Further Outpatient Care

CBC count: Perform a CBC count twice each week in patients receiving therapy by using granulocyte-colony stimulating factor (G-CSF) so that G-CSF can be discontinued when the absolute neutrophil count has reached a predetermined level (usually 1 or 5 X 10⁹/L [1000 or 5000/µL]). See the Absolute Neutrophil Count calculator.

Blood chemistry: Monitor blood chemistry results and liver function in patients receiving parenteral nutrition or in those who have a history of toxicity, especially if the patient continues to receive nephrotoxic or hepatotoxic antibiotics or other drugs.

Chemotherapy: Depending on the protocol, some chemotherapy regimens (eg, vincristine, dactinomycin in particular) can be administered on an outpatient basis.

Monitoring for recurrence: Continue to perform blood tests and radiographic scans on an outpatient basis, with the frequency decreasing over time. In general, patients should be examined every 3 months for the first year, every 6 months for the second and third years, and yearly thereafter.

Long-term follow-up care: At 5 or longer after the end of therapy, patients are considered to be long-term survivors. Patients should be examined annually at a late-effects clinic and monitored with appropriate studies depending on the type of therapy they received. Visits may include hormonal, psychosocial, and neurologic evaluations, as well as follow-up examinations by the radiotherapist.

Further Inpatient Care

Chemotherapy: Chemotherapy cycles are usually administered every 3 weeks (although vincristine is periodically given weekly) in patients with rhabdomyosarcoma (RMS), depending on recovery of the bone marrow. Patients receiving cycles that include cyclophosphamide, ifosfamide, and etoposide generally require inpatient admission for drug administration and monitoring.

Fever and neutropenia: Admission is required to administer intravenous (IV) antibiotics and to monitor patients.

Other reasons for inpatient care: Patients may require admission for a multitude of other medical problems during the chemotherapy phase of treatment, including varicella infection (to administer IV acyclovir and to monitor), mucositis (resulting from narcotics use), dehydration, meningitis, constipation, fungal pneumonia, and cystitis, among others.

Inpatient & Outpatient Medications

Inpatient and outpatient medications include the following:

Trimethoprim-sulfamethoxazole: Prophylaxis against pneumocystic pneumonia should continue until 6 months after the end of chemotherapy.
Fluconazole: Systemic fungal prophylaxis is not necessary.
Clotrimazole: Prophylactic therapy for thrush may be discontinued after chemotherapy is completed.
Chlorhexidine mouth rinse: Prophylaxis against gingivitis and other mouth infections may be discontinued after chemotherapy is completed.

Transfer

Although major cancer therapy should take place at a center with pediatric oncologists, the child's referring pediatrician or general practitioner should continue to play an important role in the child's care throughout treatment.

The referring physician can be critical in performing the first evaluation of an illness, particularly if the child lives far from an oncology center.

