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Paediatric abdominal masses

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  • Neuroblastoma is mIBG avid
  • Benign renal massesMesoblasticnephromaMultilocular cystic nephromaAngiomyolipoma
  • NephroblastomatosisSmall islands of primitive metanephricblastema, which are precursor of Wilms tumor, commonly exist in the kidneys of the normal newborn infant. These primitive cells usually spontaneously regress by 4 months of age. A diffuse and proliferative form of persistent renal blastoma is referred to as nephroblastomatosis. The abnormal tissue can form as multiple discrete nodules within the renal parenchyma or may completely replace the renal cortex. -Nephroblastomatosis appears on CT or IV pyelogram as bilateral lobulated and enlarged kidneys with marked compression, stretching, and distortion of the pelvicaliceal structures On US, the kidneys are enlarged, lobular, andechogenic, or enlarged with diffuse hypoechoic thickening of the cortex. In such cases, Wilms tumor should be suspected. Nephrogenic rests are more likely to appear cortical, tend to be homogeneous, and are of low echogenicity on US, low attenuation on CT, and low signal intensity on T1WIs . Small, focal nephrogenic rests smaller than 1 cm are difficult to visualize by US and are better evaluated with contrast-enhanced CT or T1WI
  • Sagittal image of the right kidney A rounded solid appearing mass is seen in the interpolar region of the right kidney. The mass shows no obvious calcifications.
  • Sagittal image of the right kidney The right kidney appears enlarged and lobulated. The complex right renal mass is appreciated again in this image. The mass exhibits a few scattered areas that are hypoechoic
  • Contrast enhanced axial CT scan The right renal mass is again identified in this image. The mass is seen to occupy a large portion of the kidney and invades the right renal hilum; however it does not cross the midline.
  • Wilm's Tumor. Conventional radiograph of abdomen shows a large right upper mass displacingbowel loops into the pelvis and accross the midline (blue arrows). Coronal and sagittal reformatted CTdemonstrate a large, heterogeneous mass arising from the right kidney (white arrows). A smallsliver of functioning right kidney is seen to enhance (yellow arrow).
  • Adrenal HemorrhageThe infants may present with an abdominal mass, jaundice, hypotension, or anemia, but small hemorrhages may go unnoticed. Hemorrhage occurs more frequently on the right and is occasionally bilateral. Older children develop adrenal hemorrhage as a result of accidental trauma, child abuse, meningococcemia, or anticoagulant therapyHemorrhage enlarges the gland and causes loss of the V shape. Initially, the hematoma resembles a solid, echogenic mass . As the hemorrhage resolves, it becomes increasingly hypoechoic, starting in the central region and progressing peripherally .
  • hypo-echoic hematoma in the right adrenalAdrenal hemorrhage longitudinalAdrenal hemorrhage without vascularity longitudinal
  • The etiology of neuroblastoma is not well understood. The great majority of cases are sporadic and non-familial.detect neuroblastoma is the mIBG scan (meta-iodobenzylguanidine), which is taken up by 90 to 95% of all neuroblastomas, often termed "mIBG-avid
  • Tc-99m MDP (Bone Scan)MIBG scintiscan
  • A well-defined, rounded, mildly lobulated, hypoechoic mass that is separate from the liver is noted in the right upper quadrant. The mass has areas of calcification and appears to displace the kidney downwards and medially.
  • They usually present with an abdominal massHepatoblastomas originate from immature liver precursor cells, usually unifocal and affect the right lobe of the liver more often than the left lobe, can metastasize.Surgical resection is the usual manner in which material for pathologic assessment is obtained. Open biopsy is performed when complete surgical resection is not possible. Needle biopsy is not recommended, because hepatoblastomas usually are highly vascular.
  • Precontrast CT scan pre shows a large mass encompassing both lobes of the liver, which is of lower density than normal liver. CECT demonstrates mass that appears to be multinodular, with heterogeneous enhancement less than that of the surrounding liver
  • Metastatic DiseaseNeuroblastoma is the most common childhood tumor to metastasize to the liver, followed by lymphoma, leukemia, and Wilms tumor. Metastatic lesions are usually multiple, and their imaging appearance is generally nonspecific
  • Echogenic nodular lesion in segment 7 of liver in a known case of RCC, represents metastatic nodule.
  • Liver metastases, contrast-enhanced CT. Multiple hypoattenuating lesions of varying sizes are seenin both the left and right lobes of the liver, some with indistinct margins
  • Hemangioendothelioma is a benign endothelial lined vascular mass of the liver seen in infants. It should be suspected in an infant with a large liver mass and congestive heart failure (CHF) or cutaneous hemangiomas.Clinical ManifestationInfantile hemangioendotheliomas are generally noticed within the first few days to months of life. They typically present with a palpable upper abdominal mass with bruit, abdominal distension, hepatomegaly, and high output CHF secondary to arteriovenous shunting. Fifty percent of infants have cutaneous hemangiomas. Hemangioendothelioma can be detected in utero as early as 16 weeks of gestation and is associated with polyhydramnios.
