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1. Menu-Branthomme A, Rubino C, Shamsaldin A, Hawkins MM, Grimaud E, Dondon MG, Hardiman C, Vassal G, Campbell S, Panis X, Daly-Schveitzer N, Lagrange JL, Zucker JM, Chavaudra J, Hartman O, de Vathaire F: Radiation dose, chemotherapy and risk of soft tissue sarcoma after solid tumours during childhood. Int J Cancer; 2004 May 20;110(1):87-93
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  • [Title] Radiation dose, chemotherapy and risk of soft tissue sarcoma after solid tumours during childhood.
  • Soft tissue sarcoma (STS) is one of the most frequent second primary cancer that occurs during the first 20 years following treatment for a solid cancer in childhood.
  • Our aim was to quantify the risk of STS as a second malignant neoplasm and to investigate its relationship with radiotherapy and chemotherapy.
  • A cohort study of 4,400 3-year survivors of a first solid cancer diagnosed during childhood in France or the United Kingdom, between 1942 and 1985, was followed 15 years on average.
  • In a partially nested case-control study, we matched 25 cases of STS and 121 controls for sex, type of first cancer, age at first cancer and duration of follow-up.
  • Sixteen STS occurred in the cohort, as compared to 0.3 expected from the general population (Standardized Incidence Radio, SIR = 54 (95%CI: 34-89)).
  • The SIR was 113 (95% CI: 62-185) after chemotherapy plus radiotherapy (13 STS), whereas it was 28 (95%CI: 2-125) after chemotherapy alone (1 STS) and 19 (95%CI: 3-60) after radiotherapy alone (2 STS).
  • After adjustment for treatment, there was no evidence of variation in the annual excess of incidence or in the SIR with either age at first cancer or time since 1st cancer.
  • In the case-control study, the risk of a STS was increased with the square of the dose of radiation to the site of STS development and with the administration of Procarbazine.
  • The increased risk of soft tissue sarcoma that occurred after childhood cancer is independently related to exposure to radiotherapy and Procarbazine.
  • A closer surveillance of children treated with this treatment combination is strongly recommended.
  • [MeSH-major] Antineoplastic Agents / adverse effects. Neoplasms / therapy. Neoplasms, Second Primary / etiology. Radiotherapy Dosage. Sarcoma / etiology
  • [MeSH-minor] Adolescent. Adult. Case-Control Studies. Child. Child, Preschool. Cohort Studies. Combined Modality Therapy. Humans. Middle Aged. Risk

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15054872.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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2. Sadighi S, Raafat J: Sarcoma in Iran. Asian Pac J Cancer Prev; 2003 Apr-Jun;4(2):95-8
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  • [Title] Sarcoma in Iran.
  • PURPOSE: To review the clinical characteristics of 1470 sarcoma cases and to define the factors in patients that predict out come, relapse and survival.
  • METHODS: Retrospective analysis of the database for the period 1991-2002, focusing on demographic, tumor related and treatment related variables, relapse free survival (RFS) and overall survival (OS) using the Kaplan- Meier method.
  • The bone to soft tissue sarcoma ratio was 3/1 in children and 1/3 in adults.
  • Osteosarcoma, Ewing's tumours and rhabdomyosarcomas accounted for 83% of childhood tumors.
  • Mean follow up time was 56 months.
  • Of the total, 25% had initial metastasis, 86% received chemotherapy and 41 % underwent radiotherapy.
  • The main prognostic factors for survival were tumor size, margin of surgery, neurovascular involvement in the pathological report, initial metastasis and no complete response to first therapy.
  • Adjuvant radiotherapy, small tumor size, curative surgery with chemotherapy and free surgical margins were significantly associated with reduced recurrence.
  • A complete response to primary therapy is the main independent variable for overall survival.
  • [MeSH-major] Sarcoma

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  • (PMID = 12875619.001).
  • [ISSN] 1513-7368
  • [Journal-full-title] Asian Pacific journal of cancer prevention : APJCP
  • [ISO-abbreviation] Asian Pac. J. Cancer Prev.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Thailand
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3. Bisogno G, Sotti G, Nowicki Y, Ferrari A, Garaventa A, Zanetti I, Favre C, Schiavetti A, Tamaro P, Carli M: Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group. Cancer; 2004 Apr 15;100(8):1758-65
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  • [Title] Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group.
  • BACKGROUND: Survivors of childhood malignancies have an increased risk of developing second malignant neoplasms (SMN) due to their prior treatment and/or genetic susceptibility.
  • A small proportion of SMNs are soft tissue sarcomas (STS), whose prognosis is generally thought to be poor, though publications on such patients' treatment and outcome is limited.
  • METHODS: The authors analyzed 25 patients who were registered for the Italian Cooperative Group protocols for pediatric STS from 1979 to 2000.
  • The primary tumor was STS in five patients; Hodgkin disease in five patients; leukemia in four patients; retinoblastoma, neuroblastoma, and Wilms tumor in two patients each; and other tumor types in five patients.
  • SMNs occurred after a median of 8 years (range, 1.9-15.0 years) and included rhabdomyosarcoma (RMS) in 4 patients, malignant peripheral nerve sheath tumor in 4 patients, extraosseous Ewing family tumor (EFT) in 4 patients, leiomyosarcoma in 3 patients, fibrosarcoma in 2 patients, synovial sarcoma in 2 patients, and other tumor types in 6 patients.
  • Treatment generally was administered according to the guidelines for primary STS.
  • RESULTS: Seven non-RMS patients with STS underwent surgery alone, whereas 18 patients received chemotherapy and 8 patients received radiotherapy.
  • Fifteen patients were alive in complete remission of their SMN at the time of last follow-up.
  • Responses to chemotherapy and survival were satisfactory for patients with tumors such as RMS and EFT.
  • Complete tumor resection was correlated with a favorable prognosis in patients with other types of STS and in patients with postirradiation sarcoma.
  • Two patients developed a third malignancy.
  • CONCLUSIONS: Although prior treatment may hinder the management of these patients, pediatric STS second malignancies can be cured using the same strategies used for de novo pediatric sarcomas.
  • Long-term follow-up is mandatory given the risks of further malignancies and more severe, treatment-related side effects.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neoplasms, Second Primary. Sarcoma / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Prognosis. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright 2004 American Cancer Society.
  • (PMID = 15073867.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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4. Inskip PD, Curtis RE: New malignancies following childhood cancer in the United States, 1973-2002. Int J Cancer; 2007 Nov 15;121(10):2233-40
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  • [Title] New malignancies following childhood cancer in the United States, 1973-2002.
  • The objectives of our study were to quantify risks for developing new malignancies among childhood cancer survivors, identify links between particular types of first and subsequent cancer, and evaluate the possible role of treatment.
  • A cohort of 25,965 2-month survivors of childhood cancer diagnosed in the U.S. during 1973-2002 was identified and followed through SEER cancer registries.
  • Observed-to-expected ratios (O/E) were calculated, and Poisson regression was used to compare risks among treatment groups.
  • Childhood cancer survivors were at nearly 6-fold risk of developing a new cancer relative to the general population (O/E = 5.9, 95% CI: 5.4-6.5).
  • Most common were subsequent primary cancers of the female breast, central nervous system, bone, thyroid gland and soft tissue, as well as cutaneous melanoma and acute non-lymphocytic leukemia (ANLL).
  • The greatest risks of subsequent cancers occurred among patients diagnosed previously with Hodgkin lymphoma (HL), Ewing sarcoma, primitive neuroectodermal tumor, or retinoblastoma.
  • Risk of subsequent solid cancers was higher among persons whose initial treatment for childhood cancer included radiotherapy, whereas the excess of subsequent ANLL was strongly related to chemotherapy.
  • The O/E for subsequent ANLL increased with increasing calendar year of initial cancer diagnosis among survivors of cancers other than HL, most likely due to increasing use of leukemogenic drugs for solid cancers and non-Hodgkin lymphoma.
  • Childhood cancer survivors are at markedly increased risk of developing a variety of new cancers relative to the general population, but the magnitude of excess risk and specific types of second cancer vary widely by type of first cancer.
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Metastasis. Risk Factors. Sex Characteristics. Time Factors. United States / epidemiology

