eMedicine Specialties > Pediatrics: Surgery > General Surgery
Rhabdomyomas: Differential Diagnoses & Workup
Updated: Dec 29, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Other Problems to Be Considered
Hibernoma
Reticulohistiocytoma
Tuberous sclerosis
Workup
Laboratory Studies
- Order the usual laboratory studies, including CBC, hemoglobin/hematocrit, platelet count, and urinalysis.
Imaging Studies
- Perform routine radiographic studies, including radiographs of the chest and affected areas of the body.
- An MRI of the affected area might be useful.
- Occasionally, a CT scan, particularly of the chest in cases of cardiac rhabdomyoma, might be of value.
Atrial rhabdomyoma as seen on cardiac CT scan in a patient with tuberous sclerosis.
Other Tests
- When rhabdomyoma is suspected, a biopsy of the lesion is indicated. Needle biopsies can reveal sufficient information to make a histopathologic diagnosis.
Procedures
- Any masses, such as those found in the head and neck of patients with adult rhabdomyoma, should have biopsies performed to establish a diagnosis.
- Make a small incision directly over the mass, and remove a small amount of tumor tissue. Close the wound in the usual manner.
- A needle biopsy can be performed using a Tru-Cut needle. A small stab wound is made directly over the mass. The needle is introduced into the tumor, then it is withdrawn with a small amount of tumor tissue attached. Subsequently, a dressing is applied to the wound.
Histologic Findings
The histopathologic findings from patients with adult rhabdomyoma are characterized by the presence of well-differentiated large cells, which resemble striated muscle cells. The cells are deeply eosinophilic polygonal cells with small, peripherally placed nuclei and occasional intracellular vacuoles.
Fetal rhabdomyoma is identifiable by the presence of a mixture of spindle-shaped cells with indistinct cytoplasm and muscle fibers, which resemble striated muscle tissue seen in intrauterine development at 7-12 weeks.
Genital rhabdomyoma is made up of a mixture of fibroblastlike cells with clusters of mature cells containing distinct cross-striations and a matrix containing varying amounts of collagen and mucoid material.
Cardiac rhabdomyoma consists of cells that closely resemble embryonic cardiac muscle cells.
The histopathology of rhabdomyomatous mesenchymal hamartoma of the skin reveals that the lesions contain poorly oriented or perpendicular bundles of well-differentiated skeletal muscle with islands of fat, fibrous tissue, and occasionally proliferating nerves.
Histopathology of adult rhabdomyoma. Microscopically, the adult rhabdomyoma contains deeply eosinophilic polygonal cells with peripherally placed nuclei. Cross-striations can be observed (X250).
Staging
- Benign tumors can be staged as follows:
- Stage 1, latent - Remains static or heals spontaneously
- Stage 2, active - Progressive growth but limited by natural barriers
- Stage 3, aggressive - Progressive growth not limited by natural barriers
- Grading, where G = grade; T = site; M = metastasis
- G0 = benign
- T0 = intracapsular
- T1 = extracapsular, intracompartmental
- M0 = none
- Staging
- Benign stage 1, latent = G0, T0, M0
- Benign stage 2, active = G0, T0, M0
- Benign stage 3, aggressive = G0, T1, M0
More on Rhabdomyomas |
| Overview: Rhabdomyomas |
 Differential Diagnoses & Workup: Rhabdomyomas |
| Treatment & Medication: Rhabdomyomas |
| Follow-up: Rhabdomyomas |
| Multimedia: Rhabdomyomas |
| References |
| « Previous Page | Next Page » |
References
Hansen T, Katenkamp D. Rhabdomyoma of the head and neck: morphology and differential diagnosis. Virchows Arch. Nov 2005;447(5):849-54. [Medline].
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].
Iversen UM. Two cases of benign vaginal rhabdomyoma. Case reports. APMIS. Jul-Aug 1996;104(7-8):575-8. [Medline].
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].
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].
Ashfaq R, Timmons CF. Rhabdomyomatous mesenchymal hamartoma of skin. Pediatr Pathol. Sep-Oct 1992;12(5):731-5. [Medline].
Delides A, Petrides N, Banis K. Multifocal adult rhabdomyoma of the head and neck: a case report and literature review. Eur Arch Otorhinolaryngol. Jun 2005;262(6):504-6. [Medline].
Webb DW, Thomas RD, Osborne JP. Cardiac rhabdomyomas and their association with tuberous sclerosis. Arch Dis Child. 1993;68(3):367-70. [Medline].
Bastian BC, Brocker EB. Adult rhabdomyoma of the lip. Am J Dermatopathol. Feb 1998;20(1):61-4. [Medline].
Bosi G, Lintermans JP, Pellegrino PA, et al. The natural history of cardiac rhabdomyoma with and without tuberous sclerosis. Acta Paediatr. Aug 1996;85(8):928-31. [Medline].
Burke A, Virmani R. Pediatric heart tumors. Cardiovasc Pathol. Jul-Aug 2008;17(4):193-8. [Medline].
Campanacci M. Bone and Soft Tissue Tumors. New York, NY: Springer-Verlag; 1986.
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].
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].
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].
Lapner PC, Chou S, Jimenez C. Perianal fetal rhabdomyoma: case report. Pediatr Surg Int. Sep 1997;12(7):544-7. [Medline].
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].
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].
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].
Further Reading
Keywords
rhabdomyoma, benign tumor of striated muscle, neoplastic, hamartoma, cardiac rhabdomyoma, rhabdomyomatous mesenchymal hamartomas of the skin, muscle tumor
