Primary leiomyosarcoma of the pancreas

Abstract

Primary sarcomas of the pancreas are extremely rare, accounting for 0.1% of malignant pancreatic (non-islet) neoplasms. Pancreatic leiomyosarcoma is a highly aggressive malignancy that spreads in a similar manner to gastric leiomyosarcoma, i.e., by adjacent organ invasion, hematogenous spread, and lymph node metastasis. These tumors are large at the time of diagnosis and are usually found at an advanced stage. We report a case of a 70-year-old female with intermittent right upper quadrant abdominal discomfort. Radiological, histopathological, and immunohistochemical studies revealed the tumor to be a primary leiomyosarcoma of the pancreas. Herein, we describe a patient with a primary leiomyosarcoma of the pancreas who presented with clinical and radiological findings indicative of a mass in the pancreatic head.

Keywords: Leiomyosarcoma; Pancreas; Primary.

Fig. 1

Abdominal computed tomography (CT) scan shows growth of the tumor, which is heterogeneously enhanced by contrast media (A, white arrow). CT scan shows the tumor, which is abutting to the inferior vena cava (B, black arrow). Magnetic resonance imaging shows a 5.3 × 4 cm heterogenously enhanced mass (C, D).

Fig. 2

Whole body [¹⁸F]-fluoro-deoxy glucose positron emission tomography/computed tomography demonstrates a 5.6 × 3.8 cm size hypermetabolic mass arising from the pancreatic head in the retropancreatic space.

Fig. 3

The gross pathological examination reveals a 5 × 5 cm multiseptated mass in the pancreatic head. The cut surface of the tumor is whitish and shows signs of internal hemorrhage and partial myxoid change (A). Interface of tumor (left) and compressed adjacent pancreatic tissue with fibrosis (B, H&E, ×40). The mass contains interlacing spindle-shaped cells with varying degrees of pleomorphism and atypia (C, H&E, ×200). The immunohistochemical findings show strong immunoreactivity of the tumor cells to actin (D, labeled streptavidin-biotin, original magnification ×200).