Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institute.
BACKGROUND
Malignant pheochromocytomas are rare. Many controversies still exists in clinical practice. We report the clinical and histological data of long-term follow up in 10 patients with malignant pheochromocytoma.


METHODS
The clinical charts of 10 patients with malignant pheochromocytoma from a single institute were reviewed. The diagnosis of pheochromocytoma was confirmed at surgery. All patients had metastases in sites where chromaffin tissue was normally absent.


RESULTS
The median follow-up period was 5.5 years (range, 2-20 years). Extra-adrenal tumors occurred in four patients including paraganglioma tumors in three cases and bladder tumor in one case. Regional lymph node metastases were noted in six patients. Distant metastases were located in the lungs of two patients, in the bones of two patients and in the liver of one patient. Histological characteristics were not helpful for diagnosis of malignancy and for prediction of prognosis. Metastases were present in postoperative pathology in seven patients. In another three patients, metastases were discovered 6 months to 10 years after surgery. Three patients received chemotherapy and one patient received combination therapy of high-dose (131)I-meta-iodobenzylguanidine ((131)I-MIBG) therapy and chemotherapy. All patients achieved long-term survival except for two who died of metastasis 1.5 years and 2 years after diagnosis.


CONCLUSIONS
Early complete resection and adjunctive lymphadenectomy can cure malignancy. Close long-term follow up for more than 10 years after surgery is necessary in patients with pheochromocytoma. The possibility of malignancy should be kept in mind even though the initial pathology is benign.
Huang
Kuo-How
KH
Department of Urology, National Taiwan University Hospital, No. 7 Chung-Shan S. Road, Taipei 100, Taiwan.
Chung
Shiu-Dong
SD
Chen
Shyh-Chyan
SC
Chueh
Shih-Chieh
SC
Pu
Yeong-Shiau
YS
Lai
Ming-Kuen
MK
Lin
Wei-Chou
WC
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000473:pathology* / Q000628:therapy*; D000315:Adrenalectomy; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000175:diagnosis / Q000556:secondary; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000556:secondary; D008175:Lung Neoplasms / Q000175:diagnosis / Q000556:secondary; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D010673:Pheochromocytoma / Q000175:diagnosis / Q000556:secondary / Q000628:therapy; D049268:Positron-Emission Tomography; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D001749:Urinary Bladder Neoplasms / Q000175:diagnosis / Q000556:secondary
D000970:Antineoplastic Agents