Bladder - Urachal sarcoma

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Bladder

Other tumors

Urachal sarcoma

Reviewers: Gillian Levy, M.D., Yale Medical Center (see Reviewers page)
Last Revised: 29 April 2011, last major update April 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Criteria for diagnosis: sarcoma that occurs at a site consistent with anatomical position of urachus; tumor must demonstrate relationship with urachus

Epidemiology
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● Usually < age 20 years

Sites
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● At dome of bladder or superior/anterior wall of bladder

Etiology
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● Urachal ligament is embryologic remnant connecting the dome of the bladder to the umbilicus; urachal remnant may persist and contain tubular or cystic structures (see patent urachus)

Clinical features
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● Mesenchymal lesions are rare in urachus
● Leiomyosarcomas or rhabdomyosarcomas are most common urachal sarcomas; represent 8% of urachal malignancies
● Sarcomas present with hematuria and cystic or solid structure in the bladder dome
● Propensity for distant metastasis is low, but can have local recurrence

Case reports
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● 18 month Japanese girl with huge epithelioid leiomyosarcoma arising from urachus or bladder dome (Mod Pathol 2000;13:1156)
● 6 year old girl with urachal embryonal rhabdomyosarcoma (Pediatr Hematol Oncol 2007;24:543)
● 12 year old boy with spontaneous rupture of urachal leiomyosarcoma (Int J Urol 2007;14:960)
● 66 year old man with primary urachal MFH (Arch Pathol Lab Med 2004;128:456)

Treatment
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● Partial cystectomy and en bloc resection of the urachal ligament with the umbilicus
● No standard chemotherapy/radiation regimen

Micro description (Histopathology)
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● Variable, depends on the subtype of sarcoma

Micro images
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Urachal MFH – various images

Epithelioid leiomyosarcoma

Positive stains
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● Leiomyosarcoma: desmin, vimentin, smooth muscle actin, calponin
● MFH: desmin; other stains are variable
● Rhabdomyosarcoma: myogenin

Negative stains
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● Leiomyosarcoma: S100, CD34

Electron microscopy images
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Epithelioid leiomyosarcoma

Differential diagnosis
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● Sarcomas of non-urachal origin: predominantly ages 40-59

End of Bladder > Other tumors > Urachal sarcoma

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