The role of procalcitonin in the follow-up of medullary thyroid cancer.

Objective: Calcitonin (Ct) represents the most important biochemical marker of medullary thyroid cancer (MTC), but has certain limits. We analyzed the performance of procalcitonin (ProCt) in follow-up MTC patients. Methods: In this monocentric and retrospective study, we consecutively obtained ProCt and Ct values from all MTC patients that we visited during the period from April 2021 to May 2022. Patients were defined as having structural evidence of disease (29/90, 32.2%) irrespective of Ct values or, in its absence, as not evident disease (NED) if Ct was ≤10 ng/L (47/90, 52.2%), or minimal residual disease if Ct was >10 ng/L (14/90, 15.6%). Results: Ct and ProCt values were highly correlated (r = 0.883, P < 0.01). Median ProCt values differed between NED, minimal residual disease, and structural disease, being 0.04 ng/mL, 0.26 ng/mL, and 1.98 ng/mL, respectively (P < 0.01). ProCt was undetectable (<0.04 ng/mL) in 40/47 (85.1%) of NED patients, in 3/14 (21.4%) patients with minimal residual disease and in none of the patients with a structural disease (P < 0.01). Among the 11 patients with detectable but ≤10 ng/L Ct and undetectable ProCt values, none had a structural disease. The most accurate cut-off of ProCt to distinguish between the presence or absence of a structural disease was >0.12 ng/mL (P < 0.01, area under the curve: 0.963), with the following sensitivity, specificity, positive predictive value, and negative predictive value (NPV): 100%, 83.61%, 74.4%, and 100.0%. Conclusions: ProCt and Ct have a high correlation in MTC follow-up. ProCt may be useful as an adjunct to Ct, especially for its NPV concerning the structural disease.

Association between Pancreatoblastoma and Familial Adenomatous Polyposis: Review of the Literature with an Additional Case.

BACKGROUND: Adult pancreatoblastoma (PBL) is a rare pancreatic malignancy, with recent evidence suggesting a possible link to familial adenomatous polyposis (FAP). This study aims to review the latest evidence and explore a possible association between adult PBL and FAP. METHODS: Two independent literature reviews were conducted: (1) on PBL and FAP, and (2) on PBL in the adult population not diagnosed with FAP. RESULTS: Out of 26 articles on PBL and FAP screened, 5 were selected for systematic review, including 1 additional case. We identified eight FAP-related PBL cases, with a median age of 40 (IQR: 34-50). Of these, seven (87%) occurred in adults. We found 65 cases of adult PBL not FAP-related; thus, 7 out of 65 cases (10.7%) of adult PBL reported in the literature are associated with a clinical diagnosis of FAP or were carriers of APC germline pathogenic variants (GPVs). CONCLUSION: Data suggest a non-random association between adult PBL and FAP. Further research is essential to optimise surveillance protocols and develop more effective treatment strategies.

Ifosfamide-related encephalopathy in elderly patients : report of five cases and review of the literature.

Encephalopathy is a serious adverse reaction occurring in 15-30% of patients treated with the alkylating agent ifosfamide. Patients with this adverse effect may experience seizures, drowsiness, confusion and hallucinations of different grades of severity. In this article, we describe five cases of acute CNS toxicity in patients aged > or =65 years of age treated with ifosfamide and we review data on the management and outcome of this serious complication in elderly patients. All five patients experienced symptoms of encephalopathy soon after receiving combination chemotherapy including ifosfamide for different tumours. All of the patients had been assessed by means of a Comprehensive Geriatric Assessment for the presence of associated diseases, disability, cognitive status and depression, and scores were satisfactory in all patients, although case 5 was deemed frail because of cancer-related limitation in movement. In four patients, the antidote methylene blue (methylthioninium chloride) was administered intravenously, with successful recovery in three patients and a fatal outcome in the fourth patient. The fifth patient rapidly recovered after discontinuation of ifosfamide and did not receive methylene blue. The roles of older age, peak ifosfamide concentration, low albumin levels, increased serum creatinine and bulky abdominal disease as predisposing factors for ifosfamide-related encephalopathy in retrospective series are controversial.Although methylene blue has been frequently administered in patients with ifosfamide-related encephalopathy, its efficacy in this context has not been assessed objectively. Thus, careful baseline evaluation of elderly patients and constant clinical observation during infusion, especially during the first course of therapy, are recommended to reduce the risk of severe CNS toxicity from ifosfamide.

Prediction of hepatocellular carcinoma biological behavior in patient selection for liver transplantation.

Morphological criteria have always been considered the benchmark for selecting hepatocellular carcinoma (HCC) patients for liver transplantation (LT). These criteria, which are often inappropriate to express the tumor's biological behavior and aggressiveness, offer only a static view of the disease burden and are frequently unable to correctly stratify the tumor recurrence risk after LT. Alpha-fetoprotein (AFP) and its progression as well as AFP-mRNA, AFP-L3%, des-γ-carboxyprothrombin, inflammatory markers and other serological tests appear to be correlated with post-transplant outcomes. Several other markers for patient selection including functional imaging studies such as (18)F-FDG-PET imaging, histological evaluation of tumor grade, tissue-specific biomarkers, and molecular signatures have been outlined in the literature. HCC growth rate and response to pre-transplant therapies can further contribute to the transplant evaluation process of HCC patients. While AFP, its progression, and HCC response to pre-transplant therapy have already been used as a part of an integrated prognostic model for selecting patients, the utility of other markers in the transplant setting is still under investigation. This article intends to review the data in the literature concerning predictors that could be included in an integrated LT selection model and to evaluate the importance of biological aggressiveness in the evaluation process of these patients.

