Tumors of urinary tract , college of medicine
- 1. TUMORS OF URINARY TRACT Ahmed MH A’zzaw, MD CABMS(Urology) Kirkuk University, College of Medicine, Department of Surgery
- 3. Neoplasms of the kidney • Benign Benign renal tumors include: Adenoma. Oncocytoma. Angioma. Angiomyolipoma. Leiomyoma. lipoma. Hemangioma. Juxtaglomerular tumors.
- 4. Neoplasms of the kidney • Malignant 1. 1ry renal tumors: ▪ Adenocarcinoma of the Kidney (Renal Cell Carcinoma-RCC-, Grawitz’s tumour, ,hypernephroma, clear cell carcinoma, and alveolar carcinoma ). ▪ Transitional cell carcinoma (TCC) of the renal pelvis. ▪ Wilm’s tumour (nephroblastoma). 2. 2ry tumors All masses in the kidneys should be considered malignant till proven otherwise.
- 5. BENIGN RENAL TUMOURS Angiomyolipoma (Renal Hamartoma) ❏rare benign tumour ❏round, oval, expansible ❏characterized by 3 major histologic components: blood vessels, smooth muscle and fat cells ❏many asymptomatic, may spontaneously rupture, especially in pregnant females ❏found in approximately 45-80% of patients with tuberous sclerosis syndrome which is characterized by • epilepsy • mental retardation • sebaceous adenoma • hamartomas of brain and kidney ❏diagnose by CT ––> fat (negative density on CT) observed in kidneys is pathognomonic for angiomyolipoma ❏treatment: <4cm, >4cm
- 6. BENIGN RENAL TUMOURS Renal Adenoma ❏commonly found incidentally at autopsy or after nephrectomy for an unrelated disease ❏10-20% of the population ❏asymptomatic ❏need tissue diagnosis to definitively differentiate from renal cell carcinoma Renal Oncocytoma ❏ occur in variant organ (adrenal, salivary gland, thyroid,…) ❏ represent about 3% of kidney tumor ❏has a characteristic findings on imaging studies but need histopathology for diagnosis (spoke wheel sign) Renal Angioma ❏ Angioma may cause profuse haematuria, often in young adults. ❏ The bleeding source may be difficult to diagnose without renal angiography.
- 7. MALIGNANT RENAL TUMORS ADENOCARCINOMA (Renal Cell Carcinoma, Grawitz’s Tumour, Internist’s tumor)
- 8. ADENOCARCINOMA (Renal Cell Carcinoma, Grawitz’s Tumour) ❏also known as hypernephroma ❏eighth most common malignancy (accounts for 3% of all newly diagnosed cancers) ❏85% of primary malignant tumours in kidney ❏male:female = 3:1 ❏called the “internist’s tumour” because of paraneoplastic symptomatology
- 9. ETIOLOGY ❏ The cause is unknown. ❏ There are various theories of risk factors: • environmental and occupational factors: • smoking (results in 2x increased relative risk), cadmium exposure, employment in leather industry • familial incidence seen with von Hippel Lindau syndrome which is characterized by: • RCC (present in 2/3) • headache, ataxia, and blindness due to cystic lesions of cerebellum and retinal vessel aneurysms • acquired cystic disease
- 10. PATHOLOGY • The tumor occur in equal frequency in either kidney • originates in the cortex, grow out in the perinephric tissue • originates from proximal convoluted tubule epithelial cell • it is characteristically yellow to orange because of high lipid content
- 11. PATHOGENESIS ❏methods of spread • direct • venous • lymphatic • RCC is a vascular tumor, tend to spread by direct invasion • Vascular invasion is through renal vein and IVC • About 13 of patients have metastasis at presentation • The most common site of distant metastasis is lung, opposite kidney, followed by liver, bone.
