Am J Clin Pathol. 2005 Dec;124 Suppl:S110-21.

"Undifferentiated" small round cell tumors of the sinonasal tract: differential diagnosis update.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16468421&dopt=Abstract

Iezzoni JC, Mills SE.

Robert E. Fechner Laboratory of Surgical Pathology, Department of Pathology, University of Virginia Health System, Charlottesville 22908, USA.

Sinonasal tract neoplasms composed of light microscopically seemingly "undifferentiated" small round cells often generate considerable diagnostic difficulty. Although the careful review of H&E-stained sections remains of critical and central importance in this evaluation, the recent improvements in the immunohistochemical diagnostic armamentarium and molecular diagnostic techniques applicable to paraffin-embedded tissue samples may add diagnostically valuable information. Accordingly, this review will discuss the differential diagnosis of undifferentiated small blue cell tumors of the sinonasal tract based on the light microscopic and clinical features and, as needed, the results of these ancillary studies. Tumors discussed include olfactory neuroblastoma, sinonasal undifferentiated carcinoma, small cell undifferentiated (neuroendocrine) carcinoma, undifferentiated (lymphoepithelioma-like) carcinoma, malignant melanoma, pituitary adenoma, Ewing sarcoma/peripheral neuroectodermal tumor, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, synovial sarcoma, extranodal natural killer/T-cell lymphoma, nasal type, and extramedullary plasmacytoma.

PMID: 16468421 [PubMed - in process]

Acta Orthop. 2005 Dec;76(6):899-903.
 
Long-term follow-up of 15 patients with non-metastatic Ewing's sarcoma and a skip lesion.

Jiya TU, Wuisman PI

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16470449&dopt=Abstract

Department of Orthopaedic Surgery, Vrije Universiteit Medical Centre, Amsterdam. tim.jiya@wanadoo.nl

BACKGROUND Skip lesions in Ewing's sarcoma of the bone seem to be rare; to our knowledge only 7 cases have been published in the English medical literature. METHODS: We retrospectively reviewed imaging and histological data relating to 235 patients with non-metastatic Ewing's sarcoma of the bone who participated in the cooperative Ewing's sarcoma study (CESS 86 and CESS 91), and we identified 15 patients with a skip lesion at diagnosis. RESULTS: The skip lesion was located in the same bone as the primary tumor in 13 patients, and in an adjacent juxtaarticular bone in 2 cases. The average follow-up was 11 years. Despite aggressive treatment including surgery in all cases, tumor relapse occurred in 9 patients, and 7 of these patients died due to metastatic disease. INTERPRETATION: Skip lesions in patients with otherwise non-metastatic skeletal Ewing's sarcoma may be of the same consequence as the molecular detection of marrow metastases and possibly confer a worse prognosis. Newer imaging modalities (for example PET) and careful staging work-up may indicate that skip metastases in Ewing's sarcoma are more common than previously suspected.

PMID: 16470449 [PubMed - indexed for MEDLINE]

Journal of Pediatric Psychology, Vol. 26, No. 4, 2001, pp. 225-235
© 2001 Society of Pediatric Psychology

http://jpepsy.oxfordjournals.org/cgi/content/abstract/26/4/225

Psychological Adaptation and Social Support of Parents of Pediatric Cancer Patients: A Prospective Longitudinal Study

Josette E. H. M. Hoekstra-Weebers, PhD, Jan P. C. Jaspers, PhD, Willem A. Kamps, PhD and Ed C. Klip, PhD
University Hospital Groningen, The Netherlands

All correspondence should be sent to Josette Hoekstra-Weebers, Department of Medical Psychology, University Hospital, Hanzeplein 1, P.O. Box 30.001, 9700 RB Groningen, The Netherlands. E-mail: j.hoekstra-weebers@medps.azg.nl .

Objective: To investigate levels of support and the concurrent and prospective effects of support on the psychological functioning of parents of children with cancer in a prospective longitudinal study.

Methods: Parents' (n = 128) self-perceived level of psychological distress, quantity of support, and dissatisfaction with support were assessed, at diagnosis, at 6, and at 12 months.

Results: Parents received most support at diagnosis. Self-perceived quantity decreased with time, but parents indicated they remained equally satisfied. Support significantly predicted concurrent and prospective distress of fathers, but not of mothers. Dissatisfaction with support and negative interactions were consistent risk factors for fathers. Mothers who adjusted well psychologically received more support and were less dissatisfied than mothers who remained clinically distressed. Nevertheless, no persisting effect of support was found.

Conclusions: Findings illustrate that social support varies with the stress situation and with gender. Identification of vulnerable parents at diagnosis on the basis of their perception of received quantity of and dissatisfaction with support seems difficult. Intervention efforts aimed at mobilization of needed support may be efficacious.

