Malignant anaplastic meningioma in neurofibromatosis type 1 patient: a rare case report.

Background: Meningiomas are usually associated with neurofibromatosis type 2 (NF-2), while gliomas are usually associated with neurofibromatosis type 1 (NF-1). NF-1 is an autosomal dominant genetic disorder associated with skin manifestations, bone conditions, and different types of benign and malignant tumors. Grade 3 anaplastic meningiomas are rare tumors with a poor prognosis. Systemic treatments in grade 3 meningiomas are experimental, with some reports suggestive of minimal clinical benefits. They are used occasionally for recurrent cases with no surgical or radiotherapy roles. In our case, we will focus the discussion on grade 3 anaplastic meningioma in a patient with NF-1, using chemotherapy for this aggressive, recurrent tumor. To our knowledge, this is the first case of NF-1 associated with malignant anaplastic meningioma in English literature. Case Description: In this case report, we present a 25-year-old left-handed female patient who fits the diagnostic criteria for NF-1. She presented with focal seizure and was diagnosed with grade 3 anaplastic meningioma, a highly aggressive tumor. She experienced a rapid recurrence after her initial surgery and eventually received multiple lines of treatments, including radiation and chemotherapy [temozolomide (TMZ)]. Conclusions: Systemic therapy in grade 3 meningiomas is still experimental and may have a slight clinical benefit. As a result, further prospective, multicentric studies are needed to ascertain these outcomes. Patients should be included in prospective trials because of the poor prognosis and aggressive nature of grade 3 meningiomas. In addition, discovering specific molecular biomarkers will allow us to suggest an individualized treatment. This case suggests that the differential diagnosis of a mass in a patient with NF-1 should include tumors known to be associated with the syndrome as well as sporadic, unrelated neoplasms.

Primary Intracranial Solitary Fibrous Tumor With Metachronous Pulmonary and Bone Metastasis: A Case Report.

An intracranial solitary fibrous tumor (SFT) is a rare and aggressive tumor with a high propensity for locoregional recurrence and distant metastasis. The formerly used collective term for this tumor, "solitary fibrous tumor/hemangiopericytoma", has recently fallen out of use and is now commonly replaced with the term "solitary fibrous tumor". We describe a rare case of intracranial SFT with simultaneous metastasis to the spine, the right humerus, and the lungs four years after resection and radiotherapy of the primary tumor.

Case report: Dermatofibrosarcoma of the breast.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon neoplasm of the skin and soft tissue, commonly appearing on the trunk and extremities. The occurrence of DFSP in the breast is extremely rare. It has low to intermediate malignant potential with a high rate of local recurrence and invasion. We present a case of a 28-year- old female with a skin lesion on the breast associated with an underlying breast lump. We aim to discuss the sonographic and magnetic resonance findings of breast dermatofibrosarcoma.

Ectopic sympathetic ganglia cells of the ventral root of the spinal cord: an anatomical study.

The sympathetic trunk ganglia contain the cell bodies of neurons. However, some patients who undergo sympathectomy can develop compensatory hyperhidrosis. To evaluate for ectopic pathways, the present anatomical study was performed. Ten adult cadavers underwent dissection of the spinal canal and removal of randomly selected ventral roots, which were submitted for histological analysis. Random ventral root samples were taken from cervical, thoracic, and lumbosacral regions in each specimen. Each histological section was then analyzed and the presence or absence of sympathetic cells documented for level and position within the ventral root. Of all samples, a sympathetic nerve cell was found in 80% of ventral roots. At least one sympathetic cell was found in these 80%. Most sympathetic cells were found in the proximal one-third of the ventral root. Such cells were found at all spinal levels and no specific level within a vertebral region was found to house a greater concentration of these cells. No statistical significance was found when comparing sides or sex. Our study confirmed that sympathetic cells exist in the majority of human ventral roots. Such data might better explain various clinical presentations and postoperative complications/findings.

Metastatic Cecal Adenocarcinoma to the Gallbladder Presenting with Acute Cholecystitis.

Colorectal cancer (CRC) is one of the most common cancers and the second highest cause of cancer-related deaths (Jemal et al., 2011). Common presentations of CRC include alterations in bowel habit, weight loss, and lower gastrointestinal bleeding. We report a case of a 74-year-old male who presented with fever and right upper quadrant pain, with positive Murphy's sign on examination. The case was initially managed with a routine cholecystectomy. Histological examination revealed a moderately differentiated adenocarcinoma with a superimposed histologically proven acute acalculous cholecystitis. CT scan done postsurgery showed a cecal mass with retroperitoneal lymphadenopathy. Biopsy result of cecal mass was remarkable for colon adenocarcinoma. We are not aware of any similar prior cases reported in English literature.

Cerebellar Medulloblastoma in Middle-to-Late Adulthood.

Medulloblastoma is a malignant brain tumor that is typically seen in children. It is classified as an embryonal tumor, classically located within the posterior fossa. When it involves the fourth ventricle, the patient commonly presents with signs and symptoms of raised intracranial pressure secondary to obstructive hydrocephalus. It is exceedingly rare for Medulloblastoma to occur in middle and late adulthood. In this paper, we present a case of a 51-year-old man who presented with a posterior fossa mass that was diagnosed later as Medulloblastoma.

Demographic and histopathological patterns of neuro-epithelial brain tumors in Eastern Province of Saudi Arabia.

