Infundibular Epidermoid Cyst: Case Report and Systematic Review.

BACKGROUND: Intracranial epidermoid cysts are benign, congenital, keratinizing, squamous epithelial-lined cysts filled with keratin. They are uncommon and often pose a surgical challenge owing to the adherence to surrounding structures. They are typically found at the cerebellopontine angle or in the parasellar region, where they are associated with abnormal development of the Rathke pouch; involvement of the pituitary stalk is rare. CASE DESCRIPTION: The patient's electronic health record was queried for relevant data. A systematic review of the literature using dedicated search terms for cases of infundibular epidermoid cysts was conducted. We present a unique case of a 55-year-old male who presented with vision changes and was found to have a parasellar epidermoid cyst confined to the pituitary stalk. The patient underwent endoscopic transsphenoidal resection, and gross total resection was achieved. The patient's postoperative course was significant for possible chemical meningitis and the development of panhypopituitarism. The patient's vision subjectively improved after surgery. CONCLUSIONS: Although 3 other cases of epidermoid cysts involving the pituitary stalk were identified, our patient's tumor was unique in that it was confined to the stalk. Our patient's case highlights a surgical approach to parasellar epidermoid cysts and the possible complications associated therewith.

Brain metastasis: clinical characteristics, pathological findings and molecular subtyping for therapeutic implications.

Metastases are the most common brain tumors especially in adults. Although they are generally considered a single disease entity which is universally fatal in patients with advanced cancer, brain metastases are remarkably heterogeneous both clinically and pathologically. As members of the multidisciplinary clinical team for the diagnosis and management of metastatic brain tumors, pathologists must be familiar not only with clinicopathologic features of brain metastases but also with any characteristic and clinically significant molecular findings. We discuss here the epidemiology, general gross and microscopic features of brain metastases with emphasis on how to differentiate them from primary brain tumors using immunohistochemistry (e.g., for identification of the primary site and differential diagnosis), and unique pathologic patterns of brain metastases (namely, dural metastasis, leptomeningeal carcinomatosis, miliary metastasis, ''intravascular carcinomatosis'', and tumor-to-tumor metastasis) with their clinical and radiological characteristics. We specifically address metastatic breast and non-small cell lung cancers which are the two most commonly encountered in daily practice, with emphasis on the molecular alterations related to therapy and their clinicopathologic significance.

Cerebellar pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1: a case report and literature review.

Pleomorphic xanthoastrocytoma (PXA) is an uncommon tumor of young adults that typically occurs supratentorially. It is generally considered to be a low-grade, circumscribed tumor that when treated by surgical resection has a relatively favorable outcome. Cases of cerebellar PXA are rare, and those associated with neurofibromatosis type 1 (NF1) are even less common, with only 2 cases reported to date. We present herein a third case of PXA-NF1 with unusual features. A 33-year-old woman presented with a history of headache. Her medical and family history was significant for NF1. Brain MRI revealed a 3.4 cm ill-defined lesion with a gyriform enhancing pattern in the left cerebellum, superficially mimicking Lhermitte-Duclos disease. The patient underwent a gross total resection of the lesion and had an unremarkable postoperative course. While the lesion had histological features typical of "pure" PXA (WHO grade II) it had an unusual growth pattern with thickening of the superficial cerebellar folia and predominant leptomeningeal involvement. No BRAF, IDH-1, or IDH-2 mutation was identified. Three months after surgery, local recurrence was detected, and the patient was treated with radiation therapy. One year after the first surgery, she underwent surgical resection of the recurrent/residual tumor. Histologically, the recurrent tumor showed very similar features to the initially resected tumor, with no anaplastic features. Most cerebellar PXAs have an indolent clinical behavior as do most cerebral PXAs. Whether co-existence of NF1 was a factor in altering the clinical course and biologic behavior of this patient's tumor is currently unknown.

Intravascular carcinomatosis of central nervous system due to metastatic inflammatory breast cancer: A case report.

