Epidermoid cysts of the cerebellopontine angle with extension into the middle and anterior cranial fossae: surgical strategy and review of the literature

Summary Epidermoid cysts are tumours, which contain keratin, cellular debris and cholesterol, and lined with stratified squamous epithelium. Clinically, epidermoid cysts behave like benign, slow-growing lesions. We present a 63 year-old man with a 6-month history of right periorbital pain and hypaesthesia in the area of the first and second branch of the trigeminal nerve. MRI revealed an epidermoid cyst of the cerebellopontine angle extending into the middle and anterior cranial fossae. Radical surgical removal of epidermoid cysts should be attempted, but a less aggressive surgical strategy should be considered if there is strong adherence to the surrounding brain tissue, particularly in eloquent areas. In this case, complete tumour removal was achieved via a suboccipital retrosigmoid approach.     

Enterogenous cysts of the cerebellopontine angle: short review illustrated by two new patients

Summary Intracranial enterogenous cysts are an uncommon entity rarely found in the midline within the posterior cranial fossa. The occurrence of an enterogenous cyst in the cerebellopontine angle is exceptional. We present two new cases of cerebellopontine angle (CPA) enterogenous cysts and review the literature to clarify the diagnosis and the management of these lesions. Eighteen cases of CPA intradural enterogenous cysts have been reported to date, including the two cases presented in this article. All of them were symptomatic and underwent surgical treatment. After surgery, the symptomatic recurrence occurred in 31% of the patients, most of which had partial excision. Considering our patients and the published cases in the literature we suggest that the aim of surgery should be total removal of cyst and its content whenever possible. When partial resection of the cyst is performed, we recommend long-term clinical and neuroradiological follow-up. Correspondence: Paolo Perrini, MD, Neurosurgical Department, Hope Hospital, Salford, Manchester, UK.     

Enterogenous cyst of the cerebellopontine angle cistern: case report

A case of an enterogenous cyst located in the cerebellopontine angle cistern is presented. These cysts have usually been found in the spinal canal, and their intracranial occurrence is exceptional. In the present case, the cyst was lined histologically by a single epithelial layer of cuboidal cells with some glandular structures resembling the fundic glands of the stomach. The unusual location of the cyst and its histological features are discussed.     

Ciliated hepatic foregut cyst

A male infant was found to have a large congenital hepatic cyst, first noted in late gestation by prenatal ultrasound scan. The cyst communicated with the biliary tree and was eventually removed completely by an extended right hepatectomy. Histopathologic examination showed a thick-walled, unilocular cyst lined predominantly by ciliated, stratified squamous epithelium with an outer wall composed of smooth muscle cells and fibrous tissue. These features are diagnostic of a ciliated hepatic foregut cyst, a rare congenital malformation with histologic similarities to bronchogenic cysts. The young age of our patient, prenatal detection, large size of the cyst, and a clear communication with the biliary tree have not been previously described with ciliated hepatic foregut cysts.     

Cerebellopontine angle epithelial cyst. A case report

A case of epithelial cyst in the cerebellopontine angle is reported. The cyst wall showed glandular epithelium with areas of non-keratinized stratified epithelium and flattened cuboidal cells. The glandular areas stained positively with antibodies to cytokeratin. In addition, the cyst wall contained areas of arachnoid tissue. This, and the unusual position of the cyst, suggest that the epithelial elements in the cyst wall may be metaplastic in origin. Similar previously described cysts were considered to be endodermal in origin.     

Neurenteric cyst of the posterior fossa. Case report and review of the literature

Intracranial neurenteric cysts are rare entities. The term is currently used to describe epithelial cysts that are lined with a presumed endodermal-derived epithelium and are mostly located in the posterior fossa.Preoperative diagnosis is often difficult because of their clinical presentation, which may resemble a subarachnoid hemorrhage, and the radiological aspect, which can mimic vascular pathologies.We describe a posterior fossa neurenteric cyst in a 27-year-old woman, who presented with sudden headache as the only symptom and who was addressed to our hospital for subarachnoid hemorrhage. Diagnostic angiography was negative and MRI revealed a prepontine cystic lesion. The patient underwent a posterolateral approach on the right side, with subtotal resection of the lesion.We discuss the embryologic, diagnostic and therapeutic aspects of these cysts and review the literature.     

Respiratory epithelial cyst in the cerebellopontine angle

The authors report a case with a cerebellopontine angle cystic mass. Computed tomography scan failed to demonstrate the lesion. Vertebral angiogram revealed an extra-axial lesion in the cerebellopontine angle. After successful surgical removal, it was found to be a respiratory epithelial lining cyst. The literature and histogenesis of this lesion are reviewed.     

