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3. 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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  • [Title] Vaginoscopic resection for rhabdomyosarcoma of the vagina: a case report and review of the literature.
  • 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.
  • These tumors are usually localized to the anterior vaginal wall.
  • 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.
  • Conservative excision in the past has been performed by sharp curettage of the anterior vaginal wall.
  • We present a case of a 2-yr-old child with a RMS of the vagina for which we utilized vaginoscopy not only to determine the extent of the tumor but also for precise resection using a bipolar electrode with normal saline as the distension medium.
  • [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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4. Kalu E, Creighton SM, Woodhouse CR: Vaginal restoration in survivors of childhood rhabdomyosarcoma. Int J Gynecol Cancer; 2009 Jul;19(5):981-4
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  • [Title] Vaginal restoration in survivors of childhood rhabdomyosarcoma.
  • Childhood genital rhabdomyosarcoma is a devastating diagnosis.
  • Treatment is usually rigorous, comprising extensive surgery often with adjuvant chemotherapy and radiotherapy.
  • These young women will have sexual and reproductive expectations similar to those of their peers and will present to gynecologists for advice and treatment.
  • This report describes the presenting problems seen in this group and highlights the complex nature of the investigations required and the treatments available.
  • [MeSH-major] Reconstructive Surgical Procedures / methods. Rhabdomyosarcoma / surgery. Vagina / surgery. Vaginal Neoplasms / surgery

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  • (PMID = 19574797.001).
  • [ISSN] 1525-1438
  • [Journal-full-title] International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
  • [ISO-abbreviation] Int. J. Gynecol. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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5. Bernal KL, Fahmy L, Remmenga S, Bridge J, Baker J: Embryonal rhabdomyosarcoma (sarcoma botryoides) of the cervix presenting as a cervical polyp treated with fertility-sparing surgery and adjuvant chemotherapy. Gynecol Oncol; 2004 Oct;95(1):243-6
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  • [Title] Embryonal rhabdomyosarcoma (sarcoma botryoides) of the cervix presenting as a cervical polyp treated with fertility-sparing surgery and adjuvant chemotherapy.
  • BACKGROUND: Embryonal rhabdomyosarcoma (sarcoma botryoides) of the female genital tract is rare in the cervix.
  • It tends to occur in children and young women, and appears to have a better prognosis than sarcoma botryoides of the vagina and uterus.
  • CASE: We report a case of embryonal rhabdomyosarcoma (sarcoma botryoides) presenting as a cervical polyp in a 19-year-old female.
  • The patient was successfully treated with fertility-sparing surgery and adjuvant chemotherapy.
  • Therapy has recently inclined towards conservative treatment.
  • [MeSH-major] Polyps / diagnosis. Polyps / therapy. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / therapy. Uterine Cervical Neoplasms / drug therapy. Uterine Cervical Neoplasms / therapy
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Fertility. Humans


6. Filipas D: Surgery for urogenital rhabdomyosarcoma. Curr Opin Urol; 2001 Nov;11(6):563-5
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  • [Title] Surgery for urogenital rhabdomyosarcoma.
  • The treatment and prognosis of rhabdomyosarcoma of the urogenital tract have changed dramatically over the past few decades.
  • Until the 1970s radical cystectomy and pelvic exenteration were the only therapeutic options in rhabdomyosarcoma of the bladder/prostate and of the vagina/uterus.
  • Since then, multiagent chemotherapy has achieved remarkable cure rates.
  • The available long-term survival data with these techniques demonstrate excellent survival in rhabdomyosarcoma of the vagina/uterus, with a high percentage of organ preservation.
  • On the contrary, increased rates of local recurrence and death from disease in patients with rhabdomyosarcoma of the bladder/prostate who undergo primary chemotherapy with or without tumour excision, and complications following combined radiotherapy critically question the long-term benefit of these organ-sparing surgical techniques in this group.
  • [MeSH-major] Rhabdomyosarcoma / surgery. Urogenital Neoplasms / surgery

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  • (PMID = 11734690.001).
  • [ISSN] 0963-0643
  • [Journal-full-title] Current opinion in urology
  • [ISO-abbreviation] Curr Opin Urol
  • [Language] eng
  • [Publication-type] Editorial; Review
  • [Publication-country] United States
  • [Number-of-references] 14
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7. Filipas D, Fisch M, Stein R, Gutjahr P, Hohenfellner R, Thüroff JW: Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. BJU Int; 2004 Jan;93(1):125-9
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  • [Title] Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery.
  • OBJECTIVE: To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968.
  • PATIENTS AND METHODS: From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5-18) had RMS of bladder/prostate or vagina.
  • CONCLUSION: Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.
  • [MeSH-major] Prostatic Neoplasms / surgery. Rhabdomyosarcoma / surgery. Urinary Bladder Neoplasms / surgery. Vaginal Neoplasms / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Cystectomy / methods. Female. Follow-Up Studies. Humans. Infant. Male. Prostatectomy / methods. Retrospective Studies. Treatment Outcome. Urinary Diversion / methods