OBJECTIVE: To review the demographic and pathological pattern of neuro-epithelial brain tumors in a tertiary referral center in the Eastern Province of Saudi Arabia and to compare the results of our study with other national and international studies. METHODS: This is a retrospective chart-review study of all patients with neuro-epithelial brain tumors referred and treated in our center between January 2010 and January 2015. The age, gender, tumor location, and histopathology were recorded. RESULTS: The total number of cases was 149 including 96 adult cases and 53 pediatric cases. 58% of cases were male, and 42% were female. The age group distribution showed 2 peaks; one in the first 5 years of life and the second was in the age range from 26-45 years old. Glioblastoma multiforme was the most common pathological type (32%), followed by medulloblastoma (13.3%). This study showed similar results to a previous study conducted in the Eastern Province in terms of age and gender distribution, but pathologically, the tumors diagnosed in our study were generally of a higher grading. When comparing our results to other international studies in nearby countries (Jordan and Egypt), we found similarities in pathological patterns and age distribution. However, when comparing our results to a western country (USA), we found considerable differences in the age group distribution. CONCLUSION: Neuro-epithelial brain tumors in Saudi Arabia affect younger population according to our study compared to Western countries. These findings are similar to other studies from Middle Eastern countries. In addition, our study showed a significant increase in high grade gliomas in the Eastern Province compared to an old historical study. This increase should be interpreted cautiously due to possible selection errors, changes in pathological grading, and expertise.

Disseminated choroid plexus papillomas in adults: A case series and review of the literature.

Choroid plexus papillomas (CPPs) are uncommon, usually intraventricular, low-grade tumors, accounting for less than 1% of all intracranial neoplasms and 2-4% of brain tumors in children. Dissemination of CPPs to multiple levels of the neuraxis has been seldom observed. Thus far, only 26 adult patients have been reported in the English language literature. With some exceptions, disseminated CPPs have been observed in adults and involved multiple sites along the cerebrospinal fluid pathways. Occasionally, intraparenchymal extension has been documented, and secondary involvement of the suprasellar region has been reported in only five patients. Postoperative treatment of CPPs has not been standardized. Most recommended therapies have been extrapolated from a series of atypical papillomas or carcinomas of the choroid plexus in children. We herein report a series of three patients of disseminated choroid plexus papillomas providing additional insights into this relatively rare entity.

Ciliochoroidal ganglioneuroma in neurofibromatosis type 1: Report of a case and review of the literature.

Orbitofacial neurofibromatosis (OFNF) is considered a variant of neurofibromatosis type 1 (NF1). OFNF most often affects the eye, orbit and one side of the face. It is characterized by the development of relatively aggressive and disfiguring lesions, including plexiform and diffuse neurofibromas. Ciliochoroidal ganglioneuromas have not been previously reported in patients with this syndrome. We report the case of a 50-year-old man with OFNF, ciliochoroidal ganglioneuroma and a large ipsilateral frontoethmoidal encephalocele.

Rapidly enlarging low-grade fibromyxoid sarcoma with intracranial extension in a 5-year-old girl: case report.

Low-grade fibromyxoid sarcoma (LGFMS) is a rare mesenchymal tumor that is characterized by a benign histology but potentially aggressive clinical behavior, with a high rate of recurrence and metastasis. It primarily occurs in young adults in the extremities, inguinal area, neck, or chest wall. There are rare reports of intracranial LGFMS in adults. In this report, the authors present the case of a 5-year-old girl who presented with a rapidly enlarging frontal scalp mass. Pathological examination of the resected mass demonstrated LGFMS. To the authors' knowledge, this is the only reported case of intracranial LGFMS in a child.

Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review.

BACKGROUND: Chordomas are rare, slow-growing malignant neoplasms derived from remnants of the embryological notochord. Pediatric cases comprise only 5% of all chordomas, but more than half of the reported pediatric chordomas are intracranial. For patients of all ages, intracranial chordomas typically present with symptoms such as headaches and progressive neurological deficits occurring over several weeks to many years as they compress or invade local structures. There are only reports of these tumors presenting acutely with intracranial hemorrhage in adult patients. CASE DESCRIPTION: A 10-year-old boy presented with acute onset of headache, emesis, and diplopia. Head computed tomography and magnetic resonance imaging of brain were suspicious for a hemorrhagic mass located in the left petroclival region, compressing the ventral pons. The mass was surgically resected and demonstrated acute intratumoral hemorrhage. Pathologic examination was consistent with chordoma. CONCLUSION: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors' knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus.

Microscopic dysgerminoma associated with anti-Ma2 paraneoplastic encephalitis in a patient with gonadal dysgenesis.

We present a 27-yr-old female with gonadal dysgenesis (46, XY), who presented to our hospital with poor consciousness, aphasia, restlessness, and visual hallucination. Physical examination revealed normal breast development and normal external female genetalia. Computed tomography scan of the head and neck revealed the presence of brain edema, hydrocephalous, and a localized hypodense lesion in the hypothalamus. Her serum was positive for the anti-Ma2, which is associated with paraneoplastic encephalitis syndrome. Computed tomography of the abdomen revealed the presence of a 7.5×5.3×3.0 cm solid pelvic mass. Interestingly, a single microscopic focus of dysgerminoma was identified in a background of stromal fibrosis and focal dystrophic calcifications. No ovarian stroma or testicular tissue was identified. To our knowledge, this is the first case of gonadal dysgenesis presenting with anti-Ma2 paraneoplastic encephalitis with dysgerminoma. A discussion about paraneoplastic encephalitis with a microscopic dysgerminoma associated with anti-Ma2 antibody is presented.