  • Axial CT scan post-contrast arterial phase at the level of the kidneys showed diffused hepatic masses with intense peripheral enhancement and central non-enhancing areas, B) Venous phase shows diffused enhancement of the lobulated liver segments. Axial unenhanced T1 weighted MRI image shows diffused nodules with low signal intensity, B) Coronal T2 weighted MRI image shows diffuse high signal intensity nodules occupying almost the entire abdomen.
  • Choledochal cysts are congenital malformations of the intrahepatic or extrahepatic bile ducts. majority of cysts are associated with an anomalous junction of the common bile duct and pancreatic duct Jaundice, pain, and a right upper quadrant mass comprise the classic triad of findings seen with a choledochal cyst. The most common type of choledochal cyst (type 1) is a localized, fusiform or saccular dilation of the common bile duct below the cystic duct.
  • These patients also have a normal pancreaticobiliary junction.
  • Ultrasound images reveal a large cystic collection with thickened and shaggy walls, located anterior to left kidney and in close relation to tail and body of the pancreas. These ultrasound findings suggest a diagnosis of pseudocyst of the pancreas
  • Splenomegaly is a relatively common cause of a left upper quadrant mass in children.
  • Situsambiguus, also known as heterotaxy is a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen.The normal position of the organs is known as situssolitus; situsinversusis a condition in which the usual positions of the organs are reversed from left to right as a mirror image of the normal conditionabsent or defective splenic function is associated with a high risk of fulminant bacterial infections, especially with encapsulated bacteria
  • Left IVC and multiple spleens polysplenia, azygous continuation of the IVC and retroperitoneal hepatic flexure (note no situsinvertus
  • 15-year-old male with Burkitt's lymphoma. CT with contrast. (A) The bulky heterogeneousmassBurkitt lymphoma in a 4 year-old boy infiltrating both kidneys, liver and spleen. a) Axial contrast enhanced CT reveals a homogeneous hypodense nodular lesion in the left kidney (black arrow) and another one that almost replaces the right kidney (yellow arrow). b) Two months later, after chemotherapy, the left kidney lesion was unapparent and the lesion in the right kidney shrunken significantly
  • The transverse ultrasound image of the pelvis showed a soft tissue mass deep to the urinary bladder This was a prostatic rhabdomyosarcoma.
  • Axial and coronal CT images below show a complex soft tissue mass, arising from the prostate gland
  • Ovarian Teratomacomplex cystic and solid pelvic mass containing two teeth and fat
  • Contrast-enhanced coronal CT images of the abdomen and pelvis, demonstrate a large predominantly cystic left adnexal mass containing multiple septations almost completely occupying the abdomen (black arrow). The twisted vascular pedicle in the left lower quadrant demonstrates a whirlpool sign (white arrows)
  • Claw signIt refers to the sharp angles on either side of the mass which the surrounding normal parenchyma forms when the mass has arisen from the parenchyma. As such, normal parenchyma extends some way around the mass. Examples where a claw sign is useful include:meningioma vsintraparenchymal brain tumourWilms tumour vsneuroblastomarenal angiomyolipomavs retroperitoneal liposarcoma

Transcript

  • 1. Paediatric Abdominal Masses Dr.Aftab Qadir
  • 2. 1.Renal Masses 2.Adrenal 3.Hepatobiliary 4.Pancreatic 5.Spenic 6.Gastrointestinal 7.Pelvic
  • 3. Imaging plays an important role in the diagnosis and management. Plain radiographs provide clues to the location of the mass,organomegaly and the presence of calcifications. Ultrasound is generally the most valuable procedure for the initial evaluation. Ultrasound differentiates cystic from solid masses, indicates the organ of origin, and suggests the diagnosis, also about the vascular compromise and intraluminal thrombosis. CT or MR needed when the mass is large, poorly defined, for staging or when obscured by bowel gas. Radionuclide for specific applications
  • 4. Renal Masses Congenital Pevicalyceal dilatation Infection Neoplasm Vascular Multicystic Dysplastic kidney Abscess Malignant: Wilm’s tumour Renal vein thrombosis PUJ obstruction Focal nephritis Benign Haematoma Cystic renal disease Xanthogranulomat ous pyelonephritis
  • 5. Wilm’s tumour • It arises from the primitive metanephric epithelium • Bilateral synchrous tumours occur in 5-10%. • Nephroblastomatosis as precursor • Increased incidence sporadic aniridia,hemihypertrophy,Beckwith- wiedemann syndorme,Drash syndrome,Horseshoe kidney,Family history.
