Local-regional recurrence of sporadic or syndromic abdominal extra-adrenal paraganglioma: Incidence, characteristics, and outcome

• Published in: Surgery 2009-12, Vol.146 (6), p.986-992
• Main Authors: Slycke, Sam Van, MD, Caiazzo, Robert, MD, PhD, Pigny, Pascal, PhD, Cardot-Bauters, Catherine, MD, Arnalsteen, Laurent, MD, D'Herbomez, Michele, MD, Leteurtre, Emmanuelle, MD, Rouaix-Emery, Nathalie, PhD, Ernst, Olivier, MD, PhD, Huglo, Damien, MD, PhD, Vantyghem, Marie-Christine, MD, PhD, Wemeau, Jean-Louis, MD, Carnaille, Bruno, MD, Pattou, François, MD
• Format: Article
• Language: English
• Subjects: Adult; Female; Humans; Lymphatic Metastasis; Male; Membrane Proteins - genetics; Middle Aged; Mutation; Neoplasm Recurrence, Local - etiology; Neoplasm Recurrence, Local - genetics; Neoplasm Recurrence, Local - surgery; Paraganglioma, Extra-Adrenal - etiology; Paraganglioma, Extra-Adrenal - genetics; Paraganglioma, Extra-Adrenal - secondary; Paraganglioma, Extra-Adrenal - surgery; Prognosis; Reoperation; Retrospective Studies; Succinate Dehydrogenase - genetics; Surgery; Syndrome; Von Hippel-Lindau Tumor Suppressor Protein - genetics; Young Adult
• ISSN: 0039-6060; 1532-7361
• DOI: 10.1016/j.surg.2009.10.055

Background Operative excision of abdominal extra-adrenal paragangliomas (EAPs) does not preclude the late development of local-regional recurrence. We describe the incidence, characteristics, and outcome of this rarely reported feature. Methods Retrospective analysis of local-regional recurrence that occurred during follow-up of 51 consecutive patients operated for a sporadic ( n = 26) or hereditary ( n = 25) EAP. Results Seven patients with a sporadic or syndromic EAP ( n = 4: von Hippel-Lindau syndrome and SDHB, SDHC, and SDHD gene mutations) underwent reoperation for a local-regional recurrence after a median time of 46 months (interquartile range [IQR], 16–100). The Kaplan-Meier estimated incidence of local-regional recurrence (± standard error of the mean) reached 15% ± 7% at 5 years and 23% ± 9% after 10 years. Recurrent EAPs were all secreting and 38% provoked clinical symptoms. New lesions were smaller than the primary EAP ( P = .01) and more often associated with lymph node metastases (43% vs 4%, P = .01). Operative excision seemed complete in 5 patients. Clinical remission was maintained in 4 patients after a median follow-up of 57 months (IQR, 22–102). Conclusion Local-regional recurrence of sporadic and syndromic EAPs is frequent and may be delayed beyond 10 years, requiring lifelong follow-up after the initial operation. When technically feasible, operative excision can lead to prolonged remission.