Hypercalcemia and parathyroid hormone- related peptide expression in a 3 months old boy with Colon Hemangioendothelioma

MAURY, KELLY; MARTÍN, MA. JULIA; FIGUEROA, VERÓNICA; YOGUI, ANALÍA; GENTILI, CLAUDIA; BRUNETTO, OSCAR

Conferencia; 9th International Conference on Children?s Bone Health; 2019

Introduction Epithelioid hemangioendothelioma (HEE) is a tumor of vascular origin, infrequent in the pediatric age and even more infrequent at intestinal level. To our knowledge, there are no previous reports of pediatric patients with malignanthumoral hypercalcemia associated with this tumor. Humoral mechanism is seen more often in lung, uterine ce ́rvix, skin and esophagus tumors. The presence of hypercalcemia appears to be an ominous prognostic sign.ObjectiveTo report the first case of a patient with hypercalcemia related to PTH-rPassociated with Colon HEE in a pediatric patient.Case descriptionA 3 months years old boy was admited because of clinical worsening and palpableabdominal mass. Initial laboratory investigation revealed hypercalcemia with the following workup: PTH: 1.65 pg/ml, calcium: 25.1 mg/dl, phosphorus 2.9 mg/dl25ohvitamine D:25.2 ng/ml, Urine catecholamines were normal. Ultrasoundvisualized a highly vascularized tumor with calcifications in retroperitoneum of8!6!6 cm located between liver and right kidney. Biphosphonates andCalcitonin were iniciated without improvement. Biopsy reported epithelioidhemangioendothelioma and angiography revealed tumor irrigated by the middlecolic artery. Selective embolization was performed with spongostan and 24 hourslater, tumor exeresis was achieved. PTHrP mRNA was identified in the tumor.After surgery the patient attained normocalcemic state, PTH levels normalizedand remained normocalcemic to date, 18 months later.ConclusionWe report the first case of PTH-rp related hypercalcemia, with mesocolonepithelioid hemangioendothelioma a pediatric patient. PTH-rp mRNA wasdetected at tumor level, and the patient resolved hypercalcemia with tumorresection, remaining normocalcemic and with normal PTH levels since then.Selective embolization was important in order to facilitate tumor resectionsuccessfully, and improving morbidity and mortality of this surgery.DisclosureThe authors declared no competing interests.