  • 6. Presentation • Mostly present as asymptomatic mass • Abdominal pain • Haematuria • Fever • Hypertension
  • 7. Imaging • On ultrasound Wilms tumor characteristically is a well-defined, predominantly solid mass Appears as echogenic, heterogeneous with cystic areas. Wilms tumor has a propensity to extend into the renal vein, inferior vena cava, and right atrium • On CT Wilms tumours are heterogeneous soft- tissue density masses, rarely calcifications and enhancement is patchy. • On MRI, tumor tends to have decreased signal on T1 and increased on T2 • The lung is commonest site for metastases.
  • 8. Tumor staging Determined by imaging and surgical findings. • Stage 1:Encapsulated tumor completely excised. • Stage 2:Extends beyond the kidney, completely excised • Stage 3:Residual tumor confined to abdomen and nodes • Stage 4:Haemetogenous metastasis • Stage 5:Bilateral tumours at diagnosis 5 year survival exceeds 90% in those with early stage and favorable histology. A tumor biopsy is not typically performed due to the chance of upstaging the cancer and seeding the abdomen with malignant cells.
  • 9. Treatment • The main types of treatment that can be used for Wilm's tumor are: • Surgery • Chemotherapy • Radiation therapy Nephrectomy followed by chemotherapy • Postoperative radiation therapy for stage II and IV lesions
  • 10. • Renal cell carcinoma is very rare in young children but sometimes occurs in older children and adolescents • The imaging characteristics of renal cell carcinoma are indistinguishable from those of Wilm's tumor.
  • 11. Adrenal Masses Adrenal Hemorrhage Commonest cause of adrenal mass May be unilateral or bilateral Predisposing factors include large babies, obstetric trauma, neonatal sepsis, and hypoxia Ultrasound: demonstrate an avascular heterogeneous adrenal mass that has becomes cystic and smaller over the following weeks as clot retractions occur. Serial ultrasound to document the typical course of cystic changes and resolution, usually evolves from hyper echoic to iso-echoic to hypo-echoic.
  • 12. Neuroblastoma • Malignant tumor of neural crest cells • Commonest extracranial solid malignant tumor • Approximately 70% originate in the abdomen of which 2/3rd arise in adrenal,20% in the chest and 10% in the head and neck
  • 13. • May present as palpable abdominal mass or non specific symptoms • 50 to 60% of all neuroblastoma cases present with metastases
  • 14. Radiological imaging • Ultrasound demonstrates a hyperechoic mass in the adrenal or central retroperitonuem often with flecks of calcification. Doppler for flow in encased vessels • CT confirms the calcification with low attenuation mass. • The most characteristic imaging feature is the encasement of adjacent vessels • 99m Tc MDP scintigraphy is useful for detection of bone metastasis. • mIBG scan
  • 15. Staging Involve the radiological, surgical and bone marrow aspirate: • Stage 1:confined to organ of origin • Stage 2: extending beyond the organ of origin, unilateral nodal disease • Stage 3:extending across the midline, bilateral nodal disease • Stage 4: distant metastases • Stage 4s: age<1year,localized primary(stage 1 or 2 metastases to liver, skin and/or bone marrow)
  • 16. • Treatment options for neuroblastoma largely depend on the location and size of the tumor and usually involve multimodal therapy • 1.Surgery • 2.Radiation therapy • 3.Chemotherapy • Immunotherapy • Bone marrow transplant
  • 17. Hepatobiliary masses • Accounts for 6% of abdominal masses • 2/3rd are malignant and 1/3rd are benign. Hepatoblastoma Most common malignant hepatic tumor • Majority present under 2 years • No association with cirrhosis • Increased risk with beckwith wiedemann syndrome, affected siblings, familial polyposis coli and trisomy 18. usually present with an abdominal mass
  • 18. Imaging • On ultrasound single or multiple hyperechoic masses with distortion of the adjacent vascular architecture,hepatoblastomas usually are highly vascular • On CT heterogeneous low attenuation lesion are seen with areas of necrosis and hemorrhage and often containing coarse calcification • Vascular and tumor thrombus strongly suggest malignancy • Lung is the most frequent site of metastases
  • 19. Management • Chemotherapy • Radiotherapy • Resection and transplantation
  • 20. Liver metastases • Metastases to the liver are more common than primary hepatic tumours and most frequently associated with the neuroblastoma, Wilm's tumour, lymphoma and leukemia
  • 21. Ultrasound of liver metastases can have a variety of appearances • hypoechoic: most common ~ 65% • Hyperechoic • peripheral halo • Calcified • Cystic • poorly defined (infiltrative)
  • 22. Infantile haemangioendothelioma • common benign hepatic mass in newborn. May be multifocal or solitary.
