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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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A<br />

B<br />

Fig. 4.119 Embryonal rhabdomyosarcoma. A Embryonal rhabdomyosarcoma can have a well differentiated pattern with abundant rhabdomyoblasts. B Embryonal<br />

rhabdomyosarcoma may be composed of primitive, hyperchromatic oval cells.<br />

mas {1076}. The dedifferentiation may<br />

occur at the inception or in a recurrent<br />

tumour. This component can give rise to<br />

metastases. Some liposarcomas of the<br />

scrotum can have smooth muscle elements;<br />

these have been designated as<br />

combined lipoleiomyosarcomas {2539}.<br />

Malignant fibrous histiocytoma and<br />

fibrosarcoma are diagnoses by exclusion.<br />

The former is a pleomorphic fibroblasticmyofibroblastic<br />

sarcoma, and the latter<br />

has a more uniform spindle cell pattern.<br />

The majority of paratesticular rhabdomyosarcomas<br />

are of the embryonal<br />

type, but a small percentage (10-15%)<br />

have been classified as the alveolar type<br />

in the largest clinicopathological series<br />

{753,1283,1563,2146}. A typical example<br />

of embryonal rhabdomyosarcoma contains<br />

large number of primitive round to<br />

oval cells and smaller numbers of differentiating<br />

rhabdomyoblasts with eosinophilic<br />

cytoplasm and possible cytoplasmic<br />

cross striations. However, the number<br />

of differentiating rhabdomyoblasts varies<br />

widely. Myxoid matrix is often present. A<br />

rare variant of embryonal rhabdomyosarcoma<br />

is composed of predominantly<br />

spindled cells, with some resemblance to<br />

smooth muscle cells. This type has been<br />

referred to as spindle cell or leiomyosarcoma-like<br />

rhabdomyosarcoma {1483}.<br />

Although cytoplasmic cross striations<br />

may be noted, especially in the spindle<br />

cell rhabdomyosarcoma, they are not<br />

required for the diagnosis. Diagnostic<br />

confirmation should be obtained by<br />

immunohistochemistry. Virtually all RMS<br />

are positive for desmin and muscle actins<br />

(HHF35), and most have nuclear positivity<br />

for myogenic regulatory proteins,<br />

MyoD1 and myogenin (the latter demonstrated<br />

with Myf4 antibody). Cytoplasmic<br />

positivity for MyoD1 occurs in various<br />

tumours and has no diagnostic significance.<br />

Post chemotherapy specimens<br />

can show extensive rhabdomyoblastic<br />

differentiation.<br />

276 Tumours of the testis and paratesticular tissue

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