Journal ArticleDOI
Malignant vascular tumors in children and adolescents: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group.
Andrea Ferrari,Michela Casanova,Gianni Bisogno,Giovanni Cecchetto,Cristina Meazza,Lorenza Gandola,Alberto Garaventa,A. Mattke,Jörn Treuner,Modesto Carli +9 more
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TLDR
Angiosarcoma and related malignant vascular tumors are aggressive neoplasms with a poor prognosis; their behavior in children seems no different from their adult counterparts.Abstract:
Background
Malignant vascular tumors are extremely rare in childhood and few data on their clinical management are available. We report on a series of 18 children who had malignant vascular tumors, treated from 1980 to 2000 by the Italian and German Soft Tissue Sarcoma Cooperative Group.
Procedure
Twelve patients had angiosarcoma, four had malignant hemangioendothelioma, and two had Kaposi's sarcoma. Surgical resection was completed in six cases; radiotherapy was administered to 6 children, and chemotherapy to 14.
Results
After a median follow-up of 208 months, the 5-year survival and event-free survival rates were 30.9 and 20.8%. Six patients were alive, four in first remission (three had tumor < 5 cm, grossly completely resected), and two in second remission. Response to chemotherapy was evaluable in nine cases and was: six no response, two partial remission, one complete remission.
Conclusions
Angiosarcoma and related malignant vascular tumors are aggressive neoplasms with a poor prognosis; their behavior in children seems no different from their adult counterparts. Complete surgical resection remains the mainstay of treatment, but is probably sufficient in only a minority of cases. Postoperative radiotherapy may have a role and could be added to improve local control. The role of chemotherapy is uncertain, but the high rate of metastatic spread prompts investigation into new chemotherapeutic approaches. Med Pediatr Oncol 2002;39:109–114. © 2002 Wiley-Liss, Inc.read more
Citations
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Journal ArticleDOI
Neurofibromatosis type 1.
David H. Gutmann,Rosalie E. Ferner,Robert Listernick,Bruce R. Korf,Pamela L. Wolters,Kimberly J. Johnson +5 more
TL;DR: Although considerable progress has been made in understanding this condition, numerous challenges remain; a collaborative and interdisciplinary approach is required to manage individuals with neurofibromatosis type1 and to develop effective treatments.
Journal ArticleDOI
Whole-exome sequencing-based discovery of STIM1 deficiency in a child with fatal classic Kaposi sarcoma
Minji Byun,Avinash Abhyankar,Virginie Lelarge,Sabine Plancoulaine,Sabine Plancoulaine,Ayse Palanduz,Leyla Telhan,Bertrand Boisson,Capucine Picard,Capucine Picard,Scott Dewell,Connie Zhao,Emmanuelle Jouanguy,Emmanuelle Jouanguy,Emmanuelle Jouanguy,Stefan Feske,Laurent Abel,Laurent Abel,Laurent Abel,Jean-Laurent Casanova +19 more
TL;DR: Whole-exome sequencing reveals a homozygous splice-site mutation in the gene encoding STIM1 in a child with classic Kaposi sarcoma.
Journal ArticleDOI
Forward genetic screen for malignant peripheral nerve sheath tumor formation identifies new genes and pathways driving tumorigenesis
Eric P. Rahrmann,Adrienne L. Watson,Vincent W. Keng,Kwangmin Choi,Branden S. Moriarity,Dominic A. Beckmann,Natalie K. Wolf,Aaron L. Sarver,Margaret H. Collins,Christopher L. Moertel,Margaret R. Wallace,Bernat Gel,Eduard Serra,Nancy Ratner,David A. Largaespada +14 more
TL;DR: The Sleeping Beauty transposon-based somatic mutagenesis system in mice with somatic loss of transformation-related protein p53 (Trp53) function and/or overexpression of human epidermal growth factor receptor (EGFR) was used to identify genetic drivers of MPNST development, and several new proto-oncogenes, including Foxr2 (encoding forkhead box R2), were identified.
Journal ArticleDOI
Antiangiogenetic therapy with pioglitazone, rofecoxib, and metronomic trofosfamide in patients with advanced malignant vascular tumors.
Thomas Vogt,Christian Hafner,Klaus Bross,Frauke Bataille,Karl-Walter Jauch,Anna Berand,Michael Landthaler,Reinhard Andreesen,Albrecht Reichle +8 more
TL;DR: The efficacy of metronomically scheduled, low‐dose trofosfamide in combination with the peroxisome proliferator‐activated receptor γ agonist, pioglitazone, and the selective cyclooxygenase‐2 inhibitor, rofecoxib was evaluated in patients with advanced vascular malignancies.
Journal ArticleDOI
Inherited human OX40 deficiency underlying classic Kaposi sarcoma of childhood
Minji Byun,Cindy S. Ma,Cindy S. Ma,Arzu Akcay,Vincent Pedergnana,Vincent Pedergnana,Umaimainthan Palendira,Umaimainthan Palendira,Jinjong Myoung,Danielle T. Avery,Yifang Liu,Avinash Abhyankar,Lazaro Lorenzo,Lazaro Lorenzo,Monika Schmidt,Hye Kyung Lim,Olivier Cassar,Mélanie Migaud,Mélanie Migaud,Flore Rozenberg,Nur Canpolat,Gonul Aydogan,Bernhard Fleckenstein,Jacinta Bustamante,Jacinta Bustamante,Capucine Picard,Capucine Picard,Antoine Gessain,Emmanuelle Jouanguy,Emmanuelle Jouanguy,Emmanuelle Jouanguy,Ethel Cesarman,Martin Olivier,Philippe Gros,Laurent Abel,Laurent Abel,Laurent Abel,Michael Croft,Stuart G. Tangye,Stuart G. Tangye,Jean-Laurent Casanova +40 more
TL;DR: Human OX40 is necessary for robust CD4+ T cell memory and confers selective protective immunity against HHV-8 infection in endothelial cells.
References
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Edward L. Kaplan,Paul Meier +1 more
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Angiosarcoma: A report of 67 patients and a review of the literature
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Journal ArticleDOI
Angiosarcoma of the face and scalp, prognosis and treatment
TL;DR: 72 patients with angiosarcoma of the face and scalp have been analyzed with respect of various prognostic factors and the effects of different treatment regimes, finding the overall poor prognosis and the apparent eradication of the local skin disease were poor.
Journal ArticleDOI
Angiosarcoma of skin and soft tissue: A study of forty-four cases
John C. Maddox,Harry L. Evans +1 more
TL;DR: Survition was generally poor in all groups, owing to frequent local recurrence and early metastasis, and only nine patients had long‐term, disease‐free survival.