Adrenocortical carcinoma

1. CASE PRESENTATION Dr.YazeedOwiwi Pediatric Surgery Department FCPS-II Trainee, PGR-III

2. CASE HISTORY 20th Dec, 2010 Saira - 20 months old girl Hirsutism - Moon face- Central obesity - BMI 21.6 >95thcentile -

7. BMI HEIGHT 76 CM WIGHT 12.5 KG

9. Dexamethasonesuppresion test* 24 hour urinary cortisol* * Plasma ACTH level * Ultrasound abdomen * CT Abdomen

10. Results Blood cp Serum electrolyte WNLUrea/Creatinine BSR LFTs Testosteron level 11.8 nmol/L (female: 0.3-3.0 nmol/L)

11. Serum Cortisol 8:00 am 14 ug/dl 8:00 pm 29 ug/dl Normal 2.3 -19.9 ug/dl ) ) Dexamethason Suppressive Test -ve

12. Plasma ACTH 22.20 pg/dl (Normal value 10-100 pg/dl) 24 Hrs urinary Cortisol 27.9 ug/dl ( (Normal value 43-176 ug/dl

13. Ultrasound Abdomen Right supra renal mass with evidance of focal calcification

14. CT scan Abdomen Well defined lobulated soft tissue mass lesion -- approximatly cm in right supra renal region. --Its arrising from right adrenal gland --Multiple calcific foci. --Suggestive of Neuroblastoma.

17. Further Investigation 24 hr urinary VMA 4.2 mg/24 hours (Normal value < 13.6)

18. Surgery were planned…..

19. Pre-operative manegment normal* Blood pressure. * Serum electrolyte, no hypokalemia * BSR, 89 mg/dl normal * Pt shifted to PICU prior to surgery. * 2 pints of blood crossed matched and prepared. * Pt NPO at 3 am on day of operation.

20. Peri-operative manegment * N/g tube placed. * 2 wide bore cannula placed. * Prophylactice antibiotic given. * Inj. Hydrocotisone 25mg given followed by 25mg/24 hour in infusion form. * Injhydralazin on hand.

21. Open Right Adrenalectomy

43. Post-operative manegment --Tissue biopsy sent for histopathology. --N/G tube removed 1st post-op day. --Inj. Hydrocortisone given in tapering form. --Switch to oral coticosteroid 7th post-op day. --Stitch removed at 10th post op day.

44. Histopathology Adrenal Cortical Neoplasm There is marked pleomorphism and scattered mitosis. However to establish malignant nature of the lesion, the evidence of metastasis is mandatory !

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