Is there a role for adjuvant therapy in radiation-induced angiosarcoma of the breast? A case report and review of the literature.

BACKGROUND: Angiosarcoma (AS) of the breast is very rare, accounting for 1% of all soft tissue breast tumors. AS may present as primary tumors of the breast or as secondary lesions usually associated with previous radiotherapy. Commonly, secondary AS affects older women (median age 67-71 years) with a clinical history of breast cancer. The preferred site of onset of RIAS is the edge of radiation fields, where radiation doses and tumor necrosis may be heterogeneous, resulting in a DNA damage and instability. Radical surgery is the treatment of choice, but no clear consensus exists on surgical management of breast AS. CASE REPORT: We describe an atypical case of relapsed RIAS after radical mastectomy, treated with new surgery and, considering the higher risk of recurrence, subsequent adjuvant chemotherapy with weekly paclitaxel. CONCLUSIONS: The frequency of radiation-induced angiosarcomas (RIAS) after breast-conserving surgery and radiotherapy has been increased to 0.14-0.5% among long survivors. Nevertheless, even if RIAS continues to be prognostically an extremely unfavorable cancer due to a high rate of recurrence, distant spread, and median overall survival (OS) of about 60 months, the benefits of loco-regional breast radiotherapy are clearly higher than the risk in developing angiosarcoma.

A patients' and caregivers' perspective on hepatic encephalopathy.

Awareness of previous hepatic encephalopathy (HE) and compliance with treatment can probably reduce HE recurrence. The aim of this study was to assess the degree of awareness of previous HE and its treatment in a group of cirrhotic patients and their caregivers. Thirty-five cirrhotic patients with a history of HE and their caregivers (n = 31) were enrolled. Patients underwent evaluation of HE (clinical, psychometry and electroencephalography), quality of life (SF36 questionnaire), and awareness of HE/treatment on an ad hoc questionnaire (QAE). Caregivers underwent the QAE plus the Caregiver Burden Inventory. On the day of study, 7 patients were unimpaired, 8 had minimal and 20 low-grade overt HE. Of the patients, 37 % were aware of previous HE, 6 % of being on treatment and 6 % understood treatment effects. Of the caregivers, 48 % were aware of previous HE, 6 % of their relative being on treatment and 6 % understood treatment effects. Significant correlations were observed between neuropsychiatric status/linear HE indices and both the patients' quality of life and the caregivers' burden. In conclusion, HE awareness was poor in both patients and caregivers, most likely in relation to insufficient/inadequate provision of information.

Management of systemic prostate cancer: current algorithm from castration sensitive to castration resistant setting.

In recent years, the advanced knowledge of clinical, biological and molecular features of prostate cancer have led to the introduction of new drugs and have allowed the relocation of old drugs in different settings. In this way, the new concepts of systemic disease arise: high risk or high volume vs. low risk and low volume disease castration sensitive prostate cancer (CSPC), diversifying the use of previously approved drugs (CRPC) and opening new scenarios for sequence therapy. The aim of this review is to integrate new developments into the medical management of systemic prostate cancer.

Advanced/metastatic bladder cancer: current status and future directions.

In 2015 bladder cancer was the fourth most frequent malignancy and the eighth cause of death for cancer. At diagnosis, about 30% of bladder cancer (BC) patients present a muscle-invasive bladder cancer (MIBC) and 5% a metastatic bladder carcinoma (MBC). For fit MBC patients, combination chemotherapy (CC) is the standard of care for first-line treatment. CC includes both the treatment with methotrexate, vinblastine, doxorubicin, and cisplatin (MVAC) either the classical or the dose-dense MVAC regimen, and the doublet therapy with cisplatin and gemcitabine (CG). Median progression free survival (PFS) was 7 months and median overall survival (OS) was 15 months. The present review provides an update on the management of MBC, with focus on target therapies, immune checkpoint inhibition, looking for prognostic and predictive factors.

Prognostic and predictive factors in testicular cancer.

