{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T16:43:28Z","timestamp":1787071408382,"version":"build-2736575974"},"reference-count":18,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T00:00:00Z","timestamp":1751328000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Succinate dehydrogenase (SDH) deficient gastrointestinal stromal tumor (GIST) is the most common type of wild type GIST characterized by lack of mutations in proto-oncogene receptor tyrosine kinase (KIT) or platelet-derived growth factor receptor alpha (PDFGR alpha) pathways. It has a unique predilection for females and young adults, with a relatively indolent prognosis and varied treatment modalities. Data regarding SDH GIST from the Indian subcontinent is sparse.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Materials and Methods:<\/jats:title>\n                    <jats:p>This is a retrospective study of patients with SDH deficient GIST, who presented to the GIST clinic in a tertiary care center in India from 2016\u20132022. 9 out of 120 GIST patients were SDH deficient. All patients were discussed in GIST tumor board and pathologies were re-reviewed by a dedicated GIST pathologist. Data was collected retrospectively from prospectively maintained database.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      There were nine patients with median age of 48 years (range 15\u201363), of which there were 5 males (\n                      <jats:italic toggle=\"yes\">n<\/jats:italic>\n                      = 5, 55.5%). Eight patients (\n                      <jats:italic toggle=\"yes\">n<\/jats:italic>\n                      = 8, 88.8%) had primary in stomach, one had duodenal GIST. In stomach, body was the most common location (\n                      <jats:italic toggle=\"yes\">n<\/jats:italic>\n                      = 6,75%). 3 out of 9 patients (\n                      <jats:italic toggle=\"yes\">n<\/jats:italic>\n                      = 3, 33.3%) had multifocal disease. 8 out of 9 (88.8%) had metastasis at presentation. Most common site of metastasis was liver (7 out of 8, 87.5%) Lymph node involvement was present in 4 out of 9 patients (44.4%). One patient had classical Carneys triad at presentation. 8 out of 9 (88.8%) had epithelioid morphology and 1 (11.1%) had mixed epithelioid-spindle picture. 5 out of 9 (55.5%) patients had germline SDH mutations. Out of nine patients, one was put on observation post surgery and therapy was given for eight patients (\n                      <jats:italic toggle=\"yes\">n<\/jats:italic>\n                      = 8,88.8%). Response to imatinib was very poor, 3 out of 5 patients (60%) had response with sunitinib, three were started on regorafenib out of which one had partial response and one patient had partial response with temozolomide. Median overall survival was NR- not reached (95% CI 12.96-NR).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>SDH deficient GIST is a unique subtype of gastrointestinal stromal tumor with distinct clinic-pathological features, diagnostic modalities, therapeutic strategies, and genetic implications as compared to C-kit\/PDGFR-alpha mutated GIST.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/ijc.ijc_500_24","type":"journal-article","created":{"date-parts":[[2025,11,22]],"date-time":"2025-11-22T07:00:24Z","timestamp":1763794824000},"page":"408-415","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Succinate dehydrogenase deficient GIST: Case series and review of literature from a tertiary care center in India"],"prefix":"10.4103","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6780-0406","authenticated-orcid":false,"given":"Akhil P","family":"Santhosh","sequence":"first","affiliation":[{"name":"Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4792-3083","authenticated-orcid":false,"given":"Sameer","family":"Rastogi","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8787-4285","authenticated-orcid":false,"given":"Shamim A","family":"Shamim","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7597-4895","authenticated-orcid":false,"given":"Rajni","family":"Yadav","sequence":"additional","affiliation":[{"name":"Department of Pathology, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8755-2013","authenticated-orcid":false,"given":"Dikhra","family":"Khan","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,11,21]]},"reference":[{"key":"R1-20251122","doi-asserted-by":"crossref","first-page":"1712","DOI":"10.1097\/PAS.0b013e3182260752","article-title":"Succinate dehydrogenase-deficient GISTs:A clinicopathologic, immunohistochemical, and molecular genetic study of 66 gastric GISTs with predilection to young age","volume":"35","author":"Miettinen","year":"2011","journal-title":"Am J Surg