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.     

Bilateral epidermoid cysts of the testicle.

An 11-year-old boy presented with bilateral testicular masses that consisted of hyperechoic centers with hypoechoic rims on ultrasound. Surgical exploration revealed bilateral epidermoid cysts. To our knowledge this is the first reported case of bilateral epidermoid cysts in a child. Recognition of this entity requires surgical confirmation but it may obviate removal of the testicle.     

Epidermal cysts of the cerebellopontile angle

The authors describe six cases of epidermoid cysts of the cerebellopontine angle. They insist on the progressive development, the variable duration and the polymorphism of the symptomatology. The imaging techniques relevant to diagnosis are reviewed. Among these computerized cisternotomography and N.M.R. are the methods of choice. It is suggested that surgical removal should be total whenever possible because of the risk of recurrence of these cysts but with great caution not to cause a vital or functional damage of the brain stem.     

Intradiploic epidermoid cysts of the skull: Report of 10 cases and review of the literature

Intradiploic epidermoid cysts, fairly uncommon lesions in neurosurgical practice, are, as a rule, benign and slow-growing. Some attain great size, producing major neurological signs. Correct radiological assessment and complete removal of the tumour and its capsule are essential for adequate surgical treatment and good long-term prognosis. We report ten cases of intradiploic epidermoid cysts of the skull, three of which were giant lesions and one malignant, and analyze the clinicopathological and radiological features and treatment of these lesions in the light of the most important published data.     

Transpetrosal extreme lateral suboccipital approach for extensive posterior fossa epidermoid cyst--case report.

A new transpetrosal extreme lateral suboccipital approach was adopted to totally remove an extensive posterior fossa epidermoid cyst in a 36-year-old male. The pathological behavior of intracranial epidermoid cysts may impose surgical problems during removal of the tumor. However, planning based on neuroimaging allows optimum access to tumors and microsurgery achieves safer and more complete removal.     

Epidermoid cysts of the cavernous sinus

BACKGROUND: Epidermoid cysts involving the cavernous sinus are rare lesions. They can be divided into 3 different categories: extracavernous, interdural, and true intracavernous. METHODS: This classification of cavernous epidermoid cysts is correlated with the extent of tumor resection and the clinical outcome in reported cases here and elsewhere. RESULTS: Patients with cysts invading or compressing the cavernous sinus present with an excellent functional recovery after surgery independent of the extent of tumor resection. In cases of interdural cysts, there is often an adherence of the tumor capsule to the third and fourth cranial nerves. Therefore, in this group, subtotal resection is a safe strategy to prevent surgical morbidity. However, this policy may lead to a higher incidence of tumor recurrence in these cases. In true intracavernous lesions, a higher rate of total removal is possible despite the increased occurrence of internal carotid artery encasement and cranial nerve displacement. CONCLUSION: Postoperative outcome with preservation of neural and vascular structures in cavernous epidermoid cysts is more likely related to surgical strategy than to tumor localization. Independent of the different tumor origins in these cases, a subtotal resection is usually sufficient to achieve postoperative improvement of the symptoms without additional morbidity.     

Intramedullary epidermoid cysts of the spinal cord. Case report.

Epidermoid cysts are tumors familiar to neurosurgeons, but intramedullary epidermoid cysts are rare. The authors report the case of a 6-year-old girl presenting with progressive paraparesis. A midthoracic intramedullary mass was revealed on myelography and magnetic resonance (MR) imaging and confirmed as an intramedullary epidermoid cyst at surgery, at which time the cyst was removed. This is the fourth report documenting a purely intramedullary epidermoid cyst occurring in a child. The pathology and etiology, epidemiology, clinical features, radiology (including MR image characteristics), and surgical treatment of such rare intramedullary benign tumors are discussed. Magnetic resonance imaging reduces the delay in diagnosis of spinal cord tumors but should be guided by clinical judgment.     

Prothesenrandknoten

As there are 47,000 leg amputees in Germany, the avoidance and sucessful treatment of epidermoid cysts is very important. A random sample of 100 leg amputees using prostheses (92 men, 8 women) were questioned according to a standard protocoll. Epidermoid cysts had been experienced at least once by 68% of those whose leg had been amputated above the knee and by 80% of those with below-knee amputations. In above-knee amputees all epidermoid cysts were in the inguinal region, while in below-knee amputees they were found exclusively in the popliteal fossa. In older patients epidermoid cysts were significantly less frequent than in younger patients. They tend to be more frequent in patients who do physical work, those who have problems with stability and those whose protheses are a poor fit. In above-knee amputees the newer sagitally oriented oval shape of the shaft of the prothesis has led to markedly fewer epidermoid cysts than were seen with the transversely oriented oval shaft. Epidermoid cysts in leg amputees are predominantly treated by conservative techniques. Crucial importance attaches to improved fit of the prothesis, smooth inner lining of the prothesis, consistent skin hygiene and local, and if necessary also systemic, anti-infectious measures. Surgical removal of epidermoid cysts in leg amputees should be reserved for exception cases only.     

