primary cutaneous cd30 anaplastic large cell lymphoma in a heart transplant patient case report and literature review

Solid organ transplant recipients are at risk of develop ing a wide range of viral-associated malignancies, in cluding skin tumours and lymphoproliferative disorders. The risk of a post-transplant lymphoproliferative disorder is 28–49 times the risk of a lymphoproliferative disorder in the normal population. Most cases are of B-cell phenotype and are associated with Epstein-Barr virus infection. Post-transplant lymphoproliferative disorders presenting clinically in the skin are rare and usually of B-cell phenotype. Only rare cases of cutaneous T-cell post-transplant lymphoproliferative disorder have been reported previously, mostly mycosis fungoides type. We describe here a rare primary cutaneous T-cell lymphoma CD30+ arising in a heart transplant patient who had a nodule on the right leg, several years after heart transplantation. The morphology and immunohistochemical findings were consistent with a CD30+ anaplastic large cell lymphoma with a T-cell phenotype. Excisional biopsy and radiotherapy of the affected area were performed. In this patient, the presence of a solitary lesion and th

Magnetic resonance imaging assessment of lipomatous soft-tissue tumors

Aim: To establish the accuracy of magnetic resonance imaging (MRI) in distinguishing between benign and malignant lipomatous tumors; to evaluate the reproducibility of the MRI interpretation assessing the agreement between judgments of two radiologists with the same experience in soft-tissue sarcomas; to identify an association among MRI findings (size, depth, septa, nodules, signal homogeneity) and nature of the lesion. Materials and Methods: A total of 54 patients (28 men and 26 women), with a mean age of 56 (range=27-84) were included years. All subjects followed-up by the Multidisciplinary Sarcoma Group. The following MRI findings were judged in a blind study by two radiologists: Size, localization, septa, nodules and signal homogeneity. A diagnostic indication was then given from among lipoma, atypical lipomatous tumour (ALT) and liposarcoma. Accuracy in distinguishing between benign and malignant lesions, and between lipoma and ALT (Fisher's exact test), inter-operator agreement (Cohen's kappa), association of MRI findings and malignancy of the lesion (Fisher's exact test and odds ratio) were evaluated. Results: The inter-operator agreement was complete (100%). The agreement between diagnostic hypothesis and histological diagnosis was statistically significant (p<0.05). Among the radiological findings taken into account, only septa and signal homogeneity were significantly associated with the malignancy of the lesion (p<0.05). Conclusion: MRI could be helpful in distinguishing lipomatous tumors, allowing biopsy to be avoided in some cases (negative predictive value=100%)

Cost-effectiveness of a melanoma screening programme using whole disease modelling

OBJECTIVE: To assess the potential impact of a melanoma screening programme, compared with usual care, on direct costs and life expectancy in the era of targeted drugs and cancer immunotherapy. METHODS: Using a Whole Disease Model approach, a Markov simulation model with a time horizon of 25 years was devised to analyse the cost-effectiveness of a one-time, general practitioner-based melanoma screening strategy in the population aged over 20, compared with no screening. The study considered the most up-to-date drug therapy and was conducted from the perspective of the Veneto regional healthcare system within the Italian National Health Service. Only direct costs were considered. Sensitivity analyses, both one-way and probabilistic, were performed to identify the parameters with the greatest impact on cost-effectiveness, and to assess the robustness of our model. RESULTS: Over a 25-year time horizon, the screening intervention dominated usual care. The probabilistic sensitivity analyses confirmed the robustness of these findings. The key drivers of the model were the proportion of melanomas detected by the screening procedure and the adherence of the target population to the screening programme. CONCLUSIONS: The screening programme proved to be a dominant option compared with usual care. These findings should prompt serious consideration of the design and implementation of a regional or national melanoma screening strategy within a National Health Service

Timing of sentinel node biopsy independently predicts disease-free and overall survival in clinical stage I-II melanoma patients: A multicentre study of the Italian Melanoma Intergroup (IMI)

Background: Sentinel lymph node biopsy (SNB) still remains a key procedure to appropriately stage melanoma patients and to select those who are candidate to novel treatments with immunotherapy and targeted therapy in the adjuvant setting. The impact of timing of SNB on disease-free survival (DFS) and overall survival (OS) is still unclear. Material and methods: The study was conducted at 6 Italian Melanoma Intergroup (IMI) centres and included 8953 consecutive clinical stage I-II melanoma patients who were diagnosed, treated, and followed up between November 1997 and March 2018. All patients were prospectively included in dedicated IMI database. Multivariable Cox regression analyses were performed to investigate how baseline characteristics and time interval until SNB are related to DFS and OS. Results: Considering the whole population, at multivariable analysis, after adjusting for age, gender, Breslow thickness, site, ulceration, and the SNB status, a delay in the timing of SNB was associated with a better DFS (adjusted hazard ratio [aHR, delayed versus early SNB] 0.98, 95% confidence interval [CI] 0.97\u20130.99, p < 0.001) and OS (aHR 0.98, 95% CI 0.97\u20130.99, p = 0.001). Specifically, in patients with a negative SNB status, a beneficial impact of delayed SNB (i.e. at least 32 days after primary excision) was confirmed for DFS (aHR 0.70, 95%CI 0.63\u20130.79, p < 0.001) and OS (aHR 0.69, 95%CI 0.61\u20130.78, p < 0.001), whereas in those with a positive SNB status, DFS (aHR 0.96, 95%CI 0.84\u20131.09, p = 0.534) and OS (aHR 0.94 95%CI 0.81\u20131.08, p = 0.374) were not significantly different in patients with early or delayed SNB. Conclusions: Our study does not support a strict time interval for SNB. These results may be useful for national guidelines, for counselling patients and reducing the number of high urgency referrals

