Doença de Gorham da escápula e clavícula: relato de caso e evolução de dois anos de uma afecção rara / Gorham's disease of scapula and clavicle: case report and two-year follow-up of a rare disorder

A doença de Gorham é uma rara desordem osteolítica, de etiologia controversa, que pode afetar qualquer osso. O substrato histopatológico é a substituição óssea por uma formação expansiva de natureza vascular agressiva, não neoplásica. Descrevemos os achados radiográficos, tomográficos e de ressonância magnética de um caso desta afecção acometendo a escápula esquerda e, dois anos depois, a clavícula ipsilateral.

Gorham's disease is a rare osteolytic disorder of still controversial etiology that may affect any bone. The histopathological substrate for such a condition is the replacement of normal bone by aggressive non-neoplastic expansile vascular tissue. The authors describe radiographic, computed tomography and magnetic resonance imaging findings in a case of this entity initially affecting the left scapula and, two years later, the ipsilateral clavicle.

Gorhams Disease: Vanishing Bone Syndrome.

Gorhams disease, also known as massive osteolysis or vanishing bone disease is an extremely rare bone disease. It is characterized by angiomatosis with adjacent bone resorption. We report an 8-years old boy with the disease who was managed successfully with alpha 2b interferon therapy.

Chylothorax in Gorham's Disease

A 25-yr-old woman presented with a right pleural effusion. Destruction of 9th through 12th ribs, adjacent vertebral bodies, and transverse processes was noted on plain radiograph and a large low-attenuated, irregular shaped mass lesion with peripheral rim enhancement, destroying vertebral body and transverse process, was revealed on the computed tomographic scan. Magnetic resonance imaging showed high signal on T1- weighted image and iso- and low signal on T2-weighted image for the mass lesion replacing the vertebral bony cortex and marrow space. An open rib biopsy revealed the histopathological changes of Gorham's disease (essential osteolysis), even though only bloody fluid filling the empty space and rib and vertebral transverse process destruction were grossly observed on operation. Even though there was no definite response to radiotherapy and pleurodesis, the patient showed stable condition up to 20 months after diagnosis.

Mediastinal Lymphangioma and Chylothorax: Thoracic Involvement of Gorham's Disease

We report a case of mediastinal lymphangioma associated with Gorham's disease in a 38-year-old man who had suffered recurrent clavicular fractures during a seven-year period. Mediastinal widening associated with osteolysis of the clavicles and the sternal manubrium was revealed by chest radiography, while computed tomography demonstrated a cystic anterior mediastinal mass infiltrating mediastinal fat and associated with osseous destruction of the clavicles and manubrium. Chylothorax recurred during the course of the disease.

Acroosteolisis primaria

Describimos dos casos de acroosteólisis primaria: una mujer de 50 años y un hombre de 34 años quien además presentaba neurofibromas, osteopenia y cardipatía, la cual es una variedad no informada en la literatura. Proponemos una nueva clasificación para la entidad

Artritis psoriasica asociada a acroosteolisis y neuropatia perférica

El diagnóstico de artritis psoriásica se hace cuandopsoriasis y artritis de presentan juntas, sin nódulos subcutáneos y usualmente seronegativa para factor reumatoideo (FR) (1). Wright en 1991 (2) simplificó la clasificación en tres grupos. Revisamos la literatura y no encontramos informes de neuropatía periférica ni osteólisi de la tibia, peroné y tarso en artritis psoriática. Informamos el caso de una paciente con las anteriores manifestaciones clínicas

Sindrome de Gorham: presentación de un caso con linfangiomatosis y osteolisis progresiva / Gorham syndrome: presentation of a case with lymphangiomatosis and progressive osteolysis

En definitiva se trata de un Síndrome de Gorham, con osteolisis masiva de cintura pelviana y lesiones de piel características. Desde el punto de vista histopatológico, se trata de un tumor vascular linfático sin malignidad citológica, pero arquitecturalmente de carácter disecante e infiltrante, biológicamente maligno, originado en restos embrionários. Actuarían eventualmente como factores inductores de su desarrollo el traumatismo y estímulos de carácter hormonal