scholarly journals Rectal Gastrointestinal Stromal Tumor (GIST): About a Rare and Unusual Case

2021 ◽  
Vol 6 (11) ◽  
pp. 814-817
Author(s):  
Abderrahmane Jallouli ◽  
Mariama Jarti ◽  
Marj Zohour Haida ◽  
Mouna El Bouatmani ◽  
Adil Ait Errami ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumor ◽  
Kinase Inhibitors ◽  
Stromal Tumor ◽  
Large Tumor ◽  
Lower Rectum ◽  
Local Invasion ◽  
Rectal Gist ◽  
Multidisciplinary Consultation

Rectal gastrointestinal stromal tumors (GIST) are extremely rare, accounting for approximately 0.1% of all rectal tumors. Diagnosis is based on histological and immunohistochemical confirmation. We report the case of a 38-year-old patient with a rectal GIST revealed by chronic rectal bleeding associated with rectal tenesmus, abdominal pain with painful contractions and frequent evacuations evolving in a context of altered general state. Rectoscopy was used to visualize the tumor mass and to take biopsies; their histological and immunohistochemical study revealed a rectal location of a gastrointestinal stromal tumor. The extension assessment was negative. After discussion of the case in a multidisciplinary consultation meeting, management consisted of initially putting the patient on Imatinib given the large tumor size, the degree of local invasion and the location of the mass (lower rectum), with regular follow-up in order to schedule a less invasive surgical resection later. Despite the rarity of rectal GIST, early diagnosis is necessary to avoid progression to locoregional invasion complicating some surgical resections given the anatomical constraints of the pelvic region. Hence the interest of neoadjuvant therapy with tyrosine kinase inhibitors allowing in some cases a decrease in tumor volume, a regression of the degree of local invasion and a decrease in the morbidity of the surgery in order to improve the patient's quality of life.

scholarly journals Vagus-Preserving Proximal Gastrectomy with Double- Flap Technique for Gastrointestinal Stromal Tumor involving the Esophagogastric Junction：A Case Report

2021 ◽  
Author(s):  
Wei Peng ◽  
Yi-Kai Huang ◽  
Yong-You Wu ◽  
Wei Gong ◽  
Zheng-Rong Chen ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumor ◽  
Proximal Gastrectomy ◽  
Partial Solution ◽  
Stromal Tumor ◽  
Case Presentation ◽  
Flap Technique ◽  
Enhanced Ct ◽  
Feasible Option

Abstract Background: Gastrointestinal stromal tumor (GIST) involving the esophagogastric junction (EGJ) is a relatively rare but thorny problem. It represents a dilemma between complete resection and impairment of quality of life (QOL) for loss of cardiac sphincter or even the whole stomach. Thus, emergence of function preserving gastrectomy is necessary and may provide partial solution to this hot potato. Here, we would like to introduce a method which successfully settle this matter through a clinical case. Case presentation: A 46-year-old male complaining of a progressive dysphagia. A huge GIST with 120mm in diameter was diagnosed by enhanced CT scan and pathological exam. Imatinib mesylate (IM) was initially prescribed, the tumor was shrank to a large extent while drug resistance emerged 6 months after therapy, leaving a mass approximately 90mm in diameter. We applied a method of vagus preserving proximal gastrectomy (VP-PG) and esophagogastrostomy with double-flap technique (DFT) for the patient. The operation was smooth and postoperative courses within a 12-months follow-up were uneventful. The patient had no digestive symptoms but slight leucopenia due to IM toxicity during this medication period. Conclusion: We consider that the application of VP-PG and DFT subsequent to neoadjuvant therapy with IM is likely to be an effective and feasible option for GISTs with EGJ involvement.

Fulminant Hepatic Failure Caused by Diffuse Liver Metastases following Gastrointestinal Stromal Tumor Resection

2009 ◽  
Vol 2 ◽  
pp. CGast.S3221
Author(s):  
Abdel-Rauf Zeina ◽  
Alicia Nachtigal ◽  
Eugene Vlodavsky ◽  
Jochanan E. Naschitz
Keyword(s):  
Liver Metastases ◽  
Gastrointestinal Stromal Tumor ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Tumor Resection ◽  
Stromal Tumor ◽  
Prognostic Features ◽  
Elevated Liver Enzymes ◽  
Complete Tumor

Metastatic tumors to the liver resulting in fulminant hepatic failure are a rare occurrence and have not been previously described in patients with gastrointestinal stromal tumor (GIST). A 70 year-old man was referred to hospital with postprandial discomfort. On examination a 19.5 cm large epithelioid GIST of the stomach was diagnosed. The mass exhibited unfavorable prognostic features: mitotic index 18/50 high-power fields, large primary tumor size and male sex. Complete tumor resection with negative margins was achieved and metastases were not detected. The patient presented six months later with jaundice, asterixis and elevated liver enzymes. Computerized tomography showed multiple liver hypodense lesions representing metastases. Treatment with imatinib mesylate was of no avail and the patient died 3 days later as the result of hepatic failure. Massive liver metastases can, even though rarely, be responsible for fulminant hepatic failure. Clinical and radiological follow-up are crucial in patients with GIST even after surgical resection.

