Peripheral T cell lymphoma, not otherwise specified: the stuff of genes, dreams and therapies.

Peripheral T cell lymphomas (PTCL) account for about 12% of lymphoid tumours worldwide. Almost half show such morphological and molecular variability as to hamper any further classification, and to justify their inclusion in a waste-basket category termed "not otherwise specified (NOS)". The latter term is used for neoplasms with aggressive presentation, poor response to therapy and dismal prognosis. In contrast to B cell lymphomas, PTCL have been the subject of only a limited number of studies to elucidate their pathobiology and identify novel pharmacological approaches. Herewith, the authors revise the most recent contributions on the subject based on the experience they have gained in the extensive application of microarray technologies. PTCL/NOS are characterised by erratic expression of T cell associated antigens, including CD4 and CD52, which have recently been proposed as targets for ad hoc immunotherapies. PTCL/NOS also show variable Ki-67 marking, with rates >80% heralding a worse prognosis. Gene expression profiling studies have revealed that PTCL/NOS derive from activated T lymphocytes, more often of the CD4+ type, and bear a signature composed of 155 genes and related products that play a pivotal role in cell signalling transduction, proliferation, apoptosis and matrix remodelling. This observation seems to pave the way for the use of innovative drugs such as tyrosine kinase and histone deacetylase inhibitors whose efficacy has been proven in PTCL primary cell cultures. Gene expression profiling also allows better distinction of PTCL/NOS from angioimmunoblastic T cell lymphoma, the latter being characterised by follicular T helper lymphocyte derivation and CXCL13, PD1 and vascular endothelial growth factor expression.

Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients.

Myeloid sarcoma (MS) is a rare neoplasm whose knowledge is largely based on case reports and/or technically dated contributions. Ninety-two MSs in adulthood with clinical data available were evaluated both morphologically and immunohistochemically. Seventy-four cases were also studied by fluorescent in situ hybridization on tissue sections and/or conventional karyotyping on bone marrow or peripheral blood. Histologically, 50% of the tumors were of the blastic type, 43.5% either monoblastic or myelomonocytic and 6.5% corresponded to different histotypes. CD68/KP1 was the most commonly expressed marker (100%), followed by myeloperoxidase (83.6%), CD117 (80.4%), CD99 (54.3%), CD68/PG-M1 (51%), CD34 (43.4%), terminal-deoxy-nucleotidyl-transferase (31.5%), CD56 (13%), CD61/linker for activation of T cells (2.2%), CD30 (2.2%) and CD4 (1.1%). Foci of plasmacytoid monocyte differentiation were observed in intestinal cases carrying inv16. Chromosomal aberrations were detected in about 54% of cases: monosomy 7(10.8%), trisomy 8(10.4%) and mixed lineage leukemia-splitting (8.5%) were the commonest abnormalities, whereas t(8;21) was rare (2.2%). The behavior was dramatic irrespective of presentation, age, sex, phenotype and cytogenetics. Most if not all, long survivors received bone-marrow transplantation. The present report expands the spectrum of our knowledge showing that MS has frequent monoblastic/myelomonocytic differentiation, displays distinctive phenotypic profile, carries chromosomal aberrations other than t(8;21), and requires supra-maximal therapy.

[Aneurysmal bone cysts of the nasal cavity. Description of a case and review of the literature]. / Cisti ossea aneurismatica delle cavità nasali. Descrizione di un caso e revisione della letteratura.

INTRODUCTION: Aneurysmal bone cyst (ABC) are benign tumor-like lesions of the bone that occur at any age with a slight female predominance. The most common sites are long bones and vertebrae bodies. MATERIALS AND METHODS: We report a case of 54 year-old man with nasal respiratory difficulty and a previous history of nasal fracture. RMN and TC-scan showed a voluminous nasal lesion, which was surgically removed. Tissue sample was formalin fixed (10%) and routinely processed to paraffin after decalcifying treatment (Osteodec, Bioptica). Section were stained with haematoxilin-eosin. RESULTS: At histological level the lesion was characterized by multilocular cyst-like spaces filled with erythrocytes separated by connective septa lacking the smooth muscle walls and endothelial lining of normal vessels. Histological features are consistent with a diagnosis of ABC. DISCUSSION: We report a case of ABC of nasal cavity. The previous history of nasal fracture and the fact that pre-existing or concurrent were not found lead us to suppose a relationship between the fracture and ABC. Teleangiectatic osteosarcoma, fibrous dysplasia and giant-cell tumor were excluded according to clinical and morphological features.