Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1

Abstract

The bone mineral density increments in patients with sporadic primary hyperparathyroidism after parathyroidectomy have been studied by several investigators, but few have investigated this topic in primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Further, as far as we know, only two studies have consistently evaluated bone mineral density values after parathyroidectomy in cases of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Here we revised the impact of parathyroidectomy (particularly total parathyroidectomy followed by autologous parathyroid implant into the forearm) on bone mineral density values in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Significant increases in bone mineral density in the lumbar spine and femoral neck values were found, although no short-term (15 months) improvement in bone mineral density at the proximal third of the distal radius was observed. Additionally, short-term and medium-term calcium and parathyroid hormone values after parathyroidectomy in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 are discussed. In most cases, this surgical approach was able to restore normal calcium/parathyroid hormone levels and ultimately lead to discontinuation of calcium and calcitriol supplementation.

A differential diagnosis of inherited endocrine tumors and their tumor counterparts

“…In addition, sporadic insulinoma is frequently treated with surgery by single pancreatic nodule removal, whereas cases with insulinoma/MEN1 are usually submitted to sub-total pancreatectomy (83,117). For gastrinoma in MEN1, subtotal or total duodenopancreatectomy or subtotal pancreatectomy associated with duodenotomy is applied, and partial pancreatectomy is usually the surgical choice in sporadic gastrinoma (33,34,51,52). …”

“…Patients usually have early and severe bone demineralization and renal complications that were considered an important cause of death, at least until 1998 (45,46,158). It is still a matter of debate how to operate on HPT/MEN1 patients (48,51). PTx is advised by most surgeons and clinicians as the optimal approach to HPT/MEN1.…”

“…PTx is advised by most surgeons and clinicians as the optimal approach to HPT/MEN1. However, there is a dispute as to whether subtotal or total PTx would be optimal for treating this condition (51,57,159,160). Subtotal PTx is largely applied, which usually leads to low rates of secondary hypoparathyroidism and may involve lesser surgical complications.…”

“…However, one-third of cases may present HPT relapse/recurrence during the post-surgical follow-up, and substantial surgical difficulties may appear during parathyroid re-intervention (159). Conversely, total PTx followed by parathyroid auto-transplant to the non-dominant forearm is a valuable alternative and may have some advantages (48,51,160). First, post-surgical hypoparathyroidism is most frequently observed until 6-12 months after PTx.…”

Avaliação do método de sequenciamento de nova geração no diagnóstico genético de neoplasia endócrina múltipla tipo 1

“…Estudos avaliando a evolução do tratamento cirúrgico do HPT associado a NEM1 demonstraram ganho, em curto prazo, de massa óssea na coluna e no fêmur (11,12).…”