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  • [CommentIn] J Urol. 2005 Mar;173(3):982 [15711356.001]
  • (PMID = 14678383.001).
  • [ISSN] 1464-4096
  • [Journal-full-title] BJU international
  • [ISO-abbreviation] BJU Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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8. Caruso RA, Napoli P, Villari D, Starrantino M: Anaplastic (pleomorphic) subtype embryonal rhabdomyosarcoma of the cervix. Arch Gynecol Obstet; 2004 Dec;270(4):278-80
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  • [Title] Anaplastic (pleomorphic) subtype embryonal rhabdomyosarcoma of the cervix.
  • INTRODUCTION: Embryonal rhabdomyosarcoma (RMS) of the female genital tract usually occurs in the vagina during childhood.
  • CASE REPORT: We present the case of a 13-year-old girl diagnosed with an anaplastic (pleomorphic) subtype embryonal RMS of the endocervix, who was treated successfully with polypectomy followed by ifosfamide-vincristine-actinomycin combination chemotherapy and brachytherapy.
  • The patient exhibits no evidence of recurrence and has normal menstrual function 36 months following initial diagnosis.
  • [MeSH-major] Brachytherapy. Chemotherapy, Adjuvant. Gynecologic Surgical Procedures. Rhabdomyosarcoma, Embryonal / therapy. Uterine Cervical Neoplasms / therapy
  • [MeSH-minor] Adolescent. Antibiotics, Antineoplastic / administration & dosage. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Phytogenic / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dactinomycin / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Treatment Outcome. Vincristine / administration & dosage


9. Leuschner I, Harms D, Mattke A, Koscielniak E, Treuner J: Rhabdomyosarcoma of the urinary bladder and vagina: a clinicopathologic study with emphasis on recurrent disease: a report from the Kiel Pediatric Tumor Registry and the German CWS Study. Am J Surg Pathol; 2001 Jul;25(7):856-64
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  • [Title] Rhabdomyosarcoma of the urinary bladder and vagina: a clinicopathologic study with emphasis on recurrent disease: a report from the Kiel Pediatric Tumor Registry and the German CWS Study.
  • Rhabdomyosarcomas (RMS) of the urinary bladder and vagina vary in their biologic and clinical behavior and require different types of treatment.
  • We investigated tumor specimens of 51 urinary bladder RMS and 14 vaginal RMS with regard to histologic subtype, growth pattern, differentiation, and proliferation morphologically and immunohistochemically.
  • Recurrences and/or "second look" specimens from 15 patients after chemotherapy were compared with the primary tumors.
  • BRMS is more common in the vagina (11 BRMS of 14 cases) than in the urinary bladder RMS (15 BRMS of 54 cases).
  • Classical embryonal RMS with a polypoid (exophytic) growth pattern is associated with a more favorable prognosis (92% 10-year survival) than the same type with a diffuse intramural (endophytic) growth pattern (68% 10-year survival, p = 0.02).
  • A marked maturation after chemotherapy was seen in the majority of recurrences and SL specimens, associated with lowered proliferation activity.
  • Two of 12 patients with recurrences showing chemotherapy-induced maturation died of the disease.
  • In conclusion, we determined that polypoid embryonal RMS of both the urinary bladder and the vagina have a comparably good prognosis.
  • Maturation after chemotherapy occurs frequently in RMS.
  • In contrast to the excellent prognosis reported in other studies, we had two patients with fatal outcome despite chemotherapy-induced maturation in the recurrences.
  • [MeSH-major] Rhabdomyosarcoma / pathology. Urinary Bladder Neoplasms / pathology. Vaginal Neoplasms / pathology

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  • (PMID = 11420456.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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10. Maharaj NR, Nimako D, Hadley GP: Multimodal therapy for the initial management of genital embryonal rhabdomyosarcoma in childhood. Int J Gynecol Cancer; 2008 Jan-Feb;18(1):190-2
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  • [Title] Multimodal therapy for the initial management of genital embryonal rhabdomyosarcoma in childhood.
  • Rhabdomyosarcoma is the most common soft tissue sarcoma found in children.
  • A polypoid form of the embryonal type of rhabdomyosarcoma, namely, sarcoma botryoides, is often found in girls younger than 5 years, and typically presents as a polypoid mass protruding from the vagina.
  • Over the years, there has been a shift in the treatment of this condition from radical surgery to a multimodal approach involving conservative surgery with chemotherapy and radiotherapy.
  • We discuss the initial management of a 4-year-old girl with pelvic embryonal rhabdomyosarcoma using a multimodal approach.
  • [MeSH-major] Pelvic Neoplasms / therapy. Rhabdomyosarcoma, Embryonal / therapy
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Female. Humans

