Background: Resectable adenocarcinomas in the pancreatic head, by definition "periampullary", originate from ampullary, duodenal, biliary, or ductal pancreatic epithelium. Typically, periampullary adenocarcinomas have either intestinal or pancreatobiliary type of differentiation, and the type of differentiation might be prognostically more important than the anatomic site of origin. The aim of the study was to determine whether the histologic type of differentiation is an independent prognostic factor in periampullary adenocarcinoma, and whether tumour origin predicts the prognosis in pancreatobiliary type carcinomas independently of resection margin involvement, tumour size, nodal involvement, perineural and vascular infiltration, and degree of differentiation.

Background: The retroperitoneal margin is frequently microscopically tumour positive in non-curative periampullary adenocarcinoma resections. This margin should be evaluated by serial perpendicular sectioning. The aim of the study was to determine whether retroperitoneal margin involvement independently predicts survival after pancreaticoduodenectomy within a framework of standardized assessment of the resected specimens.

We evaluated the correlation between histologic and cytologic specimens in the determination of estrogen receptor (ER) and progesterone receptor (PR) status in breast carcinoma and investigated the causes of clinically significant discrepancies. We analyzed 70 immunoassays for ER and 60 for PR from 71 patients with breast carcinoma. Concordance between cytology and histology was 89% for ER and 63% for PR using scores from pathology reports. Concordance between cytology and histology was 98% for ER and 91% for PR using consensus scores (obtained after reevaluation by the team of pathologists). Thirty of 130 (23%) tests had clinically relevant discrepancies, 53% of which were caused by wrong interpretation of cytologic findings, 10% by wrong interpretation of histologic findings, 17% by sampling error and 20% were not available for reevaluation. Wrong interpretation of the results for ER and PR status in cytology was a far more frequent cause of clinically relevant discrepancies than sampling errors. The use of strict criteria is recommended.

Background/Aim: Fusions of the ABL protooncogene 1 gene (ABL1 in 9q34) are common in leukemias but rare in solid tumors. The most notable is the t(9;22)(q34;q11)/BCR-ABL1 coding for a chimeric tyrosine kinase. We herein report an ABL1-fusion in a pediatric tumor. Materials and Methods: G-banding, fluorescence in situ hybridization, reverse transcription polymerase chain reaction and Sanger sequencing were performed on a soft tissue perineurioma found in the left musculus erector spinae of a child. Results: A der(4)t(4;9)(q31;q34) and a fusion of the GRB2 associated binding protein 1 (GAB1 in 4q31) gene with ABL1 were found. A literature search revealed 3 more cases with similar genetic and clinicopathological characteristics: a soft tissue perineurioma with t(2;9;4)(p23;q34;q31) and ABL1 rearrangement, a soft tissue angiofibroma with a GAB1-ABL1 chimeric gene, and a solitary fibrous tumor carrying a der(4)t(4;9)(q31.1;q34). Conclusion: GAB1-ABL1 is a recurrent fusion gene in benign pediatric tumors. The ABL proto-oncogene 1 gene (ABL1, previous symbol ABL) in chromosome band 9q34 is ubiquitously expressed and codes for a non-receptor tyrosine kinase which is localized at many subcellular sites including the nucleus, cytoplasm, mitochondria, and endoplasmic reticulum. It is involved in a variety of cellular processes such as cell division, adhesion, differentiation, and response to stress (1-3). ABL1 together with ABL2 (the proto-oncogene 2 gene which encodes a non-receptor tyrosine kinase and maps to chromosome band 1q25) constitute the ABL family of kinase genes (2, 4-6). Both ABL1 and ABL2 fuse with a variety of translocation partner genes in various hematological malignancies (7-10). The most notable fusion is between ABL1 and the 5' end of the breakpoint cluster region gene (BCR), located in 22q11, through the t(9;22)(q34;q11) chromosome translocation that gives rise to the Philadelphia chromosome in chronic myeloid leukemia (CML) (11, 12). The BCR-ABL1 fusion gene codes for a leukemogenic, constitutively active tyrosine kinase (11, 12). The discovery that 2-phenylaminopyrimidines inhibit the ABL protein kinase both in vitro and in vivo, led to the development of imatinib mesylate that now constitutes the first-line treatment of CML, as well as to introduction of other protein kinase inhibitors into cancer therapy (13-19). Rearrangements of the ABL1 and ABL2 genes in solid tumors have also been documented (3-6, 20). Phosphorylation and activation of ABL kinases were reported in various tumors such as breast and lung adenocarcinomas, melanomas, and cancers of the brain (3-6, 20). The mechanisms for activation of ABL1 and ABL2 kinases are in these settings not chromosome translocations/fusion genes but rather genomic amplification, increased expression of mRNA, enhanced protein expression, and increased catalytic activity (3-6, 20). Perineurioma is a tumor composed entirely of neoplastic perineurial cells with ultrastructural and immunohistochemical features similar to those of their normal counterparts (21). A...

We report the case of a newborn boy with multinodular NRAS and BRAF mutation-negative congenital melanocytic nevi and cerebral lesions compatible with congenital intraparenchymal melanosis. Histopathology from skin lesions showed atypical nodular melanocytic proliferation with marked melanocytic atypia and a large number of mitoses and apoptosis, indicating aggressive proliferation. The child developed several new subcutaneous tumors and multiple internal lesions, which were confirmed to be metastases, and died at 5 months of age. This case may represent an infantile melanoma developing from a giant congenital melanocytic nevus or a congenital melanoma.