OBJECTIVE: Testicular cancer is a relatively rare neoplasia, with an incidence of about 1,5% among male malignancies, usually in the third and fourth decade of life. Although several histological variants are known, with some histotypes affecting older patients (e.g., spermatocytic seminoma), there is a clear predominance (90-95%) of germ cell tumors among young adults patients1. Testicular Germ Cell Tumor (TGCT), undoubtedly the seminoma histological variant more than non-seminoma one, is definitely a highly curable disease, with a distinctive sensitivity to cisplatin-based therapy (and for seminomas to radiotherapy) and an outstanding cure rate of nearly 80% even for patients with advanced disease. So far, clinical and pathohistological features supported our efforts to choose the best treatment option for patients suffering from this malignancy, but we don't clearly enough know molecular and pathological features underlying different clinical behaviors, mostly in early-stage disease: by improving this knowledge, we should better "shape" therapeutic or surveillance programs for each patient, also in order to avoid unnecessary, if not harmful, treatments.

Effects of an eight-month weight-control program on body composition and lipid oxidation rate during exercise in obese children.

OBJECTIVE: To investigate the effects of an 8-month multidisciplinary weight-control program, including 2 h/week of moderate physical activity, nutritional education lessons and psychological follow-up, on body composition and lipid oxidation rate during exercise in obese children. DESIGN: Nineteen (7 boys and 12 girls) obese children, aged 8-12 yr [mean body mass index (BMI) z-score: 2.3 and fat mass: 35.8%] participated in this study. Before and at the end of the weight-control period body composition was assessed by bioelectrical impedance, lipid oxidation rate by indirect calorimetry during a graded exercise test, and time devoted to various activities and energy intake in free-living conditions by questionnaire. RESULTS: All children completed the study, at the end of which BMI decreased significantly by mean 0.6+/-0.5 and 0.5+/-0.8 kg/m2, in boys and girls, respectively (p<0.05), and fat mass (FM) decreased by 1.7+/-2.8 and 1.4+/-1.3 kg in boys and girls, respectively (p<0.05). In addition, lipid oxidation rate during exercise increased significantly throughout the graded exercise test up to 21% at maximal lipid oxidation rate which happened at 48+/-5% of maximal oxygen uptake (VO2max), corresponding to 64+/-5% of maximal heart rate. Time spent at sedentary and very light physical activities decreased (p<0.001) to the benefit of recreational activities at home. CONCLUSIONS: Multidisciplinary weight-control program, with moderate-intensity physical activities, induced decreases in FM without decreases in free FM, increases in VO2max, lipid oxidation rate during exercise, and time devoted to recreational activities in free-living conditions.

Health-related quality of life and psychosocial implications in testicular cancer survivors. A literature review.

OBJECTIVE: In this review, we focused our attention on Quality of Life (QoL) of testicular cancer survivors (TCSs), in general and in the most relevant areas. Several key findings have been highlighted in our review. MATERIALS AND METHODS: PubMed, MEDLINE and PsycINFO databases were consulted to find published studies, from 1980 to May 2017, that met our inclusion criteria. RESULTS: The majority of studies investigated older adult TCSs, while few studies on adolescent and young adult patients were available. Many studies indicate that health-related QoL (HRQoL) is similar among the TCSs and the general population. Even if QoL deteriorates so clear at the time of diagnosis and throughout treatment, afterward returns to normal levels, as defined by the matched controls. However, there are numerous chronic conditions consequent to diagnosis and treatment of testicular cancer that plague survivors and affect QoL, like Raynaud-like phenomena, peripheral neuropathy, fatigue, anxiety, sexual, fertility and body image problems. Even if these problems can have no effects on the measures of global QoL, they have an impact on the quality of life. Differences between TCSs with and without a partner bring to different outcomes in the adjustments to cancer. CONCLUSIONS: It is necessary to identify TCSs with higher risks of poorer QoL outcomes, to focus interventions on the areas with the greatest impairments. Further researches should consider the effects of testicular cancer on the impaired areas, collecting more data to better identify survivor's needs and consequent interventions, with a special focus on adolescent and young adult TCSs. Other works are requested on therapies, preventive and ameliorative, to reduce chronic side effects of testicular cancer treatment.

Stereotactic injection of herpes simplex thymidine kinase vector producer cells (PA317-G1Tk1SvNa.7) and intravenous ganciclovir for the treatment of progressive or recurrent primary supratentorial pediatric malignant brain tumors.

