Testis & paratestis

Sex cord stromal tumors

Large cell calcifying Sertoli cell tumor



Last author update: 30 March 2022
Last staff update: 30 March 2022

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PubMed Search: Large cell calcifying Sertoli cell tumor [title]

Stephanie Siegmund, M.D., Ph.D.
Andres Acosta, M.D.
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Cite this page: Siegmund S, Anderson W, Acosta A. Large cell calcifying Sertoli cell tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testissertolilargecell.html. Accessed December 7th, 2022.
Definition / general
Essential features
  • Generally presents as a benign sex cord stromal tumor found predominantly in patients < 20 years old; a subset presents in older patients and may behave aggressively
  • Associated with Carney complex in 20 - 40% of cases due to alterations in the gene PRKAR1A
  • Characterized by intratubular and sheet-like growth of large eosinophilic cells with laminated psammomatous, mulberry-like or dystrophic calcifications, a prominent neutrophilic infiltrate and lymphocytic rim
ICD coding
  • ICD-10: D29.20 - Sertoli cell tumor, male
Epidemiology
  • Male patients, generally < 20 years old
  • 20 - 40% of cases associated with Carney complex due to mutations in PRKAR1A gene (part of Carney complex with spotty cutaneous pigmentation, primary pigmented nodular adrenocortical disease, atrial myxomas, superficial angiomyxomas, malignant melanocytic nerve sheath tumors, blue nevus and thyroid carcinoma)
  • Debatable association with Peutz-Jeghers syndrome (rare reports, may represent intratubular large cell hyalinizing Sertoli cell neoplasia) and neurofibromatosis (Am J Surg Pathol 2021 Dec 16 [Epub ahead of print])
Sites
  • Testis
  • Multifocal and bilateral testicular involvement in > 50% of patients with Carney complex (J Urol 2002;167:1299)
Clinical features
Diagnosis
  • Definitive diagnosis by histopathological examination
Prognostic factors
  • Kratzer et al. proposed malignant prognostic features that include age > 25 years old and 2 or more of the following adverse features (Am J Surg Pathol 1997;21:1271):
    • Size > 4 cm
    • Extratesticular extension
    • Mitotic index > 3/10 high power fields (HPFs)
    • Coagulative tumor necrosis
    • Vascular invasion
    • High grade cytologic atypia
  • Prepubertal status may provide a protective effect (Am J Surg Pathol 2021 Dec 16 [Epub ahead of print])
  • Tumors associated with Carney complex may behave in biologically indolent fashion compared with sporadic tumors (Eur Urol 2001;40:699)
Case reports
Treatment
  • Partial or radical orchiectomy for organ confined disease
  • Retroperitoneal lymph node dissection with possible adjuvant chemotherapy or radiation therapy for metastatic disease (benefit unknown)
Gross description
Gross images

Contributed by Sara Vargas, M.D.
Ill defined testicular nodules

Ill defined testicular nodules



Images hosted on other servers:

Firm, white tan nodules

Tumor

Microscopic (histologic) description
  • Laminated psammomatous and mulberry-like calcifications
  • Prominent neutrophilic infiltrate and lymphocytic rim
  • Large epithelioid cells with eosinophilic cytoplasm, prominent nucleoli and minimal cytologic atypia
  • Cords, trabeculae, nests or sheets in a variably myxoid or fibrous stroma
  • Generally limited to testis with occasional extension into paratesticular tissue, including rete testis and hilar soft tissue
  • Malignant cases more likely to exhibit solid sheet-like growth and frequent nuclear pleomorphism ranging from mild to prominent
Microscopic (histologic) images

Contributed by Stephanie Siegmund, M.D., Ph.D.
Calcifications, neutrophils, myxoid stroma

Calcifications, neutrophils, myxoid stroma

Focus of intratubular tumor

Focus of intratubular tumor

Tumor involving rete testis

Tumor involving rete testis

Mulberry calcifications

Mulberry calcifications

Trabecular architecture

Trabecular architecture


Focal nuclear pleomorphism

Focal nuclear pleomorphism

Neutrophilic infiltrate

Neutrophilic infiltrate

Minimal cytologic atypia

Minimal cytologic atypia

Mulberry calcifications

Mulberry calcifications

PRKAR1A loss by IHC

PRKAR1A loss

Virtual slides

Images hosted on other servers:
Multifocal mass in pediatric testis

Multifocal mass in pediatric testis

Dystrophic calcifications

Dystrophic calcifications

Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Testis and spermatic cord, radical orchiectomy:
    • Large cell calcifying Sertoli cell tumor (1.0 cm) (see comment)
    • Tumor is limited to the testicular parenchyma
    • No necrosis is identified
    • Mitoses number < 1 per 10 HPFs
    • The spermatic cord and cord resection margin are negative for tumor
    • No lymphovascular invasion is identified
    • Background testis with maturing spermatogenesis
    • Comment: The slides demonstrate sheets and cords of large epithelioid cells with eosinophilic cytoplasm and prominent nucleoli. Immunohistochemistry demonstrates the following staining profile in lesional cells: positive staining for inhibin, calretinin, S100, MelanA and beta catenin (cytoplasmic, nonnuclear) and negative staining for PRKAR1A (loss), OCT 3/4 and SALL4. Overall, the findings are consistent with large cell calcifying Sertoli cell tumor. Adverse pathologic features are not identified (e.g., > 3 mitoses per 10 HPF, coagulative necrosis, vascular invasion, size > 4.0 cm, high grade cytologic atypia or extratesticular extension) (Am J Surg Pathol 1997;21:1271). Approximately 20 - 40% of cases are associated with Carney complex and genetic counseling is recommended in the appropriate clinical context.
Differential diagnosis
  • Leydig cell tumor (LCT):
    • Usually solitary and unilateral
    • Frequent Reinke crystals (diagnostic of LCT)
    • Lacks calcifications and neutrophilic infiltrate
    • Retains IHC expression of PRKAR1A
  • Sertoli cell tumor, not otherwise specified (NOS):
    • Similar population of large, polygonal Sertoli cells with eosinophilic cytoplasm
    • Usually lacks calcifications and neutrophilic infiltrate
    • Nuclear localization of beta catenin by IHC
    • Retains IHC expression of PRKAR1A
  • Intratubular large cell hyalinizing Sertoli cell neoplasia:
    • Predominantly intratubular process with expanded tubules and thickened basement membrane
    • Tubules filled by globular eosinophilic basement membrane-like material
    • Generally lacks calcifications
    • Retains IHC expression of PRKAR1A
Board review style question #1

Which of the following features is most consistent with sporadic presentation of large cell calcifying Sertoli cell tumor (LCCSCT), shown in the image, versus a syndromic association (e.g., Carney complex)?

  1. Bilateral disease
  2. Loss of PRKAR1A by IHC
  3. Multifocal disease
  4. Pediatric presentation
  5. Sibling(s) with similar presentation
Board review style answer #1
B. Loss of PRKAR1A by IHC. While PRKAR1A alterations are commonly found in patients with Carney complex, the loss of PRKAR1A is seen in most sporadic as well as syndromic LCCSCT and can frequently be demonstrated by as loss of PRKAR1A by IHC in sporadic tumors. All other listed answers (pediatric onset, bilateral and multifocal disease, sibling(s) with similar presentations) are more commonly associated with LCCSCT occurring in Carney complex patients and would suggest a syndromic association.

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Reference: Large cell calcifying Sertoli cell tumor
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