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  • (PMID = 17511807.001).
  • [ISSN] 1525-1438
  • [Journal-full-title] International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
  • [ISO-abbreviation] Int. J. Gynecol. Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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11. Ojwang SB, Rana F, Sayed S, Aruasa WK: Embryonal rhabdomyosarcoma with uterine inversion: case report. East Afr Med J; 2006 Mar;83(3):110-3
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  • [Title] Embryonal rhabdomyosarcoma with uterine inversion: case report.
  • Presented here is a 16-year-old nulliparous girl who had embryonal rhabdomyosarcoma causing uterine inversion.
  • She had been referred with a one year history of continuous vaginal bleeding and vaginal swelling.
  • Histology had shown sarcoma botyroides and had received five courses of chemotherapy at the referring hospital.
  • [MeSH-major] Rhabdomyosarcoma, Embryonal / complications. Uterine Inversion / etiology. Uterine Inversion / surgery. Uterine Neoplasms / complications
  • [MeSH-minor] Adolescent. Female. Humans. Hysterectomy. Radiotherapy, Adjuvant. Uterine Hemorrhage. Vagina / physiopathology

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  • (PMID = 16771108.001).
  • [ISSN] 0012-835X
  • [Journal-full-title] East African medical journal
  • [ISO-abbreviation] East Afr Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Kenya
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12. Stankovic ZB, Djuricić S, Stanković DS, Zdravković S, Gazikalović S, Sedlecki K: Minimal invasive treatment of cervical rhabdomyosarcoma in an adolescent girl. J BUON; 2007 Jan-Mar;12(1):121-3
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  • [Title] Minimal invasive treatment of cervical rhabdomyosarcoma in an adolescent girl.
  • Rhabdomyosarcomas are the most common soft tissue sarcomas in childhood and adolescence.
  • In cases of minimal cervical invasion, less invasive local excision in combination with adjuvant chemotherapy has resulted in excellent survival rates with complete preservation of the bladder, rectum, uterus and vagina.
  • Herein we present the case of an adolescent girl with a large rhabdomyosarcoma of the uterine cervix treated with minimal invasive therapy.
  • [MeSH-major] Gynecologic Surgical Procedures. Metrorrhagia / etiology. Rhabdomyosarcoma, Embryonal / surgery. Uterine Cervical Neoplasms / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Female. Humans. Minimally Invasive Surgical Procedures. Tomography, X-Ray Computed. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 17436413.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide
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13. Hester AL, Sweeney TA, Hudson MM, Billups CA, Krasin MJ, Spunt SL: Late effects following treatment for female pelvic rhabdomyosarcoma (RMS). J Clin Oncol; 2004 Jul 15;22(14_suppl):8547

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  • [Title] Late effects following treatment for female pelvic rhabdomyosarcoma (RMS).
  • : 8547 Background Most girls treated for RMS of the pelvic region are cured, but the effects of this treatment on long term health status are poorly documented.
  • Late effects occurring beyond 5 years from diagnosis were recorded and graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events version 3.0.
  • Results Among the 26 patients studied, the median age at diagnosis of RMS was 3.4 years (range, 0.2-17.2 years).
  • The most common tumor sites were vagina (n=7), pelvis/retroperitoneum (n=6), and bladder (n=4).
  • All patients received chemotherapy, which in some cases included an alkylating agent (n=23), doxorubicin (n=16), or etoposide (n=2).
  • Radiotherapy was administered to 22 patients (dose range 16-60.5 Gy, median 46 Gy).
  • Surgical intervention for treatment of late complications was required in fourteen patients (54%).