Recurrent Intracranial Neurenteric Cysts

Summary  Neurenteric cysts are cystic lesions lined by a columnar epithelium of endodermal derivation. They are rarely located in the central nervous system. We describe the case history of two recurrent neurenteric cysts located within the posterior fossa: one in the 4th ventricle and the second in the cerebellopontine angle. The literature is reviewed and the follow-up and recurrence of such cysts are discussed.     

Endodermal cyst of the cerebellopontine angle cistern: case report]

We report a case of a 55-year-old man with endodermal cyst located in the cerebellopontine angle cistern. The patient presented with dizziness. Magnetic resonance imaging (MRI) revealed multilocular cystic lesion at the right cerebellopontine angle. T1-weighted image showed a mass with a low signal intensity, but higher intensity than CSF. Gd-DTPA T1-weighted image showed no enhancement in the mass. Diffusion-weighted image showed a mass with no signal lesion. After successful surgical removal, it was found to be an endodermal cyst. These cysts have usually been found in the spinal canal, and their intracranial occurrence is exceptional. The unusual location of the cyst and its histological features and radiological findings are discussed.     

Acquired mirror-image cerebellopontine angle arachnoid cysts: case report.

Arachnoid cysts are neurosurgical entities that have long been considered to be congenital in origin. Many examples in the literature suggest that there is a subgroup of arachnoid cysts that are required. The authors present the clinical history of a 17-month-old girl who developed two cerebellopontine angle arachnoid cysts after posterior fossa surgery for a brain tumor. After surgical excision of the tumor the child developed a left cerebellopontine angle cyst. This was treated through a suboccipital craniectomy by evacuating the cyst and excising the cyst wall. Two months later the child developed a second right-sided cerebellopontine angle cyst. It was treated by inserting a cystoperitoneal shunt. This article presents the case with radiological evidence of the acquired nature of the cysts. It also includes a brief review of the clinical presentation, pathogenesis, radiological evaluation, and surgical treatment of arachnoid cysts with emphasis on those occurring in the posterior fossa.     

Epithelial cyst of the gallbladder associated with adenocarcinoma

Epithelial cyst of the gallbladder associated with papillary adenoma and adenocarcinoma is presented herein. A 70-year-old male patient with general malaise was admitted to our hospital during the follow-up of an elevated mucosal lesion in the fundus of the gallbladder. Endoscopic ultrasonography revealed a cystic lesion adjacent to the elevated lesion, and interruption of the hyperechoic third layer of the gallbladder wall next to the gallbladder bed of the liver. He was diagnosed as having an epithelial cyst of the gallbladder associated with gallbladder carcinoma that directly invaded the gallbladder bed. He underwent resection of the gallbladder with partial resection of the liver and regional lymph node dissection. The resected specimen, which showed multiple cysts with septum formation, measured 1.9 cm in size and was located in the subserosal layer of the gallbladder wall. The wall of the cyst was lined with columnar epithelium and papillary adenoma, and adenocarcinoma was scattered in some portions of the cyst wall. Furthermore, papillary adenoma associated with adenocarcinoma was independently identified in the luminal surface of the gallbladder epithelium covering the cysts. These histological findings were compatible with epithelial cyst of the gallbladder associated with papillary adenoma and adenocarcinoma. The tumor was diagnosed as T1bN0M0, stage I, in the TNM clinical classification. To our knowledge, this is the first report of an epithelial cyst of the gallbladder associated with papillary adenoma and adenocarcinoma, and provides some insight into the relationship between epithelial cyst and adenoma or cystadenocarcinoma of the gallbladder. Received: December 25, 2001 / Accepted: April 14, 2002     

Giant prebulbomedullary arachnoid cyst. Case report and review of the literature

Usual locations of arachnoid cyst are the middle cranial fossa in 50-60%, cerebellopontine angle (10%) and suprasellar area (10%). Most of these malformations are asymptomatic. Premedullar arachnoid cysts are extremely rare. All previous cases reported were operated. We report a case of an asymptomatic giant craniocervical junction arachnoid cyst with a follow up of five years. In 2002, an adolescent consulted for persistent cervical pain. Encephalic MR showed a giant ventral craniocervical junction arachnoid cyst. Neurologic examination was normal. Conservative treatment was decided with a clinical follow up and repeated MR in case of persistent cervicalgia. Craniocervical junction arachnoid cysts are anecdotic. Medical care cannot be standardized. Pathogenesis and management are discussed.     

