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Primary leiomyosarcoma of the pancreas is a very rare malignant tumour.

A spindle cell pattern at light microscopy,immunocytochemical  reactivity with muscle-specific actin, alpha-smooth muscle actin, desmin, alpha-1-antitrypsin and vimentin and ultrastructural features of smooth muscle differentiation help to establish the diagnosis.

It is an aggressive neoplasm characterized by short survival and a high rate of metastases.

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Pancreatic metastasis of leiomyosarcoma in the right thigh: a case report.World J Gastroenterol. 2007 Feb 21;13(7):1135-7.

Pancreatic tumors are primary in most of the cases. Pancreatic metastases associated with other primary malignancies, especially pancreatic metastasis of leiomyosarcoma, are uncommon. A 66-year-old woman underwent surgical resection of malignant mesenchymoma (70% osteosarcoma and 30% leiomyosarcoma) in the right thigh. In the postoperative period, a pancreatic mass was identified radiologically by abdominal computed tomography. Pylorus-preserving pancreaticoduodenectomy was performed. The surgical specimen revealed leiomyosarcoma metastasized to the pancreas. A metastatic nodule on the remnant pancreatic tail was discovered 9 mo after the first pancreatic resection, and distal pancreatectomy was performed. Cases of pancreatic metastasis from leiomyosarcoma are extremely rare, especially when the tumor was resectable. We report here a unique case of pancreatic metastasis from a leiomyosarcoma in the right thigh that had been treated surgically.

Primary leiomyosarcoma of the pancreas: a case report and review of literature. Arch Pathol Lab Med. 2001 Jan;125(1):152-5.

Primary leiomyosarcoma of the pancreas is a rare tumor for which only 21 reports appear in the world literature. We describe an additional case of pancreatic leiomyosarcoma in a 76-year-old man, who complained of persistent high fever. Histologic examination revealed a pleomorphic spindle cell tumor. Reactivity for muscle-specific actin, alpha-smooth muscle actin, and basement membrane components, along with negative staining for epithelial and neural markers, were consistent with a smooth muscle sarcoma. The patient died of disease 1 year after complete surgical excision. This report highlights the need to use a complete antibody panel in order to accurately immunophenotype pleomorphic malignant tumors of the pancreas. A review of the cases compiled in the literature indicates that pancreatic leiomyosarcoma, like its counterpart arising in deep soft tissues, is an aggressive neoplasm characterized by short survival and a high rate of metastases.

Pancreatic leiomyosarcoma: clinicopathohistological presentation of a rare tumor.Hepatogastroenterology. 2000 Mar-Apr;47(32):556-9.

A smooth muscle tumor originating from the pancreas is an extremely rare neoplasm characterized by peculiar histopathologic features and progressive clinical course. We report a case of such a pancreatic neoplasm occurring in a 57-year-old female who underwent surgical resection. The tumor was histologically as well as immunocytochemically analyzed. The spindle cell pattern, vimentin and actin reactivity confirmed smooth muscle differentiation. On the basis of the degree of cellular atypias and the mitotic counts, the malignant potential of the tumor was assessed. Sharp demarcation of the growth appearing characteristic for this type of pancreatic neoplasia with no invasion into the surrounding organs enabled complete excision in spite of the large dimensions of the tumor.

Leiomyosarcoma and stromal tumor of the pancreas.J Radiol. 2001 Dec;82(12 Pt 1):1723-5.

A tumor of the head of pancreas was an incidental finding on US of two patients aged 52 and 61 years presenting with abdominal pain. The tumor was studied by computed tomography, MRI and endoscopic ultrasonography. Surgical biopsy of a liver lesion was performed in one case and partial duodenopancreatectomy was performed in the other case. Histological evaluation, including immunohistochemistry, showed leiomyosarcoma and stromal tumor respectively. Whatever the site of origin, most mesenchymal pancreatic tumors are hypervascular, heterogeneous and have a necrotic center.

Metastasis of uterine leiomyosarcoma to the pancreas.J Obstet Gynaecol Res. 2005 Dec;31(6):531-4.

BACKGROUND: Metastasis of uterine leiomyosarcoma to the pancreas is extremely rare. CASE: A 46-year-old woman presented with hypermenorrhea and dysmenorrhea and underwent surgery. The histologic and immunohistochemical diagnosis was uterine leiomyosarcoma stage I with no metastasis to the ovaries or the pelvic lymph nodes. The mitotic count was very high. Thereafter, recurrences in the lung and subsequently in the pancreas were detected. The lesions in the lung and pancreas were resected and diagnosed as metastases of uterine leiomyosarcoma based on histology and immunohistochemistry. CONCLUSION: We report an extraordinarily rare case of uterine leiomyosarcoma with metastasis to the pancreas following initial metastasis to the lung, both of which were diagnosed using histology and immunohistochemistry of the specimens obtained at surgery.

