Adrenal gland and paraganglia
Other adrenal malignancies

Topic Completed: 1 May 2016

Revised: 10 June 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Adrenal leiomyosarcoma

Lan L. Gellert, M.D., Ph.D.
Page views in 2018: 267
Page views in 2019 to date: 254
Cite this page: Gellert LL. Leiomyosarcoma. website. Accessed September 17th, 2019.
Definition / general
  • A malignant neoplasm showing pure smooth muscle differentiation
Essential features
  • Malignant neoplasm showing pure smooth muscle differentiation
  • Characteristic intersecting fascicles of spindle blunt ended cells
  • SMA, desmin and h-caldesmon immunoreactivity in majority of cases
  • Tumors arising in immunocompromised patients have distinctive features
  • Very rare in adrenal gland
  • Often in elderly patients
Clinical features
  • Most present with abdominal or flank pain
Prognostic factors
  • Tend to have poor prognosis
  • Prone to local recurrence and metastasis
  • Prognostic indicators include tumor size and histological grade
  • Tumors arising in immunocompromised patients sometimes behave better
Case reports
  • Surgery is the mainstay of therapy
  • Adjuvant radiation or chemotherapy in advanced disease
Gross description
  • Fleshy grey to white tan mass
  • A whorled appearance may be evident
  • The tumor border often appears well circumscribed
Microscopic (histologic) description
  • Intersecting sharply marginated fascicles of spindle cells
  • Characteristic elongated and blunt ended neoplastic cells
  • Cytoplasm varies from brightly eosinophilic fibrillar to pale
  • Nuclear hyperchromasia and pleomorphism generally notable
  • Most cases in adrenal gland are of conventional type
  • Rare cases of pleomorphic type reported in adrenal gland (World J Surg Oncol 2012;10:64, Urology 2007;70:591)
  • Tumor in HIV+ or post-transplant patients is histologically relatively bland but can have focal primitive appearing round cell component
Microscopic (histologic) images

Images hosted on other servers (not necessarily from adrenal gland):

Intersecting fascicles of spindle cells

Tumor with hyalinization

Elongated and blunt-ended neoplastic cells

Pleomorphic leiomyosarcoma

SMA, desmin stains

Cytology description
  • Fascicles with dispersed cells or stripped nuclei
  • Spindle cells with elongated, blunt ended, segmented nuclei or round / polygonal cells with round or indented nuclei
Positive stains
Negative stains
Molecular / cytogenetics description
  • Karyotype often highly complex with genomic instability
Differential diagnosis
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