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.