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Metastatic retroperitoneal paraganglioma: Case report and review of the literature.

ABSTRACT: Paragangliomas are rare neuroendocrine tumors with 500 to 1600 new cases in the United States each year (1). The clinical presentation may range from asymptomatic to the classic triad of episodic diaphoresis, headache, and palpitations. Surgery is the hallmark of treatment when tumors are amenable to resection. When patients are found to have metastases, systemic therapies may be employed. In this case report, we present a patient found to have a large retroperitoneal paraganglioma with nodal metastases.

SUBMITTER: Tabakin AL

PROVIDER: S-EPMC6554718 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature

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Metastatic retroperitoneal paraganglioma: Case report and review of the literature.

Tabakin Alexandra L AL Weintraub Michael A MA Radadia Kushan D KD Salazar Cristo G CG Sadimin Evita E Singer Eric A EA

Clinics in oncology (Belmont, Calif.) 20190308

Paragangliomas are rare neuroendocrine tumors with 500 to 1600 new cases in the United States each year (1). The clinical presentation may range from asymptomatic to the classic triad of episodic diaphoresis, headache, and palpitations. Surgery is the hallmark of treatment when tumors are amenable to resection. When patients are found to have metastases, systemic therapies may be employed. In this case report, we present a patient found to have a large retroperitoneal paraganglioma with nodal me ...[more]

PMID: 31179416

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Project description:Context: Metastatic pheochromocytoma/paraganglioma (MPP) therapy mainly involves radionuclide therapy, chemotherapy, and targeted therapy. In recent years, temozolomide (TMZ) showed great promise in some MMP patients, especially those with SDHB germline mutation. We reported a patient with MPP who did not have any known germline genetic change and responded remarkably well to TMZ monotherapy. Case presentation: The patient was a 41-year-old woman with local and distant recurrence (soft tissues and bone metastases) of retroperitoneal paraganglioma. She suffered from dizziness, palpitation, sweating, weight loss and constipation, with the blood pressure fluctuating substantially from 130/100 mmHg to 190/120 mmHg, although she was on phenoxybenzamine and metoprolol medication. The patient showed clinical and radiological response after 3-cycle TMZ therapy. Upon 15 cycles of TMZ therapy, her symptoms were dramatically alleviated, urinary norepinephrine excretion decreased from 1,840 μg/24 h to 206 μg/24 h, and CT showed that the lesions further shrank. Molecular profiling of the tumor tissue of the patient revealed hypermethylation of the O6-methylguanine-DNA-methyltransferase (MGMT) promoter and a negative immunostaining for MGMT. Globally, only 26 cases of MPP treated with TMZ have been described so far. TMZ is effective, especially in patients with SDHB mutation, which can be explained by the silencing of MGMT expression as a consequence of MGMT promoter hypermethylation in SDHB-mutated tumors. Although, in general, patients with SDHB mutation or MGMT promoter hypermethylation have better response to TMZ, there are also exceptions. Severe side effects are uncommon, with only 17.4% patients experiencing Grade 3 toxicities, including lymphopenia, and hypertension. Conclusions: TMZ is effective and safe in MPP patients, and, it may work better on patients with SDHB-related MPP. Measurement of MGMT expression might help assess the tumor sensitivity to TMZ but this needs further systematic investigation.

Dataset Information

Metastatic retroperitoneal paraganglioma: Case report and review of the literature.

Publications

Metastatic retroperitoneal paraganglioma: Case report and review of the literature.

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