Primary hyperparathyroidism-jaw tumor syndrome: a confusing and forgotten diagnosis

Abstract

Background: Primary hyperparathyroidism is caused by the excessive growth of parathormone secretion, its consequence being hypercalcemia. The parathyroid adenoma is responsible for over half of primary hyperparathyroidism cases. The mandibular tumor can be the initial sign in the case of primary hyperparathyroidism.

Case presentation: We present the case of a 33 year old patient with history of a mandibular operated tumor, repetitive pathological fractures and hypercalcemia manifestations. The level of the parathormone at the first measurement indicated a very high value. The parathyroid scintigraphy with 99mTc-MIBI (methoxy-isobutyl-isonitrile) evidenced a high uptake of the tracerin the superior mediastinum, suggestive for an ectopic parathyroid adenoma. The histopathological examination after surgery leads to the diagnosis of parathyroid adenoma. The association between the primary hyperparathyroidism, the mandibular tumour, the clinical history and the nuclear imaging lead to the diagnosis of primary hyperparathyroidism - Jaw tumor syndrome.

Conclusion: The hyperparathyroidism - Jaw tumor syndrome has a special clinical importance because of the severe and progressive symptomatology, and because of the risk of developing neoplasia of parathyroid glands, which have a reserved prognosis.

Keywords: 99m Tc-MIBI; bone scan; jaw tumor; primary hyperparathyroidism.

Figure 1

Parathyroid scintigraphy with ^99mTc-MIBI. Ectopic parathyroid macroadenoma in the superior mediastinum.

Figure 2

Bone scan ^99mTc-MDP; multiple bone lesions.

Figure 3

Hematoxilin-eosin staining 25× of the ectopic adenoma.

Figure 4

Proliferation index ki-67 in the atypical parathyroid adenoma.