Dorsal intramedullary spinal epidermoid cysts: Report of two cases and review of literature

Intramedullary epidermoid cysts of the spinal cord are rare tumors, especially those not associated with spinal dysraphism. About 50 cases have been reported in the literature. Of these, only seven cases have had magnetic resonance imaging (MRI) studies. We report two cases of spinal intramedullary epidermoid cysts with MR imaging. Both were not associated with spina bifida. In one patient, the tumor was located at D4 vertebral level; while in the other, within the conus medullaris. The clinical features, MRI characteristics and surgical treatment of intramedullary epidermoid cyst are presented with relevant review of the literature.     

Intramedullary epidermoid cyst presenting with abnormal urological manifestations

STUDY DESIGN: Report of an epidermoid cyst with intramedullary localization. OBJECTIVE: To describe an atypical presentation of intramedullary epidermoid cyst. SUMMARY OF BACKGROUND DATA: Intramedullary epidermoid cysts are rare entities with a marked variability in the clinical presentation, essentially of neurological pertinence. METHODS: Case report of a spinal epidermoid cyst in a 13-year-old girl presenting with urological symptoms: she had a 12-month history of recurrent low urinary tract infections, urinary frequency and nocturnal enuresis. A urodynamic evaluation was performed and showed the presence of involuntary bladder contractions with detrusor instability and low bladder compliance. Magnetic resonance imaging of the spine demonstrated an intramedullary lesion of the dorsal spinal cord. RESULTS: The mass was excised and 6 months after surgical excision, urological manifestations improved with decreased detrusor hyper-reflexia, increased bladder capacity and compliance and no later report of urinary tract infections. CONCLUSIONS: In our patient, unusual clinical manifestations of the tumor have delayed the diagnosis, but its complete removal has led to remission of symptoms. Detailed neurological examination and investigations are indicated in patients with clinical and urodynamic features, suggestive of neuropathic bladder.     

Multiple epidermoid cysts located in the pineal and extracranial regions treated by neuroendoscopy

A 22-year-old woman presented with a rare case of multiple epidermoid cysts located in the pineal and extracranial regions. Magnetic resonance (MR) imaging showed a lesion in the pineal region as hypointense on the T(1)-weighted image and hyperintense on the T(2)-weighted image, without enhancement. Neuroendoscopic treatment was performed under a diagnosis of pineal cyst. However, the cyst wall was too thick to perforate, although third ventriculostomy was performed. Diffusion-weighted MR imaging demonstrated the lesions in the pineal and extracranial regions as marked hyperintensity. The diagnosis was epidermoid cyst. Subsequently, neuroendoscopic treatment of the pineal epidermoid cyst was performed. Careful preoperative diagnosis of epidermoid cysts based on diffusion-weighted MR imaging is required.     

Magnetic resonance appearance of multiple intracranial epidermoid cysts: intrathecal seeding of the cysts? Case report

A 44-year-old man presented to the hospital with multiple intracranial epidermoid cysts. The clinical manifestations of his disease included chronic headaches and one seizurelike episode. Findings determined by magnetic resonance (MR) imaging, surgery, and histological analysis indicated intrathecal and intraventricular seeding of the cysts. Spontaneous (nontraumatic) seeding of multiple daughter cysts from intracranial epidermoid cysts is still very rare and their multiple appearances on MR imaging should be distinguished from the simple scattering of oily contents due to cyst rupture.     

Posterior fossa epidermoid cysts presenting with unusual radiological appearances--two case reports

Intracranial epidermoid cysts generally appear as hypodense on computed tomography (CT), hypointense on T(1)-weighted magnetic resonance (MR) imaging, and hyperintense on diffusion-weighted MR imaging. We report two cases of posterior fossa epidermoid cysts with unusual radiological features. A 49-year-old male presented with facial dysesthesia and a 12-year-old male presented with diplopia and internuclear ophthalmoplegia. CT of both cases revealed hyperdense lesions. MR imaging showed the first case as hypointense in the posterior part and hyperintense in the anterior part of the tumor on diffusion-weighted imaging, and the second case as hyperintense on diffusion-weighted and T(1)-weighted MR imaging. Surgical exploration revealed that the tumors consisted of creamy materials, instead of the usual semi-solid or flaky texture in epidermoid cysts. Xanthochromic serous fluid was also contained in the superoposterior half of the cyst of the first case. These unusual contents of the cyst may be responsible for the unusual neuroimaging findings. Histological examination showed both cysts were lined with stratified squamous epithelium and contained keratinaceous materials. Therefore, epidermoid cysts can occasionally present with unusual radiological characteristics giving rise to a diagnostic pitfall.     

