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Urethral cancer (urethral cancer)
Last reviewed: 23.04.2024
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Urethral cancer (urethral cancer) is a rare tumor that accounts for less than 1% of all urinary tract infections. Low morbidity causes the absence of a standardized approach to managing patients with urethral cancer.
In this regard, the results of treatment of this disease remain unsatisfactory.
Epidemiology
Primary cancer of the urethra in men is extremely rare. About 600 reports have been published in the literature. The tumor is diagnosed at any age, although men over 50 years old are more likely to suffer. In women, urethral cancer (urethral cancer) is 0.02-0.5% of malignant neoplasms of the female urinary and genital area. Usually the disease develops in postmenopause. 75% of patients with cancer of the urethra are older than 50 years.
Causes of the urethral cancer (urethral cancer)
The etiology of urethral cancer has not been established. An optional precancerous disease is leukoplakia. The risk factors include bladder cancer, chronic urinary tract infection and prolonged trauma to the mucous membrane of the urethra.
Histogenesis
Histogenesis of urethral cancer depends on the type of epithelium covering the area of the urethra, in which the tumor is localized. The distal part of the urethra is lined with a flat epithelium, which serves as a source of squamous cell carcinoma, proximal to the transitional cell, from which the transitional cell tumors originate.
Adenocarcinoma arises from the glandular tissue of the prostate in men and paraurethral glands in women. In women, squamous cell carcinoma is 60%, transitional cell carcinoma is 20%. Adenocarcinoma - 10%. Melanoma - 2%. Rare tumors (sarcomas, neuroendocrine tumor, plasmacytoma, metastases of other tumors) 8% of all observations. Among men, tumors of the urethra are squamous cell carcinoma in the wave, transitional cell cancer in 15%, adenocarcinoma, melanoma and sarcomas in 5% of cases.
Growth and metastasis
Cancer of the urethra, especially when affection of its proximal parts, is prone to local invasive growth. Men can sprout spongy and cavernous bodies of the penis, urogenital diaphragm, prostate, perineum and scrotum skin. In women, the tumor tends to germinate the underlying tissues and spread to the front wall of the vagina, bladder and cervix.
For urethral cancer, lymphogenous metastasis is characteristic of the inguinal and iliac lymph nodes. Enlarged inguinal lymph nodes are detected in 1/3 of patients with urethral cancer, while the presence of metastases is confirmed in 90% of the observations. At the time of diagnosis, 20% of patients have metastases to the iliac lymph nodes. In the future, the appearance of metastases in the lymph nodes of the pelvis is noted in 15% of patients. Metastasis to distant groups of lymph nodes occurs rarely.
Hematogenous metastases to the parenchymal organs appear late. Losses of the lungs, pleura, liver, bones, adrenals, brain, salivary glands, and glans penis are described.
Symptoms of the urethral cancer (urethral cancer)
Symptoms of cancer of the urethra are variable, non-pathognomonic and largely depend on the disease, against which the malignant process develops. Symptoms of cancer of the male urethra - discharge, pain, difficulty urinating up to its delay, palpable compaction, periurethral abscesses and fistulas, malignant priapism. The symptoms of urethral cancer in women include discharge, the presence of volumetric education in the area of the external opening of the urethra, difficulty urinating, pain in the urethra and perineum, urinary incontinence, urethro-vaginal fistula bleeding from the vagina.
In a third of patients with palpation of inguinal areas, enlarged lymph nodes are revealed. Tumor thrombosis of the lymphatic vessels of the pelvis and groin area can lead to the appearance of edema of the lower half of the body.
The appearance of metastases in the parenchymal organs causes the development of the corresponding symptomatology.
Forms
TNM-classification of urethral cancer (urethral cancer).
Primary tumor (male and female)
- Tx-the primary tumor can not be evaluated.
- T0 - no signs of a primary tumor.
- Ta - non-invasive papillary, polypoid, or verruzed (warty) carcinoma.
- Tis - carcinoma in situ (preinvasive).
- T1 tumor extends to the subepithelial connective tissue.
- T2 - the tumor extends to the spongy body of the penis or prostate, or periurethral muscle.
- T3 - the tumor extends to the cavernous body or beyond the capsule of the prostate, or to the front wall of the vagina, or the neck of the bladder.
- T4 - the tumor spreads to other neighboring organs.
Regional lymph nodes
- Nx - regional lymph nodes can not be evaluated.
- N0 - there are no metastases to the regional lymph nodes.
- N1 - metastasis in one lymph node not more than 2 cm in the largest dimension.
- N2 - metastasis in one lymph node more than two in the largest measurement or multiple metastases to the lymph nodes.
Distant metastases
- Mx - distant metastases can not be evaluated.
- M0 - no distant metastases.
- Ml - distant metastases.
Pathoanatomical classification of pTNM
Categories pT, pN, pM correspond to categories T, N, M, G - histopathological gradation.
- Gx - the degree of differentiation can not be estimated.
- G1 is a highly differentiated tumor.
- G2 - moderately differentiated tumor.
- G3-4 is a low-grade / undifferentiated tumor.
