[Leiomyosarcoma of the heart--interdisciplinary therapeutic approach of systemic chemotherapy and subsequent heart transplantation].
METHODS
A 35-year-old man reported progressive exercise dyspnea, decreased exercise tolerance and weight loss of 10 kg in 2 months. Imaging tests demonstrated a tumor of maximally 7 cm diameter in the region of the left atrium. Histologically it was a poorly differentiated leiomyosarcoma G 3, which could not be completely resected (R 2 resection).
Six weeks after surgical resection a rapidly growing tumor, maximally 7.5 cm in diameter, was visualized at the right lateral region of the left atrium. It filled it almost completely and extended via the right upper pulmonary vein to the right hilus.


METHODS
As part of a multimodal therapeutic approach he was given 6 cycles of chemotherapy (doxorubicin and ifosfamide), bringing about significant reduction of the tumor size. The tumor was then completely resected, followed by an orthotopic heart transplantation with right pneumonectomy. The resected surgical specimen indicated complete remission, no vital tumor cells being demonstrated. No tumor recurrence or distal metastasis was seen during a follow-up period of 36 months. The patient died from right heart failure with advanced pulmonary hypertension 45 months after the diagnosis had first been made, 37 months after the cardiac transplantation.
D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D004317:Doxorubicin / Q000627:therapeutic use; D017809:Fatal Outcome; D006325:Heart Atria; D006338:Heart Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D007890:Leiomyosarcoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D008297:Male; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D011013:Pneumonectomy
D000970:Antineoplastic Agents; D004317:Doxorubicin; D007069:Ifosfamide