INITIAL PRESENTATION AND MANAGEMENT
SYMPTOMS AND SIGNS
The pathognomonic presentation of a primary testicular tumor is a painless testicular mass that may range in size from a few millimeters to several centimeters. However, the painless testicular mass occurs in only a minority of patients. The majority present with more diffuse testicular pain, swelling, hardness, or some combination of these findings. Because infectious epididymitis or orchitis, or a combination of the two, is more common, a trial of antibiotic therapy is often reported in questionable cases. Acute testicular pain, simulating testicular torsion, occurs less frequently and may represent intratumoral hemorrhage. If the testicular discomfort does not abate or findings do not revert to normal within 2 to 4 weeks, a testicular ultrasound is indicated. On ultrasound, the typical testicular tumor is intratesticular, and may produce one or more discrete hypoechoic masses or diffuse abnormalities with microcalcifications. The latter is more frequently found in seminoma. Delay in diagnosis, caused by either patient- or physician-related factors, or both, generally results in higher stage at presentation and presumably lower survival. These data on delay in diagnosis require that this highly curable neoplasm be properly managed at all stages.
A radical inguinal orchiectomy, using an inguinal incision with early high ligation of the spermatic cord at the deep inguinal ring, minimizes local tumor recurrence and aberrant lymphatic spread and is the only acceptable therapeutic and diagnostic procedure. The vasal and vascular components are doubly clamped and divided separately; their respective stumps are pushed into the retroperitoneal space to facilitate future removal of the gonadal vessels at the time of retroperitoneal lymph node dissection (RPLND). The testicle and spermatic cord are removed en bloc, avoiding any spillage, and meticulous hemostasis is achieved. The testis embryologically originates in the genital ridge, and descends during fetal life through the abdomen and inguinal canal into the scrotum. Therefore, the primary lymphatic and vascular drainage of the testis is to the retroperitoneal lymph nodes and the renal or great vessels, respectively. A transscrotal orchiectomy is contraindicated, because it permits the development of alternate lymphatic drainage pathways to the inguinal and pelvic lymph nodes, and leaves intact the spermatic cord from the external to the internal ring. In the rare situation when the diagnosis of a testicular tumor is in question, then an inguinal incision is required for an open biopsy. The testis can then be examined in situ in a sterile field and an appropriate biopsy taken with minimal risk of scrotal or inguinal contamination. Regardless of the preoperative diagnosis, all potential, primary testicular malignancies should be managed through an inguinal approach.
Extragonadal GCTs comprise fewer than 10% of all GCT presentations. The mediastinum and retroperitoneum are the most common primary sites. Pineal tumors, occurring most frequently in children, are usually GCT. Because of their unique access to the meninges, the metastatic pattern of pineal germ GCT includes intradural sites along the neuraxis and is infrequently systemic. The management of pineal GCT is discussed elsewhere in this text. In extremely rare circumstances, primary GCTs have been found in unusual sites such as the sacrum, thyroid, paranasal sinuses, and soft tissues of the head and neck. In patients with extragonadal presentations of GCT, a testicular ultrasound is required. The management of extragonadal and testicular GCT is the same, and primary site is an independent factor in current staging and risk classifications.