Testis & epididymis
Sex cord-stromal tumors
Sertoli cell NOS
Author: Swapnil U. Rane, M.D.
Topic Completed: 1 December 2013
Minor changes: 5 November 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Testis Sertoli cell tumor [title]
Table of Contents
Definition / general | Terminology | Epidemiology | Clinical features | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Differential diagnosisCite this page: Rane S. Sertoli cell NOS. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testisSertoliNOS.html. Accessed January 16th, 2021.
Definition / general
- Sex cord stromal tumor of testis composed of cells expressing, to a varying degree, features of fetal, prepubertal or adult Sertoli cells
Terminology
- Also called androblastoma
Epidemiology
- < 1% of testicular tumors
- Occurs in normally descended testis
- Mean age 45 years; exceptionally rare in men < age 20 years
- Most cases are sporadic but cases in infants / children are usually part of Peutz-Jeghers, androgen insensitivity or Carney syndromes
Clinical features
- Typically present with slowly enlarging testicular mass (J Urol 1980;124:757)
- Usually lack hormone related symptoms (Am J Surg Pathol 1998;22:709) but may have hyperestinism symptoms if part of Peutz-Jeghers syndrome
- Presence of bilateral testicular Leydig cell tumors requires exclusion of Peutz-Jeghers syndrome
Laboratory
- No specific laboratory finding
- Hyperestrinism is noted in syndromic cases
Radiology description
- Ultrasound is usually hypoechoic but not specific and not helpful in distinguishing from germ cell tumors
- However, large cell calcifying variant can often be distinguished on ultrasound
Prognostic factors
- 12% of cases are malignant
- Features associated with malignancy are large size ( > 5 cm) (range 2 - 18 cm), vascular invasion, marked nuclear pleomorphism, tumor necrosis, mitotic index > 5/10HPF (Current Diagnostic Pathology 2002;8:83)
Case reports
- 34 year old man with bilateral Sertoli cell tumors (Virchows Arch 2012;461:713)
- With heterologous sarcomatous component (Arch Pathol Lab Med 1998;122:907)
- Malignant Sertoli cell tumors (Am J Surg Pathol 2002;26:541)
Treatment
- Orchidectomy is treatment of choice; usually sufficient because most tumors are benign
- Benefit of chemotherapy or radiotherapy is not well documented
Gross description
- Spherical, lobulated, well circumscribed tumors
- Mean 3.5 cm (Am J Surg Pathol 1998;22:709), range 1 - 20 cm
- Cut surface usually homogenous tan yellow or greyish white
- Areas of necrosis usually not evident
- Hemorrhage may be seen
Gross images
Images hosted on other servers:
Tumor in lower pole
Microscopic (histologic) description
- Tumor cells are typically arranged in solid or hollow tubules separated by basement membrane; also retiform, tubule - glandular patterns, solid sheets or nodules
- Well developed or abortive tubules present in at least one focus
- Cytoplasm is pale eosinophillic to vacuolated due to extensive lipid
- Tumor cells are bland and uniformly round with oval, elongated nuclei; no prominent nucleoli, no nuclear grooves, no inclusions
- Rarely mild nuclear atypia and pleomorphism
- Intervening stroma is fibrous; variably cellular, hyalinized, edematous, containing dilated blood vessels
- Minor dystrophic calcifications may be seen within the stroma which may occasionally be extensive
- Typically no stromal inflammatory cells
- Mitoses are uncommon and is usually < 5 mitoses per 10 high power fields
- Increased mitoses ( > 5 per high power field) in ~15% of cases but by itself does not constitute malignancy
Microscopic (histologic) images
Images hosted on other servers:
Cystic area with tubules
Pseudoglandular pattern
Solid - cystic areas
Retiform pattern
Hyalinized blood vessels
Positive stains
- Vimentin (90%), cytokeratin (80%)
- Inhibin (40%), S100 (30%)
- Chromogranin (Mod Pathol 1998;11:774), synaptophysin (5/11) (Hum Pathol 2000;31:1055)
- CD99 (14%) (Hum Pathol 2000;31:1055)
Negative stains
Electron microscopy description
- Cells interconnected by desmosomes; abundant smooth ER, lipid droplets
- Charcot-Böttcher crystals, composed of filaments, are typical of Sertoli cells but are rarely seen
Differential diagnosis
- Adenomatoid tumor:
- Paratesticular in location
- Carcinoid:
- Strong chromogranin and synaptophysin positivity
- May be associated with teratomatous element
- Cystadenoma of rete testis:
- Confined to rete testis
- Juvenile granulosa cell tumor:
- Form follicules rather than tubules
- Large cell calcifying Sertoli cell tumor:
- Associated with Carney syndrome
- Metastatic adenocarcinoma: EMA positive
- Metastatic adenocarcinoma of prostate: PSA positive
- Metastatic melanoma:
- Positive for more specific markers such as HMB45
- Seminoma
- Sertoli - Leydig cell tumour:
- Very rare, have a more cellular stroma
- Sertoli cell nodule (Pick adenoma):
- Associated with cryptorchidism and testicular feminisation