Preoperative diagnosis of pancreatic leiomyosarcoma.Int J Pancreatol. 2000 Oct;28(2):97-100.

BACKGROUND: The low incidence of pancreatic leiomyosarcoma is responsible for the small number of cases correctly diagnosed preoperatively, the tumor being frequently confused with benign pancreatic lesions. RESULTS: We describe a symptom free 52-yr-old male bearing an abdominal mass incidentally found at physical examination. Imaging techniques revealed a nonhomogenous large mass at the head of the pancreas that dislodged the portal vein and the superior mesenteric vein. Increased metabolic activity in the tumor area demonstrated by 18F-fluorodeoxyglicose positron emission tomography scan allowed the diagnosis of a malignant lesion. The patient was operated on and a pylorus preserving pancreatoduodenectomy performed. The pathology diagnosis was a low grade leiomyosarcoma. Immunohistochemistry revealed positivity for vimentin and smooth muscle specific actin. The clinical course was uneventful after 2 yr follow-up. CONCLUSION: Pancreatic leiomyosarcoma may be preoperatively diagnosed by image techniques and differentiated from benign lesions by means of fluorodeoxyglicose positron emission tomography scanning (FDGPET).

MR imaging of primary pancreatic leiomyosarcoma.  Br J Radiol. 1998 May;71(845):561-3.

Primary leiomyosarcoma of the pancreas is a rare neoplasm with only 17 reported cases in the literature. Although the ultrasonic and computed tomographic appearances have been described, the magnetic resonance features of pancreatic leiomyosarcomas have not. In this report, we describe the MR characteristics of a primary pancreatic leiomyosarcoma which was an incidental finding on ultrasound. The unenhanced T1 weighted and T2 weighted images were the most useful in tumour localization whereas the enhanced images actually obscured the tumour within the pancreas. Differentiation from the much more commonly occurring adenocarcinoma was impossible.

Fatty replacement of the pancreatic body and tail associated with leiomyosarcoma of the pancreatic head.Pathol Int. 1997 Sep;47(9):633-6.

A case of fatty replacement of the pancreatic body and tail associated with leiomyosarcoma of the pancreatic head is presented. Clinicopathological features as well as differential diagnoses are described with a review of the literature on both leiomyosarcoma and fatty replacement of the pancreas. A possible correlation between the two lesions is also discussed.

Leiomyosarcoma of the pancreas.Int J Pancreatol. 1995 Feb;17(1):95-7.

A 45-yr-old female patient was admitted with signs and symptoms of acute pancreatitis. She had no history of gallstones, alcohol, or trauma. Her CT scan was consistent with a cystic lesion of the pancreas. Because of her presenting symptoms and signs, she was initially treated as an acute pancreatitis. Followup CT scan showed absolutely no change in the cyst. Surgical consultation was requested. A diagnosis of a neoplastic cyst was made, and the patient was taken to surgery where a distal pancreatectomy and splenectomy was performed. Pathology revealed a leiomyosarcoma of the pancreas. Only 15 other cases of leiomyosarcoma of the pancreas have been reported thus far in literature.

Leiomyosarcoma of the pancreas: report of a case diagnosed by fine needle aspiration biopsy.Jpn J Clin Oncol. 1994 Feb;24(1):42-5.

We present a case of pancreatic leiomyosarcoma with liver metastasis diagnosed by liver tumor biopsy. A 66-year-old man had a pancreatic tumor and hepatic tumors, which were hypoechoic and mixed echoic on ultrasonography, respectively. They were stained in enhanced computed tomography and angiography. A fine needle aspiration biopsy of the liver tumor was performed, and the pathological examination of the biopsied specimen suggested the tumor cells to have originated from smooth muscle. Under a diagnosis of pancreatic leiomyosarcoma with liver metastasis, the patient was treated with several anticancer agents. The tumor, however, spread to multiple organs and he dies of the disease two years nine months after the start of treatment. The diagnosis of pancreatic leiomyosarcoma was confirmed by autopsy.

Leiomyosarcoma of the pancreas. Report of a case and review of the literature.Am J Surg Pathol. 1981 Sep;5(6):597-602.

