Stomach Cancer of a 14-Year-Old Boy with Ataxia-telangiectasia

Gastric Signet Ring Carcinoma in a Patient With Ataxia-Telangiectasia

“…Further, 2 cases with gastric adenocarcinoma were described by Kondo and Horikawa and a gastric neoplasm was found who had normal IgA. 15 In our case, we detected both IgA deficiency and chronic gastritis.…”

“…8,10,11 Ten cases of gastric cancer associated with A-T have been reported in the literature; 8 of these cases were reported in the English literature. [12][13][14][15][16][17][18] Among the 10 patients with gastric carcinoma, mucinous adenocarcinoma were found in 5 patients, adenocarcinoma in 4 patients, and adenocarcinoma tubulare mucocellulare in remaining 1 patient which included many signet ring cells (Table 1). Signet ring cell carcinoma is one of the 5 types of gastric adenocarcinoma.…”

“…Furthermore, in the setting of new-onset nausea, vomiting, and anorexia, early radiologic examination and endoscopy for this tumor is recommended for early diagnosis. 15 14 y M Adenocarcinoma tubulare mucocellulare Frais 16 26 y M Adenocarcinoma Murphy et al 17 15 y F Mucinous adenocarcinoma 17 y F Mucinous adenocarcinoma Otabor et al 18 20 y F Adenocarcinoma…”

Gastric adenocarcinoma due to ataxia-telangiectasia (Louis-Bar syndrome).

“…They had reduced IgA levels. A further two cases with gastric adenocarcinoma were described by Kondo and Horikawa (1975), and a gastric neoplasm was found in a 14-year-old boy who had normal IgA (Watanabe et al, 1977). This is a further report of gastric adenocarcinoma in a young person as a consequence of ataxiatelangiectasia, and it is therefore proposed that ataxia-telangiectasia be recognised as predisposing to gastric adenocarcinoma.…”

Astrocytoma linked to familial ataxia-telangiectasia

“…For example, AT with dysgerminoma [12,21], gastric carcinoma [7,36], hepatocellular carcinoma [17], renal cell carcinoma [38], retinoblastoma [37], papillary carcinoma of thyroid [21], and adenocarcinoma of pancreas [21], are listed.…”