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  • (PMID = 28013809.001).
  • [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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14. 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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15. Ghaemmaghami F, Karimi Zarchi M, Ghasemi M: Lower genital tract rhabdomyosarcoma: case series and literature review. Arch Gynecol Obstet; 2008 Jul;278(1):65-9
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  • [Title] Lower genital tract rhabdomyosarcoma: case series and literature review.
  • OBJECTIVE: Rhabdomyosarcoma (RMS) of the lower genital tract is a rare tumor.
  • It tends to occur in childhood in the vagina and in rare cases, RMS can originate in the uterine cervix, with a peak incidence in the 2nd decade.
  • All patients presented with vaginal bleeding.
  • The lesions in vagina were clinically staged as stage I (2), stage II (1) and in cervix were stage I (2) and stage III(1).
  • All of the patients were treated with surgery and adjuvant chemotherapy.
  • Two patients with cervical RMS died from the large size and extent of the disease 9 and 11 months after diagnosis, but all patients with vaginal RMS remain alive after a mean follow-up of 38 months.
  • CONCLUSION: Most patients present with vaginal bleeding and a palpable cervical or vaginal mass.
  • While the optimal management of these tumors is uncertain, primary therapy with wide local excision and chemotherapy can result in prolonged survival and cure in patients with early stage RMS.
  • Vaginal lesions have a better prognosis than cervical lesions.
  • [MeSH-major] Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / therapy. Uterine Cervical Neoplasms / pathology. Uterine Cervical Neoplasms / therapy. Vaginal Neoplasms / pathology. Vaginal Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Female. Humans. Neoplasm Recurrence, Local / therapy. Radiotherapy, Adjuvant. Registries. Uterine Hemorrhage / etiology

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  • (PMID = 18427824.001).
  • [ISSN] 0932-0067
  • [Journal-full-title] Archives of gynecology and obstetrics
  • [ISO-abbreviation] Arch. Gynecol. Obstet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 15
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16. Godbole P, Outram A, Wilcox DT, Duffy PG, Sebire NJ: Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications. J Urol; 2006 Oct;176(4 Pt 2):1751-4
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  • [Title] Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications.
  • PURPOSE: Posttreatment genitourinary embryonal rhabdomyosarcoma often shows well differentiated rhabdomyoblasts, which are detectable on routine histological staining.
  • Definite areas of residual undifferentiated rhabdomyosarcoma indicate residual/recurrent disease.
  • However, the recent use of immunohistochemical staining with desmin and myogenin in resected specimens and surveillance biopsies following adjuvant therapy may demonstrate scant positive staining cells that appear undifferentiated on light microscopy.
  • Therefore, we reviewed our retrospective experience with genitourinary embryonal rhabdomyosarcoma to examine the relationship between immunostain positive undifferentiated cells and subsequent clinical outcome.
  • MATERIALS AND METHODS: A total of 14 children with a median age of 2.75 years (range 8 months to 7 years) with genitourinary embryonal rhabdomyosarcoma were identified in the histopathology database.
  • All had biopsy confirmation of the diagnosis, followed by multi-agent chemotherapy.
  • RESULTS: There were 14 cases of genitourinary embryonal rhabdomyosarcoma.
  • In 2 cases (14%) residual embryonal tumor was pathologically confirmed following initial treatment.
  • In 12 cases no obvious residual tumor was present following initial therapy.
  • Rhabdomyosarcoma affected the bladder in 10 cases and the vagina in 2.
  • Ten patients underwent local resection following chemotherapy and 2 underwent followup biopsies only without resection.
  • A total of 11 cases showed well differentiated, posttreatment rhabdomyoblasts that was identifiable on routine hematoxylin and eosin staining with margins apparently free of tumor and 1 showed no morphological evidence of residual rhabdomyosarcoma.
  • Four patients had no further treatment and none had clinical recurrence.
  • All were well 10 years (range 8 to 13) after treatment.
  • Eight patients received further treatment (chemotherapy and/or radiotherapy) based on clinical and pathological findings, followed by further resection in 3.
  • One patient died of disease but 7 were well a median of 7.2 years (range 8 months to 13 years) after treatment.
  • CONCLUSIONS: The significance of undifferentiated myogenin/desmin positive cells in genitourinary embryonal rhabdomyosarcoma in the absence of morphological residual/recurrent embryonal rhabdomyosarcoma remains unclear since such cells can be detected in all cases of posttreatment embryonal rhabdomyosarcoma.
  • In some cases findings are associated with clinical disease recurrence, while others with identical histopathological findings following initial treatment have no clinical sequelae even in the absence of further treatment.
  • In genitourinary embryonal rhabdomyosarcoma close and regular clinical surveillance is essential.
  • [MeSH-major] Desmin / analysis. Myogenin / analysis. Rhabdomyosarcoma, Embryonal / chemistry. Rhabdomyosarcoma, Embryonal / drug therapy. Urogenital Neoplasms / chemistry
  • [MeSH-minor] Child. Child, Preschool. Female. Follow-Up Studies. Humans. Immunohistochemistry. Infant. Male. Prostatic Neoplasms / chemistry. Prostatic Neoplasms / drug therapy. Urinary Bladder Neoplasms / chemistry. Urinary Bladder Neoplasms / drug therapy. Vaginal Neoplasms / chemistry. Vaginal Neoplasms / drug therapy

