Mathews Joseph Wolfgang, Winchester Rhonda, Alsaygh Nebras, Bartlett Anne M, Luttrell Louis
Division of Endocrinology, Diabetes, and Medical Genetics, Charleston, South Carolina.
Department of Medicine, College of Medicine, Charleston, South Carolina.
Am J Med Sci. 2016 Sep;352(3):302-5. doi: 10.1016/j.amjms.2016.06.020. Epub 2016 Jul 1.
Ossifying fibromas of the maxillofacial bones are an uncommon form of benign neoplasm usually treated by surgical excision. Up to 30% of patients with hyperparathyroidism-jaw tumor syndrome, a rare form of multiple endocrine neoplasia resulting from autosomal dominant inactivating mutation of the Hrpt2 tumor suppressor gene, initially present with ossifying fibromas. Coincident hypercalcemia because of the presence of parathyroid adenoma is common in these patients, of whom 15% may have or may develop parathyroid carcinoma. The authors present a case of severe postsurgical hypercalcemia after removal of a large maxillary ossifying fibroma in a patient with previously unrecognized hyperparathyroidism-jaw tumor AU3 syndrome.
颌面部骨化性纤维瘤是一种不常见的良性肿瘤，通常通过手术切除进行治疗。高达30%的甲状旁腺功能亢进-颌骨肿瘤综合征患者最初表现为骨化性纤维瘤，这是一种由肿瘤抑制基因Hrpt2的常染色体显性失活突变导致的罕见的多内分泌腺瘤病形式。由于甲状旁腺腺瘤的存在，这些患者中合并高钙血症很常见，其中15%可能患有或可能发展为甲状旁腺癌。作者报告了一例在切除一名先前未被识别的甲状旁腺功能亢进-颌骨肿瘤综合征患者的巨大上颌骨化性纤维瘤后发生严重术后高钙血症的病例。
