PERSPECTIVE, PATTERNS OF SPREAD, AND PATHOLOGY
The staging criteria are based on conceiving of the testes as an abdominal organ.
PERSPECTIVE AND PATTERNS OF SPREAD
An enlarged or swollen testes is common in young men. It is usually due to a benign event such as an epididymitis or a swelling indicative of some trauma. Despite the infrequent occurrence of testicular tumors, which only account for 1% of all cancers in males, it is the most common tumor in young men between 20 and 40 years of age. However, the benign appearance of hydroceles and varicoceles may disguise a testicular tumor. Thus, increased size of the testes, whether it is sudden or slow in onset, painless or tender, requires careful investigation and consideration before a course of treatment can be decided upon.
The remarkable aspect of all testicular tumors is their high curability. Seminomas traditionally treated with radical orchidectomy followed by para-aortic radiation have yielded cure rates of ≥95%. One of the first malignancies controlled by multiagent chemotherapy were the nonseminomatous cancers (NSCs) of the testes. Stages I and II NSC have 95% long-term survivals; even disseminated metastatic disease can be eradicated the majority of times (50% to 60%). The best example of success is Lance Armstrong, the record-setting cycling champion and seven-time winner of the Tour de France.
Seminomas, embryonal tumors, and teratocarcinomas tend to be confined to the testes. Direct extension through the capsule of the testis into the fascia and muscle of the scrotal wall is an uncommon pattern of spread (Fig. 41.2; Table 41.2). Occasionally, the epididymis is invaded early. Involvement of the rete testis without evidence of further extension is considered an early lesion in behavior if the involvement is contained within the epididymis.
PATHOLOGY
Testicular tumors are divided into seminomas, embryonal cancers, teratocarcinomas or teratomas, and choriocarcinomas (Table 41.1; Fig. 41.1A,B). There are two hypotheses for the generation of this large variety of tumors. The most popular thesis is that a single germ cell gives rise to seminomas and to the multipotential cells that form the other tumor types; the second thesis is that totipotent cells exist that can give rise to the nonseminomatous tumors only (Fig. 41.1C).
Figure 41.1 | A. Seminoma. Groups of tumor cells are surrounded by fibrous septa infiltrated with lymphocytes. Tumor cells have vesicular nuclei that are much larger than the small, round nuclei of the lymphocytes. B. Embryonal. Embryonal carcinoma component of a nonseminoma germ cell tumor. Because these undifferentiated cells have scant cytoplasm, their hyperchromatic nuclei impart a bluish color to the tumor. The nuclei appear crowded and seem to overlap each other. The cells form cords and sheets surrounding dilated vascular channels filled with red blood cells. C. Tumors of the testis, epididymis, and related structures.
Figure 41.2 | Coronal. Patterns of spread (cancer crab) of testes cancer are color coded for stage: T0, yellow; T1, green; T2, blue; T3, purple. The concept of visualizing patterns of spread to appreciate the surrounding anatomy is well demonstrated by the six-directional pattern (SIMLAP, Table 41.2).
TNM STAGING CRITERIA
TNM STAGING CRITERIA
The staging criteria are based on conceiving of the testes as an abdominal organ. The tunica albuginea is the equivalent of the first wrap of the serosa and, once penetrated by cancer, is T1. If the tunica vaginalis is penetrated, it is equivalent to the peritoneum and is T2. The tunica vaginalis has a visceral and a parietal layer and both need to be penetrated for the cancer to invade the scrotal wall (T4; Fig. 41.3).
The major route for local extension is through the lymphatic channels of the testes that emerge from the mediastinum of the testis and continue along the spermatic cord. The major lymphatic drainage of the testes is different on the left and right. The lymphatics of the testes follow the spermatic vessels and are very commonly the major route of spread of testicular tumors.
