Dr.Venkatesh Kumar.M,Dr. Pavithran(Medical Oncology), Dr.Praveena, Dr.C.Jayakumar, AIMS, Kochi.
Four years old male 3rd child developmentally normal and immunised from Maldives at the age of 2 years is noticed to have a swelling over his abdomen incidentally the mother The swelling was initially in the abdomen but has progressively increased in size and they have noticed more prominence
Historyof UTI 1 month back and was treated symptomatically.At Mali hospital Labs done showed
LDH272 and CECT of Abdomen and Chest done showed large round lobulated enhancing heterogenous mass lesion with necrotic areas centrally at right lobe of liver(8.7 x 6.1 x 7.7 cm in size) .
Few foci of calcification noted at the centre. The mass is abutting the right portal vein and head of pancreas posteriorly, lesser curvature of stomach left laterally and anterior rectus muscle suggestive of Hepatoblastoma.
AFP tested at that time was more than 60,000.
He was then referred to AIMS for further management.
Mother is on treatment for Leukemia.
At examination,vitals stable. General examination was within normal limits. Systemic examination was unremarkable. Auxology revealed normal growth parameters.
Differential diagnosis conisdered at this point are:
1.Neuroblastoma
2.Nephroblastoma
3.Hodgkins and Non-Hodgkins Leukemia
4.Hepatoblastoma
5.Infantile hemangioma
6.Focal Nodular hyperplasia
Investigations:
Hemogram: TC- 11.87 ku/ml, N- 33.3 %, L-55.3 %, Hb- 10.1 gm/dl, Plt- 589 ku/ml.
LFT: SGOT- 42.4 IU/L, SGPT- 14.1 IU/L, ALP- 179 IU/L, T.Protein- 7.7 gm/dl, S.Albumin- 3.9 gm/dl, S.Globulin- 3.79 gm/dl, T.Bilirubin- 0.30 mg/dl, D.Bilirubin – 0.06 mg/dl.
RFT: Urea- 24 mg/dl, Creat- 0.28 mg/dl.
Electrolytes: Na+:131.2 mmol/L, K+:4.5 mmol/L, Magnesium:1.9 mg/dl, Calcium: 9.84 mg/dl
CRP: 7.13 mg/L,S.Iron: 23.2 mcg/dl, S.Ferritin: 50.98 ng/ml, TIBC : 392.3 mcg/dl
AFP: 300086 ng/ ml ( elevated )
USG Guided Liver biopsy: Shows Neoplasm with widened trabeculae and nodules of smaller cells with rossetting.
IHC was positive for Glypican, CK 19, Beta Catenin.
CT Chest Plain & Contrast: A small focal lung nodule seen in apicoposterior segment of left upper lobe – may represent possibility of metastasis.
MDCT Abdomen: A fairly large focal mass lesion involving left lobe of liver – in view of age and elevated AFP levels ,likely to represent Hepatoblastoma.
Impression: Hepatoblastoma ( Pretext stage III with extrahepatic disease )- high risk with lung metastasis.
Treatment given:
1.Chemotherapy therapy with Super-Plado arm( Alternate cycles of Cisplatin and Carboplatin + Doxorubicin X 7 cycles)
2.Hepatectomy (Left Lobectomy ,Lobe 2 and 3 resected)
3. Adjuvent Chemotherapy with Doxorubicin + Carboplatin.
Discussion:Hepatoblastoma is the most common liver tumour in children seen in first 4 years of life.
It is tumour of immature hepatocyte progenitor cells. Survival rates have reached 80-90 percent. This rate depends upon staging at diagnosis. Patients with fully resected tumors have a greater than 85 percent chance of survival. The rate is about 60 percent for those with metastatic disease that responds to chemo.
It is anembryological tumour which recapitulates various stages of liver development. Histological types are epithelial type and Mixed epithelial and mesenchymal type.
Clinically detected as abdominal lump the parent or clinician.Investigations show elevated AFP and thrombocytosis. CT scan shows heterogenously enhancing well circumscribed lesion with occasional calcification. Biopsy is required for confirmation of diagnosis.Staging systems available are PRETEXT( Preoperative extent of tumour system) and COG(Children’s Oncology Group) classification.Multimodality treatment with surgery and chemotherapy (platinum based) are the mainstay of treatment.
Take home message: Any children presenting with abdominal lump should not be evaluated on superficial basis,detailedevaluation including AFP,CT abdomen should be done to rule out Hepatoblastoma so that increasing the survival rate and prognosis in children.