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Extramedulläres Plasmozytom des Kehlkopfs
Abstract
Zusammenfassung Ausserhalb des Knochenmarks entstehende extramedulläre Plasmozytomherde können sich als primäre (echte) Schleimhautplasmozytome oder im Rahmen eines multiplen Myeloms manifestieren und befallen in erster Linie den oberen Aerodigestivtrakt. In der vorliegenden Arbeit wird der Fall eines unifokalen, primär extramedullären Larynxplasmozytoms vorgestellt. Diese Lokalisation ist ausserordentlich selten, in der Weltliteratur werden nur etwa 60 derartige Fälle beschrieben, meist war die Supraglottis befallen. Das Larynx-plasmozytom hat im Vergleich zu extramedullären Plasmozytomherden anderer Lokalisation eine günstigere Prognose, die Therapie besteht grundsätzlich in chirurgischer Entfernung, Bestrahlung oder der Kombination beider.
... If EMP is present in the soft tissue and is locally and well operable, then surgical excision usually is sufficient. 46 Of all of the patients with EMP outside the UAD, 55.6% were treated with surgery alone. However, complete removal of an EMP often is not possible, especially in the UAD, because adjacent vital organ structures may preclude a radical intervention. ...
Extramedullary plasmacytoma (EMP) is a rare entity belonging to the category of non-Hodgkin lymphoma. EMPs make up 4% of all plasma cell tumors and occur mainly in the upper aerodigestive tract (UAD). Seven patients with EMP included in this evaluation were under the authors' care and have been clinically followed since 1990. Because there are no general guidelines for the treatment of patients with EMP, the authors tried to obtain detailed data about the occurrence of this disease and also reviewed the therapies that have been used. To do so, they evaluated all EMP cases published in the medical literature until now and included their own experience.
Extramedullary plasmacytoma (EMP) is a rare entity that belongs to the category of non-Hodgkin lymphoma. EMP make up 4% of all plasma cell tumors and occur mainly in the upper aerodigestive tract. Primary plasmacytoma involving the orbit is rare. There is a relation between solitary plasmacytoma and subsequent development of multiple myeloma. As no predictors of progression have been identified patients need indefinite follow-up. The authors report a case of EMP involving the eyelid in an otherwise healthy adult female.
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