This study will evaluate the safety and efficacy of in vivo gene transfer of the herpes simplex thymidine kinase (HSV-Tk1) gene using PA317/G1Tk1SvNa.7 vector producer cells (VPC) in pediatric patients with progressive or recurrent primary supratentorial malignant brain tumors. Insertion of the HSV-Tk1 gene confers a sensitivity to the anti-herpes drug ganciclovir. It has been demonstrated that the direct injection of HSV-Tk vector producer cells into growing tumors in animals can result in their complete destruction with ganciclovir therapy. This selective destruction of growing tumors in situ is thought to result from the transfer of the HSV-Tk gene into the tumor cells and the production of toxic ganciclovir metabolites which result from the interaction of HSV-Tk and ganciclovir. This procedure can result in the cure of some experimental animals with limited systemic toxicity due to selective gene transfer into tumors. This clinical trial will focus on maximizing the relative number of vector producer cells to the tumor mass by stereotactically injecting VPCs into the tumor mass. Children with progressive or recurrent primary supratentorial malignant brain tumor which is accessible to stereotactic injection will be evaluated for the extent and location(s) of their disease before being entered into the study. Fifteen days after stereotactic injection of the tumor mass, ganciclovir will be administered at 5 mg/kg IV b.i.d. for 14 days. Upon completion of the treatment with HSV-Tk1 vector producer cells and ganciclovir, the patient will be followed monthly for the first three months, then every two months for the next twenty-one months, and annually for life thereafter.

Changes in motor cortex excitability during ipsilateral hand muscle activation in humans.

OBJECTIVES: To test whether unilateral hand muscle activation involves changes in ipsilateral primary motor cortex (M1) excitability. METHODS: Single- and paired-pulse transcranial magnetic stimulation (TMS) of the right hemisphere was used to evoke motor evoked potentials (MEPs) from the resting left abductor pollicis brevis (APB) in 9 normal volunteers. We monitored changes in motor threshold (MT), MEP recruitment, intracortical inhibition (ICI) and intracortical facilitation (ICF) while the ipsilateral right APB was either at rest or voluntarily activated. Spinal motoneuron excitability was assessed using F-wave recording procedures. RESULTS: Voluntary muscle activation of the ipsilateral APB significantly facilitated the MEPs and F-waves recorded from the contralateral APB. Facilitation was observed with muscle activation >50% of the maximum voluntary force and with stimulus intensities >20% above the individual resting motor threshold. Intracortical inhibition significantly decreased in the ipsilateral M , while there was no significant change in intracortical facilitation during this maneuver. CONCLUSIONS: Unilateral hand muscle activation changes the excitability of homotopic hand muscle representations in both the ipsilateral M1 and the contralateral spinal cord. While the large proportion of MEP facilitation most likely occurred at a spinal level, involvement of the ipsilateral hemisphere may have contributed to the enlargement of magnetic responses.

A treatable cause of ataxia in children.

An 11-year-old black male presenting with severe subacute sensory ataxia, unusual skin hyperpigmentation, megaloblastic anemia, low serum B12 levels, and an abnormal part I Schilling test was diagnosed with pernicious anemia in the context of a polyglandular syndrome. Intrinsic factor and thyroid microsomal antibodies were positive, and thyroid-stimulating hormone levels were undetectable. There was a strong familial aggregation because the mother, a maternal aunt, the maternal grandfather, and the maternal great-grandmother had been diagnosed with pernicious anemia, albeit of unspecified etiology. Spinal magnetic resonance imaging (MRI) demonstrated extensive demyelination of the posterior columns along the entire length of the cord, as well as areas of contrast enhancement. Treatment with cobalamin produced complete remission of the neurologic deficits and normalization of the MRI findings in the short space of 2 months. Although rare, childhood pernicious anemia is a treatable disease that should be included in the differential diagnosis of the sensory ataxias in children. In this article, we review the causes of pernicious anemia in children and discuss the MRI findings.

[Predictive factors of etiology (bacterial or viral) in acute gastroenteritis in childhood]. / Fattori predittivi dell'eziologia (batterica o virale) nelle gastroenteriti acute dell'infanzia.

