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dc.contributor.authorJha, Abhishek
dc.contributor.authorde Luna, Kristine
dc.contributor.authorBalili, Charlene Ann
dc.contributor.authorMillo, Corina
dc.contributor.authorParaiso, Cecilia Angela
dc.contributor.authorLing, Alexander
dc.contributor.authorGonzales, Melissa K
dc.contributor.authorViana, Bruna
dc.contributor.authorAlrezk, Rami
dc.contributor.authorAdams, Karen T
dc.contributor.authorTena, Isabel
dc.contributor.authorChen, Alice
dc.contributor.authorNeuzil, Jiri
dc.contributor.authorRaygada, Margarita
dc.contributor.authorKebebew, Electron
dc.contributor.authorTaieb, David
dc.contributor.authorO'Dorisio, M Sue
dc.contributor.authorO'Dorisio, Thomas
dc.contributor.authorCivelek, Ali Cahid
dc.contributor.authorStratakis, Constantine A
dc.contributor.authorMercado-Asis, Leilani
dc.contributor.authorPacak, Karel
dc.date.accessioned2020-02-28T00:59:23Z
dc.date.available2020-02-28T00:59:23Z
dc.date.issued2019
dc.identifier.issn2234-943X
dc.identifier.doi10.3389/fonc.2019.00053
dc.identifier.urihttp://hdl.handle.net/10072/391999
dc.description.abstractBackground: Pheochromocytoma and paraganglioma (PHEO/PGL) are rare neuroendocrine tumors which may cause potentially life-threatening complications, with about a third of cases found to harbor specific gene mutations. Thus, early diagnosis, treatment, and meticulous monitoring are of utmost importance. Because of low incidence of succinate dehydrogenase complex subunit A (SDHA)-related metastatic PHEO/PGL, currently there exists insufficient clinical information, especially with regards to its diagnostic and treatment characteristics. Methods: Ten patients with SDHA-related metastatic PHEO/PGL were followed-up prospectively and/or retrospectively between January 2010–July 2018. They underwent biochemical tests (n = 10), 123I-MIBG (n = 9) scintigraphy, and multiple whole-body positron emission tomography/computed tomography (PET/CT) scans with 68Ga-DOTATATE (n = 10), 18F-FDG (n = 10), and 18F-FDOPA (n = 6). Results: Our findings suggest that these tumors can occur early and at extra-adrenal locations, behave aggressively, and have a tendency to develop metastatic disease within a short period of time. None of our patients had a family history of PHEO/PGL, making them appear sporadic. Nine out of 10 patients showed abnormal PHEO/PGL-specific biochemical markers with predominantly noradrenergic and/or dopaminergic phenotype, suggesting their utility in diagnosing and monitoring the disease. Per patient detection rates of 68Ga-DOTATATE (n = 10/10), 18F-FDG (n = 10/10), 18F-FDOPA (n = 5/6) PET/CT, and 123I-MIBG (n = 7/9) scintigraphy were 100, 100, 83.33, and 77.77%, respectively. Five out of 7 123I-MIBG positive patients had minimal 123I-MIBG avidity or detected very few lesions compared to widespread metastatic disease on 18F-FDG PET/CT, implying that diagnosis and treatment with 123/131I-MIBG is not a good option. 68Ga-DOTATATE PET/CT was found to be superior or equal to 18F-FDG PET/CT in 7 out of 10 patients and hence, is recommended for evaluation and follow-up of these patients. All 7 out of 7 patients who received conventional therapies (chemotherapy, somatostatin analog therapy, radiation therapy, 131I-MIBG, peptide receptor radionuclide therapy) in addition to surgery showed disease progression. Conclusion: In our cohort of patients, SDHA-related metastatic PHEO/PGL followed a disease-course similar to that of SDHB-related metastatic PHEO/PGL, showing highly aggressive behavior, similar imaging and biochemical phenotypes, and suboptimal response to conventional therapies. Therefore, we recommend careful surveillance of the affected patients and a search for effective therapies.
dc.description.peerreviewedYes
dc.languageEnglish
dc.language.isoeng
dc.publisherFrontiers Media
dc.relation.ispartofjournalFrontiers in Oncology
dc.relation.ispartofvolume9
dc.subject.fieldofresearchOncology and carcinogenesis
dc.subject.fieldofresearchcode3211
dc.subject.keywordsScience & Technology
dc.subject.keywordsLife Sciences & Biomedicine
dc.subject.keywordsOncology
dc.subject.keywordsparaganglioma
dc.subject.keywordspheochromocytoma
dc.titleClinical, Diagnostic, and Treatment Characteristics of SDHA-Related Metastatic Pheochromocytoma and Paraganglioma
dc.typeJournal article
dc.type.descriptionC1 - Articles
dcterms.bibliographicCitationJha, A; de Luna, K; Balili, CA; Millo, C; Paraiso, CA; Ling, A; Gonzales, MK; Viana, B; Alrezk, R; Adams, KT; Tena, I; Chen, A; Neuzil, J; Raygada, M; Kebebew, E; Taieb, D; O'Dorisio, MS; O'Dorisio, T; Civelek, AC; Stratakis, CA; Mercado-Asis, L; Pacak, K, Clinical, Diagnostic, and Treatment Characteristics of SDHA-Related Metastatic Pheochromocytoma and Paraganglioma, Frontiers in Oncology, 2019, 9
dcterms.dateAccepted2019-01-18
dcterms.licensehttp://creativecommons.org/licenses/by/4.0/
dc.date.updated2020-02-28T00:56:34Z
dc.description.versionVersion of Record (VoR)
gro.rights.copyright© 2019 Jha, de Luna, Balili, Millo, Paraiso, Ling, Gonzales, Viana, Alrezk, Adams, Tena, Chen, Neuzil, Raygada, Kebebew, Taieb, O'Dorisio, O'Dorisio, Civelek, Stratakis, Mercado-Asis and Pacak. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
gro.hasfulltextFull Text
gro.griffith.authorNeuzil, Jiri


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