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testicular Sertoli cell tumor

Wednesday 24 February 2010

Sertoli cell tumor of the testis

Definition: Sex cord stromal tumor composed of cells recapitulating Sertoli cells.

Sertoli cell tumor has tubules lined by epithelial cells, but may also be diffuse or trabecular. They do not produce hormones. Large cell calcifying tumor is associated with Carney complex syndrome and Peutz-Jeghers syndrome.

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:

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

- pathconsultddx

References

- Testicular sertoli cell tumours and relative sub-types. Analysis of clinical and prognostic features. Giglio M, Medica M, De Rose AF, Germinale F, Ravetti JL, Carmignani G. Urol Int. 2003;70(3):205-10. PMID: 12660458

- 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

- Higgins JP, Rouse RV. Testicular Sertoli cell tumors NOS, the final word?. Adv Anat Pathol. 1999;6:103–113.

- Hopkins GB, Parry HD. Metastasizing Sertoli-cell tumor (androblastoma). Cancer. 1969;23:463–467.

- Young RH, Koelliker DD, Scully RE. Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases. Am J Surg Pathol. 1998;22:709–721.

- Zukerberg LR, Young RH, Scully RE. Sclerosing Sertoli cell tumor of the testis. A report of 10 cases. Am J Surg Pathol. 1991;15:829–834.

- Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VLW. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine. 1985;64:270–283.

- Proppe KH, Scully RE. Large-cell calcifying Sertoli cell tumor of the testis. Am J Clin Pathol. 1980;74:607–619.

- Talerman A. Malignant Sertoli cell tumor of the testis. Cancer. 1971;28:446–455.

- Gilcrease MZ, Delgado R, Albores-Saavedra J. Testicular Sertoli cell tumor with a heterologous sarcomatous component: immunohistochemical assessment of Sertoli cell differentiation. Arch Pathol Lab Med. 1998;122:907–911.

- Jacobsen GK. Malignant Sertoli cell tumors of the testis. J Urol Pathol. 1993;1:233–255.

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- Proppe KH, Dickersin GR. Large-cell calcifying Sertoli cell tumor of the testis. Light microscopic and ultrastructural study. Hum Pathol. 1982;13:1109–1114.

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- Rey R, Sabourin J-C, Venara M, Long WQ, Jaubert F, Zeller WP, et al. Anti-Mullerian hormone is a specific marker of Sertoli- and granulosa-cell origin in gonadal tumors. Hum Pathol. 2000;31:1202–1208.

- Tanaka Y, Carney JA, Ijiri R, Kato K, Miyake T, Nakatani Y, et al. Utility of immunostaining for S-100 protein subunits in gonadal sex cord-stromal tumors, with emphasis on the large-cell calcifying Sertoli cell tumor of the testis. Hum Pathol. 2002;33:285–289.

- Teilum G. Classification of testicular and ovarian androblastoma and Sertoli cell tumors. Cancer. 1958;11:769–782.

- Mostofi FK, Theiss EA, Ashley DJB. Tumors of specialized gonadal stroma in human male patients. Androblastoma. Sertoli cell tumor, granulosa-theca cell tumor of the testis, and gonadal stromal tumor. Cancer. 1959;12:944–957.

20 Henley JD, Young RH, Ulbright TM. Malignant Sertoli cell tumors of the testis: A study of 13 examples of a neoplasm frequently misinterpreted as a seminoma. Am J Surg Pathol. 2002;26:541–550.