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Sex cord stromal tumors
Mixed germ cell - sex cord stromal tumors: other

Swapnil U. Rane, M.D.

Topic Completed: 1 August 2013

Revised: 15 December 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Mixed germ cell sex cord stromal tumors testis

Related topics: Mixed germ cell - sex cord stromal tumors: gonadoblastoma

Swapnil U. Rane, M.D.
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Table of Contents
Definition / general | Terminology | Epidemiology | Clinical features | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Comparison of tumors with and without gonadoblastoma | Differential diagnosis
Cite this page: Rane S. Mixed germ cell - sex cord stromal tumors: other. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testismixedother.html. Accessed February 20th, 2020.
Definition / general
  • Gonadal neoplasm composed of germ cells associated with sex cord stromal tumor without any evidence of gonadoblastoma
  • Most cases have been described in ovaries, only a few cases reported in testis
Terminology
  • Also called pflügeroma (Bull Soc Anat Paris 1912;14:403), epitheliomas pflügeriens (Mason P (1923) In: diagnostics de Laboratoire. Maloine Editions, Paris, pp 477), tumor of gonadal analage with female differentiation (Arch Anat Cytol Pathol 1990;38:11)
Epidemiology
  • Rare; occur in gonads of phenotypically and genetically normal subjects
  • Usually ages 30 - 69 years in normally descended testis
Clinical features
  • Presents as asymptomatic, gradually enlarging scrotal mass, often over many years
  • Normal serum AFP, CEA, β-hCG
  • Benign behavior with no metastases reported in testicular or ovarian tumors
Case reports
Treatment
  • Radical orchidectomy; nodal dissection probably not indicated unless clinical involvement
Gross description
  • Well circumscribed, white, soft, with compressed normal testicular parenchyma at periphery
  • Variable cystic change (Virchows Arch 2006;448:612)
  • No involvement of testicular adnexa or tunica albuginea
Microscopic (histologic) description
  • Germ cells arranged in groups or as solitary units within background of dense, diffuse proliferation of spindle shaped sex cord stromal cells
  • Germ cell groups are not uniformly scattered; microscopic foci can show predominance of either cell type
  • Germ cells:
    • Variable size but abundant clear cytoplasm, variably blastic nucleus with fine chromatin, 1+ inconspicuous nucleoli
    • Variable grade; may have targetoid appearance if small nuclei and abundant cytoplasm
    • Unlike ovarian counterpart, squaring off of nuclei is typically absent
  • Sex cord stromal elements:
    • Spindle cells with nuclei showing longitudinal clefts and inconspicuous nucleoli
    • Can show palisading
    • Tumor cells may be intermixed with dense collagen
    • Cells may be arranged around collagen forming Call-Exner-like bodies
  • No concentric calcifications, Leydig cells, lutein cells
Microscopic (histologic) images

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Testicular tumor and other images (scroll down)

Positive stains
  • Germ cells: variable AE1 / AE3 in a paranuclear dot pattern; variable Ki67 (depending on grade)
  • Sex cord stromal elements: inhibin
Negative stains
Molecular / cytogenetics description
  • Ovarian tumors have c-kit mutations (seen in seminomas) and excess genetic material on #12p (seen in all germ cell tumors) but no genetic aberrations identified in testicular tumors (Virchows Arch 2006;448:612)
Comparison of tumors with and without gonadoblastoma
  • MSGCT - gonadoblastoma:
    1. Associated with disorders of sexual development (gonadal dysgenesis [pure or mixed], altered karyotype, pseudohermaphrodites with streak gonads)
    2. Leydig-like cells and lutein cells
    3. Virilization
    4. Proliferative activity only in germ cells

  • MGSCT - not gonadoblastoma:
    1. Genetically and phenotypically normal individuals
    2. No Leydig-like cells and lutein cells
    3. No endocrine effects usually; if present, usually feminization
    4. Proliferative activity in sex cord stromal and germ cell components
Differential diagnosis
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