Diagnostik und Therapie primärer mediastinaler Liposarkome

Abstract

EinleitungLiposarkome gehören mit einer Inzidenz zwischen 6 und 18 % unter den Sarkomen zu den häufigsten mesenchymalen Malignomen im Erwachsenenalter [7]. Dennoch ist die primäre Lokalisation im Mediastinum eine Rarität [25]; bis jetzt wurden weniger als 100 Fälle in der Literatur mitgeteilt [2,6,11,16,23]. Extramediastinal finden sie sich sonst hauptsächlich im Bereich der unteren Extremitäten, des Schultergürtels und des Retroperitoneums [5,7,10,11] AbstractMediastinal liposarcoma are of rare entity; there are less than one hundred cases published worldwide. Because of the equivocal and inapparent symptomatology clinical signs often are falsly estimated, and in time of surgery there is generally seen a wide spread tumor growth, so that a radical resection often is impossible. Radiotherapy and chemotherapy are of limited value and mean no chance for curative therapy. The case of a 46 years old patient with an extensive primary liposarcoma of the mediastinum and the results of the review of the literature will be discussed. Kasuistik und Übersicht 22Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.

Primary liposarcoma of the heart

“…Although liposarcoma is the most common tumour of mesenchymal origin in adults, a mediastinal location is extremely rare 4 8–14. Primary mediastinal liposarcoma accounts for only 0.13–0.75% of all mediastinal tumours15 and constitutes about 13% of all cardiac sarcomas 6 7 16 17.…”

Lipossarcoma de mediastino: relato de caso

“…Although liposarcoma is the most common tumor of mesenchymal origin in adults, a mediastinal location is extremely rare. (1)(2)(3)(4)(5)(6)(7)(8) According to the experience of the Armed Forces Institute of Pathology, in collaboration with the Mayo Clinic, 75% of the cases of liposarcoma develop in deep muscle tissues of the extremities, 20% develop in the retroperitoneum, and the remainder develop at other sites. (3) Primary mediastinal liposarcoma accounts for only 0.13-0.75% of all mediastinal tumors.…”

“…(9) In 2003, there were fewer than 100 cases reported in the international literature, 36 of which occurred in Japan. (8) These tumors can present as well-differentiated neoplasms with a pseudocapsule, which makes it difficult to characterize malignancy, and it is necessary to analyze all of the surgical samples in search of cellular atypias. However, they can manifest as high-grade tumors, which makes prognosis poor.…”

“…(11) It remains asymptomatic until reaching a considerable volume, although the symptoms remain nonspecific. (2,8,12) Most patients complain of dyspnea or chest pain, and some also report cough or weight loss. (12,13) Although the radiological findings are nonspecific, computed tomography and magnetic resonance imaging provide useful data.…”

“…(3,4,14) Radiotherapy and chemotherapy have limited value and are ineffective treatment modalities. (8,11) Radiotherapy can be useful in the palliation of unresectable tumors or in the adjuvant treatment of those incompletely resected. (11,15) The role of adjuvant chemotherapy remains controversial.…”