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17557301.001).
  • [ISSN] 1097-0215
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
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5. Bhatti P, Veiga LH, Ronckers CM, Sigurdson AJ, Stovall M, Smith SA, Weathers R, Leisenring W, Mertens AC, Hammond S, Friedman DL, Neglia JP, Meadows AT, Donaldson SS, Sklar CA, Robison LL, Inskip PD: Risk of second primary thyroid cancer after radiotherapy for a childhood cancer in a large cohort study: an update from the childhood cancer survivor study. Radiat Res; 2010 Dec;174(6):741-52
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  • [Title] Risk of second primary thyroid cancer after radiotherapy for a childhood cancer in a large cohort study: an update from the childhood cancer survivor study.
  • Previous studies have indicated that thyroid cancer risk after a first childhood malignancy is curvilinear with radiation dose, increasing at low to moderate doses and decreasing at high doses.
  • Understanding factors that modify the radiation dose response over the entire therapeutic dose range is challenging and requires large numbers of subjects.
  • We quantified the long-term risk of thyroid cancer associated with radiation treatment among 12,547 5-year survivors of a childhood cancer (leukemia, Hodgkin lymphoma and non-Hodgkin lymphoma, central nervous system cancer, soft tissue sarcoma, kidney cancer, bone cancer, neuroblastoma) diagnosed between 1970 and 1986 in the Childhood Cancer Survivor Study using the most current cohort follow-up to 2005.
  • Other factors such as sex, type of first cancer, attained age, age at exposure to radiation, time since exposure to radiation, and chemotherapy (yes/no) were assessed for their effect on the linear and exponential quadratic terms describing the dose-response relationship.
  • Similar to the previous analysis, thyroid cancer risk increased linearly with radiation dose up to approximately 20 Gy, where the relative risk peaked at 14.6-fold (95% CI, 6.8-31.5).
  • At thyroid radiation doses >20 Gy, a downturn in the dose-response relationship was observed.
  • We found that age at exposure modified the ERR linear dose term (higher radiation risk with younger age) (P < 0.001) and that sex (higher radiation risk among females) (P  =  0.008) and time since exposure (higher radiation risk with longer time) (P < 0.001) modified the EAR linear dose term.
  • Sex, age at exposure and time since exposure were found to be significant modifiers of the radiation-related risk of thyroid cancer and as such are important factors to account for in clinical follow-up and thyroid cancer risk estimation among childhood cancer survivors.

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  • (PMID = 21128798.001).
  • [ISSN] 1938-5404
  • [Journal-full-title] Radiation research
  • [ISO-abbreviation] Radiat. Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U24 CA055727; United States / Intramural NIH HHS / / Z01 CP010131-13; United States / NCI NIH HHS / CA / U24 CA55727
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS269726; NLM/ PMC3080023
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6. Womer RB, Pressey JG: Rhabdomyosarcoma and soft tissue sarcoma in childhood. Curr Opin Oncol; 2000 Jul;12(4):337-44
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  • [Title] Rhabdomyosarcoma and soft tissue sarcoma in childhood.
  • The treatment of the primary tumor in rhabdomyosarcoma has been reexamined, with the roles of surgery and radiation expanding in American studies and decreasing in European ones.
  • "Megatherapy" approaches with stem cell or bone marrow autologous transplants still have not found a role in the treatment of metastatic rhabdomyosarcoma.
  • Our understanding of the natural history of nonrhabdo soft tissue sarcomas in children has increased, and molecular diagnosis is becoming established.
  • The role of chemotherapy in treatment remains controversial.
  • [MeSH-major] Rhabdomyosarcoma / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 10888419.001).
  • [ISSN] 1040-8746
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 34
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7. Brecht IB, Treuner J: [Soft tissue sarcoma in children and adolescents: experiences of the cooperative Soft Tissue Sarcoma Group Studies (CWS-81 - 96)]. Handchir Mikrochir Plast Chir; 2004 Oct;36(5):275-81
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  • [Title] [Soft tissue sarcoma in children and adolescents: experiences of the cooperative Soft Tissue Sarcoma Group Studies (CWS-81 - 96)].
  • The very heterogeneous group of paediatric soft tissue sarcomas account for approximately 7 % of all malignant childhood tumours.
  • The prognosis and biology of soft tissue sarcomas in children and adolescents vary greatly depending on histological subtype, the age of the patient, the primary site, the tumour size, tumour invasiveness and the extent of disease at diagnosis.
  • Since 1981, 2918 children and adolescents with soft tissue sarcomas were treated prospectively according to the common treatment protocols of the Cooperative Soft Tissue Sarcoma Study Group (CWS-81 - 96).
  • The multimodal treatment includes the use of surgery, chemotherapy and radiotherapy and should be planned by a multidisciplinary team.
  • [MeSH-major] Sarcoma / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Protocols. Child. Child, Preschool. Combined Modality Therapy. Connective Tissue / pathology. Female. Germany. Humans. Infant. Interdisciplinary Communication. Male. Muscle, Skeletal / pathology. Neoplasm Invasiveness / pathology. Neoplasm Staging. Patient Care Team. Prognosis. Prospective Studies. Survival Rate

