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29 May 2013 - Case of the Week #275
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Case of the Week #275
In 1986, a 13 year old girl in Pripyat, now an abandoned city in the Ukraine, was exposed to the Chernobyl nuclear plant explosion for 48 hours prior to being evacuated. She lived 3 km from the nuclear plant. Twenty two years later, at age 35 years, she developed recurring upper abdominal pain and underwent upper endoscopy with gastric biopsy.
What is your diagnosis?
Signet ring cell adenocarcinoma of the stomach, also known as diffuse type adenocarcinoma
Immunostains were performed (left CAM 5.2, right CEA):
The biopsy shows a gastric adenocarcinoma characterized by gastric mucous cells that infiltrate the stroma as single cells, or small clusters of cells with numerous signet ring cells where intracellular mucin pushes the nucleus to the periphery. The tumor cells were immunoreactive for CAM5.2 and CEA (shown above). Often the central intracellular mucin can be highlighted with mucicarmine or Alcian blue-PAS stains (intracellular mucin gives a “target pattern”). The tumor cells are also immunoreactive for EMA, villin and Ki-67 and negative for CD45 and TTF1.
The differential diagnosis includes:
• Lymphoma with artifactual signet ring cells due to cytoplasmic shrinkage. CD45 is usually positive in lymphoma.
• Metastases from breast or lung; clinical history, plus the use of appropriate immunostains such as TTF1 or GCDFP-15 is helpful.
• Reactive epithelial atypia associated with radiation treatment or chemotherapy.
The Chernobyl nuclear accident is known for having caused a large number of aggressive pediatric papillary thyroid carcinomas, especially in Belarus (1). There may be some Chernobyl-related gastric cancers that exhibit an unusually aggressive clinical course (2). The treatment usually involves surgical resection or chemotherapy. Presently the prognosis is poor.
1. Mahoney MC, Lawvere S, Falkner KL, Averkin YI, Ostapenko VA, Michalek AM, Moysich KB, McCarthy PL: Thyroid cancer incidence trends in Belarus: examining the impact of Chernobyl, Int J Epidemiol 2004;33:1025
2. Shchepotin IB, Valetsky VL, Chorny VA, Shabahang M, Nauta RJ, Buras RR, Evans SR: Carcinoma of the stomach following the Chernobyl nuclear accident, Eur J Cancer 1997;33:1413
Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
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