Questions

Punyko JA, Mertens AC, Baker KS, et al. Long-term survival probabilities for childhood rhabdomyosarcoma. A population-based evaluation. Cancer. 2005 Apr 1. 103(7):1475-83. [QxMD MEDLINE Link].
Rao AA, Naheedy JH, Chen JY, Robbins SL, Ramkumar HL. A clinical update and radiologic review of pediatric orbital and ocular tumors. J Oncol. 2013. 2013:975908. [QxMD MEDLINE Link]. [Full Text].
Rahman HA, Sedky M, Mohsen I, Taha H, Loaye I, Zaghloul MS, et al. Outcome of pediatric parameningeal rhabdomyosarcoma. The Children Cancer Hospital, Egypt, experience. J Egypt Natl Canc Inst. 2013 Jun. 25(2):79-86. [QxMD MEDLINE Link].
Dasgupta R, Fuchs J, Rodeberg D. Rhabdomyosarcoma. Semin Pediatr Surg. 2016 Oct. 25 (5):276-283. [QxMD MEDLINE Link].
Arndt CA, Crist WM. Common musculoskeletal tumors of childhood and adolescence. N Engl J Med. 1999 Jul 29. 341(5):342-52. [QxMD MEDLINE Link].
Stout AP. Rhabdomyosarcoma of the skeletal muscles. Ann Surg. 1946. 123:447-72.
El Demellawy D, McGowan-Jordan J, de Nanassy J, Chernetsova E, Nasr A. Update on molecular findings in rhabdomyosarcoma. Pathology. 2017 Apr. 49 (3):238-246. [QxMD MEDLINE Link].
Pappo AS, Shapiro DN, Crist WM, Maurer HM. Biology and therapy of pediatric rhabdomyosarcoma. J Clin Oncol. 1995 Aug. 13(8):2123-39. [QxMD MEDLINE Link].
Barr FG. Molecular genetics and pathogenesis of rhabdomyosarcoma. J Pediatr Hematol Oncol. 1997 Nov-Dec. 19(6):483-91. [QxMD MEDLINE Link].
Sumegi J, Streblow R, Frayer RW, et al. Recurrent t(2;2) and t(2;8) translocations in rhabdomyosarcoma without the canonical PAX-FOXO1 fuse PAX3 to members of the nuclear receptor transcriptional coactivator family. Genes Chromosomes Cancer. 2010 Mar. 49(3):224-36. [QxMD MEDLINE Link]. [Full Text].
Merlino G, Helman LJ. Rhabdomyosarcoma--working out the pathways. Oncogene. 1999 Sep 20. 18(38):5340-8. [QxMD MEDLINE Link].
Arndt CA. Risk stratification of rhabdomyosarcoma. Am Soc Clin Oncol Educ Book. 2013. 2013:415-9. [QxMD MEDLINE Link].
Gripp KW. Tumor predisposition in Costello syndrome. Am J Med Genet C Semin Med Genet. 2005 Aug 15. 137C(1):72-7. [QxMD MEDLINE Link].
Grufferman S, Ruymann F, Ognjanovic S, Erhardt EB, Maurer HM. Prenatal X-ray exposure and rhabdomyosarcoma in children: a report from the children''s oncology group. Cancer Epidemiol Biomarkers Prev. 2009 Apr. 18(4):1271-6. [QxMD MEDLINE Link]. [Full Text].
Oberlin O, Rey A, Lyden E, et al. Prognostic factors in metastatic rhabdomyosarcomas: results of a pooled analysis from United States and European cooperative groups. J Clin Oncol. 2008 May 10. 26(14):2384-9. [QxMD MEDLINE Link].
Bisogno G, Ferrari A, Prete A, et al. Sequential high-dose chemotherapy for children with metastatic rhabdomyosarcoma. Eur J Cancer. 2009 Nov. 45(17):3035-41. [QxMD MEDLINE Link].
Mazzoleni S, Bisogno G, Garaventa A, et al. Outcomes and prognostic factors after recurrence in children and adolescents with nonmetastatic rhabdomyosarcoma. Cancer. 2005 Jul 1. 104(1):183-90. [QxMD MEDLINE Link].
Punyko JA, Mertens AC, Gurney JG, et al. Long-term medical effects of childhood and adolescent rhabdomyosarcoma: a report from the childhood cancer survivor study. Pediatr Blood Cancer. 2005 Jun 15. 44(7):643-53. [QxMD MEDLINE Link].
Qualman SJ, Coffin CM, Newton WA, et al. Intergroup Rhabdomyosarcoma Study: update for pathologists. Pediatr Dev Pathol. 1998 Nov-Dec. 1(6):550-61. [QxMD MEDLINE Link].
