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+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.