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1. Loeb DM, Thornton K, Shokek O: Pediatric soft tissue sarcomas. Surg Clin North Am; 2008 Jun;88(3):615-27, vii

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  • [Title] Pediatric soft tissue sarcomas.
  • Soft tissue sarcomas in children are rare.
  • Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or a non-RMS soft tissue sarcoma (NRSTS).
  • Surgery is a major therapeutic modality and radiation plays a role.
  • RMS is treated with adjuvant chemotherapy, whereas chemotherapy is reserved for the NRSTS that are high grade or unresectable.
  • This review discusses the etiology, biology, and treatment of pediatric soft tissue sarcomas.
  • [MeSH-major] Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Child. Humans. Neoplasm Staging. Radiotherapy Dosage. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma, Embryonal / genetics

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  • (PMID = 18514702.001).
  • [ISSN] 0039-6109
  • [Journal-full-title] The Surgical clinics of North America
  • [ISO-abbreviation] Surg. Clin. North Am.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA006973
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 35
  • [Other-IDs] NLM/ NIHMS646294; NLM/ PMC4273573
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2. Mysorekar VV, Harish K, Kilara N, Subramanian M, Giridhar AG: Embryonal rhabdomyosarcoma of the chest wall: a case report and review of the literature. Indian J Pathol Microbiol; 2008 Apr-Jun;51(2):274-6
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  • [Title] Embryonal rhabdomyosarcoma of the chest wall: a case report and review of the literature.
  • Embryonal rhabdomyosarcoma is a soft-tissue sarcoma which has a predilection for the head and neck area, genitourinary tract and the extremities.
  • We report a rare case of embryonal rhabdomyosarcoma of the chest wall in an 8-year-old girl, presenting as a destructive tumor in the rib and clinically and radiologically mimicking Ewing's sarcoma.
  • Immunohistochemically, the positivity for muscle markers desmin and myogenin in the tumor cells proved to be useful for making a definitive diagnosis of embryonal rhabdomyosarcoma.
  • The patient is responding well to chemotherapy.
  • [MeSH-major] Rhabdomyosarcoma, Embryonal / diagnosis. Thoracic Neoplasms / diagnosis. Thoracic Wall / pathology
  • [MeSH-minor] Aneuploidy. Child. Diagnosis, Differential. Female. Humans. Sarcoma, Ewing / diagnosis

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  • (PMID = 18603708.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] India
  • [Number-of-references] 9
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3. Ferrari A, Bisogno G, Macaluso A, Casanova M, D'Angelo P, Pierani P, Zanetti I, Alaggio R, Cecchetto G, Carli M: Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1. Cancer; 2007 Apr 1;109(7):1406-12
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  • [Title] Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1.
  • BACKGROUND: Patients affected by neurofibromatosis type 1 (NF1) are at higher risk of developing soft-tissue sarcomas (STS) than the general population.
  • Two of 16 patients with evaluable disease responded to chemotherapy.
  • All 6 RMS patients were </=3 years old and had embryonal subtype, 5 of 6 arising in the genitourinary tract or pelvis (paravesical); 4 were alive in first remission at the time of the analysis, 1 was alive in second remission after a local recurrence, and 1 died of disease.
  • Cases of RMS tended to have particular features (early age, embryonal histotype, genitourinary site) and their outcome seemed to resemble that of the general RMS population.
  • [MeSH-major] Neurofibromatosis 1 / complications. Sarcoma / complications
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasms, Second Primary / complications. Neoplasms, Second Primary / diagnosis. Neoplasms, Second Primary / therapy. Nerve Sheath Neoplasms / complications. Nerve Sheath Neoplasms / diagnosis. Nerve Sheath Neoplasms / therapy. Neurofibrosarcoma / complications. Neurofibrosarcoma / diagnosis. Neurofibrosarcoma / therapy. Peripheral Nervous System Neoplasms / complications. Peripheral Nervous System Neoplasms / diagnosis. Peripheral Nervous System Neoplasms / therapy. Prognosis. Prospective Studies. Rhabdomyosarcoma / complications. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Risk Factors. Survival Rate

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  • [Copyright] (c) 2007 American Cancer Society.
  • (PMID = 17330850.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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4. Bisogno G, Pilz T, Perilongo G, Ferrari A, Harms D, Ninfo V, Treuner J, Carli M: Undifferentiated sarcoma of the liver in childhood: a curable disease. Cancer; 2002 Jan 1;94(1):252-7
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  • [Title] Undifferentiated sarcoma of the liver in childhood: a curable disease.
  • BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis.
  • METHODS: The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995.
  • They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second-look operation in cases of residual disease.
  • Eight underwent complete tumor resection either at diagnosis or after preoperative chemotherapy, and all are currently alive.
  • After initial chemotherapy tumor reduction was evident in six of nine evaluable cases.
  • Modern multimodal treatment and supportive therapy have improved survival.
  • [MeSH-major] Liver Neoplasms / therapy. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasm Staging. Remission Induction

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  • [Copyright] Copyright 2002 American Cancer Society.
  • (PMID = 11815984.001).
  • [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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5. Missaoui N, Landolsi H, Jaidene L, Anjorin A, Abdelkader AB, Yaacoubi MT, Hmissa S: Pediatric rhabdomyosarcomas in Tunisia. Asian Pac J Cancer Prev; 2010;11(5):1325-7

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  • INTRODUCTION: Rhabdomyosarcoma is the most common soft tissue sarcoma in the first two decades of life.
  • RESULTS: Rhabdomyosarcomas represented 53.6% of soft tissue sarcomas and 3.8% of all children cancer cases registered during this period.
  • The male/female ratio was 2.7 with a mean age at diagnosis of 5.9 years.
  • The embryonal subtype was the most frequent (60%) and the two most common sites of disease were the head and neck (50%) and genito-urinary tract(23.3%).
  • Chemotherapy was used in 90% of patients; 43.3% of patients had radical surgery and 26.7% of patients received radiation therapy.
  • [MeSH-major] Rhabdomyosarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Head and Neck Neoplasms / therapy. Humans. Infant. Infant, Newborn. Registries. Retrospective Studies. Tunisia / epidemiology. Urologic Neoplasms / therapy

