Primary anaplastic solitary fibrous tumor of the tentorium cerebelli

Meningeal solitary fibrous tumors (MSFT) have been described in about 80 patients as benign spindle-cell neoplasms, with few anaplastic variants. We report a 57-year-old male patient with a 4-month history of progressive headache caused by a primary anaplastic MSFT arising from the tentorium cerebelli. MRI revealed a tentorium-based tumor that extended into the occipital lobe superiorly and into the cerebellum inferiorly on the left. Following gross total resection of the tumor and postoperative radiotherapy, the patient experienced symptomatic improvement with no recurrence at the 12-month follow-up. The final tumor pathology was consistent with an anaplastic MSFT, with a Ki-67 proliferative index of 25%.     

Multiple myeloma presenting as solitary mass in the posterior fossa

Intracranial plasma cell tumors are extremely rare and can either be solitary lesions or part of systemic multiple myeloma. We report a 42-year-old woman who presented with a posterior fossa mass and successfully underwent surgical resection, leading to the diagnosis of multiple myeloma. To our knowledge, this is the first reported case of multiple myeloma presenting as a posterior fossa mass lesion. This report highlights the importance of maintaining plasma cell tumor in the differential of intracranial mass with bony involvement. Furthermore, once the diagnosis is established, further work up is critical to evaluate for systemic disease.     

Anterior C1-2 osteochondroma presenting with dysphagia and sleep apnea

Spinal osteochondromas are rare, benign tumors of the bone. These tumors can manifest as solitary lesions or as part of a hereditary syndrome. Most spinal osteochondromas occur in the posterior cervical spine and can cause myelopathy or radiculopathy. Osteochondromas of the anterior cervical spine that cause respiratory or swallowing symptoms are rare. We present the unique case of a solitary osteochondroma of the anterior C1 vertebral arch causing obstructive sleep apnea and dysphagia in a 16-year-old female. The patient underwent resection of the tumor via a left anterior transcervical approach to the spine. The patient’s symptoms resolved completely after surgery. To our knowledge, this is the first case of a sporadic osteochondroma arising from the anterior arch of the C1 vertebra causing dysphagia and obstructive apnea in a pediatric patient.     

Solitary fibrous tumor of the meninges: case report

The solitary fibrous tumor (SFT) is a rare mesenquimal neoplasm, found originally in association with the pleura. Recently, SFT was reported in others sites. The extension into adjacent structures is not uncommon. The meningeal involvement by SFT is rare and there has only twenty-six cases been reported previously in the literature. We report a case of a 25 years-old female patient with generalized tonic clonic seizures in the last six years. During the neurologic investigation, a tumor in the left occipital region of the brain was found. The patient underwent an occipital craniotomy with total resection of the tumor. The histopathological and immunohistochemical diagnosis was STF. After three years of follow-up, the patient remains stable, with a normal neurological exam. There is no sign of tumor recidive in the postoperative cranial tomography. We will briefly review the literature about STF.     

Intradural extraneural bilobate ganglion cyst of the atlanto-occipital joint compressing the hypoglossal nerve

Ganglion cysts (ganglia) are benign lesions of the soft tissue arising in the periarticular space. We present a 54-year-old woman with a 5-month history of headache and weakness of the tongue with deviation to the left side who had a rare extraneural intradural bilobate ganglion cyst of the atlanto-occipital joint compressing the hypoglossal nerve. An MRI showed a bilobate cystic lesion in the premedullary cistern on the left side at the level of the hypoglossal canal. This lesion was removed using a lateral suboccipital approach in the semi-sitting position with removal of the C1 hemiarch. The lesion proved to be a ganglion cyst on histopathology. Intracranial juxtafacet (ganglion and synovial) cysts compressing the hypoglossal nerve should be considered in the differential diagnosis with other lesions of this region. Although there was no recurrence at 30-month follow-up, there was no significant improvement of the tongue weakness. We describe our surgical strategy and discuss the pathogenesis of the cyst.     