A case of leiomyosarcoma of the pancreas, occurring in a 44-year-old male, is reported. With a preoperative diagnosis of cystadenocarcinoma, the patient underwent surgical resection of a cystic tumor in the head of the pancreas. There were no metastases noted at the time. The resected specimen was diagnosed histologically as a smooth muscle tumor originating from the pancreas. It was seemingly benign on the basis of the mitotic counts and the degree of cellular atypism. However, the tumor proved to be malignant; the patient died with metastases to the liver. The tumor at autopsy also had few mitoses (0-1/10 hpf). The previously reported cases were reviewed and the limitations of histological diagnosis in assessing malignant potential of smooth muscle tumors are discussed.

Anatomy of Normal Pancreas ; Normal Islets of Langerhans ; The Apud Concept ; 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 ; Herpes Simplex Pancreatitis Diabetes Mellitus ; Neoplasms of the Endocrine Tumours Islet Cell Tumours ; Glucagonomas  ; Insulinomas  ; Somatostatinoma ; VIPomas ; Pancreatic Polypeptide-Secreting Tumours ;  Enterochromaffin Cell (Carcinoid) Tumours ; Pancreatic Gastrinoma ; Corticotropinoma ;  Multiple Endocrine Neoplasia (MEN) Syndrome ; Carcinoma of the Pancreas ; Exocrine Pancreatic Tumours ;  Paediatric Pancreatic Tumours ;  Acinar cell carcinoma ; Pancreatoblastoma ; Adenosquamous Carcinoma of the Pancreas ; Intraductal Papillary Mucinous Tumour ;   Serous Cystic Tumours  ; Solid Pseudopapillary Tumour;  Mucinous Non-Cystic and Signet-Ring Cell Carcinoma ; Undifferentiated (anaplastic) carcinoma ; Undifferentiated carcinoma with osteoclast-like giant cell ; Oncocytic carcinoma  ; Clear cell carcinoma ; Microglandular adenocarcinoma ; Carcinoma with mixed differentiation ;
 
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A resected case of giant leiomyo sarcoma of the pancreas.J Gastro enterol. 1994 Apr;29(2):223-7.

Leiomyosarcoma of the pancreas is very rare. We report a case of giant leiomyosarcoma of the pancreas treated by distal pancreatectomy. A 53-year-old female was admitted with an large abdominal mass. Computed tomography, magnetic resonance imaging, and ultrasonography revealed a huge tumor adjacent to the pancreas. However, we could not identify the primary organ with these imagings. Angiographic findings strongly suggested that the tumor originated from the pancreas, as main feeding arteries arose from the great pancreatic artery. Fourteen days after trans-catheteric arterial embolization, we performed a distal pancreatectomy with splenectomy; the patient's postoperative course was uneventful. Histologically, we confirmed the diagnosis as a leiomyosarcoma originating from the pancreas.

Leiomyosarcoma of the pancreas. Virchows Arch A Pathol Anat Histopathol. 1993;422(5):419-22.

We report a case of leiomyosarcoma of the pancreas. A spindle cell pattern at light microscopy, immunocytochemical reactivity with desmin, alpha-1-antitrypsin, vimentin and actin and ultrastructural features of smooth muscle differentiation help to establish the diagnosis.

Primary leiomyosarcoma of the pancreas.Nippon Igaku Hoshasen Gakkai Zasshi. 1990 Oct 25;50(10):1215-23.

Primary leiomyo sarcoma of the pancreas is a very rare malignant tumor, with only 12 cases reported. A case of primary pancreatic leiomyo sarcoma in a 55 year-old female with diabetes mellitus is described. US and CT demonstrated tumor masses in the head and tail of the pancreas. By angiography, abundant tumor vessels corresponding to the pancreatic tumor masses were revealed in the pancreas. The patient underwent surgical resection of the tumor in the tail of the pancreas, and then, microscopically it was diagnosed as leiomyosarcoma. The tumor in the head of the pancreas was not resected on account of the presence of diabetes mellitus and the possibility of poor prognosis. Macroscopically, it seemed to be the same as the tumor in the pancreatic tail. By operative findings, there was invasion from the pancreatic head to the duodenum and there was nothing to justify suspicion of the extra-ductal growing type of primary leiomyosarcoma of the duodenum. There were no metastases noted at the time.

Pancreatic leiomyosarcoma. Case report with immunohistochemical and flow cytometric studies.Virchows Arch. 1998 May;432(5):469-72.

A leiomyosarcoma originating from the pancreas of a 57-year-old man is presented. A 6x5x4 cm tumour was located in the head region, and the patient underwent surgical palliation. Immuno histochemical studies excluded an epithelial origin; a myogenic origin was suggested by strong vimentin and smooth muscle actin positivity. Flow cytometric analysis revealed an aneuploid pattern (DNA index: 1,561). The patient died with widespread metastases 7 month after the operation.


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