• Named for distinctive gross features (resembles a bunch of grapes)
• Also called sarcoma botryoides

• 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

• 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)

• Resembles cluster of grapes or allergic nasal polyp
• Fleshy nodular polypoid projections of variable size into lumen

Bladder tumor

Clusters of edematous, grape-like masses that protrude into lumen of hollow organs

• 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

Figures 1 and 2

Figure 3

Figure 4

Figures 5 and 6

Figs 1&2: Polypoid or lobulated masses of cells covered by mucosa, with underlying hypercellular zone of poorly differentiated cells (cambium layer)
Fig 3: Tumor may have only focal cambium layer, and consist primarily of paucicellular edematous tissue with scattered undifferentiated or atypical large cells
Fig 4: Tumor with sheets of round or spindle cells resembling benign polyp or fibroinflammatory lesion
Figs 5&6: Deep foci of hypercellularity is common with round or spindled undifferentiated cells mixed with differentiating rhabdomyoblasts

Vaginal tumor

Condensation of tumor cells in subepithelial zone

• Desmin, MyoD1, smooth muscle actin, muscle specific actin (Pediatr Dev Pathol 2005;8:427)

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