Diagnostics of the urethral cancer (urethral cancer)
A thorough examination, palpation of the external genitalia, perineum and bimanual palpation are necessary to assess the local prevalence of the tumor. The main diagnostic method is urethrocystoscopy, which allows to determine the localization, size, color, nature of the tumor surface, the condition of the surrounding mucosa. For cancer of the urethra (cancer of the urethra) is characterized by the presence of a solid tumor on a broad base, with a light-rooting and often ulcerated surface. With a significant narrowing of the urethra by the tumor, it is possible to indirectly judge the localization, shape and size of the neoplasm, allowing the presence of a defect in the urethra filling in the ascending and umbilical urethrograms. The degree of local prevalence of the tumor process and the state of regional lymph nodes are assessed using transabdominal and transvaginal ultrasound, CT and MRI. In order to identify distant metastases, all patients undergo chest X-ray, ultrasound and CT scan of the abdominal cavity, retroperitoneum and pelvis.
Scanning of the bones of the skeleton is performed only by patients presenting the corresponding complaints. Morphological confirmation of the diagnosis is obtained by histological examination of the tumor biopsy. Cytological examination of smears-prints, scrapings from neoplasm, separated from the urethra can be possible.
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Differential diagnosis
Errors in the diagnosis of urethral cancer are met in 10% of cases. In men, a differential diagnosis of urethral cancer should be carried out with benign tumors, stricture, chronic urethritis, tuberculosis, prostate cancer, and stone. In women, urethral cancer should be distinguished from a tumor of the vulva and vagina, benign neoplasms and inflammatory diseases of the urethra, paraurethral cysts, and the prolapse of the mucous membrane of the urethra combined with the pubescence of the vaginal wall. The only reliable criterion that excludes urethral cancer (urethral cancer) - Morphological verification of diagnosis.
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Treatment of the urethral cancer (urethral cancer)
Treatment of urethral cancer depends on the stage and location of the tumor. Due to the small number of observations, the standard method of managing patients with this disease has not been developed.
Below are the most common approaches.
Treatment of urethral cancer in women
With small superficial tumors of the distal urethra T0 / Tis, Ta, it is possible to perform TUR or open resection, fulguration, destruction by niodim Nd: YAG or carbon dioxide CO2 laser. Detection of large surface (Ta-T1), as well as invasive (T2) neoplasms serves as an indication for interstitial or combined (interstitial and remote) radiation therapy. In case of cancer of the distal part of the female urethra at stage T3, as well as after relapse after surgical treatment or irradiation of this zone, anterior exenteration of the pelvis is performed with or without preoperative radiotherapy. Palpable inguinal lymph nodes serve as an indication for their removal with urgent histological examination. In cases of confirmation of their metastatic lesions, ipsilateral lymphadenectomy is performed. Routine lymph node dissection with unregistered regional lymph nodes is not indicated.
Cancer of the proximal urethra in women is an indication for the appointment of non-adjuvant radiotherapy and anterior pelvic exenteration with bilateral pelvic lymphadenectomy. Ispylateral inguinal lymphodissection is performed with positive results of cytological or histological examination of the biopsy specimen of enlarged lymph nodes of this localization.
Massive neoplasms may also require resection of the symphysis and lower branches of the pubic bones with the reconstruction of the perineum by the musculocutaneous flap. For tumors of the proximal part of the urethra less than 2 cm in the largest measurement, an attempt may be made to perform an organ-preserving radiotherapy, operative or combined treatment.
Treatment of urethral cancer in men
Surface cancer of the distal urethra T0 / Tis-Tl can be successfully treated by TUR or open resection, fulguration, destruction by niodymium Nd: YAG or carbon dioxide CO2 laser. Invasive tumors of the scaphoid fossa serve as an indication for amputation of the head, infiltrative neoplasms (T1-3), located proximally, to amputation of the penis, receding 2 cm proximal to the tumor edge. Radiation therapy for tumors of the distal part of the male urethra is considered as a compulsory alternative to surgical treatment in patients who refuse to have a pectectomy.
Cancer of the bulbomembranous and prostatic urethra in men is an indication for neoadjuvant radiation therapy followed by cystoprostatectomy with urinary diversion, pectectomy, bilateral pelvic lymphodissection with orpis lympholysis (with or without it) in the presence of verified metastases in enlarged inguinal lymph nodes. With locally advanced tumors, the symphysis and lower branches of the pubic bones are removed to increase the radicalism of the intervention.
A common cancer of the urethra serves as an indication for chemoradiation. When receiving a pronounced clinical response to therapy, an attempt at subsequent radical intervention is possible. The chemotherapy regimen is determined by tumor histogenesis.
- In transitional cell carcinoma, the M-VAC scheme is used (methotrexate 30 mg / m2 - days 1, 15, 22, vinblastine 3 mg / m2 - 2 nd, 15 th, 22 nd days, adriamycin 30 mg / m2 - 2 nd day and cisplatin 70 mg / m2 - 2 nd day).
- When squamous - chemotherapy with the inclusion of 5-FU (375 mg / m2 - 1-3 days), cisplatin (100 mg / m2 - 1 st day) and calcium folinate (20 mg / m2 - 1-3 days) ).
- In adenocarcinoma - a scheme based on 5-FU (375 mg / mg - 1-3 days), cisplatin (100 mg / m2 - the first day).
Complex treatment of urethral cancer (cancer of the urethra) and chemotherapy interfere with cell repair after sublethal doses of radiation. Surgery is performed 4-6 weeks after the end of neoadjuvant treatment.