Intracranial Extraskeletal Myxoid Chondrosarcoma : Case Report and Literature Review

Intracranial extraskeletal myxoid chondrosarcoma is extremely rare, with only seven patients previously reported. We present a case report of a 21-year-old woman admitted for weakness in her right extremities and symptoms of increased intracranial pressure. Magnetic resonance imaging (MRI) revealed hydrocephalus and a well-enhanced large mass around her left thalamus. A left parietal craniotomy and a cortisectomy at the superior parietal lobule were performed. Total surgical resection was also performed, and pathology results confirmed an extraskeletal myxoid chondrosarcoma. Postoperative MRI showed no residual tumor, and the patient underwent radiotherapy. After six months of radiotherapy, the patient''s headache and weakness had improved to grade IV. This malignant tumor showed high rates of recurrence in previous reports. We here report another occurrence of this highly malignant and rare tumor in a patient treated using total surgical excision and adjuvant radiotherapy.     

Epidermoid tumors of the temporal lobe as epileptogenic foci

Intracerebral epidermoid tumors of the temporal lobe are exceedingly rare. The vast majority of these slow-growing tumors remain clinically silent while only a select few are associated with overt symptomatology. We report two patients with epidermoid tumors whose unique location in the temporal lobe resulted in secondarily generalized seizures. Our first patient, a 19-year-old female, presented with a secondarily generalized tonic–clonic seizure and MRI revealed a superior temporal lobe lesion that was subsequently treated with an awake craniotomy and gross total resection. Our second patient was a 71-year-old male with a recent seizure history and known left temporal lobe lesion. The patient underwent craniotomy for gross total resection of the mass. In these two patients, we found that MRI proved diagnostic and surgical resection was curative. Our clinical experience and review of the literature indicate that gross total resection of these lesions confers control of localization-related epilepsy.     

Primary Rathke’s cleft cyst in the cerebellopontine angle associated with apoplexy

Rathke’s cleft cyst (RCC) is a congenital, benign, epithelial tumor and mainly occurs in sellar region and occasionally in suprasellar region; ectopic RCC is exceedingly rare. We report an uncommon RCC in cerebellopontine angle (CPA) associated with RCC apoplexy and investigated the possible hypothesis of its origin. A 12-year-old female student was admitted to hospital for 3-month history of vertigo, headache, nausea, and vomiting and aggravated for 1 week. Magnetic resonance imaging (MRI) revealed a space-occupying lesion with short T1 and long T2 signals in the left CPA and an intracystic floating nodule with hypointensity on T1- and T2-weighted imaging. The patient underwent the total tumor removal via the retrosigmoid approach with a good recovery. Primary RCC was confirmed by pathology. Follow-up MRI showed no recurrence 3.5 years after craniotomy. Primary RCC can occur in CPA and present special neuroimaging features associated with RCC apoplexy. We presumed that a mimicking mechanism of ectopic craniopharyngioma in CPA leads to the formation in the present case. Microsurgical resection is the optimal strategy for management. Further research and longer follow-up are helpful to better understanding the pathogenesis and development history of RCC in CPA.     

Thyroid carcinoma metastasis to the choroid plexus of the lateral ventricle

We present a rare case of a lateral ventricle choroid plexus metastasis arising from thyroid carcinoma in a 62-year-old man. The patient underwent subtotal excision of the intracranial tumour followed by total thyroidectomy with good outcome. We review previous reports of intracranial thyroid metastases and discuss the role of surgical resection, radiosurgery, whole brain radiotherapy and radioactive iodine therapy. There is no consensus regarding treatment in the literature due to small numbers of patients reported. We recommend surgical resection for single accessible lesions.     

Lumbar Spinal Extradural Angiolipoma: Case Report and Review of the Literature

Angiolipomas in the lumbar spinal region are extremely rare. The present report describes the identification of such a tumor and its removal, and discusses the tumor characteristics and prognosis. A 74-year-old woman was presented with a 5-month history of lower back pain. Severe radiculopathy was experienced in the left leg for 5 days prior to the presentation, and there were no neurological deficits. Magnetic resonance (MR) images showed an approximately 3.5 cm heterogeneously enhanced and elongated mass at the left L5-S1 level. A portion of the mass appeared with high signal intensity on T2-weighted MR images, with low signal intensity on T1-weighted images, and with high signal intensity on T1 fat suppression enhancement images. Resection of the tumor was approached via an L5 and S1 laminectomy. A fibrous sticky yellowish hypervascular tumor was identified. Histological study revealed the tumor as an angiolipoma. Symptoms were relieved after tumor excision, and there were no neurological sequelae. Although extremely rare, lumbar epidural angiolipoma should be considered in the differential diagnosis of lumbar spinal epidural lesions. The prognosis after surgical management of this lesion is favorable.     

