Multimodal combination of interferon and loco-regional treatment for disease control in progressive metastatic pheochromocytoma/paraganglioma patients

Julien Hadoux; Desiree Deandreis; Caroline Caramella; Sophie Leboulleux; Ghuzlan Abir Al; Cecile Chougnet; Frederic Dumont; Frederic Deschamps; Martin Schlumberger; Eric Baudin

doi:10.1530/endoabs.32.P544

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Endocrine Abstracts (2013) 32 P544 | DOI: 10.1530/endoabs.32.P544

ECE2013 Poster Presentations Endocrine tumours and neoplasia (66 abstracts)

Multimodal combination of interferon and loco-regional treatment for disease control in progressive metastatic pheochromocytoma/paraganglioma patients

Julien Hadoux , Désirée Déandréis , Caroline Caramella , Sophie Leboulleux , Abir Al Ghuzlan , Cécile Chougnet , Frédéric Dumont , Frédéric Deschamps , Martin Schlumberger & Eric Baudin

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Institut Gustave Roussy, Villejuif, France.

Interferon-α (IFN-α) has shown some activity in neuroendocrine tumors with disease stabilizations. Malignant pheochromocytoma and paraganglioma (MPPGLs) have a heterogeneous behavior with a slow progression rate, most of the time and a high frequency of bone metastases. Stabilizing disease and preventing skeletal-related events are two goals to achieve in the management of MPPGLs patients.

This retrospective study evaluated a multimodal strategy of IFN-α combined to loco-regional treatments (LRT) (radiation and/or interventional radiology and/or surgery) for disease control in patients with progressive MPPGLs. Progression free survival (PFS) was primary endpoint; response rate, safety and symptomatic efficacy were secondary endpoints.

Eleven consecutive patients received peg-IFN-α 2A (90–180 μg/week) or IFN-α 2B (1.5–3 MU×3/week) at our institution between December 2005 and May 2010 as first (n=3), second (n=5) or subsequent line (n=3) of treatment. Six patients were men (55%); median age was 41. At the beginning of treatment, ten patients had progressive disease demonstrated by PET scan (n=8), MIBG (n=3) or CT-scan (n=2); data were missing for one patient. Nine patients had bone-predominant disease (bone-only, n=4). During IFN-α therapy, a mean number of 3 (range 1–8) bone directed LRT were performed. Most frequent all grade IFN-α-related toxicities were asthenia (n=9), anemia (n=5), lymphopenia (n=5), diarrhea (n=3). One patient had cardiac arrest while on therapy and survived. Symptomatic relief of pain, headaches, diarrhea or sweating occurred in 43% of seven symptomatic patients. Response was evaluable in ten patients: one partial response, eight stable diseases and one progressive disease (PERCIST or RECIST1.1) were seen. With a median follow up of 54 months, median overall survival was not reached and median PFS was 14.4 months.

This study demonstrates the symptomatic and stabilizing effect of a multimodal treatment combination of IFN-α and LRT in progressive metastatic PPGLs.