Una enfermedad granulomatosa “sarcoidosis-like”. A propósito de un caso clínico / A sarcoidosis-like granulomatosis disease. Based on a clinical case

La asociación de sarcoidosis e inmunodeficiencia variable común (IVC) se ha considerado como una enfermedad con entidad propia denominada síndrome “sarcoidosis-like”, cuya verdadera incidencia y su mecanismo responsable se desconocen.La clave podría encontrarse en aspectos inmunológicos que ambos procesos comparten. Presentamos el caso de unapaciente con diagnóstico de síndrome de Larsen1, en la que documentamos la concurrencia de una IVC y una sarcoidosis. Su evolución clínica y radiológica fue satisfactoria, tras iniciar tratamiento sustitutivo con inmunoglobulinas y corticoides

The association of sarcoidosis and common variable immunodeficiency (CVI) has been considered as a disease with its own entity called “sarcoidosis-like”syndrome. Its real incidence and responsible mechanism are unknown. The key could be found in immunological features shared by both conditions. We present the case of a female patient diagnosed oh Larsen1syndrome in which we document the concurrence of CVI and sarcoidosis. Its clinical and radiological course wassatisfactory after immunoglobulin and corticosteroid replacement treatment was begun (AU)

Paget's disease of the skull with osteosarcoma and neurological symptoms associated.

Paget's disease is a common entity but osteosarcoma arising in Paget's disease is an infrequent complication. Primary osteosarcomas are typically long bone tumours and the skull is rarely affected. A case with malignant transformation of Paget's disease of the skull and an unusual clinical presentation is reported. Computed tomography scans of the tumour area show interesting results. Main features are: an intensive sclerotic reaction of the host bone, an inner and outer spiculation of the calvarium, radial ossifications in the soft extracranial mass, and spotty calcifications and ossifications of the osteosarcomatous portion infiltrating the brain.

Hemangiopericytoma of the breast: mammographic and sonographic findings.

The authors describe the mammographic and B-mode and color Doppler sonographic findings in a breast hemangiopericytoma in a 49-year-old woman. High vascularity was noted on color Doppler scans with the presence of a single vascular feeding pedicle. An update of the literature is included.