These pathlets are edutainment. This site collects no information about visitors, and cannot substitute for your own doctor's care. There are many questions without clear right-or-wrong answers.
Upon physical exam, she is noted to be within normal height and weight for her age, strong but petite, has not yet had onset of menses, and otherwise appears healthy. She has a moderate amount of acne on her forehead and face, which started appearing about six months ago. Lung and bowel sounds are normal, and the heart has a regular rate and rhythm with no murmurs. Abdominal exam reveals right lower quadrant, rebound tenderness to palpation (a slight but definite wince by the patient), and fluid wave. Percussion demonstrated shifting dullness.
If you hadn't palpated the mass or found the fluid wave, which would you consider as the cause of her acne? Choose all that apply.
Normal teenage development Polycystic ovary syndrome Adrenal hyperplasia Ovarian tumor Cushing’s syndrome
For this patient, which diagnostic studies would you perform? Choose all that apply.
Ovarian tumors that produce male hormones usually produce testosterone, and there would be little reason at this time to test for other steroids.
We're not big on spot-ACTH or spot-cortisol levels for Cushingism, but prefer the dexamethasone suppression test and the even-more-inconvenient 24 hour urine cortisol if this is a serious consideration. In a young woman who almost certainly has an ovarian tumor, this would seem to be overtesting.
Ultrasound confirmed the presence of a 13 cm solid, right ovarian mass and fluid in the peritoneal cavity. Serum testosterone was moderately elevated.
Which is your best action right now?
Upon opening the abdomen, hemorrhagic asitic fluid was present. The fluid, collected from the sterile field, was sent for cytology and culture.
The right ovary had a firm-to-rubbery mass attached to it that was hemorrhagic in some areas. The ovary was then excised and sent to pathology for further evaluation. Multiple tumor nodules were noted in the omentum, peritoneum, appendix and were simultaneously biopsied then sent to pathology.
The pathologist received a specimen labeled as right ovary. Examination revealed a 14 X 10 X 5 cm tan-white, firm to rubbery white oval mass with a ruptured area that is hemorrhagic. The cut surface was also tan white with areas of punctuated hemorrhage. The biopsied omental, peritoneal and appendiceal nodules had a similar gross appearance.
Histologic sections reveal a poorly differentiated tumor, with a diffuse growth pattern composed of spindle-shaped cells and a relatively high mitotic rate. Heterologous elements, were not seen.
Once you are done, what is your diagnosis?
Brenner tumor Dysgerminoma Fibroma Mucinous cystadenoma Serous carcinoma Sertoli cell tumor, anaplastic Sertoli-Leydig tumor, well-differentiated Thecoma
The triangular cells arranged in ribbons is typical of Sertoli-cell tumors. The staining with vimentin, keratin, and inhibin also fits. The cells are poorly-differentiated and there are mitotic figures so it's no surprise that the tumor had already metastasized when it was discovered.
The tumor was probably the source of the extra androgens that caused the acne before menarche. These malignant tumors are not especially responsible to therapy.
Sertoli-Leydig tumors make up only around 0.5% of ovarian tumors, and these anaplastic tumors are even less common.
Peter Marogil KCUMB '15
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