Hyper Parathyroidisim Jaw Tumor Syndrome: A Rare Condition of Incongruous Features

Ethiop J Health Sci. 2017 May;27(3):309-313. doi: 10.4314/ejhs.v27i3.14.

Abstract

Background: Hyperparathyroidism-Jaw Tumor (HPT-JT) syndrome is a rare genetic disorder bearing both a germline and a somatic CDC73 mutation (formerly known as HRPT2), which has been mapped to chromosome 1q25-q31. The association of jaw ossifying fibroma with primary hyperparathyroidisim (PHPT) is typical of HPT-JT. It may also include cystic and neoplastic renal abnormalities and uterine tumors.

Case details: Here, we report a case of HPT-JT with an initial presentation of declination in reproductive fitness. Extensive literature search and thorough investigation helped us parturitate the underlying syndrome, thereby predictively improving the prognosis.

Conclusion: The features of HPT-JT are clinically difficult to ascertain because the parathyroid disease, ossifying fibroma in the jaw and other abnormalities, often occurs asynchronously and may be diagnosed and treated separately.

Keywords: HPT-JT; Hyperparathyroidism; Ossifying fibroma; Syndrome.

MeSH terms

  • Adenoma / complications
  • Adenoma / diagnosis
  • Adenoma / pathology*
  • Adult
  • Female
  • Fibroma / complications
  • Fibroma / diagnosis
  • Fibroma / pathology*
  • Genetic Fitness
  • Humans
  • Hyperparathyroidism / complications
  • Hyperparathyroidism / diagnosis
  • Hyperparathyroidism / pathology*
  • Jaw / pathology*
  • Jaw Neoplasms / complications
  • Jaw Neoplasms / diagnosis
  • Jaw Neoplasms / pathology*
  • Parathyroid Diseases
  • Prognosis
  • Rare Diseases
  • Reproductive Health*
  • Tumor Suppressor Proteins / genetics
  • Young Adult

Substances

  • CDC73 protein, human
  • Tumor Suppressor Proteins

Supplementary concepts

  • Hyperparathyroidism 2