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Soft tissue tumors

Skeletal muscle

Genital type rhabdomyoma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 21 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Rare benign tumor with skeletal muscle differentiation in vagina, vulva or cervix, usually in middle aged women and rarely urethra
● Extracardiac rhabdomyomas are divided into adult, fetal, and genital (below) histologic types
● Extracardiac tumors are not associated with tuberous sclerosis


● Rarely occurs in males in paratesticular region or epididymis or in the tunica vaginalis of the testis or prostate
● Mean age 42 years

Case reports

● Infant girl with genital rhabdomyoma of the urethra (Hum Pathol 2012;43:597)
● 17 year old boy with locally invasive tumor (J Pediatr Surg 2007;42:E5)
● 19 year old man with testicular tumor (Mod Pathol 1997;10:608)
● 20 year old man with epididymal tumor (Arch Pathol Lab Med 2000;124:1518)
● 48 year old woman with lower abdominal pain and vaginal bleeding (Hum Pathol 2005;36:433)


● Local excision is curative

Gross description

● Well circumscribed, solitary, up to 3 cm
● Resembles polyp
● Covered by smooth mucosa

Micro description

● Submucosal, polypoid, well circumscribed, no capsule
● Haphazard strap-like or round striated muscle fibers in fibrous stroma with dilated vessels
● Cells have abundant eosinophilic cytoplasm with glycogen, cross striations, longitudinal myofibrils
● Nucleus is round, vesicular, central and uniform
● May have bi- or multinucleated cells
● No/rare mitotic figures, no cambium layer, no spider cells, no spindle cells or rhabdomyoblasts, no necrosis, no nuclear pleomorphism

Micro images

Submucosal proliferation of haphazard skeletal muscle cells with prominent cross striations and fibromyxoid stroma


Cervix (various images)

Positive stains

● Muscle specific actin and desmin highlight rod-like structures; myoglobin (100%)

Negative stains

● Keratin, EMA, CD68

Differential diagnosis

Botyroid variant of embryonal rhabdomyosarcoma: usually <25 years old with rapidly growing mass, cambium layer, atypia, mitotic activity
● Vaginal polyp: may have atypical cells, but no cross striations

End of Soft tissue tumors > Skeletal muscle > Genital type rhabdomyoma

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