Multiple pulmonary chemodectomas in a child: results of four different therapeutic regimens.
OBJECTIVE
Chemodectomas (or paragangliomas) are rare tumors of neuroendocrine chemoreceptors, such as the carotid body. This report describes a case of multiple pulmonary chemodectomas in an adolescent and discusses the results of four therapeutic regimens.


METHODS
At 15 years of age, the patient had cough and fatigue. Investigation revealed numerous 1- to 2-cm diameter nodules throughout both lungs. Biopsy revealed multiple pulmonary chemodectomas of uncertain malignant potential. No extrapulmonary primary site could be found.


RESULTS
Because of deteriorating pulmonary function, she was treated with courses of etoposide-cisplatin and subsequently somatostatin without effect. She finally responded to a course of doxorubicin and streptozocin. She is currently maintained on interferon-alpha 2B but her measured vital capacity continues to fall slowly, reflecting increased tumor growth. Because there is still no evidence of extrapulmonary spread, she is considered to be a candidate for lung transplantation.


CONCLUSIONS
A doxorubicin-streptozocin combination produced a temporary remission of this patient's multiple pulmonary chemodectomas.
Chow
S N
SN
Department of Pediatrics, University of British Columbia, Vancouver, Canada.
Seear
M
M
Anderson
R
R
Magee
F
F
D000293:Adolescent; D000782:Aneuploidy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002869:Chromosome Aberrations; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging / Q000628:therapy; D010236:Paraganglioma, Extra-Adrenal / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics; D012129:Respiratory Function Tests; D013311:Streptozocin / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity
D013311:Streptozocin; D004317:Doxorubicin