Paediatric cutaneous lymphomas including rare subtypes: A 40-year experience at a tertiary referral centre.

BACKGROUND: Primary cutaneous lymphomas are neoplasms of the immune system with a distinct tropism for the skin and an absence of extracutaneous manifestations at the time of diagnosis. Studies focusing on cutaneous lymphomas in children and adolescents remain scarce and often do not encompass the rare subtypes. OBJECTIVES: To address this knowledge gap by describing the clinical, histological and molecular characteristics of a large group of paediatric patients affected by primary cutaneous lymphoma. We also provided the Paediatric Primary Cutaneous Lymphoma Atlas that illustrates the clinicopathological spectrum of observed presentations, in the hope of supporting other physicians in the diagnostic process. METHODS: Retrospective chart review of paediatric patients diagnosed with primary cutaneous lymphomas between 1980 and 2022 at the Paediatric Dermatology Unit of Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan. RESULTS: A total of 101 patients (58 males, 43 females) met the inclusion criteria. The most common subtypes were lymphomatoid papulosis (n = 48) and mycosis fungoides (n = 31). These were followed by primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders (n = 7), primary cutaneous anaplastic large-cell lymphomas (n = 5), primary cutaneous marginal zone B-cell lymphomas (n = 3), primary cutaneous follicle centre lymphomas (n = 2), subcutaneous panniculitis-like T-cell lymphomas (n = 2), primary cutaneous peripheral T-cell lymphoma not otherwise specified (n = 1), primary cutaneous precursor B-lymphoblastic lymphoma (n = 1) and Sézary syndrome (n = 1). Clinical follow-up data covering a median of 70.8 months (range 1-324) were available for 74 patients, of whom three died due to cutaneous lymphoma. CONCLUSIONS: Our findings shed light on the peculiar aspects and long-term outcomes of paediatric cutaneous lymphomas, particularly emphasizing their distinctive features in comparison to their adult counterparts and exploring the less common subtypes. Further larger-scale studies are warranted to better characterize these entities and to achieve a more rapid and accurate diagnosis.

Prognostic evaluation in palliative care: final results from a prospective cohort study.

Prognostic characterization in the initial assessment of patients with advanced cancer disease is an essential step to plan the most appropriate therapeutic program. Since clinical prediction of survival (CPS) may be of limited value, some authors have tried to integrate specific prognostic factors into prognostic multidimensional scores. We carried out a prospective cohort study in two palliative care units to compare the accuracy of the Palliative Prognostic (PaP) Score, the Objective Prognostic Score (OPS), and the Palliative Prognostic Index (PPI). In addition, we compared the accuracy of the CPS independently estimated by different healthcare professionals and we tested the role of laboratory results, together with clinical and social factors in predicting survival. Clinical and laboratory data of 334 advanced cancer patients were prospectively collected from the time of in-hospital admission. PaP Score was the most accurate index of survival prediction, followed by PPI; CPS estimates' accuracy was similar among physicians and nurse. All healthcare professionals tended to underestimate the real survival. Integrating CPS with multidimensional indexes may further improve the patient's management. The degree of autonomy and the number of metastatic sites were independent prognostic factors for 30-days mortality and overall survival in multivariate analysis.

Sézary Syndrome in a 17-Year-Old Boy: Clinicopathologic Features and Genomic Profile.

We describe the case of a 17-year-old Hispanic boy who had had erythroderma and diffuse lymphadenopathy for approximately 6 months. A diagnosis of Sézary syndrome was made on the basis of the histologic features of the skin; the presence of the same T-cell clone on the skin, blood, and bone marrow; and the high CD4(+) lymphocyte count with an aberrant phenotype in peripheral blood; bone marrow involvement was also present. The patient was treated with systemic gemcitabine and achieved partial remission.

Primary Cutaneous Gamma-Delta T Cell Lymphomas: A Case Series and Overview of the Literature.

Primary cutaneous gamma-delta T cell lymphomas (PCGDTCLs) are rare and aggressive cutaneous malignancies that have been diagnostically challenging for dermopathologists and clinicians since their first published descriptions in 1991. Since then, the availability of immunostaining for T cell receptors γ and δ in formalin-fixed paraffin-embedded samples has greatly increased our knowledge of the gamma-delta phenotype by showing that it may also be present in the context of indolent entities, such as mycosis fungoides (MFs) and lymphomatoid papulosis, and this has raised questions concerning its diagnostic and prognostic implications. We here describe the histological and clinical differences between the dermo-epidermal and subcutaneous sub-groups of PCGDTCL observed in a cohort of 20 patients attending a single experienced centre, with particular focus on cases with an MF-like presentation, which are still less well defined than those of classic MF.