Harel M, Ferrer FA, Shapiro LH, Makari JH. Future directions in risk stratification and therapy for advanced pediatric genitourinary rhabdomyosarcoma. Urol Oncol. 2016 Feb. 34 (2):103-15. [QxMD MEDLINE Link].
Williamson D, Missiaglia E, de Reyniès A, Pierron G, Thuille B, Palenzuela G, et al. Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma. J Clin Oncol. 2010 May 1. 28 (13):2151-8. [QxMD MEDLINE Link].
Seki M, Nishimura R, Yoshida K, et al. Integrated genetic and epigenetic analysis defines novel molecular subgroups in rhabdomyosarcoma. Nat Commun. 2015 Jul 3. 6:7557. [QxMD MEDLINE Link].
Shern JF, Chen L, Chmielecki J, et al. Comprehensive genomic analysis of rhabdomyosarcoma reveals a landscape of alterations affecting a common genetic axis in fusion-positive and fusion-negative tumors. Cancer Discov. 2014 Feb. 4 (2):216-31. [QxMD MEDLINE Link].
Weigel BJ, Lyden E, Anderson JR, Meyer WH, Parham DM, Rodeberg DA, et al. Intensive Multiagent Therapy, Including Dose-Compressed Cycles of Ifosfamide/Etoposide and Vincristine/Doxorubicin/Cyclophosphamide, Irinotecan, and Radiation, in Patients With High-Risk Rhabdomyosarcoma: A Report From the Children's Oncology Group. J Clin Oncol. 2016 Jan 10. 34 (2):117-22. [QxMD MEDLINE Link].
Mulcahy N. Trial Caps 4 Decades of Failure With Pediatric Sarcoma. Medscape Medical News. Available at http://www.medscape.com/viewarticle/856680#vp_2. December 31, 2015; Accessed: May 1, 2016.
Nelson R. New Drug Combos Promising in Higher-Risk Rhabdomyosarcoma. Medscape Medical News. Available at http://www.medscape.com/viewarticle/826251. Accessed: June 9, 2014.
Mascarenhas L, Meyer WH, Lyden E. et al. Randomized phase II trial of bevacizumab and temsirolimus in combination with vinorelbine (V) and cyclophosphamide (C) for first relapse/disease progression of rhabdomyosarcoma (RMS): A report from the Children’s Oncology Group (COG). ASCO Meeting Abstracts. Available at http://abstracts.asco.org/144/AbstView_144_126151.html. Accessed: June 9, 2014.
Hawkins DS, Anderson JR, Mascarenhas L, et al. Vincristine, dactinomycin, cyclophosphamide (VAC) versus VAC/V plus irinotecan (VI) for intermediate-risk rhabdomyosarcoma (IRRMS): A report from the Children’s Oncology Group Soft Tissue Sarcoma Committee. ASCO Meeting Abstracts. Available at http://abstracts.asco.org/144/AbstView_144_126983.html. Accessed: June 9, 2014.
Bisogno G, De Salvo GL, Bergeron C, et al. Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005): a multicentre, open-label, randomised, phase 3 trial. Lancet Oncol. 2019 Nov. 20 (11):1566-75. [QxMD MEDLINE Link]. [Full Text].
Nelson R. New Standard of Care in Rhabdomyosarcoma?. Medscape Medical News. 2019 Oct 18. Available at https://www.medscape.com/viewarticle/920057.
De Corti F, Dall'Igna P, Bisogno G, et al. Sentinel node biopsy in pediatric soft tissue sarcomas of extremities. Pediatr Blood Cancer. 2009 Jan. 52(1):51-4. [QxMD MEDLINE Link].
Wolden SL, Wexler LH, Kraus DH, et al. Intensity-modulated radiotherapy for head-and-neck rhabdomyosarcoma. Int J Radiat Oncol Biol Phys. 2005 Apr 1. 61(5):1432-8. [QxMD MEDLINE Link].
Yock T, Schneider R, Friedmann A, et al. Proton radiotherapy for orbital rhabdomyosarcoma: clinical outcome and a dosimetric comparison with photons. Int J Radiat Oncol Biol Phys. 2005 Nov 15. 63(4):1161-8. [QxMD MEDLINE Link].
Million L, Anderson J, Breneman J, et al. Influence of Noncompliance With Radiation Therapy Protocol Guidelines and Operative Bed Recurrences for Children With Rhabdomyosarcoma and Microscopic Residual Disease: A Report From the Children's Oncology Group. Int J Radiat Oncol Biol Phys. 2011 Jun 1. 80(2):333-8. [QxMD MEDLINE Link].