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  • (PMID = 15503257.001).
  • [ISSN] 0722-1819
  • [Journal-full-title] Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Mikrochirurgie der Peripheren Nerven und Gefässe : Organ der Vereinigung der Deutschen Plastischen Chirurgen
  • [ISO-abbreviation] Handchir Mikrochir Plast Chir
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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8. Ferrari A, Bisogno G, Casanova M, Meazza C, Cecchetto G, Mancini MA, Zanetti I, Alaggio R, Ninfo V, Carli M: Childhood leiomyosarcoma: a report from the soft tissue sarcoma Italian Cooperative Group. Ann Oncol; 2001 Aug;12(8):1163-8
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  • [Title] Childhood leiomyosarcoma: a report from the soft tissue sarcoma Italian Cooperative Group.
  • BACKGROUND: Only a few reports on the clinical features and management of childhood leiomyosarcoma are available.
  • To contribute additional information on the management of this rare tumor, we report on a series of 16 pediatric patients treated from 1982 to 1998 by the Soft Tissue Sarcoma Italian Cooperative Group.
  • PATIENTS AND METHODS: Primary surgery was conservative in all but two patients, and consisted of biopsy--three cases, non-radical excision--four, and radical resection--nine (involving a primary re-excision in 4 of 9).
  • In two cases secondary radical surgery was performed after primary chemotherapy.
  • Chemotherapy was administered to 9 of 16 patients, radiotherapy to three.
  • CONCLUSIONS: Complete surgical resection is the mainstay of treatment for leiomyosarcoma.
  • The role of both adjuvant chemotherapy and radiotherapy has yet to be established, and awaits cooperative multicentric studies.

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  • (PMID = 11583201.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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9. Martin RC 2nd, Brennan MF: Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival? Arch Surg; 2003 Mar;138(3):281-5
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  • [Title] Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival?
  • BACKGROUND: Ewing sarcoma (ES) is the second most common primary osseous malignancy in childhood and adolescence.
  • The improvement in survival is primarily associated with the combination of surgery and chemotherapy.
  • HYPOTHESIS: Little is known about the outcome of adults with soft tissue ES or primitive neuroectodermal tumors (PNET).
  • Certain prognostic factors from soft tissue sarcomas (tumor size, tumor location, margin status, and initial presentation) in adults (>16 years) with ES/PNET will help to identify factors associated with outcome.
  • METHODS: Between July 1, 1982, and June 30, 2000, we identified 59 adult patients with primary soft tissue ES/PNET.
  • Clinicopathologic factors were correlated with the end points studied: patient factors, tumor factors, pathologic factors, status of surgical margins, adjuvant chemotherapy, and radiation therapy.
  • The median follow-up was 29 months (range, 6-222 months), with local recurrence identified in 13 patients (22%), with a median time to recurrence of 15 months (range, 5-200 months).
  • Initial presentation was the only predictor of long-term survival, with primary tumor-only presentation having a 5-year survival of 60% (median not reached) compared with primary tumor plus metastatic disease having a 5-year survival of 33% (median, 17 months) (P =.02).
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / mortality. Sarcoma, Ewing / mortality. Soft Tissue Neoplasms / mortality
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local

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  • (PMID = 12611575.001).
  • [ISSN] 0004-0010
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. Modritz D, Ladenstein R, Pötschger U, Amman G, Dieckmann K, Horcher E, Urban C, Meister B, Schmitt K, Jones R, Kaulfersch W, Haas H, Moser R, Stöllinger O, Peham M, Gadner H, Koscielniak E, Treuner J: Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study. Wien Klin Wochenschr; 2005 Mar;117(5-6):196-209
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  • [Title] Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study.
  • OBJECTIVE: The aim of the CWS 96 Study was to achieve an optimal treatment in children and adolescents with soft tissue sarcoma (STS) implementing a further refinement of risk-adapted allocation to chemotherapy, surgery and radiotherapy.
  • METHODS: Treatment stratification was based on tumour histology, TNM status, postsurgical stage, localisation and age.
  • 59/89 patients had localised RMS-like (rhabdomayosarcoma) STS (EFS 73% +/- 7%), 14 had localised NON-RMS STS (EFS 54% +/- 16%) and 15 patients had metastatic disease at diagnosis (EFS 33% +/- 12%), 1 patient had fibromatosis.
  • Favourable primary tumour sites of nonmetastatic RMS-like STS i.e. orbit, head/neck nonparameningeal or genitourinary non-bladder/prostate were diagnosed in 15 patients (1/15 patients died).
  • The 3 year EFS according to histology in patients with RMS-like STS was 61% +/- 11% for RME (embryonal RMS ) (28 patients) and 71% +/- 15% for RMA (alveolar RMS) (10 patients).
  • The most common treatment failure was local relapse occurring in 21% of patients in the high-risk group.
  • CONCLUSION: Risk-adapted individualisation of treatment led to a reduction of chemotherapy in the low and standard risk group without compromising survival.
  • These preliminary results after a median observation time of 2.5 years confirm the CWS 96 strategy.
  • [MeSH-major] Risk Assessment / methods. Sarcoma / mortality. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Adult. Austria / epidemiology. Child. Child, Preschool. Cohort Studies. Disease-Free Survival. Female. Humans. Incidence. Infant. Infant, Newborn. Male. Prognosis. Risk Factors. Survival Analysis. Treatment Outcome

