322 CHAPTER 6 Urological neoplasia

Urethral cancer

Primary urethral cancer is rare, occurring in elderly patients. It is 4 times more common in women than in men.

Risk factors

Urethral stricture and sexually transmitted disease are implicated. Direct spread from tumor in the bladder or prostate is more common.

Pathology and staging

Seventy-five percent of cases are SCC, occurring in the anterior urethra; 15% are UC, occurring in the posterior/prostatic urethra; 8% are adenocarcinoma; and the remainder include sarcoma and melanoma.

Urethral cancer metastasizes to the pelvic lymph nodes from the posterior urethra and to the inguinal nodes from the anterior urethra in 50% of patients.

Staging is by the TNM system (see Table 6.21) following histological confirmation of the diagnosis.

Presentation

This is often late; many patients have metastatic disease at presentation

•Painless hematuria; initial, terminal, or a bloody urethral discharge

•Voiding-type LUTS (less common)

•Perineal pain (less common)

•Periurethral abscess or urethrocutaneous fistula (rare)

•Past history of sexually transmitted or stricture disease

•Examination may reveal a hard, palpable mass at the female urethral meatus or along the course of the male anterior urethra. Inguinal lymphadenopathy, chest signs, and hepatomegaly may suggest metastatic disease.

Differential diagnosis

In men

•Urethral stricture

•Perineal abscess

•Metastatic disease involving the corpora cavernosa

•Urethrocutaneous fistula (secondary to benign stricture disease)

In women

•Urethral caruncle

•Urethral cyst

•Urethral diverticulum

•Urethral wart (condylomata acuminata)

•Urethral prolapse

•Urethral vein thrombosis

•Periurethral abscess

Investigations

Cystourethroscopy, biopsy, and bimanual examination under anesthesia will obtain a diagnosis and local clinical staging. Chest radiography and abdominopelvic CT scan will enable distant staging.

Treatment

For localized anterior urethral cancer, radical surgery or radiotherapy are the options. Results are better with anterior urethral disease (see Box 6.8). Male patients would require perineal urethrostomy. Postoperative incontinence due to disruption of the external sphincter mechanism is minimal unless the bladder neck is involved, but the patient would need to sit to void.

For posterior/prostatic urethral cancer, cystoprostatourethrectomy should be considered for men in good overall health, while anterior pelvic exenteration (excision of the pelvic lymph nodes, bladder, urethra, uterus, ovaries, and part of the vagina) should be considered for women.

324 CHAPTER 6 Urological neoplasia

In the absence of distant metastases, inguinal lymphadenectomy is performed if nodes are palpable, since 80% contain metastatic tumor.

For locally advanced disease, a combination of preoperative radiotherapy and surgery is recommended.

For metastatic disease, chemotherapy is the only option with regimens of systemic cisplatin, bleomycin, and methotrexate or 5-fluorouracil and methotrexate in addition to surgical resection in the treatment of metastatic urethral SCC.

Staging

Staging is by the TNM (2002) classification following histological confirmation of the diagnosis (see Table 6.21). All cases rely on physical examination and imaging, the pathological classification (prefixed p) corresponding to the TNM categories.

Box 6.8 5-year survival

This page intentionally left blank

326 CHAPTER 6 Urological neoplasia

Retroperitoneal fibrosis

Retroperitoneal fibrosis (RPF) was first described by the French urologist Albarran at the beginning of the 20th century. The condition is also known as Ormond disease.

Benign causes

Idiopathic RPF comprises two-thirds of benign cases. A fibrous plaque extends laterally and downward from the renal arteries, encasing the aorta, inferior vena cava, and ureters, but rarely extends into the pelvis. The central portion of the plaque consists of woody scar tissue, while the growing margins have the histological appearance of chronic inflammation. It may be associated with the following:

•Mediastinal, mesenteric, or bile-duct fibrosis

•Drugs, including methysergide, B-blockers, haloperidol, amphetamines, and LSD

•Chronic urinary infections including TB and syphilis

•Inflammatory conditions such as Crohn’s disease or sarcoidosis

•Abdominal aortic aneurysm (AAA), intra-arterial stents, and angioplasty may induce idiopathic fibrosis due to periaortitis,

hemorrhage, or an immune response to insoluble lipoprotein.

Malignant causes

•Lymphoma is the most common cause; RPF is also sometimes due to sarcoma.

•Metastatic or locally infiltrative carcinoma of the breast, stomach, pancreas, colon, bladder, prostate and carcinoid tumors

•Radiotherapy may cause RPF, although this is rare today with more precise field localization.

•Chemotherapy, especially following treatment of metastatic testicular tumors, may leave fibrous masses encasing the ureters. These may or may not contain residual tumor.

Presentation

Idiopathic RPF classically occurs in the fifth or sixth decade of life.

Men are affected twice as commonly as women. In the early stage, symptoms are relatively nonspecific, including loss of appetite and weight, low-grade fever, sweating, and malaise. Lower limb swelling may develop. Dull, non-colicky abdominal or back pain is described in up to 90% of patients. Later, the major complication of the disease develops: bilateral ureteral obstruction causing anuria and renal failure.

Examination may reveal hypertension in up to 60% of patients and an underlying cause such as an AAA.

Investigations

Inflammatory serum markers are elevated in idiopathic RPF (60–90% elevated ESR). Pyuria or bacteriuria is common.

Ultrasound will demonstrate unior bilateral hydronephrosis.

RETROPERITONEAL FIBROSIS 327

CT, IVP, or retrograde ureterography will reveal tapering medial displacement of the ureters with proximal dilatation and will exclude calculus disease. Up to one-third of patients will have a nonfunctioning kidney at the time of presentation due to long-standing obstruction.

CT-guided fine needle or laparoscopic biopsy of the mass may confirm the presence of malignant disease, but a negative result does not exclude malignancy.

Management

Emergency management of a patient presenting with established renal failure requires relief of the obstruction by percutaneous nephrostomy or ureteral stenting. Fluid and electrolyte losses need to be replaced following relief of bilateral ureteral obstruction and postobstructive diuresis. Assess with daily weighing and measurement of blood pressure lying and standing.

Steroids may decrease the edema often associated with RPF and may help reduce the obstruction. If used, they are usually discontinued when inflammatory markers (such as ESR or CRP) return to normal. The antiestrogen tamoxifen and cyclophosphamide have been used successfully in some patients but are not considered the standard of care.

Surgical ureterolysis with omental wrap is often necessary to free and insulate the ureters from the encasing fibrous tissue. This can be accomplished by the open, laparoscopic or hand-assisted laparoscopic approaches. Biopsies are taken to exclude malignancy. If malignancy is noted, treatment is directed at the cause.

Monitor patient for recurrent disease with serum creatinine and ultrasound every 3–6 months for 5 years.