CLINICAL HISTORY
The patient was a 40-year-old female presented with abdominal pain and new-onset hyperglycemia. Past history is significant for infertility and total abdominal hysterectomy/bilateral salpingo-oopherectomy for benign disease in 2000. A CT scan of abdomen with contrast showed a retroperitoneal mass, which is inseparable from several organs including the pancreas, stomach, spleen, left kidney.
Resection of retroperitoneal mass, distal pancreas, left adrenal and left kidney were performed.
LABORATORY DATA
24-hour urine free catecholamines: negative.
Glucose: 175 mg/dl [70-110]
GROSS PATHOLOGY
Retroperitoneal mass measured 27x23x15 cm and weighed 4414 g (Figure 1). Cut surface showed a variegated appearance with areas of necrosis and hemorrhage.
MICROSCOPIC PATHOLOGY
H&E sections show rhabdoid appearing cells with eosinophilic cytoplasm (>75%) (Figure 2) and cells with clear cytoplasm (Figure 3).
Areas of sinusoidal invasion (Figure 4), necrosis (Figure 5), calcifications (Figure 6) and intratumoral and peritumoral vascular invasion are present.
IMMUNOHISTOCHEMISTRY
Melan A (+) (Figure 7A), NSE (+) (Figure 7B), a-Inhibin (+) (Figure 7C), Ki-67 index 14% (Figure 7D).
CD 117 (C-kit) (-)
Vimentin (-)
S100 (-)
Chromogranin (-)
SMA (-)
Desmin (-)
Synaptophysin (-)
EMA (-)
Pankeratin (-)
PAS-D (-)
P53 (-)
ELECTRON MICROSCOPY
Abundant smooth endoplasmic reticulum consistent with steroid cell tumors is seen. |
