Gastrointestinal Stromal Tumours (GIST): Indian Experience of Rare Malignancy

Malik, Kanuj; Seshadri, Ramakrishnan A; Sundersingh, Shirley; Dhanushkodi, Manikandan

doi:10.1007/s13193-020-01095-6

Cited by 2 publications

(3 citation statements)

References 36 publications

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“…7 In our study GIST was more common in males than females consistent with the studies conducted in other parts of the country. 7,8 Age of the patients varied from 25 years to 76 years with 50 years as median age of diagnosis. The various sites of lesion in our study were stomach, ileum, jejunum, esophagus, rectum and in two patients the exact origin could not be ascertained.…”

Section: Discussionmentioning

confidence: 99%

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Clinical, pathological, radiological characteristics, risk stratification and immunohistochemistry profile of gastrointestinal stromal tumors treated in a tertiary cancer centre located in Sub-Himalayan region: institutional experience of 20 patients and review of literature

Sharma¹,

Negi²,

Gupta³

et al. 2021

Int Surg J

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GIST (gastrointestinal stromal tumors) are the rare mesenchymal tumors. Treatment includes curative surgery along with targeted agents like TKI in neoadjuvant/adjuvant settings. A total of 20 patients of histology proven GIST who were registered between 2014 to 2020 were reviewed for clinico-pathological data, endoscopic and radiological investigations, sites, primary treatment received, histology, immunohistochemistry, stage, risk stratification and imatinib therapy. GIST was more common in males than females. Age group varied between 25 years to 76 years. Majority of patients consumed non vegetarian diet, half of them being smokers and consumed alcohol. Pain abdomen, abdominal lump, dysphagia, haematemesis, melena and blood in stools were presenting complaints. CECT revealed heterogeneously enhancing mass with necrosis as most common finding. Upper GI endoscopy/colonoscopy revealed extrinsic bulge, polypoidal growth or ulcers as main findings. Stomach was the most common site followed by jejunum. Few patients presented with metastatic disease to liver and lungs. HPE revealed spindle cell GIST as main histology (with one patient with mixed spindle and epithelioid cells) with all patients having immunoreactivity to CD117. Majority of patients belonged to stage III and high-risk category by NIH stratification criteria. In majority of patients treatment received was surgery followed by adjuvant imatinib. Few patients had unresectable disease at presentation and received imatinib as upfront therapy. Imatinib was well tolerated in majority of patients. Few experienced manageable side effects like headache, irritability, leukopenia, pain abdomen, vomiting. Duration of treatment was one to three years. GIST is a rare tumor with varied presentations. Surgery is the mainstay of treatment offering chances of cure and revolutionary adjuvant imatinib is well tolerated with mild and manageable side effects in our centre. Being a resource limited centre, affordability for special investigations like IHC (immunohistochemistry) for CD117 (which helps in further confirmation of the diagnosis), remains a challenge for the patient and so does the 2nd line agent like sunitinib in case of recurrence.

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Section: Discussionmentioning

confidence: 99%

“…Sharma J et al Int Surg J. 2021 Aug;8(8):2414-2419 sort of treatment advised (N=1, 5%). Out of sixteen patients who received surgery as the primary treatment 14 patients underwent R0 resection, one patient each underwent R1 and R2 resection.…”

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confidence: 99%

Clinical, pathological, radiological characteristics, risk stratification and immunohistochemistry profile of gastrointestinal stromal tumors treated in a tertiary cancer centre located in Sub-Himalayan region: institutional experience of 20 patients and review of literature

Sharma¹,

Negi²,

Gupta³

et al. 2021

Int Surg J

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“…There are numerous challenges that developing countries are facing as reflected by sparse data, fewer reports of mutation testing, lack of GIST new trials, the absence of reporting of wild-type GIST, difficulty in procuring newer drugs, few support groups, lack of dedicated centres and lack of collaboration. Indian data describing and analysing the mutations is sparse [11][12][13][14]. Minhas et al [15] and Cyriac et al [16] described a cohort of 31 and 24 patients, respectively, wherein c-kit was the most common mutation seen.…”

Section: Introductionmentioning

confidence: 99%

Insights into the medical management of gastrointestinal stromal tumours: lessons learnt from a dedicated gastrointestinal stromal tumour clinic in North India

Baa¹,

Rastogi²,

Fernandes³

et al. 2023

ecancer

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Background:The advent of molecular driver alterations has brought in a revolutionary transformation in the treatment landscape of gastrointestinal stromal tumour (GIST). However, there is a paucity of data regarding mutational testing prevalence and associated outcomes from India. Methods:It was a retrospective study. We reviewed the case records of all patients diagnosed with GIST in a tertiary care centre from 2015 to 2021. The clinicopathological, mutational analysis and treatment plans were recorded. The study cohort was characterised by descriptive statistics.Results: Our study included 120 patients with a median age of 53 years (range: 28-77), with a male preponderance of 2:1. The most common site of the primary was the stomach (50%), followed by the small intestine (37%), with 55.8% of the patients having disseminated disease at presentation with a predominance of liver metastasis (67%). The prevalence of mutational analysis among patients prior to referral was 4%. 60.8% of the patients at our clinic had mutational analysis performed, and unavailability of analysis in the rest was due to financial constraints (12.5%), exhaustion of tissue (7.5%), reluctance to repeat biopsy (4.1%) and low-risk patients. We report c-kit in the majority (52%), platelet-derived growth factor receptor (PDGFR) in 19.2% and wild type in 16.4% along with the rarer subtypes: succinate dehydrogenase (SDH)-deficient GIST in 10.9% and Neurotrophic tyrosine receptor kinase (NTRK) fusion in 1.3%. Four of the eight SDH-deficient GIST patients had germline mutations (50%). The knowledge of driver mutations led to a change of treatment in 39.7% (29/73), i.e. stoppage of tyrosine kinase inhibitor (TKI) in 3, switch of TKI in 23, increase in TKI dose in 2 and upfront surgery in 1. The most common change was the use of sunitinib and regorafenib in patients with SDH-deficient GIST. Conclusion:Our study is one of the largest comprehensive series describing the clinical and mutational profile of GIST from India. The mutation testing rates at primary care centres continue to be low. Despite the hurdles, a large percentage of our patients underwent molecular testing, aiding in therapeutic decision-making.

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Gastrointestinal Stromal Tumours (GIST): Indian Experience of Rare Malignancy

Cited by 2 publications

References 36 publications

Clinical, pathological, radiological characteristics, risk stratification and immunohistochemistry profile of gastrointestinal stromal tumors treated in a tertiary cancer centre located in Sub-Himalayan region: institutional experience of 20 patients and review of literature

Clinical, pathological, radiological characteristics, risk stratification and immunohistochemistry profile of gastrointestinal stromal tumors treated in a tertiary cancer centre located in Sub-Himalayan region: institutional experience of 20 patients and review of literature

Insights into the medical management of gastrointestinal stromal tumours: lessons learnt from a dedicated gastrointestinal stromal tumour clinic in North India

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