Adenoma paratiroideo mediastínico gigante. Abordaje cervical y toracoscópico. Caso clínico / Gigant Mediastinal Parathyroid Adenoma. Cervical and Thoracoscopic Approach. Case Report

El hiperparatiroidismo primario es el tercer trastorno endocrino más común, alrededor del 85% de los casos se debe a adenomas paratiroideos. El tratamiento definitivo es la paratiroidectomía, siendo la causa más común de fracaso la resección inadecuada y la localización de tejido ectópico, representando un desafío para el cirujano. En el presente trabajo se presenta el caso de un paciente con diagnóstico de adenoma paratiroideo mediastínico gigante, siendo este el de mayor longitud descrito en la literatura en los últimos 10 años. Caso clínico: Paciente masculino de 70 años de edad quien consulta por presentar convulsiones, polidipsia y poliuria. Se determinan niveles elevados de PTH, hipercalcemia e hipofosfatemia. Estudios de imagen describen lesión alargada localizada en espacio paratraqueal derecho extendiéndose hasta el polo inferior de lóbulo tiroideo derecho. Se decide resolución quirúrgica, mediante la realización de cervicotomía y toracoscopia con evolución satisfactoria del paciente. Conclusión: Los adenomas paratiroideos ectópicos constituyen una causa común de falla quirúrgica e hiperparatiroidismo persistente, su sospecha es de gran importancia. El tratamiento definitivo es la cirugía. La localización preoperatoria por pruebas de imagen es fundamental para seleccionar correctamente la técnica quirúrgica y garantizar el éxito de la cirugía. El abordaje cervical y toracoscópico es una alternativa segura y eficaz(AU)

Primary hyperparathyroidism is the third most common endocrine disorder, about 85% of cases are due to parathyroid adenomas. The definitive treatment is parathyroidectomy, being the most frequent cause of failure the inadequate resection and the location of ectopic tissue. The ectopic parathyroid adenomas represent a challenge for the surgeon. In this paper a case of a patient diagnosed with giant mediastinal parathyroid adenoma is presented, and is the largest reported in the literature in the last 10 years. Clinical case: 70-year-old male patient presented with seizures, polydipsia and polyuria, reason for which he consults. Elevated PTH levels, hypercalcemia, and hypophosphatemia are determined. Imaging studies report an elongated lesion located in the right paratracheal space that extends to the lower pole of the right thyroid lobe. Surgical resolution was decided, by performing cervicotomy and thoracoscopy with satisfactory recovery of the patient. Conclusion: Ectopic parathyroid adenomas are a common cause of surgical failure and persistent hyperparathyroidism; their suspicion is of great importance. The definitive treatment is surgery. Preoperative localization through imaging tests is essential to correctly select the surgical technique and guarantee the success of the surgery, the cervical and thoracoscopic approach is a safe and effective alternative(AU)

Biochemical, bone and renal patterns in hyperparathyroidism associated with multiple endocrine neoplasia type 1

Primary hyperparathyroidism associated with multiple endocrine neoplasia type I (hyperparathyroidism/multiple endocrine neoplasia type 1) differs in many aspects from sporadic hyperparathyroidism, which is the most frequently occurring form of hyperparathyroidism. Bone mineral density has frequently been studied in sporadic hyperparathyroidism but it has very rarely been examined in cases of hyperparathyroidism/multiple endocrine neoplasia type 1. Cortical bone mineral density in hyperparathyroidism/multiple endocrine neoplasia type 1 cases has only recently been examined, and early, severe and frequent bone mineral losses have been documented at this site. Early bone mineral losses are highly prevalent in the trabecular bone of patients with hyperparathyroidism/multiple endocrine neoplasia type 1. In summary, bone mineral disease in multiple endocrine neoplasia type 1related hyperparathyroidism is an early, frequent and severe disturbance, occurring in both the cortical and trabecular bones. In addition, renal complications secondary to sporadic hyperparathyroidism are often studied, but very little work has been done on this issue in hyperparathyroidism/multiple endocrine neoplasia type 1. It has been recently verified that early, frequent, and severe renal lesions occur in patients with hyperparathyroidism/multiple endocrine neoplasia type 1, which may lead to increased morbidity and mortality. In this article we review the few available studies on bone mineral and renal disturbances in the setting of hyperparathyroidism/multiple endocrine neoplasia type 1. We performed a meta-analysis of the available data on bone mineral and renal disease in cases of multiple endocrine neoplasia type 1-related hyperparathyroidism.

