Case description:
A 6year old male, presented with left flank swelling and excessive facial, axillary and pubic hair, increased appetite and weight gain for 2 months. On examination he had hyper tension, flank mass and virilization. Abdomen and chest CT was suggestive of a left sided adrenal mass with calcification and pulmonary metastasis. Serum dehydroepiandrosterone level was > 1500.0 ug/dL leading to the diagnosis of adrenocortical carcinoma. Due to upfront un-resectability he was started on mitotane and CED (Cisplatin-Etoposide-Doxorubicin) chemotherapy.
Two weeks after starting chemotherapy he had multiple episodes of left focal seizure with secondary generalisation. Patient also had behavioural alterations, frightfulness, deterioration of sensorium, dystonia and overall increased extremity tones. MRI brain showed focal areas of hyperintensity and diffusion restriction in thalami, posterior parietal and subcortical and insular cortex. FDG-PET of the brain was suggestive of hypermetabolism in frontal-temporal lobe and thalami with hypometabolism in occipital lobes (Fig-1). EEG was suggestive of left hemispheric near continuous discharges with occipital predominance (Fig-2).
The patient was started on antiepileptics bud had poor responses in EEG. Ultimately the patient went on to require five antiepileptics: valproate, levetiracetam, topiramate, lacosamide and lorazepam. Initial cerebrospinal fluid (CSF) analysis was suggestive of neutrophilic pleocytosis, and was sent for both infective and autoimmune panel. All the CSF workup including biochemistry, bacterial, tubercular, viral, parasitic and autoimmune panel were negative. With the clinical possibility of seronegative autoimmune encephalitis, child received IVIG and pulse steroids. Child showed neurological improvement in the form of improved sensorium, normalization of behaviour and seizure control. Unfortunately, interim response was suggestive of progression of ACC at primary and metastatic site, hence parents opted for palliation.