Abstract Ref Number = APCP851
RARE ENDOCRINE TUMOR IN CHILDREN PRESENTING WITH THE SAME NEUROLOGICAL SYMPTOMS ADRENOCORTICAL CARCINOMA AND OVARIAN STEROID CELL TUMOR
Agatha Febrina Christie,Setyo Handryastuti,Bambang Tridjaja,Aman Bhakti Pulungan,Ahmad Rafli
Faculty of Medicine Universitas Indonesia Cipto Mangunkusumo Hospital
Background : Tumor in the adrenal glands can cause ACTH-independent Cushing’s syndrome (CS). Adrenocortical carcinoma (AC) is a very rare tumor in pediatric, less than 0.2% of all pediatric tumors. A tumor located far from the adrenal area may synthesize adrenocortical hormones, mimicking perfectly adrenal secretion (cortisol) causing CS with metastases are exceptional.
Case Presentation Summary : We report two cases with loss of consciousness as a chief complaint. The first case, a 3-year-old boy with decreased of consciousness and history of tonic-clonic seizure three months before admission. Physical examination noted somnolence, cortical blindness, malignant hypertension, precocious puberty, and Cushingoid appearance. Laboratory result of cortisol were 33.53 (morning) and 31.7 ng/dL (afternoon). Head CT-scan showed subgaleal hematoma on right temporoparietal with subfalcine herniation, right parietal intracranial bleeding, and right lateral intraventricular bleeding. Adrenocortical carcinoma was confirmed by abdominal MRI with solid lobulated mass left adrenal (4.8x3.9x3.1 cm). Postoperative he was subjected to adjuvant chemotherapy but unfortunately the patient passed away. The second case, a 2-year-old girl with 3 weeks of decreased consciousness. She was somnolent, manifesting hypertension, hirsutism, Cushingoid appearance, and clitoromegaly with cortisol level of 26.6 ng/dL (morning). Abdominal CT-scan revealed 4.7x3.7x4.9 cm solid mass in the right lower abdomen, well-enhanced, with left uterine artery as tumor feeding-artery. Head MRI showed multiple bleeding lesions at right temporo-parieto-occipital lobes, left occipital lobes, and left posterior subfalcine hernia. She was diagnosed with ovarian steroid cell tumor and underwent salphingoopherectomy. Currently, she is under follow-up without notable problems.
Learning Points/Discussion : Loss of consciousness accompanied with hypertension is not merely a neurological problem but should be a warning to pediatrician of rare endocrine tumor.
Keywords: adrenocortical carcinoma steroid cell children