scholarly journals Signet ring cell cancer of Ampulla of Vater—first ever case reported in a teenager and a review of literature

Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110077
Author(s):  
Asad Ali Kerawala ◽  
Abid Jamal ◽  
Lubna Saleem

Ampullary cancers are rare accounting for 0.2% of all gastrointestinal cancers. Signet ring is a rare variant of adenocarcinoma, characterized by having more than 50% of mucin secreting cells and clinically having a worse prognosis. We present the case of a teenage girl with this tumor, the youngest ever reported in medical literature. An 18 years old girl with no significant past medical history presented to our clinic with symptoms of upper abdominal pain and jaundice. Her upper GI endoscopy showed an ampullary lesion which was biopsied—diagnosing it as adenocarcinoma. She underwent a pancreato-duodenectomy (Whipple’s procedure) with Child’s reconstruction and a feeding jejunostomy. Her final histopathology report was documented as infiltrating adenocarcinoma of Signet Ring variety arising from the Ampulla of Vater. Being such a rare entity, there is a lack of randomized trials advising the optimum treatment for such cases. Till then anecdotal experiences will drive the optimum management of this rare disease.

2018 ◽  
Vol 9 (1) ◽  
Author(s):  
Yang Shu ◽  
Weihan Zhang ◽  
Qianqian Hou ◽  
Linyong Zhao ◽  
Shouyue Zhang ◽  
...  

2019 ◽  
Vol 51 (1) ◽  
pp. 354-358 ◽  
Author(s):  
Chandana Javvaji ◽  
Jacob R. Bledsoe ◽  
Lloyd Hutchinson ◽  
Ediz F. Cosar ◽  
Lacey J. McIntosh ◽  
...  

2011 ◽  
Vol 29 (4_suppl) ◽  
pp. 58-58 ◽  
Author(s):  
R. Thota ◽  
T. Tashi ◽  
W. Gonsalves ◽  
V. Murukesan ◽  
P. Townley ◽  
...  

58 Background: Signet ring cell carcinoma accounts for less than 1% of all colon cancers. We examined the clinical pathological features and prognosis of signet ring cell carcinoma of colon and compare it with mucinous and non-mucinous adenocarcinoma of colon. Methods: A total of 206 patients diagnosed with signet ring cell carcinoma from 1995 to 2009 were identified from the VA Central Cancer Registry (VACCR) database. Age, race, histology, grade, lymph node status, stage and type of treatment received data were collected. Results: Out of 206 patients, 173 (83.9%) were white, 31 (15%) were black, and 2 patients were listed as unknown. Median age of diagnosis was 67 years as compared to 70 years for both mucinous and non-mucinous adenocarcinoma of colon. Pathological T-stages were as follows: T1 = 2.9%, T2=5.3%, T3=33.9%, T4= 25.7%, and unknown 32%. Of the total, 22.3% were located in caecum, 21.8% in ascending colon, 15.5% in sigmoid colon, 7.7% in appendix and hepatic flexure of colon, 11.1% in transverse colon, 2.9% in splenic flexure and 4.4% in descending colon. 33.5% were lymph node positive, 34.6% were lymph node negative, and 31.8% were unknown. Histologically grade 3 (55.4%) was most commonly reported followed by grade 2 (7.3%), grade 1 (2.5%), grade 4 (1.9%)and in 33% grade was unknown. 41.3% patients received only surgery while 34% received surgery with adjuvant chemotherapy, 7.3% received chemotherapy alone and 7.8% patients received either chemotherapy, radiation or hormonal therapy alone, 9% did not receive any therapy. 1 year, 3 year and 5 year survivals for signet ring cell cancer compared to adeno carcinoma was 60% vs 80%, 33% vs 60%, and 24% vs 47% respectively. Median survival of signet ring cell carcinoma compared to mucinous and non mucinous adenocarcinoma was 19 months, 48 months and 62 months respectively. Conclusions: Signet ring cell carcinoma of colon has poor survival rates than the other histological subtypes. Signet ring cell carcinoma presents at an earlier age, higher tumor grade and advanced stage at diagnosis when compared to mucinous and non-mucinous adenocarcinoma of colon. Due to rarity of this disease further multi-institute studies are required for in-depth understanding and analysis of this disease. No significant financial relationships to disclose.


2015 ◽  
Vol 72 (6) ◽  
pp. 381
Author(s):  
Minchul Kim ◽  
Yoon Young Jung ◽  
Myung-Won You ◽  
Dong Hee Kim ◽  
Won Mee Lee ◽  
...  

2012 ◽  
Vol 104 (9) ◽  
pp. 501-502 ◽  
Author(s):  
Lucía Lesquereux-Martínez ◽  
Aquilino Fernández-Pérez ◽  
Manuel Bustamante-Montalvo

2020 ◽  
Vol 13 (4) ◽  
pp. 607-614
Author(s):  
Chisaki Ikeda ◽  
Naohiko Makino ◽  
Akiko Matsuda ◽  
Yasuharu Kakizaki ◽  
Tetsuya Ishizawa ◽  
...  

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