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. If airway obstruction is diagnosed, surgical intervention should be considered. 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. Patients with advanced cardiac rhabdomyomas should be placed in a cardiac care unit. Anecdotal case reports show significant regression of a cardiac rhabdomyoma after receiving everolimus, an mTOR (mammalian target of rapamycin) inhibitor. [16, 17, 18, 19, 20, 21, 22]
Patients with genital rhabdomyomas may require catheterization if they have symptoms of urinary tract obstruction. Patients with genital rhabdomyomas who become pregnant need to be monitored closely. They may require a cesarean delivery.
Studies have explored the expression of anaplastic lymphoma kinase (ALK) and insulinlike growth factor-1 receptor (IGF-1R) as potential therapeutic targets in rhabdomyosarcoma. In one study, IGF-1R and ALK expression was observed in 72% and 92% of alveolar rhabdomyosarcomas and 61% and 39% of embryonal rhabdomyosarcomas, respectively. [23] Coexpression was observed in 68% of alveolar rhabdomyosarcomas and 32% of embryonal rhabdomyosarcomas. Combined inhibition reveals synergistic cytotoxic effects and continues to be a promising area for future study; further investigation is needed.
Patients with adult rhabdomyoma and problems related to swallowing may need to be placed on a liquid diet.
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.
Surgical Care
Patients with adult rhabdomyoma should be treated with 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. [24]
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.
Postoperative Care
Patients who have had surgical resection of their rhabdomyomas should have routine postoperative care (eg, dressing changes and suture removal when indicated).
Acetaminophen and codeine or oxycodone and acetaminophen may be used to control postoperative pain. Oxycodone with acetaminophen can produce drug dependence of the morphine type. Accordingly, the potential for drug abuse exists.
Complications
Any surgical procedure can be complicated by infection.
Patients with cardiac rhabdomyoma may develop congestive heart failure or arrhythmia.
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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Histopathology of adult rhabdomyoma. Microscopically, the adult rhabdomyoma contains deeply eosinophilic polygonal cells with peripherally placed nuclei. Cross-striations can be observed (X250).
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Atrial rhabdomyoma as seen on cardiac CT scan in a patient with tuberous sclerosis.
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Nonobstructive ventricular rhabdomyomas in a patient with tuberous sclerosis.
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Contrast-enhanced cardiac-gated T1-weighted MRI shows an enhancing left ventricular mass. At autopsy, this mass was found to be a cardiac rhabdomyoma.