Journal of Pain and Symptom Management
Volume 30, Issue 6 , December 2005, Pages 570-577

http://www.mdlinx.com/readArticle.cfm?art_id=1447035
      
Copyright © 2005 U.S. Cancer Pain Relief Committee Published by Elsevier Inc.
Clinical Note

When Nothing Helps: Propofol as Sedative and Antiemetic in Palliative Cancer Care

Staffan Lundström MDCorresponding Author Contact Information, Ulla Zachrisson MD and Carl Johan Fürst MD, PhD

Palliative Care Services (S.L., U.Z., C.J.F.), Stockholms Sjukhem Foundation; and Department of Oncology-Pathology (S.L., C.J.F.), Karolinska Institutet, Stockholm, Sweden

Accepted 27 May 2005.  Available online 21 December 2005.


Abstract

Benzodiazepines, neuroleptics, and barbiturates are commonly used for sedation to achieve symptom control in end-of-life care. Propofol has several advantages over traditional sedating agents that would indicate its use in treatment-refractory situations. We report on the use of propofol in 35 patients. In 22 patients, propofol was used for palliative sedation when treatment with benzodiazepines had failed. The mean dose range during treatment was between 0.90 and 2.13 mg/kg/h. The effect was assessed as good or very good in 91% of the patients. Thirteen patients were treated with propofol due to intractable nausea and vomiting. The mean dose range during the infusion period was 0.67–1.01 mg/kg/h. The effect was judged as good or very good in 69% of the patients. Based on our experience, we propose clinical guidelines on the safe use of propofol in specialized palliative inpatient units.

Key Words: Propofol; sedative; sedation; antiemetic; nausea; vomiting; palliative care


Corresponding Author Contact InformationAddress reprint requests to: Staffan Lundström, MD, Stockholms Sjukhem Foundation, Mariebergsgatan 22, S-112 35 Stockholm, Sweden.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16455477&dopt=Abstract

Lancet Oncol. 2006 Feb;7(2):132-40.

Survival from rare cancer in adults: a population-based study.

Gatta G, Ciccolallo L, Kunkler I, Capocaccia R, Berrino F, Coleman MP, De Angelis R, Faivre J, Lutz JM, Martinez C, Moller T, Sankila R; EUROCARE Working Group.

Epidemiology Unit, National Cancer Institute, Milan, Italy. gemma.gatta@istitutotumori.mi.it

BACKGROUND: Rare cancers are a challenge to clinical practice, and treatment experience, even in major cancer centres to which rare cancers are usually referred, is often limited. We aimed to study the epidemiology of rare cancers in a large population of several countries. METHODS: We analysed survival by age, sex, subsite, and morphology in 57,144 adults with 14 selected rare cancers diagnosed 1983-94. Variations in survival over time and between European regions were also assessed for variations in quality of care. We also estimated the adjusted relative excess risk of death for every rare cancer. FINDINGS: Overall 5-year relative survival was good (ie, >65%) for placental choriocarcinoma (85.4% [95% CI 81.4-89.5]), thyroid medullary carcinoma (72.4% [69.2-75.5]), ovarian germ-cell cancer (73.0% [70.0-76.0]), lung carcinoid (70.1% [67.3-72.9]), and cervical adenocarcinoma (65.5% [64.3-66.6]); intermediate (ie, 35-65%) for testicular cancer at age 65 years or older (64.0% [59.3-68.7]), sarcoma of extremities (60.0% [58.9-61.2]), digestive-system endocrine cancers (55.6% [54.9-56.3]), anal squamous-cell carcinoma (53.1% [51.5-54.8]), and uterine sarcoma (43.5% [42.0-44.9]); low for carcinoma of adrenal-gland cortex (32.7% [28.3-37.2]) and bladder squamous-cell carcinoma (20.4% [18.8-22.0]); and poor for angiosarcoma of liver (6.4% [1.8-11.0]) and mesothelioma (4.7% [4.3-5.2]). Survival was usually better for women than men and poor in those aged 75 years or older. Survival significantly improved over time for ovarian germ-cell cancer, sarcomas of extremities, digestive-system endocrine tumours, anal squamous-cell carcinoma, and angiosarcoma of liver. Survival in northern Europe was higher than in the other geographic groupings for most cancers. INTERPRETATION: Because effective treatments are available for several of the rare cancers we assessed, further research is needed to ascertain why survival is lower in some European countries than in others, particularly in older patients. Audit of best practice for rare cancers with treatment protocols would be useful.

PMID: 16455477 [PubMed - in process]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16400337&dopt=Abstract

Bone Marrow Transplant. 2006 Feb;37(4):345-51.

Population pharmacokinetics of intravenous busulfan in patients undergoing hematopoietic stem cell transplantation.

Takama H, Tanaka H, Nakashima D, Ueda R, Takaue Y.

1Product Development Department, Pharmaceutical Division, Kirin Brewery Company Ltd, Shibuya-ku, Tokyo, Japan.

