A 53-year-old woman presented to our institution with progressive abdominal distention and irregular vaginal bleeding of several weeks’ duration.
A contrast CT abdomen and pelvis revealed an ill-defined, irregular mass in the lower uterine/cervical region (Figure 1).
There were multiple peritoneal and omental masses, enlarged pelvic and paraaortic lymph nodes, and large-volume ascites.
The initial CBC was significant for hemoglobin of 9.2 g/dL, leukocytosis of 36 790 per microliter, and thrombocytosis of 625 000 per microliter.
Serum calcium was elevated at 13.1 mg/dL.
BUN and creatinine were within normal limits.
Hepatic function was within normal limits.
A pelvic exam revealed a fungating vaginal mass.
The patient underwent biopsies of the endometrium and the vaginal mass, which revealed a high-grade tumor with predominantly oval-shaped cells.
Immunohistochemical staining was positive for vimentin, CD10, and cyclin D1, consistent with a diagnosis of high-grade endometrial stromal sarcoma (Figure 2).
The tumor was negative for ER, with rare cells positive for PR.
Although immunohistochemical staining for cytokeratins was negative, a biphasic component could not be entirely ruled out given the small size of the biopsy specimen relative to the tumor.
The patient’s Ca125 was markedly elevated at 1624 U/mL (nl ≤34).
Contrast CT chest revealed a right-sided pleural effusion but no suspicious nodules or lymphadenopathy.
The patient was deemed to have operable disease and was scheduled for debulking surgery.
However, preoperative laboratory studies showed that her serum calcium had further increased to 19.2 mg/dL.
She had also developed evidence of acute kidney injury, with her BUN rising to 37 mg/dL and creatinine to 1.81 mg/dL.
On physical exam, she was noted to be lethargic, with dry mucous membranes.
The surgery was cancelled due to the patient’s medical instability.
Further workup revealed an elevated PTHrP of 301 pg/mL (nl 14–27), a depressed PTH level of 3 pg/mL (nl 15–65), and a depressed 25-OH vitamin D level of 16 ng/mL (nl 30–100), consistent with humoral hypercalcemia of malignancy.
The patient was treated with pamidronate, calcitonin, and intravenous fluids.
She eventually required temporary hemodialysis and denosumab for refractory hypercalcemia.
The patient improved with regard to her electrolyte abnormalities.
Given the extent of her disease, she was planned for neoadjuvant chemotherapy followed by surgery if a good response was achieved.
She was started on a regimen of carboplatin and paclitaxel, which she received for 3 cycles.
She also had palliative radiation to the uterus to control bleeding.
However, her disease failed to respond significantly to chemotherapy, and she ultimately died from complications of malignancy.