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Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other
Genital type rhabdomyoma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 22 July 2009, last major update July 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Rare benign tumor with skeletal muscle differentiation in vagina, vulva or cervix, usually in middle aged women
● Extracardiac rhabdomyomas are divided into adult, fetal, and genital (below) histologic types
● Extracardiac tumors are not associated with tuberous sclerosis
Epidemiology
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● Rarely occurs in males in paratesticular region or epididymis
● Mean age 42 years
Case reports
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● 17 year old boy with locally invasive tumor (J Pediatr Surg 2007;42:E5)
● 19 year old man with testicular tumor (Mod Path 1997;10:608)
● 20 year old man with epididymal tumor (Archives 2000;124:1518)
● Ovarian serous cystadenoma with mural nodules of genital rhabdomyoma (Hum Pathol 2005;36:433)
Treatment and prognosis
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● Local excision is curative
Gross description
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● Well circumscribed, solitary, up to 3 cm
● Resembles polyp
● Covered by smooth mucosa
Micro description
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● 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
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Submucosal proliferation of haphazard skeletal muscle cells with prominent cross striations and fibromyxoid stroma
Epididymis Cervix (various images)
Positive stains
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● Muscle specific actin and desmin highlight rod-like structures; myoglobin (100%)
Negative stains
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● Keratin, EMA, CD68
Differential Diagnoses
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● Vaginal polyp - may have atypical cells, but no cross striations
● Botyroid variant of embryonal rhabdomyosarcoma - usually <25 years old with rapidly growing mass, cambium layer, atypia, mitotic activity
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Genital type rhabdomyoma
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