Differentiating Brown Tumor From Bone Metastasis in Parathyroid Cancer Using 18 F-FDG PET and 99m Tc-MIBI SPECT.

ABSTRACT: A 69-year-old woman presented with a right clavicle pain. CT revealed a pathological fracture of the right clavicle, multiple osteolytic lesions, and a left cervical mass. 18 F-FDG PET/CT demonstrated a marked FDG uptake in the cervical mass and osteolytic lesions indicative of metastatic parathyroid cancer. 99m Tc-MIBI SPECT/CT revealed either faint or no uptake in the osteolytic lesions. However, a histopathological analysis after a parathyroidectomy and right clavicle biopsy confirmed the diagnosis of parathyroid cancer and the presence of benign brown tumors secondary to hyperparathyroidism. Postoperative imaging showed sclerotic change and a decreased FDG uptake in the bone lesions.

Bone disease in primary hyperparathyroidism / Doença óssea no hiperparatiroidismo primário

Bone disease in severe primary hyperparathyroidism (PHPT) is described classically as osteitis fibrosa cystica (OFC). Bone pain, skeletal deformities and pathological fractures are features of OFC. Bone mineral density is usually extremely low in OFC, but it is reversible after surgical cure. The signs and symptoms of severe bone disease include bone pain, pathologic fractures, proximal muscle weakness with hyperreflexia. Bone involvement is typically characterized as salt-and-pepper appearance in the skull, bone erosions and bone resorption of the phalanges, brown tumors and cysts. In the radiography, diffuse demineralization is observed, along with pathological fractures, particularly in the long bones of the extremities. In severe, symptomatic PHPT, marked elevation of the serum calcium and PTH concentrations are seen and renal involvement is manifested by nephrolithiasis and nephrocalcinosis. A new technology, recently approved for clinical use in the United States and Europe, is likely to become more widely available because it is an adaptation of the lumbar spine DXA image. Trabecular bone score (TBS) is a gray-level textural analysis that provides an indirect index of trabecular microarchitecture. Newer technologies, such as high-resolution peripheral quantitative computed tomography (HR-pQCT), have provided further understanding of the microstructural skeletal features in PHPT.

A doença óssea no hiperparatiroidismo primário grave é representada pela osteíte fibrosa cística (OFC). Dor óssea, deformidades esqueléticas e fraturas patológicas são achados comuns na OFC. A densidade mineral óssea está, usualmente, extremamente diminuída na OFC, mas é reversível após a cura cirúrgica. Os sinais e sintomas da doença óssea grave incluem dor óssea, fraturas patológicas e fraqueza muscular proximal com hiper-reflexia. O comprometimento ósseo é tipicamente caracterizado pela aparência em “sal-e-pimenta” nos ossos do crânio, erosões ósseas e reabsorção das falanges, tumores marrons e cistos. Na radiografia, observam-se desmineralização difusa e fraturas patológicas especialmente nos ossos longos das extremidades. No hiperparatiroidismo primário (HPTP) sintomático grave, as concentrações séricas de cálcio e PTH estão usualmente bem elevadas e o comprometimento renal se caracteriza pela presença de urolitíase e nefrocalcinose. Uma nova tecnologia, recentemente aprovada para uso clínico nos Estados Unidos e na Europa, torna-se provável se difundir rapidamente, pois utiliza as imagens geradas pela densitometria DXA. O escore trabecular ósseo (TBS), obtido por meio da análise do nível da textura cinza das imagens dos corpos vertebrais, fornece informações indiretas sobre a microarquitetura trabecular. Novos métodos, como a tomografia de alta resolução quantitativa periférica computadorizada (HRpqCT), têm proporcionado conhecimentos adicionais sobre os achados da microarquitetura esquelética no HPTP.

