Testis & epididymis
Sex cord stromal tumors
Intratubular large cell hyalinizing Sertoli cell neoplasia


Topic Completed: 18 November 2019

Revised: 19 November 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Intratubular large cell Sertoli

Erica Vormittag-Nocito, M.D.
Andre Kajdacsy-Balla, M.D., Ph.D.
Page views in 2019 to date: 131
Cite this page: Vormittag-Nocito E, Kajdacsy-Balla A. Intratubular large cell hyalinizing Sertoli cell neoplasia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/testisitlchscn.html. Accessed December 10th, 2019.
Definition / general
Essential features
  • Benign, multifocal and often bilateral testicular lesion
  • Found in prepubertal males, usually associated with gynecomastia
  • Associated with Peutz-Jeghers syndrome
Terminology
  • Historically: sex cord tumor with annular tubules (not recommended)
ICD coding
  • ICD-10: D29.20 - benign Sertoli cell tumor of testis
  • ICD-11: unknown
  • ICD-O-3: 8640 / 1 - Sertoli cell tumor, NOS
Sites
  • Seminiferous tubules
Pathophysiology
  • Unknown
Etiology
Clinical features
Diagnosis
Laboratory
  • Increased estradiol level
Radiology description
Prognostic factors
  • Considered a benign condition, unknown what makes rare cases invasive
Case reports
Treatment
  • Aromatase inhibitor therapy initial treatment
  • Orchiectomy for nonresponders with continued / progressive clinical estrogen symptoms (Am J Surg Pathol 2007;31:827)
Gross description
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Vikas Mehta, M.D.

Seminiferous tubules

Basement membrane deposition

Calretinin

Inhibin A

Collagen IV

PAS

Negative stains
  • Not well studied in literature
Molecular / cytogenetics description
Sample pathology report
  • Testis, right, core needle biopsy:
    • Intratubular large cell hyalinizing Sertoli cell neoplasia
Differential diagnosis
Board review question #1
What diagnosis is associated with a 4 year old patient who presents with pigmented macules around the lips and oral mucosa, gynecomastia and the following findings on testis biopsy?



  1. Gonadoblastoma
  2. Intratubular large cell hyalinizing Sertoli cell neoplasia
  3. Large cell calcifying Sertoli cell neoplasm
  4. Sertoli cell nodule
Board review answer #1
B. Intratubular large cell hyalinizing Sertoli cell neoplasia. The patient has Peutz-Jeghers syndrome and the testicle shows intratubular large cell hyalinizing Sertoli cell neoplasia. The image shows a tubule composed of large Sertoli cells without normal spermatogonia and spherules of basement membrane-like material characteristic of this entity.

Reference: Intratubular large cell hyalinizing Sertoli cell neoplasia

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Board review question #2
Which of the following symptoms is most associated with intratubular large cell hyalinizing Sertoli cell neoplasia?

  1. Gynecomastia
  2. Mucosal ulcers
  3. Normal age of puberty
  4. Short stature
Board review answer #2
A. Gynecomastia. This is a symptom seen due to the elevated serum estradiol level in these patients.

Reference: Intratubular large cell hyalinizing Sertoli cell neoplasia

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