Testis & epididymis
Sex cord-stromal tumors
Adult granulosa cell tumor

Editorial Board Member: Maria Tretiakova, M.D., Ph.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Kristine M. Cornejo, M.D.

Topic Completed: 1 February 2021

Minor changes: 12 April 2021

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PubMed Search: Adult granulosa cell tumor testis pathology

Related topics: Juvenile granulosa cell tumor

Kristine M. Cornejo, M.D.
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Cite this page: Cornejo KM. Adult granulosa cell tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testisgranulosa.html. Accessed April 15th, 2021.
Definition / general
  • Sex cord stromal tumor resembling granulosa cells of the Graafian follicles of the ovary
Essential features
  • Rare, low grade, indolent sex cord stromal tumor; 10% have malignant potential
  • Most common architectural patterns are diffuse / solid, insular and spindled; pathognomonic Call-Exner bodies, which are a part of the microfollicular pattern, are less common
  • Morphologically, the tumor cells characteristically display uniformly round to ovoid nuclei with conspicuous, longitudinal nuclear grooves
  • FOXL2 gene mutations occur in a minor subset of cases, which is substantially less frequent than in its ovarian counterpart
ICD coding
  • ICD-O: 8620/1 - Granulosa cell, adult type
  • ICD-O: 8620/3 - Granulosa cell, malignant
  • ICD-10: D40.1 - Neoplasm of uncertain behavior of unspecified testis
  • ICD-10: C62.9 - Malignant neoplasm of testis, unspecified whether descended or undescended
Epidemiology
Sites
  • Testicular parenchyma
Pathophysiology
  • Derived from granulosa cells in which FOXL2, a granulosa cell expressed gene, plays a role in regulating cell fate
  • Missense mutation (402C → G, C134W) causes a negative effect by dysregulating gene expression, leading to increased cell proliferation and reducing apoptosis (Oncol Lett 2020;20:293)
Etiology
  • Unknown
Clinical features
Diagnosis
  • Imaging studies (e.g. ultrasound) may identify a testicular mass
  • Definitive diagnosis is made by histopathologic examination only
Laboratory
Radiology description
  • Nonspecific findings but typically shows a well demarcated, hypoechoic mass with hyperechoic foci and calcification (Radiol Case Rep 2015;3:188)
Radiology images

Images hosted on other servers:
Hypoechoic lesion

Hypoechoic lesion

Hypoechoic lesion and increased vascularity

Hypoechoic lesion /
increased vascularity

Increased vascularity

Increased vascularity

Prognostic factors
Case reports
Treatment
Gross description
  • Mean size: 2.8 cm (0.5 - 6.0 cm) (Am J Surg Pathol 2014;38:1242)
  • Well circumscribed, predominantly solid; occasionally solid and cystic
  • Lobulated, yellow-tan or tan-white cut surface
  • Infiltrative borders / extratesticular involvement, hemorrhage and necrosis are rarely seen and often associated with more aggressive behavior
Gross images

Contributed by Kristine M. Cornejo, M.D.
Tan-yellow solid mass

Tan-yellow solid mass

Lobulated tan-yellow mass

Lobulated tan-yellow mass

Microscopic (histologic) description
  • Low power: typically displays a nodular / lobulated appearance
  • High power: tumor cells contain uniformly round to ovoid nuclei with conspicuous longitudinal nuclear grooves, indistinct cell borders, variable amounts of eosinophilic cytoplasm
  • Most common patterns: diffuse / solid, insular and spindled (Am J Surg Pathol 2014;38:1242)
    • Diffuse / solid: sheets of round cells
    • Insular: nests of tumor cells surrounded by stroma
    • Spindled: sheets of fusiform cells
    • Cords / trabecular: regularly to irregularly arranged thin cords or thick columns of tumor cells
    • Water silk / gyriform: interweaving cords and undulating columns of cells
    • Microfollicular: numerous small spaces with hyalinized basement membrane material or eosinophilic fluid (Call-Exner bodies)
    • Herringbone / fascicular: spindled cells with fibrocollagenous stroma
    • Pseudopapillary: tumor cells surrounding fibrovascular cores
    • Palisading: tumor cells arranged parallel to each other, resembling a fence
    • Luteinized: more abundant clear to eosinophilic cytoplasm with rounded nuclei
  • Admixed with varying amounts of fibrocollagenous or edematous stroma
  • Mitotic count: mean 4.9/10 high power fields (0 - 18/10 high power fields); suggests the presence of mitoses are not of prognostic significance (Am J Surg Pathol 2014;38:1242)
  • May contain hemorrhage or hemosiderin deposition
  • Rarely displays necrosis and lymphovascular invasion; features of aggressive behavior
  • Tumor often infiltrates between seminiferous tubules and may involve rete testis and tunica albuginea
    • Rarely involves paratesticular or extratesticular tissue
Microscopic (histologic) images

