LETTER TO THE EDITOR Extramedullary myeloid tumour of the stomach and duodenum presenting without acute myeloblastic leukemia: A diagnostic and therapeutic challenge ENRICO DERENZINI 1 , STEFANIA PAOLINI 1 , GIOVANNI MARTINELLI 1 , CRISTINA CAMPIDELLI 2 , GIAN LUCA GRAZI 3 , CARLO CALABRESE 4 , PIER LUIGI ZINZANI 1 , & MICHELE BACCARANI 1 1 Institute of Haematology and Medical Oncology L.A. Seragnoli, University of Bologna, Bologna, Italy, 2 Unit of Haematopathology, University of Bologna, Bologna, Italy, 3 Department of General Surgery, University of Bologna, Bologna, Italy, and 4 Department of Internal Medicine, University of Bologna, Bologna, Italy (Received 2 September 2007; revised 17 September 2007; accepted 20 September 2007) Extramedullary myeloid tumours (EMMTs) are haematological tumours of malignant myeloid cells arising in extramedullary sites. The occurrence of EMMT in patients without leukemia is rare and in the majority of cases, AML develops within 2 years of diagnosis (average 10.5 m) [1,2]. Common sites of onset are the bone, periosteum lymphnodes and skin, while GI involvement is rare, and still rarer is the isolated gastro-doudenal localization, there being only a few cases reported in the literature (less than 20 cases described) [3]. The prognosis with GI EMMT is poor, particularly because of the high rate of early complications such as bleeding and perfora- tion (Table I). We report the case of a 40-year-old man whose medical history was unremarkable. In December 2004 the patient began to complain of dyspeptic symptoms with nausea, anorexia and abdominal pain to the upper quadrants, accompanied by fever, sweating by night and pruritus. After an initial misdiagnosis of gastric MALT lymphoma and ineffective antibiotic therapy, the patient came to our centre where another EGDS showed thickening of the gastric wall with increased density of the gastric plicas at fundum and body level (Figure 1). Multiple biopsies revealed a diffuse infiltration by large-sized neoplastic cells, with dispersed chromatin, evident nucleoli and a variably basophilic cytoplasmic rim. Immunostains showed an intense reactivity to CD34 þ , myeloperoxidase (MPO), CD117/CKIT and KI67/MIB1 (90%) (Figure 1). A diagnosis of EMMT was made with no evidence of blood or bone marrow infiltration (bone marrow aspiration and biopsy were normal, with a 46 XY karyotype). Laboratory examination showed haemoglobin con- centration 11.3 g/dL, white blood cell count 5.5610 9 /mL, platelets 309610 9 /mL, total/direct bilirubin 3.4/3.04 mg/dL, alkaline phosphatase 2500 U/L, gGT 329 U/L, AST 93 U/L, ALT 249 U/L, albumin 3.3 g/dL, PT 76%, aPTT 0.98, INR 1.18, fibrinogen 718 mg/dL, erythrocyte sedi- mentation rate 70 mm/h, PCR 14.1 mg/L, HbsAg and anti-HCV negative. The CT scan showed a diffuse thickening of the gastric and duodenal wall, with evidence of intrahepatic and extrahepatic biliary duct dilatation (Figure 1), and the PET scan showed that the stomach and duodenum were the only site of the disease (SUV max ¼ 11). The patient received combination induction chemotherapy for acute myeloid leukemia (Ara-C 3200 mg/mq/die on days 1 – 5, etoposide 160 mg/mq/die on days 1 – 5 and idarubicine 10.5 mg/mq/die on days 1, 3 and 5) and parenteral nutrition was started to prevent GI bleeding. During hospitalisation the clinical condi- tion of the patient progressively improved, with disappearance of the epigastric pain and dyspeptic Correspondence: Enrico Derenzini, Institute of Haematology and Medical Oncology L.A. Seragnoli, University of Bologna, Bologna, Italy. Tel: þ390516363680. Fax: þ390516364037. E-mail: enrico.derenzini@libero.it Leukemia & Lymphoma, January 2008; 49(1): 159 – 162 ISSN 1042-8194 print/ISSN 1029-2403 online Ó 2008 Informa UK Ltd. DOI: 10.1080/10428190701704621 Leuk Lymphoma Downloaded from informahealthcare.com by D S Diffusioni Scientifiche - Unical on 12/02/10 For personal use only.