Myeloid and lymphoid neoplasms with fibroblastic growth factor receptor-1 (FGFR1) abnormalities originate from mutated pluripotent stem cells and have a heterogeneous clinical presentation. There are 12 identified partner genes commonly involved in FGFR1 translocation at an 8p11 breakpoint. In FGFR1-related neoplasms, T-lymphoblastic lymphoma with eosinophilia is the most common clinical scenario, whereas acute B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) is rare. To date, only 7 cases of B-ALL/LBL with FGFR1 abnormalities have been reported. Here, we report an additional case of a 64-year-old gentleman with leukocytosis, eosinophilia and diffuse mediastinal and general lymphadenopathy. Bone marrow examination showed patchy infiltrates of immature precursors/blasts, along with myeloid/eosinophilic hyperplasia. Immunophenotyping confirmed increased B lymphoblasts (30-40%). Karyotyping revealed cytogenetic abnormalities, including t(8;13)(p11;q12)/ZMYM2 (ZNF198)-FGFR1 and trisomy 21. The patient did not respond to hyper-CVAD chemotherapy and within 4 months developed acute myelomonocytic leukemia and expired 11 months after the initial diagnosis. Similar cases from the literature are reviewed.
A 68-year-old woman with a history of follicular lymphoma had pathological findings of grade 3B follicular lymphoma, mantle cell lymphoma (MCL), and diffuse large B-cell lymphoma (DLBCL) identified in 1 lymph node. The DLBCL appeared to be a transformation of the follicular lymphoma. The nodules were diffusely and strongly positive for CD20, BCL6, and BCL2. CD43 highlighted smaller lymphocytes in a fraction of the nodules. BCL1 staining was variable with a mixture of nodular and mantle zone patterns. The diffuse areas showed weaker positivity for CD10, BCL2, and BCL6. CD3 and CD5 highlighted intermixed T cells. The Ki-67 proliferative index was overall estimated to be 60%. Fluorescent in situ hybridization performed on the lymph node was positive for CCND1/IGH. The patterns of BCL1 and BCL6 staining demonstrated 2 separate populations of neoplastic B lymphocytes.
Adenocarcinoma arising from esophageal submucosal gland duct (ESGD) is a rare entity. We found several reports of esophageal adenomas originating from these glands, but there is only one case report in the literature describing adencarcinoma arising from ESGD. Here, we report the histomorphological patterns of 10 cases of esophageal submucosal gland duct adenocarcinoma. In our collection we found 2 major histological patterns. One pattern was characterized by infiltrating dilated glands lined by flattened eosinophilic epithelium with dysplastic cells; occasionally displaying central necrosis. This pattern had a "swiss cheese"• appearance at low power. The second pattern was characterized by dilated glands lined by eosinophilic dysplastic cells exhibiting a "serrated lumen•." In some cases however both patterns were present simultaneously. In all cases the tumor glands were associated with underlying submucosal gland ducts. We believe that identification of this type of esophageal adenocarcinoma is important to avoid overestimation of the frequency of Barrett's-associated adenocarcinoma in the population. Additional studies are warranted to further characterized the immunohistochemical profile of this entity.
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