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

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 pilot study on hypothalamo-pituitary-adrenocortical axis in primary hyperparathyroidism.

Background & objectives: Parathormone (PTH) and calcium, both have been shown to stimulate adrenal steroidogenesis in animal models and in vitro experiments. This is attributed to structural similarity between 15-25 amino acid region of the parathyroid hormone (PTH) and 1-11 amino acid region of adrenocorticotropin (ACTH). However, there are no in vivo human data regarding the effect of PTHcalcium axis on adrenocortical function. Materials: Ten patients with primary hyperparathyroidism underwent evaluation for cortisol dynamics including 0800 h and 2000 h plasma cortisol on day 1, cortisol response to insulin induced hypoglycaemia (IIH) on day 2, and 1 mg overnight dexamethasone suppression test (ONDST) on day 4. Serum aldosterone was also measured at 0800 h in fasting state on salt ad libitum for three days. These parameters were repeated 3 months after curative parathyroidectomy. Results: Basal plasma cortisol level at 0800 h and 2000 h were within upper normal range and loss of circadian rhythm in cortisol secretion was observed in half and forty per cent of patients had nonsuppressibility with ONDST. The defined peak cortisol response to insulin induced hypoglycaemia (>550 nmol/l) was achieved in all and nearly one third of patients had exaggerated response (>2000 nmol/l). After curative parathyroidectomy, the abnormalities in circadian rhythm and non-suppressibility with ONDST continued to prevail in 40 per cent of patients. The peak cortisol response to IIH showed a decrement but remained higher than normal. No correlation was observed between circulating parathyroid hormone and calcium with cortisol levels. Serum aldosterone was in upper normal range pre - and postoperatively, though it decreased postoperatively, but it could not attain a statistical significance (p = 0.5). Interpretation & conclusion: Abnormalities in hypothalamo-pituitary-adrenocortical axis in primary hyperparathyroidism do occur, however these are inconsistent and do not recover in majority of patients even after 3 months of curative parathyroidectomy.

Paratireóides: estrutura, funções e patologia / Parathyroid glands: structure, functions and pathology

Os autores tecem considerações sobre a estrutura e funções normais das glândulas paratireóides como introdução à patologia e as repercussões clinico - patológicas tanto do excesso como da redução do paratormônio. Maior ênfase é dedicada ao hiperparatireoidismo primário quanto às causas, a fisiopatologia das alterações, os aspectos anatomopatológicos macro e microscópicos das lesões e sua patogenia, na "Osteite fibrocistica" ou "doença de von Recklinghausen dos ossos" com a correlação aos aspectos radiográficos. Apresentam caso de paciente, cuja história clinica demonstra as dificuldades encontradas para o diagnóstico da doença. Referem-se ainda às alterações e patogenia das formas de hiperparatireoidismo secundário e terciário e ao hipoparatireoidismo.

The authors present a summary on the normal anatomy and function of the parathyroid glands as well as a brief review of clinical and pathological repercussions of higher and lower parathyroid hormone production. The emphasis is given on the causes, physiopathology, anatomy, macroscopy and microscopy of the lesions and their role in the genesis of fibrocystic osteitis, also known as Von Recklinhausen disease of the bones. Radiological correlation is also given. The authors show the challenges for the diagnosis in the same cases. We also write about secondary and tertiary hyperparathyroidism, as well as hypoparathyroidism.