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Interferon-alpha Treatment for Disease Control in Metastatic Pheochromocytoma/Paraganglioma Patients
Julien Hadoux1, Marie Terroir2, Sophie Leboulleux2
1Département d'imagerie, service de médecine nucléaire et cancérologie endocrinienne, Gustave Roussy, Université Paris-Saclay, 114 rue Edouard Vaillant, F-94805, Villejuif, France. Julien.hadoux@gustaveroussy.fr.
Abstract:
Interferon-alpha (IFN-alpha) is recommended in neuroendocrine tumors (NET). Malignant pheochromocytoma and paragangliomas (MPPGLs) constitute a rare subgroup of NET with few treatment options. IFN-alpha efficacy in patients with MPPGLs was evaluated in a single-center retrospective study. Progression-free survival (PFS) was the primary endpoint according to RECIST 1.1 and/or PERCIST 1.0, and response rate, safety, and symptomatic efficacy were secondary endpoints. Fourteen patients received peginterferon alfa-2a (90 to 180 μg/week) or interferon alfa-2b (1.5 to 3 million units × 3/week) at our institution between December 2005 and February 2014 as the first (n = 7), second (n = 3), or subsequent line (n = 4) of treatment. Most of the patients had a slowly progressive disease before IFN-alpha initiation. Eight patients were men (57%); the median age was 44. At the beginning of treatment, 12 patients had progressive disease demonstrated by FDG-PET (n = 9), MIBG (n = 1), or CT scan (n = 2). Most of the patients treated (64%) had metastatic disease limited to or predominantly located in the bones. During IFN-alpha therapy, bone-directed loco-regional treatments were performed in 9 patients (range 1-4). Median PFS was 17.2 months (95% CI [12.1-58.3]). We observed 3 partial metabolic responses, 9 stable diseases, and 2 progressive diseases. No partial response according to RECIST 1.1 was observed. Symptomatic relief of pain, headaches, diarrhea, or sweating occurred in 6 out of 10 symptomatic pts. Most frequent all grade IFN-α-related toxicities were asthenia (n = 10), lymphopenia (n = 7), thrombopenia (n = 6), and anemia (n = 5). Median overall survival was 7.5 years (95% CI [4-NR]). This study suggests symptomatic response and tumor control effect with interferon-alpha in progressive MPPGLs.
Insights
Interferon-alpha (IFN-alpha) showed a tumor control effect and symptomatic relief in patients with malignant pheochromocytoma and paragangliomas (MPPGLs). This rare neuroendocrine tumor subgroup may benefit from IFN-alpha therapy, offering a new treatment avenue.
Area of Science:
- Oncology
- Endocrinology
- Pharmacology
Background:
- Neuroendocrine tumors (NET) treatment often includes Interferon-alpha (IFN-alpha).
- Malignant pheochromocytoma and paragangliomas (MPPGLs) are rare NETs with limited therapeutic options.
- The efficacy of IFN-alpha in MPPGLs has not been extensively studied.
Purpose of the Study:
- To evaluate the efficacy and safety of Interferon-alpha (IFN-alpha) in patients with malignant pheochromocytoma and paragangliomas (MPPGLs).
- To assess progression-free survival (PFS), response rates, and symptomatic benefits of IFN-alpha therapy in this rare NET subgroup.
Main Methods:
- A single-center retrospective study involving 14 patients with MPPGLs treated with peginterferon alfa-2a or interferon alfa-2b.
- Patients received IFN-alpha as first, second, or subsequent line of treatment between December 2005 and February 2014.
- Primary endpoint: Progression-free survival (PFS) by RECIST 1.1/PERCIST 1.0. Secondary endpoints: response rate, safety, and symptomatic efficacy.
Main Results:
- Median PFS was 17.2 months. Three partial metabolic responses and nine stable diseases were observed.
- No partial response according to RECIST 1.1 criteria was noted.
- Symptomatic relief (pain, headaches, diarrhea, sweating) occurred in 60% of symptomatic patients. Common toxicities included asthenia, lymphopenia, and thrombopenia.
Conclusions:
- Interferon-alpha (IFN-alpha) demonstrates a tumor control effect and provides symptomatic relief in progressive malignant pheochromocytoma and paragangliomas (MPPGLs).
- IFN-alpha may be a valuable therapeutic option for patients with this rare neuroendocrine tumor subgroup, particularly those with bone metastases.
- Further prospective studies are warranted to confirm the role of IFN-alpha in MPPGL management.
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