Biopsy diagnosis of CD30-positive anaplastic large T-cell lymphoma of ileum in necrotic biopsies

Abstract

CD30 (Ki-1)-positive anaplastic large cell lymphoma (ALCL) has been reported only once in ileum. A 72-year-old presented with melena for 3 weeks duration. Endoscopy identified a large ulcerated tumor (3 cm × 4 cm × 4 cm) in ileum proximal to Bauhin’s valve. Punch biopsies showed broad coagulative necrosis without apparently viable cells, but cell contours were recognizable. Diagnosis was impossible on HE-section, but it suggested malignancy. Since the patient denied second endoscopy, the author managed to make the diagnosis of necrotic punches. Immunohistocheimcally, the coagulative necrosis cells were positive for vimentin, CD45, CD3, CD4, CD5, CD10, CD15, CD30, CD43, CD45RO, p53, and Ki-67 (labeling = 78%). They were negative for cytokeratin (CK) AE 1/3, CK CAM5.2, CK5, CK6, CK7, CK8, CK18, CK19, CK20, EMA, p63, CD8, CD20, CD38, CD138, CD23, CD79α, κ-chain, λ-chain, bcl-2, bcl-6, cyclinD1, CD56, CD57, TdT, chromogranin, synaptophysin, NSE, S100 protein, smooth muscle actin, MUC1, MUC2, MUC5AC, MUC6, KIT and PDGFRA. Since CD30 and CD15 were strongly positive, Hodgkin’s disease and ALCL were considered. However, Hodgkin’s disease was unlikely because no apparent Hodgkin’s or Reed-Sternberg cells were seen. Therefore, CD30-positive ALCL was diagnosed. In addition, because the tumor cells expressed only T-cell markers, the diagnosis of CD30-positive T-cell ALCL was confirmed. The present study could not detect ALK, ALK/NPN, ALK-1, p80, and tissue destructive molecules such as perforins, TIA-1, and granzyme B because tumor cells were totally necrotic. Chromosomal translocation of t (2; 5) and TCR rearrangement could not be performed due to no viable cells. Post-diagnosis whole body examinations using CT, MRI, and PET showed bladder metastasis and multiple lymph node swellings in the para-aortic areas (Ann Arber Stage 4). The patient is now treated by chemotherapy and radiation for 5 months. The present study showed a very rare case of ileal CD30-positive T-cell ALCL and indicated that lymphoma diagnosis is possible even in coagulatively necrotic tissues.