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Germ Cell Tumours
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== Special scenarios == === <span style="color:#ff0000">Brain metastases</span> === * '''<span style="color:#ff0000">Associated with choriocarcinoma; should be suspected in any patient with a very high serum hCG level</span>''' ** '''Choriocarcinomas are highly vascular and tend to hemorrhage during chemotherapy, with death rates of 4-10% secondary to intracranial hemorrhage''' * Management ** Brain metastases at diagnosis: BEP×4 chemotherapy followed by resection of residual masses. ** Relapse in the brain after first-line chemotherapy: second-line chemotherapy followed by resection and/or radiation therapy *** Relapse in the brain after achieving a complete response to chemotherapy have a worse prognosis than patients with brain involvement at diagnosis. === <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''' === Small (<10 mm), impalpable intratesticular lesions === *'''Small (<10 mm), impalpable intratesticular lesions in the absence of disseminated GCT or elevated serum tumor markers are a diagnostic dilemma''' * '''<span style="color:#ff0000">Most of these lesions are benign (testicular cysts, small infarcts, or small Leydig cell or Sertoli cell tumors), however, 20-50% may represent small GCTs (usually seminomas).</span>''' ** '''Risk of malignancy increases with the size of the lesion.''' * '''Management options (3):''' *# '''Inguinal orchiectomy''' *# '''Testis-sparing surgery involving inguinal exploration and excision (with frozen-section analysis to rule out GCT)''' *#* '''Intraoperative ultrasonography is useful during surgical exploration of the testis to locate the lesion.''' *# '''Close observation with serial ultrasound scans (with exploration of growing lesions).'''
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