Primary rare anaplastic large cell lymphoma, ALK positive in small intestine: case report and review of the literature

BACKGROUND: Primary anaplastic large cell lymphoma, ALK positive in small intestine is clinically rare and the clinical, radiological and pathological information are generally not available. Here, we report a case of 32-year-old male with ALK positive anaplastic large cell lymphoma at the junction of jejunum and ileum, and highlight the clinicopathological features and the differential diagnosis of this type lymphoma. CASE PRESENTATION: The patient presented with right middle abdominal mass for 1 month with sporadic pain. Computed tomography (CT) showed a mass measured 8.5 × 7.4 × 4 cm at the junction of jejunum and ileum. The diagnosis was made after pathological examination of the excised tissue by enterectomy. Grossly, the mass was located predominately in intestinal wall with grayish appearance and blurry boundary. Microscopically, almost all layers of the intestinal wall were infiltrated by pleomorphic tumor cells with diffuse and cohesive growth pattern. The neoplastic cells were mainly medium to large size with moderate basophilic cytoplasm. Most of them had hyperchromatic nuclei and prominent nucleoli. "Hallmark" cells were easily detected. Immunohistochemically, tumor cells are characterized by CD30, ALK, CD5, TIA-1, Granzyme B, EMA positive staining, and CD2, CD3, CD7, CD4, CD8, CD20, CD79a negative staining. The Epstein-Barr virus encoded RNAs (EBERs) genome was also negative. A diagnosis as primary small intestinal ALK positive anaplastic large cell lymphoma was finally made. The patient received CHOP chemotherapy and is alive till now without recurrence 5 months after enterectomy. CONCLUSIONS: Primary small intestinal ALK positive anaplastic large cell lymphoma is rare. The accurate diagnosis should be based on combined consideration of clinical characteristics, CT image and pathological features, and should be distinguished from other lymphomas or solid tumors in small intestine.