Isolierte Amyloidose der Hiluslymphknoten

Abstract

Pseudotumoral mediastinal and pulmonary amyloidosis occur infrequently and may be confused with other tumors that are found more commonly in this region. Enlargement of hilar lymph nodes in the absence of pulmonary involvement is extremely rare. We report a case of nodular amyloidosis of the hilar lymph nodes that was studied using different diagnostic and radiologic methods. A previously healthy 79-year-old man presented with a large right hilar mass detected on routine chest roentgenography. The extensive mass behaved like a solid tumor and mimicked a bronchogenic carcinoma. At thoracotomy, frozen sections revealed the tumor to consist of kappa-light-chain amyloid. In light of the irresectibility of the tumor, thoracotomy was ended as open biopsy. Immunoelectrophoretic analyses of the plasma and urine showed no evidence of a paraprotein immunoglobulin or light chains. The patient was discharged without symptoms of disease. Different diagnostic procedures before and after surgery are discussed.

Zusammenfassung

Die isolierte hiläre Amyloidose des Mediastinums ohne pulmonale Beteiligung gehört zu den Raritäten der Thoraxchirurgie. Anhand eines Fallberichts wird die präoperativ schwierige Abgrenzung zum zentralen Bronchuskarzinom und zu mediastinalen Lymphomen demonstriert. Trotz umfangreicher bildgebender sowie bioptischer Diagnostik ergab erst die offene Biopsie mit anschließender Immunhistochemie das Vorliegen einer κ-Leichtkettenamyloidose der hilären Lymphknoten. Neben einer Literaturübersicht werden prä- und postoperative Diagnoseverfahren bei der Amyloidose diskutiert.