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Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other
Botryoid variant of embryonal rhabdomyosarcoma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 17 July 2009, last major update July 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Terminology
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● Named for distinctive gross features (resembles a bunch of grapes)
● Also called sarcoma botryoides
Clinical
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● 25% of rhabdomyosarcomas, 10% of embryonal subtype
● Tumors are beneath mucosal membrane in walls of hollow structures (bladder, vagina, nasal cavity), extrahepatic bile ducts or near a space; rarely in eyelid or anal region
Treatment and prognosis
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● Very good prognosis (Zhonghua Bing Li Xue Za Zhi 2004;33:225, Am J Surg Pathol 2001;25:856), although may have late relapse (Pediatr Blood Cancer 2008;51:140)
● Treatment is conservative surgery plus radiation and chemotherapy (Int J Gynecol Cancer 2008;18:190)
Gross description
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● Resembles cluster of grapes or allergic nasal polyp
● Fleshy nodular polypoid projections of variable size into lumen
Gross images
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Clusters of edematous, grape-like masses Bladder tumors
that protrude into lumen of hollow organs
Tumor of bile duct
Micro description
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● Hypercellular zone immediately beneath epithelium (Nicholson’s cambium layer - resembles hypercellular zones that produce growth rings in trees)
● Cells are undifferentiated, round or spindled with minimal cytoplasm, frequent mitotic figures
● Less cellular in deeper layers, composed of differentiating and undifferentiated cells resembling embryonal NOS
Micro images
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Condensation of tumor cells Polypoid mass protruding Cambium layer and underlying
in subepithelial zone beneath flattened epithelium nevoid cells
AFIP images
Polypoid or lobulated masses of cells covered by mucosa, with
underlying hypercellular zone of poorly differentiated cells (cambium layer)
Tumor may have only focal cambium layer, and consist primarily
of paucicellular edematous tissue with scattered undifferentiated or atypical large cells
Tumor with sheets of round or spindle cells
resembling benign polyp or fibroinflammatory lesion
Deep foci of hypercellularity is common with round or spindled undifferentiated cells mixed with differentiating rhabdomyoblasts
Other images: vaginal tumor
Positive stains
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● Desmin, MyoD1, smooth muscle actin, muscle specific actin (Pediatr Dev Pathol 2005;8:427)
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Botryoid variant of embryonal rhabdomyosarcoma
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