scholarly journals Synchronous detection of extra-adrenal paraganglioma in a follow-up case of high-grade gastrointestinal stromal tumor

2021 ◽  
Vol 36 (2) ◽  
pp. 205
Author(s):  
Anurag Jain ◽  
Azhar Husain ◽  
Harkirat Singh ◽  
Arun Hegde
2004 ◽  
Vol 7 (4) ◽  
pp. 380-384 ◽  
Author(s):  
Liliane Boccon-Gibod ◽  
Françoise Boman ◽  
Sabah Boudjemaa ◽  
Monique Fabre ◽  
Guy Leverger ◽  
...  

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.


2006 ◽  
Vol 67 (7) ◽  
pp. 1541-1545 ◽  
Author(s):  
Hisashi ISHIKURA ◽  
Hiroshi OKITSU ◽  
Akihiro SAKATA ◽  
Masashi ISHIKAWA ◽  
Jyunnichi SEIKE ◽  
...  

Author(s):  
Abderrahmane Jallouli ◽  
Mariama Jarti ◽  
Marj Zohour Haida ◽  
Mouna El Bouatmani ◽  
Adil Ait Errami ◽  
...  

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.


Author(s):  
Edward C. Rosenow

1. Pulmonary chondroma 2. Gastrointestinal stromal tumor (GIST), formerly, gastric epithelioid leiomyosarcoma 3. Functioning extra-adrenal paraganglioma • Triad eventually present in more than half of patients • 〉90% of patients are female • Mean age: 17 years • Mechanism: unknown (disease absent in 1 identical twin)...


2009 ◽  
Vol 2 ◽  
pp. CGast.S3221
Author(s):  
Abdel-Rauf Zeina ◽  
Alicia Nachtigal ◽  
Eugene Vlodavsky ◽  
Jochanan E. Naschitz

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.


2015 ◽  
Vol 81 (5) ◽  
pp. AB254-AB255
Author(s):  
Min Jae Yang ◽  
Sun Gyo Lim ◽  
Sung Jae Shin ◽  
Kee Myung Lee

2011 ◽  
Vol 2 (6) ◽  
pp. 114-117 ◽  
Author(s):  
Marcelo Eller Miranda ◽  
Luiz Ronaldo Alberti ◽  
Edson Samesima Tatsuo ◽  
Clécio Piçarro ◽  
Marcelo Rausch

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