Successful induction therapy with sequential CVD followed by high-dose lanreotide in for metastatic SDHB paraganglioma: Case report

Isabel Tena, José Luis Ponce, Marcos Tajahuerce, José Luis Vercher-Conejero, Manuel Cifrián, Katherine I. Wolf, Marcela Miret, Maribel del Olmo, Rosa Reboll, Antonio Conde, Francisca Moreno, Julia Balaguer, Adela Cañete, Rosana Palasí, Alfredo Marco, Juan Francisco Merino Torres, Carlos Ferrer, Alfredo Sánchez, José Luis Soto, Antonio LlombartKarel Pacak

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Objective Pheochromocytomas (PHEOs) and paragangliomas (PGLs) arise from adrenal extra-adrenal paraganglia. They often secrete catecholamine and approximately 1/3 are hereditary. Almost 50% of metastatic PGLs are caused by mutations in the succinate dehydrogenase gene, particularly in subunits B and D (SDHB/D). These tumors remain a diagnostic and therapeutic challenge due to a limited number of effective treatment options. To our best knowledge, we present the first report that uses induction therapy to elicit a significant response in both primary and metastatic lesions, followed by complete surgical resection of a primary SDHB-related PGL. Case description A 45-year-old female presented with an 8.5 cm unresectable primary SDHB-related PGL with multiple bone metastases. She had an initial blood pressure of 210/130 mmHg and a heart rate of 150 bpm. Initially, she was treated with cisplatin, vinblastine, and dacarbazine (CVD) chemotherapy and lanreotide (120 mg/sc/28d), which resulted in tumor shrinkage. The patient later progressed, and monotherapy with a shortened interval of lanreotide (120 mg/sc/14d) was initiated. Following 2.5 months at this interval, the patient nearly achieved complete control of her clinical symptoms, and experienced a 30% reduction in 18F-flurodeoxyglucose uptake in her primary and metastatic lesions. Following the surgical resection of her primary PGL, all prior antihypertensive medications were stopped. Conclusions CVD, together with a 14-day regimen of high dose lanreotide, may be an effective treatment option for SDHB-related metastatic PGLs. Therefore, further evaluation of somatostatin analogues, preferably lanreotide, in the treatment of metastatic SDHB-related PGLs is warranted. We believe that ours is the first report detailing the successful use of lanreotide treatment prior to a surgical rescue after systemic treatment.

Original languageEnglish
Pages (from-to)8-13
Number of pages6
JournalJournal of Clinical and Translational Endocrinology: Case Reports
Volume7
DOIs
StatePublished - Mar 2018

Bibliographical note

Funding Information:
This work was supported in part by the Spanish Society of Medical Oncology (SEOM Grant for 2 years in a foreign reference center, 2015), the Eunice Kennedy Shriver National Institute of Child Health and Human Development of the National Institutes of Health , and the PHEiPAS Alliance .

Publisher Copyright:
© 2017

Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.

Keywords

  • Lanreotide
  • Metastatic
  • Paraganglioma
  • SDHB

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