Brown Tumors of the Facial Bones as an Early Manifestation of Primary Hyperparathyroidism due to Sporadic Single Adenomas in Three Young Adults

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 234-256-Bone & Calcium Metabolism: Clinical Trials & Case Series
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-241
Manuela GM Rocha-Braz*, Adriana M Fernandes, Pedro Henrique S Correa and Bruno Ferraz-de-Souza
University of Sao Paulo Sch of Medicine (FMUSP), Sao Paulo, Brazil
Background: Primary hyperparathyroidism (PHPT) is more frequent in post-menopausal women and currently mainly diagnosed through biochemical abnormalities. Osteitis fibrosa cystica and brown tumors (BTs) are rare findings considered to be late features of advanced disease. BTs are more frequently found in long bones, ribs and pelvis. Here we describe 3 cases of PHPT presenting in the 3rd & 4th decades of life with BTs of facial bones as an early manifestation.

Clinical Cases: Case 1 is a 27-yr-old man with 2-mo history of painless right inferior jaw mass followed by proximal weakness and polyuria. Blood analyses revealed: total serum calcium levels (Ca) 14.2 mg/dl (ref range 8.6-10.2), phosphate (P) 2.1 mg/dl (2.7-4.5), alkaline phosphatase (AP) 450 U/L (40-129) and PTH 1,217 pg/ml (16-87). CT scan revealed lytic lesions in the mandible and maxilla, with max 35 mm diameter. Biopsy showed a giant cell lesion compatible with BT, excluding ossifying fibromas typical of HPT-jaw tumor syndrome.

Case 2 is a 37-yr-old woman with 7-mo history of right mandibular painful swelling, weakness and memory loss. Blood tests showed Ca 14.0 mg/dl, P 2.2 mg/dl, AP 618 U/L, and PTH 2,399 pg/ml. Head CT revealed multiple lytic expansive bone lesions, the 2 largest in the right maxilla and mandible (24 and 21 mm), confirmed to be giant cell lesions by biopsy.

Case 3 is a 32-yr-old woman with 2-yr history of an enlarging cervical nodule followed by progressive weakness and muscular pain. She complained of a painful swelling of the right inferior gum in the previous month. Blood tests revealed Ca 15.7 mg/dl, P 2.0 mg/dl, AP 580 U/L, and PTH 1,472 pg/ml. Head CT showed mandibular and maxillary expansive lytic lesions with up to 35 mm, confirmed to be giant cell lesions.

All 3 subjects had multiple skeletal lytic lesions, low BMD in the distal radius (Z-score -6.4, -4.0, and -4.6 respectively) and negative screening for familial forms of PHPT. Single parathyroid (PT) adenomas measuring 33, 40 and 71 mm, respectively, were surgically resected, leading to resolution of PHPT.

Clinical Lesson: We describe 3 cases of rapid-onset PHPT presenting at young age with severe biochemical and skeletal disease, including histologically confirmed BTs in facial bones. Other rare reports in the literature with similar presentation suggest that a more aggressive form of PHPT with facial BTs might be more frequent in a younger population, potentially due to different mechanisms of disease.

Nothing to Disclose: MGR, AMF, PHSC, BF

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm