Malignant paraganglioma of the retroperitoneum with lung metastases: a 13-year survivor after radical surgery.
We present a 23-year-old patient with extra-adrenal retroperitoneal paraganglioma with lung metastases who was successfully treated by complete removal of the tumour. Lung metastases were the first manifestation of the disease, and an abdominal computed tomography scan showed a large mass in the retroperitoneum with marked contrast enhancement. Angiography demonstrated a hypervascular mass with many feeding arteries, but vascular invasion was not apparent. The retroperitoneal tumour was resected completely followed by resection of lung metastases after 1 month of observation. The patient was disease free for 13 years after this radical surgery. The survival rate in patients with retroperitoneal paraganglioma with lung metastasis is low, and this case represents the longest surviving period reported in the literature. These tumours are usually large and located in the para-aortic region, and hence resection is sometimes challenging. We believe that a complete and meticulous surgical procedure is a prerequisite for long survival from this rare disease.
Matsui
Hideo
H
Research Institute for Life Extension and Department of Surgery, Eiju General Hospital, Tokyo, and Department of Pathology, Fujita Health University School of Medicine, Aichi, Japan. maheday@u01.gate01.com
Ikeuchi
Shunji
S
Onoda
Noboru
N
Tsutsumi
Yutaka
Y
D000328:Adult; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary* / Q000601:surgery*; D008297:Male; D010236:Paraganglioma, Extra-Adrenal / Q000556:secondary* / Q000601:surgery*; D012186:Retroperitoneal Neoplasms / Q000473:pathology / Q000601:surgery*