Patient journey and quality of life (QOL) among diffuse-type TGCT in the U.S.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e23565-e23565
Author(s):  
Nicholas M. Bernthal ◽  
Geert Spierenburg ◽  
Michiel van de Sande ◽  
John H. Healey ◽  
Silvia Stacchiotti ◽  
...  
Keyword(s):  
Kinase Inhibitors ◽  
Diagnostic Delay ◽  
Tendon Sheath ◽  
Real World Data ◽  
Patient Journey ◽  
Delay In Diagnosis ◽  
Frequent Use ◽  
The Us

e23565 Background: Diffuse Tenosynovial Giant Cell Tumor (d-TGCT) is a rare, locally aggressive neoplasm of joint and tendon sheath synovia, which may severely affect patients (pts) QOL due to pain, limited joint function and destruction of bone and local tissues. This study analyzes real-world data to describe d-TGCT pt journey and QOL in the US. Methods: The TGCT Observational Platform Project (TOPP), the first non-interventional prospective disease registry, enrolled d-TGCT pts at 10 Western European (EU) and 2 US sarcoma centers from Nov 2016 to Mar 2019, with prospective follow-up of 24 mo. Pts demographics, disease history and ongoing medical treatment and pt-reported outcomes (PROs) (pain, stiffness, PROMIS Physical Function [PF] and EQ-5D) were collected at baseline and every 6 mo during follow-up periods. Descriptive analyses from baseline compared US pts with Western EU pts (Table). Results: Of 166 d-TGCT pts, 30 were enrolled in the US. Mean age was 40.6 ± 12.82 y, with 63.3% female and 66.7% with tumor in knee. Median time from first symptom to diagnosis was 12 mo (range; 0–153). 28 pts (93.3%) had surgery, 4 pts (13.3%) had tyrosine kinase inhibitors (TKI), no pts had radiotherapy. 18 pts (60%) experienced >1 locoregional recurrence. Median Pain Severity Score (PSS) and Pain Interference Score (PIS) were 2.8 and 3.0, respectively. Median PROMIS PF Score was 39.8. Mean EQ-5D index score and visual analogue scale (VAS) were 0.73 ±0.229 and 75.5 ±13.31, respectively. Compared with Western EU pts, US pts reported similar PROs and QOL, were younger, had shorter delay in diagnosis, and lower use of systemic therapy or radiotherapy (Table). Conclusions: d-TGCT affects a relatively young population in the US, similar to Western EU, resulting in serious impairment in daily activities and QOL. Shorter diagnostic delay and less frequent use of non–surgical approach was observed in the US. [Table: see text]

Sa1543 Long Term Follow-Up Result of Endoscopic Resection of Gastric Gastrointestinal Stromal Tumor

2015 ◽  
Vol 81 (5) ◽  
pp. AB254-AB255
Author(s):  
Min Jae Yang ◽  
Sun Gyo Lim ◽  
Sung Jae Shin ◽  
Kee Myung Lee
Keyword(s):  
Gastrointestinal Stromal Tumor ◽  
Endoscopic Resection ◽  
Stromal Tumor ◽  
Long Term Follow Up

Separate Occurrence of Extra-adrenal Paraganglioma and Gastrointestinal Stromal Tumor in Monozygotic Twins: Probable Familial Carney Syndrome

2004 ◽  
Vol 7 (4) ◽  
pp. 380-384 ◽  
Author(s):  
Liliane Boccon-Gibod ◽  
Françoise Boman ◽  
Sabah Boudjemaa ◽  
Monique Fabre ◽  
Guy Leverger ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumor ◽  
Monozygotic Twins ◽  
Monozygotic Twin ◽  
Stromal Tumor ◽  
Gastric Gist ◽  
Carney Triad ◽  
Pulmonary Chondroma ◽  
Carney Syndrome ◽  
Extra Adrenal Paraganglioma

The nonfamilial Carney triad includes paraganglioma, gastrointestinal stromal tumor (GIST), and pulmonary chondroma. Some paraganglioma-GIST diads are familial and inherited in an apparent autosomal dominant manner. The familial paraganglioma-GIST syndrome differs from the Carney triad by the absence of female predilection and predominance of paragangliomas. We report the cases of a 12-year-old boy with a paraganglioma of the organ of Zuckerkandl, and his 13-year-old monozygotic twin with a gastric GIST. These two patients, to our knowledge, are the first to be reported as likely having the familial paraganglioma-GIST syndrome following its description by Carney and Stratakis (Am J Med Genet 2002;108:132–139) in 12 patients from five families. A lifetime follow-up and a periodic search for both tumors are indicated in these patients and their families.

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