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  • [CommentIn] Wien Klin Wochenschr. 2005 Mar;117(5-6):176-9 [15875755.001]
  • (PMID = 15875759.001).
  • [ISSN] 0043-5325
  • [Journal-full-title] Wiener klinische Wochenschrift
  • [ISO-abbreviation] Wien. Klin. Wochenschr.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Austria
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11. Bien E, Kazanowska B, Dantonello T, Adamkiewicz-Drozynska E, Balcerska A, Madziara W, Rybczynska A, Nurzynska-Flak J, Solarz E, Kurylak A, Zalewska-Szewczyk B, Krawczyk M, Izycka-Swieszewska E, Rapala M, Koscielniak E: Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature. Ann Surg Oncol; 2010 Jul;17(7):1878-89
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  • [Title] Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature.
  • BACKGROUND: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival.
  • METHODS: A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols.
  • Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis.
  • Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses.
  • Response to systemic treatment was poor (44%) and did not prevent local and distant relapses.
  • CONCLUSIONS: The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS.
  • An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy.
  • The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.
  • [MeSH-major] Hemangioendothelioma / mortality. Hemangiopericytoma / mortality. Hemangiosarcoma / mortality. Sarcoma / mortality. Vascular Neoplasms / mortality

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  • (PMID = 20333551.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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12. Krawczuk-Rybak M, Leszczyńska E, Wysocka J, Zelazowska-Rutkowska B: [Anti-mullerian hormone in young women after chemotherapy and infradiaphragmatic radiotherapy for childhood cancer]. Pediatr Endocrinol Diabetes Metab; 2008;14(2):99-103
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  • [Title] [Anti-mullerian hormone in young women after chemotherapy and infradiaphragmatic radiotherapy for childhood cancer].
  • INTRODUCTION: Composed anticancer treatment leads to different late effects, such as ovarian failure, causing infertility or premature menopause.
  • AIM OF THE STUDY: was to analyse ovarian function, particularly anti-mullerian hormone levels, in young females after anticancer treatment.
  • PATIENTS AND METHODS: We analysed FSH, LH, estradiol and anti-mullerian hormone (AMH) levels on days 3-5 of a menstrual cycle in thirty three cancer survivors in mean age 19.1+/-4.7 years treated in age 12.0+/-5.6 years for Hodgkin Lymphoma (HL) (n=16), nephroblastoma (n=7), soft tissue sarcoma (n=4), germinal tumor (n=3), neuroblastoma (n=2), histiocytosis (n=1).
  • Particular analysis of all cases showed higher (>2 SD) FSH levels in 8 patients: 5 patients treated for HL with radiotherapy and higher total doses of procarbazine, nitrogen mustard and vinblastine; 2 patients treated for soft tissue sarcoma and one patient for Wilms tumor (all received radiotherapy).
  • Lowered AMH levels were found in 8 patients treated with chemo- and radiotherapy (4 - for HL, 2 - for Wilms tumor and 2 - for soft tissue sarcoma).
  • CONCLUSION: Composed anticancer treatment, especially radiotherapy, leads to ovarian failure.
  • All causes and first symptoms of ovary damage should be known to the doctors who take care of the patients after anticancer treatment.
  • [MeSH-major] Anti-Mullerian Hormone / metabolism. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Infertility, Female / diagnosis. Menopause, Premature. Primary Ovarian Insufficiency / diagnosis. Radiotherapy / adverse effects
  • [MeSH-minor] Adolescent. Adult. Combined Modality Therapy / adverse effects. Female. Follicle Stimulating Hormone / metabolism. Hodgkin Disease / drug therapy. Hodgkin Disease / radiotherapy. Humans. Neoplasms / therapy. Ovary / drug effects. Ovary / radiation effects. Survivors


13. Bien E, Rapala M, Krawczyk M, Balcerska A: The serum levels of soluble interleukin-2 receptor alpha and lactate dehydrogenase but not of B2-microglobulin correlate with selected clinico-pathological prognostic factors and response to therapy in childhood soft tissue sarcomas. J Cancer Res Clin Oncol; 2010 Feb;136(2):293-305
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  • [Title] The serum levels of soluble interleukin-2 receptor alpha and lactate dehydrogenase but not of B2-microglobulin correlate with selected clinico-pathological prognostic factors and response to therapy in childhood soft tissue sarcomas.
  • PURPOSE: To establish the clinical utility of serum soluble IL-2 receptor (sIL-2R alpha), lactate dehydrogenase (LDH) [corrected] and B2-microglobulin [corrected] (B2-M) as markers for diagnosis, prognosis and treatment monitoring in childhood soft tissue sarcomas (STS).
  • METHODS: The markers[corrected] were measured prospectively before treatment, in complete remission (CR) during and after therapy and at relapse [corrected] in 35 children with STS and in 50 healthy children [corrected] (once).
  • Pre-treatment levels [corrected] of sIL-2R alpha and LDH [corrected] but not of B2-M exceeded significantly those of controls.
  • [corrected] Elevated [corrected] sIL-2R alpha levels correlated with more [corrected] advanced stages, poor-risk histology and poor response to chemotherapy, higher LDH with incomplete primary tumour [corrected] resection and increased B2-M with poor-risk histology.
  • [corrected] Good response to chemotherapy was paralleled by significant decline of sIL-2R alpha and LDH pre-treatment levels while relapse--by sIL-2R alpha and LDH increase to values similar to those at diagnosis.
  • [corrected] CONCLUSIONS: sIL-2R alpha and LDH were [corrected] proven to be promising markers [corrected] for diagnosis and treatment monitoring in children with STS.
  • The markers [corrected] correlated also with some [corrected] important prognostic clinico-pathological factors for childhood [corrected] STS; however, they [corrected] failed to predict EFS and OS.
  • Measurements of serum [corrected] B2-M were shown [corrected] to have no clinical value in the diagnostics, prognostics and treatment monitoring in paediatric STS.
  • [MeSH-major] Biomarkers, Tumor / blood. Interleukin-2 Receptor alpha Subunit / blood. L-Lactate Dehydrogenase / blood. Sarcoma / diagnosis. Sarcoma / therapy. beta 2-Microglobulin / blood
  • [MeSH-minor] Adolescent. Age Factors. Case-Control Studies. Child. Child, Preschool. Enzyme-Linked Immunosorbent Assay. Female. Humans. Male. Predictive Value of Tests. Prognosis. Prospective Studies. Recurrence. Remission Induction. Risk Assessment. Risk Factors. Treatment Outcome

