Sex cord stromal tumors
Steroid cell tumor, NOS

Topic Completed: 1 December 2012

Minor changes: 10 March 2020

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PubMed search: Ovarian steroid cell tumor

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Cite this page: Ehdaivand S. Steroid cell tumor, NOS. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorsteroidcelltumorNOS.html. Accessed September 21st, 2020.
Definition / general
  • < 0.1% of all ovarian tumors
  • Mean age 42 years, range 2 to 80 years
  • Usually associated with androgenic changes (56-77%); estrogen secretion in 6-23%, Cushing syndrome in 6-10%
  • Usually benign in prepubertal children
  • Clinically malignant in 25-43%, associated with 2+ mitosis per high-power field (92% malignant); necrosis (86% malignant); 7 cm or larger (78% malignant); hemorrhage (77% malignant); and grade 2 or 3 nuclear atypia (64% malignant); some clinically malignant cases are histologically benign (Am J Surg Pathol 1987;11:835)
Case reports
Gross description
  • Up to 45 cm, solid, well-circumscribed, yellow-orange to red-brown to brown-black cut surface (depending on amount of lipid and lipochrome pigment)
  • 6% bilateral; occasional hemorrhage and cystic degeneration
Gross images

AFIP images

Lobulated and yellow-orange

Dark brown with large amount of intracytoplasmic lipochrome pigment

Necrosis, hemorrhage and cystic degeneration

Images hosted on other servers:

Tan brown, well circumscribed tumor with hemorrhage

Bright yellow lobulated mass (fig 1)

Well circumscribed, golden yellow, slightly lobulated, soft tumor

Microscopic (histologic) description
  • Usually diffusely arranged cells, also nests, clusters, cords and columns
  • Scant stroma may be fibromatous, edematous or myxoid
  • Tumor cells are round / polygonal with spongy to granular, eosinophilic cytoplasm with clear intracytoplasmic vacuoles
  • Distinct cell borders; central nuclei, prominent nucleoli
  • No Reinke crystals, no significant nuclear atypia or mitotic activity
Microscopic (histologic) images

AFIP images

Diffuse pattern

Growing in large aggregates

Round tumor cells, uniform nuclei, prominent nucleoli

Abundant pale (lipid-rich) cytoplasm

Abundant lipid (oil red-O stain)

Two atypical mitotic figures

Images hosted on other servers:

H&E, Oil Red O (fig 4)

Diffuse sheets of large polygonal cells with vacuolated
cytoplasm, vesicular nuclei and nuclear pleomorphism

Fig 2: medium-large polygonal cells with eosinophilic granular
cytoplasm in delicate vascularized connective tissue network
Fig 3: vimentin+
Fig 4: inhibin+

Positive stains
  • Inhibin, fat stains (75%), vimentin (75%), CAM 5.2 (46%), AE1/AE3 (37%), EMA (8%) and S100 (7%)
Negative stains
  • Chromogranin A, CD15 (Leu-M1), myoglobin, neurofilament, AFP, CEA and HMB-45
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