Cancer from the germ cells of the testis usually develops during the years of greatest sexual activity. The undescended testis has a greater tendency to undergo carcinomatous change, even after an orchiopexy has been performed. The associated hormonal secretions and the amount of the hormone may produce endocrine effects, including gynecomastia and altered laboratory determinations (human chorionic gonadotropin). In addition, because of their embryonal origin, testicular tumors may produce the tumor-associated antigen alpha fetoprotein. A third serum marker is lactic acid dehydrogenase. Collectively, these three serum biomarkers are incorporated into the staging system as S1, S2, or S3, depending on their level of elevation. Particularly noteworthy is this is the first and only staging system that incorporates serum bio-markers into its progression.
SUMMARY OF CHANGES SEVENTH EDITION AJCC
The definition of TNM and the Stage Grouping for this chapter have not changed from the Sixth Edition (Fig. 41.3). T stage determines stage group (Table 41.3).
GENESIS AND EVOLUTION
Testes cancers are indicative of the robustness and the impact that molecular markers can have in determining stage groups. Including them in the staging system has altered the 4 stages into 13 stage subgroupings. Stage IS is a unique stage with no visible tumor in the TNM compartments. Detection of a serum marker category S1, S2, or S3 depends on titer levels.
TESTES
Figure 41.3 | TNM testes cancer diagram. Testes cancers are unique in having serum markers, which have a greater impact on stage groupings and substages than anatomic extent. Vertically arranged with T definitions on the left and stage groupings on the right. Color bars are coded for stage: stage 0, yellow; I, green; II, blue; III, purple; IV, red; and metastatic, black.
T-ONCOANATOMY
ORIENTATION OF THREE-PLANAR ONCOANATOMY
The isocenter is taken at the center of the scrotum inferior to the bony pelvis (Fig. 41.4).
The testes are composed of convoluted seminiferous tubules with a stroma containing functional endocrine interstitial cells.
T-oncoanatomy
The T-oncoanatomy is displayed in three planar views in Fig. 41.5:
• Coronal: Both are encased in a dense barrier capsule, the tunica albuginea, with fibrous septa extending into and separating the testes into lobules.
• Transverse: The testis is surrounded by a remnant of the peritoneum, the tunica vaginalis, which explains the occurrence of hydroceles, which account for approximately 10% of testicular tumors.
• Sagittal: The tubules converge and exit at the mediastinum of the testis into the rete testis and efferent ducts, which join a single tubule. This tubule, the epididymis, is coiled outside the upper and lower poles of the testicle, then joins the muscular vas deferens conduit that accompanies the vessels and lymphatic channels of the spermatic cord.
Figure 41.4 | Orientation of T-oncoanatomy. The anatomic isocenter for three-planar anatomy of testes is below the pelvis. A. Coronal. B. Sagittal.
Figure 41.5 | T-oncoanatomy. Connecting the dots. Structures are color coded for cancer stage progression. The color code for the anatomic sites correlates with the color code for the stage group (Fig. 41.3) and the patterns of spread (Fig. 41.2) and SIMLAP table (Table 41.2). Connecting the dots in similar colors will provide an appreciation for the three-dimensional oncoanatomy.
N-ONCOANATOMY AND M-ONCOANATOMY
N-ONCOANATOMY
The spermatic lymphatic collecting ducts on the right side tend to follow the vascular components of the cord and drain into the paracaval lymph nodes in the area where the spermatic vein enters the inferior vena cava and the artery arises from the aorta. The spermatic lymphatic collecting ducts on the left side also tend to follow the vascular components of the cord and drain into the para-aortic nodes in the region where the spermatic and the inferior mesenteric arteries arise out of the aorta and also into the nodes of the left renal hilum in the region where the left spermatic vein joins the left renal vein. Juxtaregional or second station nodes are those of the pelvis and mediastinal and supraclavicular regions (Fig. 41.6A; Table 41.4).
The left and right testicles demonstrate different patterns of primary drainage that mirror the differences in venous drainage. The left testicle primarily drains to the para-aortic lymph nodes and the right testicle primarily drains to the interaortocaval lymph nodes. The intrapelvic, external iliac, and inguinal nodes are considered regional only after scrotal or inguinal surgery prior to the presentation of the testis tumor. All nodes outside the regional nodes are distant. Nodes along the spermatic vein are considered regional. A varicoel can result from the left renal vein being compressed by metastatic renal hilar and para aortic lymph nodes.