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  • [ErratumIn] J Cancer Res Clin Oncol. 2010 Feb;136(2):307
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  • (PMID = 19693535.001).
  • [ISSN] 1432-1335
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Interleukin-2 Receptor alpha Subunit; 0 / beta 2-Microglobulin; EC 1.1.1.27 / L-Lactate Dehydrogenase
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14. McCarville MB, Christie R, Daw NC, Spunt SL, Kaste SC: PET/CT in the evaluation of childhood sarcomas. AJR Am J Roentgenol; 2005 Apr;184(4):1293-304
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  • [Title] PET/CT in the evaluation of childhood sarcomas.
  • OBJECTIVE: Our objective was to review our preliminary experience with PET/CT in evaluating childhood sarcomas including rhabdomyosarcoma (n = 28), the Ewing's sarcoma family of tumors (n = 14), nonrhabdomyosarcoma soft-tissue sarcoma (n = 9), osteosarcoma (n = 8), chondrosarcoma (n = 1), and embryonal sarcoma (n = 1).
  • CONCLUSION: We found PET/CT useful in depicting an unknown primary rhabdomyosarcoma and detecting unsuspected and unusual metastatic sites of childhood sarcomas.
  • It was useful in monitoring response to chemotherapy, radiation therapy, and radiofrequency ablation and aided the postoperative evaluation of tumor resection sites.
  • [MeSH-major] Positron-Emission Tomography. Sarcoma / radiography. Tomography, X-Ray Computed

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  • [CommentIn] AJR Am J Roentgenol. 2006 Feb;186(2):581; author reply 581-2 [16423975.001]
  • (PMID = 15788613.001).
  • [ISSN] 0361-803X
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-21765; United States / NCI NIH HHS / CA / CA-23099
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Number-of-references] 8
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15. Jakubcová T, Jakubec P: [Primary pulmonary sarcomas]. Klin Onkol; 2009;22(4):139-53
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  • [Title] [Primary pulmonary sarcomas].
  • Primary pulmonary sarcomas are rare diseases unlike lung carcinomas.
  • They are flowing from the soft tissue of lung.
  • Their morfologic structure does not digger from the sarcomas of soft tissue.
  • The primary pulmonary sarcomas occur more often in childhood and in young people unlike lung carcinomas.
  • Some gene mutations, infectious pathoghens and contraception have a possible impact on the origin of some types of the sarcomas.
  • The basic diagnostic problem is exclusion of a secondary origin of sarcomatic cells in the lung, because pulmonary metastasis of extrapulmonary sarcomas are more often than the primary pulmonary involvement.The optimal treatment is a resection of the tumour.The other therapeutic modalities are radiotherapy and chemotherapy, but results of these modalities are unsatisfactory.
  • Problems of the chemotherapy are high toxicity and relatively low curative effect about 20%.The first studies with biological treatment of the sarcomas of soft tissue have been published recently.This types of drugs could be a part of the complex management of these primary pulmonary tumours in the future.
  • The primary pulmonary sarcomas have mostly aggresive course and often recur.
  • Prognostic factors are the size of tumour, histological type, grading, clinical stage and measure of a surgery major.
  • [MeSH-major] Lung Neoplasms. Sarcoma

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  • (PMID = 19731876.001).
  • [ISSN] 0862-495X
  • [Journal-full-title] Klinická onkologie : casopis Ceské a Slovenské onkologické spolecnosti
  • [ISO-abbreviation] Klin Onkol
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 68
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16. Lahl M, Fisher VL, Laschinger K: Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship. Clin J Oncol Nurs; 2008 Feb;12(1):89-97
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  • [Title] Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship.
  • The Ewing's sarcoma family of tumors (ESFT) is a malignant primary bone tumor often involving soft tissue that affects not only children but also young adults.
  • Since 1992, with the addition of ifosfamide and etoposide to standard chemotherapy for primary tumors, much improvement has been made in the treatment of ESFT, with a primary focus on children.
  • Though often recognized as a childhood cancer, it can affect individuals into the middle years of their lives, but little is known about the outcomes of adults with ESFT.
  • ESFT, which includes Ewing's sarcoma, extraosseous Ewing's sarcoma, Askin tumor, and primitive neuroectodermal tumor, is the second most common primary malignant bone tumor in children and adolescents.
  • It accounts for 10% of primary malignant bone tumors in children and 3% of all childhood malignancies.
  • Treatment for ESFT consists of a multimodal approach, including chemotherapy, radiation therapy, and surgery.
  • Children and young adults with Ewing's sarcoma face many physical challenges from their illness and the complications of their treatments.
  • [MeSH-major] Bone Neoplasms. Sarcoma, Ewing
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy / adverse effects. Combined Modality Therapy / nursing. Humans. Incidence. Nurse's Role. Oncology Nursing / organization & administration. Prognosis. Radiotherapy, Adjuvant. Risk Factors. Stem Cell Transplantation. Survival Rate. Treatment Outcome

