scholarly journals Gastrointestinal Stromal Tumor of the Ampulla of Vater: A Narrative Review

2021 ◽  
pp. 1-6
Author(s):  
Bita Geramizadeh ◽  
Alireza Shojazadeh

<b><i>Background:</i></b> Gastrointestinal stromal tumor (GIST) of the ampulla of Vater is a rare occurrence. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. <b><i>Summary:</i></b> In this review, we will discuss all the reported details of the published cases, including demography, clinical presentation, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome of cases with the diagnosis GIST from the ampulla of Vater in the last 20 years. <b><i>Key Message:</i></b> Twenty-five cases of GIST in the ampulla of Vater have been reported in the last 20 years in the English literature. GIST in the ampulla of Vater are usually small tumors (&#x3c;5 cm) in middle-age patients. The majority of the patients present with lower GI bleeding and abdominal pain. Imaging findings are not characteristic, and most of the patients without biopsy and with no histologic diagnosis were operated with the primary impression of adenocarcinoma, neuroendocrine tumor, and GIST. Perioperative tissue biopsy has been accurate in &#x3c;70% of the cases. The majority of the reported cases of GISTs in the ampulla of Vater have been low risk with spindle-cell morphology, low mitotic figures, and minimal atypia; reactive for C-KIT and DOG-1; and nonreactive for SMA, desmin, and S100. In the majority of the cases, duodenectomy with or without Whipple’s operation has been performed, and most of the cases showed good prognosis.

2019 ◽  
Vol 128 (11) ◽  
pp. 1086-1091 ◽  
Author(s):  
Jonathan Reid ◽  
Bret Wehrli ◽  
Leigh J. Sowerby

Background: Spindle cell lipomas (SCLs) are benign tumors that are characteristically present on the upper back and neck, but in rare cases present throughout the respiratory mucosa, causing hoarseness, stridor, dyspnea, and obstruction. Objective: To highlight the importance of considering SCL in the diagnosis of benign respiratory tract tumors, a literature review identified all published cases of respiratory tract SCLs, including 2 from our institution: one case in the nasopharynx and 1 in the nasal valve. Methods: All case reports, series and literature reviews from the English literature from 1975 through March 2018 were systematically identified for review in the MEDLINE, EMBASE, and Scopus databases. Two additional cases from our institution were described. Results: In total, 24 cases of SCL in the respiratory tract were identified for review. Two cases from our institution are described here, bringing the total of reported cases to 26. Extensive analyses of oral cavity SCLs already exist, so we excluded this site from our review and focused on sites where SCLs may present with respiratory symptoms. Excluding the oral cavity and oropharynx, the most common location described is the larynx. All 26 cases were treated with excision. One tumor required a second surgery, but there were no other complications nor recurrences. Conclusion: Although rare, SCLs may arise from throughout the respiratory tract and cause dyspnea, hoarseness and stridor. Spindle cell lipoma should be considered in the differential diagnosis of a respiratory tract mass. This diagnosis confers a good prognosis and patients may be reassured that surgery is almost always curative.


2010 ◽  
Vol 56 (1) ◽  
pp. 39
Author(s):  
Jeung Eun Park ◽  
Seok-Ho Dong ◽  
Kun Hyung Cho ◽  
Jae Young Jang ◽  
Hyo-Jong Kim ◽  
...  

2014 ◽  
Vol 32 (3_suppl) ◽  
pp. 437-437
Author(s):  
I-Jen Chiang ◽  
Wen-Yi Shau ◽  
Wei-Tse Fang ◽  
Chi-Hui Fang ◽  
Yen-Yang Chen

437 Background: Gastrointestinal stromal tumor (GIST) is a visceral sarcoma that arises from the gastrointestinal tract. Currently, imatinib (first-line) and sunitinib (second-line) are two reimbursed targeted therapies for GIST in Taiwan. This study aimed to evaluate the real world data of targeted therapies in GIST treatment among Taiwanese population. Methods: We conducted a nationwide retrospective cohort study based on data from the National Health Insurance Research Database (NHIRD) between January 2005 and December 2010. NHIRD is a comprehensive database of administrative and claim data which covers over 99% of entire population in Taiwan. Patients were selected with ICD codes and targeted therapy utilization, imatinib (IMA) or sunitinib (SUN). Time to treatment discontinuation (DC) due to any reasons (such as death, intolerance and disease progression) was estimated using Kaplan-Meier analysis. Results: From 2005 to 2010, the incidence rate of GIST in Taiwan was between 20 and 30 cases per million. A total of 3,436 GIST patients were identified; the mean age was 63.2 years and 57 % were male. Most patients (94.7%) received standard dose IMA (…400mg/day). Among these patients, 136 patients (4.0%) required IMA dose escalation and 44 (1.3%) patients switched to SUN after IMA. The probability of the 1st-line treatment DC was 20%, 30.7%, 38.9%, 43.2%, and 47.1% for years 1 to 5, respectively. The probability of a change in treatment pattern (defined as either IMA dose escalation or switch to SUN) was 4% 6.8%, 9.6%, 10.5% and 11.7% for years 1 to 5. Lastly, the probability of the 2nd-line treatment DC was 20.5%, 33.7%, 41.1%, 44.8%, 51.7% for years 1 to 5, respectively. Conclusions: First-line IMA plus other treatment modalities may provide additional benefits to progression-free survival. Taiwanese GIST patients who failed first-line treatment still gained benefit from either IMA dose escalation or a switch to SUN. Additional analysis is required for the detailed comparison in different treatment patterns.


2001 ◽  
Vol 125 (5) ◽  
pp. 665-668
Author(s):  
En Yoneda ◽  
Kazuhiro Teramura ◽  
Shingo Hiruma ◽  
Takao Satou ◽  
Shigeo Hashimoto

Abstract A case of malignant gastrointestinal stromal tumor (GIST) is reported. Histologically, spindle cell proliferation with remarkable whorl formations was predominant in the tumor. Immunohistochemically, the tumor cells were diffusely positive for CD117 (c-Kit) and vimentin and partially positive for CD34. Ultrastructurally, the desmosome-like structures and interdigitations occurred much more frequently in the areas with whorl formations. These organelles were considered to be closely associated with the whorl formations. Various kinds of cellular arrangements are revealed in GISTs, but remarkable whorl formations, such as in our case, are a rare variant pattern. Herein, we discuss the histopathologic differences between this and other tumors showing whorl formations and describe the meaning of this unique arrangement. GISTs are thought to be immature tumors, and, therefore, variations in histopathologic findings are recognized. Finally, the ultrastructural study of GISTs is useful for understanding the mechanisms forming whorl formations and the differentiation or pathogenesis of GISTs.


2013 ◽  
Vol 17 (4) ◽  
pp. 372-376 ◽  
Author(s):  
Fernando Candanedo-Gonzalez ◽  
Leslie Camacho-Rebollar ◽  
Candelaria Cordova Uscanga ◽  
Alejandra Romero Utrilla ◽  
Maria Eugenia Palmerin Bucio ◽  
...  

2013 ◽  
Vol 6 (3) ◽  
pp. 198-201 ◽  
Author(s):  
Yi Mou ◽  
Chuncheng Wu ◽  
Hang Yi ◽  
Wei Liu ◽  
Li Jing ◽  
...  

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