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  • (PMID = 16945640.001).
  • [ISSN] 0022-5347
  • [Journal-full-title] The Journal of urology
  • [ISO-abbreviation] J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Desmin; 0 / Myogenin
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17. Spunt SL, Sweeney TA, Hudson MM, Billups CA, Krasin MJ, Hester AL: Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors. J Clin Oncol; 2005 Oct 1;23(28):7143-51
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  • [Title] Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors.
  • PURPOSE: To document the spectrum and severity of late effects in female survivors of pelvic rhabdomyosarcoma.
  • PATIENTS AND METHODS: We reviewed the demographic, diagnostic, treatment, and outcome data of the 26 females treated for pelvic rhabdomyosarcoma at our institution between March 1962 and December 1996 who survived free of disease for 5 or more years.
  • Adverse effects that occurred 5 or more years after diagnosis were graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events, version 3.0.
  • RESULTS: The most common tumor sites were vagina (n = 7), pelvis/retroperitoneum (n = 6), and bladder (n = 4).
  • All patients received chemotherapy (alkylating agent, n = 23; doxorubicin, n = 16); 22 received radiotherapy (median dose, 46 Gy).
  • CONCLUSION: The burden of late effects in girls treated for pelvic rhabdomyosarcoma is significant and does not seem to be diminishing with advances in treatment.
  • Prospective studies are needed to better assess the impact of these late effects on quality of life and functional outcome, and to refine the treatment approach to pelvic rhabdomyosarcoma.
  • [MeSH-major] Cost of Illness. Pelvic Neoplasms / pathology. Rhabdomyosarcoma / pathology

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  • (PMID = 16192598.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 23099; United States / NCI NIH HHS / CA / P30 CA 21765
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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18. Kusafuka T, Oue T, Tazuke Y, Kuroda S, Udatsu Y, Shimizu Y, Okada A: Vaginal reconstruction in a patient with rhabdomyosarcoma previously treated by total vaginectomy. J Pediatr Surg; 2002 Sep;37(9):1365-7

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  • [Title] Vaginal reconstruction in a patient with rhabdomyosarcoma previously treated by total vaginectomy.
  • In vaginal rhabdomyosarcoma, because of effectiveness of multiple-agent chemotherapy and brachytherapy, function-preserving treatment without radical surgery has been recently recommended.
  • The authors report experience of successful vaginal reconstruction in a rhabdomyosarcoma patient who had total vaginectomy with preservation of the uterus.
  • Because continuity from the vaginal introitus to the uterus was established, fertility can be expected for this patient.
  • [MeSH-major] Vagina / surgery
  • [MeSH-minor] Child. Female. Gynecologic Surgical Procedures / methods. Humans. Rhabdomyosarcoma, Embryonal / surgery. Vaginal Neoplasms / surgery

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  • [Copyright] Copyright 2002, Elsevier Science (USA). All rights reserved.
  • (PMID = 12194137.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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19. Segura Huerta A, López Tendero P, Yuste Izquierdo A, Gironés Sarrió R, Pérez Fidalgo J, Aparicio Urtasun J: [Embryonal rhabdomyosarcoma of the prostate. Report of a case and review of the literature]. Actas Urol Esp; 2001 Feb;25(2):144-9
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  • [Title] [Embryonal rhabdomyosarcoma of the prostate. Report of a case and review of the literature].
  • The rhabdomyosarcoma (RMS) is a tumour derived from the mesenchymal cells that give origin to striate muscle.
  • There are two principal types: alveolar RMS and embrionary RMS.
  • It can appear in any localization, being the most frequent the periorbitary area and the paranasal sinuses, and the second in frequency the genitourinary system (bladder, prostate and vagina).
  • The therapeutic management is multidisciplinary and local therapies must always be accompanied with polychemotherapy.
  • With combined treatments a curation rate of almost 90% can be reached in good prognosis cases, although it drops to 20% in those with disfavourable prognostic factors.
  • The protocols with higher chemotherapy doses and subsequent rescue with blood-borne progenitor cells have not yet proved their efficiency.
  • We present the case of a fifteen-year old patient with an embrionary rhabdomyosarcoma of the prostate, we analyze the clinical evolution and we revise the present therapeutic management of these neoplasies.
  • [MeSH-major] Bone Marrow Neoplasms / secondary. Prostatic Neoplasms / pathology. Rhabdomyosarcoma / secondary