- 14. TUMOR STAGING • Stage I: tumor is confined within kidney parenchyma • StageII:tumor involve perinephric fat but is confined within Gerota’s fascia, or adrenals • Stage IIIa : tumor involve the main renal vein or inferior vena cava, byeond Gerotas • Stage IIIb :tumor involve regional lymph node • Stage IIIc : tumor involve both local vessels and regional lymph node • Stage IVa : tumor involves adjacent organs (colon,pancreas,….) • Stage IVb :distant metastasis
- 16. CLINICAL PICTURE It has a wide variety of presentation ❏symptoms and signs • increasingly diagnosed incidentally with U/S and CT • poor prognostic indicators include • weight loss • weakness • anemia • bone pain • classic triad (too late triad!) found in 10-15% • gross hematuria 50% (sometimes associated with clot colic) • flank pain < 50% (dragging discomfort in the loin ) • palpable mass < 30% (In men rapidly developing varicocele is a rare but impressive sign (mostly left)) • Abdominal pain • 30% have metastases when first seen: dyspnea, cough, headache, bone pain
- 17. CLINICAL PICTURE Para-neoplastic syndrome ❑3-10% of RCC present by paraneoplastic syndrome ❑hematopoietic disturbances: anemia, polycythemia; raised ESR: RCC is the most common cause of paraneoplastic erythrocytosis ❑endocrinopathies: hypercalcemia 20% (production of PTH), production of other hormones including erythropoietin, renin, prolactin, gonadotropins, TSH, insulin, and cortisol. ❑PUO ❑ hemodynamic alterations: systolic hypertension 40% (due to AV shunting, production of renin), and peripheral edema (due to caval obstruction) ❑Non-metastatic hepatic dysfunction: “Staufer’s syndrome”: abnormal liver function tests, decreased WBC count, fever, areas of hepatic necrosis; reversible following removal of primary tumour
- 18. Laboratory FINDINGS • routine labs for paraneoplastic syndromes • CBC anemia (30%) • High ESR • Serum calcium • S. PTH • LFT • urinalysis (60-75% have hematuria)
- 19. Imaging • US: Doppler: solid or cystic • IVP 75% accurate: soft tissue shadow, calcification overlying the renal shadow & distorted renal outline. • CT scan with contrast: it is the leader for diagnosis and staging and detect distant metastasis. ▪ RCC becomes enhanced with the use of intravenous contrast media. ▪ the method of choice in staging the patient by visualizing the renal hilum, perinephric space, renal vein and vena cava, adrenals, regional lymphatics, and adjacent organs.
- 20. Imaging • MRI: is equivalent to CT for staging of RCC. • MRI angiography (MRA): tumor thrombus in renal vein and IVC • Renal angiography: no longer routinely done ▪ pre operative embolisation for size reduction or controlling of bleeding • Radionuclide bone scan: Determination of metastases to bones. • CXR: to detect lung secondaries
- 21. FNA/Bx • Fine needle aspiration or percut. Bx are forbidden (to avoid local dissemination ), but • indicated in: ▪ metastatic disease, planned for nonsurgical management ▪ establishing diagnosis in patients who are not surgical candidate ▪ bilateral renal tumors: suspecion of renal lymphoma ▪ secondary renal tumors: differentiating a primary RCC from a renal metastasis in patients with known primary cancers of non-renal origin. ▪ evaluating some radiographically indeterminate lesions.
- 25. Differential diagnosis • Urothelial carcinoma of renal pelvis • Renal lymphoma • Adrenal cancer • Benign renal tumor • Renal cysts • Renal abscess
- 26. TREATMENT LOCALISED DISEASE ❑Stage (I, II , IIIa )→ • surgical (mainstay): • partial Nx: • small < 4cm, polar, or bilateral tumors • radical nephrectomy: • en bloc removal of kidney, tumour, ipsilateral adrenal gland and intact Gerota’s capsule and periaortic lymphadenectomy • Cryoablation, high-intensity focused US, and radiofrequency ablation: for the treatment of multiple small tumors, incidentally discovered renal lesions, individuals with many comorbidities. • Observation as treatment should also be mentioned for small (< 3.0 cm) lesions, particularly in elderly patient.
- 28. DISSEMINATED DISEASE • 30% of RCC are metastatic • Surgical: – the role of radical nephrectomy is limited. It is a palliative therapy – • surgical removal of solitary metastasis may be considered • Immunotherapy→15% response rate – cytokine interleukin-2 – α- INF – Tyrosin kinase inhibitors---monoclonal Abs • Radiotherapy (RCC is a radioresistant): • • radiation for palliation • • for painful bony lesions • Chemotherapy (is also chemoresistant )
- 29. Prognosis • stage at diagnosis is the single most important predictor of survival: • 5 year survival of T1 is 90-100% • 5 year survival of T2-T3 is approximately 60% • 5 year survival of patients presenting with metastasis is 0-20%
- 30. UROTHELIAL CARCINOMA OF THE RENAL PELVIS AND URETER
- 31. UROTHELIAL CARCINOMA OF THE RENAL PELVIS AND URETER ❏incidence • rare, accounts for 4% of all urothelial cancers • frequently multifocal • papillary transitional cell cancer 85% • The mean age at diagnosis is 65 years • male:female = 3:1 ❏relative incidence • bladder:pelvis:ureter = 100:10:1 ❏predisposing factors • chemical exposure (industrial dyes and solvents) • smoking • analgesic abuse (acetaminophen, aspirin, and phenacetin) • Balkan nephropathy Patients with single upper tract carcinoma are at risk of developing bladder carcinoma (30-50%) and contralateral upper tract (2-4%).
