Document Type: Case Reports
Dept. of Pathology, Melaka Manipal Medical College, Manipal University, Manipal, India
Dept. of Pathology, KMC International Centre, Manipal University, Manipal, India
Dept. of Paediatric Surgery, Kasturba Medical College and Hospital, Manipal University, Manipal, India
Nonfunctional adrenocortical carcinoma is an extremely rare malignant tumor in children. Unlike the functional tumor which is detected early due to its hormonal presentation, nonfunctional tumor is detected at a later stage. Here we report a case of a 10 year old girl who presented with abdominal mass and symptoms of short duration. No hypertension and cushingoid features were seen. Serum alpha-fetoprotein, urine vanillyl mandelic acid and homovanillic acid levels were not elevated. CT scan showed multiple pulmonary nodules suggestive of metastatic deposits. With gross and light microscopic findings differential diagnoses of hepatoblastoma, paraganglioma, renal cell carcinoma, adrenal cortical and medullary tumours were made. An array of immunohistochemical markers was done and the final diagnosis given was nonfunctional adrenocortical carcinoma with foci of osseous metaplasia.
- Patel VV, Shah DS, Raychaudhari CR, Patel KB. Giant non-functioning adrenocortical carcinoma: A rare childhood tumor. Indian J Med Paediatr Oncol 2010 Apr;31(2):65-8.
- Lele SM, Mittal BV, Vora IM, Kulkarni HS. Nonfunctioning adrenocortical carcinoma. J Postgrad Med 1992;38(3):145-7.
- Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003;169(1):5-11.
- Mayer SK, Oligny LL, Deal C, Yazbeck S, Gagne N, Blanchard H. Childhood adrenocortical tumors: case series and reevaluation of prognosis--a 24-year experience. J Pediatr Surg 1997;32(6):911-5.
- Budhwani K, Ghritlaharey R, Debbarma M. Adrenal cortex tumor in a six year girl- A case report and review of literature. Indian J Med Paediatr Oncol 2004;(25):71-5.
- Volante M, Buttigliero C, Greco E, Berruti A, Papotti M. Pathological and molecular features of adrenocortical carcinoma: an update. J Clin Pathol 2008;61(7):787-93.
- Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A. Weiss criteria in large adrenocortical tumors: a validation study. Indian J Pathol Microbiol 2010;53(2):222-6.
- Poian C, Carsote M, Baloescu R, Stanescu B, Petrescu R, Corneci C, et al. The adrenalectomy in rare endocrine tumors – 2 cases report. Jurnalul de Chirurgie, Iasi 201;6(1):47-53.