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  • (PMID = 21198286.001).
  • [ISSN] 2476-762X
  • [Journal-full-title] Asian Pacific journal of cancer prevention : APJCP
  • [ISO-abbreviation] Asian Pac. J. Cancer Prev.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Thailand
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6. Hummel T, Hord JD: Favorable response to soft tissue sarcoma therapy in an adolescent with embryonal renal sarcoma. Pediatr Blood Cancer; 2004 Jul;43(1):70-2
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  • [Title] Favorable response to soft tissue sarcoma therapy in an adolescent with embryonal renal sarcoma.
  • Embryonal renal sarcomas were first identified in 1995 among banked tumor samples originally classified as adult Wilms tumor.
  • Few long-term remissions were observed when these rare tumors were treated with chemotherapy usually used for childhood Wilms.
  • Data were collected from the medical record of an adolescent female with embryonal renal sarcoma and treated with sarcoma-directed chemotherapy and radiation.
  • At 66 months following diagnosis, the patient has no evidence of tumor but has experienced severe renal dysfunction and ovarian failure.
  • We believe there is a subset of patients with disseminated embryonal renal sarcoma that respond to intense sarcoma-directed therapy.
  • [MeSH-major] Kidney Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / therapy. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Female. Humans


7. Durisin M, Mengel M, Beilken A, Donnerstag F, Lenarz T, Stöver T: [Embryonal rhabdomyosarcoma of the orbita]. Laryngorhinootologie; 2006 Feb;85(2):124-7
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  • [Title] [Embryonal rhabdomyosarcoma of the orbita].
  • CT and MRT scanning revealed a tumor which by open biopsy disclosed embryonal rhabdomyosarcoma.
  • By means of this case we present the current diagnostic and therapeutic approaches for childhood soft tissue sarcomas.
  • At present our patient receives a poly-chemotherapy which has already shown a good response.
  • This case demonstrates that in a child with a unilateral, progressive orbital tumor, the differential diagnosis of a soft tissue sarcoma has to be ruled out.
  • [MeSH-major] Orbital Neoplasms. Rhabdomyosarcoma, Embryonal
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biopsy. Child, Preschool. Diagnosis, Differential. Exophthalmos / etiology. Female. Humans. Orbit / pathology. Prognosis. Treatment Outcome

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  • (PMID = 16498541.001).
  • [ISSN] 0935-8943
  • [Journal-full-title] Laryngo- rhino- otologie
  • [ISO-abbreviation] Laryngorhinootologie
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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8. Rozen WM, Galloway S, Salinas C, Allen P, Schlicht S, Mann GB: Fasciitis ossificans with a radial neuropathy: a benign differential diagnosis for soft tissue sarcoma. J Clin Neurosci; 2007 Apr;14(4):391-4
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  • [Title] Fasciitis ossificans with a radial neuropathy: a benign differential diagnosis for soft tissue sarcoma.
  • A soft tissue mass with an associated neuropathy suggests a malignancy with nerve compression or infiltration.
  • We report a case of an axillary mass with a radial nerve neuropathy, initially suspected to be a soft tissue sarcoma.
  • The final diagnosis was fasciitis ossificans.
  • [MeSH-major] Fasciitis / diagnosis. Nerve Compression Syndromes / diagnosis. Ossification, Heterotopic / diagnosis. Radial Neuropathy / etiology. Sarcoma / diagnosis
  • [MeSH-minor] Adult. Carcinoma, Embryonal / drug therapy. Diagnosis, Differential. Humans. Male. Testicular Neoplasms / drug therapy

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  • (PMID = 17240146.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
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9. Andrés AM, Avila LF, Luis AL, Encinas JL, Sastre A, López-Gutiérrez JC, Martínez L, Queizán A, Martínez-Urrutia MJ, Jaureguizar E, Tovar JA: [Soft tissue sarcomas (1991-2004)]. Cir Pediatr; 2006 Oct;19(4):210-6
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  • [Title] [Soft tissue sarcomas (1991-2004)].
  • BACKGROUND: The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years.
  • MATERIAL AND METHODS: Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated.
  • The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests.
  • RESULTS: 1. RMS: Median age at diagnosis was 2.3 years (range 6 m-16y).
  • Histologically, 13 were embryonal, 5 botryoid, 1 alveolar and 1 fusiform.
  • At diagnosis, 74% were in stages I or II.
  • Surgical biopsy was made before the definitive surgery in 12 cases.
  • In the remaining 8 children the diagnosis was made only after surgical resection.
  • 2. Other sarcomas: Median age at diagnosis was 10.9 years (range 4 days-15 years).
  • Only 9 received chemotherapy and one radiotherapy.
  • CONCLUSIONS: Although the role of surgery is crucial, it is necessary to refine the initial histological diagnosis, because neither the PAAF or the biopsy have always been correct.
  • The negative prognostic factors in our series were metastases present at diagnosis, genitourinary location and alveolar (RMS), desmoplastic or indifferenciated histology.
  • [MeSH-major] Sarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology

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  • (PMID = 17352109.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Spain
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10. Viswanatha B: Embryonal rhabdomyosarcoma of the temporal bone. Ear Nose Throat J; 2007 Apr;86(4):218, 220-2
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  • [Title] Embryonal rhabdomyosarcoma of the temporal bone.
  • The most common soft-tissue sarcoma in infants and children is rhabdomyosarcoma.
  • Multimodality therapy--surgery, multiagent chemotherapy, and radiotherapy-yields sufficiently good results.
  • The author reports a case of embryonal rhabdomyosarcoma of the temporal bone with cranial nerve palsies and extension into the parapharyngeal space in a 4-year-old boy.
  • Despite surgery and chemotherapy, the patient died of his disease within 3 months of presentation.
  • [MeSH-major] Ear Canal. Ear Neoplasms / diagnosis. Ear, Middle. Facial Paralysis / etiology. Mastoid. Ophthalmoplegia / etiology. Rhabdomyosarcoma, Embryonal / diagnosis. Skull Neoplasms / diagnosis. Temporal Bone
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Diagnosis, Differential. Fatal Outcome. Humans. Male. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / therapy. Palliative Care. Pharyngeal Neoplasms / diagnosis. Pharyngeal Neoplasms / pathology. Pharyngeal Neoplasms / therapy

