Die druckdolente Schwellung in der Kniekehle als Wegweiser einer monoklonalen Gammopathie unklarer Spezifität (MGUS)

Zusammenfassung

Die AL-Amyloidose tritt häufig in Assoziation mit einer hämatoproliferativen Grunderkrankung wie dem Multiplen Myelom oder dem Morbus Waldenström auf. Das sporadische Auftreten einer AL-Amyloidose ist eine Rarität. In unserer Kasuistik berichten wir über eine 71-jährige Patientin, die sich mit einer schmerzhaften bewegungseinschränkenden Schwellung in der linken Kniekehle in unserer Klinik vorstellte. Weiterführende Diagnostik, die Exstirpation sowie die histologisch/immunhistochemische Aufarbeitung dieser Raumforderung zeigten den Nachweis eines AL-Amyloidtumors. Als ursächlichen Grund hierfür konnte im weiteren diagnostischen Verlauf eine monoklonale Gammopathie unklarer Spezifität (MGUS) nachgewiesen werden. Die vollständige Exstirpation und feingeweblich pathologische Aufarbeitung eines jeden Weichteiltumors mit ggf. weiterführenden Untersuchungen ist erforderlich, um eine Diagnose zu erzwingen. Nur dadurch können mögliche bereits zuvor bestehende, noch nicht diagnostizierte, seltene, ggf. maligne Grunderkrankungen erkannt werden.

Abstract

AL amyloidosis (AL-A) is seen in about 6–15 % of patients with multiple myeloma. In contrast, the sporadic occurrence of AL-A is a rarity. The presence of amyloid is often found in the respiratory tract followed by the lung, the eyelids, the skin and the lower urinary tract, while the presence in soft tissues or bone is rather uncommon. We here describe a 71-year-old woman who presented with a pressure-sensitive swelling in the left knee at our hospital. The clinical examination on admission showed a tumourous alteration of the popliteal soft tissues without any signs of neurological deficits. However, the range of motion (ROM) of the left knee was restricted with 0–0–100 degrees for extension and flexion. A dialysis-dependent renal insufficiency, heart insufficiency with the necessity of permanent anticoagulation and glaucoma were noted as pre-existing illnesses. Further diagnostic examinations including ultrasound and magnetic resonance imaging (MRI) of the left knee revealed a solid tumourous structure with a radiological suspicion of pigmented villonodular synovitis. A complete surgical extirpation of the tumourous structure was performed. The further performed diagnostic examinations including histological and immunohistochemical analyses showed evidence of an AL-A tumour. A monocloncal gammopathy of unknown specificity (MGUS) was detected as an origin for the AL-A. This case underscores the necessity of complete extirpation and histological and immunohistochemical analyses of any soft tissue tumour. Possible previously existing rare malignant diseases can be detected only in this way.

Schlüsselwörter

Amyloidose - Knieschwellung - monoklonale Gammopathie unklarer Spezifität

Key words

amyloidosis - knee swelling - monoclonal gammopathy of undetermined significance

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