Rhabdomyoma epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]
Overview
Rhabdomyoma is a benign tumor of striated muscle. Rhabdomyomas are rare and can be classified into cardiac type and extracardiac type.The most common primary benign pediatric tumor of the heart is cardiac rhabdomyoma which can be seen mainly in fetal life and children, second most common primary benign cardiac tumor in children is fibroma. Most tumors regress spontaneously, prognosis depends on the location of tumor and size. Cardiac rhabdomyoma is strongly associated with tuberous sclerosis. Cardiac rhabdomyoma is seen almost always in the pediatric age group and is associated with tuberous sclerosis, neurofibromatosis, and sebaceous adenomas. Extracardiac rhabdomyoma can be divided into three groups (adult, fetal, and genital types) with distinct clinical and morphological differences. The adult type is a slowly growing mass which typically involves the head and neck. Fetal type is seen in the head and neck region. The genital type is almost always found in the vulvovaginal region of older women. Treatment of adult type rhabdomyoma of head and neck depends upon the severity of clinical symptoms. Conservative management or surgery may be necessary depending upon the type and tumor stage.
Epidemiology and Demographics
- Cardiac rhabdomyomas are usually detected during the first year of life or before birth and accounts for majority of all primary cardiac tumors.[1][2][3]
- Worldwide, rhabdomyoma is rare.
- Most of patients with tuberous sclerosis develop a cardiac rhabdomyoma. Similarly, children diagnosed with cardiac rhabdomyomas demonstrate radiologic or clinical findings of tuberous sclerosis or have a positive family history. Rhabdomyoma is extremely rare in the United States. Rhabdomyoma has a relative incidence of 5.8%.
Age
- Adult rhabdomyoma is more commonly observed among patients aged greater than 40 years old.[4]
- Fetal rhabdomyoma is more commonly observed among patients aged between birth and 3 years.
- Cardiac rhabdomyoma is more commonly observed among patients in the pediatric age group.
- Genital rhabdomyoma is more commonly observed among patients in the young and middle-aged women.
- Rhabdomyomatous mesenchymal hamartomas of the skin is more commonly observed among newborns and infants.[5]
Gender
- Cardiac rhabdomyoma affects men and women equally.[6][7]
- Rhabdomyomatous mesenchymal hamartoma of skin is extremely rare in both genders.
- Males are more commonly affected with adult rhabdomyoma than females.
- Males are more commonly affected with fetal rhabdomyoma than females.
- Females are more commonly affected with genital rhabdomyoma than males.
Race
- There is no racial predilection for rhabdomyomas.
References
- ↑ Isaacs H (2004). "Fetal and neonatal cardiac tumors". Pediatr Cardiol. 25 (3): 252–73. doi:10.1007/s00246-003-0590-4. PMID 15360117.
- ↑ Delides A, Petrides N, Banis K (2005). "Multifocal adult rhabdomyoma of the head and neck: a case report and literature review". Eur Arch Otorhinolaryngol. 262 (6): 504–6. doi:10.1007/s00405-004-0840-y. PMID 15942804.
- ↑ Ramadani N, Kreshnike KD, Muçaj S, Kabashi S, Hoxhaj A, Jerliu N, Bejiçi R (April 2016). "MRI Verification of a Case of Huge Infantile Rhabdomyoma". Acta Inform Med. 24 (2): 146–8. doi:10.5455/aim.2016.24.146-148. PMC 4851540. PMID 27147810.
- ↑ Bejiqi R, Retkoceri R, Bejiqi H (April 2017). "Prenatally Diagnosis and Outcome of Fetuses with Cardiac Rhabdomyoma - Single Centre Experience". Open Access Maced J Med Sci. 5 (2): 193–196. doi:10.3889/oamjms.2017.040. PMC 5420773. PMID 28507627.
- ↑ McKinnon EL, Rand AJ, Selim MA, Fuchs HE, Buckley AF, Cummings TJ (October 2015). "Rhabdomyomatous mesenchymal hamartoma presenting as a sacral skin tag in two neonates with spinal dysraphism". J. Cutan. Pathol. 42 (10): 774–8. doi:10.1111/cup.12538. PMID 25989364.
- ↑ de Trey LA, Schmid S, Huber GF (2013). "Multifocal adult rhabdomyoma of the head and neck manifestation in 7 locations and review of the literature". Case Rep Otolaryngol. 2013: 758416. doi:10.1155/2013/758416. PMC 3697226. PMID 23841004.
- ↑ Takeyama J, Hayashi T, Sanada T, Shimanuki Y, Saito M, Shirane R (April 2005). "Rhabdomyomatous mesenchymal hamartoma associated with nasofrontal meningocele and dermoid cyst". J. Cutan. Pathol. 32 (4): 310–3. doi:10.1111/j.0303-6987.2005.00312.x. PMID 15769282.