Currier MA, Adams LC, Mahller YY, et al. Widespread intratumoral virus distribution with fractionated injection enables local control of large human rhabdomyosarcoma xenografts by oncolytic herpes simplex viruses. Cancer Gene Ther. 2005 Apr. 12(4):407-16. [QxMD MEDLINE Link].
Modak S, Guo HF, Humm JL, et al. Radioimmunotargeting of human rhabdomyosarcoma using monoclonal antibody 8H9. Cancer Biother Radiopharm. 2005 Oct. 20(5):534-46. [QxMD MEDLINE Link].
Bersani F, Taulli R, Accornero P, et al. Bortezomib-mediated proteasome inhibition as a potential strategy for the treatment of rhabdomyosarcoma. Eur J Cancer. 2008 Apr. 44(6):876-84. [QxMD MEDLINE Link].
Cao L, Yu Y, Darko I, et al. Addiction to elevated insulin-like growth factor i receptor and initial modulation of the AKT pathway define the responsiveness of rhabdomyosarcoma to the targeting antibody. Cancer Res. 2008 Oct 1. 68(19):8039-48. [QxMD MEDLINE Link].
Klingebiel T, Boos J, Beske F, et al. Treatment of children with metastatic soft tissue sarcoma with oral maintenance compared to high dose chemotherapy: report of the HD CWS-96 trial. Pediatr Blood Cancer. 2008 Apr. 50(4):739-45. [QxMD MEDLINE Link].
Fulda S. Molecular Targeted Therapies for Rhabdomyosarcoma: Focus on Hedgehog and Apoptosis Signaling. Klin Padiatr. 2013 May. 225(3):115-119. [QxMD MEDLINE Link].
Pappo AS, Lyden E, Breitfeld P, et al. Two consecutive phase II window trials of irinotecan alone or in combination with vincristine for the treatment of metastatic rhabdomyosarcoma: the Children's Oncology Group. J Clin Oncol. 2007 Feb 1. 25(4):362-9. [QxMD MEDLINE Link].
Weiss AR, Lyden ER, Anderson JR, Hawkins DS, Spunt SL, Walterhouse DO, et al. Histologic and Clinical Characteristics Can Guide Staging Evaluations for Children and Adolescents With Rhabdomyosarcoma: A Report From the Children's Oncology Group Soft Tissue Sarcoma Committee. J Clin Oncol. 2013 Sep 10. 31(26):3226-3232. [QxMD MEDLINE Link].

Media Gallery

Axial CT scan of rhabdomyosarcoma in the left middle ear. Image provided by Suresh Muhkerji, MD, Department of Radiology, University of North Carolina Hospitals.

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Author

Timothy P Cripe, MD, PhD, FAAP Chief, Division of Hematology/Oncology/BMT, Gordon Teter Endowed Chair in Pediatric Cancer, Nationwide Children's Hospital; Professor of Pediatrics, Ohio State University College of Medicine

Timothy P Cripe, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Gene and Cell Therapy, American Society of Pediatric Hematology/Oncology, Connective Tissue Oncology Society, Society for Pediatric Research, Children's Oncology Group

Disclosure: Nothing to disclose.

Coauthor(s)

Bhuvana Setty, MD Assistant Professor of Clinical Pediatrics, Division of Pediatric Hematology/Oncology/BMT, Nationwide Children's Hospital, Ohio State University College of Medicine

Bhuvana Setty, MD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, Connective Tissue Oncology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Acknowledgements

Samuel Gross, MD Professor Emeritus, Department of Pediatrics, University of Florida College of Medicine; Clinical Professor, Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine; Adjunct Professor, Department of Pediatrics, Duke University School of Medicine

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.