Primary Leiomyosarcoma of the Pancreas—a Case Report and a Comprehensive Review

Abstract

PurposePrimary mesenchymal tumors of the pancreas are rare, with leiomyosarcomas the most encountered entities among the pancreatic sarcomas. With few exceptions, single case reports published over the last six decades constitute the entire scientific literature on this topic. Thus, evidence regarding clinical decision-making is scant.MethodsBased on a case report and an extensive literature search in PubMed, we discuss the clinical aspects and current management of this rare malignancy.ResultsWe identified only two papers with more than a single case presentation; these institutional patient series were limited to five and nine patients. Additionally, a few papers sought to summarize the individual case reports published in the English and/or Chinese language. The clinical presentation is rather non-specific. Moreover, modern imaging modalities are insufficiently accurate to diagnose leiomyosarcoma of the pancreas. Treatment goals include a complete resection with free margins. Proper morphologic examination using immunohistochemistry and the application of a grading system are clinically important for prognostication. The efficacy of adjuvant treatments has not been established.ConclusionPrimary pancreatic leiomyosarcoma is extremely rare, and the scientific literature is primarily based on single case reports. Conclusions on management and prognosis should be drawn with caution. A multidisciplinary team consultation is warranted to discuss a thorough individual treatment plan based on the available scientific literature, despite its low evidence level.

Irreversible electroporation in a case of pancreatic leiomyosarcoma: a novel weapon versus a rare malignancy?

“…It is well known that pancreatic adenocarcinoma is the most common histologic type of malignancy of the pancreas, representing more than 90–95% of all pancreatic tumors. Pancreatic sarcomas are encountered with a reported incidence of about 0.1% of all malignant pancreatic tumors, with leiomyosarcoma being the most common type reported [1]. Pancreatic leiomyosarcoma, which was first described by Ross in 1951, is an extremely rare tumor with nonspecific clinical and radiological features and also poor prognosis [2].…”

“…Pancreatic leiomyosarcomas represent 0.1% of all malignant pancreatic tumors. They seem to be more prevalent in the fifth decade of life, with nonspecific symptoms at their initial presentation [1]. The tumor is usually aggressive in nature, and 25% of patients suffer from distant metastases at the time of diagnosis [2].…”

“…Median survival is reported to be around 48 months with a 5-year associated survival of about 43.9% [3]. A complete resection is considered the sole and gold standard treatment for this type of malignancy [1]. However, in cases of advanced and/or metastatic pancreatic leiomyosarcoma, an established consensus for the management of these tumors does not exist, whereas efficacy of adjuvant and neo-adjuvant treatment with chemotherapy and/or radiation remains questionable.…”

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

“…Therefore, extensive surgical resection should be advocated, even when morphological results show a low- grade lesion [5] , [8] . It is particularly important to recognize that complete resectability of the lesion is the only viable hope for improving long-term survival of the patients [2] , [4] , [5] .…”

“…The first report of pancreatic leiomyosarcoma was described by Ross in 1951 [1] , and only 52 cases have been reported in the English literature [2] , [3] , [4] . The tumor is considered to derive from the vasculature or ductal smooth muscle in the pancreas [5] , [6] , [7] , [8] .…”