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  • (PMID = 17500393.001).
  • [ISSN] 0145-5613
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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11. Ruymann FB, Grovas AC: Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas. Cancer Invest; 2000;18(3):223-41
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  • [Title] Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas.
  • Advances in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas continue in the Intergroup Rhabdomyosarcoma Study Group (IRSG) and European cooperative groups.
  • The use of molecular biology techniques in soft tissue sarcomas are redefining the classic pathology of these small blue cell tumors.
  • These advances confound the interpretation of consecutively run chemotherapy trials using historical comparisons.
  • The IRSG has reported improvement in the prognosis of both nonmetastatic and metastatic embryonal rhabdomyosarcoma as attributable to three, three-drug regimens that use cyclophosphamide at 2.2 g/m2 in either maintenance or induction and maintenance therapy.
  • Patients of any age with metastatic, nonembryonal, and those over 10 years of age with metastatic embryonal rhabdomyosarcoma continue to have a poor prognosis, which even megatherapy has failed to change.
  • The doublet of ifosfamide and etoposide in combination with vincristine, actinomycin D, and cyclophosphamide at 2.2 g/m2 achieved a remarkable 3-year survival of 58% in patients with metastatic rhabdomyosarcoma and undifferentiated soft tissue sarcoma.
  • Molecular studies in IRSG-V will be applied in the detection of occult bone marrow metastases and the evaluation of resection margins at initial and second-look surgery.
  • Long-term follow-up will be required in patients with gross residual sarcoma randomized to conventional and hyperfractionated radiotherapy in IRSG-IV to assess late effects.
  • As molecular discoveries advance the diagnosis and detection of rhabdomyosarcoma, it is hoped that the futuristic molecular based treatment strategies in development and early testing will further improve survival in high-risk patients with metastatic soft tissue sarcoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Sarcoma / diagnosis. Sarcoma / therapy. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / therapy

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  • (PMID = 10754991.001).
  • [ISSN] 0735-7907
  • [Journal-full-title] Cancer investigation
  • [ISO-abbreviation] Cancer Invest.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA16058; United States / NCI NIH HHS / CA / U10 CA03750-39; United States / NCI NIH HHS / CA / U10 CA24507-17
  • [Publication-type] Clinical Trial; 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] 121
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12. Anavi BL, Moumdjiev IN, Stoyanova AA, Sapunarova KG, Tashev PV, Dureva-Popova MK: A case of undifferentiated (embryonal) sarcoma of the liver: fine needle aspiration cytology diagnosis by one cell type. Folia Med (Plovdiv); 2001;43(3):53-8
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  • [Title] A case of undifferentiated (embryonal) sarcoma of the liver: fine needle aspiration cytology diagnosis by one cell type.
  • We present the first case in Bulgaria of cytologically verified undifferentiated (embryonal) sarcoma of the liver.
  • In the scanty specimen obtained by fine-needle aspiration biopsy under ultrasound control a characteristic prevalence of polygonal cells within mesenchyme tissue was found.
  • The cells are pathognomonic of undifferentiated (embryonal) sarcoma of the liver.
  • After preoperative chemotherapy with ifosfamide, vincristine and actinomycin D (CEVAIE, CWS--96) resection of the tumor including IV, V, VI and VII liver segments was performed.
  • A 30 x 30-cm soft gray-whitish tumor with fine capsule was found.
  • The cytological diagnosis was histologically verified.
  • The patient had a mechanical subileus episode after the 6th course of chemotherapy.
  • The case indicates that the unique cell appearance in embryonal sarcoma of the liver provides a correct diagnosis even in a scanty specimen.
  • [MeSH-major] Liver Neoplasms / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Sarcoma / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Biopsy, Needle. Child. Combined Modality Therapy. Female. Humans

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  • (PMID = 11930835.001).
  • [ISSN] 0204-8043
  • [Journal-full-title] Folia medica
  • [ISO-abbreviation] Folia Med (Plovdiv)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Bulgaria
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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13. 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.
  • 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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14. Kazanowska B, Mikołajewska A, Balcerska A, Balwierz W, Bodalski J, Dłuzniewska A, Drozyńska E, Kurylak A, Reich A, Stencel D, Szewczyk B, Wachowiak J, Wysocki M, Chybicka A: [Soft tissue sarcoma of the bladder and prostate. A report of the Polish Paediatric Solid Tumour Group (PPSTG)]. Med Wieku Rozwoj; 2004 Oct-Dec;8(4 Pt 2):1091-8
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  • [Title] [Soft tissue sarcoma of the bladder and prostate. A report of the Polish Paediatric Solid Tumour Group (PPSTG)].
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children younger then 15 years of age.
  • The treatment of RMS localized in bladder or prostate still remains controversial.
  • The aim of this study was analysis of treatment results in children with soft tissue sarcoma of bladder and prostate.
  • From 1993 to 2001 the PPSTG has used three protocols to treat soft tissue sarcomas in children.
  • After biopsy confirmation of the diagnosis patients were treated with chemotherapy and subsequent surgery.
  • The median follow-up time was 42 months.
  • RMS-embryonal was diagnosed in 11 patients, RMS-alveolare in 4 and others types in 4.
  • After induction chemotherapy two patients received partial cystectomy, 5 complete cystectomy and 3 complete cystectomy with genitourinary reconstructive.
  • The most common treatment failure was isolated, local relapse in 8 children particularly in patients with any second surgery.
  • Significant prognostic factors are the initial tumours volume, the lymph nodes infiltration and the response to the first chemotherapy cycle.
  • Surgery is the most important procedure in local control of soft tissue sarcoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Prostatic Neoplasms. Rhabdomyosarcoma. Urinary Bladder Neoplasms
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Cystectomy / methods. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Staging. Poland. Radiotherapy, Adjuvant. Treatment Outcome

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  • (PMID = 15951604.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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15. Stevens M, Rey A, Bouvet N, Ellershaw C, Sanchez de Toledo J, Oberlin O: SIOP MMT 95: Intensified (6 drug) versus standard (IVA) chemotherapy for high risk non metastatic rhabdomyosarcoma (RMS). J Clin Oncol; 2004 Jul 15;22(14_suppl):8515

