Pancreatic leiomyosarcoma: a diagnostic challenge and literature review

Abstract

A 71-year-old woman was referred with abdominal pain and weight loss. An abdominal CT showed a 5-cm heterogeneous mass in the head of the pancreas with involvement of the superior mesenteric vein and artery. Her carcinoembryonic antigen (CEA) and CA 19-9 were normal. Two endoscopic ultrasound/fine needle aspirates (EUS/FNAs) of the mass diagnosed her with a mesenchymal tumour of myogenic origin but did not show features of malignancy. Frozen section analysis of laparoscopic core biopsies also failed to show malignant features, hence requiring an open biopsy which confirmed the diagnosis of pancreatic leiomyosarcoma (PLMS). She was eventually treated with radiotherapy. To our knowledge this is the only case in recent English literature of inoperable locally advanced PLMS that has required an open biopsy to formalise the diagnosis despite prior EUS FNAs. We include a review of the literature, highlighting the deficiencies of various biopsy techniques.

Pancreatic Leiomyosarcoma With Schistosomiasis Hematobia: A Case Report and Literature Review

“…PL is a rare malignant neoplasm, first reported by Ross in 1951 ( 2 ). Fewer than 100 cases of PL have been documented in the literature ( 7 ). The mean age of patients is 53.9 ± 14.7 years old (range = 14–87 years) with an equal incidence rate between men and women ( 3 , 7 ).…”

“…Fewer than 100 cases of PL have been documented in the literature ( 7 ). The mean age of patients is 53.9 ± 14.7 years old (range = 14–87 years) with an equal incidence rate between men and women ( 3 , 7 ). The most common symptoms of PL include abdominal pain, weight loss, epigastric tenderness, and abdominal mass formation ( 8 , 9 ).…”

“…What were the imaging features of PL with Schistosoma japonicum , and why it was misdiagnosed by first MRI? PL is characterized by a large heterogeneous mass with peripheral enhancement via CT scanning while MRI scanning shows hyperintensity of a mass on T2 weighted images with peripheral enhancement ( 7 , 11 , 12 ). In the above case, the main imaging characteristic was a heterogeneous mass with a cystic component due to necrosis at pancreatic body and tail.…”

Malignant biliary obstruction due to metastatic non-hepato-pancreato-biliary cancer

“…Cystic features may lead to misdiagnoses as pseudocysts or cystic neoplasms and to false-negative EUS-FNA results. There are also 23 reports of primary pancreatic carcinosarcoma as well as reports of various subtypes of sarcoma originating in the bile duct as well as the ampulla of Vater[ 190 - 194 ]. More than half of pancreatic carcinosarcomas occur in the pancreatic head, of which about half cause obstructive jaundice[ 190 ].…”

“…There are also 23 reports of primary pancreatic carcinosarcoma as well as reports of various subtypes of sarcoma originating in the bile duct as well as the ampulla of Vater[ 190 - 194 ]. More than half of pancreatic carcinosarcomas occur in the pancreatic head, of which about half cause obstructive jaundice[ 190 ]. Thus, sarcoma should be included in the differential diagnosis of isolated pancreato-biliary tumors even in the absence of a coexisting primary tumor.…”