A Subtle Presentation
Pathlet 005
Peter Marogil '15
with the pathology team

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.


Elizabeth Gardner
Young Girl with Grapes
A 13-year-old female presents to her primary care physician because of abdominal pain and an enlarging mass in the right lower quadrant. With a slightly deep but, somber voice, the patient describes herself as being in “good health until the recent onset of abdominal pain several months ago”. She has noticed some hair growth on her face, which she is embarrassed about but does not understand. Says that her mom told her it was part of growing up and it would go away.

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

"All are correct." If acne in a young girl is the sign of serious disease, there will be, or soon will be, other signs and symptoms. In this case, there's a mass.

For this patient, which diagnostic studies would you perform? Choose all that apply.

Abdominal ultrasound
MRI of the abdomen
CT of the abdomen
Serum ACTH
Metyrapone test for Cushing's
Dexamethasone suppression test for Cushing's
Spot serum Cortisol
24 hour urine for cortisol
Serum testosterone
Serum dehydroepiandrosterone (DHEA)
Serum 17-hydroxyprogesterone

We think the ABDOMINAL ULTRASOUND and the SERUM TESTOSTERONE are the best for starters.

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?

Order antibiotics for 12 days
Recommend bed rest
Get a pediatric surgical consult
Perform muscle energy until the patient gets pain relief

Surgery was performed by a renowned pediatric surgeon (a KCUMB alumnus of course).

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.

 

You may order your immunohistochemical tests here.

A103
   (stains melanoma)
     
Alpha-fetoprotein
   (stains liver, some germ cells)
     
Calretinin
   (stains mesothelioma, others)
     
CK7
   (stains urothelium)
     
CK20
   (stains glands, signet-ring CA)
     
CD56
   (stains neural, others)
     
CD99
   (stains Ewing, granulosa)
     
Cytokeratin
   (stains epithelium)
     
Epithelial membrane antigen
   (stains ducts & acini)
     
Estrogen Receptor
   (stains many breast, some other)
     
Inhibin
   (sex-cord stromal)
     
Leukocyte common antigen
   (stains white cells)
     
Neuron-specific enolase
   (stains neural, some other)
     
Progesterone Receptor
   (stains many breast, some other)
     
Synaptophysin
   (stains some neural)
     
Vimentin
   (stains most mesenchymal)
     

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

FINAL DIAGNOSIS: MALIGNANT SERTOLI CELL TUMOR OF RIGHT OVARY

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.


"Sunset Girl"
Painter Unknown


Peter Marogil KCUMB '15

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