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  • [Title] SIOP MMT 95: Intensified (6 drug) versus standard (IVA) chemotherapy for high risk non metastatic rhabdomyosarcoma (RMS).
  • Principal study objectives were: low and standard risk patients, to maintain excellent survival with limited chemotherapy and very selective use of local therapy.
  • ; high risk patients, to explore survival advantage for an intensified chemotherapy strategy in a randomised trial.
  • METHODS: Eligibility for randomisation included age ≥ 6 months ≤18 years, no distant metastases, diagnosis within previous 8 weeks without prior treatment except surgery, pathology available for central review, written consent according to institutional requirement.
  • From July 1995 to July 2003, 456 high risk patients (incompletely resected embryonal RMS, undifferentiated sarcoma and soft tissue PNET at all sites except paratesticular, vagina and uterus, and all alveolar RMS) were randomised to receive IVA (ifosfamide, vincristine, actinomycin D) or a 6 drug combination (IVA + carboplatin, epirubicin, etoposide) both delivered over 27 weeks.
  • Cumulative dose / m<sup>2</sup> = ifosfamide 54g (both arms), epirubicin 450 mg, etoposide 1350 mg (6 drug).
  • Delivery of radiotherapy was determined according to site and / or response to chemotherapy ± surgery.
  • Non randomised exceptions were: orbital tumours (allocated IVA); SIOP stage III (node positive) and young (age < 3yr) parameningeal tumours (allocated 6 drugs).
  • RESULTS: Data given only for randomised patients [Figure: see text] Toxicity was significantly greater (infection, myelosuppression, mucositis) for the 6 drug arm.
  • CONCLUSIONS: Intensification of chemotherapy provides no overall advantage for non metastatic RMS / other chemosensitive STS, and adds toxicity.

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  • (PMID = 28013776.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. Malerba M, Garofalo A: [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)]. Tumori; 2003 Jul-Aug;89(4 Suppl):246-50

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  • [Title] [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)].
  • [Transliterated title] Un raro caso di sarcoma delle guaine nervose a differenziazione rabdomioblastica (malignant Triton tumor).
  • Malignant triton tumor (MTT) is a subtype of MPNST that also contain tissue with skeletal muscle differentiation (embryonal, plemorphic and botryoid rhabdomyosarcoma).
  • Seventeen months before, when the patient underwent surgery at the same Department for both a left-sided paravertebral inferior mediastinal neurofibroma and a right-sided axillary neurofibroma, diagnosis of von Recklinghausen disease (NF1) was made, according to the criteria established by the NIH Consensus Development.
  • The popliteal mass was resected too and resulted to be a neurofibroma just like the tumors resected 17 months before when diagnosis of von Recklinghausen disease was made.
  • Sarcoma arising in anatomic site other than extremity and superficial trunk are often more difficult to control because of anatomic constraints, delayed disease presentation, proximity to neurovascular and osseous structures and toxicity for normal adjacent tissues that limits the use of adequate radiation doses.
  • In contrast to the benefit most patients with high grade soft tissue sarcomas of the extremities receive from adjuvant radiation and chemotherapy, these modalities have been of little value for retroperitoneal tumors.
  • Current chemotherapy for retroperitoneal sarcomas is ineffective.
  • Local adjuvant therapy such as intraperitoneal chemotherapy or experimental immunotherapy seems to be attractive in theory, but needs further investigations through prospective randomized multicentric trials.
  • In conclusion, to date aggressive surgical management remains the most effective modality for selected primary and recurrent retroperitoneal soft tissue sarcomas including MPNSTs and the subtype MTT.
  • Patients with incomplete resection and other risk factors such as younger age and high grade tumors may be suitable candidates for investigational adjuvant therapy.
  • [MeSH-minor] Adult. Axilla. Cell Differentiation. Humans. Knee. Male. Mediastinal Neoplasms / pathology. Mediastinal Neoplasms / surgery. Muscles / pathology. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Spinal Nerve Roots / pathology. Spinal Nerve Roots / surgery

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  • (PMID = 12903608.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] United States
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17. Nicol K, Savell V, Moore J, Teot L, Spunt SL, Qualman S, Children's Oncology Group, Soft Tissue Sarcoma Committee: Distinguishing undifferentiated embryonal sarcoma of the liver from biliary tract rhabdomyosarcoma: a Children's Oncology Group study. Pediatr Dev Pathol; 2007 Mar-Apr;10(2):89-97
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Distinguishing undifferentiated embryonal sarcoma of the liver from biliary tract rhabdomyosarcoma: a Children's Oncology Group study.
  • Morphologically, the distinction between undifferentiated embryonal sarcoma of the liver (UESL) and biliary tract rhabdomyosarcoma (RMS) can be uncertain because of some shared pathologic similarities.
  • Patients with UESL have been consistently but erroneously enrolled in Children's Oncology Group (COG) treatment protocols because UESL was equated with RMS, despite the differing primary treatment modalities of these entities.
  • Establishing the correct diagnosis of these distinct clinical and pathologic entities is important, as surgery alone may be curative in UESL, whereas initial chemotherapy is often recommended for the treatment of biliary tract RMS.
  • [MeSH-major] Bile Duct Neoplasms / diagnosis. Biomarkers, Tumor / analysis. Liver Neoplasms / diagnosis. Rhabdomyosarcoma / diagnosis. Sarcoma / diagnosis
  • [MeSH-minor] Child. Child, Preschool. Diagnosis, Differential. Disease-Free Survival. Female. Follow-Up Studies. Humans. Immunohistochemistry. Male. MyoD Protein / metabolism. Myogenin / metabolism. Neoplasm Proteins / metabolism. Retrospective Studies. Survival Analysis. Time Factors. Treatment Outcome


18. Hardaway CA, Graham BS, Barnette DJ, Feldman BD: Embryonal rhabdomyosarcoma presenting in an adult: a case report and discussion of immunohistochemical staining. Am J Dermatopathol; 2003 Feb;25(1):45-52
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  • [Title] Embryonal rhabdomyosarcoma presenting in an adult: a case report and discussion of immunohistochemical staining.
  • Embryonal rhabdomyosarcoma is the most common soft tissue sarcoma of childhood but is rarely seen in adults.
  • We report an embryonal rhabdomyosarcoma of the cheek in a 21-year-old Filipino man presenting as a rapidly enlarging mass.
  • An incisional biopsy was consistent with embryonal rhabdomyosarcoma.
  • The patient failed to respond to one cycle of chemotherapy after initial diagnosis and workup for metastatic disease.
  • The patient subsequently underwent radiation therapy for a total of 6,000 cGy, followed by two cycles of chemotherapy.
  • [MeSH-major] Rhabdomyosarcoma, Embryonal / secondary. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols. Biomarkers, Tumor / analysis. Chemotherapy, Adjuvant. Combined Modality Therapy. Etoposide / therapeutic use. Humans. Ifosfamide / therapeutic use. Immunohistochemistry. Male. Neoplasm Proteins / chemistry. Radiotherapy, Adjuvant. Vincristine / therapeutic use

