ABSTRACTconventional CT scan less appropriate for adequate staging and less suitable for image-guided biopsy.Recently, 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) combined with CT has emerged as the most sensitive and specific imaging modality for diagnosis and staging of both Hodgkin's and different subtypes of aggressive non-Hodgkin's lymphoma. 4 The role of FDG-PET scan in PTLD, however, has not yet been established.Our aim was to evaluate the accuracy and clinical performance of FDG-PET scan in the diagnosis of PTLD in a large cohort of transplant recipients.
Background: The treatment of advanced desmoid-type fibromatosis (DF) is poorly standardized and primarily based on physician's choice. We assessed systemic treatment preferences for advanced DF among European experts, with the aim to define a control treatment for prospective randomized trials. Material and Methods: A structured questionnaire was sent to a group of physicians involved in DF treatment. Results: 54 experts from 14 countries (Europe, Israel) responded. Disease progression and failure of local therapy were typical indications for systemic therapy. Treatment preferences for patients with sporadic DF versus DF associated with Gardner's syndrome were similar. Physicians use at least 5 different classes of drugs (27 agents). The most frequently used compounds were anti-estrogens and non-steroidal anti-inflammatory agents (NSAIDs), in combination or as single agents. The second and third most common systemic approach was chemotherapy based on methotrexate or an anthracycline. Trial activity was limited to 1 country/1 multicentric study. Conclusions: There is an unmet medical need for evidence-based treatments and well-designed studies. Clinical trials with systemic agents should ideally select a homogeneous DF population with advanced, progressive, ideally symptomatic disease and/or functional impairment after failure of wait-and-see and/or local treatments, and should be randomized, with placebo, anti-estrogens, NSAIDs, or physician's choice as comparator.
The treatment of Ewing sarcoma (ES) in adult patients requires a multidisciplinary approach. Systemic therapy remains an important component of clinical management of this disease. ES is extremely rare in adult patients. Due to the rarity of the disease, no standard of care in terms of chemotherapy for the adult population exists, and the level of evidence for individual agents or some multidrug combinations is limited. Most regimens that are used in both adults and children include anthracyclines, etoposide, vincristine, cyclophosphamide, and ifosfamide. In this report, we describe our experience with the alternating use of triple combination therapies based on vincristine, ifosfamide, and doxorubicin (VIA) and an etoposide, ifosfamide, and cisplatin combination (VIP). We retrospectively evaluated the response rates, outcome, and tolerance of adult patients (n = 64) treated with VIA/VIP between 1990 and 2014. The patients included were treated with perioperative chemotherapy (53.1% neoadjuvant therapy and 17.2% adjuvant therapy) or had synchronous metastases at diagnosis (29.7%). Five-year overall survival rate was 52.2% for all patients, 72.2% for patients with localized disease, and 5.3% in patients with synchronous metastases. Overall response rate (ORR) was 37% after 2 cycles of VIA and 2 cycles of VIP. There were no patients with progressive disease (PD).
A 64-year-old woman was scheduled for a diagnostic laparoscopy to explore two ovarian masses that were recently discovered during a workup for diarrhea, abdominal pain, and weight loss (Fig. 1A). She presented with progressive dyspnea and hypoxemia on arterial blood gas (pO 2 48 mmHg and saturation 87% on room air). Transthoracic (movie clip S1, Fig. 2) and transesophageal (movie clips S2-S4, Figs. 3 and 4) echocardiography were performed. 1,2 There was no evidence of left-sided valvular involvement. There were no lesions in the liver on positron emission tomography-computed tomography (PET-CT). A positive octreotide scan (Fig. 1B) and elevated urinary 5-hydroxyindoleacetic acid level confirmed the diagnosis of carcinoid heart disease due to an ovarian carcinoid tumor. Although in 95% of cases the primary tumor is located in the gastrointestinal tract and hepatic inactivation of serotonin prevent symptoms from occurring until liver metastases are present, 3 carcinoid heart disease in patients with primary ovarian carcinoid tumors may develop in the absence of liver metastases as the venous drainage of the ovaries bypasses the portal circulation. Sandostatin intramuscular was initiated Figure 1. A. CT abdomen indicating two nodular masses (maximal diameter 12 cm), originating from both ovaries. B. Octreotide scan indicating the presence of somatostatine receptors in these masses and confirming the diagnosis of ovarian carcinoid tumor.
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