Vaccination with HPV-18 E7-pulsed dendritic cells in a patient with metastatic cervical cancer [7]

To the Editor: The anagement of disseminated carcinoma of the cervix that is no longer amenable to control with surgery or radiation therapy has not improved significantly with the advent of modern chemotherapy. The one-year survival rate remains between 10 percent and 15 percent. Studies have provided a rationale for using dendritic cells as natural adjuvants for human immunotherapy

Case Report: SATB2-Associated Syndrome Overlapping With Clinical Mitochondrial Disease Presentation: Report of Two Cases

SATB2-associated syndrome (SAS) is an autosomal dominant neurogenetic multisystemic disorder. We describe two individuals with global developmental delay and hypotonia who underwent an extensive evaluation to rule out an underlying mitochondrial disorder before their eventual diagnosis of SAS. Although the strict application of the clinical mitochondrial disease score only led to the designation of “possible” mitochondrial disorder for these two individuals, other documented abnormalities included nonspecific neuroimaging findings on magnetic resonance imaging and magnetic resonance spectroscopy, decreased complex I activity on muscle biopsy for patient 2, and variation in the size and relative proportion of types of muscle fibers in the muscle biopsies that were aligned with mitochondrial diseases. SAS should be in the differential diagnoses of mitochondrial disorders, and broad-spectrum diagnostic tests such as exome sequencing need to be considered early in the evaluation process of undiagnosed neurodevelopmental disorders

Prostate carcinoma mimicking a sphenoid wing meningioma

AbstractIntroductionWe report here on a rare case of a large, lateral sphenoid wing tumor with radiographic and intraoperative findings highly suggestive of meningioma, yet pathology was in fact consistent with metastatic prostate adenocarcinoma.Presentation of caseAn 81 year-old male presented with expressive dysphasia, right-sided weakness and headaches. Imaging revealed a heterogeneously-enhancing lesion based on the left lateral sphenoid wing. The presumed diagnosis was strongly in favor of meningioma, and the patient underwent complete resection of the dural-based lesion. Final pathology confirmed the unexpected finding of a metastatic prostate adenocarcinoma. Although he tolerated surgery well, the patient was subsequently referred for palliative therapy given findings of widespread systemic disease.DiscussionIntracranial metastases may involve the dura, at times presenting with rare radiographic features highly suggestive for meningioma, as in our case here. This makes differentiation, at least based on imaging, a challenge. Elderly patients presenting with neurological deficits secondary to a newly-diagnosed, dural-based lesion should thus be considered for metastasis, prompting additional imaging studies (including body CT, MRI or PET) to rule out a primary lesion elsewhere. In some cases, this may affect the overall decision to proceed with surgical resection, or alternatively, to proceed directly to palliative therapy (the latter decision made in the context of widespread metastatic disease).ConclusionWe conclude that dural-based metastatic lesions may mimic meningiomas, warranting thorough pre-operative work-up to exclude the possibility of metastasis. In certain cases, identification of widespread disease might preclude surgery and favor palliation, instead

Frontal epidural metastasis of follicular carcinoma

Papillary-follicular thyroid carcinoma usually remains localized to the thyroid bed, and in cases of metastasis, almost always involves the lungs, bones, or liver. Brain metastasis is rare and there has been no reported case of epidural metastasis of thyroid carcinomas in the literature up to date. A frontal epidural mass lesion was excised and histologically found to be a follicular thyroid carcinoma metastasis

