24 research outputs found
STAT3 pathway is activated in ALK-positive large B-cell lymphoma carrying SQSTM1-ALK rearrangement and provides a possible therapeutic target
No full textALK-positive large B-cell lymphomas usually harbor clathrin (CLTC)-ALK rearrangement or, more rarely, nucleophosmin (NPM)-ALK fusion gene. Here we report a large B-cell lymphoma with a peculiar pattern of diffuse and cytoplasmic immunohistochemical staining and carrying sequestosome 1 (SQSTM1)-ALK rearrangement, identified by reverse transcription polymerase chain reaction analysis and Rapid Amplification of cDNA Ends analysis and confirmed by fluorescence in situ hybridization with specific dual-color fusion probes. The gene fusion product and the transcription factor STAT3 are both phosphorylated, and thereby the pathogenetic mechanism of this case shows important analogies with that of NPM-ALK and CLTC-ALK lymphomas, in which STAT3 plays a central role in the lymphomagenesis. Consequently, STAT3 inhibition provides a possible therapeutic target also for lymphomas with SQSTM1-ALK variant translocation
Ruolo del trascritto chimerico ETV6-NTRK3 nella diagnosi e prognosi del fibrosarcoma infantile congenito
No full textIntraoperative examination (IOE) in pediatric extracranial tumors
No full textBACKGROUND AND OBJECTIVES: Intraoperative evaluation of surgical specimens by frozen sections (IOE) is required to distinguish benign and malignant lesions, assess surgical margins, and determine sample adequacy of biopsies. In the last years, it has been used also for therapeutic decisions, particularly in children, who may need other ancillary procedures, in case of malignancies. Our purpose was the evaluation of diagnostic accuracy, limits, and different role of IOE in pediatric pathology.
PATIENTS AND METHODS: From 1990 to 2001, 416 IOEs were performed in 341 children, affected by lymph node pathology, soft tissue tumors, neuroblastic tumors, gonadal germ cell, and stromal tumors, hepatic lesions, renal tumors, and others; the technique was also used to assess surgical margins during major surgeries. IOEs were obtained from Tru-cut biopsies (<1 cm(3)), wide biopsies (>1 cm(3)), or from the whole lesions, and the subsequent final diagnoses were classified as conordant, discordant, and deferred.
RESULTS: Three hundred seventy cases (88.9%) were concordant, 10 (2.4%) discordant, and 36 (8.6%) deferred. The disagreement was found in two small lymph node samples, three soft tissue tumors, one hepatoblastoma, one metastasis, and three surgical margins. The deferred diagnoses were related to lymph node and soft tissue lesions.
CONCLUSIONS: IOE in pediatric oncology may integrate the diagnostic process and supports the therapeutic guidelines of different tumors. In our study, the diagnostic concordance was satisfactory. A rational use of the technique and the awareness of its limits are, however, prerequisites to avoid the risk of overtreatment
Ruolo del trascritto chimerico ETV6-NTRK3 nella diagnosi e prognosi del fibrosarcoma infantile congenito
No full text
Intraoperative examination (IOE) in pediatric extracranial tumors
No full textBACKGROUND AND OBJECTIVES: Intraoperative evaluation of surgical specimens by frozen sections (IOE) is required to distinguish benign and malignant lesions, assess surgical margins, and determine sample adequacy of biopsies. In the last years, it has been used also for therapeutic decisions, particularly in children, who may need other ancillary procedures, in case of malignancies. Our purpose was the evaluation of diagnostic accuracy, limits, and different role of IOE in pediatric pathology.
PATIENTS AND METHODS: From 1990 to 2001, 416 IOEs were performed in 341 children, affected by lymph node pathology, soft tissue tumors, neuroblastic tumors, gonadal germ cell, and stromal tumors, hepatic lesions, renal tumors, and others; the technique was also used to assess surgical margins during major surgeries. IOEs were obtained from Tru-cut biopsies (1 cm(3)), or from the whole lesions, and the subsequent final diagnoses were classified as conordant, discordant, and deferred.
RESULTS: Three hundred seventy cases (88.9%) were concordant, 10 (2.4%) discordant, and 36 (8.6%) deferred. The disagreement was found in two small lymph node samples, three soft tissue tumors, one hepatoblastoma, one metastasis, and three surgical margins. The deferred diagnoses were related to lymph node and soft tissue lesions.
CONCLUSIONS: IOE in pediatric oncology may integrate the diagnostic process and supports the therapeutic guidelines of different tumors. In our study, the diagnostic concordance was satisfactory. A rational use of the technique and the awareness of its limits are, however, prerequisites to avoid the risk of overtreatment
Three cases of pancreatic serous cystadenoma and endocrine tumour
No full textTo report three cases of serous cystadenoma and endocrine tumour in the same pancreas, to review the literature and to evaluate the clinicopathological features of the tumours. Cases: Three women (71, 57 and 31 years old) were admitted to hospital, two for diseases unrelated to the pancreas and the third for increasing obstructive jaundice in von Hippel-Lindau disease. Preoperative examination showed two distinct lesions in the first patient and only cystic lesions in the other two.
RESULTS:
Histological examination of the pancreas showed one serous oligocystic adenoma associated with a benign, well-differentiated endocrine tumour, one serous oligocystic adenoma associated with an endocrine microadenoma, and a von Hippel-Lindau-related cystic neoplasm with a well-differentiated endocrine carcinoma.
CONCLUSIONS:
Serous cystadenoma associated with endocrine tumour shows some clinicopathological differences with respect to the two tumours considered separately, and with respect to von Hippel-Lindau-related cases, although there is no convincing evidence at present to justify considering this association as a separate entity
Rituximab, Bendamustine and Cytarabine (R-BAC) in patients with relapsed-refractory aggressive B-cell lymphoma
No full textRituximab, bendamustine and cytarabine (R-BAC) in patients with relapsed-refractory aggressive B-cell lymphoma.Management of diffuse large B-cell lymphoma (DLBCL) after failure of front-line immunochemotherapy is a treatment challenge, without a clear optimal salvage strategy, especially for patients not candidate to autologous stem cell transplantation (ASCT). 1 Few patients achieve long-term disease control and no standard therapy exists. The British Columbia Cancer Agency reported a median progression-free survival (PFS) and overall survival (OS) of 2.1 and 3.9 months, respectively, in 326 patients who were not candidate for ASCT. 2 Among most widely used salvage regimens, the combination of rituximab and bendamus- tine (BR) was associated with overall response (OR) ranging from 32 to 63%, and PFS of 3-8 months. 3,4 When combined with cytara- bine, bendamustine has demonstrated significant synergistic activity in preclinical studies on DLBCL. 5 Thus, we conducted a pilot trial to explore the efficacy and tolerability of R-BAC (rituximab, bendamus- tine, and cytarabine) in a cohort of patients with B cell relapsed/ refractory (R/R) aggressive lymphoma
La malattia linfoproliferativa post-trapianto (PTLD): uno studio clinico-patologico sui riceventi di trapianto d'organo e di midollo osseo in eta' pediatrica
No full textXXX Congresso Nazionale AIEOP - Verona, 14-16 novembre 200
