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Primary leiomyosarcoma of the pancreas: report of a case treated by local excision and review of the literature.

Milanetto AC, Liço V, Blandamura S, Pasquali C - Surg Case Rep (2015)

Bottom Line: The patient is alive and asymptomatic 19 months after surgery, without evidences of disease.In the English literature, only 44 cases of primary pancreatic leiomyosarcoma have been reported.If a pancreatic mass suspected for primary pancreatic leiomyosarcoma has no adjacent organ/vessel invasion or distant metastases, surgical resection is the therapy of choice.

View Article: PubMed Central - PubMed

Affiliation: Pancreatic and Digestive Endocrine Surgical Unit-Department of Surgery, Gastroenterology and Oncology, University of Padua, Via Giustiniani, 2-35128, Padua, Italy. ac.milanetto@email.it.

ABSTRACT

Background: First described by Ross in 1951, primary pancreatic leiomyosarcoma is a rare mesenchymal tumour of the pancreas, with nonspecific clinical and radiological features and a poor prognosis, if unresectable.

Case report: A 60-year-old woman presented with abdominal pain. Magnetic resonance imaging (MRI) and computed tomography (CT) scan detected a dishomogeneous egg-shaped 8-cm mass, arising from the pancreatic head, with duodenal compression, without dilation of the Wirsung duct. (18)F-FDG positron-emission tomography (PET)-CT showed a moderate tracer uptake, and the endoscopic ultrasound (US) showed a hypoechoic lesion, arising from the duodenal wall, suspected to be a gastrointestinal stromal tumour (GIST). CEA, CA19-9, NSE, and chromogranin A were normal. At the surgical exploration, a 10-cm mass, adherent to the anterior aspect of the pancreatic head, was found. The lesion was easily separable from the duodenal wall and was totally excised. The frozen intraoperative examination showed a mesenchymal tumour, with spindle-shaped cells, suggesting that a GIST diagnosis was likely. Postoperative course was uneventful. Histology and immunohistochemistry demonstrated a well-differentiated leiomyosarcoma, with five to six mitotic counts per 10 high-power field (HPF) and proliferative index (MIB-1) 10 % (grade 2 according to Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC)), with positive smooth muscle actin, desmin, and caldesmon but negative CD117 (c-kit) and S-100. The patient is alive and asymptomatic 19 months after surgery, without evidences of disease.

Conclusions: In the English literature, only 44 cases of primary pancreatic leiomyosarcoma have been reported. If a pancreatic mass suspected for primary pancreatic leiomyosarcoma has no adjacent organ/vessel invasion or distant metastases, surgical resection is the therapy of choice.

No MeSH data available.


Related in: MedlinePlus

Histology and immunohistochemical analysis. Haematoxylin-eosin stain (original magnification ×50) (a). Mib1 (proliferative index) (b). Strong immunoreactivity to smooth muscle actin (c). No immunoreactivity to CD117 (c-kit) (d)
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Fig3: Histology and immunohistochemical analysis. Haematoxylin-eosin stain (original magnification ×50) (a). Mib1 (proliferative index) (b). Strong immunoreactivity to smooth muscle actin (c). No immunoreactivity to CD117 (c-kit) (d)

Mentions: Histology revealed a well-differentiated leiomyosarcoma (Fig. 3a), composed by spindle cells with a fasciculated growth pattern, with five to six mitotic counts per 10 high-power field (HPF) and proliferative index (MIB-1) 10 % (grade 2 according to (FNCLCC) system [35]) (Fig. 3b). Surgical margins were negative, and no pancreatic tissue was included in the specimen.Fig. 3


Primary leiomyosarcoma of the pancreas: report of a case treated by local excision and review of the literature.

Milanetto AC, Liço V, Blandamura S, Pasquali C - Surg Case Rep (2015)

Histology and immunohistochemical analysis. Haematoxylin-eosin stain (original magnification ×50) (a). Mib1 (proliferative index) (b). Strong immunoreactivity to smooth muscle actin (c). No immunoreactivity to CD117 (c-kit) (d)
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4595416&req=5

Fig3: Histology and immunohistochemical analysis. Haematoxylin-eosin stain (original magnification ×50) (a). Mib1 (proliferative index) (b). Strong immunoreactivity to smooth muscle actin (c). No immunoreactivity to CD117 (c-kit) (d)
Mentions: Histology revealed a well-differentiated leiomyosarcoma (Fig. 3a), composed by spindle cells with a fasciculated growth pattern, with five to six mitotic counts per 10 high-power field (HPF) and proliferative index (MIB-1) 10 % (grade 2 according to (FNCLCC) system [35]) (Fig. 3b). Surgical margins were negative, and no pancreatic tissue was included in the specimen.Fig. 3

Bottom Line: The patient is alive and asymptomatic 19 months after surgery, without evidences of disease.In the English literature, only 44 cases of primary pancreatic leiomyosarcoma have been reported.If a pancreatic mass suspected for primary pancreatic leiomyosarcoma has no adjacent organ/vessel invasion or distant metastases, surgical resection is the therapy of choice.

View Article: PubMed Central - PubMed

Affiliation: Pancreatic and Digestive Endocrine Surgical Unit-Department of Surgery, Gastroenterology and Oncology, University of Padua, Via Giustiniani, 2-35128, Padua, Italy. ac.milanetto@email.it.

ABSTRACT

Background: First described by Ross in 1951, primary pancreatic leiomyosarcoma is a rare mesenchymal tumour of the pancreas, with nonspecific clinical and radiological features and a poor prognosis, if unresectable.

Case report: A 60-year-old woman presented with abdominal pain. Magnetic resonance imaging (MRI) and computed tomography (CT) scan detected a dishomogeneous egg-shaped 8-cm mass, arising from the pancreatic head, with duodenal compression, without dilation of the Wirsung duct. (18)F-FDG positron-emission tomography (PET)-CT showed a moderate tracer uptake, and the endoscopic ultrasound (US) showed a hypoechoic lesion, arising from the duodenal wall, suspected to be a gastrointestinal stromal tumour (GIST). CEA, CA19-9, NSE, and chromogranin A were normal. At the surgical exploration, a 10-cm mass, adherent to the anterior aspect of the pancreatic head, was found. The lesion was easily separable from the duodenal wall and was totally excised. The frozen intraoperative examination showed a mesenchymal tumour, with spindle-shaped cells, suggesting that a GIST diagnosis was likely. Postoperative course was uneventful. Histology and immunohistochemistry demonstrated a well-differentiated leiomyosarcoma, with five to six mitotic counts per 10 high-power field (HPF) and proliferative index (MIB-1) 10 % (grade 2 according to Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC)), with positive smooth muscle actin, desmin, and caldesmon but negative CD117 (c-kit) and S-100. The patient is alive and asymptomatic 19 months after surgery, without evidences of disease.

Conclusions: In the English literature, only 44 cases of primary pancreatic leiomyosarcoma have been reported. If a pancreatic mass suspected for primary pancreatic leiomyosarcoma has no adjacent organ/vessel invasion or distant metastases, surgical resection is the therapy of choice.

No MeSH data available.


Related in: MedlinePlus