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  • (PMID = 12544100.001).
  • [ISSN] 0193-1091
  • [Journal-full-title] The American Journal of dermatopathology
  • [ISO-abbreviation] Am J Dermatopathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; UM20QQM95Y / Ifosfamide
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19. Antillon F, Castellanos M, Valverde P, Luna-Fineman S, Garrido C, Serrato T, Rodriguez-Galindo C, Casanova M, Ferrari A: Treating Pediatric soft tissue sarcomas in a country with limited resources: the experience of the Unidad Nacional de Oncologia Pediatrica in Guatemala. Pediatr Blood Cancer; 2008 Dec;51(6):760-4
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  • [Title] Treating Pediatric soft tissue sarcomas in a country with limited resources: the experience of the Unidad Nacional de Oncologia Pediatrica in Guatemala.
  • BACKGROUND: About 250-300 children with newly diagnosed cancer are treated each year at the Unidad Nacional de Oncologia Pediatrica in Guatemala City; less than 5% of them have soft tissue sarcomas (STS).
  • The aim of the article was to evaluate whether the therapeutic standards achieved in STS in developed countries could be reproduced in a low-income country.
  • PATIENTS AND METHODS: We reviewed the clinical data, treatment and outcome of 80 patients, 47 cases of rhabdomyosarcoma (RMS) and 33 of non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), treated between January 2000 and October 2007.
  • RESULTS: Most of the RMS patients had advanced disease at diagnosis (87% groups III-IV).
  • Their 3-year event-free survival rate (EFS) was 26.4% if abandoning the treatment was considered as an event, or 32.4% if it was censored (14 patients abandoned the treatment), and the 3-year overall survival rate (OS) was 43.5%.
  • Local progression/relapse was the main cause of treatment failure.
  • Among the patients with NRSTS, the EFS at 3 years was 36.4% (when abandoning the treatment was considered as an event) or 43.3% (when it was censored), and the OS was 44.2%.
  • Outcome was satisfactory for synovial sarcoma patients, those with tumors < or =5 cm, and those with localized disease.
  • CONCLUSIONS: Overall results were unsatisfactory compared to results reported from developed countries.
  • Late diagnosis and the consequently high proportion of cases of advanced disease at diagnosis, the large number of patients failing to complete the treatment, and the poor quality of local control (in RMS) adversely influence outcome.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Developing Countries. Rhabdomyosarcoma, Alveolar / drug therapy. Rhabdomyosarcoma, Embryonal / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Female. Guatemala. Humans. Infant. Male. Prognosis. Survival Rate. Treatment Outcome

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  • (PMID = 18680154.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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20. Raney RB, Anderson JR, Andrassy RJ, Crist WM, Donaldson SS, Maurer HM, Intergroup Rhabdomyosarcoma Study Group: Soft-tissue sarcomas of the diaphragm: a report from the Intergroup Rhabdomyosarcoma Study Group from 1972 to 1997. J Pediatr Hematol Oncol; 2000 Nov-Dec;22(6):510-4
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  • [Title] Soft-tissue sarcomas of the diaphragm: a report from the Intergroup Rhabdomyosarcoma Study Group from 1972 to 1997.
  • PATIENTS AND METHODS: We reviewed the records of 15 patients with sarcoma of the diaphragm who were entered on IRS Group protocols between 1972 and 1997.
  • Patient ages at diagnosis ranged from 0.5 to 20 years (median, 13 yrs), and 10 were girls.
  • RESULTS: Localized, gross residual disease after initial surgery was present in 10 patients, and five had metastases at diagnosis (pleura, 3; pericardium, 1; lungs and bones, 1).
  • Tumor subtypes were alveolar rhabdomyosarcoma (RMS) in five cases, embryonal RMS in three, undifferentiated sarcoma in three, extraosseous Ewing sarcoma in three, and unclassified sarcoma in one.
  • Treatment consisted of radiation therapy to the primary tumor and metastases when feasible, and combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide with or without doxorubicin, ifosfamide, cisplatin, and etoposide.
  • Five patients (33%) are continuously failure-free and alive at a median of 8.8 years from diagnosis (range, 1.1-15 yrs).
  • However, the other 10 patients experienced relapse at 0.3 to 2 years from start of therapy (median, 1 yr).
  • Death after relapse occurred at 0.39 to 2.6 years (median, 1.6 yrs) from diagnosis.
  • CONCLUSIONS: Sarcomas of the diaphragm are generally deemed unresectable at diagnosis and/or are metastatic.
  • Most of them are not embryonal rhabdomyosarcomas.
  • Treatment with more effective primary chemotherapy to shrink the tumor, followed-up by surgical resection and radiation therapy, should improve the prognosis for patients with sarcomas arising in the diaphragm, especially for the majority who have localized tumors.
  • [MeSH-major] Muscle Neoplasms / therapy. Rhabdomyosarcoma / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Diaphragm. Female. Humans. Infant. Male. Radiotherapy Dosage. Recurrence. Retrospective Studies. Survival Rate

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  • (PMID = 11132218.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-24507; United States / NCI NIH HHS / CA / CA-72989
  • [Publication-type] Journal Article; Multicenter Study; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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21. Raney B, Anderson J, Breneman J, Donaldson SS, Huh W, Maurer H, Michalski J, Qualman S, Ullrich F, Wharam M, Meyer W, Soft-Tissue Sarcoma Committee of the Children's Oncology Group, Arcadia, California, USA: Results in patients with cranial parameningeal sarcoma and metastases (Stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV, 1978-1997: report from the Children's Oncology Group. Pediatr Blood Cancer; 2008 Jul;51(1):17-22
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  • [Title] Results in patients with cranial parameningeal sarcoma and metastases (Stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV, 1978-1997: report from the Children's Oncology Group.
  • PURPOSE: Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV.
  • PATIENTS: We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail.
  • RESULTS: The 54 males and 37 females were <1-19 years at diagnosis.
  • Major metastatic sites at diagnosis were lung (63%), bone marrow (33%), and bone (27%).
  • Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues.
  • Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died.
  • Factors indicating likelihood of 10-year FFS included tumor arising in "better" versus "worse" sites (FFS 46% vs. 18%, P = 0.02) and embryonal versus other histology (FFS 37% vs. 19%, P = 0.06).
  • CONCLUSIONS: Cure was possible for some patients with metastatic cranial parameningeal sarcoma.
  • Patients with the best outlook had embryonal RMS located in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal region.
  • Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease.