The aim of the study was to identify immediate clinical and/or laboratory findings able to differentiate bacterial from viral etiology of acute gastroenteritis in pediatric patients. We studied 52 children, aged between 5 months and 12 years, consecutively admitted to hospital with acute diarrhoea lasting less than 5 days. All the patients were divided into 4 groups according to etiologic agent, subsequently demonstrated by culture: salmonellae (group A), rotavirus (group B); combined salmonellae and rotavirus (group C) and no pathogen (group D). The contemporary presence of fever > 39 degrees C, number of daily liquid stools > 6, presence of bloody diarrhoea, positivity of C-reactive protein and hyponatremia (< 135 mEq/l) allowed to recognize the etiology (viral or bacterial) before results of culture (sensitivity was 71% and specificity was 97%). In particular, hyponatremia resulted significantly lower in group A and C than in group B and D. We concluded that hyponatremia can be considered a marker for acute gastroenteritis caused by salmonellae.

[Retrospective analysis of Henoch-Schonlein purpura in children: diagnostic difficulties and renal outcome]. / Analisi di una casistica pediatrica di porpora di Schönlein-Henoch: difficoltà diagnostiche iniziali e follow-up a distanza.

OBJECTIVE: To assess clinical presentation, diagnostic difficulties, natural history and prognostic indicators of renal involvement of Henoch Schönlein Purpura, a ten-years retrospective analysis of all patients case records was performed. METHODS: After a variable interval of 1 to 10 years following the vasculitic episode all the children came to the outpatient clinic for history taking, clinical examination, blood pressure measurements, urine analysis and renal function assessment. RESULTS: 54 children, mean age 5.9 +/- 2.5, were identified. The purpuric rash was associated with arthritis in 46 patients (85%), abdominal pain in 28 (51.8%), temperature > 38 degrees C in 11 (20%). The purpuric rash was present at on presentation in 39 subjects (72%) whereas in 15 children (28%) it followed the subsequent signs or symptoms that were arthritis in 8 (53.3%), joint pain in 2 (13.3%), abdominal pain in 5 (30.3%). Abdominal pain was the only symptom of presentation in 5 patients; two of them underwent surgical laparotomy.

[Kluver Bucy syndrome and central diabetes insipidus: two uncommon complications of herpes simplex encephalitis]. / Sindrome di Kluver Bucy e diabete insipido centrale: due rare complicanze dell'encefalite erpetica.

Herpes Simplex Encephalitis (HSE) is an uncommon but severe disease with high mortality and morbidity. The major clinical manifestations are deteriorating consciousness with confusion, drowsiness or coma, altered behaviour, convulsions and a variety of neurological signs (hemiplegia, aphasia, ataxia, etc.). An uncommon complication of HSE is Kluver Bucy syndrome (KBS), characterized by hyperorality, bulimia and changes in emotional behaviour. Neuroimaging studies frequently show an involvement of the temporal lobes and limbic areas. Another uncommon complication of HSE is central diabetes insipidus as a result of herpes simplex infection of the hypothalamus. We report two pediatric cases of HSE complicated with Kluver Bucy syndrome and central diabetes insipidus.

[Periodic fever, aphthous stomatitis, pharyngitis, and lymphadenopathy: a pediatric caseload]. / Febbre periodica, stomatite aftosa, faringite, linfoadenopatia (PFAPA): una casistica pediatrica.

We report 5 patients (3F; 2M; age 19-60 months) affected by a syndrome characterized by recurrent episodes of abrupt onset of fever, aphtous stomatitis, pharyngitis and cervical adenopathy named by the acronym PFAPA (periodic fever, aphtous stomatitis, pharyngitis and adenopathy). The episodes had clockwork periodicity (3-4 weeks) and the fever was unaccompanied by remarkable respiratory signs or symptoms. All children were doing well between episodes. The inflammatory markers were elevated only during attacks in all children. Steroid therapy was highly effective in controlling symptoms in 5/5 patients and tonsillectomy was associated with remission in two of five patients. In one child the syndrome resolved spontaneously, in two patients resolved after tonsillectomy and in two children persisted. No long-term sequelae were observed.