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Primary tumors of the paratesticular region are rare, with
sarcomas constituting a major proportion, particularly in the old age. The
paratesticular region consists of mesothelial, various epithelial and
mesenchymal cells and may therefore give rise to a variety of tumors.
Epithelioid sarcoma usually occurs at the distal extremities of young adults.
The proximal-type variant is characterized by a more aggressive course and
resistance to treatment. We herein report a case of 32 years male with scrotal
swelling diagnosed as Proximal-type epithelioid sarcoma of paratesticular
region after histopathological examination and immunohistochemistry. To the
best of our knowledge, this is only the second case of proximal epithelioid
sarcoma of paratesticular region in the English literature. As these tumors are
rare, it has been difficult to establish their optimal treatment. Also, these
can be easily misdiagnosed as other epithelioid lesions.
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