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.
Physical Examination
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.
For patients presenting with acute abdominal pain, a careful speculum examination should be performed to help rule out infectious etiologies. Wet preparation and testing 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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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.
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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.
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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.
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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.
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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.
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Luteinized thecoma. Vacuolated theca cells with an abundant fibromatous stroma (200X). Image courtesy of James B. Farnum, MD, TriHealth Department of Pathology.
