A case report of primary pancreatic leiomyosarcoma requiring six additional resections for recurrences

Abstract

Introduction: Primary pancreatic leiomyosarcoma is extremely rare. We report a case in which six additional resections were required to treat recurrent tumors in a 5-year period following the primary operation.

Presentation of case: A 69-year-old man presented with a pancreatic tumor. Abdominal computed tomography scan showed a large heterogeneous mass with a necrotic area arising from the pancreatic body. We performed distal pancreatectomy, splenectomy, and wide resection of the transverse mesocolon. Histopathological examination confirmed the diagnosis of a pancreatic leiomyosarcoma. We repeatedly performed surgery on recurrent tumors.

Discussion: Primary pancreatic leiomyosarcoma is considered to be a highly aggressive malignancy. The most effective treatment is complete surgical resection with tumor-free margins. Even when tumors recur, it is possible to improve the prognosis by further resection.

Conclusion: Long-term survival is achievable by repeated resection of recurrent tumors.

Keywords: Dissemination; Leiomyosarcoma; Metastasis; Pancreas; Recurrence.

Fig. 1

Radiologic imaging modalities in the diagnosis of leiomyosarcoma. Coronal (a) and axial (b) abdominal CT scans showing an 8.2 × 7.2 cm large heterogeneous mass with a central necrotic area in the body of the pancreas (arrows). (c) Ultrasonography showing a 9.7 × 7.2 cm large hypoechoic mass with cystic change in the body of the pancreas. (d) PET-CT showing a strong accumulation in the body of the pancreas (arrow).

Fig. 2

Resected specimen and histological analysis. (a) Macroscopically, there was an 8 × 7 cm tumor in the body of the pancreas (arrow). (b) The cross-section of the tumor is whitish and shows signs of internal hemorrhage and partial myxoid changes. (c) Histological examination showing interlacing bundles of spindle-shaped cells (Hematoxylin and Eosin staining x10). (d) Spindle-shaped cells with varying degrees of pleomorphism and a few mitotic figures. (Hematoxylin and Eosin staining x40).

Fig. 3

Immunohistochemical examination. Immunohistochemical analysis showing positive staining for α-smooth muscle actin (a), caldesmon (b), and HHF35 (c) (a → cx20).

Fig. 4

Thoracic and abdominal CT imaging of recurrent tumors. (a) Abdominal CT scan revealing a metastatic tumor measuring 4.5 cm in the caudal resion of the stomach of middle part of the greater curvature side (arrow) at the time of the first re-operation. (b) Abdominal CT scan showing a liver metastasis measuring 5 cm in diameter (arrow) at the time of the second re-operation. Thoracic CT scan at the third and fourth re-operations shows three metastatic tumors in the upper and lower lobes of the right lung (c,d), and in the lower lobe of the left lung (d) (arrows).

Fig. 5

CT imaging of recurrent tumors. Thoracic and abdominal CT scans at the fifth and sixth re-operations show multiple metastatic tumors in the abdominal cavity (a), chest wall (b, c), and buttocks (d, e) (arrows).