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A) Demonstrates conglomerate of para-aortic retroperitoneal lymphadenopathy at diagnosis of primitive neuroectodermal tumor. Patient went on to receive cheomtherapy with VAC/IE. B) Demonstrates parital response to chemotherapy prior to potentially curative surgical resection.
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Imaging studies showed widespread recurrent disease, including supraclavicular lymphadenopathy, a new right inguinal soft tissue mass and an enlarging conglomerate of retroperitoneal lymphadenopathy in the para-aortic region (Figure 1A). At completion of his neoadjuvant chemotherapy, his imaging studies demonstrated complete response in his neck and mediastinal lymphadenopathy and partial response of his retroperitoneal lymphadenopathy (Figure 1B) and right inguinal mass.
Primitive neuroectodermal tumor (Case 1) consisting of a large confluent aggregate of immature neuroepithelium that forms tubules lined by stratified cells (inset picture) was identified in the orchiectomy sample.
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The RPLND, as well as the supraclavicular lymph node biopsy, confirmed PNET in both specimens (Figure 2).
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