History

The clinical presentation of rhabdomyosarcoma (RMS) varies according to the site of presentation. Most patients, however, present with a painless mass, often discovered after minor trauma. Growth of the mass may impact adjacent structures, thus causing symptoms, including pain. When large, RMS may impinge on adjacent structures and lead to secondary problems such as bowel obstruction or respiratory distress.

Other symptoms are determined by location of the mass. Tumors originating in the head and neck may present as a mass or as signs and symptoms of central nervous system involvement due to intracranial extension of the tumor or infiltration of the cranial nerves, meninges, or brainstem.^{[22, 23]} RMS of the orbit may result in disconjugate gaze, whereas RMS of the uterus, cervix, or bladder may result in menorrhagia or metrorrhagia or difficulty voiding.

Physical Examination

In many instances, physical examination may be positive only for lymphadenoapathy in the region of the tumor. Other findings are dependent on tumor location. Secondary signs may include anemia, thrombocytopenia and neutropenia. Many patients have no physical examination findings at the time of diagnosis.

Differential Diagnoses

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Media Gallery

Proper orientation of the biopsy allows complete resection at second operation.
The histologic findings in rhabdomyosarcoma.
Sentinel node biopsy after lymphatic mapping in a child with rhabdomyosarcoma. Notice that the incision is oriented to allow extension or incorporation of the incision should further dissection be necessary. The sentinel node should be blue and should have high counts of radioactive tracer signal when checked with the gamma probe.

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Table 1. TNM Classification of Rhabdomyosarcoma

Tumor (T)	T1 T1a T1b	Tumor confined to site of origin < 5 cm ≥5 cm
	T2 T2a T2b	Tumor extending into surrounding tissue < 5 cm ≥5 cm
Node (N)	N0	No lymph node involvement
	N1	Clinical involvement of lymph nodes
	NX	Unknown lymph node status
Metastasis (M)	M0	No metastasis
	M1	Metastasis present

Table 2. Pretreatment Staging of Rhabdomyosarcoma.

Stage	Sites	T	Size	N	M
1	Orbit, head/neck (no parameningeal involvement), genitourinary (no bladder/prostate involvement)	T1 or T2	< 5 cm or ≥5 cm	N0 or N1 or Nx	M0
2	Bladder/prostate, extremity, cranial, head/neck parameningeal, other (trunk, retroperitoneum, thorax)	T1 or T2	< 5 cm	N0 or Nx	M0
3	Bladder/prostate, extremity, cranial, head/neck parameningeal, other (trunk, retroperitoneum, thorax)	T1 or T2	< 5 cm	N1	M0
3		T1 or T2	≥5 cm	N0 or N1 or Nx	M0
4	Any	T1 or T2	< 5 cm or ≥5 cm	N0or N1	M1

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Author

Roshni Dasgupta, MD, MPH Associate Professor of Surgery, University of Cincinnati College of Medicine; Attending Surgeon, Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center

Roshni Dasgupta, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Canadian Association of Pediatric Surgeons, Children's Oncology Group, International Pediatric Endosurgery Group, International Society of Pediatric Oncology, Massachusetts General Hospital Surgical Society, Society of American Gastrointestinal and Endoscopic Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Inna Lobeck, MD Resident Physician, Department of General Surgery, Detroit Medical Center, Wayne State University School of Medicine

Inna Lobeck, MD is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, Detroit Surgical 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

Eugene S Kim, MD, FACS, FAAP Director, Division of Pediatric Surgery, Vice Chair, Department of Surgery, Cedars Sinai Medical Center; Professor of Surgery and Pediatrics, Division of Pediatric Surgery, Division of Hematology, Oncology, Blood and Marrow Transplantation, Keck School of Medicine of the University of Southern California

Eugene S Kim, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Surgical Association, Association for Academic Surgery, Children's Oncology Group, Society of Laparoscopic and Robotic Surgeons, Society of University Surgeons, Texas Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

Richard Andrassy, MD Denton A Cooley, MD Chair and Professor, Department of Surgery, University of Texas at Houston Health Science Center, Chief, Section of Pediatric Surgery, MD Anderson Cancer Center, Chief Surgeon, Department of Surgery, Memorial-Hermann Hospital

Richard Andrassy, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Physician Leadership, American College of Surgeons, American Society for Nutrition, American Society of Pediatric Hematology/Oncology, American Trauma Society, Association for Academic Surgery, International College of Surgeons, Society of Surgical Oncology, Southwestern Surgical Congress

Disclosure: Nothing to disclose.

Diana Farmer, MD Professor and Chief of Pediatric Surgery, Vice Chair, Department of Surgery, University of California, San Francisco, School of Medicine; Surgeon-in-Chief, UCSF Children's Hospital

Diana Farmer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association

Disclosure: Nothing to disclose.

Holly L Neville, MD Associate Professor of Clinical Surgery, Division of Pediatric Surgery, University of Miami, Leonard M Miller School of Medicine

Holly L Neville, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association of Women Surgeons

Disclosure: Nothing to disclose.

Acknowledgements

Nicholas A Shorter, MD Professor of Clinical Surgery and Clinical Pediatrics, State University of New York Downstate University; Division Chief, Department of Surgery, Division of Pediatric Surgery, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Sections Pediatric Rhabdomyosarcoma Surgery
Overview
Presentation
DDx
Workup
Treatment
Questions & Answers
Media Gallery
Tables
References

Pediatric Rhabdomyosarcoma Surgery Clinical Presentation

History

Physical Examination

References

Tables

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