Anaplastic Ki-1-positive Large Cell Lymphoma of the Pancreas: a Case Report and Review of the Literature
Anaplastic Ki-1-positive Large Cell Lymphoma of the Pancreas: a Case Report and Review of the Literature HiroshiMaruyama1, NaoyukiNakatsuji2, SeiichiSugihara2, MasahideAtsumi3, KazuhikoShimamoto3, KunioHayashi3, MasahiroTsutsumi4 and YoichiKonishi4
Departments of 1Pathology, 2Surgery and 3Internal Medicine, Hoshigaoka Koseinenkin Hospital, Hirakata, Osaka; 4Department of Oncological Pathology, Cancer Center, Nara Medical University, Kashihara, Nara, Japan
A case of Ki-1-positive anaplastic large cell lymphoma of the pancreas is presented. The patient complained of abdominal pain and was jaundiced. Examination of a biopsy specimen obtained by duodenal endoscopy revealed malignant lymphoma, and surgery confirmed a large mass located in the region from the intra-pancreatic tissue around the lower common bile duct to the peri-pancreatic lymph nodes. Histologically, this tumor was composed mainly of large and giant neoplastic cells. Immunohistochemically, these cells were diffusely positive for Ki-1 and CD45RO antigens, indicating the features of Ki-1 anaplastic large cell lymphoma with a T-cell phenotype among non-Hodgkin's lymphoma. The histologic types of the majority of malignant lymphomas of the pancreas reported previously were considered to be diffuse-type non-Hodgkin lymphoma (probably with predominance of the B-cell phenotype), except for a single Japanese lymphoma case with a T-cell phenotype. This is therefore the first known case of Ki-1 anaplastic large cell lymphoma of the pancreas.
Key words: pancreas - malignant lymphoma - Ki-1 antigen - anaplastic large cell lymphoma - surgery
Malignant lymphomas arising primarily in the pancreas, involving only the pancreatic parenchyma and/or peri-pancreatic lymph nodes, are rare, constituting less than 0.7% of all pancreatic malignancies (1) and 1% of non-Hodgkin's lymphomas (2). Ki-1-positive anaplastic large cell lymphoma (Ki-1 ALCL) is an uncommon type of non-Hodgkin's malignant lymphoma first described by Stein et al. (3) in 1985. Since then, following the description in the updated Kiel classification (4) of 1988, the criteria for this tumor have been added to the recently published Revised European-American Classification of Lymphoid Neoplasms (REAL classification) (5). Morphologically, this tumor is composed mainly of large blastic cells, often with horseshoe-shaped or multiple nuclei and multiple or single prominent nucleoli. Moreover, the tumor cells grow in a cohesive pattern and often preferentially involve the lymph node sinuses, as well as extranodal sites such as soft tissue, bone and skin.
Here we report the first known case of Ki-1 ALCL of the pancreas and review the literature concerning pancreatic lymphomas.
A 46-year-old woman was admitted to the Medical Department of Hoshigaoka Koseinenkin Hospital in May 1994, complaining of right lumbar back pain and anorexia of one week's duration. Abdominal ultrasonography revealed a hypoechoic mass in the pancreatic head with a mildly dilated common bile duct (CBD). Physical examination demonstrated no superficial lymphadenopathy or anemia, but icteric conjunctivae, a flat and soft abdomen, and mild tenderness in the right upper quadrant. The RBC count was 479 * 104/mm3 and Hb level 12.0 g/dl. The WBC count was 5900/mm3 with a normal lymphocyte percentage (23%). Aspiration of bone marrow cells revealed no abnormality (nucleocyte count: 30.7 * 104/mm3). The level of total protein was 6.7 g/dl. The total bilirubin level was 4.5 mg/dl (normal range 0.2-1.0 mg dl) with a direct bilirubin volume of 2.9 mg/dl. Other values included GOT 110 IU, GPT 182 IU, alkaline phosphatase 26.3 King-Armstrong units (KAU) (normal range 2-10 KAU), LAP 441 Goldbarg-Rutenberg units (GR), [gamma]-GTP 145 IU/L and cholinesterase of 0.57 [Delta]pH. All these were abnormal, suggesting obstructive jaundice. Serum amylase was low at 100 IU (normal range 150-400) and urinary amylase was normal at 644 IU (normal range 100-1000). Although the serum levels of carbohydrate antigen 19-9, CEA, AFP and elastase 1 were all within normal limits at 6 U/ml, 1.7 ng/ml, 1 ng/ml and 270 U/ml respectively, serum carbohydrate antigen 125, normal value <35, was mildly elevated at 40 U/ml. Serum was negative for HTLV-1 antibody.
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