Primary plasma cell granuloma of petrous bone.

Plasma cell granulomas are rare intracranial lesions that can mimic a variety of intracranial tumours. As they are usually benign lesions, their identification assumes importance. We report a case of plasma cell granuloma in a 52-year-old man presenting with features of left sided V, VII and bilateral VIII nerve involvement who underwent subtotal excision of the lesion. The relevant literature is reviewed.     

A case of giant skull myofibroma occupying left anterior cranial fossa]

We report a rare case of giant skull myofibroma occupying left anterior cranial fossa. A 53-year-old woman presented with left exophthalmos for 2 years. Neurological examination showed left exophthalmos, disturbance of bilateral visual acuity, and bitemporal hemianopsia. A CT scan revealed an ossifing mass at left anterior cranial fossa. On magnetic resonance images, the tumor showed iso-intensity on T 1-weighted image, heterogeneous high intensity on T 2-weighted image, and was heterogeneously well-enhanced after administration of Gd-DTPA. The tumor was fed mainly by middle meningeal artery. The patient underwent surgery and the tumor was removed totally. Histological diagnosis of the tumor was myofibroma. The patient has been followed every other month by MRI without any adjuvant therapy. There has been no tumor recurrence for 19 months. There is no other myofibroma in her body, therefore the patient was diagnosed as solitary myofibroma of the skull. Our case is the first report of solitary myofibroma of the skull because we could not find any reports on solitary myofibroma of the skull in the past literature.     

Solitary fibrous tumor of the meninges

Intracranial fibrous tumors are unusual growths that occur almost exclusively in young individuals. Most of these lesions are intra-axial, however, on extremely rare occasions they arise from the leptomeninges and compress the adjacent brain. The authors report the case of a benign solitary fibrous tumor of the meninges in a 14-year-old girl who presented with a 5-month history of generalized headaches. NMR imaging displayed a left parietal mass, which mimicked a meningioma.     

Primary alveolar soft part sarcoma arising from the cerebellopontine angle

Introduction

Alveolar soft part sarcoma (ASPS), a rare soft tissue malignant neoplasm, frequently metastasizes to the brain. However, primary intracranial ASPS is extremely rare. We present a case of primary intracranial ASPS arising from the cerebellopontine angle (CPA) without demonstrable systemic lesions.

Case report

An 11-year-old girl presented with a recurrent tumor in the right CPA after a partial resection and radiation therapy (RT). Near-total resection with a minimal tumor left in the jugular foramen was performed. The pathological diagnosis was ASPS. There was no evidence of primary extracranial tumors. She underwent adjuvant chemotherapy and gamma knife surgery. At 29 months after the second surgery, magnetic resonance imaging revealed multifocal enhancing lesions at the prepontine cistern, right CPA and medulla oblongata, despite intensive treatment. However, extracranial metastasis was not noted. This case suggested a poor outcome of primary intracranial ASPS, similar to extracranial ASPS.     

Giant intradiploic epidermoid cyst of the occipital bone

Epidermoid cysts are uncommon, benign and slow-growing lesions. They often reach an enormous size without producing neurologic symptoms. We describe a 35-year-old female who had a giant intradiploic epidermoid cyst of the occipital bone. She underwent posterior cranial fossa tumor resection. Pathology confirmed epidermoid cyst. There was no recurrence at 13-month follow-up. Total removal of these cysts and repeated washing of the cavity with 0.9% saline may prevent recurrence and aseptic meningitis.     

Brain metastasis from primary peritoneal carcinoma: Case report and literature review

Here, we describe a patient with primary peritoneal carcinoma (PPC) who developed a late, solitary brain metastasis. It is a case of a 68-year-old female presented with a 1-month history of progressive gait disturbance who had undergone a surgical resection of PPC five years prior, followed by adjuvant chemotherapy. An MRI revealed a 4.6 cm mass lesion in her right cerebellum. The patient underwent total removal of the tumour. The histopathology was consistent with a brain metastasis due to PPC. This condition is a rare malignancy of peritoneum, and only six cases of brain metastasis have been reported in PPC patients to date. Due to the prolongation of survival resulting from advanced chemotherapy for PPC, more patients will live long enough to develop brain metastases.     

Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus

Introduction Langerhans cell histiocytosis (histiocytosis X) is a rare disorder that can manifest with solitary bony lesions or disseminated disease in various tissues. A common manifestation of the disease is diabetes insipidus even in the absence of neural involvement. Disseminated disease often involves the central nervous system, but only six isolated lesions in the infundibulum have been reported.Case report An 18-year-old male had failed to develop secondary sexual characteristics. He also had diabetes insipidus for more than 1 year. Magnetic resonance imaging (MRI) of the brain showed a 1-cm homogeneously enhancing mass in the region of the infundibulum. No other lesions were found in other organ systems. The patient underwent an orbitozygomatic osteotomy and pterional craniotomy. The lesion, which was adherent to the infundibulum, was partially resected. Microscopic analysis showed multiple lymphocytes and eosinophils with Langerhans cells that stained positive for S-100. Langerhans cell histiocytosis was diagnosed, and the patient was discharged on postoperative day 6 with no neurological deficits. His diabetes insipidus persisted, and he underwent intensity-modulated radiation therapy to the sellar region.Conclusions Isolated Langerhans cell histiocytosis of the infundibulum is rare. The usual presentation is hypothalamic-pituitary axis dysfunction and diabetes insipidus. Patients with diabetes insipidus of unknown origin should undergo MRI of the sellar region to rule out infundibular abnormalities.     

Orbital Solitary Fibrous Tumor : A Case Report and Diagnostic Clues

Orbital solitary fibrous tumor (SFT) is a rare tumor originating from the mesenchyme. We describe the clinical presentations, radiological and operative findings, and pathological features of a patient with orbital SFT. The patient was a 46-year-old female who presented with progressive proptosis advanced for 20 months. On ophthalmological examination, no visual impairment was detected, but left eye was found to be obviously protruded on exophthalmometry. Orbital magnetic resonance imaging showed a 2.5 × 2 × 2 cm, intensely enhanced mass in the left orbit, which compressed the eyeball forward and the optic nerve downward. The patient underwent frontal craniotomy with superior orbitotomy and gross total resection was performed for the tumor. The histopathological diagnosis including immunohistochemistry was a SFT. After the surgery, proptosis was markedly relieved without visual impairment. Although orbital SFT is extremely rare, it should be considered in the differential diagnosis of orbital tumors. Clinical presentations such as painless proptosis and CD34 immunoreactivity play a significant role in differentiating orbital SFT from other spindle-cell neoplasms of the orbit.     

Expanded endonasal endoscopic approach for resection of a large skull base aneurysmal bone cyst in a pediatric patient with extensive cranial fibrous dysplasia

Aneurysmal bone cysts (ABCs) are uncommon non-neoplastic, hemorrhagic, and expansile osseous lesions. These lesions most commonly occur in the first two decades of life and affect the long bones and spinal column. Skull base involvement is rare. The authors report the case of a 16-year-old boy who presented with acute visual decline and was found to have a large skull base ABC centered in the sphenoid sinus. In addition, the patient had extensive cranial fibrous dysplasia. The patient underwent a staged expanded endonasal endoscopic approach for complete resection of this lesion with excellent return of his vision. This case adds to the growing body of evidence supporting a role for expanded endonasal endoscopic procedures in pediatric patients with skull base pathologies.     

Sincronía de diferentes tumores radioinducidos en superviviente de leucemia linfoblástica aguda

The cranial radiation-induced tumors appearance in pediatric patients treated for hematological diseases such as leukemia/T-lymphoblastic lymphoma (T-ALL/LBL) is a known phenomenon that may include lesions of different malignant degrees and require neurosurgical treatment.We present the case of a 38-year-old man referred to our department for a sudden diplopia with 6-month progressive left hemiparesis and frequent falls. After imaging tests, different intra and extraxial lesions with different radiological behavior were observed, performing a single surgical approach for their resection. The pathological anatomy confirms four histological variants: meningioma (grade 1 and 2 [atypical]), subependymoma, and cavernoma. We discuss the teratogenic role of ionizing radiation.