Thirty-six tumors from 33 patients (mean age: 60.4, range: 26 to 88; 17 men and 16 women) were studied. Three patients had bilateral tumors and 1 patient had von Hippel-Lindau disease. Follow-up was available in 60% (20/33) of the patients for a mean of 27.4 (range 1 to 85) months. No patient had evidence of the disease after surgery except for the patient with von Hippel-Lindau disease, who was alive with stable disease in the contralateral kidney. All 36 tumors were small (mean size 2.4 cm; range 0.9 to 4.5 cm) and low stage (pT1). The majority was cystic and had prominent fibrous capsule and stroma. The tumors were composed of variable amount of cysts, papillae,
tubules, acini, and solid nests. The most characteristic histologic features were branching tubules and acini and anastomosing clear cell ribbons
with low-grade nuclei. All tumors were strongly positive for selleck products CK7 and variably positive for CA9, but largely negative for CD10, and negative for alpha-methylacyl-CoA racemase and TFE-3. All but 1 tumor had no gains of GTPL8918 chromosomes 7 and 17 and deletion of 3p. Only 1 tumor had low copy number gains of chromosomes 7 and 17. VHL gene mutation and promoter methylation were negative in 2 tumors analyzed. We show that these tumors, which we term as “clear cell tubulopapillary renal cell carcinoma,” constitute a unique subtype in the spectrum of renal epithelial neoplasia based on their characteristic morphologic and immunohistochemical features.”
“Adult lymphoblastic lymphoma (LBL) is
an aggressive form of non-Hodgkin lymphoma occurring in predominantly adolescent and young adult men. Lymphoblastic lymphoma is rare, accounting for 1% to 2% of all non-Hodgkin lymphomas and is of T-cell phenotype in 90% of cases. Lymphoblastic lymphoma is morphologically indistinct from acute lymphoblastic leukemia (ALL). Both express their lineage-specific markers as well as terminal deoxynucleotidyl transferase. The differences are often made on clinical grounds. Lymphoblastic lymphoma is characterized by a predominantly nodal distribution of disease, often with a large mediastinal mass. Patients with less than 25% bone marrow involvement have typically been categorized as LBL rather than ALL, although this has not been applied consistently in the literature. Gene expression studies have identified differences in gene expression, 17DMAG with LBL expressing higher levels of genes associated with cytoskeleton, adhesion, angiogenesis, and chemotaxis than ALL. Although LBL and ALL can be distinct clinically, chemotherapy strategies are often very similar. Acute lymphoblastic leukemia regimens, which incorporate intensive multidrug induction, consolidation, delayed intensification, and maintenance, have been shown to be superior to standard lymphoma regimens. As central nervous system (CNS) relapse is common, CNS prophylaxis with high-dose chemotherapy and intrathecal therapy is also standard.