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       Atypical Fibroxanthoma


 

                  

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Non-epithelial tumours of the pancreas are exceedingly rare and include.

Benign:

-Clear Cell (Sugar) Tumour of the Pancreas

- Pancreatic Schwannoma

Malignant:

- Malignant Lymphoma of the Pancreas

- Leiomyosarcoma of the Pancreas

- Primitive Neuroectodermal Tumour of the Pancreas

- Carcinosarcoma of the Pancreas

Carcinosarcoma of the pancreas: a case report and review of the literature. Arch Pathol Lab Med. 2002 Sep;126(9):1114-7.

We report the case of a 74-year-old white man with a mass in the head of the pancreas, which was found incidentally on computerized tomographic scan during a workup for deep vein thrombosis. Endoscopy with pancreatic duct brushings yielded a diagnosis of adenocarcinoma. A pancreaticoduodenectomy followed, with complete resection of the tumor. Pathologic examination showed 2 distinct components. One component was a conventional infiltrating pancreatic ductal adenocarcinoma, and the other component was high-grade sarcoma with features of malignant fibrous histiocytoma. To our knowledge, this carcinosarcoma is the seventh reported case of a primary pancreatic neoplasm with mixed carcinomatous and sarcomatous elements.

Whatever the site of origin, most mesenchymal pancreatic tumours

are hypervascular, heterogeneous and have a necrotic center.

                   

Clinicopathologic features of two rare cases of mesenchymal metastatic tumors in the pancreas: review of the literature.Pancreas. 2006 Oct;33(3): 301-3.

OBJECTIVES: A clinicopathologic presentation of 2 unusual cases of metastatic mesenchymal neoplasms in the pancreas. METHODS: The first case concerns a 26-year-old man with a history of intracranial mesenchymal chondrosarcoma (since the age of 17), 2 left lung operations, and 3 right thigh operations. Distal pancreatectomy and splenectomy was performed because of suspicious mass in the pancreas. The second case concerns a 66-year-old woman with a history of uterus leiomyosarcoma (10 years ago) with left axillary and right femoral metastases. She underwent distal pancreatectomy and splenectomy because of suspicious mass measuring 4 x 4 cm, in the pancreatic body. RESULTS: In the first case, the pathological examination revealed a tumor measuring 3.8 x 3.5 cm and histologically compatible with mesenchymal chondrosarcoma, developing in a vessel lumen and invading into the pancreatic parenchyma. In the second case, the pathological examination showed metastatic leiomyosarcoma of high-grade malignancy. The incidence of metastatic pancreatic tumors has been reported to be only 1.6% to 3%. Most of these tumors were of epithelial origin, and the most common sites of the primary lesions were the lung, kidney, and gastrointestinal tract. CONCLUSIONS: The cases of metastatic uterus leiomyosarcoma and the metastatic intracranial chondrosarcoma are, to our knowledge, the first to be described.

Mesenchymal tumors of the pancreas: computed tomography patterns. Radiol Med (Torino). 1999 Oct;98(4):295-9.

PURPOSE: To report the CT patterns of pancreatic mesenchymal lesions and to investigate the capabilities and limitations of this technique in terms of characterization. MATERIAL AND METHODS: We selected the CT examinations of 23 patients from all CT examinations of the pancreas performed from 1986 to 1998. The patients were 10 to 85 years old and all lesions but two (1 lipoma and 1 lymphoma) had pathologic confirmation. Two lymphangiomas, 2 lipomas, 4 Schwannomas (SCH), 1 plexiform neurofibroma, 1 cystic teratoma, 1 pancreatoblastoma, 9 non-Hodgkin's lymphomas (NHL), 1 undifferentiated sarcoma and 2 leiomyosarcomas were diagnosed. RESULTS: Lipomas had typically homogeneous negative HU values, and NHL a homogeneous hypodense pattern with mild contrast enhancement, with no necrosis or calcifications in both diffuse and nodular forms; SCH had variable appearance, with water/slightly negative HU numbers or highly enhanced patterns with a central necrotic core depending on the main Antoni A or B histology. Lymphangiomas appeared as complex cysts, with thin and regular or calcified walls and intracystic septa, while the plexiform neurofibroma had near-water homogeneous density with mild contrast enhancement and an infiltrating growth pattern. Our teratoma had multiple calcifications, mucinous and lipid components. The pancreatoblastoma, sarcomas, and leiomyosarcomas exhibited no specific findings. CONCLUSIONS: In selected cases, CT can provide virtually diagnostic information (lipoma and teratoma), or at least highly suggestive findings (NHL, plexiform neurofibroma, lymphangioma). Moreover, knowledge of the variable morphostructural patterns of mesenchymal histotypes (SCH, sarcoma etc.) permits to include them in the differential diagnosis of pancreatic masses.

Malignant non-epithelial tumors of the pancreas.Pathologe. 1997 May; 18(3): 233-7.

Nonepithelial malignant tumors of the pancreas are extremely rare neoplasms with a frequency of approximately 0.6%. They are always explored because of a suspected diagnosis of pancreatic carcinoma. Amongst the more than 600 primary pancreatic neoplasms in our pancreatic tumor archive only 5 neoplasms were of nonepithelial origin (one was a malignant peripheral nerve sheath tumor [MPNST], one a leiomyosarcoma, one a malignant mesothelioma, and two were peripheral neuroectodermal tumors [PNET]. The differential diagnosis includes secondary infiltration of the pancreas by mesenchymal tumors of the retroperitoneum, undifferentiated pancreatic carcinoma and, especially in the case of PNET, malignant lymphoma. Preoperative chemotherapy and down-staging can improve the operability and prognosis, especially in PNET.


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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

Nesidioblastosis

Pancreas Divisum

Aberrant(Ectopic)Pancreas

Annular Pancreas

Pancreatic Agenesis

Non-Neoplastic Pancreatic Cysts 

Pancreatitis

Acute Pancreatitis

Chronic Pancreatitis

Autoimmune Pancreatitis