Three Signs Suggest Rare Renal Tumor in Young Adults
Case studies and literature review shed light on differential diagnosis of aggressive tumor
THURSDAY, Aug. 24 (HealthDay News) -- Physicians should consider renal peripheral primitive neuroectodermal tumor (PNET) in young adults who present with the classic triad of renal cancer, hematuria, and pain and palpable tumor, according to three case studies and a corresponding literature review in the August issue of Urology.
Jörg Ellinger, M.D., of Universitätsklinikum Bonn in Bonn, Germany, and colleagues, conducted two case studies of renal and one case study of bladder PNET along with a literature review for PNET in which 52 renal PNET patients were identified and analyzed.
Renal PNET is diagnosed in young adults with a median age of 24. They found that 84 percent of renal PNET patients present with pain, 60 percent present with palpable tumor and 38 percent have hematuria. Computed tomography and magnetic resonance imaging findings of PNET are useful only for local assessment of resectability and to determine if the cancer has spread, therefore diagnosis is based on histologic and immunohistochemistry findings. Palpable tumor masses and synaptophysin expression are markers of shorter cancer specific-survival. By contrast, bladder PNET is associated with previous immunosuppression.
"Once PNET is diagnosed, multimodal treatment must be initiated," the study authors concluded. "Despite this, the prognosis is poor if distant metastases are present."