The 62-year-old female patient's chronic lymphoid leukemia (CLL) was diagnosed 10 years earlier and was non-progressive under treatment. A lesion with the appearance of an atheroma was removed from the right upper arm 2 years before her admission to the Department of Neurology. The lesion recurred and was reoperated. Histology with immunohistochemistry confirmed the diagnosis MCC. Sentinel lymph node biopsy did not show infiltration from MCC, but progression of CLL. The patient later had right axillary block dissection which showed metastases from both CLL and MCC. Combined chemotherapy based on National Comprehensive Cancer Network (NCCN) guidelines was discontinued because of bone marrow depression. Postoperative radiotherapy was performed, and etoposide and cisplatin chemotherapy of CLL was continued. The patient's condition deteriorated. A right axillary soft tissue conglomerate mass infiltrated the right lateral thoracic wall and pathologic upper mediastinal and parasternal lymph nodes appeared. She was admitted to our Department of Neurology due to her first epileptic seizure (GM). The last chemotherapy session (vincristin) was administered 3 days earlier. The patient complained about headache during the week before admission.
General medical state: BP: 115/75 mmHg, HR: 75/min. No bruit over carotid or subclavian arteries. An oblique surgical scar with primary healing on the right upper arm. Liver edge at the costal margin. No other abnormality was found during physical examination.
Neurological status: Normally shaped skull. No meningeal signs. Intact cranial nerves. Symmetric, normal deep tendon reflexes. No pathologic reflexes. Intact motor and sensory system, and coordination. No aphasia or lobe syndrome. Normal vigilance.
Mental state: no abnormality was found.
Laboratory findings: WBC: 11.48 10*9/L; Neutrophils (%): 5.9, ratio of stab cells: 1%; Lymphocytes (%): 86.4%; Monocytes (%): 1.0%; Eosinophiles (%): 0.1%; Basophiles (%): 0.4%; RBC: 3.90 10*12/L; Hgb: 112 g/L; Hct: 0.335 L/L; Platelets: 85 10*9/L; Blood smear: Gumprecth's shadows.
Normal serum glucose, creatinine and electrolyte levels. Gamma-GT: 24 U/L; Alkaline phosphatase: 162 U/L; LDH: 1768 U/L; Amylase: 75 U/L; CRP: 1.59 mg/L.
Cranial CT: showed a 37x9 mm hypodensity in the right parietal region, at the posterior horn of the lateral ventricle, with marked cerebral and cerebellar satrophy.
Cranial MR (axial FLAIR, T2, sagittal 3D T1 (with and without contrast application): a solid epidural 2x2.8x5 cm mass, originating from the bone, in the right parietal region, showing homogeneously low signal intensity on T1-weighted images (Figure 1/a) and homogeneously high signal intensity on T2 and FLAIR images (Figure 1/b), with an intense contrast enhancement (mainly) in the peripheral zone, and smaller necrotic areas in the central zone. Moderate oedema around the mass is present, with higher apparent diffusion coefficient (ADC) value on the ADC images. A 6.2x3.2x1.3 cm mass, infiltrating the bone, with similar structure and contrast enhancement is seen in mainly the left frontal area (Figure 1/c-d). The mass shows higher ADC values in diffusion images. Lacunar encephalopathy, cerebral and cerebellar atrophy were also noted.
Lumbar cerebrospinal fluid (CSF): Clear and transparent; Cell count: 5 WBC/microliter, Total protein: 368 mg/L; CSF glucose: 3.8 mmol/L; Serum glucose (simultaneous): 7.5 mmol/L
CSF cytology: few monocytoid cells and leukocytes. No tumor cells are detected in the sample.
CSF immunology: intact blood-liquor barrier. Intrathecal IgG synthesis is not detected, but oligoclonal gammopathy (OGP) is found.
Flow cytometry of CSF did not reveal metastasis of MCC. CLL was only present in peripheral blood, where 50% of the cells showed a corresponding flow cytometry phenotype.
Based on the clinical picture, the patient was started on carbamazepine. She did not have further epileptic seizures, and did not develop any neurological deficit. During an oncology follow-up visit, the patient announced that she and her husband had decided to continue her treatment abroad. We have no knowledge of her since then.
The diagnosis at the time of discharge was: Chronic lymphoid leukemia, Merkel cell carcinoma, metastatic bone tumors of bilateral frontal bones and right parietal bone, symptomatic epilepsy, lacunar encephalopathy, cerebral and cerebellar atrophy.