脊髓内胆脂瘤的诊断和手术治疗

目的提高脊髓内胆脂瘤的诊断水平及选择治疗方法。方法总结１９７８～１９９５年收治的经手术和病理证实的１０例脊髓内胆脂瘤的经验。结果患者临床表现无特异性。本组ＣＴ检查８例，显示肿瘤呈现均匀低密度影像，边界清楚，强化扫描多无增强。ＭＲ检查４例，肿瘤呈现信号强度变化不定的特点，边界清楚。１０例患者均行手术治疗，肿瘤全切除１例，次全切除９例。８例术后得以随访，无复发。结论肿瘤全切是治疗本病的最好选择。     

Schwanzdarmzyste

Tumors of the retrorectal space are rare entities. The case report of a retrorectal tailgut cyst serves to illustrate the clinical and diagnostic findings, differential diagnosis, and therapy of retrorectal tumors. Tumors in this area can be identified by palpation. The subsequent investigations include ultrasound, endosonography, endoscopy, and by choice computed tomography or magnetic resonance imaging. Usually tailgut cysts are characterized as cystic tumors, well delineated with multicystic formation. The diagnosis has to differentiate between carcinomas of the colorectum, hamartomas, lymphomas, teratomas, chordomas, abscess formation, dermoid cysts, epidermoid cysts, and enteral cysts. An elevated level of CEA may point to malignancy. Complete surgical resection is the therapy of choice.     

Intramedullary neurenteric cyst without an associated malformation. Case report

The authors report a case of an intramedullary neurenteric cyst without any associated dysraphic lesion. Unlike extramedullary intradural forms, this type of malformation remains rare. Our observation is one of the first to have been evaluated by magnetic resonance imaging. Theories concerning the embryogenesis of neurenteric cysts as well as their clinical characteristics and surgical treatment are discussed.     

Intradiploic arachnoid cyst identified by diffusion-weighted magnetic resonance imaging--case report

A 72-year-old woman presented with an intradiploic arachnoid cyst in the occipital intradiploic space which was found incidentally by magnetic resonance (MR) imaging. Computed tomography revealed a widened diploic space and thinning of the inner and outer tables of the occipital bone. The cyst appeared as isointense to the cerebrospinal fluid on both T1- and T2-weighted images. The differential diagnosis of intradiploic epidermoid cyst could be excluded because the lesion was low intensity on diffusion-weighted MR images. Arachnoid cyst is a benign lesion, so exploratory surgery should be avoided unless the cyst is symptomatic. Diffusion-weighted MR imaging is an effective modality to distinguish diploic epidermoid cysts from arachnoid cysts.     

Dermoid cyst: A rare intramedullary inclusion cyst

Intramedullary dermoid cysts are rare tumors, especially those not associated with spinal dysraphism. Only six cases have been reported in the literature. Of these, only two cases have had magnetic resonance imaging studies. We report a case of an 18-year-old female patient, who presented with progressive weakness of both the lower limbs and wasting of both the upper limbs. Magnetic resonance imaging (MRI) showed an intramedullary lesion extending from C3 to D2 with peripheral enhancement on contrast. Decompression of the cystic contents with partial removal of cyst wall was done. Hair with oily cholesterol and keratin debris was encountered. Histopathology confirmed the diagnosis of dermoid cyst. This case adds to the previous reported cases of the rare and uncommon intramedullary space occupying lesions of the spinal cord.     

Multiple intramedullary lipomas with conal intramedullary dermoid: magnetic resonance appearances.

