Contributed by Marian Rollins-Raval, MD MPH and Rohit Bhargava, MBBS
The patient is a 61-year-old, gravida 5, para 5, who presented with a history of hypertension, hypercholesterolemia, coronary artery disease, arthritis and gingivitis. She had a surgical history significant for right coronary artery stent that was placed approximately 10 years ago in 1999. She underwent an exploratory laparotomy, total abdominal hysterectomy and unilateral salpingo-oophorectomy (TAH/USO) at the age of 35 for menorrhagia, and also underwent a removal of a breast cyst. The patient presented with increased hair growth on her neck, face and abdomen for the past 2 years and initially presented to a dermatologist for hair removal. In addition, she has had some loss of hair on her head with a receding hairline. She noted that her voice has been deeper over the past year. She has had increased irritability and decreased libido. She had clitoromegaly. As part of her workup for this hirsutism, she had several hormone levels sent, which were concerning for an elevated dehydroepiandrosterone (DHEA) of 2974 ng/dL with a normal being less than 370 ng/dL in her age group, an elevated androstenedione at 2672 ng/dL (upper level of normal 268 ng/dL) and an elevated free and total serum testosterone with a total testosterone of 1054 ng/dL and an elevated free testosterone of 38.0 pg/mL. Given these findings, the patient was referred to an endocrinologist. The endocrinologist repeated this laboratory work, which confirmed the above findings. She also had an elevated CA-125 noted to be 50.8 units/mL. Subsequently, the patient underwent a transvaginal ultrasound, which showed a heterogenous mass with cystic foci with the epicenter at the right adnexa. The mass measured 7.7 x 5.8 x 5.6 cm and was concerning for a malignancy (Figure 1). She then underwent a CT scan, which showed a 10 x 6 cm lobulated heterogenous solid mass in the pelvis, slightly to the right of midline (Figure 2). There were no abnormalities noted of the liver, spleen, pancreas or adrenal glands, and she had no abdominal or retroperitoneal lymphadenopathy.
The surgeon and patient decided to proceed with removal of the adnexal mass. Based on intra-operative consulatation with the pathologist, the surgeon performed resection of the right pelvic mass, removal of the left fallopian tube, bilateral pelvic and periaortic lymph node sampling, omentectomy, peritoneal biopsies for staging and an appendectomy. There was no gross residual disease at the completion of surgery. There was retroperitoneal fibrosis and pelvic adhesive disease.
Gross Pathologic Findings
The pelvic mass measured 9.0x 7.0 x 5.0 cm and consisted of a tan-pink lobulated the mass. The external surface of the mass displayed a prominent 3.5 x 3.0 x 2.5 cm yellow orange surface nodule (Figure 3). On cut section, the tumor demonstrated yellow-orange, mottled lobulated soft tissue. Several of the lobules are separated by dense, white, fibrous septae. No necrosis was identified. No grossly normal ovarian tissue was identified (Figure 4).
Microscopic Pathologic Findings
Sections of the tumor demonstrate sheets of neoplastic cells. There are two distinct populations, one containing eosinophilic cytoplasm and the other showing clear cytoplasm. In some areas these populations are continuous (Figures 5 and 6) while others are separated by broad fibrous septae (Figure 7). The nuclei demonstrate a moderate degree of pleomorphism, particularly in the eosinophilic areas, with varying shapes and sizes (nuclear grade 2) (Figure 8). The tumor mitotic activity is 3-4 mitotic figures per 10 high power fields (Figure 9).
Immunohistochemical studies differed somewhat between the two populations of cells (Figure 10) with the clear cell population staining much more weakly than the eosinophilic population, but with both populations still showing positivity for inhibin (Figure 11) and calretinin (Figure 12). Immunohistochemical studies for epithelial membrane antigen, S100, HMB-45 and CD99 were all negative in the tumor cells. Melan-A showed patchy positivity in the cells with eosinophilic cytoplasm.
In addition, several areas were suspicious for vascular space invasion (Figure 13), which was confirmed with CD31 (Figure 14). D2-40 was also performed which highlighted the lymphatic endothelium and confirmed the absence of lymphatic space involvement.