  • 23. Imaging • Infantile haemangioendotheliomas have a variable sonographic appearance and may be either hypoechoic or hyperechoic or may have mixed echogenicity • Colour Doppler sonographic evaluation will show increased flow. • On CT enhancement is typical • On MRI lesions are low signal on T1 and high on T2 with large vascular signal voids
  • 24. Choledochal Cysts Congenital dilatations of the biliary tree • Most cause symptoms in childhood and adult life. There are four types: • Type 1A: Fusiform dilatation of the CBD below the cystic duct • Type 1B: Fusiform dilation of the common hepatic duct and CBD • Type 2: Eccentric diverticulum off the CBD • Type 3: Choledococele-Dilatation of the distal intramural portion of the CBD • Type 4: Caroli’s disease-saccular dilations of the hepatic bile ducts
  • 25. Complications include cholangitis, biliary calculi, pancreatitis and biliary cirrhosis. • On ultrasound or CT the biliary tree dilatation or cyst can be seen. • 99mTc-HIDA scinitraphy will show accumulation of tracer within the cyst. • Percutanous or endoscopic cholangiography and MRCP are helpful in preoperative planning.
  • 26. Pancreatic masses • Pancreatic pseudocysts occurring as the sequelae of previous pancreatitis or trauma • True epithelial line congenital pancreatic cysts are less common • Primary pancreatic tumours are rare in childhood including the pancreticoblastoma,papillary epithelial neoplasm and endocrine adenomas
  • 27. Splenic masses Causes of splenomegaly Infections Mononucleosis,tuberculosis,septicemia,typhoid,malaria Portal hypertension Haemolytic anemia Thalassaemia Haematological malignancies Leukemia,lymphoma Infiltrative disorders Gaucer’s,niemann-pick,Langerhans cell histiocystosis Collagen vascular disorders Rheumatoid arthritis CCF
  • 28. • Asplenia and polysplenia occurs as part of heterotaxy syndrome.(Situs ambiguus) • Asplenia is associated with right isomerism,malrotation and severe congenital cardiac defects with majority of infants dying in the first year of life • Polysplenia in which multiple well defined masses are found in the left upper quadrant. • Wandering spleen is ectopic location outside the left upper quadrant
  • 29. Wandering spleen
  • 30. Focal splenic lesions • Splenic cysts may be congenital or acquired secondary to trauma, infarction or hydatid disease. • Benign focal splenic masses include haemangiomas,lymphangiomas, AVM and hamartomas
  • 31. Gastrointestinal Masses • Duplication cysts and mesenteric/omental cysts • Commonest malignancy is Burkitt’s non Hodgkin’s lymphoma ▫ Ill defined mass of adherent bowel loops with infiltration of the adjacent mesentery and with lymphadenopathy
  • 32. Pelvic masses • Rhabdomyosarcoma • Sacrococcygeal teratomas • Ovarian masses • Pelvic inflammatory disease
  • 33. Rhabdomyosarcoma • Commonest pediatric soft tissue sarcoma • Pelvis most frequent site of origin • In boys mostly arises from the prostate or bladder base • In girls from the urinary bladder, uterus or vagina
  • 34. • These are aggressive tumors • Invasion of adjacent viscera and pelvic wall • Distant spread to lymph nodes, lung and bone
  • 35. Ultrasound • shows heterogeneous well-defined irregular mass of low to medium echogenicity CT • soft tissue density • some enhancement with contrast • adjacent bony destruction seen in over 20% of cases
  • 36. Ovarian tumors • 65% are benign and 35% are malignant • Ovarian teratomas usually present at puberty and 90% are benign • The majority of ovarian malignancies are germ cell tumour • Most are large >15cm at presentation
  • 37. Serology • CA-125 levels - elevated in most ovarian malignancies (~ 80% in general) • AFP levels - elevated particularly with immature ovarian teratomas (~ 50% of cases) and ovarian yolk sac tumours
  • 38. Imaging • On ultrasound solid and cystic component can be demonstrated • CT can demonstrate the solid-cystic mass with fat and calcific components • It is not possible to determine whether a mass is benign or malignant unless local invasion or distant metastasis is present on imaging
  • 39. Adnexal torsion • Can occur at any age but is most frequent in the first two decades of life. The affected ovary may be normal or containing a cyst or tumour. • Patients present with acute lower abdominal pain and vomiting. • Ultrasound demonstrate an enlarged swollen ovary with peripheral cysts and free fluid in the pouch of Douglas • Doppler signal is usually absent.
  • 40. Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth disorder: • macroglossia - most common clinical finding • omphalocoele • localised gigantism / macrosomia • hemihypertrophy • cardiac anomalies • pancreatic islet cell hyperplasia • organomegalies • nephromegaly • hepatosplenomegaly
  • 41. Associations • Wilms tumour • Neuroblastoma • Hepatoblastoma • Rhabdomyosarcoma
  • 42. •Thank You