Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of leukemia/lymphoma, whose diagnosis can be difficult to achieve due to its clinical and biological heterogeneity, as well as its overlapping features with other hematologic malignancies. In this study we investigated whether the association between the maturational stage of tumor cells and the clinico-biological and prognostic features of the disease, based on the analysis of 46 BPDCN cases classified into three maturation-associated subgroups on immunophenotypic grounds. Our results show that blasts from cases with an immature plasmacytoid dendritic cell (pDC) phenotype exhibit an uncommon CD56- phenotype, coexisting with CD34+ non-pDC tumor cells, typically in the absence of extramedullary (e.g. skin) disease at presentation. Conversely, patients with a more mature blast cell phenotype more frequently displayed skin/extramedullary involvement and spread into secondary lymphoid tissues. Despite the dismal outcome, acute lymphoblastic leukemia-type therapy (with central nervous system prophylaxis) and/or allogeneic stem cell transplantation appeared to be the only effective therapies. Overall, our findings indicate that the maturational profile of pDC blasts in BPDCN is highly heterogeneous and translates into a wide clinical spectrum -from acute leukemia to mature lymphoma-like behavior-, which may also lead to variable diagnosis and treatment.

Osteoma Osteóide Cervical - Radiofrequência ou a Clássica Curetagem?

Os Osteomas Osteóides são tumores benignos, osteoblásticos, cuja incidência ronda os 3% do total de todos os tumores. Ocorrem mais frequentemente no fémur e tíbia, e o esqueleto axial é afetado em apenas 10% dos casos, maioritariamente ao nível da coluna lombar (56,1%), sendo a coluna cervical afetada em apenas 26,8% dos casos. O clássico tratamento cirúrgico está a ser substituído por técnicas ablativas mini-invasivas, como a termoablação por radiofrequência por via percutânea. No entanto, na coluna cervical, torna-se difícil prever o efeito do calor quando aplicado na proximidade de estruturas nervosas e vasculares. Apresenta-se o caso clínico de uma doente do sexo feminino de 24 anos, observada por cervicalgia persistente, cuja tomografia computorizada realizada demonstrou um Osteoma Osteóide localizado a nível do pedículo esquerdo da 6ª vértebra cervical. A doente foi submetida a tratamento cirúrgico, através de curetagem transpedicular guiada por fluoroscopia, tendo sido obtida uma resolução imediata, completa e sustentada da dor.