Granulosa-Theca Cell Tumors Clinical Presentation

  • Author: Chad M Michener, MD; Chief Editor: Warner K Huh, MD   more...
 
Updated: Jan 4, 2010
 

History

Many patients with GCTs present with manifestations of hyperestrogenism. Approximately 70% of these tumors are hormonally active. Hormonal influences can cause different presenting symptoms depending on patient age and menstrual status. Although these symptoms can be quite profound, often they may be secondary findings in patients with complaints relating to the abdomen and pelvis.

Reports of increasing abdominal girth and abdominal discomfort are quite common. Most patients have a palpable mass found during examination. Abdominal symptoms may be due to enlargement of the mass but also can be due to the production of ascites, which occurs in approximately 10% of patients. Increasing size of the mass also can lead to symptoms associated with compression of adjacent structures, such as abdominal pain, dysuria, urinary frequency, and constipation.

Acute onset of abdominal pain also can occur, although rarely. Acute abdominal or pelvic pain may be observed in combination with nausea, vomiting, dizziness, and shoulder pain. These symptoms may be due to adnexal torsion, rupture of a partially cystic GCT, or hemorrhage either within the tumor or into the peritoneum.

  • Prepubertal girls
    • Patients usually present with precocious pseudopuberty (70-80%) and have secondary sex characteristics at a very early age. These may include increased linear growth, breast enlargement, clitoral enlargement, pubic hair development, increased vaginal secretions, and vaginal bleeding.
    • In a few instances, patients present with virilizing symptoms as a result of testosterone production by the tumor cells. Many of these hormone-induced symptoms abate following resection of the tumor.
  • Premenopausal women
    • Increasing abdominal girth and other symptoms related to an enlarging adnexal mass may be seen in this group of patients.
    • Menstrual irregularities such as oligomenorrhea, menorrhagia, and secondary amenorrhea tend to be the hallmark of these tumors in reproductive-aged women.
  • Postmenopausal women
    • The most common endocrine manifestation of GCTs in postmenopausal women is abnormal uterine bleeding. This is caused by resumption of endometrial proliferation due to estrogen production by the tumor. For this reason, endometrial hyperplasia and/or endometrial adenocarcinoma may be a concomitant finding in women with GCT.
    • Patients also can have breast tenderness and increased vaginal secretions from estrogenic stimulation of the breast and vaginal tissues, respectively.
    • Rarely, a patient may present with virilizing symptoms such as acne, hirsutism, deepening of the voice, and clitoral enlargement. This is due to testosterone and/or androstenedione production in a minority of these tumors.
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Physical

  • Pelvic mass is the most consistent finding on pelvic and rectal examination in patients of all ages with GCT. A palpable mass can be found in 85-97% of patients. A bimanual examination and a rectovaginal examination should be performed to evaluate the pelvis and lower abdomen for masses, the posterior cul-de-sac for nodularity, and any other areas associated with tenderness. During the rectal examination, a stool sample should be obtained for guaiac testing, which can be helpful in narrowing the differential of GI disorders.
  • For patients presenting with acute abdominal pain, a careful speculum examination should be performed to help rule out infectious etiologies. Wet preparation and cultures for Neisseria gonorrhoeae and Chlamydia trachomatis should be considered. Gram stain for gram-negative diplococci can be helpful if other findings are consistent with a diagnosis of pelvic inflammatory disease and/or cervicitis.
  • Other findings generally relate to endocrine manifestations of hyperestrogenic and/or hyperandrogenic states.
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Causes

No definite etiologies for GCTs have been found. Proposed etiologies include chromosomal anomalies and/or autocrine and endocrine signaling abnormalities. A multifactorial etiology has been postulated.

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Contributor Information and Disclosures
Author

Chad M Michener, MD  Assistant Professor, Obstetrics/ Gynecology and Women's Health Institute, Section of Gynecologic Oncology, The Cleveland Clinic

Chad M Michener, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists and Society of Gynecologist Oncologists

Disclosure: Nothing to disclose.

Coauthor(s)

David C Starks, MD  Fellow, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Cleveland Clinic Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Bruce A Meyer, MD, MBA  Executive Vice President for Health System Affairs, Chief Clinical Officer, Interim CEO, University Hospitals; Professor, Department of Obstetrics and Gynecology, University of Texas Southwestern Medical School

Bruce A Meyer, MD, MBA is a member of the following medical societies: American College of Obstetricians and Gynecologists, American College of Physician Executives, American Institute of Ultrasound in Medicine, Association of Professors of Gynecology and Obstetrics, Massachusetts Medical Society, Medical Group Management Association, and Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Frederick B Gaupp, MD  Consulting Staff, Department of Family Practice, Hancock Medical Center

Frederick B Gaupp, MD is a member of the following medical societies: American Academy of Family Physicians

Disclosure: Nothing to disclose.

Chief Editor

Warner K Huh, MD  Professor, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Senior Scientist, Comprehensive Cancer Center, University of Alabama School of Medicine

Warner K Huh, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American College of Surgeons, American Society of Clinical Oncology, Massachusetts Medical Society, and Society of Gynecologist Oncologists

Disclosure: MERCK Consulting fee Consulting; GSK Consulting fee Consulting; ROCHE PHARMA/DIAGNOSTICS Consulting fee Consulting; HOLOGICS Consulting fee Consulting; HELIX BIOPHARMA Consulting fee Consulting; COVIDIEN Consulting fee Consulting; INTUITIVE SURGICAL Surgical Proctor

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Microfollicular pattern of an adult granulosa cell tumor at 100X magnification. Inset is characteristic Call-Exner bodies and nuclear grooves (400X). Image courtesy of James B. Farnum, MD, TriHealth Department of Pathology.
Less well-differentiated diffuse pattern of adult granulosa cell tumor. Monotonous pattern can be confused with low-grade stromal sarcoma (200X). Inset is high-power magnification demonstrating nuclear grooves and nuclear atypia. Image courtesy of James B. Farnum, MD, TriHealth Department of Pathology.
Juvenile granulosa cell tumor. Multiple follicles in various shapes and sizes (200X). Inset shows nuclei that are rounded, hyperchromatic, lacking grooves and showing atypia, and are abnormal mitotic figures (400X). Image courtesy of James B. Farnum, MD, TriHealth Department of Pathology.
Gyriform pattern of adult granulosa cell tumor. Undulating single-file rows of granulosa cells (200X). Image courtesy of James B. Farnum, MD, TriHealth Department of Pathology.
Theca cell tumor. Typical thecoma with lipid-rich cytoplasm, pale nuclei, and intervening hyaline bands (200X). Image courtesy of James B. Farnum, MD, TriHealth Department of Pathology.
Luteinized thecoma. Vacuolated theca cells with an abundant fibromatous stroma (200X). Image courtesy of James B. Farnum, MD, TriHealth Department of Pathology.
 
 
 
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