Aggressive Pituitary Tumor with Crooke's Cells and Invasion of the Posterior Fossa.

BACKGROUND: Pituitary adenomas are mostly benign in character and are managed via a transsphenoidal approach in most cases. Crooke's cell adenoma (CCA) is a particular variant accounting for less than 1% of the pituitary adenomas. They have a distinctive histopathologic pattern and behavior. CASE DESCRIPTION: We present a case of a 56-year-old man with recurrent pituitary adenoma and complicated neurosurgical history. Imaging follow-up showed a suprasellar mass with progressive growth into the posterior fossa. Surgical management via retrosigmoid craniectomy was performed, and histopathology elucidated Crooke's cells. CONCLUSIONS: CCA is recognized by its local aggressiveness and high recurrence rates. They tend to be locally invasive; however, posterior fossa invasion has not been reported to date. We aim to contribute to the arsenal of differential diagnosis of similar pituitary tumor cases.

Cured from a Cardiac Myxoma? Brain Intraparenchymal Hemorrhage in a Patient with a 10-Year Successfully Resected Cardiac Myxoma.

BACKGROUND: A cardiac myxoma (CM) is the most common primary tumor of the heart. This case report highlights that for metastatic CMs, even after a successful total resection, there is a small but real risk of recurrence that can manifest as late as 10 years after initial tumor resection. CASE DESCRIPTION: We present the case of a 53-year-old woman who visited the emergency room after a 4-day worsening right-sided weakness. The patient was diagnosed with a left CM 10 years previously, and a complete surgical resection was performed at that time. A noncontrast computed tomography of the head revealed a left parietal hematoma and a contrast computed tomography scan of the head revealed an enlarged left parieto-occipital vein that was subsequently shown to be part of an arteriovenous fistula. After embolization of the fistula was performed, postprocedure magnetic resonance imaging of the brain showed redemonstration of acute intraparenchymal hemorrhage with vasogenic edema. The history of a previously resected CM and the multifocal distribution of brain lesions opened the possibility of slow-growing metastasis from the previous myxoma. CONCLUSIONS: Our case report demonstrates the metastatic nature of CMs to the central nervous system, even after successful gross total resection and no local relapsing mass in subsequent ultrasonographic follow-ups. A comprehensive evaluation on clinical and imagological grounds is mandatory to rule out the presence of myxomatous metastatic disease. Awareness and recognition of the potential neurologic manifestations of a metastatic CM will prevent unnecessary diagnostic workup and treatments.

Hashimoto's encephalopathy - presenting with epilepsia partialis continua and a frontal lobe lesion.

We report a case of Hashimoto's encephalopathy (HE), who presented with epilepsia partialis continua (EPC) and a frontal lobe lesion. The diagnosis of HE remained elusive until the serum thyroid antibodies became positive 7 months after the onset of EPC. The histopathology of this frontal lesion showed nonvasculitic inflammation.

Pure akinesia with gait freezing: a clinicopathologic study.

BACKGROUND: Pure akinesia with gait freezing is a rare syndrome with few autopsied cases. Severe freezing of gait occurs in the absence of bradykinesia and rigidity. Most autopsies have revealed progressive supranuclear palsy. We report the clinical and postmortem findings of two patients with pure akinesia with gait freezing, provide video recordings of these patients, and review the literature describing similar cases. We also discuss bradykinesia, hypokinesia and akinesia in the context of this clinical syndrome. CASE PRESENTATION: Two patients with the syndrome of pure akinesia with gait freezing were examined by the same movement disorder specialist at least annually for 9 and 18 years. Both patients initially exhibited freezing, tachyphemia, micrographia and festination without bradykinesia and rigidity. Both autopsies revealed characteristic tau pathology of progressive supranuclear palsy, with nearly total neuronal loss and gliosis in the subthalamus and severe neuronal loss and gliosis in the globus pallidus and substantia nigra. Previously published postmortem studies revealed that most patients with this syndrome had progressive supranuclear palsy or pallidonigroluysian atrophy. CONCLUSIONS: Pallidonigroluysian degeneration produces freezing and festination in the absence of generalized slowing (bradykinesia). Freezing and festination are commonly regarded as features of akinesia. Akinesia literally means absence of movement, and akinesia is commonly viewed as an extreme of bradykinesia. The pure akinesia with gait freezing phenotype illustrates that bradykinesia and akinesia should be viewed as separate phenomena.

Trigeminal Ganglioneuroma: A Rare Case of Trigeminal Neuralgia Caused by Cerebellopontine Angle Tumor.

BACKGROUND: Intracranial ganglioneuromas are very rare benign tumors of neural crest origin and generally arise from the peripheral nervous system or adrenal glands. Very few cases of intracranial ganglioneuroma arising from the trigeminal nerve have been reported in the literature, all in East Asia. CASE DESCRIPTION: A 52-year-old male presented to his primary care physician for evaluation of right facial and periorbital pain for over 18 months. He also reported a two-week history of diplopia with right lateral gaze, which resolved spontaneously. An MRI brain with and without contrast revealed a 3.0 × 2.6 × 2.4 cm enhancing extra-axial mass involving right Meckel's cave with a posterior 1.0 × 1.4 cm lobular component protruding into the prepontine cistern. The patient underwent a right middle fossa craniotomy for a subtemporal surgical resection of the tumor. Histologic section of the tumor demonstrated mature ganglion cells with surrounding satellite cells in a schwannian rich stroma, consistent with mature type ganglioneuroma. CONCLUSIONS: To the best of our knowledge, this is the sixth case of trigeminal ganglioneuroma; however, it is the first case reported in the United States.

Stillbirth: Correlations Between Brain Injury and Placental Pathology.

