancreas discovered in an asymptomatic subject.Int J Pancreatol. 2000 Jun;27(3):249-57.

A 50-yr-old Japanese man was found to have a hypoechoic mass 3 cm in diameter in the pancreatic head on an ultrasonography (US) examination without symptoms. A computed tomography (CT) scan demonstrated a 3-cm solid mass in the pancreatic head, and it was more clearly delineated as a low-density area on enhanced CT. Angiography showed a tumorlike stain, 3 cm in size, in the pancreatic head. The preoperative diagnosis was "special type of pancreatic tumor such as acinar cell carcinoma or non-functioning islet cell tumor." The patient was treated by pylorus-preserving pancreatoduodenectomy. Histological, immunohistochemical, and electron-microscopic studies of the surgical specimen led to a definitive diagnosis of a mixed acinar-endocrine carcinoma. The patient is currently well, with no signs of tumor recurrence, 18 mo after the operation. Our search of the Japanese and English-language literature retrieved only 15 well-documented cases of mixed acinar-endocrine carcinoma. Imaging in the reported cases revealed features of either acinar cell carcinoma or islet cell tumor, or both, which can may be detected even in small tumors more easily than conventional invasive ductal carcinoma of the pancreas because the detectability of this rare tumor on US and CT seems to be good.

Mixed exocrine-endocrine tumors of the pancreas.Semin Diagn Pathol. 2000 May;17(2):104-8.

Neoplasms of the pancreas usually show either ductal, acinar, or endocrine differentiation. Mixed exocrine-endocrine pancreatic neoplasms, in which the endocrine component is significant and comprises one-third to one-half of the tumor tissue, are rare. Truly mixed tumors have to be distinguished from exocrine neoplasms with scattered endocrine cells. In ductal adenocarcinomas, the scattered endocrine cells seem to be nonneoplastic. In other malignancies such as acinar cell carcinoma and pancreatoblastoma, scattered endocrine cells most likely represent an integral component of the tumor.

Mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with Zollinger-Ellison syndrome: an autopsy case with a 24-year survival period.Virchows Arch. 1999 Dec;435(6):606-11.

We report an autopsy case of mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with Zollinger-Ellison syndrome. A 38-year-old Japanese male was found to have Zollinger-Ellison syndrome and pancreatic gastrinoma, and gastrectomy and resection of the pancreatic tumor were performed. However, hypergastrinemia persisted, and the patient died of disseminated carcinomatosis at 62 years of age, 24 years after the onset of Zollinger-Ellison syndrome. At autopsy, the main tumor was present in the residual pancreas, and metastases were noted in many organs. In the pancreas and other organs, ductal and endocrine carcinoma areas were mixed and there was a gradual transition between the two. No acinar differentiation was noted. The ductal elements were positive for mucins and carcinoembryonic antigen but negative for neuroendocrine markers, while endocrine elements were positive for chromogranin A and synaptophysin and to a lesser extent for gastrin, but negative for mucins and carcinoembryonic antigen. The ductal elements comprised about 30% of the tumor cells, and endocrine elements 70%. According to the revised World Health Organization classification, our case was diagnosed as mixed ductal-endocrine carcinoma. Our case is rare because the tumor manifested as gastrinoma with Zollinger-Ellison syndrome and the patient survived for 24 years. To the best of our knowledge, no such case has been reported. Our case suggests that pancreatic endocrine tumors may evolve into mixed ductal-endocrine carcinomas.

Malignant mixed exocrine-endocrine tumor of the pancreas with unusual intracytoplasmic inclusions. Ultrastruct Pathol. 1993 Sep-Oct;17(5):483-93.

A case of malignant mixed exocrine-endocrine tumor of the pancreas is reported. Electron microscopy revealed abundant neurosecretory granules in most cels. Zymogen granules indicating acinar differentiation were seen in a few cells. Ductal features, including microvilli with prominent filamentous cores and intracytoplasmic mucin granules, were also noted in this lesion. immunocytochemical strains were positive for serotonin and glucagon. Unusual intracytoplasmic fibrillary inclusions are described and their possible origin discussed.

Pancreatic mixed ductal-islet tumors. Is this an entity?Int J Pancreatol. 1992 Feb;11(1):23-9.

Thirty-eight human pancreatic cancer specimens were studied for the reactivity of cancer cells with monoclonal antibodies against insulin, glucagon, somatostatin, pancreatic polypeptide (PP), vasoactive intestinal peptide (VIP), gastrin, calcitonin, and with argyrophilic reactivity. Immunoreactivity with one or several antibodies or argyrophilic reactivity were found in 30 (79%) cases. In 17 cases, the number of endocrine cells was excessive and morphologically consistent with the mixed ductal-islet tumor. Although most immunoreactive cells were located at the base of the malignant glands, some had intraepithelial location and were also present in the invasive portion of cancers, indicating their malignant nature. Endocrine cell proliferation were found in the pancreatic tissue adjacent to the carcinoma in 8 out of 12 specimens examined. In these cases, the immunoreactive cells were either distributed among the acinar cells or ductal cells. More endocrine cells were found in the hyperplastic ducts; however, no correlation was found between the degree of hyperplasia and the occurrence of any type of immunoreactive cells. Although several types of endocrine cells occurred in different pancreatic regions (head, body, and tail), PP cells were restricted to tissues taken from the head of the pancreas. Experimental data and similar observations by other investigators led us to conclude that participation of endocrine cells in ductal-type carcinomas is a general phenomenon and does not justify the classification of these lesions to mixed ductal-islet entity. However, because immunoreactive cells were more common and numerous in well-differentiated carcinomas, they may have some prognostic values.
 


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Anatomy of Normal Pancreas

Normal Islets of Langerhans

An approach to reporting of pancreatic specimen

Reporting of pancreatic biopsies for the diagnosis of neoplastic lesions

Reporting of ampullary and periampullary biopsies for the diagnosis of neoplastic lesions

Reporting of Pancreatico duodenectomy (Whipple's operation) specimen

Reporting of Distal Pancreatectomy Specimen

Developmental Defects of Pancreas

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Pancreas Divisum

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Pancreatic Agenesis

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Neoplasms of the Endocrine Tumours

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Enterochromaffin Cell (Carcinoid) Tumours

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Carcinoma of the Pancreas