Abstract
This case report presents the clinical evolution and management of a patient with a hereditary paraganglioma syndrome. This disease is characterized by rare tumors of neural crest origin that are symmetrically distributed along the paravertebral axis from the base of the skull and neck to the pelvis. In addition, these patients may develop renal cancer, gastrointestinal stromal tumors, pituitary adenomas, and bone metastasis in some cases. To date no successful therapeutic treatment has been reported. Total resection with postoperative radiotherapy and chemotherapy have been advocated, especially for the multiple metastasis. Here we show how the combination of high doses of the beta blocker propranolol (3 mg/Kg/day) and the DNA intercalating agent, temozolomide, has been successful in the treatment of a SDHA metastatic paraganglioma.
Highlights
Hereditary paraganglioma and pheochromocytoma syndromes (HPP) are characterized by rare tumors called pheochromocytomas (PHEOs) and paragangliomas (PGLs). These are highly vascularized catecholamine-secreting tumors of neural crest origin, distributed symmetrically along the paravertebral axis
There are some literature reports on the synergy between chemotherapeutic agents inducing DNA damage, and blocking DNA replication and β-blockers, which have shown antiangiogenic and pro-apoptotic effects in tumoral cells[9,10]. Recent examples of these combinations found in the literature include the action of β-blockers increasing response to different chemotherapeutic agents
Propranolol in combination with vinblastine, proved to be a successful treatment for advanced angiosarcoma[12], with optimal results in seven patients following a clinical trial[13]
Summary
Hereditary paraganglioma and pheochromocytoma syndromes (HPP) are characterized by rare tumors called pheochromocytomas (PHEOs) and paragangliomas (PGLs). In October 2017, 4 months since the last dose of temozolomide, and propranolol as the only treatment (240mg/day), the disease remains controlled according to the last PET-TAC (F18-FDG) (Figure 1G). This imaging study suggests the presence of two neoplastic bone lesions in the dorsal spine and right acetabulum; regarding the previous examination (July 2017), the lesions described in D6 and acetabulum persist without major changes, whereas that of vertebra D10 has disappeared
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