Schwannoma benigno del cuello

Objetivos: Descripción de un nuevo caso clínico de Schwannoma del cuello. Métodos: Presentamos un caso clínico de tumoración del cuello en un paciente de 64 años y la conducta terapéutica seguida ante el mismo. Resultado: El estudio anatomopatológico demostró la presencia de un schwannoma sin signos de malignidad. Conclusiones: El schwannoma del cuello es una entidad infrecuente. El diagnóstico preoperatorio es difícil y su tratamiento es la exéresis quirúrgica (AU)

Tumores pardos en múltiples localizaciones: manifestación de osteodistrofia renal severa. Caso clínico / Brown tumors in multiple locations: manifestation of severe renal osteodystrophy. Case report

We report a 25 years old woman with a four years history of end stage renal disease on hemodialysis. During the last two years of follow up, multiple tender nodules appeared in the left clavicle and ribs. These were diagnosed as brown tumors, secondary to osteitis fibrosa cystica, in the context of a secondary hyperparathyroidism. A subtotal parathyroidectomy was performed and a homologous parathyroid implant was done in the right forearm. The pathological study of the surgical pieces confirmed the presence of the brown tumors and parathyroid hyperplasia. Nine months after surgery, parathormone levels and brown tumor calcifications decreased significantly and bone mineral density increased by 20 per cent.

Feocromocitoma bilateral asociado con enfermedad de Von Hippel-Lindau: estudio genético / Bilateral pheochromocytoma associated with von hippel-lindau syndrome: genetic study

Un 10 por ciento de los feocromocitomas son bilaterales.Además el 10 por ciento es familiar y puede asociarse de forma más frecuente con el síndrome MEN 2a o 2b, o bien con la enfermedad de Von Hippel-Lindau, la enfermedad de Von Recklinghausen y el síndrome de Sturge-Weber. Presentamos el caso de una mujer de 18 años con dos tumoraciones dependientes de ambas glándulas adrenales que se visualizan en una resonancia magnética (RM). Se realiza una adrenalectomía bilateral, así como un estudio genético de la paciente, en el que se detecta una mutación en el exón 3 del gen VHL. El estudio genético de los progenitores fue negativo (AU)

Cytokine accumulation in osteitis fibrosa of renal osteodystrophy

Bone marrow fibrosis occurs in association with a number of pathological states. Despite the extensive fibrosis that sometimes characterizes renal osteodystrophy, little is known about the factors that contribute to marrow accumulation of fibrous tissue. Because circulating cytokines are elevated in uremia, possibly in response to elevated parathyroid hormone levels, we have examined bone biopsies from 21 patients with end-stage renal disease and secondary hyperparathyroidism. Bone sections were stained with antibodies to human interleukin-1alpha (IL-1alpha), IL-6, IL-11, tumor necrosis factor-alpha (TNF-alpha) and transforming growth factor-ß (TGF-ß) using an undecalcified plastic embedding method. Intense staining for IL-1alpha, IL-6, TNF-alpha and TGF-ß was evident within the fibrotic tissue of the bone marrow while minimal IL-11 was detected. The extent of cytokine deposition corresponded to the severity of fibrosis, suggesting their possible involvement in the local regulation of the fibrotic response. Because immunoreactive TGF-ß and IL-6 were also detected in osteoblasts and osteocytes, we conclude that selective cytokine accumulation may have a role in modulating bone and marrow cell function in parathyroid-mediated uremic bone disease

Síndrome de Albright asociado a hipotiroidismo / Albright's syndrome asociated with hyperthyroidism

La asociación del síndrome de Albright (displasia fibrosa poliostótica, máculas hipercrómicas y trastornos endocrinológicos) con hipotiroidismo aún no han sido descrito. Se trata de una venezolana de 26 años con el clásico síndrome de Albright e hipotiroidismo primario. La paciente tenía una historia de deformidad craneana y de cadera, pubertad precoz, hipotiroidismo y anemia ferropénica. Estudios endocrinológicos demostraron un nivel bajo de tiroxina libre (T4-L) y valores elevados de hormonas estimulante de tiroides (TSH)