Tumors of the cup and pelvis system

Tumors of the cup-and-pelvic system develop from urothelium and in the overwhelming majority are cancer of varying degrees of malignancy; they occur 10 times less often than tumors of the renal parenchyma.

Tumors of the cup and pelvis system and ureter proceed from the transitional epithelium lining the upper urinary tract; this, as a rule, exophytally growing papillary neoplasms.

Epidemiology

These neoplasms are relatively rare and account for 6-7% of primary kidney tumors. The vast majority of them (82-90%) are transitional cell carcinomas; squamous cell carcinoma is observed in 10-17%, adenocarcinoma - in less than 1% of cases. The annual increase in morbidity is about 3%, which may be due to the deterioration of environmental conditions, although it may be the result of improved diagnostics.

Men are ill 2-3 times more often than women, the age peak of incidence falls on the 6th to 7th decades of life. In childhood, these neoplasms are extremely rare. Tumors of calyx and pelvis are diagnosed 2 times more often than tumors of the ureter. When localized in the ureter, its lower third is more often affected. Tumor formations can be single, but more often they register multifocal growth. Bilateral lesions of the upper urinary tract are observed in 2-4% of cases, but mostly it develops in patients with Balkan nephropathy - a risk factor for this disease.

[1], [2], [3], [4], [5], [6], [7]

Causes of the tumors of the pulmonary system

The causes of tumors of the calyx and pelvic system and ureter, as well as tumors of the bladder, are largely known. The influence of environmental factors has been established, the effect of which can be significantly delayed. These include the effects of aniline dyes, beta-naphthylamines. The incidence rate is 70 times higher, and the average time from the onset of exposure to the development of the tumor is about 18 years.

The systematic use of phenacetin-containing analgesics for decades with the onset of nephropathy increases the risk of such neoplasms by 150 times, and the time until the appearance of the tumor can last up to 22 years. A prominent place in the development of the disease is Balkan endemic nephropathy: men and women, usually employed in agricultural production in Romania, Bulgaria, the countries of the former Yugoslavia, suffer equally often; latent period of the disease is up to 20 years; The peak incidence falls on the 5th-6th decade of life. The risk of disease in this endemic area is 100 times higher; tumors occur in 40% of persons suffering from Balkan nephropathy. In 10% of cases, neoplasms are bilateral, most of them are low-grade transitional-cellular cancer.

An important predisposing factor in the development of these tumors is contact with organic solvents, petroleum products, car exhausts. Studies in recent years have shown that urban residents have a higher incidence of morbidity than rural ones; in the city the drivers of motor transport, auto repairmen and car inspectors are most vulnerable. Smoking increases the risk of the disease 2.6-6.5 times for men and 1.6-2.4 times for women compared with non-smokers. A possible connection is the development of neoplasms with chronic inflammatory processes in the wall of the upper urinary tract.

Pathomorphological features of tumors of the calyx-pelvis system

Tumors most often (82-90%) are papillary tumors having the structure of transitional cell carcinoma high (30%), medium (40%) and low (30%) degree of differentiation, often with multicentric growth. 60-65% of the neoplasm is located in the pelvis, 35-40% in the ureter (15% in the upper and middle and 70% in the lower third). The histological type distinguishes urothelial, squamous, epidermoid and adenocarcinoma.

Tumors metastasize lymphogenically to the nodes of the kidney, paracaval (right), para-aortic (left), retroperitoneal, corresponding periureteral, iliac and pelvic. Lymph node involvement is an extremely unfavorable prognostic sign, while the size, quantity and localization of lymphogenous metastases have little effect on the outcome of the disease. There is a point of view about the possibility of implantation metastasis down the ureter in the bladder, but the intra-wall lymphogenous pathway is more likely. Tumors are insensitive to chemotherapy and radiation therapy, they have an unfavorable prognosis.

[8], [9], [10],

Symptoms of the tumors of the pulmonary system

Most patients note total macuraturia with the departure of vermicular clots. Hematuria can be initially painless, but with occlusion of the ureter, clots can be accompanied by a pain attack by the type of renal colic on the side of the lesion, which stops as the clots retreat. Constant blunt aching pain  is a sign of chronic impairment of urine outflow with the development of hydronephrosis. In this case, bleeding into the lumen of the cup-and-pelvis system can be accompanied by the development of hematohydronephrosis with a tamponade of the cup-and-pelvic system with blood clots, the development of acute pyelonephritis.

The classic triad of symptoms described in a kidney tumor (hematuria, pain, palpable formation), as well as anorexia, weakness, weight loss, anemia, indicate the neglected nature of the tumor and a poor prognosis of the disease. According to the literature, 10-25% of patients may not have any clinical symptoms.

Forms

The clinical classification is designed to assess the depth of the lesion, the prevalence and severity of the cancer process. As with parenchyma, the International Classification of the TNM system has been adopted.

T (tumor) is the primary tumor:

• T - papillary noninvasive carcinoma.
• T1 - the tumor sprouts into the subepithelial connective tissue.
• T2 - the tumor sprouts into the muscle layer.
• ТЗ (pelvis) - the tumor grows into okololohanochnuyu cellulose and / or parenchyma of the kidney.
• TK (ureter) - the tumor sprouts into the peri-cellular tissue.
• T4 - the tumor sprouts into the neighboring organs or through the kidney into the paranephric fiber.