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  • (PMID = 18258578.001).
  • [ISSN] 1092-1095
  • [Journal-full-title] Clinical journal of oncology nursing
  • [ISO-abbreviation] Clin J Oncol Nurs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 25
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17. Guérin S, Hawkins M, Shamsaldin A, Guibout C, Diallo I, Oberlin O, Brugières L, de Vathaire F: Treatment-adjusted predisposition to second malignant neoplasms after a solid cancer in childhood: a case-control study. J Clin Oncol; 2007 Jul 1;25(19):2833-9
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  • [Title] Treatment-adjusted predisposition to second malignant neoplasms after a solid cancer in childhood: a case-control study.
  • PURPOSE: Previous therapy, genetic susceptibility, and the type of first malignant neoplasm (FMN) are known to be associated with the risk of second malignant neoplasm (SMN) among patients treated for a childhood cancer.
  • PATIENTS AND METHODS: A case-control study nested in a European cohort of 4,581 patients treated for a solid cancer during childhood was conducted.
  • One hundred forty-six patients with an SMN and 417 controls were matched for sex, age at FMN, chemotherapy, radiotherapy, the local radiation dose received at the site of SMN for patient cases and at the same site for the matched controls, and follow-up.
  • RESULTS: A significantly increased risk of developing any SMN was observed after Hodgkin's lymphoma, retinoblastoma, malignant bone tumor, soft tissue sarcoma (STS), and germ cell tumor as FMN, after adjustment for chemotherapy and family cancer syndrome.
  • No significant risk of developing a carcinoma was observed among patients who had developed Hodgkin's lymphoma as FMN.
  • A significantly increased risk of developing a sarcoma was observed among patients who had developed a retinoblastoma (adjusted odds ratio [ORa] = 7.5; 95% CI, 1.2 to 46), a malignant bone tumor (ORa = 13.3; 95% CI, 1.5 to 117), an STS (ORa = 4.8; 95% CI, 1.3 to 18), or a carcinoma (ORa = 9.4; 95% CI, 1.1 to 82) as FMN.
  • CONCLUSION: Survivors of Hodgkin's lymphoma, retinoblastoma, malignant bone tumor, STS, and germ cell tumor should receive close surveillance because they are at increased risk of developing any SMN.
  • [MeSH-major] Hodgkin Disease / therapy. Neoplasms / drug therapy. Neoplasms / radiotherapy. Neoplasms, Germ Cell and Embryonal / therapy. Neoplasms, Second Primary / etiology. Retinoblastoma / therapy. Sarcoma / therapy


18. Schultze-Mosgau S, Thorwarth M, Wehrhan F, Holter W, Stachel KD, Grabenbauer G, Amann K, Beck JD: Ewing sarcoma of the mandible in a child: interdisciplinary treatment concepts and surgical reconstruction. J Craniofac Surg; 2005 Nov;16(6):1140-6
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  • [Title] Ewing sarcoma of the mandible in a child: interdisciplinary treatment concepts and surgical reconstruction.
  • Ewing's sarcoma is the second most common primary bone malignancy in childhood and adolescence.
  • We present a standardized interdisciplinary treatment protocol according to the EURO-E.W.I.N.G.
  • 99 study, applied in the treatment of a 7-year-old patient with localized Ewing's sarcoma of the left mandible.
  • After six blocks of VIDE (vincristine/ifosfamide/doxorubicin/etoposide) chemotherapy and stem cells rescue, intensity modulated external radiation with 48.6 Gy and subsequent high dose therapy with busulphan-melphalan were administered.
  • Because of the effective neoadjuvant treatment, no extensive soft tissue resection was necessary.
  • The presented case underlines the requirement for multidisciplinary protocols involving radiologists, pathologists, oncologists, radiation oncologists, and surgeons for accurate diagnosis and appropriate therapy.
  • [MeSH-major] Mandibular Neoplasms / surgery. Sarcoma, Ewing / surgery
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Transplantation. Child. Esthetics. Follow-Up Studies. Humans. Male. Microsurgery. Neoadjuvant Therapy. Peripheral Blood Stem Cell Transplantation. Radiotherapy Dosage. Reconstructive Surgical Procedures. Skin Transplantation. Surgical Flaps. Treatment Outcome. Wound Healing / physiology

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  • (PMID = 16327573.001).
  • [ISSN] 1049-2275
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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19. Kebudi R, Görgün O, Ayan I: Oral etoposide for recurrent/progressive sarcomas of childhood. Pediatr Blood Cancer; 2004 Apr;42(4):320-4
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  • [Title] Oral etoposide for recurrent/progressive sarcomas of childhood.
  • BACKGROUND: Etoposide (VP-16) is a topoisomerase II inhibitor that is effective in a broad spectrum of pediatric and adult malignancies.
  • PROCEDURE: Twenty-one children (10 girls and 11 boys) with R/P sarcomas and a median age of 11 years (range 3-16 years) were enrolled in this study.
  • The diagnosis was Ewing sarcoma family tumor (ESFT) in seven, osteosarcoma in eight, rhabdomyosarcoma in four, clear cell sarcoma of soft tissue in one, fibrosarcoma in one patient.
  • They are alive with no evidence of disease (NED) 79 and 94 months from time of relapse/progressive disease (PD).
  • A patient developed acute myeloid leukemia and died.
  • CONCLUSIONS: Oral VP-16 therapy is simple, relatively nontoxic, and does not necessitate hospitalization.
  • Given the risk of second malignancy, especially in children with previous exposure to topoisomerase II inhibitors and alkylating agents, this regimen may be used as a palliative treatment or in patients with poor prognosis.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / administration & dosage. Etoposide / administration & dosage. Sarcoma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Child. Child, Preschool. Disease Progression. Female. Humans. Male. Neoplasms, Second Primary / chemically induced. Palliative Care. Recurrence. Remission Induction. Survival Rate. Treatment Outcome