Pure alcohol injection of a congenital splenic cyst: a valid alternative?

Congenital splenic cysts are a rare entity. They are more frequent in children and young adults. They are true cysts, lined by epithelium with a typical trabeculation. Aspiration and injection with antibiotics (minocycline-tetracycline) or pure alcohol has been reported sparsely with variable results. The purpose of this case study is to report our experience with ultrasound (US)-guided aspiration and injection of a congenital splenic cyst, which, in our case, did not prove a viable alternative to surgical defenestration.     

Large bronchogenic cyst of stomach: A case report

IntroductionBronchogenic cysts are congenital cysts arising as an abnormal budding from primitive tracheobronchial tree. They are lined by pseudostratified columnar or cuboidal ciliated epithelium and contain smooth muscle fibers, submucosal bronchial glands and/or cartilage. They are most frequently located in the mediastinum or the lung parenchyma. Intramural occurrence of bronchogenic cyst in the gastric wall is very rare.Presentation of caseWe present a case of 65-year-old lady with a 7 × 8 cm lesion in the gastric cardia suspicious of gastrointestinal stromal tumor. Because of the large size, total gastrectomy with Roux-en-Y esophagojejunal anastomosis was performed. The postoperative course was uneventful. Histopathological examination revealed a sub-mucosal cyst lined by PCCE with presence of smooth muscle fibers and focal mucous glands. Final diagnosis of bronchogenic cyst was made. On the last follow up at one year, she was symptom free.DiscussionOn extensive Medline/Pubmed search, only 38 cases of gastric bronchogenic cysts were found to be reported till date. They are typically located in the posterior gastric wall close to the gastric cardia. On radiological imaging, they appear as well defined intramural cystic lesion without any characteristic features. Surgical resection is considered in symptomatic cases or in case of diagnostic dilemma.ConclusionGastric bronchogenic cysts often mimic gastrointestinal stromal tumor on preoperative imaging. They should be included in the differential diagnosis while dealing with an intramural gastric lesion close to the cardia or gastroesophageal junction.     

Spontaneous disappearance of cerebellopontine angle arachnoid cyst: report of a case

A case of a cerebellopontine angle arachnoid cyst spontaneously disappeared is reported. A 1-year-and-11-month old boy was suffered from sudden onset of left facial palsy. CT scan demonstrated dilatation of left internal auditory canal and a cystic lesion in the left cerebellopontine angle. Neurological examination disclosed only left facial palsy and left hearing loss. There was no signs and symptoms of increased intracranial pressure. He was followed up by CT scan. Repeated CT scan showed non-enhanced cystic lesion, the attenuation value of which was similar to that of cerebrospinal fluid. The cyst expanded gradually, and the brain stem was severely compressed. Then operation was planned under the diagnosis of left cerebellopontine angle arachnoid cyst about 2 years after the onset. But CT scan performed before operation showed disappearance of the cyst. Without operation the patient was followed by CT scan. There is no recurrence of the cyst. Natural history of arachnoid cyst will be well understood with repeated CT scan.     

Multicystic Mesothelial Cyst of the Mediastinum: Report of a Case

We report the rare case of a multicystic mesothelial cyst of the mediastinum. A 43-year-old man was referred to our department after a routine chest X-ray showed an anterior mediastinum mass. The lesion was resected by thoracoscopy, which revealed multicystic, thin-walled tense masses arising in the anterior mediastinum. The cysts were not adherent to the pericardium or to the thymus of the right lobe. Immunohistochemistry revealed that the cyst was lined with a layer of mesothelial cells and there were no malignant foci. The lining cells were positive for keratin and negative for the lymphocyte marker. These findings were consistent with a diagnosis of multicystic mesothelial cyst. Received: March 19, 2002 / Accepted: July 2, 2002 Acknowledgment. We would like to thank Ms. Miyazaki for her excellent technical assistance in pathology. Reprint requests to: H. Sasaki     

Extradural neurenteric cyst of the cerebellopontine angle. Case report

Neurenteric cyst is a developmental malformation found mainly in the spinal canal. The authors report on a 47-year-old man with a neurenteric cyst of the cerebellopontine angle (CPA) who presented with progressive hearing disturbance and facial palsy. The tumor was located extradurally with marked destruction of the petrous bone around the internal auditory canal and demonstrated irregular and heterogeneous high-intensity signals on T1- and T2-weighted on MR images, which is atypical for neurenteric cysts. The pathological findings in samples obtained after resection disclosed a single epithelial layer (a feature of neurenteric cyst), which was accompanied by marked xanthogranulomatous changes. Although several neurenteric cysts have been reported in the CPA, extradural lesions with unusual imaging features and marked bone destruction have not been reported previously. This benign developmental lesion should be considered, although it is extremely rare, in patients harboring an extradural temporal bone tumor around the CPA.     

