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Pathologic Classification

Updated February 8, 2018

3. Pathologic classifications

Sex Cord Stromal Tumours (SCST)

Granulosa-Stromal Cell Tumours

  1. Granulosa cell tumour
    1. juvenile
    2. adult
  2. Tumours in the thecoma-fibroma group
    1. thecoma
      1. typical
      2. luteinized (a rare tumour has the features of luteinized thecoma and additionally, crystals of Reinke in the steroid cell component. This tumour has been called "stromal Leydig cell tumour".)
    2. fibroma
    3. cellular fibroma
    4. fibrosarcoma
    5. stromal tumour with minor sex cord elements
    6. sclerosing stromal tumour
    7. (stromal luteoma) see Steroid (Lipid) Cell Tumours, stromal luteoma
    8. unclassified (fibrothecoma)
    9. others (This category includes the rare signet ring cell stromal tumour and the myxoma).

Sertoli-Leydig Cell Tumours; Androblastomas

  1. Well differentiated
    1. Sertoli cell tumour (tubular androblastoma)
    2. Sertoli-Leydig cell tumour
    3. (Leydig cell tumour) - see Steroid (Lipid) Cell Tumours, Leydig cell tumour
  2. Of intermediate differentiation
    1. variant - with heterologous elements (specify type)
  3. Poorly differentiated (sarcomatoid)
    1. variant - with heterologous elements (specify type)
  4. Retiform
    1. variant - with heterologous elements (specify type)

Sex Cord Tumour with Annular Tubules



Steroid (Lipid Cell Tumours)

  1. Stromal luteoma
    1. Leydig cell tumour (hilus cell tumour). Some Leydig cell tumours do not arise from hilus cells but within the ovarian stroma. These tumours have been called "Leydig cell tumours, non-hilar type".

Germ Cell Tumours (GCT)

  1. Dysgerminoma
    1. Variant - with syncytiotrophoblast cells
  2. Yolk Sac Tumour (Endodermal Sinus Tumour)
    1. Variants
      1. Polyvesicular vitelline tumour
      2. Hepatoid
      3. Glandular (some glandular yolk sac tumours resemble endometrioid adenocarcinoma and have been called "endometrioid-like".
  3. Embryonal Carcinoma
  4. Polyembryoma
  5. Choriocarcinoma
  6. Teratomas
    1. Immature
    2. Mature
      1. solid
      2. cystic (dermoid cyst)
      3. with secondary tumour (e.g. to squamous cell carcinoma)
      4. retiform (homunculus)
    3. Monodermal and highly specialized
      1. struma ovarii – need to check thyroid function
        • variant - with secondary tumour (specify type)
      2. carcinoid
        • insular
        • trabecular
      3. struma carcinoid
      4. mucinous carcinoid
      5. neuroectodermal tumours (specify type)
      6. sebaceous tumours
      7. others
  7. Mixed (specify types)


  1. Variant - with dysgerminoma or other term cell tumour

Germ Cell - Sex Cord-Stromal Tumour

  1. Variant - with dysgerminoma or other term cell tumour

Tumours of Rete Ovarii

  1. Adenomatoid tumour
  2. Mesothelioma

Mesothelial Tumours

  1. Adenomatoid tumour
  2. Mesothelioma

Tumours of Uncertain Origin

  1. Small cell carcinoma (This tumour is of two types - one that is usually associated with paraendocrine hypercalcemia and has been called "small cell carcinoma, hypercalcemic type", and the other that has neuroendocrine features and has been designated "small cell carcinoma, pulmonary type". Probably most of the latter type belong in the surface epithelial-stromal category.)
  2. Tumour of probable Wolffian origin
  3. Hepatoid carcinoma
  4. Oncocytoma

Soft Tissue Tumours not Specific to Ovary

Malignant Lymphomas and Leukemias

Unclassified Tumours

Secondary (Metastatic) Tumours


  1. Colombo N, Peiretti M, Castiglione M, Group EGW. Non-epithelial ovarian cancer: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 2009; 20 Suppl 4: 24-26.
  2. Shah SP, Kobel M, Senz J et al. Mutation of FOXL2 in granulosa-cell tumors of the ovary. N Engl J Med 2009; 360: 2719-2729.
  3. Heravi-Moussavi A, Anglesio MS, Cheng SW et al. Recurrent somatic DICER1 mutations in nonepithelial ovarian cancers. N Engl J Med 2012; 366: 234-242.
  4. Korach J, Perri T, Beiner M et al. Promising effect of aromatase inhibitors on recurrent granulosa cell tumors. Int J Gynecol Cancer 2009; 19: 830-833.
  5. Schwartz M, Huang GS. Retreatment with aromatase inhibitor therapy in the management of granulosa cell tumor. Gynecol Oncol Rep 2016; 15: 20-21.
  6. Tao X, Sood AK, Deavers MT et al. Anti-angiogenesis therapy with bevacizumab for patients with ovarian granulosa cell tumors. Gynecol Oncol 2009; 114: 431-436.
  7. Meisel JL, Woo KM, Sudarsan N et al. Development of a risk stratification system to guide treatment for female germ cell tumors. Gynecol Oncol 2015; 138: 566-572.

SOURCE: Pathologic Classification ( )
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