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18266224.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA-98543; United States / NCI NIH HHS / CA / CA-24507; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / CA-72989; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / CA-29511
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
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22. Huh WW, Skapek SX: Childhood rhabdomyosarcoma: new insight on biology and treatment. Curr Oncol Rep; 2010 Nov;12(6):402-10
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  • [Title] Childhood rhabdomyosarcoma: new insight on biology and treatment.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood.
  • The two most common histologic variants are the embryonal and alveolar subtypes.
  • Other studies have implicated several cellular mechanisms and pathways being involved in RMS pathogenesis and survival, such as the cyclin-dependent kinase inhibitors, insulin-like growth factor pathway, and the mammalian target of rapamycin pathway, thus providing potential avenues for targeted therapy.
  • Recent clinical trials have tried to improve risk stratification and prediction of clinical outcome based upon clinical or radiographic response to initial therapy and also to determine the role of high-dose chemotherapy with stem cell rescue in high-risk RMS patients.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / therapy. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics. Rhabdomyosarcoma, Embryonal / therapy. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Animals. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Child. Child, Preschool. Clinical Trials as Topic. Combined Modality Therapy. Disease Progression. Female. Humans. Male. Mesenchymal Stromal Cells / pathology. Mice. Neoplasm Metastasis. Neoplasm Staging. Neoplastic Stem Cells / metabolism. Neoplastic Stem Cells / pathology. Prognosis. Protein Kinase Inhibitors / administration & dosage. Risk Factors. Secondary Prevention. Signal Transduction / drug effects. Signal Transduction / genetics. Sirolimus / administration & dosage. Survival Rate. TOR Serine-Threonine Kinases / antagonists & inhibitors. Tomography

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  • (PMID = 20820958.001).
  • [ISSN] 1534-6269
  • [Journal-full-title] Current oncology reports
  • [ISO-abbreviation] Curr Oncol Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors; EC 2.7.1.1 / TOR Serine-Threonine Kinases; W36ZG6FT64 / Sirolimus
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23. Kebudi R, Ayan I, Görgün O, Ağaoğlu FY, Vural S, Darendeliler E: Brain metastasis in pediatric extracranial solid tumors: survey and literature review. J Neurooncol; 2005 Jan;71(1):43-8
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  • The aim of this study is to investigate the incidence, treatment, prognosis of brain metastasis in extracranial pediatric malignant tumors in a single institution and to review the literature.
  • RESULTS: Sixteen (10 female, 6 male) of 1100 patients (1.45%) with extracranial solid tumors developed brain metastases.
  • The diagnosis was sarcomas in 12 patients: 5 osteosarcomas, 4 Ewing's sarcoma family tumors, 1 rhabdomyosarcoma, 1 clear cell sarcoma of the soft tissue, 1 alveolar soft part sarcoma.
  • Four patients (25%) had brain metastasis at diagnosis.
  • Twelve (75%) developed brain metastasis during therapy or relapse at a median duration of 16 (1-70) months from initial diagnosis.
  • All patients had metastases to various sites, mostly lung, at the time the brain metastases were detected.
  • Treatment included surgery, followed by postoperative radiotherapy (RT) and chemotherapy (CT) in 1, S and RT in 1, S in 1, RT and CT in 6, RT in 1, CT in 1 and no treatment in 5.
  • Only one patient with alveolar soft part sarcoma is alive with disease 20 months from diagnosis of brain metastasis.
  • All other patients died at a median time of 2 months (2 days-6 months) from the time of brain metastasis.
  • Although, the outcome for these patients is dismal in this series and in the literature; reports of long term survival in a few cases with Wilms' tumor, osteosarcoma and alveolar soft part sarcoma who had isolated brain metastasis, suggest that a subset of patients may benefit from therapy.
  • [MeSH-major] Brain Neoplasms / epidemiology. Brain Neoplasms / secondary. Sarcoma / epidemiology. Sarcoma / secondary
  • [MeSH-minor] Adolescent. Antineoplastic Agents / therapeutic use. Bone Neoplasms / epidemiology. Bone Neoplasms / secondary. Bone Neoplasms / therapy. Child. Child, Preschool. Female. Humans. Infant. Lung Neoplasms / epidemiology. Lung Neoplasms / secondary. Lung Neoplasms / therapy. Male. Mediastinal Neoplasms / epidemiology. Mediastinal Neoplasms / secondary. Mediastinal Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / epidemiology. Neoplasms, Germ Cell and Embryonal / secondary. Neoplasms, Germ Cell and Embryonal / therapy. Prognosis. Radiotherapy, Adjuvant. Survival Analysis. Wilms Tumor / epidemiology. Wilms Tumor / secondary. Wilms Tumor / therapy

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  • (PMID = 15719274.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 43
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24. Alaggio R, Ninfo V, Rosolen A, Coffin CM: Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases. Am J Surg Pathol; 2006 Mar;30(3):388-94