A Bronchogenic Cyst Masquerading as a Tongue Mass

Bronchogenic cysts are foregut-derived developmental anomalies found along the developmental pathway of the foregut. The putative theory of pathogenesis is abnormal budding or branching of epithelial cells during the development of tracheobronchial tree. Over 99 % of cases occur in the mediastinum and lung while the head and neck area is affected in less than 1 % of cases with only rare cases reported in the oral cavity. This is a report of a case of a bronchogenic cyst arising in a 6-year-old male. The lesion presented as a painless swelling of the left underside of the tongue. Microscopically, the cyst was lined by pseudostratified columnar epithelium exhibiting many ciliated and mucous cells. A focus of cartilage and discontinuous bundles of smooth muscle were present adjacent to the lining. Where there was cyst rupture, there was granulation tissue associated with many foamy macrophages and acute and chronic inflammation. Four other cases, three in the tongue and one in the lower lip vestibule with cutaneous extension, all in the midline, have been reported in a 1 day-old male, 4 year-old male, 6 year-old female and 3 year-old male. There was no recurrence after excision and this is in keeping with the behavior in previous reports. Other developmental cysts including foregut cysts may be focally lined with respiratory epithelium but the presence of cartilage is the sine qua non for the diagnosis of a bronchogenic cyst.     

Cerebellopontine angle epidermoid cysts: clinical presentations and surgical outcome

Epidermoid cysts constitute less than 1 % of intracranial tumors with the majority of them involving cerebellopontine angle (CPA). Although several mechanisms for cranial nerve dysfunction due to these tumors have been proposed, no direct evaluation for hyper- or hypoactive dysfunction has been done. In this case series, pathophysiology of cranial nerve dysfunction in CPA epidermoid cysts was evaluated with special attention to a new mechanism of capsule strangulation caused by stratified tumor capsule. Twenty-two cases with epidermoid cysts of CPA micro-neurosurgically treated in our departments since 2005 were reviewed. Clinical status of the patients before the surgery and post-operative functional outcome were recorded. Available data from the English literature were summarized for comparison. Mass reduction of cyst contents in most cases was usually associated with prompt and marked improvement of the symptoms suggesting neuroapraxia caused by compression of the tumor content and/or mild ischemia. Among them, two cases showed strangulation of the affected nerves by the tumor capsule whose preoperative dysfunction did not improve after surgery in spite of meticulous microsurgical removal of the lesion. Involved facial and abducent nerves in these two cases showed distortion of nerve axis and nerve atrophy distal to the strangulation site. We report the first direct evidence of etiology of cranial nerve dysfunction caused by cerebellopontine angle epidermoid tumors. Young age and rapidly progressive neurological deficit might be the characteristics for strangulation of the affected nerves by the cyst capsule. Even though the number of cases might be limited, immediate decompression and release of the strangulating band might be urged in such patients to prevent irreversible deficits.     

Peripontomedullary hydatid cyst: Case report and literature review

Introduction: Hydatid cyst represents the parasitic infection by Genus Echenococcus Granulosis. This disease usually involves liver followed by lungs and rarely the CNS. The CNS involvement by the Hydatid Cyst is present in 1–2% of all hydatidosis. Even when it is found in the Brain it presents usually in the supratentorial compartment. However this case was unique in having the Hydatid cyst within the infratentorial fossa. With multiple small cysts, causing mass effect and challenging for surgical resection.Presentation: A 44 years female presented with headache, diplobia and bulbar symptoms, followed by ataxia. Full examination, proper investigations showed the peripontomedullary hydatid cysts. Surgical management is illustrated.Discussion: It is still challenging for the neurosurgeons to operate on these lesions in spite of modern technologies and fancy approaches due to its delicate nature, associated risk of allergic reaction, cyst’s material dissemination and irreversible injury of multiple neurological structure due to prolonged compression of cranial nerves crossing the cerebellopontine angle.Conclusion: In this case report we are presenting a rare case of Multiple Hydatid cysts involving a rare location in the brain; peripontomedullary area and extending all the way down to the foramen magnum. Supported with a literature review in relation to disease etiology, epidemiology, clinical presentation and management.