Rara localización de tumor pardo en paciente pediátrico / Rare brown tumor location in chronic renal failure

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Natural history and surgical treatment of brown tumor lesions at various sites in refractory primary hyperparathyroidism.

OBJECTIVE: Nowadays, the occurrence of brown tumor lesions or osteitis fibrosa cystica caused by long-lasting primary hyperparathyroidism are very rare, since measuring serum calcium became available routinely in the mid-1970s. It is a tumor-like lesion that may affect the entire skeleton, often presenting with diffuse focal bone pain or by pathological fracture. METHODS: We describe our experience of brown tumor lesions at different skeletal sites that were treated at our trauma centre within the last two years. This included surgical therapy for the indications (i) pain at the pelvis, (ii) increased risk for pathological fracture at the tibia and (iii) acute radicular symptoms at the lumbar spine. The literature was reviewed for the current understanding of the pathophysiology as well as therapy of brown tumor lesions in primary hyperparathyroidism. RESULTS: Curettage of a left-sided iliac crest brown tumor terminated focal pain. A less invasive stabilisation system and bone cement decreased both patient pain and the fracture risk of brown tumor lesion sites of the shinbone; and internal fixator including laminectomy at the lumbar spine ended radicular symptoms. CONCLUSION: Patients with refractory primary hyperparathyroidism should be monitored closely by endocrinologists and the patient's serum calcium level should be adjusted as far as possible. Radiography is required only if focal bone pain or pathological fractures or radicular symptoms occur. Surgery should be considered if large bone defects with spontaneous fracture risk or increasing pain are present. Tumor curettage, Palacos plombage and less invasive stabilisation systems have proved to be acceptable surgical options.

Development of renal bone disease.

Renal osteodystrophy (ROD) develops as the early stages of chronic renal failure (CRF) and covers a spectrum of bone changes observed in the uraemic patient, which extend from high remodelling bone disease (frequently known as osteitis fibrosa) to low turnover, or adynamic disease. Between these two extremes there are also cases of bone mineralization compromised in variable degrees, as is the case of 'mixed bone disease' and osteomalacia. The dynamic process of bone remodelling is compromised in CRF, and a positive or negative bone balance can be observed in uraemic patients. In addition to the classic modulators of bone remodelling, like parathyroid hormone, calcitriol and calcitonin, other factors were recently identified as significant modulators of osteoblast and osteoclast activation in uraemic patients. In fact, different cytokines and growth factors, acting at an autocrine or paracrine level, seem to play a relevant role in the bone and mineral changes observed in uraemia. Recently, observations have been made of the development of more sensitive and specific techniques to assay different biochemical markers of bone turnover and mineral metabolism. Analogously, new contributions of conventional bone histology, bone immunocytochemistry and molecular biology, which enabled the understanding of some etiopathogenic mechanisms of ROD, were observed.

[Imitation of metastatic lesions in patients with brown tumor]. / Imitacja przerzutów nowotworowych u chorych z guzem brunatnym.

The authors have presented two cases of hyperparathyroidism in male patients at the age of 52 and 77 years in whom radiological findings imitated osteolytic, metastatic bone lesions. It has been emphasised that the difficulties with proper diagnosis of brown tumor often occur and the final diagnosis is based on the exact pathology test. In patients with pathological fracture resection with margins and intramedullary fixation, as well as total hip replacement were performed. Postoperative observation revealed that the pain and radiological changes stopped after parathyroid gland resection as the main reason of the brown tumor. The authors have confirmed that open biopsy should be a routine procedure in patients with suspicion of brown tumor.

Primary hyperparathyroidism: pathophysiology and impact on bone.

Primary hyperparathyroidism has been associated with bone loss, especially at cortical skeletal sites. Results from studies evaluating the mineral density of cancellous bone have been more difficult to interpret. Most densitometry studies support the concept that the parathyroid hormone appears to be catabolic at cortical sites and may have anabolic effects at cancellous bone sites. Studies completed to date, however, have been limited by design, definitions of fracture and inadequate control groups. Primary hyperparathyroidism is now increasingly being detected during the asymptomatic phase. The need for parathyroidectomy has been questioned in such patients because there may be no disease progression in the absence of surgery. Medical management of primary hyperparathyroidism has to date been limited to estrogen replacement therapy in postmenopausal women. Identification of the calcium receptor has improved our understanding of calcium homeostasis, and significant reductions in calcium receptor levels have been detected in parathyroid adenomas. Thus, a new class of therapeutics may include the calcimimetic agents. Bisphosphonates are also currently being evaluated with regard to their impact on fracture prevention and their beneficial effects on bone mineral density.

Osteitis fibrosa: treatment trends.

Renal osteodystrophy is frequently seen in patients with end stage renal disease. Osteitis fibrosa associated with secondary hyperparathyroidism is often diagnosed. Treatment options vary based on disease severity. Individual patient considerations need to be addressed to determine the best therapeutic plan. Medical management with calcitriol, dietary modifications, and administration of phosphate binders continues to be the best treatment for most patients. Parathyroidectomy should be reserved for patients with complications related to severe irreversible hyperparathyroidism and/or failure of medical management. Total resection of all parathyroid tissue with or without autotransplant is the most common surgery for hyperparathyroid bone disease.

[Spontaneous pulmonary hernia and Recklinghausen disease]. / Hernie pulmonaire spontanée et maladie de Recklinghausen.

The pulmonary hernia is a rare entity defined by protrusion of pulmonary tissue through an abnormal weakness of the thoracic wall. We describe a case of spontaneous pulmonary hernia associated with osseous sternocostal abnormalities in a patient with a Recklinghausen disease.