Chronic placental pathologic processes such as fetal thrombotic vasculopathy have been linked to brain injury in neonates. We hypothesize that using stillbirth as a model, placental pathology can predict risk for hypoxic-ischemic brain injury. From a single institutional database of stillbirths ≥23 weeks' gestational age, we included cases with full autopsy and neuropathology examination. Bivariable analyses were performed to identify whether there was an association between placental pathologic findings and neuropathologic findings. Logistic regression was used to control for potential confounders. Among 97 potential cases, adequate tissue was analyzable from 79 cases (mean gestational age  =  33 weeks). Acute central nervous system hemorrhage and acute neuronal necrosis were the most common neuropathologic processes seen in this cohort (57% for each). Maternal vascular underperfusion was the most common placental pathology but was not significantly associated with a specific neuropathologic finding. High-grade chronic villitis (HGCV) and fetal thrombotic vasculopathy (FTV) were significantly associated with increased risk for pontosubicular necrosis (odds ratios, 15.73 and 3.79, respectively). These associations persisted after controlling for potential confounders. Chronic placental pathologies, specifically HGCV and FTV, were associated with pontosubicular necrosis, suggesting that placental pathology involving the fetal vasculature and altered fetoplacental blood flow carry the greatest likelihood of hypoxic/ischemic brain injury.

Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature.

Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury) and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20). Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment.

A novel GRN mutation (GRN c.708+6_+9delTGAG) in frontotemporal lobar degeneration with TDP-43-positive inclusions: clinicopathologic report of 6 cases.

Understanding of frontotemporal lobar degeneration, the underlying pathology most often linked to the clinical diagnosis of frontotemporal dementia, is rapidly increasing. Mutations in 7 known genes (MAPT, GRN, C9orf72, VCP, CHMP2B, and, rarely, TARDBP and FUS) are associated with frontotemporal dementia, and the pathologic classification of frontotemporal lobar degeneration has recently been modified to reflect these discoveries. Mutations in one of these genes (GRN), which encodes progranulin, have been implicated in up to a quarter of cases of frontotemporal lobar degeneration with TDP-43 (TAR DNA-binding protein 43)-positive inclusions; currently, there are more than 60 known pathogenic mutations of the gene. We present the clinical, pathologic, and genetic findings on 6 cases from 4 families, 5 of which were shown to have a novel GRN c.708+6_+9delTGAG mutation.

Intracranial aneurysms in previously irradiated fields: literature review and case report.

OBJECTIVE: Radiotherapy is a common treatment for a variety of disease processes in the central nervous system; it has an ever-increasing number of indications and applications. With the life expectancy of cancer patients increasing, delayed complications of radiation have become more apparent. One such potential complication is the appearance of intracranial aneurysms in the irradiated field. The incidence and natural history of these aneurysms is not well understood. To this end, we performed a review of the literature to analyze the current state of knowledge of these rare aneurysms. Furthermore, we present a case treated at our center. METHODS: We reviewed the literature for all reported cases of intracranial aneurysms appearing in an irradiated field, including any available histopathologic analysis. All papers were included irrespective of the language in which it was published. We calculated the mean age at radiation exposure, the interval between radiation exposure, and aneurysm development and the rate of presentation. Herein we also present a case of an intracranial aneurysm in a 38-year-old patient detected in an irradiation field 33 years after the patient underwent craniospinal irradiation for a medulloblastoma. RESULTS: A total of 46 patients with 69 intracranial aneurysms in irradiation fields were reported between 1978 and 2013. The mean age at radiation exposure was 34 years, and the mean lag time between exposure and diagnosis was 12 years (range, 4 months to 50 years). The median lag time between exposure and diagnosis was shorter in patients older than 40 (6 years). Among the reported aneurysms, 83% were saccular, 9% were fusiform, and 9% were considered pseudo-aneurysms. The Median lag time was 20 years for brachytherapy, 8 years for focused radiation, 9 years for whole brain radiation, and 6 years for SRS. Among reported aneurysms, 55% presented with some form of hemorrhage: intracranial rupture with subarachnoid hemorrhage, epistaxis, or otorrhagia. Only 13% were discovered on routine follow-up or were found incidentally for work-up of unrelated neurologic symptoms. CONCLUSION: Although rarely reported, intracranial aneurysms in irradiation fields may warrant special attention when diagnosed. These aneurysms may have an inherently weaker structure and may be more prone to rupture. Their repair may also be complicated by more fragile and irregular morphology. The increasing longevity of cancer patients suggests that screening for aneurysms at irradiation sites may be warranted, but further studies are needed to validate this approach.

Multifocal and multicentric glioblastoma with leptomeningeal gliomatosis: a case report and review of the literature.

Glioblastoma (GBM) rarely presents as an infratentorial tumor in adults. The authors present a case of concomitant supratentorial and infratentorial GBM in an adult. A 72-year-old man presented with headache, nausea, vomiting, and lightheadedness. Initial MR images revealed enhancing masses in the right cerebellum and right posterior periventricular region. The patient underwent a suboccipital craniotomy and resection of the cerebellar lesion. Final histopathology was consistent with glioblastoma. The patient went on to receive standard radiation treatment for GBM with concurrent and adjuvant temozolomide. However, the patient experienced clinical deterioration within a few days after starting radiotherapy. He and his family decided to forego treatment and pursue palliative care. The patient expired three months after the initial diagnosis. Autopsy findings supported the diagnosis of GBM with leptomeningeal gliomatosis and involvement of the cerebrum, cerebellum, and spinal cord. The authors review the literature and propose that the pathogenesis of multiple and multicentric GBM may involve neural stem cells within the subventricular zone or could result from tumor dissemination along established CNS routes, such as white matter tracts and CSF pathways.