Primary leiomyosarcoma of the pancreas: Study of 9 cases and review of literature

Hongying Zhang, Mark H. Jensen, Michael B. Farnell, Thomas Christopher Smyrk, Lizhi Zhang

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

Primary pancreatic leiomyosarcomas are rare lesions and not well described, yet they are the most common primary pancreatic sarcoma. English-language medical literature reports 29 cases as single cases or small series. A systematized nomenclature of medicine (SNOMED) search of Mayo Clinic surgical pathology files from 1994 to 2006 identified 22 primary pancreatic leiomyosarcomas. Nine patients with pancreatic leiomyosarcoma were diagnosed and treated at our institution (5 males and 4 females; mean age at diagnosis, 63 y; range, 39 to 87 y) are described, with a literature review. In situ hybridization for Epstein-Barr virus (EBV)-encoded RNA (EBER) was conducted in all cases to exclude EBV-associated smooth muscle tumor (EBV-SMT). Seven of the 9 patients presented with abdominal pain, weight loss, and jaundice. Seven tumors (mean, 10.7 cm; range, 1.0 to 30 cm) were located in the pancreatic head and 2 in the tail. Histologic findings of primary pancreatic leiomyosarcomas (7 spindle and 2 epithelioid) were similar to leiomyosarcomas of other sites. All tumors stained positive for smooth muscle actin and desmin and negative for KIT. No case showed EBER positivity. Pancreaticoduodenectomy was done in 4 patients; 3 patients had palliative procedures, and 2 had biopsy only. No lymph node metastasis was identified in 4 resected tumors, but liver metastases were present in 4 patients. All patients died; 5 deaths were known to be disease related (overall mean survival, 31 months; range, 5 to 98 mo). Historical cases showed similar clinicopathologic findings. These pancreatic leiomyosarcoma lesions have the same morphologic features as their counterparts of other sites. EBER testing should be conducted-especially for pediatric patients-to rule out EBV-SMT. The tumor is likely to metastasize to liver but not regional lymph nodes. Extensive surgical resection should be advocated, even when morphologic results show a low-grade lesion.

Original languageEnglish (US)
Pages (from-to)1849-1856
Number of pages8
JournalAmerican Journal of Surgical Pathology
Volume34
Issue number12
DOIs
StatePublished - Dec 2010

Fingerprint

Leiomyosarcoma
Pancreas
Human Herpesvirus 4
RNA
Neoplasms
Systematized Nomenclature of Medicine
Lymph Nodes
Smooth Muscle Tumor
Neoplasm Metastasis
Surgical Pathology
Pancreaticoduodenectomy
Desmin
Liver
Jaundice
Sarcoma
Abdominal Pain
In Situ Hybridization
Smooth Muscle
Actins
Weight Loss

Keywords

  • leiomyosarcoma
  • pancreatic neoplasms
  • pancreaticoduodenectomy

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery

Cite this

Primary leiomyosarcoma of the pancreas : Study of 9 cases and review of literature. / Zhang, Hongying; Jensen, Mark H.; Farnell, Michael B.; Smyrk, Thomas Christopher; Zhang, Lizhi.

In: American Journal of Surgical Pathology, Vol. 34, No. 12, 12.2010, p. 1849-1856.

Research output: Contribution to journalArticle

Zhang, Hongying ; Jensen, Mark H. ; Farnell, Michael B. ; Smyrk, Thomas Christopher ; Zhang, Lizhi. / Primary leiomyosarcoma of the pancreas : Study of 9 cases and review of literature. In: American Journal of Surgical Pathology. 2010 ; Vol. 34, No. 12. pp. 1849-1856.
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