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Diagnosis: Types of Tumors

Sex Cord-Stromal Tumors

Introduction | Classification
Cancer is not a single disease, but encompasses well over a hundred distinct diseases of different organs. Normally cells divide only when additional cells are required for normal body function. However, at certain times the controls that regulate when a cell divides are lost. This results in accumulation of more and more cells without order. Eventually these cells grow into a mass and this is termed a 'tumor'. It is important to understand that not all tumors are cancer:

Benign tumors are NOT cancer. Benign tumors are only very rarely life-threatening. They do not spread and invade other tissues. Benign tumors can usually be removed and only infrequently grow back.

Borderline or Low Malignant Potential (LMP) tumors are a borderline form of cancer that may eventually spread and invade other tissues. This is a gray zone. Most of these tumors are benign but a few spread and progress. There are certain features that allow the pathologist to predict with some degree of confidence how one of these tumors will behave.

Malignant tumors are cancer. Malignant cancer will spread beyond the ovary, invading and damaging other organs of the body. The spread of cancer beyond its tissue of origin is called metastasis.

Sex cord-stromal neoplasms account for ~10% of all ovarian neoplasms. They are composed of various cell types derived from gonadal stroma and sex cords. They account for most of the hormonally active ovarian tumors. Granulosa cell tumors and Sertoli-Leydig cell tumors are the most common. Unlike patients with common epithelial tumors, in which 75% are considered to be at stage III or IV at diagnosis, patients with these tumors are at stage I at diagnosis 70% of the time. Also unlike common epithelial tumors, sex cord-stromal tumors often have more specific symptoms. Granulosa cell tumors, which are most common in postmenopausal women, may cause vaginal bleeding and an elevated level of the tumor marker inhibin in the blood. Sertoli-Leydig cell tumors are rare; the average age of patients diagnosed with these tumors is 25, and only 10% of patients are over 50. About 33% of these tumors produce signs of virilism (infrequent menstrual periods, cessation of menstrual periods before menopause, hoarse voice, and appearance of facial hair).

Classification of sex cord-stromal neoplasms

Granulosa cell tumor
  • 1-2% of ovarian tumors
  • adult form typically occurs at any age after puberty but is more common in postmenopausal women
  • most common clinically estrogenic ovarian tumor
  • can present with abnormal vaginal bleeding
  • can be associated with endometrial hyperplasia and carcinoma
  • typically a unilateral solid or solid and cystic tumor, often with hemorrhagic areas
  • microscopically displays a variety of patterns (microfollicular, macrofollicular, trabecular, insular, solid-tubular, diffuse); characteristic features include Call-Exner bodies (small cavities filled with eosinophilic material) and nuclei with longitudinal grooves ("coffee bean" appearance)
  • all types have a malignant potential and recurrences can be late
  • juvenile form occurs in the first three decades, can present with isosexual precocity, contains immature follicle-like structures, and does not display nuclear grooves


Sertoli-Leydig cell tumor
  • accounts for less than 0.5% of ovarian tumors
  • occurs in all age groups but encountered most often in young women
  • present with virilization in ~1/3 of cases (oligomenorrhea, amenorrhea, loss of female secondary sex characteristics with hirsutism, clitoromegaly, deepening of voice)
  • almost always a unilateral tumor that can be solid, solid and cystic, or even papillary
  • microscopically can range from well-differentiated to poorly differentiated; better differentiated forms contain tubular structures resembling seminiferous tubules lined by Sertoli cells and surrounding Leydig cells
  • behavior correlates with degree of differentiation and stage

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