A European project on incidence, treatment, and outcome of sarcoma

Abstract BACKGROUND: Sarcomas are rare tumors (1-2% of all cancers) of mesenchymal origin that may develop in soft tissues and viscera. Since the International Classification of Disease (ICD) attributes visceral sarcomas (VS) to the organ of origin, the incidence of sarcoma is grossly underestimated. The rarity of the disease and the variety of histological types (more than 70) or locations account for the difficulty in acquiring sufficient personal experience. In view of the above the European Commission funded the project called Connective Tissues Cancers Network (CONTICANET), to improve the prognosis of sarcoma patients by increasing the level of standardization of diagnostic and therapeutic procedures through a multicentre collaboration. METHODS/DESIGN: Two protocols of epidemiological researches are here presented. The first investigation aims to build the population-based incidence of sarcoma in a two-year period, using the new 2002 WHO classification and the "second opinion" given by an expert regional pathologist on the initial diagnosis by a local pathologist. A three to five year survival rate will also be determined. Pathology reports and clinical records will be the sources of information.The second study aims to compare the effects on survival or relapse-free period - allowing for histological subtypes, clinical stage, primary site, age and gender - when the disease was treated or not according to the clinical practice guidelines (CPGs). DISCUSSION: Within CONTICANET, each group was asked to design a particular study on a specific objective, the partners of the network being free to accept or not the proposed protocol. The first protocol was accepted by the other researchers, therefore the incidence of sarcoma will be assessed in three European regions, Rhone-Alpes and Aquitaine (France) and Veneto (Italy), where the geographic distribution of sarcoma will be compared after taking into account age and gender. The conformity of the clinical practice with the recommended guidelines will be investigated in a French (Rhone Alps) and Italian (Veneto) region since the CPGs were similar in both areas

A gene expression signature associated with survival in metastatic melanoma

BACKGROUND: Current clinical and histopathological criteria used to define the prognosis of melanoma patients are inadequate for accurate prediction of clinical outcome. We investigated whether genome screening by means of high-throughput gene microarray might provide clinically useful information on patient survival. METHODS: Forty-three tumor tissues from 38 patients with stage III and stage IV melanoma were profiled with a 17,500 element cDNA microarray. Expression data were analyzed using significance analysis of microarrays (SAM) to identify genes associated with patient survival, and supervised principal components (SPC) to determine survival prediction. RESULTS: SAM analysis revealed a set of 80 probes, corresponding to 70 genes, associated with survival, i.e. 45 probes characterizing longer and 35 shorter survival times, respectively. These transcripts were included in a survival prediction model designed using SPC and cross-validation which allowed identifying 30 predicting probes out of the 80 associated with survival. CONCLUSION: The longer-survival group of genes included those expressed in immune cells, both innate and acquired, confirming the interplay between immunological mechanisms and the natural history of melanoma. Genes linked to immune cells were totally lacking in the poor-survival group, which was instead associated with a number of genes related to highly proliferative and invasive tumor cells

Fine-needle aspiration cytology and frozen section examination in pre- and intraoperative diagnosis of thyroid cancer

In contrast to thyroid nodules thyroid cancer is a rare condition, which requires an early diagnosis and treatment. Patients with nontoxic solitary thyroid nodules (NSTN) both fine-needle aspiration cytology (FNAC) and intraoperative frozen-section examination (IFSE) are usually requested for the adequate surgical planning. The aim of this study was to evaluate the usefulness of FNAC and IFSE in patients with NSTN undergoing surgery. We retrospectively analyzed a series of 606 patients with a SNTN who underwent both preoperative FNAC and IFSE prior to partial or total thyroidectomy. There were 118 (19.5%) men and 488 (80.5%) women, with an overall median age of 44 years (range 16-81 years). Final pathologic examination showed 500 (82.5%) benign nodules, including 239 (39.4%) follicular adenomas, and 106 (17.5%) thyroid carcinomas, of which 80 (75.5%) papillary, 18 (17.0%) follicular, 5 (4.7%) undifferentiated, and 3 (2.8%) medullary carcinomas. Patients with benign tumors were significantly (p=0.002) younger. In the preoperative differential diagnosis between hyperplastic thyroid nodules and thyroid tumors, sensitivity, specificity, positive predictive value (PPV), negative-predictive value (NPV) of FNAC were 93.6%, 98.9%, 92.1%, 99.4% and 95.9%, respectively. In the detection of malignancy sensitivity, specificity, specificity, PPV, NPV, and accuracy were 94.3%, 99.8%, 98.8%, 99.0%, and 98.8% for FNAC, and 95.3%, 99.0%, 100%. and 99.2% for IFSE (p=NS, chi-squared test). The combination of FNAC and IFSE did not improve significantly (p=NS) the results. In fact, IFSE suggested a thyroid cancer in only one of the 6 patients with false negative FNAC, and failed to detect malignancy in 5 of 18 (27.8%) follicular carcinomas. In conclusions, in patients with NSTN and an adequate FNAC suggesting malignancy IFSE may be unnecessary, land in those with follicular tumors the results of both FNAC and IFSE should not affect the final intraoperative decision-making

Successful treatment of gangrene in systemic necrotizing vasculitis with iloprost

Systemic necrotizing vasculitis is uncommon in children and may be rarely associated with gangrene. We describe a 3-yr-old girl with parvovirus B19-induced necrotizing vasculitis whose digital gangrene was successfully treated with iloprost, a prostacyclin analogue