BACKGROUND CONTEXT: Intramedullary fat-containing benign childhood tumors of the cord include lipomas, dermoid cysts, and teratomas. These are embryonal tumors. Most intramedullary fat-containing tumors are solitary. Multiple intramedullary lipomas are rare and may represent a spinal lipomatous malformation. The presence of another intramedullary dermoid tumor in the same case is rare. PURPOSE: The intent of this case report is to look at magnetic resonance features and possible mechanisms of association of these fat-containing intramedullary tumors. STUDY DESIGN/SETTING: A 3-year-old male child presented with spastic quadriplegia. METHODS: Magnetic resonance imaging (MRI) of spine was done on a 1.5-T scanner in different planes. RESULTS: MRI showed multiple intramedullary spinal lipomas with an intramedullary dermoid involving the conus, cord atrophy, and subarachnoid fat droplets. CONCLUSION: Multiple intramedullary lipomas with an intramedullary dermoid represent a form of spinal lipomatous malformation. Both may represent embryogenic mesenchymal inclusions and hamartomatous growth, which can be accurately diagnosed with MRI.     

Malignant squamous degeneration of a cerebellopontine angle epidermoid tumor. Case report

The authors present the case of a woman with a cerebellopontine angle (CPA) epidermoid cyst that degenerated into a squamous cell carcinoma. Malignant degeneration of an epidermoid cyst is an extremely rare occurrence. Malignant transformation must be considered in the differential diagnosis when new contrast enhancement on imaging studies and progressive neurological deficit are seen in a patient harboring an epidermoid cyst. The patient initially presented with a 10-year history of left trigeminal neuralgia, subacute left-sided hearing loss, and with facial weakness of 3 weeks' duration. Initial magnetic resonance (MR) imaging revealed a left CPA mass, consistent with an epidermoid. There was faint contrast enhancement where the tumor was in contact with the lateral brainstem. A subtotal resection was performed. Histopathological findings were consistent with an epidermoid tumor. One year after initial presentation, the patient's neurological deficit had increased, and follow-up MR imaging demonstrated a large contrast-enhancing tumor filling the left CPA and compressing the brainstem. At repeated surgery a squamous cell carcinoma arising from the previous epidermoid was found. The patient was subsequently treated with external-beam radiotherapy and stereotactic radiosurgery. Her tumor stabilized. Three years and 8 months after the patient's initial presentation, a new area of tumor developed at the torcular Herophili. The patient died shortly thereafter. Malignant squamous degeneration is a rare cause of enhancement on MR images, as is progressive neurological deficit in a patient with an epidermoid. The combination of subtotal resection, external-beam radiotherapy, and stereotactic radiosurgery may be useful for local tumor control but the long-term prognosis is guarded.     

Intracerebral epidermoid tumor: a case report and review of the literature

BACKGROUND: Intracerebral epidermoid cysts are rare lesions and may account for only 1.5% of intracranial epidermoid tumors. Cell entrapment from the mesectodermal origin of the neural crest within the primitive cerebral hemisphere may lead to the formation of such rare intracerebral lesions. METHODS: An intracerebral epidermoid cyst located in the right temporal lobe that was surgically treated is presented. The origin, clinical course, radiological features, and surgical treatment of such uncommon lesions are discussed based on a review of the literature.RESULTS: Intraoperative findings revealed an epidermoid tumor. The postoperative course was uneventful and the patient was discharged with no neurological deficits. On long-term follow-up (2 years), there were no signs of recurrence. CONCLUSIONS: Truly intracerebral epidermoid tumors are rarely found. Cells originating from mesectodermal lines may migrate and remain trapped within the primitive cerebral hemisphere while the neural tube is closing, leading to the formation of such intraparenchymal epidermoid tumors. Accurate preoperative diagnosis can be very difficult due to the radiological similarities to other common intracerebral cysts (e.g., astrocytomas or gliomas). Magnetic resonance imaging (MRI) studies, especially with diffusion-weighted images, allow greater accuracy in the preoperative differential diagnosis. Radical surgical removal should be attempted, but a less aggressive surgical strategy should be considered if there is strong adherence between the tumor capsule and the brain tissue, particularly in eloquent areas.     

