Paediatric Abdominal Masses

Dr.Aftab Qadir

1.Renal Masses 2.Adrenal 3.Hepatobiliary 4.Pancreatic 5.Spenic 6.Gastrointestinal 7.Pelvic

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

Renal Masses
Congenital Pevicalyceal dilatation Infection Neoplasm Vascular

Multicystic Dysplastic kidney

Abscess

Malignant: Wilms tumour

Renal vein thrombosis

PUJ obstruction

Focal nephritis

Benign

Haematoma

Cystic renal disease

Xanthogranulomat ous pyelonephritis

Wilms tumour
It arises from the primitive metanephric epithelium Bilateral synchrous tumours occur in 5-10%. Nephroblastomatosis as precursor Increased incidence sporadic aniridia,hemihypertrophy,Beckwithwiedemann syndorme,Drash syndrome,Horseshoe kidney,Family history.

Presentation
Mostly present as asymptomatic mass Abdominal pain Haematuria Fever Hypertension

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 softtissue 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.

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.

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

 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.

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.

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

 May present as palpable abdominal mass or non specific symptoms 50 to 60% of all neuroblastoma cases present with metastases

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

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)

 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

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

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

Management Chemotherapy Radiotherapy Resection and transplantation

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

Ultrasound of liver metastases can have a variety of appearances hypoechoic: most common ~ 65% Hyperechoic peripheral halo Calcified Cystic poorly defined (infiltrative)

Infantile haemangioendothelioma
common benign hepatic mass in newborn. May be multifocal or solitary.

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

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: Carolis disease-saccular dilations of the hepatic bile ducts

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.

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

Splenic masses
Causes of splenomegaly
Infections Portal hypertension Haemolytic anemia Haematological malignancies Infiltrative disorders Collagen vascular disorders CCF Thalassaemia Leukemia,lymphoma Gaucers,niemann-pick,Langerhans cell histiocystosis Rheumatoid arthritis Mononucleosis,tuberculosis,septicemia,typhoid,malaria

 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

Wandering spleen

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

Gastrointestinal Masses
Duplication cysts and mesenteric/omental cysts Commonest malignancy is Burkitts non Hodgkins lymphoma
Ill defined mass of adherent bowel loops with infiltration of the adjacent mesentery and with lymphadenopathy

Pelvic masses
Rhabdomyosarcoma Sacrococcygeal teratomas Ovarian masses Pelvic inflammatory disease

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

 These are aggressive tumors Invasion of adjacent viscera and pelvic wall Distant spread to lymph nodes, lung and bone

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

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

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

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

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.

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

Associations
Wilms tumour Neuroblastoma Hepatoblastoma Rhabdomyosarcoma

 Thank You