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 14966827.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide
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20. Sagar TG, Chandra A, Raman SG, Swaminathan R: Paucity of hematological neoplasia after treatment of Hodgkin disease: observation after long-term follow-up at Cancer Institute, Chennai, south India. Pediatr Hematol Oncol; 2002 Apr-May;19(3):197-203
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  • [Title] Paucity of hematological neoplasia after treatment of Hodgkin disease: observation after long-term follow-up at Cancer Institute, Chennai, south India.
  • The purpose of the study was to evaluate the incidence of second malignancies in childhood Hodgkin disease treated with COPP and COPP/ABV (replacing mechlorethamine by cyclophosphamide) chemotherapy.
  • All 5 were solid tumors: one each of soft tissue sarcoma, dermatofibrosarcoma, micropapillary carcinoma of thyroid, malignant phylloides tumor of breast, and chondrosarcoma of ilium.
  • All patients had received combination chemotherapy and radiotherapy.
  • All these patients had advanced stage of cancer and were 7-14 years of age at the time of diagnosis of first primary Hodgkin disease.
  • COPP and COPP/ABV are effective therapeutic regimens.
  • The paucity of secondary hematological malignancies is unique in this series and may be attributed to the substitution of nitrogen mustard with cyclophosphamide in the chemotherapy combination.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Hematologic Neoplasms / chemically induced. Hodgkin Disease / drug therapy
  • [MeSH-minor] Bleomycin / administration & dosage. Child, Preschool. Cyclophosphamide / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Female. Humans. Incidence. Infant. Infant, Newborn. Male. Neoplasms, Second Primary / chemically induced. Neoplasms, Second Primary / prevention & control. Prednisone / administration & dosage. Procarbazine / administration & dosage. Survival Rate. Treatment Outcome. Vinblastine / administration & dosage. Vincristine / administration & dosage

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  • (PMID = 11936733.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 5V9KLZ54CY / Vinblastine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; CVPPABO protocol
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21. Gunes D, Uysal KM, Cetinkaya H, Tekin HG, Yuceer N, Sarialioglu F, Olgun N: Paravertebral malignant tumors of childhood: analysis of 28 pediatric patients. Childs Nerv Syst; 2009 Jan;25(1):63-9
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  • [Title] Paravertebral malignant tumors of childhood: analysis of 28 pediatric patients.
  • PURPOSE: To evaluate the clinical features and treatment results of the primary paravertebral malignant tumors (PMTs) in our department.
  • METHODS: Medical records of 28 children with primary PMTs treated between 1988-2007 were analyzed retrospectively.
  • RESULTS: Primary PMTs constituted 4.8% of the cancer cases in our department.
  • Tumor diagnoses were mostly neuroblastoma (46.4%) and soft tissue sarcomas (35.7%).
  • Seven were managed by surgical departments by primary surgery (three unresponsive).
  • Others were managed by pediatric oncology: five with corticosteroids+/-chemotherapy (one unresponsive), one with radiotherapy (RT), and two with surgery for the clinical CC.
  • Surgery was tumor excision in nine, laminectomy in nine, laminotomy in one, and delayed surgery after chemotherapy in two cases.
  • In chemotherapy and surgery groups, there were neurologic sequela associated with the advanced disease at diagnosis in 38% and 37%, respectively.
  • The CC caused by PMTs should be initially managed with corticosteroids +/- chemotherapy to avoid the adverse late effects of RT and surgery.
  • [MeSH-major] Neuroblastoma / therapy. Spinal Cord Neoplasms / therapy. Spinal Neoplasms / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Infant. Male. Recovery of Function / physiology. Retrospective Studies. Sarcoma / therapy. Treatment Outcome

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  • (PMID = 18843494.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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22. Liu AX, Zhou JH, Jin HM, Zhu CK, Cheng XD: Primary rhabdomyosarcoma of urethra in a 5-year-old girl: case report and literature review. Urology; 2007 Jun;69(6):1208.e17-9
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  • [Title] Primary rhabdomyosarcoma of urethra in a 5-year-old girl: case report and literature review.
  • Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood, while RMS from the urinary tract has rarely been reported.
  • Aspects of the manifestation, diagnosis, and treatment of these tumors are discussed in the case of a girl with rapidly progressive RMS from the urethral tract.
  • She was treated with a two-stage surgical procedure and chemotherapy.
  • At the latest follow-up visit at 18 months after treatment, the patient had no evidence of disease on clinical examination or imaging studies.
  • The present case underscores that careful history, physical examination, and laboratory tests should be performed, in additional to using adequate tissue for routine pathologic examination, before making the diagnosis.
  • A combined approach to treating RMS using multidrug chemotherapy and surgery has markedly improved survival.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Urethral Neoplasms / diagnosis. Urethral Neoplasms / therapy
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Female. Humans. Immunohistochemistry. Treatment Outcome. Urologic Surgical Procedures. Vincristine / administration & dosage

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  • (PMID = 17572220.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide
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23. Hosoi H, Tsuchiya K, Sugimoto T: [Rhabdomyosarcoma]. Gan To Kagaku Ryoho; 2007 Feb;34(2):181-6
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  • Rhabdomyosarcoma (RMS) is the most common type of soft-tissue sarcoma in childhood.
  • In Europe and the United States, survival rates of RMS patients, especially those with local or regional RMS, have dramatically improved from around 25% in 1970 to over 70% in 2001 as a result of the introduction of multimodality therapy and cooperative group trials.
  • RMS patients require multimodal therapy including appropriate surgery at the primary site, histological diagnosis supported by molecular genetics, and a combination of chemotherapy and radiation therapy based on their risk group.
  • An analysis of registration data from the Japanese Society for Pediatric Surgery as well as our recent retrospective study indicates that the survival rates of each risk group are all lower than those in the US or Europe, even in the "low-risk" group.
  • We have recently established the Japan Rhabdomyosarcoma Study Group (JRSG) and continued several studies in each risk group to improve the outcome and to establish a standard therapy in Japan.
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Dactinomycin / administration & dosage. Drug Administration Schedule. Humans. Lymph Nodes / pathology. Lymphatic Metastasis. Neoplasm Staging / classification. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate. Vincristine / administration & dosage