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  • (PMID = 11345801.001).
  • [ISSN] 0210-4806
  • [Journal-full-title] Actas urologicas espanolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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20. Ong LY, Hwang WS, Wong A, Chan MY, Chui CH: Perivascular epithelioid cell tumour of the vagina in an 8 year old girl. J Pediatr Surg; 2007 Mar;42(3):564-6
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  • [Title] Perivascular epithelioid cell tumour of the vagina in an 8 year old girl.
  • We present the first reported case of PEComa of the vagina diagnosed in an 8-year-old girl.
  • This was initially diagnosed on biopsy as an embryonal rhabdomyosarcoma, and the patient underwent 3 cycles of chemotherapy according to the Mesenchymal Malignant Tumour 1989 protocol.
  • Six months after surgery, she remains free of tumor and is on follow-up with regular local examinations under anesthesia and computed tomography scans.
  • Our case highlights the importance of an accurate initial diagnosis, and we stress the importance of regular surveillance over a significant period.
  • [MeSH-major] Epithelioid Cells / pathology. Sarcoma / therapy. Vaginal Neoplasms / therapy

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  • (PMID = 17336201.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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21. Cecchetto G, Carretto E, Bisogno G, Dall'Igna P, Ferrari A, Scarzello G, Donfrancesco A, Alaggio R, Indolfi P, Carli M: Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee. Pediatr Blood Cancer; 2008 Nov;51(5):593-7
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  • [Title] Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee.
  • PROCEDURE: We analyzed data from 39 patients (age: 0.5-194 months, median 52) who were enrolled between 1988 and 2005 in 2 consecutive Italian Studies, RMS 88 and RMS 96.
  • All achieved a complete resection of the residual tumor after neoadjuvant chemotherapy; 27 did not receive any other local treatment: pelvic 8, extremities 6, head-neck-non-parameningeal 5, orbit 1, genito-urinary-bladder-prostate 3, trunk 2, abdomen 1, vagina 1; 12 were given RT (32-45 Gy), 5 before and 7 after the operation: genito-urinary-bladder-prostate 3, pelvic 3, abdominal 1, extremities 1, head-neck-parameningeal 1, head-neck-non-parameningeal 1, vagina 1, orbit 1.
  • All received postoperative chemotherapy.
  • Without RT: 16/27 maintained the first complete remission, however 1/16 died due to a second tumor; 8 suffered from local relapse (4 pelvic, 1 orbit, 1 vagina, 1 head-neck-non-parameningeal, 1 abdomen) and 3 of them died, 3 showed a metastatic recurrence (2 extremities, 1 pelvic) and 1 died.
  • [MeSH-major] Rhabdomyosarcoma / radiotherapy. Rhabdomyosarcoma / surgery. Second-Look Surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Child. Child, Preschool. Clinical Trials as Topic. Combined Modality Therapy. Female. Humans. Infant. Kaplan-Meier Estimate. Male. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / therapy. Radiotherapy, Adjuvant. Sarcoma

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18668515.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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22. Perez-Brayfield MR, Smith EA, Gatti J, Bergsagel J, Namnoun J: Vaginal reconstruction using a vertical rectus abdominis myocutaneous flap in a child: a case report. J Pediatr Surg; 2001 Jul;36(7):1070-2
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  • [Title] Vaginal reconstruction using a vertical rectus abdominis myocutaneous flap in a child: a case report.
  • Rhabdomyosarcoma is the most common soft tissue sarcoma in infants and children younger than 15 years old.
  • Multimodal treatment using chemotherapy, radiation, and surgery have increased survival.
  • Because of the potential for long-term survival, the importance of organ preservation and vaginal and pelvic reconstruction has been recognized.
  • The authors report a case in which special circumstances led to vaginal reconstruction concomitantly with exenteration surgery.
  • [MeSH-major] Reconstructive Surgical Procedures / methods. Rhabdomyosarcoma, Embryonal / surgery. Surgical Flaps. Urogenital Neoplasms / surgery. Vagina / surgery

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  • [Copyright] Copyright 2001 by W.B. Saunders Company.
  • (PMID = 11431782.001).
  • [ISSN] 0022-3468
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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23. Raspagliesi F, Ditto A, Martinelli F, Hanozet F, Solima E, Grijuela B, Haeusler E, Fontanelli R: Nerve-sparing radical vaginectomy: two case reports and description of the surgical technique. Int J Gynecol Cancer; 2009 May;19(4):794-7
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  • CASE REPORTS: A 58-year-old woman with a diagnosis of locally advanced cervical cancer underwent neoadjuvant chemotherapy with local and nodal progression of disease.
  • A 14-year-old adolescent girl with a diagnosis of recurrent embryonal-botryoidal rhabdomyosarcoma was referred to us.
  • She underwent 6 courses of chemotherapy with persistent disease.
  • Two months after the surgical procedure, she was able to void the bladder.
  • [MeSH-major] Pelvis / innervation. Rhabdomyosarcoma, Embryonal / surgery. Uterine Cervical Neoplasms / surgery. Vagina / surgery. Vaginal Neoplasms / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Gynecologic Surgical Procedures / methods. Humans. Middle Aged. Neoadjuvant Therapy. Urinary Bladder / innervation