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma.
  • PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass.
  • Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy.
  • One patient is alive with persistent locally aggressive disease, 2 are alive with no evidence of recurrence, 1 had a recurrence treated surgically without further follow-up information, 1 patient died with persistent tumor and sepsis 6 weeks after diagnosis, and 1 patient was lost to follow-up.
  • [MeSH-major] Fibrosarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Dermatofibrosarcoma / pathology. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Infant. Infant, Newborn. Male. Microscopy, Electron, Transmission. Neoplasm Recurrence, Local / pathology. Nerve Sheath Neoplasms / pathology. Oncogene Proteins, Fusion / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16538060.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ETV6-NTRK3 fusion protein, human; 0 / Oncogene Proteins, Fusion
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25. Mentzel T, Kuhnen C: Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases. Virchows Arch; 2006 Nov;449(5):554-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Rhabdomyosarcoma (RMS) is currently classified into embryonal RMS, including its botryoid and spindle cell variants, alveolar RMS, including a solid variant, and pleomorphic RMS.
  • In children and adolescents embryonal RMS occurs in a younger age group than alveolar RMS, and pleomorphic RMS is almost always seen in older adults.
  • Five neoplasms were completely excised, in one incompletely excised neoplasm additional chemotherapy was given, and in one patient a biopsy was done only so far.
  • All neoplasms arose in subcutaneous and deep soft tissues with dermal involvement in one case, and the size of the neoplasms ranged from 4 to 19 cm in largest diameter.
  • Immunohistochemically, tumour cells in RMS stain positively for CD 99 and WT1 as well, which is of importance in the differential diagnosis to other mesenchymal neoplasms, whereas fast myosin does not represent a reliable marker for RMS in adults.
  • [MeSH-major] Rhabdomyosarcoma / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 17013628.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD99; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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26. Donaldson SS, Meza J, Breneman JC, Crist WM, Laurie F, Qualman SJ, Wharam M, Children's Oncology Group Soft Tissue Sarcoma Committee (formely Intergroup Rhabdomyosarcoma Group) representing the Children's Oncology Group and the Quality Assurance Review Center: Results from the IRS-IV randomized trial of hyperfractionated radiotherapy in children with rhabdomyosarcoma--a report from the IRSG. Int J Radiat Oncol Biol Phys; 2001 Nov 1;51(3):718-28
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Of the 490 remaining, 239 were randomized to HFRT (59.4 Gy in 54 1.1-Gy twice daily fractions) and 251 to CFRT (50.4 Gy in 28 1.8-Gy daily fractions).
  • All patients received chemotherapy.
  • RT began at Week 9 after induction chemotherapy for all but those with high-risk parameningeal tumors who received RT during induction chemotherapy.
  • RESULTS: Analysis by randomized treatment assignment (intent to treat) revealed an estimated 5-year failure-free survival (FFS) rate of 70% and overall survival (OS) of 75%.
  • In the univariate analysis, the factors associated with the best outcome were age 1-9 years at diagnosis; noninvasive tumors; tumor size <5 cm; uninvolved lymph nodes; Stage 1 or 2 disease; primary site in the orbit or head and neck; and embryonal histologic features (p = 0.001 for all factors).
  • No differences in the FFS or OS between the two RT treatment methods and no differences in the FFS or OS between HFRT and CFRT were found when analyzed by age, gender, tumor size, tumor invasiveness, nodal status, histologic features, stage, or primary site.
  • Treatment compliance differed by age.
  • Of the children >or=5 years, 88% assigned to both HFRT and CFRT received their assigned treatment.
  • The reasons for not receiving the appropriate randomized treatment were progressive disease, early death, parent or physician refusal, young age, or surgery.
  • The analysis by treatment actually received revealed a 5-year FFS rate of 73% and OS rate of 77%, with no difference between CFRT and HFRT.
  • The standard of care for Group III RMS continues to be CFRT with chemotherapy.
  • [MeSH-minor] Adolescent. Adult. Analysis of Variance. Child. Child, Preschool. Female. Humans. Infant. Male. Patient Compliance. Radiation Injuries / classification. Radiation Injuries / pathology. Remission Induction. Treatment Outcome

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  • [CommentIn] Int J Radiat Oncol Biol Phys. 2002 Dec 1;54(5):1579-80; author reply 1580 [12459396.001]
  • (PMID = 11597814.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-24507
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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27. Mikuz G: [WHO classification of testicular tumors]. Verh Dtsch Ges Pathol; 2002;86:67-75
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Such atypical cells appear in the tubules adjacent to the germ cell tumors, in some few cases (6%) also in the contra lateral healthy gonad and rarely in infertile men (1%).
  • The precursor lesion can progress to franc germ cell tumor starting probably with seminoma, which still maintain the capability of differentiation (pluripotente cells) in all other types of non-seminomatous germ cell tumors.
  • This is a harmless name for an extremely dangerous tumor in which one tissue overgrows the other and gives rise to somatic type sarcomas or carcinomas.
  • Such tumors do not respond like germ cell tumors to the usual chemotherapy.
  • Treatment should be tailored according to that used in standard management of the respective sarcoma or carcinoma.
  • In the comments it is mentioned that the testis carcinoid could be a part of teratoma, but the diagnosis is listed in the group of "miscellaneous" tumors together with tumors of ovarian epithelial type.
  • This is a very questionable decision because the normal testis does not contain neuroendocrine cells from which carcinoids would have to be able to develop.
  • The patients have cardiac myxomas, spotty skin pigmentation, hormone active nodular hyperplasia of the adrenals and soft tissue myxomas.
  • For the therapy of germ cell tumor an assessment of risk factors found by the pathologists is extremely important.
  • The most important independent predictors of relapse are tumor invasion of blood or lymph-vessels, absence of yolk sac elements and the presence of an embryonal carcinoma component.
  • In the absence of such predictors a surveillance policy allows some patients to forgo chemotherapy.

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  • (PMID = 12647353.001).
  • [ISSN] 0070-4113
  • [Journal-full-title] Verhandlungen der Deutschen Gesellschaft für Pathologie
  • [ISO-abbreviation] Verh Dtsch Ges Pathol
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 48
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28. Solomon LA, Zurawin RK, Edwards CL: Vaginoscopic resection for rhabdomyosarcoma of the vagina: a case report and review of the literature. J Pediatr Adolesc Gynecol; 2003 Jun;16(3):139-42
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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 soft-tissue sarcoma found in children.
  • A polypoid form of the embryonal type of rhabdomyosarcoma, sarcoma botyroides, is often found in girls under age 5.
  • Their superficial location and clinical symptoms lead to early diagnosis, and these tumors are therefore considered to be the easiest to treat and most likely to be cured.
  • In the past 30 years we have seen a shift in treatment from radical surgery to conservative surgery with chemotherapy and radiation, with improved survival and preservation of normal anatomy and improved postoperative body imagery.
  • [MeSH-major] Rhabdomyosarcoma / diagnosis. Vaginal Neoplasms / diagnosis

