Adrenal gland and paraganglia
Other adrenal malignancies
Leiomyosarcoma

Author: Lan L. Gellert, M.D., Ph.D. (see Authors page)

Revised: 24 May 2016, last major update May 2016

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Adrenal leiomyosarcoma

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
Epidemiology
  • Very rare in adrenal gland
  • Often in elderly patients
Etiology
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
Treatment
  • 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
Micro 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
Micro 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