title: Primary anaplastic large cell lymphoma of the small intestine.
abstract: We describe the clinicopathologic, immunophenotypic, and molecular findings in 4 cases of anaplastic large cell lymphoma (ALCL) arising in the small intestine. All patients were men with acute symptoms of gastrointestinal tract obstruction. The clinical preoperative diagnosis was gastrointestinal carcinoma in 3 cases, and pancreatic carcinoma in 1 case. Histologic examination revealed cohesive aggregates of neoplastic cells, with multiple vesicular nuclei, prominent nucleoli, and abundant amphophilic cytoplasm. There was no clinical or histopathologic evidence of enteropathy. All cases were CD30+, and all showed evidence of T-cell lineage with cytotoxic potential by expression of CD3, CD43, or CD45RO; T-cell intracellular antigen-1; or perforin. One tumor showed p80 and anaplastic lymphoma kinase (ALK) overexpression corroborated by the presence of the t(2:5). One tumor expressed Epstein-Barr virus latent membrane protein. In all cases, the tumor cells were negative for CD20, CD15, CD56, and cytokeratin. Polymerase chain reaction revealed clonal rearrangements of the T-cell receptor gamma-chain gene, without evidence of immunoglobulin heavy-chain gene rearrangement. The diagnosis of primary bowel ALCL is facilitated by immunophenotypic and molecular studies. With 24 months of clinical follow-up, only the patient with the t(2:5)-positive tumor is alive and free of disease, suggesting that p80/ALK overexpression may be a good prognostic indicator.
authors.affiliation: Department of Pathology, University of Utah Health Sciences Center, Salt Lake City 84132, USA.
authors.forename: M J
authors.initials: MJ
authors.lastname: Carey
authors.forename: L J
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: J E
authors.initials: JE
authors.lastname: Roepke
authors.forename: C R
authors.initials: CR
authors.lastname: Kjeldsberg
authors.forename: K S
authors.initials: KS
authors.lastname: Elenitoba-Johnson
mesh_terms: D000328:Adult; D000368:Aged; D000951:Antigens, Neoplasm / Q000032:analysis; D000956:Antigens, Viral / Q000235:genetics; D003937:Diagnosis, Differential; D004379:Duodenal Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000821:virology; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007124:Immunoenzyme Techniques; D016130:Immunophenotyping; D017403:In Situ Hybridization; D007580:Jejunal Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000821:virology; D017730:Ki-1 Antigen / Q000032:analysis; D017728:Lymphoma, Large-Cell, Anaplastic / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D012367:RNA, Viral / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014763:Viral Matrix Proteins / Q000235:genetics / Q000302:isolation & purification
chemical_list: D000951:Antigens, Neoplasm; D000956:Antigens, Viral; C037662:EBV-associated membrane antigen, Epstein-Barr virus; D017730:Ki-1 Antigen; D012367:RNA, Viral; D014763:Viral Matrix Proteins