RENAL TUMOR

OLEH : I MADE ANDI SAPUTRA

PEMBIMBING : DR. MUNAWIR, SP.BA
INTRODUCTION

 2nd most common abdominal tumor in infants childern behind neuroblastoma

 Varies a wide spectrum from benign to extremly malignant
 Study: COG/NWTSG and SIOP
CLASSIFICATION
WILMS TUMOR

 also referred to as nephroblastoma or renal embryoma

 WT is the most frequent tumor of the kidney in infants and children
 7.6 cases for every million children younger than 15 years
MOLECULAR GENETICSMOLECULAR GENETICS

 Multiple mutated WT genes have been identified

 Few were evaluated to define the prognostic of the disease
ASSOCIATED SYNDROMES

make up about 10% of all WT cases

 Denys–Drash syndrome (nephropathy, renal failure, male pseudohermaphroditism, and WT)
 Beckwith–Wiedemann syndrome (BWS; visceromegaly, macroglossia, omphalocele, and hyperinsulinemic
hypoglycemia in infancy)
 WAGR complex (WT with aniridia, genitourinary malformations, and mental retardation)
PATHOLOGIC PRECURSORS:

 NEPHROGENIC RESTS
 NEPHROBLASTOMATOSIS
 MULTICYSTIC DYSPLASTIC KIDNEYS
PATOLOGY

 Favorable histology (90%) “classic WT” consisting of three elements: blastemal, stromal, and epithelial tubules
 Unfavorable histology, consist focal or diffuse anaplasia
STAGING

Two principal staging systems are used for children with WT

 COG
 SIOP
COG
SIOP
CLINICAL PRESENTATION

 Most common presentation :

 Abdominal mass/ abdominal swelling

 Other signs and symptomp

 Hematuria
 Abdominal pain
 Fever
 Hypertension
DIAGNOSTIC

 Radiographic imaging is performed to determine the anatomic location and extent of the mass
 CT/MRI scan of the abdomen will confirm a renal origin to the mass and whether there are bilateral tumors.
 thin lip of renal parenchyma often can be seen extending over the neoplasm in a WT, known as the claw sign
 there are no characteristic radiographic findings allow a completely accurate diagnosis.
 common sites of metastatic spread are the lungs and the liver;
 CT scans of the chest should be included in the staging studies.
TREATMENT

The treatment for WT includes

 resection, chemotherapy, and, in some cases, RT
 effective agents have been subsequently identified, including vincristine, doxorubicin, cyclophosphamide,
ifosfamide, carboplatin, irinotecan, and etoposi
RENAL CELL CARCINOMA

 RCC are generally older than those with WT

 Radical nephrectomy and regional lymphadenectomy : primary modality for cure,
 children with distant spread have a very poor prognosis
 Recent use of monoclonal antibody therapy for metastatic disease has been promising
 Trials of immunomodulating therapy with interferon-α and interleukin-2 have demonstrated efficacy in some
studies,
MESOBLASTIC NEPHROMA

 the most common renal tumor identified in the neonatal period

 initially diagnosed and treated as a congenital WT
 found most frequently in the neonatal period as a palpable flank mass, which can be massive
 Additional symptoms seen at presentation include hematuria, hypertension, vomiting, and jaundice
 treatment of a neonate with an extensively infil-
 trating tumor with 8 weekly courses of vincristine before resection
CYSTIC NEPHROMA

 All lesions are composed of purely cystic masses characterized by multiple thin-walled septations

 Nephrectomy will be curative

OSSIFYING RENAL TUMOR OF INFANCY

 Ossifying renal tumor of infancy is a relatively rare tumor occurring entirely in infancy.
 gross hematuria, and a palpable mass
 lesions are attached to a renal papilla
 confused occasionally with staghorn calculi.
 Histologically contain osteoid, osteoblastic cells, and spindle cells.
ANGIOMYOLIPOMAS

 Angiomyolipomas (AMLs) are benign tumors that contain vascular, smooth muscle, and adipose tissues
 less than 1% of renal tumors.
 most AMLs are asymptomatic, but can lead to symptoms and renal failure with a higher rate in adulthood
 Presenting symptoms include an abdominal mass, urinary infections, hematuria, hypertension, and secondary
infections
 surgery is the
 treatment of choice and includes nephron-sparing surgery
 Prognosis is excelent
RENAL MEDULLARY CARCINOMA

 Renal medullary carcinoma (RMC) is a rare and aggressive type of renal cancer that primarily affects young adults
 (11–40 years of age)
 median survival was 8 months