Metastatic malignant phaeochromocytoma: A rare entity that underlies a therapeutic quandary.
Phaeochromocytoma is a rare condition that provides a diagnostic challenge as a result of its variable presentation. Treatment of metastatic malignant phaeochromocytoma is also not well defined owing to its rarity. We present four such cases and a review of the literature. The database of the Singapore Cancer Registry was used to trace all cases of metastatic malignant phaeochromocytoma from 1984 to 2004, and the case records were then reviewed retrospectively. There were four patients with metastatic malignant phaeochromocytoma seen in Singapore in the last 20 years. Their variable clinical courses were reviewed and compared with current knowledge and overseas experience in the literature. We further discuss the difficulties in diagnosis, and the dilemma in appropriate management of such cases. Phaeochromocytoma remains a commonly missed diagnosis unless a high index of suspicion is maintained. Malignant phaeochromocytoma has a variable clinical course. There is a place for radical surgery if this can render the patient free of gross disease, or when it can achieve symptom control for palliation and improvement in quality of life. In the metastatic context, debulking surgery does not appear to be of curative benefit, although it may be undertaken for good palliation.
Wan
Wei-Hwang
WH
Department of General Surgery, Changi General Hospital, Singapore. wanweihwang@yahoo.com.sg
Tan
Kok-Yang
KY
Ng
Chin
C
Tay
Khoon-Hean
KH
Mancer
Kent
K
Tay
Miah-Hiang
MH
Chia
Whay-Kuang John
WK
Soo
Khee-Chee
KC
Ooi
London Lucien
LL
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000601:surgery; D000311:Adrenal Glands / Q000473:pathology; D000315:Adrenalectomy; D000328:Adult; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis / Q000628:therapy; D010166:Palliative Care; D010673:Pheochromocytoma / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000601:surgery; D011788:Quality of Life; D012189:Retrospective Studies; D013997:Time Factors