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Gastric outlet obstruction due to adenocarcinoma in a patient with Ataxia-Telangiectasia syndrome: a case report and review of the literature.

Otabor IA, Abdessalam SF, Erdman SH, Hammond S, Besner GE - World J Surg Oncol (2009)

Bottom Line: Ataxia-Telangiectasia syndrome is characterized by progressive cerebellar dysfunction, conjuctival and cutaneous telangiectasias, severe immune deficiencies, premature aging and predisposition to cancer.All patients presented with non-specific gastrointestinal complaints suggestive of ulcer disease.Although there was no correlation between immunoglobulin levels and development of gastric adenocarcinoma, the presence of chronic gastritis and intestinal metaplasia seem to lead to the development of gastric adenocarcinoma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatric Surgery, Nationwide Children's Hospital and The Ohio State University College of Medicine, Columbus, OH 43205, USA. iyore.otabor@nationwidechildrens.org

ABSTRACT

Background: Ataxia-Telangiectasia syndrome is characterized by progressive cerebellar dysfunction, conjuctival and cutaneous telangiectasias, severe immune deficiencies, premature aging and predisposition to cancer. Clinical and radiographic evaluation for malignancy in ataxia-telangiectasia patients is usually atypical, leading to delays in diagnosis.

Case presentation: We report the case of a 20 year old ataxia-telangiectasia patient with gastric adenocarcinoma that presented as complete gastric outlet obstruction.

Conclusion: A literature search of adenocarcinoma associated with ataxia-telangiectasia revealed 6 cases. All patients presented with non-specific gastrointestinal complaints suggestive of ulcer disease. Although there was no correlation between immunoglobulin levels and development of gastric adenocarcinoma, the presence of chronic gastritis and intestinal metaplasia seem to lead to the development of gastric adenocarcinoma. One should consider adenocarcinoma in any patient with ataxia-telangiectasia who presents with non-specific gastrointestinal complaints, since this can lead to earlier diagnosis.

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Hematoxylin and eosin stained sections of the gastric adenocarcinoma resected from our patient. [A] Cytological features of malignant glands; the cells are irregularly shaped with high nucleus to cytoplasm ratio and loss of nuclear polarity. The small dark cells are inflammatory cells (100× enlargement) [B] This area of tumor is in the serosa; there is redemonstration of irregular glands formed by tumor cells of varying sizes and orientation, with prominent nucleoli. The large clear spaces are fat cells (200× enlargement).
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Figure 3: Hematoxylin and eosin stained sections of the gastric adenocarcinoma resected from our patient. [A] Cytological features of malignant glands; the cells are irregularly shaped with high nucleus to cytoplasm ratio and loss of nuclear polarity. The small dark cells are inflammatory cells (100× enlargement) [B] This area of tumor is in the serosa; there is redemonstration of irregular glands formed by tumor cells of varying sizes and orientation, with prominent nucleoli. The large clear spaces are fat cells (200× enlargement).

Mentions: Gastric adenocarcinoma accounts for the majority of malignant gastric cancer. It arises from the glandular epithelium of the gastric mucosa. The most widely used Lauren histologic classification system divides gastric adenocarcinoma into two types – intestinal and diffuse [12]. The intestinal type, which is usually well-differentiated, originates from recognizable precancerous conditions such as gastric atrophy or intestinal metaplasia. It has a tendency to form glandular structures and spreads to distant organs hematogenously. The diffuse type is typically poorly differentiated, lacks gland formation and is composed of signet ring cells. Early metastases via lymphatic invasion commonly occur. Our patient had moderately differentiated intestinal type adenocarcinoma (Figure 3).


Gastric outlet obstruction due to adenocarcinoma in a patient with Ataxia-Telangiectasia syndrome: a case report and review of the literature.

Otabor IA, Abdessalam SF, Erdman SH, Hammond S, Besner GE - World J Surg Oncol (2009)

Hematoxylin and eosin stained sections of the gastric adenocarcinoma resected from our patient. [A] Cytological features of malignant glands; the cells are irregularly shaped with high nucleus to cytoplasm ratio and loss of nuclear polarity. The small dark cells are inflammatory cells (100× enlargement) [B] This area of tumor is in the serosa; there is redemonstration of irregular glands formed by tumor cells of varying sizes and orientation, with prominent nucleoli. The large clear spaces are fat cells (200× enlargement).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2662841&req=5

Figure 3: Hematoxylin and eosin stained sections of the gastric adenocarcinoma resected from our patient. [A] Cytological features of malignant glands; the cells are irregularly shaped with high nucleus to cytoplasm ratio and loss of nuclear polarity. The small dark cells are inflammatory cells (100× enlargement) [B] This area of tumor is in the serosa; there is redemonstration of irregular glands formed by tumor cells of varying sizes and orientation, with prominent nucleoli. The large clear spaces are fat cells (200× enlargement).
Mentions: Gastric adenocarcinoma accounts for the majority of malignant gastric cancer. It arises from the glandular epithelium of the gastric mucosa. The most widely used Lauren histologic classification system divides gastric adenocarcinoma into two types – intestinal and diffuse [12]. The intestinal type, which is usually well-differentiated, originates from recognizable precancerous conditions such as gastric atrophy or intestinal metaplasia. It has a tendency to form glandular structures and spreads to distant organs hematogenously. The diffuse type is typically poorly differentiated, lacks gland formation and is composed of signet ring cells. Early metastases via lymphatic invasion commonly occur. Our patient had moderately differentiated intestinal type adenocarcinoma (Figure 3).

Bottom Line: Ataxia-Telangiectasia syndrome is characterized by progressive cerebellar dysfunction, conjuctival and cutaneous telangiectasias, severe immune deficiencies, premature aging and predisposition to cancer.All patients presented with non-specific gastrointestinal complaints suggestive of ulcer disease.Although there was no correlation between immunoglobulin levels and development of gastric adenocarcinoma, the presence of chronic gastritis and intestinal metaplasia seem to lead to the development of gastric adenocarcinoma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatric Surgery, Nationwide Children's Hospital and The Ohio State University College of Medicine, Columbus, OH 43205, USA. iyore.otabor@nationwidechildrens.org

ABSTRACT

Background: Ataxia-Telangiectasia syndrome is characterized by progressive cerebellar dysfunction, conjuctival and cutaneous telangiectasias, severe immune deficiencies, premature aging and predisposition to cancer. Clinical and radiographic evaluation for malignancy in ataxia-telangiectasia patients is usually atypical, leading to delays in diagnosis.

Case presentation: We report the case of a 20 year old ataxia-telangiectasia patient with gastric adenocarcinoma that presented as complete gastric outlet obstruction.

Conclusion: A literature search of adenocarcinoma associated with ataxia-telangiectasia revealed 6 cases. All patients presented with non-specific gastrointestinal complaints suggestive of ulcer disease. Although there was no correlation between immunoglobulin levels and development of gastric adenocarcinoma, the presence of chronic gastritis and intestinal metaplasia seem to lead to the development of gastric adenocarcinoma. One should consider adenocarcinoma in any patient with ataxia-telangiectasia who presents with non-specific gastrointestinal complaints, since this can lead to earlier diagnosis.

Show MeSH
Related in: MedlinePlus