Assistant lecturer of Pediatrics

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Presentation transcript:

Assistant lecturer of Pediatrics CASE PRESENTATION BY AMANY IBRAHIM Assistant lecturer of Pediatrics CAIRO UNIVERSITY

Female patient 6yrs old with a history of progressive weight gain and increasing hair growth of 3 months duration. On examination She looks depressed. She had moon plethoric face. Buffalo hump, striae rubra. Hirsuite, pubic hairs ( P4), acne, mild cliteromegaly. Her height was 123.5 (on 97th centile = +2 SDS). Weight was 29.5 kg (above 97th centile = +3 SDS). BMI 19.3 (97th centile). BP: 140/70 on different occasions. Large firm mass in the right hypochondrium.

Based on the above data, the mass detected could be : Pheochromocytoma. Hepatocellular carcinoma. Adrenal tumor.

January 2002 April 2002

Investigations S.cortisol ACTH Free urinary Free urinary cortisol 287ug /24hr(N 20-80) S.cortisol ACTH Free urinary (ug/dl) (pg /ml) (ug/ml) am 50 (N 6-16) 3.7 (N<82) 280 (N 20-80) pm 37 (N 3-11) 2.0 (N<82) LDDS 36 3 HDDS 37 2.2 180 DHEAS 456u/dl (N 5-55u/dl). Testosterone 7.2 ng/dl (N <0.5ng/dl). Na, K & PRA are normal.

Based on the above data, this patient suffers from: Ectopic ACTH syndrome. Cushing’s disease. Adrenal tumor.

WHAT WOULD YOU DO NEXT?

U/S & CT a large retroperitoneal mostly right adrenal mass.

ADRENAL TUMORS Two main characteristics of adrenal tumors: They are autonomously secreting hormones. They are unresponsive to manipulation of the hypothalamic pituitary adrenal axis with pharmacological agents. Two main types of adrenal tumors: Adenomas: present alone with manifestations of glucocorticoid excess (Cushing syndrome) with no manifestations of androgen excess. Carcinomas: secrete multiple adrenocortical steroids (cortisol and androgen) even androgens in excess.

Suggested treatment modality for this case: Surgical excision only. Chemotherapy only. Radiotherapy only. Combined surgery, chemotherapy and radiotherapy.

WHAT WOULD YOU DO NEXT?

PREOPERATIVELY The patient received propranolol to control her blood pressure. She had done an MRI abdomen, chest and bone scan to exclude metastasis. All were normal, apart from adrenal mass.

TREATMENT Unilateral right adrenalectomy (adrenal carcinoma) followed by chemotherapy.

POSTOPERATIVELY She received steroid immediately after the operation and for 2 months, then withdrawn successfully. She received mitotane followed by radiotherapy for with no recurrence. She is now 10 years old and she is performing a yearly follow up of an MRI abdomen and chest, and serum DHEA-s, testosterone, cortisol which are so far normal.

THANK YOU