A population pharmacokinetic analysis was performed in 30 patients who received an intravenous busulfan and cyclophosphamide regimen before hematopoietic stem cell transplantation. Each patient received 0.8 mg/kg as a 2 h infusion every 6 h for 16 doses. A total of 690 concentration measurements were analyzed using the nonlinear mixed effect model (NONMEM) program. A one-compartment model with an additive error model as an intraindividual variability including an interoccasion variability (IOV) in clearance (CL) was sufficient to describe the concentration-time profile of busulfan. Actual body weight (ABW) was found to be the determinant for CL and the volume of distribution (V) according to NONMEM analysis. In this limited study, the age (range 7-53 years old; median, 30 years old) had no significant effect on busulfan pharmacokinetics. For a patient weighting 60 kg, the typical CL and V were estimated to be 8.87 l/h and 33.8 l, respectively. The interindividual variability of CL and V were 13.6 and 6.3%, respectively. The IOV (6.6%) in CL was estimated to be less than the intraindividual variability. These results indicate high interpatient and intrapatient consistency of busulfan pharmacokinetics after intravenous administration, which may eliminate the requirement for pharmacokinetic monitoring.Bone Marrow Transplantation (2006) 37, 345-351. doi:10.1038/sj.bmt.1705252; published online 9 January 2006.

PMID: 16400337 [PubMed - in process]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16450380&dopt=Abstract

Int J Cancer. 2006 Jan 31; [Epub ahead of print] 

Generation of tumoricidal PAX3 peptide antigen specific cytotoxic T lymphocytes.

Rodeberg DA, Nuss RA, Elsawa SF, Erskine CL, Celis E.

Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

The transcription factor PAX3 is expressed during early embryogenesis and in multiple cancer types, including embryonal rhabdomyosarcoma (ERMS), Ewing sarcoma (ES) and malignant melanoma (MEL), suggesting that it could function as a general tumor associated antigen. Major histocompatibility complex (MHC) peptide binding algorithms were used to predict potential epitopes in PAX3 capable of stimulating in vitro naive HLA-A0201 restricted cytotoxic T-lymphocytes (CTLs). Two peptides, PAX3-282 (QLMAFNHLI) and a modified version of this peptide PAX3-282.9V (QLMAFNHLV), were capable of inducing antigen-specific CTLs. Of these peptides, PAX3-282.9V was the most efficient inducer of primary CTL response. These CTLs were able to lyse HLA-A0201 expressing target cells that were pulsed with peptide, and more importantly, were effective in killing tumor cells that express PAX3, including ERMS, ES and MEL cell lines. These findings provide compelling evidence that peptide PAX3-282 is naturally processed by tumors and is presented in the context of HLA-A0201 in adequate amounts to allow CTL recognition. Also, PAX3-282.9V is an effective immunogenic peptide able to induce CTL recognition of PAX3-containing tumors and may be used as an antitumor peptide vaccine. (c) 2006 Wiley-Liss, Inc.

PMID: 16450380 [PubMed - as supplied by publisher]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16446642&dopt=Abstract

Ann Dermatol Venereol. 2005 Dec;132(12 Pt 1):986-9.

Primary cutaneous extraskeletal Ewing's sarcoma

[Article in French]

Kourda M, Chatti S, Sfia M, Kraiem W, Ben Brahim E.

Service de Dermatologie, Hopital de Nabeul, Tunisie. kourda_mouna@yahoo.fr

BACKGROUND: Cutaneous extraskeletal Ewing's sarcoma is rare, being seen principally in children. We report a case of cutaneous sarcoma in the sole of the foot in a child. CASE REPORT: A 9-year-old child with no medical history of note was presenting a skin tumor for 3 months on the heel of the right foot. This tumor was burgeoning and painful and measured 3.5 cm in diameter; it was ulcerative at the surface and covered with a crust. Histological and immunohistochemical examinations confirmed the diagnosis of Ewing's sarcoma. Staging examinations proved negative and the patient underwent polychemotherapy, resulting in complete regression of the tumor. COMMENTS: Until 1998, 37 cases of cutaneous and subcutaneous Ewing's sarcoma were reported, being seen in 21 girls and 16 boys. Mean age at diagnosis was 15 years and mean tumor size was 3 cm (range: 1 to 12 cm). The tumors were observed throughout the body, being seen in the sole of the foot in 2 cases. Confirmation of the diagnosis was made by histological examination (malignant proliferation of small round cells in the dermis), immunohistochemical examination (CD99+) and cytogenetic analysis (translocation between chromosomes 22 and 11). The prognosis for cutaneous Ewing's sarcoma appears more favorable than that of Ewing's sarcoma in bone. Of the 37 patients treated, 7 had metastases and 2 presented relapse. Treatment for cutaneous Ewing's sarcoma, though not codified, consists of polychemotherapy associated with surgery and/or radiotherapy.

PMID: 16446642 [PubMed - in process]