Case 1 : Tall, Obese , Virilized Girl
History:
KJ 4 year and 9 month old female, brought by parents with complaints of rapid gain
in weight for last 6 months and development of pubic hair for last 8 months. Born of a
non-consanguineous marriage, KJ had a normal birth and developmental history.
There was history of headaches, acne and blackening over the neck. No history of
back-aches or muscle weakness. No history of steroid or any other drug ingestion.
Examination :
Height 113.5cm [above 97th percentile], weight 27.9kg [above 97th percentile], BMI
20.88kg/m2 [WHO Z-score between +2 and +3] and weight for height [WHO Z score
+3]. BP 140/90mmHg. Her face was plethoric, had acne, with acanthosis over the
neck. She had axillary hair and pubic hair stage 4 with clitoromegaly. These were no
mucosal pigmentation or cafe-au-lait spots and striae were absent.
Investigations:
8 AM cortisol was 33mcg/dL, Overnight Dexamethasone suppresion test was
unsuppressible at 28mcg/dL. Testosterone was 166ng/dL. CT abdomen showed a
left adrenal mass 9.7x8.5x6.5cm, calcification within, extending inferomedially along
left paraaortic upto renal hilum and encasing renal vein. Chest and Liver were
normal.
ClInical Diagnosis: Cushing’s Syndrome due to Adrenal Tumour, most likely
carcinoma
Management: Blood pressure was controlled with Amlodepin with Spironolactone.
She was started on ketoconazole 200mg BD. Got operated for adrenalectomy with
nephrectomy as the mass was adhering the kidney.There was a rupture of the tumor
intraoperatively but a lavage was given. Patient was started with physiological doses
of hydrocortisone post operatively. Biopsy histopath examination showed high grade
adrenocortical carcinoma, <20 mitotic figures per 50hpf with foci of necrosis and
calcification, absence of vascular invasion but presence of capsular invasion.
Final Diagnosis: Adrenocartical Carcinoma
Follow-up: She was referred to onchlogist for chemotherapy but patient lost follow
up for next 5 months. She was admitted with unconsciousness with convulsions at 6
months post operatively. She was stabilized with anti-hypertensive management but
was diagnosed to have local recurrence as well as metastasis in the chest. Her
parents then chose for palliative care. KJ succumbed to death after another 2
months.