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Special Feature
January 2002

Pathological Case of the Month

Arch Pediatr Adolesc Med. 2002;156(1):83. doi:10.1001/archpedi.156.1.83

A PREVIOUSLY healthy 13-year-old girl had nausea, vomiting, fatigue, weakness, and a 1.13-kg weight loss over 1 week without fever or diarrhea. Menarche was at 12 years of age, and she had regular periods.

The patient was admitted to the hospital with a diagnosis of acute gastritis and dehydration. She was afebrile, normotensive, mildly dehydrated, and in no distress. Her sexual maturation was Tanner stage IV. Pelvic examination was deferred initially, and the remaining physical examination results were normal. Results of initial laboratory tests were normal except for a total serum calcium level of 14.1 mg/dL, an ionized calcium level of 7.46 mg/dL (reference range, 4.60-5.30 mg/dL), and a serum phosphorus concentration of 2.2 mg/dL. The urine calcium/creatinine ratio was elevated at 0.53 (reference range, 0.05-0.25), and the result of a pregnancy test was negative. A technetium Tc-99m sestamibi scan showed no evidence of parathyroid adenoma or ectopic parathyroid. A thyroid function test, electrocardiogram, and an abdominal radiograph were all normal. The patient responded well to therapy for symptomatic control of hypercalcemia (ie, intravenous fluids, potassium phosphate, furosemide, calcitonin, and pamidronate). The total serum calcium level fell to 9.6 mg/dL.

The patient's serum parathyroid hormone (intact) level was low (<10 pg/mL) when total calcium was 13.4 mg/dL. The parathyroid hormone–related protein level was elevated at 45.5 pg/mL (reference level, <12.9 pg/mL), and 1,25-dihydroxyvitamin D was slightly elevated at 110 pg/mL (reference range, 15-90 pg/mL). A right lower pelvic mass was found on pelvic examination. Pelvic ultrasound examination found a 15 × 10 × 10 cm solid/cystic mass involving the right ovary. A 15 × 12 × 12 cm (865 g) solid right adnexal mass was found on laparotomy (Figure 1). Frozen sections were highly suspicious for malignancy. The patient underwent a right salpingo-oophorectomy, pelvic and periaortic lymphadenectomy, omentectomy, multiple peritoneal biopsies, and a left ovarian biopsy. The pathologist reported stage IA small-cell carcinoma of the ovary (Figure 2), and specimens stained positive for parathyroid hormone–related protein (1.96 pmol per gram of tissue). All nodes, omentum, peritoneum, and pelvic fluid were negative for parathyroid hormone–related protein. The left ovarian biopsy revealed a benign stromal cortical nodule. The patient developed transient hypocalcemia postoperatively, which was corrected by calcium supplement for a brief period.

At follow-up 10 months later, the patient had completed 6 cycles of chemotherapy with paclitaxel, carboplatin, and oral etoposide without any evidence of recurrence. Serum calcium concentrations were followed as a tumor marker and remained at 8.9 to 9.4 mg/dL. Cancer antigen 125 and alkaline phosphatase levels were normal. Pelvic ultrasound examinations showed no recurrence.

At 13 months' follow-up, the patient had recurrence of hypercalcemia (serum calcium level, 11 mg/dL). A laparotomy showed recurrent small cell carcinoma involving the left ovary, left fallopian tube, rectosigmoid surface, bladder, peritoneum, and abdominal wall. The patient underwent a left salpingo-oophorectomy, hysterectomy, and tumor debulking with no residual tumor visible. She resumed chemotherapy with ifosfamide and topotecan hydrochloride without success. The patient died 36 months after the initial diagnosis.

Accepted for publication September 15, 1999.

We thank Edward C. Kohaut, MD, Nemours Children's Clinic, Pensacola, Fla, and Franklin Abbott, MD (Department of Radiology), William R. Bell, Jr, MD, and Everett Havard, MD (Department of Pathology), Sacred Heart Hospital, Pensacola, for their kind and expedient assistance.

Reprints: Helen Y. Hsiang, MD, 5153 N Ninth Ave, Pensacola, FL 32504 (e-mail: hhsiang@nemours.org).