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Germ Cell Tumours
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=== <span style="color:#ff0000">Primary extra-gonadal GCTs</span> === * '''<span style="color:#ff0000">Site of origin of GCTs: gonadal (95%) vs. extra-gonadal (5%)</span>''' * '''<span style="color:#ff0000">Develop in midline anatomic locations</span>''' ** '''<span style="color:#ff0000">Most common sites (descending order) of origin: mediastinum, retroperitoneum,</span>''' sacrococcygeal region, and pineal gland, although many unusual sites have also been reported [SASP 2014] ** '''Primary mediastinal GCT''' *** '''NSGCT''' **** '''Compared to NSGCTs originating in the testicle or retroperitoneum, primary mediastinal NSGCTs are (4):''' ****# '''More likely to have yolk sac tumour components and be associated with elevated AFP''' ****# '''Associated with Klinefelter syndrome''' ****# '''Less sensitive to chemotherapy''' ****# '''Associated with a poor prognosis''' *** '''Seminoma''' **** '''Primary mediastinal seminomas have similar prognosis to testicular seminomas''' ** '''Primary retroperitoneal GCT''' *** '''Behave similarly to testicular GCTs and carry the same prognosis.''' * Of patients with metastatic GCT without a testis mass: ** 1/3 definitively have a primary extra-gonadal GCT ** 1/3 have ITGCN in the testis ** 1/3 have sonographic evidence of a “burned-out” primary tumor * '''Diagnosis and evaluation''' ** '''If midline mass in male age < 40, consider GCT''' *** Midline mass in male age < 40 with elevated AFP and/or hCG is diagnostic of GCT, even if testicular evaluation is normal; histologic confirmation by biopsy is unnecessary before starting treatment *** Midline mass in male age < 40 with normal serum tumor markers, perform biopsy of the midline mass to confirm the diagnosis before beginning treatment * '''Management''' ** '''Inguinal orchiectomy''' *** '''Indications in patients with suspected extra-gonadal GCT (2):''' **# '''Pattern of metastasis is consistent with a right-sided or left-sided testicular primary tumor''' **# '''Sonographic evidence of a “burned-out” primary tumor'''
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