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  • (PMID = 19509590.001).
  • [ISSN] 1525-1438
  • [Journal-full-title] International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
  • [ISO-abbreviation] Int. J. Gynecol. Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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24. Gruessner SE, Omwandho CO, Dreyer T, Blütters-Sawatzki R, Reiter A, Tinneberg HR, Bohle RM: Management of stage I cervical sarcoma botryoides in childhood and adolescence. Eur J Pediatr; 2004 Aug;163(8):452-6
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  • Rhabdomyosarcomas are the most common soft tissue sarcomas in childhood.
  • The botryoid variant arises in infancy from the vagina or urinary bladder and extremely rarely from the uterine cervix.
  • Treatment regimes range from local excision of the tumour to radical hysterectomy with adjuvant multidrug therapy and/or radiotherapy.
  • In cases of minimal cervical invasion, the less invasive local excision in combination with adjuvant chemotherapy has resulted in excellent survival rates with complete functional preservation of the bladder, rectum, vagina, and ovaries.
  • CONCLUSION: based on the literature review and our own observation, we recommend minor surgical approaches in combination with chemotherapy as the treatment of choice for early stage I cervical rhabdomyosarcoma.
  • [MeSH-major] Rhabdomyosarcoma, Embryonal / pathology. Rhabdomyosarcoma, Embryonal / therapy. Uterine Cervical Neoplasms / pathology. Uterine Cervical Neoplasms / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols. Chemotherapy, Adjuvant. Child, Preschool. Dactinomycin / therapeutic use. Female. Humans. Ifosfamide / therapeutic use. Neoplasm Staging. Vincristine / therapeutic use

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  • [Copyright] Copyright 2004 Springer-Verlag
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  • (PMID = 15173941.001).
  • [ISSN] 0340-6199
  • [Journal-full-title] European journal of pediatrics
  • [ISO-abbreviation] Eur. J. Pediatr.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; UM20QQM95Y / Ifosfamide
  • [Number-of-references] 32
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25. Atlante M, Dionisi B, Cioni M, Di Ruzza D, Sedati P, Mariani L: Sarcoma botryoides of the uterine cervix in a young woman: a case report. Eur J Gynaecol Oncol; 2000;21(5):504-6
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  • Among the rhabdomyosarcomas, which represent an uncommon neoplasm of mesoderm origin comprising about 1% of all female genital malignancies, sarcoma botryoides (SB) is an embrional variant usually found in the vagina of infants and young children under 16 years of age.
  • The authors report a case of embryonal rhabdomyosarcoma of the uterine cervix which occurred in a young woman treated by radical surgery, followed by adjuvant chemotherapy and radiotherapy.
  • [MeSH-major] Rhabdomyosarcoma, Embryonal / therapy. Uterine Cervical Neoplasms / therapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Female. Humans. Radiotherapy, Adjuvant

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  • (PMID = 11198044.001).
  • [ISSN] 0392-2936
  • [Journal-full-title] European journal of gynaecological oncology
  • [ISO-abbreviation] Eur. J. Gynaecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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26. Miyamoto T, Shiozawa T, Nakamura T, Konishi I: Sarcoma botryoides of the uterine cervix in a 46-year-old woman: case report and literature review. Int J Gynecol Pathol; 2004 Jan;23(1):78-82
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  • Sarcoma botryoides, which usually occurs in the vagina in childhood, is rare in the female reproductive tract of adult women, especially those over the age of 40.
  • Postoperative chemotherapy (vincristine and actinomycin D) was given according to the Intergroup Rhabdomyosarcoma Study protocol, and the patient was alive without recurrence 45 months postoperatively.
  • [MeSH-major] Rhabdomyosarcoma, Embryonal / pathology. Uterine Cervical Neoplasms / pathology
  • [MeSH-minor] Age of Onset. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Middle Aged. MyoD Protein / metabolism. Myogenin / metabolism. Neoplasm Recurrence, Local. Polyps / pathology