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  • (PMID = 12804937.001).
  • [ISSN] 1083-3188
  • [Journal-full-title] Journal of pediatric and adolescent gynecology
  • [ISO-abbreviation] J Pediatr Adolesc Gynecol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 10
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29. Cribbs RK, Shehata BM, Ricketts RR: Primary ovarian rhabdomyosarcoma in children. Pediatr Surg Int; 2008 May;24(5):593-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Although rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, primary rhabdomyosarcomas of the ovary are extremely rare, with only eight well-documented pediatric cases previously reported in the literature.
  • We present two additional cases: an alveolar RMS originating in the right ovary with metastatic spread to the splenic flexure of the colon and to both lungs in a 13-year-old African American girl, and an embryonal RMS arising in the right ovary of a 6-year-old Caucasian girl with pre-operative intra-abdominal rupture and a malignant right pleural effusion.
  • Both patients had complete resection of their primary tumors and received chemotherapy including vincristine, doxorubicin and cyclophosphamide with good response to therapy.
  • [MeSH-major] Ovarian Neoplasms / diagnosis. Rhabdomyosarcoma / diagnosis
  • [MeSH-minor] Adolescent. Biopsy, Needle. Chemotherapy, Adjuvant. Child. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Laparoscopy. Ovariectomy / methods. Tomography, X-Ray Computed

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  • (PMID = 18004572.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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30. Ayadi L, Chaabouni S, Chabchoub I, Ayadi A, Kallel R, Fakhfakh I, Hachicha M, Boudawara T: [Primary rhabdomyosarcoma of the pleura presenting as recurrent pneumothorax]. Rev Mal Respir; 2009 Mar;26(3):333-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • INTRODUCTION: Rhabdomyosarcoma is the most common soft tissue sarcoma in the first two decades of life.
  • Our aim is to discuss the clinical presentation, treatment and prognosis of this uncommon location of rhabdomyosarcoma.
  • CASE REPORT: We report a case of primary embryonal rhabdomyosarcoma of the pleura in a boy of 21 months.
  • A computed tomography scan showed only pleural detachment with no evidence of any pleural disease.
  • Despite chemotherapy, the tumour quickly increased in size and the infant died from acute respiratory failure.
  • CONCLUSION: Thoracic rhabdomyosarcoma is rare and remains clinically silent for a long time.
  • [MeSH-major] Pleural Neoplasms / diagnosis. Pneumothorax / etiology. Rhabdomyosarcoma / diagnosis

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  • (PMID = 19367209.001).
  • [ISSN] 0761-8425
  • [Journal-full-title] Revue des maladies respiratoires
  • [ISO-abbreviation] Rev Mal Respir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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31. Sinha R, Bandyopadhyay SN, Pathak K, Hati G: Rhabdomyosarcoma of nose and paranasal sinuses--a therapeutic challenge. J Indian Med Assoc; 2006 Sep;104(9):519-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rhabdomyosarcoma of nose and paranasal sinuses--a therapeutic challenge.
  • Rhabdomyosarcoma is a highly malignant tumour of striated muscle and it is the most common soft tissue sarcoma in the paediatric age group.
  • Histologically embryonal rhabdomyosarcoma is the most common variant.
  • The present series deals with seven such cases of different histopathological types including the embryonal, botryoid, and alveolar varieties in the paediatric age group.
  • Multimodal treatment including limited surgery followed by radiotherapy and chemotherapy resulted in survival ranging from 3 days to more than 3 years, which was also influenced by the severity of the disease at presentation and its aggressiveness.
  • [MeSH-major] Nose Neoplasms / therapy. Paranasal Sinus Neoplasms / therapy. Rhabdomyosarcoma / therapy
  • [MeSH-minor] Biopsy. Child. Child, Preschool. Combined Modality Therapy. Diagnosis, Differential. Female. Follow-Up Studies. Humans. India / epidemiology. Male. Retrospective Studies. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 17388011.001).
  • [ISSN] 0019-5847
  • [Journal-full-title] Journal of the Indian Medical Association
  • [ISO-abbreviation] J Indian Med Assoc
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] India
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32. Hessissen L, Kanouni L, Kili A, Nachef MN, El Khorassani M, Benjaafar N, Khattab M, El Gueddari Bel K: Pediatric rhabdomyosarcoma in Morocco. Pediatr Blood Cancer; 2010 Jan;54(1):25-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the first two decades of life.
  • The male/female ratio was 2:1 with a mean age at diagnosis of 5 years.
  • The embryonal subtype was the most frequent (73%) and the head and neck was the most common site of disease, followed by the genito-urinary tract and limbs.
  • Chemotherapy was used in all patients; 44% also had a radical surgery and 23% radiation therapy.
  • The event-free survival (EFS) at 10 years was 39% with relapse as the first cause of treatment failure.
  • The rate of treatment abandonment was 37%.
  • However, EFS is lower than that reported elsewhere due to occasional lack of availability of drugs, inadequate local control, and abandonment.
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Morocco / epidemiology. Neoplasm Staging. Prognosis. Radiotherapy. Retrospective Studies. Survival Rate. Treatment Outcome

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
  • (PMID = 19746454.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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33. Sbeity S, Abella A, Arcand P, Quintal MC, Saliba I: Temporal bone rhabdomyosarcoma in children. Int J Pediatr Otorhinolaryngol; 2007 May;71(5):807-14
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: Rhabdomyosarcoma is the most frequent soft tissue sarcoma in the pediatric age group.
  • A thorough review of medical and surgical charts was performed to obtain demographic, clinical, paraclinical, and therapeutic data, which were subsequently analyzed and compared to published results.
  • The mean age at the time of diagnosis was 4.15 years.
  • Five children had the embryonal subtype and one had the botryoid subtype on histology.
  • All patients except two received combined chemotherapy and radiotherapy as treatment.
  • A high index of suspicion should be raised in the context of otitis media that is unresponsive to ordinary medical treatment.
  • A biopsy is hence recommended in the presence of polyps in the external auditory canal that are resistant to medical treatment.
  • Early diagnosis and the adoption of multimodal therapy offer the best outcome.

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  • (PMID = 17346806.001).
  • [ISSN] 0165-5876
  • [Journal-full-title] International journal of pediatric otorhinolaryngology
  • [ISO-abbreviation] Int. J. Pediatr. Otorhinolaryngol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Ireland
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