Cholangiocarcinoma Metastasis to the Spine and Cranium.

Background: Cholangiocarcinoma (CCC), a rare tumor arising from the viscera, has a poor prognosis. Although CCC is prone to metastasis, spread to the cranium and spine is exceedingly rare. Treatment for metastatic disease is palliative, with total resection of the primary lesion the only cure. We describe a case of metastatic CCC to the spine and cranium treated with surgical resection. Case Report: A 61-year-old male with a history of hepatitis C with liver transplant and incidental discovery of CCC presented with gradually increasing back pain. Physical examination revealed a palpable nontender mass in the parieto-occipital area. Computed tomography survey of the spine and head revealed mixed sclerotic and lytic lesions of the T9, T11, L2, and L5 vertebral bodies, a lytic lesion on the T6 vertebral body, and a 1.4-cm lesion in the right occipital calvarium. The patient underwent right occipital craniotomy for excisional biopsy of the calvarial mass with gross total resection and immunohistochemical confirmation of CCC. The patient was started on gemcitabine chemotherapy and radiation therapy for spinal metastases. Three months later, the patient died from metastatic disease complications. Conclusion: To our knowledge, only 6 cases of cranial CCC have been reported, and only 2 reported mixed cranial/spinal involvement. We report a rare case of CCC metastasis to the spine and cranium that was treated with surgery, chemotherapy, and radiotherapy. CCC should be considered an exceedingly rare etiology with treatment options aimed solely at palliation. This case supplements the existing literature to inform medical and surgical decision-making.

Pleomorphic Xanthoastrocytoma of the Pineal Region in a Pediatric Patient With Neurofibromatosis Type 1.

Background: Pleomorphic xanthoastrocytoma (PXA) is a rare and often focal glioma that most commonly affects children and young adults. Lesions are preferentially supratentorial and superficial, although infratentorial masses have been described, along with occasional involvement of the leptomeninges. The World Health Organization (WHO) categorizes these tumors as grade II, with surgical resection carrying a favorable prognosis. However, these tumors may undergo malignant degeneration and must be identified for appropriate treatment and prognosis. PXA has been associated with neurofibromatosis type 1 (NF1), although it is not the classic phenotype of NF1. We present a novel report of PXA, atypically located in the pineal region of a patient with a history of NF1. Case Report: A 17-year-old male with a history of NF1 presented with 1 month of bifrontal headaches. Magnetic resonance imaging was significant for a heterogeneous tectal mass, suspicious for a glioma extending to the fourth ventricle and causing displacement of the cerebral aqueduct without obstructive hydrocephalus. Following an infratentorial-supracerebellar approach for tumor resection, histopathology confirmed a low-grade variable neoplasm consistent with PXA. Postoperative imaging confirmed gross total resection with no evidence of recurrence at 9 months postoperatively. Conclusion: To our knowledge, this case is the fifth report of pineal PXA and the first associated with NF1. Because PXA presents similarly to other NF1-related intracranial tumors, careful diagnosis via immunohistochemistry is imperative. Gross tumor resection is usually curative; however, PXA has the propensity to undergo malignant degeneration and may require adjuvant treatment.

Long-term outcomes of deep brain stimulation in severe Parkinson's disease utilizing UPDRS III and modified Hoehn and Yahr as a severity scale.

OBJECTIVES: Deep brain stimulation (DBS) is the surgical treatment of choice for moderate to severe Parkinson's Disease (PD). However, few studies have assessed its efficacy in severe PD as defined by the modified Hoehn and Yahr scale (HY). This study evaluates long-term and medication outcomes of DBS in severe PD. PATIENTS AND METHODS: We retrospectively collected the data of 15 patients from 2008 to 2014 with severe PD treated with DBS. Retrospective assessment with the modified Hoehn and Yahr scale and motor subset of the Unified Parkinson's Disease Rating Scale (UPDRS III) were used to objectively track severity and motor function improvement, respectively. Levodopa equivalence daily doses (LEDD), number of anti-PD medications and number of daily medication doses were used to measure improvements in medication burden. Data was evaluated using univariate analyses, one sample paired t-test, two sample paired t-test, and Wilcoxon signed-rank test. RESULTS: The mean post-operative follow-up was 44.63 months, average age at diagnosis and the average age at time of DBS was 51.3 years and 61.5 years, respectively, and the time from diagnosis to treatment was 13.2 years. Significant decreases were seen in UPDRS III scores (pre-op = 44.533; post-op = 26.13; p = 0.0094), LEDD (pre-op = 1679.34 mg; post-op = 837.48 mg; p = 0.0049), and number of daily doses (pre-op = 21.266; post-op 12.2; p = 0.0046). No significant decrease was seen in the number of anti-PD medications (pre-op = 3.8; post-op = 3.2; p = 0.16). CONCLUSION: Following DBS, severe PD patients demonstrated significant improvements in motor function and medication burden during long-term follow-up. We believe our results prove that DBS is efficacious in the management of severe PD, and that further research should follow to expand DBS criteria to include severe disease.

Comparison of elderly and young patient populations treated with deep brain stimulation for Parkinson's disease: long-term outcomes with up to 7 years of follow-up.

OBJECTIVE: Deep brain stimulation (DBS) is the procedure of choice for Parkinson's disease (PD). It has been used in PD patients younger than 70 years because of better perceived intra- and postoperative outcomes than in patients 70 years or older. However, previous studies with limited follow-up have demonstrated benefits associated with the treatment of elderly patients. This study aims to evaluate the long-term outcomes in elderly PD patients treated with DBS in comparison with a younger population. METHODS: PD patients treated with DBS at the authors' institution from 2008 to 2014 were divided into 2 groups: 1) elderly patients, defined as having an age at surgery ≥ 70 years, and 2) young patients, defined as those < 70 years at surgery. Functional and medical treatment outcomes were evaluated using the Unified Parkinson's Disease Rating Scale part III (UPDRS III), levodopa-equivalent daily dose (LEDD), number of daily doses, and number of anti-PD medications. Study outcomes were compared using univariate analyses, 1-sample paired t-tests, and 2-sample t-tests. RESULTS: A total of 151 patients were studied, of whom 24.5% were ≥ 70 years. The most common preoperative Hoehn and Yahr stages for both groups were 2 and 3. On average, elderly patients had more comorbidities at the time of surgery than their younger counterparts (1 vs 0, p = 0.0001) as well as a higher average LEDD (891 mg vs 665 mg, p = 0.008). Both groups experienced significant decreases in LEDD following surgery (elderly 331.38 mg, p = 0.0001; and young 108.6 mg, p = 0.0439), with a more significant decrease seen in elderly patients (young 108.6 mg vs elderly 331.38 mg, p = 0.0153). Elderly patients also experienced more significant reductions in daily doses (young 0.65 vs elderly 3.567, p = 0.0344). Both groups experienced significant improvements in motor function determined by reductions in UPDRS III scores (elderly 16.29 vs young 12.85, p < 0.0001); however, reductions in motor score between groups were not significant. Improvement in motor function was present for a mean follow-up of 3.383 years postsurgery for the young group and 3.51 years for the elderly group. The average follow-up was 40.6 months in the young group and 42.2 months in the elderly group. CONCLUSIONS: This study found long-term improvements in motor function and medication requirements in both elderly and young PD patients treated with DBS. These outcomes suggest that DBS can be successfully used in PD patients ≥ 70 years. Further studies will expand on these findings.