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  • (PMID = 14668557.001).
  • [ISSN] 0277-1691
  • [Journal-full-title] International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists
  • [ISO-abbreviation] Int. J. Gynecol. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MYOG protein, human; 0 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / Myogenin
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27. Polish Paediatric Solid Tumours Study Group, Bień E, Stachowicz-Stencel T, Kazanowska B, Balcerska A, Balwierz W, Chybicka A, Dłuzniewska A, Drozyńska E, Kurylak A, Matysiaks M, Krawczuk-Rybak M, Rychłowska M, Solarz E, Sopyło B, Stencels D, Wachowiaks J, Wieczorek M, Woźniak W, Wysocki M: [Genitourinary soft tissue sarcomas located outside bladder and prostate in children treated according to the CWS-96 protocol--report from the Polish Paediatric Solid Tumours Study Group]. Med Wieku Rozwoj; 2005 Jul-Sep;9(3 Pt 2):507-15
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  • [Title] [Genitourinary soft tissue sarcomas located outside bladder and prostate in children treated according to the CWS-96 protocol--report from the Polish Paediatric Solid Tumours Study Group].
  • AIM: Analysis of therapy efficacy in non-bladder/prostate genitourinary sarcomas in children treated from I'1997 to VI'2003 with CWS-96 protocol in Poland.
  • RESULTS: Primary site: testes - 9 patients, paratesticular region - 6, uterus - 2, vagina and ovary-1 of each.
  • Primary tumour exceeded 5cm and/or invaded surrounding tissues in 7 patients (37%).
  • Six of 7 patients with macroscopic tumour residues responded to chemotherapy (CR-4, GR-2).
  • Radiotherapy (23,5-54 Gy) was given to 8 patients.
  • 3 children developed local relapse, 3 patients died (16%): 2 due to neoplasm progression, 1 of neutropenia-related sepsis.
  • 2) chemotherapy and radiotherapy were accompanied by severe but transient myelosupression in the HR group.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / drug therapy. Sarcoma / diagnosis. Sarcoma / drug therapy. Urogenital Neoplasms / diagnosis. Urogenital Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Lymphatic Metastasis. Male. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Treatment Outcome

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  • (PMID = 16719163.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] Clinical Trial; English Abstract; Journal Article
  • [Publication-country] Poland
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28. Calleja Aguayo E, Delgado Alvira R, Elías Pollina J, Romeo Ulecia M, González Esgueda A, Esteban Ibarz JA: [Differential diagnosis of the interlabial masses in girls under 5-years-old]. Cir Pediatr; 2010 Jan;23(1):28-31
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  • [Title] [Differential diagnosis of the interlabial masses in girls under 5-years-old].
  • The masses that we can be found at interlabial in a girl are a extensive spectrum of heterogenous lesions and often there is a confusion in diagnosis, management and prognosis.
  • RESULTS: A prolapsed urethra is presented in a 4-year-old black girl with vaginal bleeding from edamatous periurethral mass.
  • The botryoid sarcoma appears in a 1-year-old girl with ulcerated polypoid mass of 2 cm from vagina.
  • All cases were treated with surgery except the paraurethral cyst that drained spontaneously and the rhabdomyosarcoma was also treated with chemotherapy.
  • The prognosis of rhabdomyosarcoma vaginal is good with surgery and chemotherapy.
  • [MeSH-major] Vulvar Diseases / diagnosis
  • [MeSH-minor] Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Infant, Newborn

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  • (PMID = 20578574.001).
  • [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] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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29. Groff DB: Pelvic neoplasms in children. J Surg Oncol; 2001 May;77(1):65-71
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  • The most common neoplasms are rhabdomyosarcomas of the bladder, prostate, and vagina; sacrococcygeal teratoma; and the germ cell tumors, including teratomas, endodermal sinus tumors, and the choriocarcinomas.
  • Rapidly improving chemotherapy for all of these lesions has resulted in a changed role for the surgeon.
  • Survival for all types of childhood pelvic neoplasms has improved dramatically under the influence of the combined children's cancer study groups, which have been functioning for the last 20 years.
  • Because of the complexities of diagnosis and treatment of these pelvic neoplasms in childhood and because the survival rates have dramatically improved with the most current therapy, these children should be cared for in a children's center that is part of the major children's cancer study groups.
  • [MeSH-major] Pelvic Neoplasms. Pelvis / anatomy & histology. Rhabdomyosarcoma
  • [MeSH-minor] Child. Female. Humans. Infant. Male. Neoplasms, Germ Cell and Embryonal / pathology. Prostatic Neoplasms / pathology. Urinary Bladder Neoplasms / pathology. Uterine Neoplasms / pathology. Vaginal Neoplasms / pathology. Vulvar Neoplasms / pathology

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  • [Copyright] Copyright 2001 Wiley-Liss, Inc.
  • (PMID = 11344486.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 36
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