Contributed by Cecilia Lezcano, M.D. and David J. Dabbs, M.D.
CLINICAL HISTORY 24-year old female patient reports gradual onset of abdominal pain approximately 24 hours before admission. She reports the pain is cramping and sharp, that begins in the right flank and radiates to the front or will occasionally start in her lower abdomen and radiate to her back. She reports that the pain is similar to previous episodes of kidney stones. She reports three episodes of emesis overnight and ongoing nausea. She denies fevers, chills, shortness of breath, headache, diarrhea, irregular vaginal bleeding, vaginal discharge, change in bowel/bladder habits, dysuria, hematuria, hematochezia.
The patient reports past medical history of nephrolithiasis only. The patient denies prior surgical procedures. She denies family history of colon, uterine, breast and ovarian carcinomas. Relevant obstetric-gynecological history consists of G3P2012, two full term spontaneous vaginal deliveries. The patient has no history of sexually transmitted infectious diseases and is currently sexually active, taking oral contraceptive pills. No history of abnormal cervical PAP smears.
CT scan with contrast of the abdomen and pelvis was significant for a heterogeneously enhancing predominantly solid right adnexal mass that measured 7.5 x 7.3 x 7.2 cm. Appearing contiguous with the mass, an oval tubular predominantly cystic structure extending superiorly adjacent to the psoas measuring 3.5 x 7.7 cm was observed. It seemed unclear whether the cystic structure was part of the mass or corresponded to a dilated fallopian tube. No calcifications or adipose tissue was identified within this mass. A clear fat plane was identified between the mass and the uterus. No infiltration of the fat was seen. No ascites was observed. The left ovary was deemed to have a normal radiological appearance. The uterus was unremarkable. The bladder and rectum were radiologically unremarkable.
Multiple specimens were received during intraoperative consultation that consisted of aggregates of irregular tissue fragments that measured 3.5 to 14.0 cm in greatest dimension. The tissue was grossly heterogeneous showing soft, friable, hemorrhagic, and necrotic areas as well as tan-white rubbery fragments of firmer consistency.
INTRAOPERATIVE CONSULTATION DIAGNOSIS
Mixed epithelioid neoplasm.
The relevant microscopic features are illustrated in images 1 & 2 (H&E, 100X), 3 (H&E, 200X), 4 (H&E, 400X), 5 (CD99, 200X), 6 (AE1/AE3, 200X), 7 (CAM 5.2, 200X), 8 (EMA, 200X), and 9 (Ber-EP4, 200X), with the immunohistochemistry results summarized in the following table:
Fluorescence in situ hybridization (FISH) was positive for the SS18 (SYT) gene rearrangement that suggests the t(X;18) (p11.2; q11.2) translocation in 25 of the 32 interphase cells available for analysis (78.1%) in the patient's sample. The result is illustrated in image (1F1R1G= one fusion, one red and one green signal).