A 30-year-old male with no previous medical history presented to the endocrinology department in 2013 with loss of body hair. The clinical examination showed a loss of axillary and facial hair (beard). The general examination of the patient was unremarkable. In particular, the neurological exam was normal.
The endocrine workup showed decreased testosterone levels with inappropriate gonadotropin levels (testosterone: 0.18 ng/mL [3-12 ng/mL], FSH: 0.63 mUI/mL [1.3-11 mUI/mL], and LH: 0.59 mUI/mL [1.1-10 mUI/mL]) and a slightly elevated prolactin level (20.3 ng/mL [2-10 ng/mL]) concluding to an isolated hypogonadotropic hypogonadism (TSH: 0.99 muUI/mL [0.25-4.5 muUI/mL] and FT4: 16.11 pmol/L [9-24 pmol/L]). The 1 mug synacthen test revealed appropriate cortisol levels in response to the stimulation test.
The ophthalmologic examination showed preserved visual acuity and a normal fundus examination. MRI of the brain and sella showed a large suprasellar mass with cerebral and spinal leptomeningeal spread (Figures 1-3).
A lumbar puncture revealed no pathological findings. Polymerase Chain Reaction (PCR) evaluation for Mycobacterium tuberculosis in the cerebrospinal fluid (CSF) was negative. Tumor markers in the blood and the CSF were also negative. The converting enzyme dosage revealed normal levels, making the diagnosis of sarcoidosis unlikely. A thoracoabdominal-pelvic CT scan did not show any associated lesions.
The patient was referred to the neurosurgery department, and a biopsy of the arachnoid leaflet and the dura mater was taken. On exploration, the arachnoid was thick and blackish.
The histopathological examination was suggestive of a grade I meningeal pigmented melanocytoma.
An androgen replacement therapy was instaured, and then, a dopaminergic agonist (bromocriptine) was added due to the increase in prolactin levels reaching 50-60 ng/mL.
Total resection of the tumor, as well as the meningeal spread, was not possible. The patient refused any radiotherapy. Owing to the benign nature of the lesion and the absence of neurological or ophthalmological impairment, we opted for clinical and radiological monitoring. The patient was then lost to follow-up.
He consulted in 2020 for a sudden drop in visual acuity. The fundoscopy showed stage 3 papillary edema. The neurological exam of the patient was normal. An MRI was urgently done to reveal an increase in the size and number of leptomeningeal seeding complicated by obstructive hydrocephalus (Figure 4). The CT scan showed no abnormalities other than well-defined intraspinal masses (Figure 5). The patient was urgently rereferred to neurosurgery, and treatment with radiotherapy is currently being discussed.