Gastric Signet Ring Carcinoma in a Patient With Ataxia-Telangiectasia

Patıroğlu, Türkan; Gungor, Hatice; Arslan, Duran; Denız, Kemal; Ünal, Ekrem; Çoşkun, Abdulhakim

doi:10.1097/mph.0b013e318279b3f7

Cited by 12 publications

(6 citation statements)

References 16 publications

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“…In conclusion, the study suggests that lymphocyte-rich gastric adenocarcinoma, in younger patients, especially if arising on a background of a rather unique severe, metaplastic, atrophic pangastritis, should evoke the search for CVID-associated morphological traits. Our CVID-based observations join the exceptional case reports of other immunodeficiency conditions (such as IPEX syndrome 19 and ataxia teleangectasia 20 ), with severe gastritis and pediatric gastric cancer. It is suggested that the astute surgical pathologist should consider immunodeficiencies in the assessment of young patients with severe atrophic gastritis and/or neoplasia of the stomach.…”

Section: Discussionsupporting

confidence: 82%

Gastric Adenocarcinoma in Common Variable Immunodeficiency

et al. 2014

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Common variable immunodeficiency (CVID) is associated with an increased risk of gastric cancer. The aim of the study was to determine the morphological features of CVID-associated gastric adenocarcinoma (CAGA) and of the background gastritis. The population of gastric cancer patients with CVID of Mayo Clinic in the period 2000-2010 was studied; 6 cases of CVID (2 males, 4 females, average age 47 years, age range 26-71 years) were found in 5793 patients with gastric cancer in the study period. Each patient underwent gastric resection for which histology slides were reviewed. Chronic gastritis variables, CVID-related findings, and features of the adenocarcinoma were recorded. CAGA was of intestinal type, with high number of intratumoral lymphocytes (ITLs). Cancer was diagnosed in younger patients than in the overall population of gastric cancer. Severe atrophic metaplastic pangastritis with extensive dysplasia was present in the background in 4 cases, with features of lymphocytic gastritis in 2 cases. Features of CVID (plasma cells paucity in 4 of 6 cases, lymphoid nodules prominent in four cases) could be detected. In summary, gastric adenocarcinoma at young age with ITLs, accompanied by atrophic metaplastic pangastritis, should alert the pathologist of the possibility of CAGA. It follows that, in presence of those characteristics, the search of CVID-associated abnormalities should be undertaken in the nonneoplastic tissues.

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Section: Discussionsupporting

confidence: 82%

Gastric Adenocarcinoma in Common Variable Immunodeficiency

et al. 2014

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“…A gastric operation was planned, but permission for further treatment and the operation was refused by the family, and she died 15 days after their refusal. 6 Case 3. A 7-year-old boy was referred to hospital because of a left inguinal mass.…”

Section: Cases 1-3: A-t Associated Malignanciesmentioning

confidence: 98%

Malignancies in Primary Immunodeficiencies: A Single Center Experience

PatirogluTurkan¹,

Haluk²,

Ünal³

et al. 2015

Pediatric Allergy, Immunology, and Pulmonology

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“…Patients' admission findings are mostly nonspecific, and cases with vomiting, hematemesis, abdominal pain, anemia, weight loss, pulmonary hypertension, thrombotic microangiopathy, and massive ascites have been reported (2,(5)(6)(7). Patıroğlu et al (8) reported that they diagnosed gastric signet ring cell carcinoma in an 18-year-old patient with ataxia telangiectasia and Hashimato thyroiditis. Ataxia telangiectasia is a primary immunodeficiency disease whose susceptibility to cancer is increasing.…”

Section: Discussionmentioning

confidence: 99%