Adopting integrated care pathways in non-small-cell lung cancer: from theory to practice.

INTRODUCTION: Integrated care pathways (ICPs) have been proposed as effective strategies for quality improvement. To date, limited data are available that detail the methodology to design an optimal care pathway for patients with non-small-cell lung cancer (NSCLC). The main aim of this study was to assess the quality of health care delivered to lung cancer patients referred to a hub university hospital. METHODS: All professionals involved with the management of NSCLC patients, in cooperation with health care researchers, identified 11 quality indicators and associated benchmarks. These were used to estimate the quality and efficiency of health care delivered to a cohort of 175 NSCLC patients. RESULTS: The gap between "desired" and "actual" performance has been measured by benchmarking current practice against key quality indicators. Diagnostic workup, multidisciplinary team care and medical treatment of advanced disease have emerged as areas of good performance. Conversely, the management of early-stage disease offers room for improvement, in terms of both accuracy of nodal staging and surgical timeliness. CONCLUSIONS: Analyzing the process of caring for NSCLC patients is feasible and offers room for improvement. Acquired knowledge may be shared with hospital administrators, guide the revision of ICPs, and enable the delivery of consistent, high-quality clinical standards.

EGFR pathway in advanced non-small cell lung cancer.

Chemotherapy is the standard of care for patients with advanced stage non-small cell lung cancer (NSCLC) because of the a modest improvement in survival and quality of life but its efficacy has already reached a plateau. Several molecular targets involved in the uncontrolled growth of lung cancer cells has been recently discovered and Epidermal Growth Factor Receptor (EGFR) pathway is as a key therapeutic target. Strategies to block such pathway include tyrosine kinase inhibitors (TKIs) and monoclonal antibodies. Erlotinib and gefitinib are two EGFR-TKI active against NSCLC. Their efficacy has been proven to be definitively superior in presence of activating EGFR mutation in the tumor. This evidence does not apply to the monoclonal antibody cetuximab, which efficacy in NSCLC was recently demonstrated in a single phase III study. The good tolerability profile of EGFR inhibitors make these agents suitable for maintenance and adjuvant setting, while sequencing of EGFR-TKIs and chemotherapy seems to be preferred. This article reviews the role of EGFR inhibitors focusing mainly on compounds in phase III clinical development.

A randomised phase II study of docetaxel/oxaliplatin and docetaxel in patients with previously treated non-small cell lung cancer: an Alpe-Adria Thoracic Oncology Multidisciplinary group trial (ATOM 019).

INTRODUCTION: To date, no combination regimen has proven superior to single agent chemotherapy as a second-line treatment for non-small cell lung cancer (NSCLC). METHODS: This multicenter, non-comparative randomised phase II trial evaluated the activity of docetaxel (75 mg/m(2) on day 1) with oxaliplatin (70 mg/m(2) on day 2) every 3 weeks in previously treated NSCLC patients; the reference arm was single-agent docetaxel (75 mg/m(2) on day 1 every 3 weeks). It was designed as a one-stage, three-outcome phase II trial; 21 evaluable patients were required in each arm. The primary end-point was response rate; secondary end-points were toxicity, progression free survival (PFS) and overall survival. RESULTS: Fifty patients were enrolled. Patient characteristics included male/female, 76/24%; median age 62 years; ECOG PS 0/1, 36/64%; previous platinum-based chemotherapy, 98%. Partial response was seen in 20% and 8%, stable disease in 52% and 32%, of patients treated with docetaxel/oxaliplatin and docetaxel, respectively. Main grade 3-4 toxicities were neutropenia 56% and 64%; febrile neutropenia 4% and 8%; diarrhoea 12% and 4% for docetaxel/oxaliplatin and docetaxel, respectively. Median PFS was 5.0 and 1.7 months, median survival 11.0 and 7.1 months, and 1-year survival 44% and 32% for docetaxel/oxaliplatin and docetaxel, respectively. CONCLUSIONS: The study met its pre-defined study end-point; docetaxel/oxaliplatin and more generally platinum-containing doublets warrant further evaluation as second-line therapy for patients with NSCLC.