Epidermoid cyst traversing the pons into the fourth ventricle. Case report

A rare case of epidermoid cyst traversing the brain stem is presented. Computed tomography revealed the mass within the fourth ventricle; magnetic resonance imaging showed the dumbbell-shaped mass extending from the prepontine cistern to the fourth ventricle. Despite the high mortality and morbidity of brain stem epidermoid cysts in the literature, the tumor was successfully removed. The advantages of magnetic resonance imaging over computed tomography are described.     

MRI of epidermoid cyst of the conus medullaris

STUDY DESIGN: A case report of an epidermoid cyst in the conus medullaris with characteristic magnetic resonance imaging (MRI) findings. OBJECTIVE: To describe an epidermoid cyst in the conus medullaris with characteristic MRI findings and point out these findings that correlated well with histologic findings. SETTING: Taiwan. CASE REPORT: A 49-year-old man who suffered from pain and weakness in his right leg for 16 years. MRI showed a heterogeneous signal mass in the conus medullaris. The hyperintense signal area within the lesion may be attributed to the keratin content, whereas the hypointense signal area was related to calcification and soft-tissue component. The histologic findings were compatible with an epidermoid cyst. CONCLUSION: Epidermoid cysts in the conus medullaris must be considered in the differential diagnosis of mass lesions with long duration of related symptoms. The characteristic MRI findings are useful to differentiate epidermoid cysts from other tumors.     

Intramedullary epidermoid cyst: preoperative diagnosis and surgical management after MRI introduction. Case report and updating of the literature

Many patients with spinal tumours of developmental origin do not receive preoperative diagnosis and the surgical management, especially as for capsule resection, is often unplanned. Like other uncommon tumours, the intramedullary epidermoid cyst is often an operative or histological finding. Since magnetic resonance imaging (MRI) introduction, evidence has accumulated that they may be preoperatively suspected. In reporting the case of a young patient with a T3-T4 intramedullary epidermoid cyst, the authors present an overview of the clinical, radiological and surgical aspects of such tumors and review the latest literature in which MRI and microsurgical excision were performed. Despite the introduction of new diagnostic and surgical instrumentation, the preoperative diagnosis and surgical management of such tumours need further discussion.     

Spinal intramedullary ependymal cyst: a case report.

BACKGROUND: Spinal intramedullary ependymal cysts are extremely rare. Only seven pathologically proven cases have been reported in the literature. METHOD: We present an 18-month-old female with thoracic spinal intramedullary ependymal cyst that was diagnosed pathologically. RESULTS: Histological diagnosis was made by light microscopy after immunostaining. After partially removing the cyst wall and establishing communication between the cyst and the subarachnoid space, the patient improved neurologically. CONCLUSIONS: For spinal intramedullary ependymal cyst we recommend diagnosis by MR imaging without myelography, then enucleation of the cyst, if possible. Otherwise, we remove the cyst wall as much as possible and create adequate communication between the cyst and the subarachnoid space.     

Intramedullary neurenteric cyst with a false mural nodule: case report

OBJECTIVE AND IMPORTANCE: Spinal neurenteric cysts are rare congenital lesions that may occur either alone or in the context of a complex malformative disorder including typical vertebral and cutaneous abnormalities. The interest of the case of a spinal neurenteric cyst described here lies in its rare intramedullary location and in the false mural nodule image on the preoperative magnetic resonance imaging scan. A further distinctive feature is the association with a cleft spinal cord. CLINICAL PRESENTATION: A 28-year-old woman presented with a 2-year history of progressive paraparesis and urinary retention. A magnetic resonance imaging study disclosed a T8-T9 intramedullary cystic lesion with a mural nodulelike formation on the posterior face. INTERVENTION: A posterior midline myelotomy exposed a cystic lesion that had translucent walls and contained a milky fluid. No mural nodules were found. Once the cyst had been emptied, a collateral finding was a cleft that was clearly observed in the anterior spinal cord. The histological diagnosis was a neurenteric cyst. Retrospectively, the nodular lesion found on the preoperative scan was attributed to mucinous clots deposited at the bottom of the cyst. CONCLUSION: This case report demonstrates that neurenteric cysts can vary widely in radiological appearance, depending on the contents of the cyst. These differences become especially important if the associated stigmata are lacking and the preoperative diagnosis rests on magnetic resonance imaging scan appearance alone.