Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma

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2 Citations (Scopus)

Abstract

Pheochromocytomas (PCCs) and paragangliomas (PGLs) are neuroendocrine tumors with a strong genetic background. The mainstay of treatment for PCC/PGLs is surgery. However, for unresectable lesions, no curative treatment is currently available. Temozolomide (TMZ) has been shown to determine radiological and biochemical response in malignant PCC/PGLs. We report two cases of PCC/PGLs treated with TMZ. Case 1 is a 51-year-old man with local and distant recurrence (liver and bone metastases) of right adrenal PCC. Case 2 is a 54-year-old woman with a PCC/PGL syndrome caused by a mutation in MAX gene (c.171 + 1G > A), operated on for bilateral adrenal PCC and presenting with a large unresectable abdominal PGL. Both patients presented hypertension due to catecholamine hypersecretion. TMZ determined radiological response according to RECIST criteria, reduction of urinary catecholamine levels, and controlled hypertension in both patients. Furthermore, the current study demonstrates, for the first time, that MAX-related PGLs are responsive to TMZ.

Original languageEnglish
Pages (from-to)102-105
Number of pages4
JournalAnti-Cancer Drugs
Volume29
Issue number1
DOIs
Publication statusPublished - Jan 1 2018

Fingerprint

temozolomide
Paraganglioma
Pheochromocytoma
Therapeutics
Catecholamines
Hypertension
Neuroendocrine Tumors

Keywords

  • Abdominal paraganglioma
  • Malignant pheochromocytoma
  • MAX gene
  • Metanephrine
  • Normetanephrine
  • Radiological response
  • Temozolomide

ASJC Scopus subject areas

  • Oncology
  • Pharmacology
  • Pharmacology (medical)
  • Cancer Research

Cite this

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title = "Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma",
abstract = "Pheochromocytomas (PCCs) and paragangliomas (PGLs) are neuroendocrine tumors with a strong genetic background. The mainstay of treatment for PCC/PGLs is surgery. However, for unresectable lesions, no curative treatment is currently available. Temozolomide (TMZ) has been shown to determine radiological and biochemical response in malignant PCC/PGLs. We report two cases of PCC/PGLs treated with TMZ. Case 1 is a 51-year-old man with local and distant recurrence (liver and bone metastases) of right adrenal PCC. Case 2 is a 54-year-old woman with a PCC/PGL syndrome caused by a mutation in MAX gene (c.171 + 1G > A), operated on for bilateral adrenal PCC and presenting with a large unresectable abdominal PGL. Both patients presented hypertension due to catecholamine hypersecretion. TMZ determined radiological response according to RECIST criteria, reduction of urinary catecholamine levels, and controlled hypertension in both patients. Furthermore, the current study demonstrates, for the first time, that MAX-related PGLs are responsive to TMZ.",
keywords = "Abdominal paraganglioma, Malignant pheochromocytoma, MAX gene, Metanephrine, Normetanephrine, Radiological response, Temozolomide",
author = "Ferrara, {Alfonso M.} and Giuseppe Lombardi and Ardi Pambuku and Domenico Meringolo and Roberta Bertorelle and Margherita Nardin and Francesca Schiavi and Maurizio Iacobone and Giuseppe Opocher and Vittorina Zagonel and Stefania Zovato",
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T1 - Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma

AU - Ferrara, Alfonso M.

AU - Lombardi, Giuseppe

AU - Pambuku, Ardi

AU - Meringolo, Domenico

AU - Bertorelle, Roberta

AU - Nardin, Margherita

AU - Schiavi, Francesca

AU - Iacobone, Maurizio

AU - Opocher, Giuseppe

AU - Zagonel, Vittorina

AU - Zovato, Stefania

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Pheochromocytomas (PCCs) and paragangliomas (PGLs) are neuroendocrine tumors with a strong genetic background. The mainstay of treatment for PCC/PGLs is surgery. However, for unresectable lesions, no curative treatment is currently available. Temozolomide (TMZ) has been shown to determine radiological and biochemical response in malignant PCC/PGLs. We report two cases of PCC/PGLs treated with TMZ. Case 1 is a 51-year-old man with local and distant recurrence (liver and bone metastases) of right adrenal PCC. Case 2 is a 54-year-old woman with a PCC/PGL syndrome caused by a mutation in MAX gene (c.171 + 1G > A), operated on for bilateral adrenal PCC and presenting with a large unresectable abdominal PGL. Both patients presented hypertension due to catecholamine hypersecretion. TMZ determined radiological response according to RECIST criteria, reduction of urinary catecholamine levels, and controlled hypertension in both patients. Furthermore, the current study demonstrates, for the first time, that MAX-related PGLs are responsive to TMZ.

AB - Pheochromocytomas (PCCs) and paragangliomas (PGLs) are neuroendocrine tumors with a strong genetic background. The mainstay of treatment for PCC/PGLs is surgery. However, for unresectable lesions, no curative treatment is currently available. Temozolomide (TMZ) has been shown to determine radiological and biochemical response in malignant PCC/PGLs. We report two cases of PCC/PGLs treated with TMZ. Case 1 is a 51-year-old man with local and distant recurrence (liver and bone metastases) of right adrenal PCC. Case 2 is a 54-year-old woman with a PCC/PGL syndrome caused by a mutation in MAX gene (c.171 + 1G > A), operated on for bilateral adrenal PCC and presenting with a large unresectable abdominal PGL. Both patients presented hypertension due to catecholamine hypersecretion. TMZ determined radiological response according to RECIST criteria, reduction of urinary catecholamine levels, and controlled hypertension in both patients. Furthermore, the current study demonstrates, for the first time, that MAX-related PGLs are responsive to TMZ.

KW - Abdominal paraganglioma

KW - Malignant pheochromocytoma

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KW - Normetanephrine

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