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Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma (ALK+LBCL) is a rare, distinct and aggressive entity defined as a neoplasm of ALK-positive monomorphic large immunoblastic-like B cells.1 The tumour cells are considered to be derived from post-germinal centre B cells with plasmablastic differentiation and frequently show a cytoplasmic granular pattern of ALK.1 Nodal (most commonly to be cervical nodes) and extranodal presentation (including bone, head and neck, liver and spleen, gastrointestinal tract) have been reported.2 Here we reported a case of intestinal ALK+LBCL with systemic involvement mimicking the epithelioid variant of inflammatory myofibroblastic tumour (epiIMT).
A 46-year-old woman presented in March 2014 with repeated episodes of melaena for 1 month associated with general weakness, vomiting, body weight loss and pitting oedema of bilateral lower limbs. At the Emergency Department, ileus and anaemia (haemoglobin at 6.4 g/dL) were noted, and chest X-ray revealed a mass in the right lower lobe of lung. CT scans of chest and abdomen showed a 7 cm tumour in the right lower lobe of lung, a 3 cm nodule in the abdominal wall and a 12 cm polypoid tumour in the jejunum with adhesion to sigmoid colon (figure 1). She underwent resection of the tumours in the jejunum and abdominal wall. The upper jejunal tumour was located 40 cm distal to Treitz ligament with …