Response to Paclitaxel in an Adult Patient with Advanced Kaposiform Hemangioendothelioma.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare neoplasm of vascular origin that typically arises from the skin or soft tissues as a solitary tumor. The optimal therapy for this disease is still unknown. We report the case of an adult patient presenting with metastatic KHE of the spleen, who had a partial response after treatment with paclitaxel. CASE PRESENTATION: A 36-year-old man presented in November 2012 with a nontraumatic rupture of the spleen. A splenectomy was performed, and the pathology was consistent with a nonspecific vascular proliferation. Follow-up scans revealed lytic bone lesions and liver metastasis. A biopsy of the liver was performed and confirmed KHE. The decision was made to proceed with treatment with gemcitabine and docetaxel, which was discontinued due to myelotoxicity. The patient was then transferred to our institution, and a pathology review supported the diagnosis of metastatic KHE. His disease remained stable until February 2014, when he developed progression in the liver. Chemotherapy was restarted with paclitaxel, and a partial response was documented after 3 cycles. Unfortunately, disease progression occurred after 24 weeks, and subsequent treatments included prednisone, doxorubicin, interferon-α, gemcitabine, and ifosfamide, without any response. The patient developed Kasabach-Merritt phenomenon and passed away 1 week later due to a major gastrointestinal bleeding. CONCLUSIONS: This case report suggests that paclitaxel could be considered as a treatment option for advanced KHE, a rare condition for which no standard treatment exists.

Validation of questionnaire on the Spiritual Needs Assessment for Patients (SNAP) questionnaire in Brazilian Portuguese.

OBJECTIVES: Spirituality is related to the care and the quality of life of cancer patients. Thus, it is very important to assess their needs. The objective of this study was the translation and cultural adjustment of the Spiritual Needs Assessment for Patients (SNAP) questionnaire to the Brazilian Portuguese language. METHODOLOGY: The translation and cultural adjustment of the SNAP questionnaire involved six stages: backtranslation, revision of backtranslation, translation to the original language and adjustments, pre-test on ten patients, and test and retest with 30 patients after three weeks. Adult patients, with a solid tumour and literate with a minimum of four years schooling were included. For analysis and consistency we used the calculation of the Cronbach alpha coefficient and the Pearson linear correlation. RESULTS: The final questionnaire had some language and content adjustments compared to the original version in English. The correlation analysis of each item with the total score of the questionnaire showed coefficients above 0.99. The calculation of the Cronbach alpha coefficient was 0.9. The calculation of the Pearson linear correlation with the test and retest of the questionnaire was equal to 0.95. CONCLUSION: The SNAP questionnaire translated into Brazilian Portuguese is adequately reliable and consistent. This instrument allows adequate access to spiritual needs and can help patient care.

Successful Intravascular Correction of Intratumoral Pseudoaneurysm by Erosion of the Aorta in a Patient with Thoracic Giant Cell Tumor of Bone Responding to Denosumab.

Giant cell tumor of bone (GCT) is a rare, locally aggressive neoplasm characterized by the presence of giant cells with osteoclast activity. Its biology involves the overexpression of the Receptor Activator of Nuclear Factor kB Ligand (RANKL) by osteoclast-like giant cells and tumor stromal cells, which has been shown to be an actionable target in this disease. In cases amenable to surgical resection, very few therapeutic options were available until the recent demonstration of significant activity of the anti-RANK-ligand monoclonal antibody denosumab. Here we present a case of a patient with advanced GCT arising in the spine, recurring after multiple resections and embolization. Following initiation of denosumab, which resulted in unequivocal clinical improvement, computed tomography of the chest done for reassessment purposes revealed an intratumoral pseudoaneurysm by erosion of the aorta, further corrected by endovascular approach and stent placement. Patient had an unremarkable recovery from the procedure and continued benefit from therapy with denosumab and remains on treatment 24 months after the first dose.

Living better or living longer? Perceptions of patients and health care professionals in oncology.

BACKGROUND: Cancer can influence the views of patients on treatment goals and make them different from those of health care professionals (HCPs). It is crucial to understand patient expectations regarding cancer treatment. METHODS: We performed a cross-sectional survey of patients with cancer and HCPs to evaluate their perceptions about treatment priorities and to analyse variables that might influence their opinions. To identify treatment choices, we interviewed all participants using a structured questionnaire with fictitious case vignettes. RESULTS: We enrolled a total of 230 participants, including 144 patients and 86 HCPs (35 nurses, 21 physicians, 30 others). Treatment priority between survival time (28.5% for patients vs. 8.1% for HCP) and quality of life (45.8% vs. 87.2%) differed significantly, with the remaining participants stating they were uncertain or unwilling to respond, or providing invalid responses (P < 0.01). In logistic regression, prioritising survival time was more frequent in patients, adjusting for age and gender (odds ratio (OR) = 3.95; P < 0.01). The view that the physician alone should be responsible for treatment choices was more frequent among patients than HCPs (18.8 vs. 5.8%; P = 0.01). CONCLUSIONS: In Brazil, our results suggest that survival time is more important for patients with cancer than for HCPs, whereas quality of life is more important for HCPs than for patients with cancer, who place great emphasis on physicians as decision-makers. Given that Brazilian patients often rely on physicians for decisions, the potential impact of different priorities between survival time and quality of life when patients and HCPs are compared is unknown.

Phase I trials of antitumour agents: fundamental concepts.

Phase I trials are an important step in the development of new drugs. Because of the advancing knowledge of cancer's molecular biology, these trials offer an important platform for the development of new agents and also for patient treatment. Therefore, comprehension of their peculiar terminology and methodology are increasingly important. Our objectives were to review the fundamental concepts of phase I designs and to critically contextualise this type of study as a therapeutic option for patients with refractory cancer.