Ovarian Sex Cord Stromal Tumor

Sex cord stromal tumors of the ovary arise from the sex cords or the stromal cells and account for 5 to 12% of ovarian tumors. Stromal cells are present around the eggs (ova) in the ovaries (Stroma– surrounding supportive tissue). These mimic a variety of ovarian tumors (both cancerous and noncancerous). Unlike the ovarian tumors whose incidence is more common is older age group; these tumors are seen in broad age range.

Classification and Symptoms

There are several different types of ovarian sex cord stromal tumors; important ones are listed below:

• Granulosa cell tumor ovary has two variants:

  • Adult type granulosa cell tumor :
    • These are seen in adults
    • Symptoms are distended abdomen
    • Irregularities in menstrual cycles
    • In menopausal women vaginal bleeding or spotting is seen
  • Juvenile type granulosa cell tumor:
    • Occur at early age : during puberty.
    • They present with symptoms of precocious puberty (early onset of puberty with odevelopment of breasts, vaginal bleeding etc).
    • Other symptoms are distension of abdomen and ascites (fluid collection in abdomen), and pain in pelvic region.

• SertoliLeydig cell tumors/Sertoli cell tumor:

  • Very rare type of stromal sex cord tumor of ovary
  • Occur in children, adolescents, and young women
  • On microscopy, presence of Sertoli cell tubules and Leydig cells, hence the name.
  • Signs of male hormone production called virilisation (baldness, thick body hair, hoarse voice).
  • In menstruating women/girls heavy bleeding and shorter cycles.
  • Shrinking in the size of breasts.
  • Increase in the size of clitoris.

• Gynandroblastomas:

  • Have characteristics of both Sertoli and Juvenile type tumors.

• Sex cord tumor with annular tubules:

  • Sertoli cells arranged in tubules (SCTAT).
  • Majority of these tumors are noncancerous.
  • Symptoms of precocious puberty.
  • Menstrual irregularities, like bleeding in between cycles.

Juvenile type granulosa cell tumor and Sertoli cell tumor constitute 85% of the sex cord stromal tumor of the ovaries. Abdominal or pelvic pain might be present in all types.

Diagnosis

The majority of them (90%) are diagnosed at early stages because unlike ovarian tumors these stromal cell tumors show good amount of signs and symptoms. The following investigations help:

• Physical examination by the doctor
• Ultrasonography of the pelvis
• CT and/or MRI
• Tumor markers (substances found in the body whose presence or elevated levels confirm certain type of cancers). Here CD56 is a sensitive marker, although many others are present.

Treatment of Sex Cord Stromal Cell Tumors of the Ovary

Surgery is the primary treatment of Sex Cord Stromal Cell Tumors. Although the prognosis is good with long-term survival rate of 75-80%, with rare and distant recurrences, but the chances of these tumors turning cancerous is high. Fortunately, they are detected early in majority of the cases. The treatment plans vary depending on the stage of disease and age of the patient:

1. Surgery :

   • Laparotomy – Opening the abdomen to diagnose as well treat the condition. Done in young patients for fertility sparing purpose if in the first stage of cancer.
   • Removal of the tube and ovary (affected side), in low grade stage 1 tumors; saphingo-oophorectomy.
   • Patients are watched carefully after the above mentioned surgeries.
   • In stages 2, 3 and 4, removal of the ovary along with the structures to which the cancer has spread (debulking) is done and also may require chemotherapy or rarely radiation therapy.

2. Adjuvant chemotherapy :

   A combination of drugs is used to kill any cancer cells present in the body after surgery.

3. Radiation therapy :

   Use of X rays to kill cancer cells (radiation therapy) is rarely required but can help advanced stage and also in combination with other treatment procedures

4. Hormone therapy :

   Is useful in treating advanced stages when chemotherapy is not tolerable to the patient (Tamoxifen).

Prognosis

If detected early, which generally is the case, they have good prognosis. Compared to the ovarian germ cell tumors these are slow growing tumors; hence the relapse is slower. Surgery and chemotherapy to treat relapses are helpful. There is 90% chance of complete cure after treatment.

Hospital Stay and Cost

Hospital stay depends on the type of surgery performed:

• Laparoscopic procedures : 2-3 days
• More invasive surgeries : 5-7 days