{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T20:43:12Z","timestamp":1780605792672,"version":"3.54.1"},"reference-count":20,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T00:00:00Z","timestamp":1751328000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Current Oncology"],"abstract":"<jats:p>Primary hepatic gastrointestinal stromal tumours (PHGISTs) are rare and frequently misdiagnosed due to their atypical presentation and uncertain origin. The purpose of this article is to present the case of a 79-year-old female patient with a gigantic PHGIST characterized by a predominantly cystic nature\u2014an extremely rare presentation, as most cases of PHGIST are solid. Despite extensive imaging and exploratory laparotomy, the primary origin remained uncertain, leading to questioning about whether it was a true primary hepatic GIST or an atypical metastatic lesion. The initial therapeutic approach involved a surgical procedure aimed to confirm the diagnosis and achieve reductive tumourectomy. Following the surgery, the patient was administered imatinib with a favourable clinical response for four and a half years\u2014an atypical pattern of resistance, as most patients typically develop therapeutic resistance within two to three years. A second surgical intervention was performed to address a cystic lesion localized in the left hepatic lobe, followed by an atypical segment III hepatectomy to achieve macroscopic resection. Subsequently, the patient received sunitinib for two and a half years, which resulted in temporary disease stabilization. However, the sunitinib treatment was associated with hypertension and leukopenia. The patient\u2019s overall survival was 8 years, suggesting that individualized therapeutic strategies and close monitoring might be the key in such cases. Furthermore, this case confirms the role of surgical intervention even in advanced disease stages, with multiple major resections contributing significantly to prolonged survival. The interplay between surgical and oncologic therapies remains essential to guiding clinical decisions. Given the unusual cystic presentation, this case highlights the necessity to expand the pathological and molecular profiling of PHGISTs. Furthermore, the atypical timeline of resistance development and treatment-related toxicity emphasizes the importance of further research into the genetic and pharmacological determinants of PHGISTs. These findings advocate for the refinement of diagnostic, therapeutic, and surveillance protocols tailored to rare GIST subtypes.<\/jats:p>","DOI":"10.3390\/curroncol32070383","type":"journal-article","created":{"date-parts":[[2025,7,2]],"date-time":"2025-07-02T04:11:04Z","timestamp":1751429464000},"page":"383","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Atypical Cystic Primary Hepatic GIST: A Case Report of Rare Presentation and Long-Term Survival"],"prefix":"10.3390","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1307-6624","authenticated-orcid":false,"given":"Mirela Claudia","family":"Rimbu","sequence":"first","affiliation":[{"name":"Medical Doctoral School, Titu Maiorescu University of Bucharest, 040317 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florin Dan","family":"Ungureanu","sequence":"additional","affiliation":[{"name":"Medical Doctoral School, Titu Maiorescu University of Bucharest, 040317 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1362-6427","authenticated-orcid":false,"given":"Cosmin","family":"Moldovan","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Witting Clinical Hospital, 010243 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madalina Elena","family":"Toba","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Witting Clinical Hospital, 010243 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4590-5957","authenticated-orcid":false,"given":"Marinela","family":"Chirila","sequence":"additional","affiliation":[{"name":"Faculty of Pharmacy, Titu Maiorescu University, Gheorghe Sincai Blv. 16, 040314 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Truta","sequence":"additional","affiliation":[{"name":"Departament of Pharmacy, Emergency Clinical Hospital Bucharest, 8. Floreasca Street, 014461 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Cord","sequence":"additional","affiliation":[{"name":"Medical Doctoral School, Titu Maiorescu University of Bucharest, 040317 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,1]]},"reference":[{"key":"ref_1","first-page":"942","article-title":"Primary Hepatic Extra-gastrointestinal Stromal Tumors: Molecular Pathogenesis, Immunohistopathology, and Treatment","volume":"11","author":"Becker","year":"2023","journal-title":"J. Clin. Transl. Hepatol."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Holthuis, E.I., Slijkhuis, V., van der Graaf, W.T.A., Drabbe, C., van Houdt, W.J., Schrage, Y.M., Olde Hartman, T.C., Uijen, A., Steeghs, N., and Bos, I. (2025). The Prediagnostic General Practitioners\u2019 Pathway of Gastrointestinal Stromal Tumor Patients: A Real-World Data Study. Cancers, 17.","DOI":"10.3390\/cancers17091391"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"224","DOI":"10.13105\/wjma.v7.i5.224","article-title":"Hepatic gastrointestinal stromal tumor: Systematic review of an exceptional location","volume":"7","author":"Ramia","year":"2019","journal-title":"World J. Meta Anal."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2268","DOI":"10.1002\/cam4.808","article-title":"Clinicopathological feature and prognosis of primary hepatic gastrointestinal stromal tumor","volume":"5","author":"Liu","year":"2016","journal-title":"Cancer Med."