Primary gastric T-cell lymphomas are extremely rare. We report a Ki-1 negative primary gastric T-cell lymphoma of the anaplastic large cell type associated with extensive involvement of the skin, gastrointestinal tract, and other organ involvement in a patient not known to be immunocompromized. Biopsy of the stomach and distal esophagus showed infiltration of gastric mucosa and the squamous epithelium by nests of large atypical cells with vacuolated or clear cytoplasm, consistent with large cell lymphoma. Immunohistochemical staining of these specimens revealed neoplastic cells bearing LCA (CD45) and T-cell markers (UCHL1/CD45R0 and CD3). This represents the only case so far with synchronous presentation of gastric, esophageal, and cutaneous involvement without evidence of regional lymph node involvement. There was no association with HTLV-1 or HIV infection. The patient had a fulminant course and a poor response to chemotherapy.
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