122 Pediatric Radiation-Associated Meningiomas: Distinct Clinical, Pathological, and Cytogenetic Characteristics

INTRODUCTION:Radiation-associated meningiomas (RAMs) arise after treatment with radiation to the cranium and are recognized as clinically separate from sporadic meningiomas. Limited information exists about the clinical, pathological, and cytogenetic features of RAMs in pediatric patients. We report the findings in 9 children with meningiomas following therapeutic radiation to the cranium. METHODS:Medical files were searched for patients who demonstrated meningiomas after a history of radiation to the brain. Only those patients in whom a meningioma occurred before the age of 18 years were included in this study. Clinical and demographic data along with the MIB-1 labeling index and cytogenetic studies were evaluated. RESULTS:The patients consisted of 5 males and 4 females with a median age of 5 years (range 2 10 years) at radiation therapy. The latency period was a median of 10 years after radiation therapy (range 6 13 years). MIB-1 labeling index was a median of 6.6% (range 4% 10%). There was no statistical difference in the MIB-1 LI between patients in whom the tumor recurred vs those who were disease free (P = .29). Five patients (55.6%) displayed multiple meningiomas at the first presentation. Histological types included clear cell meningioma in 1 patient, fibroblastic meningioma in 2, chordoid meningioma in 2, meningothelial meningioma in 7 (atypical in 2 cases), xanthomatous meningioma in 1 and chordoid meningioma in 1. Cytogenetic studies showed that the loss of 22q12.2 was the most common abnormality (3 patients), followed by complex cytogenetic abnormalities (2 patients) and rearrangements between chromosomes 1 and 12 (1 patient) and 1p deletion (1 patient). CONCLUSION:In contrast to RAMs occurring in adults, those in pediatric patients show an increased incidence of multiplicity on first presentation and unusual histological variants, some of which are described here for the first time. There was no difference in the MIB-1 labeling index in children with RAMs as compared with that in children with non-RAMs

Neurosarcoidosis Mimicking Viral Meningoencephalitis

Neurosarcoidosis is a rare disease more commonly associated with systemic sarcoidosis and affects the cranial nerves preferentially; (1). In a retrospective study of sarcoidosis patients, isolated neurosarcoidosis has been cited to have an incidence of around ten; percent, making its diagnosis problematic (2)

Radiation-associated meningiomas in children: clinical, pathological, and cytogenetic characteristics with a critical review of the literature Clinical article

Object. Radiation-associated meningiomas (RAMs) arise after treatment with radiation to the cranium and are recognized as clinically separate from sporadic meningiomas. Compared with their sporadic counterparts, RAMs are often aggressive or malignant, likely to be multiple, and have a high recurrence rate. However, limited information exists about the clinical, pathological, and cytogenetic features of RAMs in pediatric patients. The authors report the findings in 9 children with meningiomas following therapeutic radiation to the cranium. In addition, they performed a critical review of the English language literature on pediatric RAMs. Methods. Medical tiles were searched for patients who demonstrated meningiomas after a history of radiation to the brain. Only those patients in whom a meningioma occurred before the age of 18 years were included in this study. Clinical and demographic data along with the MIB-1 labeling index and cytogenetic studies were evaluated. Results. The patients consisted of 5 males and 4 females with a median age of 5 years (range 2-10 years) at radiation therapy. The latency period was a median of 10 years after radiation therapy (range 6-13 years). The MIB-1 labeling index was a median of 6.6% (range 4%-10%). Five patients (55.6%) displayed multiple meningiomas at the first presentation. Histological types included clear cell meningioma in I patient, fibroblastic meningioma in 2, chordoid meningioma in 2, meningothelial meningioma in 7 (atypical in 2 cases), xanthomatous meningioma in 1, and chordoid meningioma in 1. Cytogenetic studies showed that the loss of 22q12.2 was the most common abnormality (3 patients), followed by complex cytogenetic abnormalities (2 patients) and rearrangements between chromosomes I and 12 (I patient) and a 1p deletion (1 patient). Conclusions. In contrast to RAMs occurring in adults, those in pediatric patients show an increased incidence of multiplicity on first presentation and unusual histological variants, some of which are described here for the first time. There was no difference in the MIB-1 labeling index in children with RAMs as compared with that in children with non-RAMs. (http//thejns.org/doi/abs/10.3171/2012.7.PEDS1251

Diffuse Leptomeningeal Glioneuronal Tumor Presenting as Progressive Cranial Neuropathies

We present a unique case of Diffuse leptomeningeal glioneuronal tumor (DLGT) presenting as stroke-like symptoms with progressive cranial neuropathies