M-ONCOANATOMY
The spermatic vein on the right side drains directly into the inferior vena cava. On the left side, it drains into and through the renal vein. This causes a higher pressure gradient in the left spermatic vein, which leads to slight dilation of the pampini-form venous plexus that accounts for the lower-lying position of the left testicle as compared to the right (Fig. 41.6B).
Venous drainage of the spermatic veins is into the systemic circulation by way of the inferior vena cava, placing the lungs as the major metastatic target organ. The lung is the major target organ for nonseminoma testicular cancers. Seminomas tend to follow lymphatic progression, giving rise to transdiaphragmatic nodes if retroperitoneal nodes are involved. Either mediastinal nodes or a left supraclavicular node can be involved if the thoracic duct is infiltrated.
Figure 41.6 | A. N-oncoanatomy. Regional lymph nodes are in the para-aortic area. Left: Renal hilar nodes; right: Paracaval nodes. B. M-oncoanatomy: Great vessels, kidneys, and suprarenal glands.
STAGING WORKUP
RULES OF CLASSIFICATION AND STAGING
Clinical Staging and Imaging
Clinical examination of primary can be enhanced by sonograms, computed tomography (CT), and magnetic resonance imaging (MRI)/coil examination. In addition, nodal assessment entails CT of abdomen and pelvis for para-aortic and renal hilar nodes and chest for mediastinal nodes and lung. Serum for marker should be collected before treatment (Table 41.5; Fig. 41.7).
Pathologic Staging
Full assessment of radical orchidectomy specimen for spread, that is, intratesticular or extratesticular, is worth noting, as is invasion of epididymis and for spermatic cord. Multiple tissue sections include one distant from the tumor to determine whether tubular germ cell neoplasia (cancer in situ) is present. Careful evaluation for vascular invasion in multiple sections should be noted. If a retroperitoneal node is evaluated, location, size, and extranodal soft tissue extension should be recorded.
Oncoimaging Annotations
• Scrotal ultrasonographic scanning is an extension of the physical examination. It should be used in most patients with a scrotal mass, especially when the physical examination is difficult or inconclusive.
• MRI is considered an adjunct to ultrasonography for indeterminate testicular lesions or in patients with a discrepancy between ultrasonographic findings and the physical examination. Cross-sectional imaging (CT preferred over MRI) is helpful in the evaluation of nodal disease and metastatic spread.
• The finding of the nodal location (regional nodes), nodal size (>10 mm), and number of detected nodes all play a role in CT/MRI diagnosis of nodal disease.
• Neither CT nor MRI can differentiate benign from malignant nodal involvement, and both understage the early metastatic disease in up to 30% of patients.
• Neither CT nor MRI can differentiate between active tumor and posttreatment fibrosis.
PROGNOSIS AND CANCER SURVIVAL
Figure 41.7 | 1. testis 2. tunica albuginea 3. epididymis
PROGNOSIS
The limited number of prognostic factors are listed in Table 41.6.
CANCER STATISTICS AND SURVIVAL
When considered together, the male genital and urinary systems are the major sites of malignancy. Prostate cancer alone accounts for 200,000 new patients annually. There are 100,000 new urinary tract cancers and 2.5-fold more of male genital cancers, or 250,000 cases annually.
The dramatic gains in survival are due to multidisciplinary achievements in screening, early detection, precise diagnoses, and effective multimodal therapies. The cancer statistics reveal perhaps the greatest gains in survival in oncology over the last five decades. In local stage I, male genitourinary tumors are 90% to 100% curable according to the latest Surveillance Epidemiology and End Results data: kidney, 90%; bladder, 94%; testes, 99%; and prostate, 100%. Death and mortality rates are declining. The pediatric Wilms’ tumor was the first malignancy in childhood to be cured, achieving >90% long-term survival, heralding the success of multimodal treatment that would be achieved in adult tumors in urology.
SECTION 5
Gynecologic Primary Sites