Treatments of AIDS-related Kaposi's sarcoma.

Although Kaposi's sarcoma (KS) has decreased in countries where the highly active antiretroviral therapy (HAART) regimen is available, however it remains, after non-Hodgkin's lymphomas, the most common malignancy in HIV+ patients. Advances in the treatment of AIDS-KS have been achieved, even though a gold standard therapy has not been yet defined. With the availability of HAART, a dramatic KS clinical response has been documented, making HAART essential in all patients. In case of aggressive and/or life threatening KS, more complex therapeutic schedules have to be taken into account, including chemotherapy and/or immunotherapy. Liposomal anthracyclines and paclitaxel have been approved by FDA as first line and second line mono-therapy, respectively. Interferon-alpha (INF-alpha) is the only immunomodulant agent to have shown a therapeutic effect. Among the new drugs, many antiangiogenetic agents have produced encouraging responses. Finally, the identification of the HHV-8 as a causative agent and new metalloproteinase inhibitors may offer promising targets for the KS treatment.

OECI accreditation at Veneto Institute of Oncology IOV -- IRCCS, general framework and multidisciplinary approach.

The aim of this article is to describe the accreditation process of the Veneto Institute of Oncology (IOV-IRCCS) according to the Organisation of European Cancer Institutes (OECI) model, with particular reference to the standards for the multidisciplinary approach. Through the analysis of the process and the activities of each multidisciplinary team (MDT) and the development, at a regional level, of diagnostic, therapeutic, and care pathways (PDTA), all the necessary steps to meet the OECI standards have been determined. Adjustment is ongoing. We are working on the inclusion of the MDT registration forms in the electronic medical records and on the possibility to extend the OECI model to the MDT not based at IOV, but participated in by IOV professionals. The sarcoma MDT has achieved results demonstrating that the OECI framework has allowed the professionals involved in the multidisciplinary meeting to systematically share the clinical information of the patient, who can benefit from better continuity of care. The model has also provided greater clarity in the management of patients who are enrolled in clinical trials and deviate from Guide Lines (GL)/PDTA. The accreditation process according to the OECI model has added value to the IOV's already well-developed multidisciplinary activities.

Increasing chemotherapy in small-cell lung cancer: from dose intensity and density to megadoses.

The hypothesis that increasing cytotoxic dose intensity will improve cancer cure rates is compelling. Although supporting evidence for this hypothesis has accrued for several tumor types, including lymphomas, breast cancer, and testicular cancers, it remains unproven. Small-cell lung cancer is extremely chemo- and radiosensitive, with a response rate of 80% achieved routinely, but few patients are cured by chemoradiotherapy. In this setting, increased cytotoxic dose intensity might improve cure rates. The finding that response rates in small-cell lung cancer correlate with received cytotoxic dose intensity merely confirms that "less is worse" and "more is better." Within conventional ranges, dose intensity can be increased with the support of hematopoietic growth factors and/or by shortening treatments intervals; however, dose intensity could be increased by only 20%-30%, and a survival advantage has not been clearly demonstrated. Given its high chemosensitivity, small-cell lung cancer was one of the first malignancies deemed suitable for increasing dose intensity and even for the use of a megadose with the support of autologous bone marrow transplantation. Some interest is emerging again due to improvements in supportive care, such as the availability of hematopoietic growth factors and peripheral blood progenitor cells.

Respiratory syncytial virus-related pneumonia after stem cell transplantation successfully treated with palivizumab and steroid therapy.

A case is reported of a 56-y-old woman with a second relapse of Hodgkin's disease who early developed after autologous stem cell transplantation (ASCT) a severe RSV-related interstitial pneumonia successfully treated with 1-d intravenous palivizumab 8 mg/kg plus low-dose systemic steroid therapy. B-cells suppression with CMV antigenaemia were then observed and required treatment with ganciclovir and liposomal amfotericine B.

Stanford V regimen plus consolidative radiotherapy is an effective therapeutic program for bulky or advanced-stage Hodgkin's disease.

Since September 1996, 48 untreated patients with bulky or advanced-stage Hodgkin's disease received the 12-week Stanford V chemotherapy regimen followed by consolidation radiotherapy at a dose of 36 Gy to bulky mediastinal disease and 30.6 Gy to the initial sites of disease > or =3 cm in transverse diameter. After the combined therapy, 46 of 48 (96%) achieved complete remissions. With a median follow-up of 48 months, the 5-year overall survival was 95% and freedom from progression 86%. There were no treatment-related deaths. All but one premenopausal female patient (who received pelvic and inguinal irradiation) recovered normal menses. Until now no case of secondary leukemia or myelodysplasia was observed. Our results confirm that the Stanford V regimen with consolidation radiotherapy is safe and effective in patients with bulky or advanced-stage Hodgkin's disease, achieving very high remission and overall 5-year survival rates. Longer follow-up is necessary to evaluate the extent of all complications.