Background
Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor of the skin. It is associated with advanced age, ultraviolet (UV) radiation, and Merkel cell polyomavirus. It has a predilection for the lymphatic system, but rarely spreads to the central nervous system.
Case presentation
A 71-year-old Caucasian man with a history of rheumatoid arthritis and MCC of the right lower eyelid and cheek presented with left-sided hemineglect and word-finding difficulty. Twenty months earlier he had undergone local excision of a 3 cm lesion with negative margins, negative sentinel lymph node biopsy, and external beam radiation. On presentation he was found to have a 6.3 cm mass in the right frontotemporal region. He underwent prompt resection, with pathological analysis consistent with metastatic MCC. He subsequently underwent stereotactic radiosurgery (SRS) and adjunctive immunotherapy with pembrolizumab. He has since tolerated the therapy well and is currently without neurological symptoms or evidence of recurrence.
Conclusions
Cerebral metastasis of MCC is a rare event and should be considered when a patient with a history of MCC presents with neurological symptoms. Optimal treatment regimens of these rare cases are unclear; however, prompt resection, stereotactic radiosurgery, and adjunctive immunotherapy have shown an initial positive response in this patient.
Introduction:
Current guidelines recommend prophylactic implantable cardioverter-defibrillator (ICD) for patients with an ejection fraction <35%. Whether this applies to patients with transposition of the great arteries (TGA) and ≥moderately depressed systemic ventricular dysfunction is uncertain.
Methods:
From an electrophysiologic database at a large, tertiary care medical center, we identified 71 patients with TGA (40 D-TGA and 31 L-TGA) receiving an implantable cardiac device from 1996-2014. Electronic and paper medical records were fully reviewed and device interrogation data was collected to assess the frequency of sustained ventricular arrhythmia.
Results:
Patients were 31±15 years of age, 32% were women, 53% had ≥moderate systemic ventricular dysfunction and 38% had ≥moderate systemic atrioventricular valve regurgitation. Sixteen (23%) had an ICD placed initially. Of the 55 with a pacemaker as their first device 9 were later upgraded to an ICD for primary prevention of sudden death and 1 patient had sustained arrhythmia and an ICD was placed afterwards. Thus, of the 26 patients who eventually underwent ICD implant, 20 received it for primary prevention and 6 for secondary prevention. Interrogation follow-up data spanned 214 patient-years for primary prevention and 41 patient-years for secondary prevention. Only 2 patients received ICD discharges in the primary prevention group and both were inappropriate. In the secondary prevention group, 67% of patients underwent appropriate ICD discharge (0.39 shocks per patient-year, p<0.001 compared to primary prevention). Patients with ICD discharge were older (49±5 vs. 30±15 years, p=0.011) and more likely to have concomitant heart defects (100% vs. 30%, p=0.011).
Conclusion:
Patients with TGA and sustained ventricular arrhythmia are at high risk for subsequent events and benefit from ICD implantation. The role of prophylactic ICD implant in this patient population, however, remains uncertain.
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