Endocrine Abstracts

Introduction

Parathyromatosis is a potential cause of recurrent hyperparathyroidism, of rare etiology, described as multiple nodules of hyperfunctioning benign parathyroid tissue, it constitutes less than 1% of the cases of recurrent hyperparathyroidism and since its description in 1975, less than 40 cases have been reported Worldwide. The presentation in primary hyperparathyroidism is extremely rare, with around 20 cases reported in the English literature. In the databases reviewed, no cases published in Cuba or in the rest of Latin America were found.

Case report

43-year-old female patient, with 18-years history of recurrent and persistent primary hyperparathyroidism due to secondary parathyromatosis that debuted with a brown cell tumor; currently with multiple spontaneous fractures, bone deformations and the onset of of stage II chronic kidney disease secondary to bilateral nephrolithiasis and moderate right hydronephrosis. Parathyroidectomy of 6 glands was performed prior to the identification of the lesions by imaging studies, at four surgical times in a period of 18 years, associated with right hemithyroidectomy due to incidental finding of papillary thyroid carcinoma. In the last admission with serum parathyroid hormone of 1000 pg/ml, serum calcium of 3.9 mmol/l, serum phosphorus of 0.73 mmol/l. A radioguided left lower parathyroidectomy was performed, obtaining a 4.8 × 2.7 cm gland. The pathological study reported parathyroid tissue with proliferation of oxyphilic cells with lesion limited to the capsule. After the intervention, parathyroid hormone levels decreased by 40% (610 pg/ml) but remained high, with normalization of serum calcium and phosphorus. He continues to be monitored for external consultation.

Conclusion

The treatment of choice is cytoreduction of all identifiable parathyroid tissue remains, while treatment aimed at removing all disseminated nodules is rarely successful, patients are initially controlled with subtotal parathyroidectomy, however reintervention is often necessary, as noted in this case, cinacalcet is a medical option. Parathyromatosis is a rare cause of recurrent and persistent hyperparathyroidism. Preoperative diagnosis continues to be a challenge even with the advent of new technologies, even after 40 years of being described. Its pathophysiology continues to be studied and discussed. Lack of knowledge about this entity can result in unsuccessful attempts to control and eradicate the disease. It is important that it be considered especially in patients with persistent hyperparathyroidism.