Anomalía linfática generalizada en un paciente adulto, un diagnóstico eminentemente radiológico / Generalized lymphatic anomaly in adult patients: an eminently radiological diagnosis

La anomalía linfática generalizada (ALG) es una enfermedad congénita poco frecuente, secundaria a la proliferación de vasos linfáticos en cualquier órgano a excepción del sistema nervioso central, mostrando un amplio abanico de formas de presentación clínicas y radiológicas. Las lesiones osteolíticas son una constante, y las costillas, el hueso más frecuentemente afectado. Se diagnostica principalmente en niños y adultos jóvenes; no obstante, en raras ocasiones la enfermedad puede ser asintomática y detectarse de forma incidental en pacientes de mayor edad. Presentamos un caso inusual de ALG en un paciente de 54 años, asintomático y con presencia de lesiones óseas de distribución atípica en la tomografía computarizada (TC). La naturaleza puramente quística de las lesiones, evidenciada mediante la medición de las unidades Hounsfield, permitió establecer el diagnóstico de sospecha de ALG, que posteriormente se confirmó con resonancia magnética, tomografía por emisión de positrones/TC, punción aspiración con aguja fina guiada por TC y biopsia vertebral percutánea con guía fluoroscópica. Finalmente, se obtuvo el diagnóstico anatomopatológico definitivo tras la resección quirúrgica de una de las lesiones.(AU)

Generalized lymphatic anomaly (GLA) is an uncommon congenital disease secondary to the proliferation of lymphatic vessels in any organ except the central nervous system. GLA has a wide spectrum of clinical and radiological presentations, among which osteolytic lesions are the most widespread, being the ribs the most commonly affected bone. GLA is diagnosed mainly in children and young adults; nevertheless, on rare occasions it can remain asymptomatic and be detected incidentally in older patients. We present an unusual case of GLA in an asymptomatic 54-year-old man who had atypically distributed, purely cystic bone lesions on CT; measuring the Hounsfield (HU) of these lesions enabled us to suspect GLA. This suspicion was confirmed with MRI, PET/CT, CT-guided fine-needle aspiration biopsy, and fluoroscopy-guided percutaneous vertebral biopsy. After surgical resection of one of the lesions, histologic study provided the definitive diagnosis.(AU)

Generalized lymphatic anomaly in adult patients: An eminently radiological diagnosis.

Generalized lymphatic anomaly (GLA) is an uncommon congenital disease secondary to the proliferation of lymphatic vessels in any organ except the central nervous system. GLA has a wide spectrum of clinical and radiological presentations, among which osteolytic lesions are the most widespread, being the ribs the most commonly affected bone. GLA is diagnosed mainly in children and young adults; nevertheless, on rare occasions it can remain asymptomatic and be detected incidentally in older patients. We present an unusual case of GLA in an asymptomatic 54-year-old man who had atypically distributed, purely cystic bone lesions on CT; measuring the Hounsfield (HU) of these lesions enabled us to suspect GLA. This suspicion was confirmed with MRI, PET/CT, CT-guided fine-needle aspiration biopsy, and fluoroscopy-guided percutaneous vertebral biopsy. After surgical resection of one of the lesions, histologic study provided the definitive diagnosis.