Endocrine Abstracts

Background

Hypercalcaemia is commonly encountered during clinical practice. SCCOHT is a rare ovarian malignancy typically found in young women. In two thirds of patients, it causes a paraneoplastic hypercalcaemia which is usually asymptomatic.

Case Report

A thirty-seven-year-old lady, presented to casualty with a one -week history of worsening nausea, vomiting, anorexia, abdominal pain, polydipsia, and polyuria. On examination all clinical parameters were within normal range. Her abdomen was distended but soft. Blood investigations revealed a corrected calcium level of 3.9 mmol/l. She was started on intravenous normal saline at one litre every six hours as acute management for hypercalcaemia. Additional tests revealed a PTH of <5 (15–65 pg/ml), a PTHrP of 5.7 ( <1.5 pmol/l), a 25-hydroxyvitamin D level of 17 (30–100 ng/ml) and normal renal function. The combination of a low PTH together with a high parathyroid hormone related protein (PTHrP) suggests humoral hypercalcaemia of malignancy. A computed tomography scan of the abdomen showed a large, hypodense, soft tissue pelvic mass arising from the left ovary. A magnetic resonance scan of the pelvis confirmed an 18×12×5 cm mass arising from left tubo-ovarian region with areas of internal cystic change, internal necrosis, prominent venous drainage, and a peripheral enhancement pattern A bone scan showed no evidence of abnormal foci of increased tracer uptake throughout the skeletal system. Despite three days of continuous fluid replacement her serum calcium remained high. She received one dose of the intravenous bisphosphonate Zoledronic acid 4 mg which reduced her calcium to 2.38 mmol/l after 48 h reaching a nadir of 1.94 mmol/l within five days. She underwent a bilateral salpingo-oopherectomy and total abdominal hysterectomy. Histology showed a left SCCOHT with sheets of small, uniform, hyperchromatic cells together with a single cystic pseudofollicle; very typical for SCCOHT. She is receiving Cisplatin/Etoposide combination chemotherapy to be followed by pelvic radiotherapy. A repeat CT scan after 3 cycles showed no distant metastases. Her calcium levels have since remained normal, repeat PTHrP and PTH levels have normalised.

Conclusion

SCCOHT was initially reported in 1979 by Scully. Fewer than 500 cases have been reported worldwide. Abdominal pain is the most frequent symptom at 68%. At surgery 50% of patients have extra ovarian spread. It has a high rate of recurrence and overall survival is less than 10%. A combination of surgery, chemotherapy ± stem cell transplant and radiotherapy produce the best results.