Hypercalcemic Crisis Secondary to a Parathyroid Hormone Secreting Neuroendocrine Ovarian Tumor

Presentation Number: SAT 327
Date of Presentation: April 1st, 2017

James Castillo Paningbatan*
Makati Medical Center, Philippines


Background: The diagnosis of hypercalcemic crisis can be difficult to make clinically when associated with malignancy. Hypercalcemia of malignancy may be explained by four mechanisms: tumor secretion of Parathyroid Hormone-related Peptide, local release of osteolytic cytokines, ectopic Parathyroid Hormone (PTH) and increased Vitamin D production. Very few cases have been reported in which the production and secretion of intact PTH by a non-parathyroid tumor has been authenticated. A high index of suspicion for a hypercalcemic state should be considered in a patient with a big tumor or cancer in a confusional state.

Clinical Case: A 45 year old woman, was transferred to our institution due to five days history of increasing weakness, lethargy, fever, disorientation and incoherence after falling out of bed. She was initially seen in another institution where a CT scan done showed no signs of acute infarct or bleeding.

Patient was seen weak, delirious and febrile. There was note of a palpable, firm, nontender hypogastric mass. A lumbar tap was done and showed a non xanthochromic, clear CSF. CSF gram stain, fungal smear, india ink, KOH, CALAS, MTB gene expert, Listeria, enteroviral PCR, Herpes simplex, all of which turned out to be negative. CSF sample was examined for anti-NMDA encephalitis and turned out to be negative. Chemistry showed severe hypercalcemia (17.24 mmol/L), hypokalemia (3.3mg/dl), low Mg (1.22 mg/dl), and elevated Creatinine (1.52 mg/dl). Repeat calcium showed hypercalcemia (16.6 mmol/L). Measurement of the PTH revealed a markedly elevated intact PTH (306.7 pg/ml). Patient was hydrated adequately, was given Calcitonin, Cinacalcet and underwent hemodialysis. Ultrasound of the neck and thyroid was negative. Sestamibi scan was negative for a parathyroid adenoma. CT scan of the whole abdomen showed a heterogeneously enhancing foci within the uterine wall 2.8 x 5.2 cm in the posterior wall and 1.8 x 2.5 cm in the anterior wall; a 2.9 x 2.4 cm hypoenhancing focus in the cervical region. There were heterogeneously enhancing masses noted in the bilateral hemipelvis measuring 8.4 x 5.0 x 6.4 cm in the right and 3.4 x 2.5 x 4.1 cm in the left. The patient underwent extrafascial hysterectomy, bilateral salpingooophorectomy, bilateral lymphadenectomy, omentectomy, peritoneal fluid cytology. PTH level was monitored preoperatively (326.89 pg/ml), 6 hours post op (78.375 pg/ml) and 24 hours post op (77.0 pg/ml). Histopathologic diagnosis was a large cell neuroendocrine carcinoma involving the right and left ovary with metastasis to the myometrium; well differentiated endometrial adenocarcinoma. Post operatively, patient started chemotherapy with Carboplatin and Paclitaxel.

Conclusion: These results support the ectopic production of intact PTH by a neuroendocrine tumor and indicate a rare neoplastic cause of hyperparathyroidism.


Nothing to Disclose: JCP