Central nervous system (CNS) involvement by metastatic cancer is well-recognized and typically presents with multifocal solid tumors within the brain parenchyma or leptomeningeal dissemination. We describe herein a histologically very rare case of CNS metastasis in a 52-year-old woman who presented with mental status changes. Post mortem examination revealed extensive CNS involvement by metastatic inflammatory breast carcinoma, characterized by the presence of single tumor cells diffusely present within capillaries without parenchymal or perivascular invasion, and acute ischemic changes/infarcts bilaterally involving multiple areas. The cancer cells were found predominantly in the cerebral cortices and deep gray matter structures. Pre-mortem magnetic resonance and CT imaging of the brain did not identify metastatic cancer; however, widespread ischemic changes (i.e. brain infarcts) were identified. Inflammatory breast carcinoma is well-known to have a predilection for spread through lymphovascular spaces. Post mortem examination revealed tumor involvement of bilateral lungs, heart and bladder, with sinusoidal spread in the liver and lymph nodes and prominent involvement of the splenic red pulp in addition to extensive vascular involvement of the brain and spinal cord without a discrete mass, despite the presence of widely metastatic disease. The tumor cells in the CNS were strongly immunoreactive for pancytokeratin, E-cadherin, cytokeratin-7, epithelial membrane antigen and CAM 5.2. This unique histologic pattern of tumor spread is considered to represent "intravascular carcinomatosis" in the CNS, and most likely the cause of the patient's widespread ischemic injuries.

Cerebral sinus thrombosis: a fatal neurological complication of ulcerative colitis.

Cerebral sinus thrombosis has been reported as an uncommon complication of ulcerative colitis (UC), occurring in up to 7.5% of cases. It is suspected to be a consequence of genetic predisposition and the hypercoagulable state occurring during disease relapse. We report a case of a 23-year-old male patient with one-year history of UC. He presented to the Emergency Room with left-sided progressive hemiparesis, numbness, hemiparesthesia, and pain, which followed a recent exacerbation of UC. The patient died 3 days after admission and an autopsy revealed superior and inferior sagittal sinus and cortical vein thrombosis with associated cerebral edema, hemorrhagic infarction, and herniation. The gastrointestinal tract had continuous cobblestone appearance extending from rectum to cecum, with hemorrhage and ulceration, consistent with active UC. Awareness of this rare complication of UC can contribute to early recognition and attempts at treatment of this serious and often fatal condition.

Whipple disease of the central nervous system: an unusual occurrence in association with acquired immune deficiency syndrome.

Whipple disease is a multisystem infectious disease caused by Tropheryma whippleii. It commonly affects the CNS and produces neurological symptoms in 10-20% of cases. Central nervous system Whipple disease occurring in patients with AIDS is extremely rare. The authors present a case of a newly diagnosed AIDS patient in whom intracranial Whipple disease was diagnosed by stereotactic brain biopsy.

A supratentorial, hemorrhagic, intraparenchymal epidermoid cyst.

Epidermoid cysts are slow growing benign tumors that represent < 1-2% of all intracranial tumors and rarely present as supratentorial, intraparenchymal masses. We present the first report of a supratentorial, hemorrhagic, intraparenchymal epidermoid cyst with its presentation, our operative approach, post-operative course, radiographic features, and a literature review.

Nocardiosis with brain abscess due to an unusual species, Nocardia transvalensis.

The identification of Nocardia transvalensis, an unusual and probably underrecognized cause of nocardial infection, is clinically significant because of this species' resistance to aminoglycosides, a standard antinocardial therapy. Diagnosis requires analytic methods available predominately in reference laboratories. We report a case of disseminated infection with N transvalensis with primary pulmonary involvement and subsequent development of brain abscesses, and review the literature to date. Familiarity with the epidemiology, pathologic findings, and clinical significance of this and other unusual Nocardia species may increase early identification and antibiotic susceptibility testing in cases of nocardial infection.