Rhabdomyoma

Cardiac rhabdomyoma

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Photomicrograph of fetal-type rhabdomyoma: Note the plump, pink benign skeletal muscle cells.

A rhabdomyoma is a benign tumor that originates from striated muscle. These tumors can be categorized based on their location: either "cardiac" (within the heart) or "extracardiac" (outside the heart). Rhabdomyomas play a significant role in pediatric and adult oncology due to their associations with various conditions and potential complications.

Classification

Rhabdomyomas are classified based on their occurrence location. The main categories include:

• Cardiac rhabdomyomas: These are the most prevalent primary tumors of the heart in infants and children.
• Extracardiac rhabdomyomas: These manifest outside the heart and can be further subdivided into:
  • Adult type
  • Fetal type
  • Genital type

Associations and Manifestations

Cardiac rhabdomyomas have a known link with tuberous sclerosis, a genetic disorder that affects multiple systems. In individuals diagnosed with tuberous sclerosis, rhabdomyomas might either regress and vanish entirely or maintain a consistent size without significant growth.

While rhabdomyomas are chiefly associated with the heart and tongue, they can also develop in other parts of the body, such as the vagina.

Malignant Potential

Malignant tumors arising from skeletal muscle are termed rhabdomyosarcoma. It is crucial to differentiate between benign rhabdomyomas and malignant rhabdomyosarcomas. Only in rare instances has malignant transformation been observed in fetal rhabdomyomas. When diagnosing rhabdomyomas in the tongue, another differential diagnosis to consider is the ectomesenchymal chondromyxoid tumor.

Conclusion

Rhabdomyomas are benign muscle tumors that can have significant clinical implications, especially in pediatric populations. Proper diagnosis and differentiation from malignant counterparts are vital for appropriate management and prognosis.

External links

Classification	ICD-10: D21 (ILDS D21.M50) ICD-9-CM: 215 MeSH: D012207
External resources	eMedicine: med/2021

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