Rhabdomyomas Treatment & Management

  • Author: Karl J D'Silva; Chief Editor: Mary C Mancini, MD, PhD   more...
 
Updated: Aug 11, 2011
 

Medical Care

Patients with adult rhabdomyoma may experience progressive difficulties in breathing and swallowing. In such instances, nasal oxygen may help patients with breathing difficulties. In circumstances in which swallowing is extremely difficult, supplemental intravenous fluids may be administered until surgery is performed. Patients with cardiac rhabdomyomas should be under the care of a cardiologist.

Anecdotal case reports show significant regression of a cardiac rhabdomyoma after receiving everolimus, an mTOR inhibitor.[9]

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Surgical Care

  • Patients with adult rhabdomyoma should have surgical resection of head and neck lesions, especially those lesions that compress or displace the tongue and those that may protrude and partially obstruct the pharynx or larynx.
  • Fetal rhabdomyomas are usually located in the subcutaneous tissues. In most instances, they can be excised from various parts of the body without much difficulty.
  • Local excision is the treatment of choice for genital rhabdomyomas.
  • Open heart surgery may be necessary for the treatment of cardiac rhabdomyomas.
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Consultations

  • Patients with adult rhabdomyomas should be cared for in consultation with ear, nose, and throat (ENT) specialists.
  • Patients with genital rhabdomyoma should be cared for in consultation with gynecologists and urologists.
  • Patients with cardiac rhabdomyomas should be cared for by cardiologists and cardiothoracic surgeons.
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Diet

Patients with adult rhabdomyoma and problems related to swallowing may need to be placed on a liquid diet.

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Activity

Patients with adult rhabdomyoma who are experiencing breathing difficulties should restrict their activities until appropriate treatment can be undertaken. Patients with cardiac rhabdomyoma also must restrict their activities.

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Contributor Information and Disclosures
Author

Karl J D'Silva  MD, Assistant Clinical Professor of Medicine, Department of Hematology/Oncology, Lahey Clinic, Sophia Gordon Cancer Center

Karl J D'Silva is a member of the following medical societies: Massachusetts Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Vijay R Karia, MD  Fellow, Department of Rheumatology, Louisiana State University

Vijay R Karia, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Richard V Worrell, MD  Vice Chairman Emeritus, Professor Emeritus, Department of Orthopedics, University of New Mexico School of Medicine

Richard V Worrell, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American College of Surgeons, American Orthopaedic Association, American Society for Clinical Pathology, American Trauma Society, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Madan L Arora, MD  Assistant Professor of Medicine, Michigan State University College of Human Medicine; Consulting Staff, Division of Hematology/Oncology, Great Lakes Cancer Institute, McLaren Regional Medical Center

Madan L Arora, MD is a member of the following medical societies: Michigan State Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Clarence Sarkodee-Adoo, MD  Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program

Disclosure: Takeda Millenium Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Benjamin Movsas, MD  Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center

Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, and American Society for Therapeutic Radiology and Oncology

Disclosure: Nothing to disclose.

J Stuart Wolf Jr, MD, FACS  The David A Bloom Professor of Urology, Director, Division of Endourology and Stone Disease, Department of Urology, University of Michigan Medical School

J Stuart Wolf Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, Society of University Urologists, and Society of Urologic Oncology

Disclosure: Nothing to disclose.

Chief Editor

Mary C Mancini, MD, PhD  Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association

Disclosure: Nothing to disclose.

References

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  2. Bjorndal Sorensen K, Godballe C, Ostergaard B, Krogdahl A. Adult extracardiac rhabdomyoma: light and immunohistochemical studies of two cases in the parapharyngeal space. Head Neck. Mar 2006;28(3):275-9. [Medline].

  3. Iversen UM. Two cases of benign vaginal rhabdomyoma. Case reports. APMIS. Jul-Aug 1996;104(7-8):575-8. [Medline].

  4. Kelekci S, Yazicioglu HF, Yilmaz B, Aygün M, Omeroglu RE. Cardiac rhabdomyoma with tuberous sclerosis: a case report. J Reprod Med. Jul 2005;50(7):550-2. [Medline].

  5. Jozwiak S, Domanska-Pakiela D, Kwiatkowski DJ, Kotulska K. Multiple cardiac rhabdomyomas as a sole symptom of tuberous sclerosis complex: case report with molecular confirmation. J Child Neurol. Dec 2005;20(12):988-9. [Medline].

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  14. Furihata M, Fujimori T, Imura J, Ono Y, Furihata T, Shimoda M. Malignant stromal tumor, so called "gastrointestinal stromal tumor", with rhabdomyomatous differentiation occurring in the gallbladder. Pathol Res Pract. 2005;201(8-9):609-13. [Medline].

  15. Gunther T, Schreiber C, Noebauer C, Eicken A, Lange R. Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: a 30-year review. Pediatr Cardiol. Nov 2008;29(6):1071-6. [Medline].

  16. Koutsimpelas D, Weber A, Lippert BM, Mann WJ. Multifocal adult rhabdomyoma of the head and neck: a case report and literature review. Auris Nasus Larynx. Jun 2008;35(2):313-7. [Medline].

  17. Lapner PC, Chou S, Jimenez C. Perianal fetal rhabdomyoma: case report. Pediatr Surg Int. Sep 1997;12(7):544-7. [Medline].

  18. Motara F, Cilliers AM, Papeta L, Adams PE, Ntsinjana H, Vanderdonck K, et al. A giant rhabdomyoma in a neonate with tuberous sclerosis. Cardiovasc J Afr. Sep-Oct 2008;19(5 Suppl):S24-5. [Medline].

  19. O'Callaghan FJ, Clarke AC, Joffe H, et al. Tuberous sclerosis complex and Wolff-Parkinson-White syndrome. Arch Dis Child. Feb 1998;78(2):159-62. [Medline].

  20. Tanda F, Rocca PC, Bosincu L. Rhabdomyoma of the tunica vaginalis of the testis: a histologic, immunohistochemical, and ultrastructural study. Mod Pathol. Jun 1997;10(6):608-11. [Medline].

 
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Histopathology of adult rhabdomyoma. Microscopically, the adult rhabdomyoma contains deeply eosinophilic polygonal cells with peripherally placed nuclei. Cross-striations can be observed (X250).
Atrial rhabdomyoma as seen on cardiac CT scan in a patient with tuberous sclerosis.
Nonobstructive ventricular rhabdomyomas in a patient with tuberous sclerosis.
Contrast-enhanced cardiac-gated T1-weighted MRI shows an enhancing left ventricular mass. At autopsy, this mass was found to be a cardiac rhabdomyoma.
 
 
 
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