Giant epidermoid cysts of the skull

The authors describe two cases of giant intradiploic epidermoid cysts of the cranial vault and a case of a large epidermoid arising from the frontal sinus and extending intracranially. The aetiology of epithelial cysts is reviewed. The characteristic radiological and CT scan appearances of diploic epidermoid cysts are described, their clinical presentation and surgical management briefly discussed.     

Transtentorial epidermoid cysts

Summary Epidermoid cysts may occur simultaneously above and below the tentorium. Eleven patients with involvement of both infra- and supra-tentorial cisterns are presented. In two cases the epidermoid, located mainly in the cerebello-pontine angle, spread into the middle cranial fossa; in three the epidermoid extended from the parasellar cisterns to the posterior cranial fossa; in six patients the epidermoid, enlarging the tentorial notch, occupied extensively both cranial fossae.The surgical approach was influenced both by the experience of the surgeon and by the main extension of the epidermoid. Total removal was feasible in two patients only but only one of the eleven patients had a recurrence of the epidermoid. The long term results appear to be unrelated to the size of the epidermoid and to the choice of approach.     

Dysembryogenetic spinal tumours in adults without dysraphism

The authors report 15 patients with spinal intradural dysembryogenetic tumours with clinical onset in adult age in the absence of clinical and radiological signs of dysraphism. The series includes seven lipomas, four epidermoid cysts, three dermoid cysts and one teratoma. The tumour site was the thoracic region in three cases, the lumbar cord and conus in six, the cauda equina in four and the filum terminale in two. Among 14 patients operated upon, the surgical removal was complete in eight cases, subtotal in two and partial in four. Recurrence was noticed only in one subtotally resected thoracic epidermoid cyst. Magnetic resonance imaging allows a precise diagnosis of these lesions, mainly of small lipomas and dermoids of the conus and filum, where a tethered conus is responsible for clinical symptoms. Dysembryogenetic spinal rumours that become symptomatic in adult age may require surgical treatment. Reduction of the mass and release of any associated tethered neural elements are the goal of surgery for spinal lipomas, whereas epidermoid and dermoid cysts require a more radical treatment. However, even partial resections to avoid neural damage result in a good clinical outcome and very low risk of recurrence.     

Operative treatment of intracranial epidermoid cysts and cholesterol granulomas: report of 21 cases

Thirteen patients had operations to remove intracranial epidermoid cysts, and long-term follow-up was obtained. Total or nearly total tumor and capsule removal was accomplished in 7 patients during the initial operation. This group required no additional operations. The other 6 underwent subtotal tumor removal and required multiple operations for symptomatic tumor recurrence. This latter group had a poorer neurological outcome. We conclude that initial total or near-total tumor resection is highly desirable in treating intracranial epidermoid cysts, particularly in physiologically young individuals. Five patients were followed after operations to remove pure cholesterol granulomas of the petroclival bone, and 3 additional patients were followed after operations to remove tumors with combined histopathological features of both an epidermoid cyst and cholesterol granuloma. Four patients with some component of a cholesterol granuloma had concurrent middle ear infections, and 4 did not. Intracranial subtotal excision and drainage of these lesions was the initial operative management in 7 patients, 5 of whom have required multiple operations for symptomatic tumor recurrence. Therefore, we conclude that subtotal excisional procedures for tumors with histopathological features of cholesterol granulomas are not usually successful in establishing long-term cures. Total excision, as recommended for epidermoid cysts, tumors frequently confused with cholesterol granulomas when occupying the petroclival region, may be warranted for these tumors as well. We postulate that when a congenital epidermoid cyst occurs in the petroclival bone, it may incite a local inflammatory reaction, producing lesions which have the histological features of both epidermoid cysts and cholesterol granulomas.     

Pineal epidermoid cysts: diagnosis and management.

OBJECT: The results of surgical treatment of epidermoid cysts of the pineal region in six cases are presented. METHODS: Six patients with pineal epidermoid cysts underwent surgery at the Institute of Neurosurgery "N. N. Burdenko," in Moscow, during the period 1976 to 1995. The duration of the patients' preadmission clinical history varied from 6 months to 2 years (average 1.4 years). Headache, diplopia, and vertigo were the most frequently occurring symptoms. Neurological examination demonstrated papilledema, impaired pupillary reaction, ataxia, and long-pathways deficit; Parinaud's syndrome was found in only one case. Computerized tomography and magnetic resonance imaging constituted the primary diagnostic tools. Surgery was performed using either an infratentorial-supracerebellar approach (two cases) or an occipital-transtentorial approach (four cases). CONCLUSIONS: Surgical results can be excellent if a removal, as extensive as possible, is performed using either the supracerebellar or occipital-transtentorial approach.     

