Background
Primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD) is a rare complication following solid organ transplantation (SOT). With increasing rates of SOT, PCNS-PTLD incidence is increasing.
Objective
Describe the characteristics of PCNS-PTLD patients requiring neurosurgical intervention.
Methods
From 2000-2011 ten patients with prior SOT underwent biopsy for evaluation of brain lesions and were diagnosed with PCNS-PTLD. Data collected included imaging characteristics, pathology, treatments administered, and survival outcomes.
Results
All patients had kidney transplantation, and 3 had concurrent pancreas transplantation. Median age at diagnosis was 49 years, with a median of 4.5 years from SOT to diagnosis (range 1.8-11.4 years). Presenting symptoms most often included focal neurologic deficits (n=6), although several patients had non-specific symptoms of headache and altered mental status. Brain lesions were generally multiple (n=7), supratentorial (n=8), and lobar or periventricular in distribution with ring-enhancement. Diagnosis was established by stereotactic (n=4) and open surgical biopsy (n=6). Treatments most frequently administered included reduction of immunosuppression (n=10), dexamethasone (n=10), rituximab (n=8), high-dose methotrexate (n=3), and whole-brain radiotherapy (n=6). Six patients remain alive without PCNS-PTLD relapse, including 4 patients who have sustained remissions beyond 2 years from diagnosis of PCNS-PTLD. Of 4 observed deaths, 1 was related to progressive PCNS-PTLD.
Conclusion
PCNS-PTLD must be considered in the differential diagnosis of any patient with prior SOT presenting with an intracranial lesion. Histologic diagnosis with brain biopsy is imperative given the risk for opportunistic infections that may have similar imaging findings and presentation. Prognosis is variable, although long-term survival has been reported.
Diseases of the cerebral vasculature represent a heterogeneous group of ischemic and hemorrhagic etiologies, which often manifest clinically as an acute neurologic deficit also known as stroke or less commonly with symptoms such as headache or seizures. Stroke is the fourth leading cause of death and is a leading cause of serious long-term disability in the United States. Eighty-seven percent of strokes are ischemic, 10% are due to intracerebral hemorrhage, and 3% are secondary to subarachnoid hemorrhage. The past two decades have seen significant developments in the screening, diagnosis, and treatment of ischemic and hemorrhagic causes of stroke with advancements in CT and MRI technology and novel treatment devices and techniques. Multiple different imaging modalities can be used in the evaluation of cerebrovascular disease. The different imaging modalities all have their own niches and their own advantages and disadvantages in the evaluation of cerebrovascular disease. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
Noncutaneous primary melanomas are a complex group of malignancies with biologic findings that are unique when compared with findings for cutaneous melanomas. Each noncutaneous primary melanoma has its own specific diagnostic and management challenges, depending on the anatomic location where they arise.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.