Single-Agent Thalidomide for Treatment of Malignant Paraganglioma of the Organ of Zuckerkandl

The standard of care for benign pheochromocytomas and paragangliomas is surgical resection; however, there is no definitive curative or standard therapy for the treatment of malignant pheochromocytomas or paragangliomas. Current therapeutic options include surgical resection, chemotherapy (CVD thera...

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Bibliographic Details
Published in:Case reports in medicine 2019, Vol.2019 (2019), p.1-4
Main Authors: Shindorf, Mackenzie L., Chaudhuri, Prabir K.
Format: Article
Language:English
Subjects:
Biology
Cancer
Care and treatment
Case Report
Case reports
Chemotherapy
Endocrinology
Health aspects
Liver
Lungs
Medical prognosis
Metabolism
Metastases
Metastasis
Neuroendocrine tumors
Palliation
Paraganglioma
Patients
Pheochromocytoma
Radiation therapy
Radiotherapy
Risk factors
Somatostatin
Statistical analysis
Temozolomide
Thalidomide
Vincristine
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Description
Summary:The standard of care for benign pheochromocytomas and paragangliomas is surgical resection; however, there is no definitive curative or standard therapy for the treatment of malignant pheochromocytomas or paragangliomas. Current therapeutic options include surgical resection, chemotherapy (CVD therapy), MIBG-radiotherapy, somatostatin analogues, and combination targeted therapies such as temozolomide and thalidomide. Although some patients demonstrate short-lived responses to these various therapies, there has been no statistically significant survival benefit with any of the current regimens and therefore the aim is palliation and symptom control. Here, we present a 38-year-old man found to have an unresectable metastatic paraganglioma located in the Organ of Zuckerkandl who has been treated with single-agent thalidomide for over 15 years with minimal growth and stabilization of metastatic lesions. The management of our patient with single-agent therapy for this extended period of time challenges the efficacy and role of chemotherapy and other accepted therapies for malignant pheochromocytoma and paraganglioma.
ISSN:1687-9627
1687-9635
DOI:10.1155/2019/7185973