A 51-year-old Portuguese female visited A&E with a headache and vomiting. She had a transurethral resection for superficial bladder cancer 2 years ago and a pacemaker for mobitz-type-2 heartblock. She had a 35-pack-year smoking history and drank 10 units of alcohol per week. Over the preceding 3 weeks she had several hospital attendances with epigastric pain and vomiting. Investigations had been normal:she was diagnosed with gastroenteritis and discharged.
This occasion, she reported ongoing epigastric pain, vomiting and new postural headaches associated with neck pain and photophobia. She had noticed progressive vision loss and unsteady gait. She denied fevers or weight-loss. On examination her GCS was 15, she had bilateral papilloedema and visual acuity reduced to hand-movement on the left. The rest of the cranial nerves III-XII and peripheral neurological examination were unremarkable, apart from an ataxic gait. She had normal observations, blood tests and x-rays. CT head and lumbar puncture were performed and she was commenced on antibiotics and antivirals to cover infective meningitis.
CT head revealed a contrast-enhancing lesion in the left pre-pontine region, likely to be a trigeminal schwannoma or metastatic deposit (Fig. 1). Lumbar puncture found clear CSF, normal opening pressures, WCC 4, raised protein 2.51 and low glucose 0.3.
Since the CT findings did not explain the clinical picture, an MRI head was recommended. This had to be performed at another trust since we did not have a pacemaker-compatible scanner.
The patient was reviewed by neurology, infectious-diseases, ophthalmology and microbiology. Differentials included infective (particularly TB and fungal), inflammatory and neoplastic diseases. CT-venography showed no evidence of venous sinus thrombosis. Further tests included: B12/folate, LDH, ESR, hormones, ACE, immunoglobulins, complement, autoimmune and porphyria screen, tumour markers, myeloma-screen, TB ellipspot, hepatitis, HIV, CMV, cryptococcal, aspergillus, toxoplasmosis and Borrelia burgdorferi. All results were unremarkable.
Further CSF results revealed no bacterial, acid-fast-bacilli or fungal growth, viral and TB PCR were negative. CSF cytology showed malignant cells (Fig. 2).
CT chest, abdomen and pelvis found a solitary 15 mm parenchymal lung nodule and a 5 mm endobronchial lesion in the right lower lobe.
In light of these findings; antimicrobials were stopped, dexamethasone plus a proton-pump-inhibitor started and an MRI spine, head and orbits requested. The working diagnosis was malignant meningitis of unknown primary cancer. Bronchoscopy confirmed an endobronchial tumour. The patient was transferred to oncology while awaiting histology.
Further examination found no lymphadenopathy, suspicious skin or ophthalmic lesions. She had left nipple inversion (longstanding), but no breast lumps or skin changes. An urgent breast clinic appointment and mammogram were arranged and ER/PR/HER-2 status requested on CSF.
During this time she deteriorated with confusion (GCS 14) and rapidly progressive neurology: tinnitus, hearing loss and bilateral proximal lower limb weakness (power 3-4/5). She lost 5 kg of weight in 2 weeks.
The patient became agitated and complained of severe headaches. She had decreased vision in the right eye and complete blindness in the left. She developed worsening leg weakness (power 2/5) and urinary incontinence. Detailed neurological examination became difficult due to confusion and agitation. Morphine and Midazolam were started for comfort.
On Day 10 her GCS decreased to 12 and symptoms progressed further. Despite best efforts, she was too unwell to attend the MRI appointment. Histopathological analysis revealed melanoma (cells expressed: S100p, Melan A and HMB45 and were immunonegative for: AE1/3, p63 and TTF1) (Figs 3-5). Given her poor performance-status and rapid decline, the patient was not fit for any cancer treatment. She was transferred to a hospice on Day 12.