Contributed by Kristine M. Cornejo, M.D.
Nodular appearance

Nodular appearance

Nuclear grooves

Nuclear grooves

Diffuse pattern

Diffuse pattern

Spindled pattern

Spindled pattern

Corded pattern

Corded pattern

Gyriform / watered-silk pattern

Gyriform / watered silk pattern


Microfollicular pattern (Call-Exner Bodies) Microfollicular pattern (Call-Exner Bodies) Microfollicular pattern (Call-Exner Bodies) Microfollicular pattern (Call-Exner Bodies)

Microfollicular pattern (Call-Exner bodies)

Insular pattern

Insular pattern

Palisading pattern

Palisading pattern


Pseudopapillary pattern

Pseudopapillary pattern

Herringbone / fascicular pattern

Herringbone / fascicular pattern

Leutinized cells

Luteinized cells

Inhibin A stain

Inhibin A stain

Positive stains
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Left testis, radical orchiectomy:
    • Adult granulosa cell tumor (3.0 cm), confined to the testis (see comment)
    • Comment: The tumor is small, well circumscribed and lacks features associated with a more aggressive behavior, such as significant cytologic atypia, conspicuous mitoses, presence of lymphovascular invasion or necrosis.
Differential diagnosis
  • Juvenile granulosa cell tumor:
    • Typically occurs in the first 6 months of life; rare after 1 year of age
    • Associated with ambiguous external genitalia and karyotypes
    • Most common patterns are solid and follicular, in which the follicles are irregular in size and shape lined by granulosa cells with an outer thecal layer; contains mucinous material that is not found in adult granulosa cell tumor and the follicles tend to be more uniform
    • Usually lacks Call-Exner bodies and nuclear grooves
  • Leydig cell tumor:
    • Presents at a similar age (mean: 46.5 years)
    • Similarly presents with sheets or lobular aggregates of cells with abundant eosinophilic cytoplasm arranged with variable amounts of fibrous stroma
    • Cells may be vacuolated and contain Reinke crystals, which are not seen in adult granulosa cell tumors
  • Sertoli cell tumor:
    • Presents at a similar age (mean: 45 years)
    • Displays a variety of patterns, such as trabecular, corded and diffuse, in addition to varying amounts of well formed tubules; the latter is typically not seen in adult granulosa cell tumors and lacks the typical microfollicular pattern or Call-Exner bodies
    • Composed of tumor cells with abundant pale to lightly eosinophilic cytoplasm, which may display nuclear grooves
    • Positive for nuclear beta catenin (Am J Surg Pathol 2015;39:1390)
  • Fibrothecoma:
    • Presents at a similar age (mean: 44 years)
    • Composed of ovoid or spindled cells in varying amounts of collagenous stroma
    • Lacks varied architectural patterns and nuclear grooves
    • Inhibin A positive (patchy to diffuse pattern) (Am J Surg Pathol 2013;37:1208)
  • Sex cord stromal tumor, unclassified:
    • Tumors that have features of indeterminate differentiation; lack findings characteristic of adult granulosa cell tumors or other sex cord stromal tumors
  • Sex cord stromal tumor, mixed:
    • Tumors that contain characteristics of 2 or more tumor subtypes
Board review style question #1

A 47 year old man presented with a right testicular swelling. Histologic examination revealed the tumor shown above. This tumor typically shows which of the following immunoprofiles?

  1. OCT4+ / SALL4+ / inhibin A- / FOX2L-
  2. OCT4- / SALL4+ / inhibin A+ / FOX2L-
  3. OCT4- / SALL4- / inhibin A+ / FOX2L+
  4. OCT4- / SALL4- / inhibin A- / FOX2L-
Board review style answer #1
C. OCT4- / SALL4- / inhibin A+ / FOX2L+. Histology shows Call-Exner bodies, which are pathognomonic for adult granulosa cell tumor. Adult granulosa cell tumor expresses general sex cord stromal tumor immunohistochemical markers, such as inhibin A. FOX2L has also been found to be a marker of sex cord stromal tumors and does not necessarily correlate with a FOX2L gene mutation, which has been identified in a small subset of testicular adult granulosa cell tumors. OCT4 and SALL4 are markers of germ cell tumors.

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Reference: Adult granulosa cell
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