},{"key":"ref_5","first-page":"110449","article-title":"Advanced imaging techniques for the evaluation of gastrointestinal stromal tumors: A review of current practices and future directions","volume":"154","author":"Matsumoto","year":"2023","journal-title":"Eur. J. Radiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1007\/s10120-015-0526-8","article-title":"The standard diagnosis, treatment, and follow-up of gastrointestinal stromal tumors based on guidelines","volume":"19","author":"Nishida","year":"2015","journal-title":"Gastric Cancer"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"6195","DOI":"10.3748\/wjg.v26.i40.6195","article-title":"Prevalence, diagnosis, and treatment of primary hepatic gastrointestinal stromal tumors","volume":"26","author":"Qian","year":"2020","journal-title":"World J. Gastroenterol."},{"key":"ref_8","first-page":"32","article-title":"The role of neoadjuvant and adjuvant therapies in the management of gastrointestinal stromal tumors: A comprehensive review","volume":"49","author":"Liu","year":"2023","journal-title":"Oncol. Rep."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"R\u00eembu, M.C., Popescu, L., Mih\u0103il\u0103, M., Sandulovici, R.C., Cord, D., Mih\u0103ilescu, C.-M., G\u0103l\u0103\u021banu, M.L., Pan\u021buroiu, M., Manea, C.-E., and Boldeiu, A. (2025). Synergistic Effects of Green Nanoparticles on Antitumor Drug Efficacy in Hepatocellular Cancer. Biomedicines, 13.","DOI":"10.20944\/preprints202501.1828.v1"},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"B\u00e9chade, D. (2025). Clinical Utility of Endoscopic Ultrasound (EUS) and Endobronchial Ultrasound (EBUS) in the Evaluation of Mediastinal Lymphadenopathy. Diagnostics, 15.","DOI":"10.3390\/diagnostics15030349"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/S0140-6736(12)61857-1","article-title":"Efficacy and safety of regorafenib for advanced gastrointestinal stromal tumours after failure of imatinib and sunitinib (GRID): An international, multicentre, randomised, placebo-controlled, phase 3 trial","volume":"381","author":"Demetri","year":"2013","journal-title":"Lancet"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1001\/jamaoncol.2016.5751","article-title":"KIT and PDGFRA Mutations in Patients With Gastrointestinal Stromal Tumor","volume":"3","author":"Joensuu","year":"2017","journal-title":"JAMA Oncol."},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Mohammadi, M., Roets, E., Bleckman, R.F., Oosten, A.W., Grunhagen, D., Desar, I.M.E., Bonenkamp, H., Reyners, A.K.L., van Etten, B., and Hartgrink, H. (2025). Impact of Mutation Profile on Outcomes of Neoadjuvant Therapy in GIST. Cancers, 17.","DOI":"10.3390\/cancers17040634"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1093\/annonc\/mdq076","article-title":"A phase I-II study of everolimus (RAD001) in combination with imatinib in patients with imatinib-resistant gastrointestinal stromal tumors","volume":"21","author":"Reichardt","year":"2010","journal-title":"Ann. Oncol."},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Catalano, F., Cremante, M., Dalmasso, B., Pirrone, C., Lagodin D\u2019Amato, A., Grassi, M., and Comandini, D. (2023). Molecular Tailored Therapeutic Options for Advanced Gastrointestinal Stromal Tumors (GISTs): Current Practice and Future Perspectives. Cancers, 15.","DOI":"10.3390\/cancers15072074"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1016\/S0140-6736(06)69446-4","article-title":"Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: A randomised controlled trial","volume":"368","author":"Demetri","year":"2006","journal-title":"Lancet"},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Handra, C.M., Chirila, M., Smarandescu, R.A., and Ghita, I. (2022). Near Missed Case of Occupational Pleural Malignant Mesothelioma, a Case Report and Latest Therapeutic Options. Int. J. Environ. Res. Public Health, 19.","DOI":"10.3390\/ijerph192214763"},{"key":"ref_18","first-page":"2290","article-title":"Surgical Management of Gastrointestinal Stromal Tumors: A Review of Current Evidence","volume":"27","author":"Ryu","year":"2020","journal-title":"Ann. Surg. Oncol."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Ran, P., Zhou, H., Li, J., Tan, T., Yang, H., Li, J., and Zhang, J. (2023). Improving Outcomes in the Advanced Gastrointestinal Stromal Tumors: The Role of the Multidisciplinary Team Discussion Intervention. J. Pers. Med., 13.","DOI":"10.3390\/jpm13030417"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1016\/j.hoc.2025.04.006","article-title":"Gastrointestinal Stromal Tumor: Current Approaches and Future Directions in the Treatment of Advanced Disease","volume":"39","author":"Cicala","year":"2025","journal-title":"Hematol. Oncol. Clin. N. Am."}],"container-title":["Current Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1718-7729\/32\/7\/383\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T18:02:40Z","timestamp":1760032960000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1718-7729\/32\/7\/383"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7,1]]},"references-count":20,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2025,7]]}},"alternative-id":["curroncol32070383"],"URL":"https:\/\/doi.org\/10.3390\/curroncol32070383","relation":{},"ISSN":["1718-7729"],"issn-type":[{"value":"1718-7729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7,1]]}}}