Epidermoid cyst of the corpus callosum]

Epidermoid cysts are most commonly located in the cerebellopontine angle and the parasellar regions. The authors report a case of an epidermoid cyst of unusual location: it arises in the midline and involves the body of the corpus callosum with interhemispheric exophytic growth. Reviewing the literature they did not find any similar report. C.T. scan and M.R.I. are the methods of choice for establishing the diagnosis and differentiating between epidermoid cyst, dermoid cyst, lipoma and arachnoid cyst. M.R.I. is highly sensitive and is especially useful in determining tumour extension, particularly in the sagittal and coronal planes, essential for surgical treatment planning. Treatment should be complete surgical resection in order to avoid recurrence and chemical meningitis.     

Giant intradiploic epidermoid cysts of the skull. Report of two cases

The authors describe two cases of giant intradiploic epidermoid cysts of the cranial vault in which there was massive intracranial extension causing signs of neurological involvement. The very slow growth and the benign histological nature of these tumors explain their long preoperative evolution and the mild neurological signs in some cases. Roentgenographic and computerized tomography findings permit a correct diagnosis. Complete removal of these cysts and their capsules can be easily accomplished, despite their large size. Total removal of these cysts is associated with a very good long-term prognosis.     

Giant earlobe epidermoid cyst

Epidermoid cysts represent the most common cutaneous cysts. They are usually small and benign; however, sometimes they can grow to giant epidermoid cists, and occasionally malignancies develop. Giant epidermoid cysts at the earlobe have never been described but in other locations. We describe a case of a giant epidermoid cyst at the earlobe, a location where such a large cyst has never been reported before. The mass was completely resected and the wound of the pedunculated base was sutured with four stitches of nylon 5/0. Histopathology confirmed the presumptive diagnosis of an epidermoid cyst. Six months after the resection, the patient did not have any relapse of the epidermoid cyst. The earlobe is a potential location for giant epidermoid cysts. Although the clinical diagnosis could be enough, due to the possibility of malignancy and to ensure appropriate diagnosis, we consider that all cysts should be sent to the anatomic pathology laboratory for histological evaluation.     

Intraspinal epidermoid and dermoid cysts. Surgical results of seven cases

Seven cases of intraspinal inclusion cysts, surgically treated between 1970 and 1984, are reported in this paper. Four were epidermoid cysts and three dermoid. Two cysts were located in the thoracic spine (one dermoid and one epidermoid) and five in the lumbosacral region (three epidermoid and two dermoid). Associated spinal dysraphia was noted in five cases. Congenital dermal sinus was found in two cases, spina bifida cystica in one, spina bifida occulta in one, and diastematomyelia in one case. The average postoperative follow-up period was 5 years. No neurological symptoms were found in the five patients who received early surgical treatment, but for the two patients whose operations were late, there were some residual neurological deficits. Therefore, early and accurate diagnosis and radical surgical extirpation are emphasized.     

Paramedian hourglass epidermoid cysts extending in the middle and posterior cranial fossa

Four cases of large paramedian hourglass epidermoid tumors extending both in the middle and posterior cranial fossa are reported and other 16 cases from the literature are reviewed. The length of the clinical history and the triviality of neurological symptoms in spite of the size of the tumor are emphasized. CT scanning well documents the extension of these epidermoids and usually consents to differentiate them from the arachnoid cysts. The subtemporal transtentorial approach is more advisable, although a two-stage operation by subtemporal and suboccipital route can be necessary in some cases. Nevertheless the difficulties and the risk of the surgical treatment make very hard the complete removal of the tumor in most cases.     

Epidermoid cyst of the brain stem. Case report

Brain stem epidermoid cysts are extremely rare lesions. Only nine cases have been reported. Management of the epidermoid cyst is decompression of cyst contents and removal of cyst capsule. But in some cases resection of the cyst may result in a poor outcome because of cyst wall adhesion into the brain stem.     

Epidermoid cyst of the fourth ventricle

The case of a 52-year-old patient with a fourth ventricle epidermoid is reported. The initial presentation included long-standing headaches, progressive anomalies of gait and slight impairment of mentation. CT showed a hypodense mass enhancing peripherally after contrast infusion. Brain auditory evoked responses (BAER) showed asymmetric increased latencies. At operation, total removal of an extensive fourth ventricle epidermoid was achieved. A delayed meningitis occurred postoperatively. Physical examination was normal at the 2 year follow-up and BAER were improved. The etiological and clinical features of fourth ventricular epidermoids are briefly reviewed. The diagnostic value of CT is emphasised but the possibility of CT-dense epidermoid cysts deserves a special mention. Total removal of the neoplasm is the theoretical aim of operative treatment, but this purpose may be harmful when the ventricular floor is involved by the capsule.