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  • (PMID = 17301524.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine
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24. Walterhouse D, Watson A: Optimal management strategies for rhabdomyosarcoma in children. Paediatr Drugs; 2007;9(6):391-400
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Rhabdomyosarcoma is the most common sarcoma of childhood.
  • The fact that the tumor arises in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph node spread, and therapeutic response, requires physicians to be familiar with site-specific staging and treatment details.
  • In addition, rhabdomyosarcoma requires multimodality therapy that can be associated with significant acute toxicities and long-term effects, particularly when administered to young children.
  • Appropriate management begins with establishing the correct pathologic diagnosis, histologic subtype, primary site, extent of disease (International Society of Pediatric Oncology [SIOP]-TNM-Union Internationale Contre le Cancer stage or Intergroup Rhabdomyosarcoma Study Group [IRSG] stage), and extent of resection (IRSG group).
  • Cooperative groups throughout North America and Europe have defined risk-adapted treatment based on these factors; this treatment requires a coordinated management plan that includes surgery, chemotherapy, and usually radiotherapy.
  • The surgical approach for rhabdomyosarcoma is to excise the primary tumor whenever possible without causing major functional or cosmetic deficits.
  • Wide excision is difficult in some primary sites and can be complicated by the fact that the tumor grows in a locally infiltrative manner so that complete resection is often neither possible nor medically indicated.
  • The cooperative groups reduce the dose of radiation based on the response of the tumor to chemotherapy and delayed primary resection to differing degrees.
  • All patients with rhabdomyosarcoma require chemotherapy.
  • Risk-adapted treatment involves reducing or eliminating the alklyating agent for patients with the most favorable disease characteristics.
  • Acute and long-term toxicities associated with these chemotherapy regimens include myelosuppression, febrile neutropenia, hepatopathy, infertility, and second malignant neoplasms.
  • It is hoped that newer therapies directed at specific molecular genetic defects will benefit all patients with rhabdomyosarcoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Rhabdomyosarcoma. Soft Tissue Neoplasms
  • [MeSH-minor] Child. Combined Modality Therapy. Diagnosis, Differential. Dose Fractionation. Humans. Treatment Outcome

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  • (PMID = 18052409.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 58
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25. Jeon IS, Yi DY: Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus. Pediatr Hematol Oncol; 2009 Mar;26(2):85-8
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  • Acute lymphoblastic leukemia (ALL), a primary hematologic malignancy that is especially common in childhood, occurs relatively rarely as a secondary malignant neoplasm.
  • Available data indicate that ALL often follows chemoradiotherapy for soft tissue sarcoma.
  • Perivascular epithelioid tumor (PEComa), a primitive mesenchymal tissue origin, can be classified as a soft tissue sarcoma.
  • An 11-year-old girl was diagnosed with ALL secondary to chemoradiotherapy (vincristine, ifosfamide, and anthracycline) and radiotherapy comprising 45 Gy to the whole pelvis for PEComa.
  • ALL, FAB L2, and immunophenotypically pro-B developed 16 months after the final chemotherapy treatment.
  • Herein, the authors report a case of secondary ALL that might be related to a previously used intercalating DNA topoisomerase II inhibitor (anthracycline) for a very rare sarcoma, PEComa.
  • [MeSH-major] Neoplasms, Second Primary / etiology. Perivascular Epithelioid Cell Neoplasms / complications. Precursor Cell Lymphoblastic Leukemia-Lymphoma / etiology. Uterine Neoplasms / complications

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  • (PMID = 19322738.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anthracyclines; 0 / Topoisomerase II Inhibitors
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26. Bersani F, Taulli R, Accornero P, Morotti A, Miretti S, Crepaldi T, Ponzetto C: Bortezomib-mediated proteasome inhibition as a potential strategy for the treatment of rhabdomyosarcoma. Eur J Cancer; 2008 Apr;44(6):876-84
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  • [Title] Bortezomib-mediated proteasome inhibition as a potential strategy for the treatment of rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood, divided into two major histological subtypes, embryonal (ERMS) and alveolar (ARMS).
  • To explore the possibility that the proteasome could be a target of therapeutic value in rhabdomyosarcoma, we treated several RMS cell lines with the proteasome inhibitor bortezomib (Velcade or PS-341) at a concentration of 13-26 nM.
  • RMS cells showed high sensitivity to the drug, whereas no toxic effect was observed in primary human myoblasts.
  • In conclusion, our data indicate that bortezomib could represent a novel drug against RMS tumours.
  • [MeSH-major] Boronic Acids / therapeutic use. Protease Inhibitors / therapeutic use. Proteasome Inhibitors. Pyrazines / therapeutic use. Rhabdomyosarcoma / drug therapy
  • [MeSH-minor] Angiogenesis Inhibitors / therapeutic use. Animals. Apoptosis / drug effects. Blotting, Western. Bortezomib. Cell Proliferation. Cell Survival / drug effects. Humans. Mice. Mice, Nude. Neoplasm Transplantation. Neovascularization, Pathologic / prevention & control. Transplantation, Heterologous. Tumor Cells, Cultured

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  • (PMID = 18342500.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Boronic Acids; 0 / Protease Inhibitors; 0 / Proteasome Inhibitors; 0 / Pyrazines; 69G8BD63PP / Bortezomib
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27. Weber K, Damron TA, Frassica FJ, Sim FH: Malignant bone tumors. Instr Course Lect; 2008;57:673-88
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  • Malignant bone tumors represent a small percentage of cancers nationwide and also are much less common than malignant soft-tissue tumors.
  • The rarity of the condition makes it imperative that orthopaedic surgeons in nononcologic practices are able to recognize the symptoms that suggest a possible bony malignancy to avoid inappropriate or delayed treatment.
  • The most common primary malignant bone tumors, osteosarcoma and Ewing's sarcoma, occur in childhood.
  • The primary symptom of a patient with a malignant bone tumor is pain, which often occurs at rest or at night.
  • Knowledge of specific tumor characteristics and treatment options for osteosarcoma, Ewing's sarcoma, chondrosarcoma, malignant fibrous histiocytoma, chordoma, and adamantinoma is important.
  • Patients with osteosarcoma and resectable Ewing's sarcoma are treated with chemotherapy followed by surgical resection.
  • Secondary sarcomas can occur in previously benign bone lesions and require aggressive treatment.
  • The care of patients with primary malignant bone tumors requires a multidisciplinary approach to treatment.
  • The orthopaedic oncologist is a vital member of a team composed of musculoskeletal radiologists and pathologists, radiation oncologists, medical and pediatric oncologists, and microvascular surgeons.

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  • (PMID = 18399615.001).
  • [ISSN] 0065-6895
  • [Journal-full-title] Instructional course lectures
  • [ISO-abbreviation] Instr Course Lect
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 42
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