N (nodnlus) - regional lymph nodes:

• N0 - there are no metastases in the regional lymph nodes.
• N1 - metastasis in a single lymph node from 2 to 5 cm, multiple in size not more than 5 cm.
• N3 - metastasis in the lymph node more than 5 cm.

M (methastases) - distant metastases:

• M0 - distant metastases are absent.
• Ml - distant metastases.

[11]

Diagnostics of the tumors of the pulmonary system

Diagnosis of tumors of the calyx-pelvis and ureter is based on clinical, laboratory, ultrasound, X-ray, magnetic resonance, endoscopic and morphological data.

Laboratory analysis and instrumental diagnostics of tumors of the bowl-and-pelvis system

The most common and persistent signs are microhematuria of different intensity, associated with it false proteinuria, as well as the detection of atypical cells in the urine sediment. Leukocyturia and bacteriuria testify to the attachment of the inflammatory process, and hypoisostenuria and azotemia - on the reduction of the total renal function. Repeated massive hematuria may cause anemia. An extremely unfavorable prognostic sign is the acceleration of ESR.

Ultrasonic diagnostics of tumors of the calyx-pelvis system

Indirect signs of a tumor are manifestations of impaired urine outflow in the form of hydrocalicosis, pyeloectasia and hydronephrosis in lesions of the pelvis, ureterohydronephrosis with involvement of the ureter in the process. Against the background of the expansion of the cup-and-pelvic system, it is possible to reveal wall-wall defects of filling, characteristic for exophytic tumors. In the absence of the image of cups and pelvis, the informative value of the study increases against the background of drug-induced polyuria after the administration of 10 mg furosemide.

An important role in diagnosis since recently began to play endoluminal  ultrasound, significantly complementary endoscopic. The scanning sensor, reminiscent of the ureteral catheter, can be passed through the ureter to the pelvis. The appearance of a parietal filling defect with changes in the underlying tissues allows not only to diagnose the neoplasm but also to clarify the nature and depth of the invasion of the wall.

Radiographic diagnosis of tumors of the calyx-pelvis system

X-ray studies are traditionally widespread in the diagnosis of neoplasms of the upper urinary tract. In the picture, papillary tumors can be seen only in cases of calcification, usually against necrosis and inflammation. On excretory urograms, a symptom of these tumors is the parietal filling defect in pictures in the direct and semi-lateral projections, which should be differentiated from the X-ray negative stone. Invaluable help in this has an ultrasound. Absence of signs of concrement with ultrasound and a defect of filling on the urogram are characteristic for a papillary tumor.

CT scan

Computed tomography  at the present time, especially with the introduction of multislice CT, is gaining increasing importance in the diagnosis of papillary neoplasm of the calyx and pelvic system and ureter. An invaluable role in this is played not only by transverse contrasted sections at the level of the supposed lesion, but also by the possibility of constructing three-dimensional images of the upper urinary tract and the so-called virtual endoscopy, which allows using digital X-ray image processing techniques to construct an image of the inner surface of a given segment of the upper urinary tract (calyx, pelvis, ureter).

[12], [13], [14]

Magnetic resonance imaging

The advantages of this method are the possibility of detailed analysis of images along the boundary between dense and liquid media, which is very effective in assessing filling defects in the cup-and-pelvis system. Obtaining very demonstrative and useful diagnostic information for papillary tumors of the upper urinary tract allows avoiding retrograde pyeloureterography, which is fraught with inflammatory complications.

Endoscopic examinations

Modern endoscopic diagnostics with the use of thin rigid and flexible ureteropyeloscopes under general or spinal anesthesia makes it possible to examine the inner surface of the calyx, pelvis, ureter, bladder and urethra and, in most cases, see the neoplasm. According to the state of the mucosa, covering the tumor and surrounding it, a visual assessment of the stage of the tumor process is possible. With the help of special instruments it is possible to carry out biopsy of the neoplasm, as well as for small superficial tumors - organ-preserving treatment - electrosurgical resection of the wall of the pelvis, ureter with removal of the tumor within the healthy tissues with the help of special miniature loops (endoscopic electroresection).

[15], [16], [17], [18]

Morphological studies

With the help of a cytological study of a centrifuged urine sediment, atypical cells characteristic of transitional cell carcinoma can be identified. Histological examination of the biopsy specimen obtained by endoscopy allows identifying the tumor.

Treatment of the tumors of the pulmonary system

In addition to endoscopic electrosurgery, which is possible only with small superficial tumors and in large medical institutions equipped with special endoscopic and endosurgical equipment, the main method of treatment of the papillary neoplasms of the upper urinary tract is the operation: the kidney, the ureter are removed all over, and the bladder is resected around the mouth of the corresponding ureter with removal of fascia and regional lymph nodes. The scope of the operation is associated with the possible descending spread of the tumor in the form of daughter tumor formations along the ureter. In the presence of daughter tumors in the bladder they are removed endosurgically. Radiation and chemotherapy in these patients is ineffective.

Clinical examination of patients with tumors of the calyx-pelvis system

Clinical examination of patients who underwent nephrureterectomy with a bladder resection for papillary neoplasms of the upper urinary tract, in addition to examination, clinical analyzes of blood and urine should necessarily include cystoscopy every 3 months for 1 year after surgery, every 6 months - 2 nd and 3 years, and then once a year for life. Endoscopic studies aim to timely identify and remove daughter tumors of the bladder, which can occur quite late after nephrenorelectomy.

[19], [20], [21], [22]