Pathol"},{"key":"R2-20251122","doi-asserted-by":"crossref","first-page":"2303","DOI":"10.3748\/wjg.v21.i8.2303","article-title":"Succinate dehydrogenase-deficient gastrointestinal stromal tumors","volume":"21","author":"Wang","year":"2015","journal-title":"World J Gastroenterol"},{"key":"R3-20251122","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1259\/bjr.20140476","article-title":"MDCT features of succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumours","volume":"87","author":"Tirumani","year":"2014","journal-title":"Br J Radiol"},{"key":"R4-20251122","doi-asserted-by":"crossref","first-page":"655","DOI":"10.5858\/arpa.2018-0370-RS","article-title":"Succinate dehydrogenase-deficient gastrointestinal stromal tumors","volume":"144","author":"Ibrahim","year":"2020","journal-title":"Arch Pathol Lab 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Rep"},{"key":"R8-20251122","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3389\/fendo.2021.680609","article-title":"Carney triad, Carney-Stratakis syndrome, 3PAS and other tumors due to SDH deficiency","volume":"12","author":"Pitsava","year":"2021","journal-title":"Front Endocrinol (Lausanne)"},{"key":"R9-20251122","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1007\/s10555-019-09818-0","article-title":"Current management of succinate dehydrogenase\u2013deficient gastrointestinal stromal tumors","volume":"38","author":"Neppala","year":"2019","journal-title":"Cancer Metastasis Rev"},{"key":"R10-20251122","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.1210\/jc.2017-00165","article-title":"A Duodenal SDH-deficient gastrointestinal stromal tumor in a patient with a germline SDH-B mutation","volume":"102","author":"Elston","year":"2017","journal-title":"J Clin Endocrinol Metab"},{"key":"R11-20251122","first-page":"1","article-title":"Clinicopathologic study of succinate-dehydrogenase-deficient gastrointestinal stromal tumors","volume":"96","author":"Liu","year":"2017","journal-title":"Med (United States)"},{"key":"R12-20251122","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1038\/ejhg.2015.142","article-title":"Carney triad can be (rarely) associated with germline succinate dehydrogenase defects","volume":"24","author":"Boikos","year":"2016","journal-title":"Eur J Hum Genet"},{"key":"R13-20251122","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1001\/jamaoncol.2016.0256","article-title":"Molecular subtypes of KIT\/PDGFRA wild-type gastrointestinal stromal tumors a report from the national institutes of health gastrointestinal stromal tumor clinic","volume":"2","author":"Boikos","year":"2016","journal-title":"JAMA Oncol"},{"key":"R14-20251122","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1002\/pbc.24539","article-title":"A novel WT1 mutation in familial wilms tumor","volume":"60","author":"Melchionda","year":"2013","journal-title":"Pediatr Blood Cancer"},{"key":"R15-20251122","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1177\/17588359211023278","article-title":"Targeted therapy in SDH-deficient GIST","volume":"13","author":"Nannini","year":"2021","journal-title":"Ther Adv Med Oncol"},{"key":"R16-20251122","doi-asserted-by":"crossref","first-page":"2516865719842534","DOI":"10.1177\/2516865719842534","article-title":"Succinate dehydrogenase-deficient gastrointestinal stromal tumors:Small steps toward personalized medicine?","volume":"12","author":"Ravegnini","year":"2019","journal-title":"Epigenetics Insights"},{"key":"R17-20251122","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1038\/modpathol.2012.77","article-title":"Succinate dehydrogenase-deficient GISTs are characterized by IGF1R overexpression","volume":"25","author":"Chou","year":"2012","journal-title":"Mod Pathol"},{"key":"R18-20251122","first-page":"e1710","article-title":"Somatostatin receptor avidity in gastrointestinal stromal tumors:Theranostic implications of gallium-68 scan and eligibility for peptide receptor radionuclide therapy","volume":"9","author":"Loaiza-Bonilla","year":"2017","journal-title":"Cureus"}],"container-title":["Indian Journal of Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/ijc.ijc_500_24","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T15:45:42Z","timestamp":1787067942000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/ijc.ijc_500_24"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7]]},"references-count":18,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025]]}},"URL":"https:\/\/doi.org\/10.4103\/ijc.ijc_500_24","relation":{},"ISSN":["0019-509X","1998-4774"],"issn-type":[{"value":"0019-509X","type":"print"},{"value":"1998-4774","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7]]},"assertion":[{"value":"2024-07-06","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication 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