- 33. ❏symptoms and signs • gross painless hematuria (70-90% of patients) • microscopic hematuria found incidentally • flank pain 50% • tenderness over kidney • flank mass caused by either tumour or associated hydronephrosis (10-20% of patients) • irritative symptoms • weight loss
- 34. LABORATORY FINDINGS • Hematuria is identified in majority of cases • Anemia • Elevated liver function • Urine cytology
- 35. IMAGING • diagnosis is made by noting a radiolucent filling defect on IVP • differential diagnosis of filling defect • transitional cell carcinoma (differentiate via cytology and CT scan) • uric acid stone (differentiate via cytology and CT scan) • blood clot • pyelitis cystica • papillary necrosis • fungus ball • gas bubble from gas producing organisms • RETROGRADE PYELOGRAPHY is more accurate • CT scan identify soft tissue abnormality of renal pelvis • URETEROPYELOSCOPY allow direct visualization of upper urinary tract and tissue sampling
- 39. TREATMENT • Based on: grade, stage, position and multiplicity • The standard therapy is radical ureteronephrectomy with cuff of bladder owing to the possibility of multifocal disease within the ipsilateral collecting system
- 41. Tumor of distal ureter : • distal ureterectomy and ureter reimplantation • Minimally invasive tech: endoscopic electro-resection, snaring or laser fulguration for small superficial papillary tumor
- 42. Advanced disease: • Radiotherapy • Chemotherapy
- 44. NEPHROBLASTOMA (WILMS TUMOUR) ❏The most common solid renal tumor of childhood ❏arises from metanephric blastema ❏5% of all childhood cancers ❏5% bilateral ❏average age of incidence is 3 years ❏No sex predilection. ❏1/3 hereditary and 2/3 sporadic • familial form associated with: ▪ other congenital abnormalities as beckwith- wiedmann and hemihypertrophy syndromes ▪ gene defects as mutation in the Wilms’ tumour suppressor gene (WT1) is responsible for some cases.
- 45. WILMS TUMOUR ❏Pathogenesis & Pathology: • The typical Wilms tumor consists of blastemal, epithelial, and stromal elements in varying proportions • It arise from congenital nephrogenic rests ❏ Tumor dissemination can occur by: • Direct extension through the renal capsule. • Hematogenously via the renal vein and vena cava. • Lymphatic spread.
- 47. Clinical Findings • A. SYMPTOMS AND SIGNS • The diagnosis of Wilms tumor is most commonly made after the discovery of an asymptomatic mass by a family member or a physician during a routine physical examination. • Common symptoms at presentation include abdominal pain and distention, anorexia, nausea and vomiting, fever, and hematuria. • The most common sign is an abdominal mass. • Hypertension is seen in 25–60% of cases and is caused by elevated renin levels • Up to 30% of patients demonstrate hematuria and coagulopathy can occur in 10%.
- 48. B. LABORATORY ANALYSIS • Urinalysis may show evidence of hematuria, and • anemia may be present, particularly in patients with evidence of subcapsular hemorrhage. • Patients with liver metastases may have abnormal serum chemistries.
- 49. C. X-RAY IMAGING • Abdominal US and CT scanning are performed initially to evaluate the mass. • MRI can also provide important information in defining the extent of tumor into the inferior vena cava, including those with intra cardiac extension. • Chest x-ray remains the initial examination of choice to evaluate for the presence of lung metastases.
- 52. D. NEEDLE BIOPSY • Preoperative biopsy is indicated routinely only in tumors deemed too large for safe primary surgical resection and for which preoperative chemotherapy or radiation therapy is Planned.
- 57. Differential Diagnosis • The differential diagnosis of a flank mass in a child includes: – Hydronephrosis – Cystic kidneys – Intrarenal neuroblastoma – Mesoblastic nephroma – Various very rare sarcomas
- 58. Treatment Of Wilms Tumor • A. SURGICAL MEASURES • For patients with unilateral kidney involvement whose tumors are deemed surgically resectable (tumors not crossing the midline or involving adjacent visceral organs), radical nephrectomy via a transabdominal incision is the procedure of choice.
- 59. B. CHEMOTHERAPY • favorable histology tumors undergo surgical resection and have adjuvant chemotherapy with vincristine and dactinomycin combinations. • Patients with Unfavorable tumors are receiving vincristine,doxorubicin, cyclophosphamide, and etoposide.
- 60. C. RADIATION THERAPY • Postoperative radiation is recommended for patients with stage III or IV disease with favorable histology, stages II–IV with focal anaplasia and clear cell sarcoma, and all stages of rhabdoid tumor of the kidney.
- 61. Differentiation between Wilms & Neuroblastoma • In contrast to Wilms tumors, which are typically confined to one side of the abdomen, neuroblastomas usually cross the midline. • Wilms tumors are intrarenal masses and rarely cause a change in the axis of the kidney, while neuroblastomas may cause an outward and downward displacement of the kidney (drooping lily). • Children with neuroblastomas are more likely to present with metastatic disease.This tumour has an unexplained tendency to metastasise to the bony orbit, and thus periorbital ecchymoses (raccoon eyes), proptosis, or both. • these tumors have a higher frequency of calcification observed radiographically (egg-shell vs punctuation) • neuroblastomas may produce various tumor markers including vanillylmandelic acid and other catecholamines that are not seen in patients with Wilms tumor.