testicular Sertoli cell tumor
Definition: Sex cord stromal tumor composed of cells recapitulating Sertoli cells.
Clinical Features
In the descended testis of normal individuals.
Sometimes associated with gynecomastia.
Microscopy
Tubular formations
- lined by elongated cells having the appearance of Sertoli cells
- In other areas, the tumor is solid and can be confused with seminoma
- Cytoplasm:
- moderate to abundant
- pale to intense eosinophilic quality
- large cytoplasmic vacuoles in ≈ 50% of cases
- Exceptionally accompanied by a heterologous sarcomatous component
- Sertoli cell adenoma with sarcomatoid features.
- The Sertoli cell nature of the tumor is apparent.
Features suggesting malignancy, particularly when occurring together include:
- mitotic figures
- pleomorphism
- large tumor size
- necrosis
Immunohistochemistry
Diagnostically more significant is frequent positivity for:
- inhibin
- CD99
- anti-Müllerian hormone (AMH) (allegedly produced only by Sertoli cells and granulosa cells)
Reactivity for:
- vimentin
- keratin
- α1-antitrypsin
- neuron-specific enolase (NSE)
Negative for:
- PLAP
- CD117 (c-kit)
Diagnosis
Diagnostic feature is the presence of tubular formations lined by elongated cells having the appearance of Sertoli cells.
Testicular tumors reported as androblastomas and as tumors of specialized gonadal stroma probably belong to the same or a closely related category, the Sertoli cell features not always being apparent.
Misinterpretation of a Sertoli cell tumor as a seminoma is more likely with malignant forms.
Variants
Large Cell Calcifying Sertoli Cell Tumor
- Usually under 20 years of age
- Commonly: bilateral and multifocal
- cardiac myxomas
- spotty pigmentation of skin
- Often part of Carney syndrome, which may also include:
- testicular Leydig cell tumors
- pituitary tumors
- pigmented nodular hyperplasia of the adrenal cortex
- other abnormalities
- Usually immunoreactive for both α and β subunits of S-100 protein (a calcium-binding protein):
- may be related to the characteristic calcification
- a useful tool in the differential diagnosis with Leydig cell tumor
sclerosing Sertoli Cell Tumor
- adults
- Well-demarcated
- Hard
- Yellow–white to tan
- No evidence of estrogen production
- No malignant behavior in any reported case
Differential diagnosis
seminoma
- The distinction of Sertoli cell tumors from seminoma is critical to ensure proper treatment.
- The nested growth pattern, prominence of clear cells, lymphoid infiltrate, inconspicuous tubular differentiation, cytoplasmic glycogen, and prominent nucleoli caused these tumors to be mistaken for seminomas.
- The smaller, less pleomorphic nuclei of Sertoli cell tumors, their lower mitotic rate, and the absence of intratubular germ cell neoplasia are helpful differential features.
- Immunohistochemistry is a useful adjunct in confirming the diagnosis of Sertoli cell tumor, but only if the overlapping features are appreciated by conventional microscopy and the diagnosis of Sertoli cell tumor included in the differential.
testicular yolk Sac Tumor
testicular embryonal Carcinoma
Variants
testicular malignant Sertoli cell tumor
Management
Orchiectomy
Surgical excision of metastatic lesions
Radiation therapy and chemotherapy have not proved particularly effective.
Prognosis
≈ 10% behave in a malignant fashion
Credits
References
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Malignant Sertoli cell tumors of the testis: a study of 13 examples of a neoplasm frequently misinterpreted as seminoma. Henley JD, Young RH, Ulbright TM. Am J Surg Pathol. 2002 May;26(5):541-50. PMID: #11979085#
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