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Huge intradiploic epidermoid cyst
Abstract
A 60-year-old man presented with an occipital mass under the scalp and complained of headache, nausea, and dizziness. Magnetic resonance imaging showed a well-defined mass in the occipital scalp extending from the scalp through the cranium and several centimetres into the posterior fossa. There were well-defined margins in the deep portion and the mass was totally removed. Histological examination showed that the cystic structure was lined by squamous epithelium containing laminated keratin material. The pathological findings were consistent with the diagnosis of an epidermoid cyst. The patient was discharged free of symptoms.
... Intradiploic epidermoid cysts are rare, non-malignant lesions which arise between the two tables of cranial bones . [1] They are encountered more often in the frontal and parietal regions. They may often reach an enormous size without producing neurological symptoms. ...
... Hydatid cysts lack bone erosion and calcifications that are seen in epidermoids. Diffusion weighted MR imaging can be used to differentiate arachnoid cysts from epidermoid cysts whereas arachnoid cysts have low signal intensity [1][2][3]. ...
The incidence of cranial epidermoid cysts is less than 1% among all cranial tumor’s was first described by Muller
in 1838 such cysts that develop in record to the spongy cancellous bone separating the inner and outer layers of the
cortical bone of the skull. They are derived from ectodermal vestiges that stay within the cranial bones during embryonic
development. Having a male performance, they can be located in anywhere of the skull.
... The post-operative course of the patient was uneventful and the patient was doing well at follow-up. Epidermoid cysts are thought to result from a displacement of ectodermal tissue during the third to fifth week of embryogenesis [2]. Epidermoid tumours account for less than 1% of the cranial tumors. ...
... The most common presentation of the calvarial epidermoid is a long-standing, asymptomatic lump on the head [5]. Surgical excision is the preferred treatment for diagnosis, to relieve any symptoms and for cosmetics with the goal being total removal of the capsule [1][2][3]5]. Spontaneous regression of epidermoids and dermoids of the calvarian bones has been reported during early childhood [6]. ...
... Other studies have documented cases with unusual features, such as intracranial extension, spontaneous fistulization, and even coexistence with other pathologies like hematomas or craniosynostosis, as seen in cases by Choo et al., Nakamoto et al.,and Yildiz et al. [ 8,10,20 ]. Authors such as Hasturk et al., Zheng et al., and Turkoglu et al. have reported on giant cysts that posed surgical challenges due to their size and proximity to critical anatomical structures [11][12][13]. Unique presentations, such as hemorrhagic cysts and those with orbital or intraorbital extension, have also been reported, adding to the variability in clinical manifestation of these cysts [ 21,23,27 ]. Rarely, they can mimic other conditions, such as metastases or fibrous dysplasia, complicating diagnosis as illustrated by Dhull et al. and Mandelbaum et al. [ 15,18 ]. ...
Intradiploic epidermoid cysts are rare, benign tumors, accounting for less than 1% of all intracranial tumors. Due to their scarcity, we are reporting a case of an intradiploic epidermoid cyst and reviewing several similar cases in the literature to provide a better description of this tumor and analyze its clinical and radiological features. We conducted a search on the PubMed database for studies published between January 2010 and February 2023, including studies of nontraumatic and noniatrogenic calvarial cysts. We found a total of 34 studies and 41 patients, with an average patient age of 42.5 years. Common symptoms included painless masses, headaches, and seizures. The occipital, frontal, and parietal regions were the most common locations for these tumors. Some patients experienced complications such as otitis media, infection, or extension into the orbit and brain. Surgical resection was performed in all cases except for 2 patients with contraindications to surgery. Histological examination confirmed the diagnosis of an intradiploic epidermoid cyst. These cysts are rare skull lesions and can potentially cause neurological deficits due to their mass effect. If incompletely resected, they may also undergo malignant transformation. No single institution has accumulated enough cases to describe this rare tumor accurately. Therefore, through this review, we aim to highlight this tumor's epidemiological, clinical, and radiological features.
... Se ha reportado en la literatura que el tratamiento definitivo de los quistes epidermoides es la resección completa de la lesión para así evitar la recurrencia y el riesgo de meningitis aséptica, por otro lado, Kakane V et al. reportan que puede considerarse una resección conservadora de la lesión si esta se encuentra en proximidad a estructuras neurovasculares o parénquima cerebral vital, dado que los quistes epidermoides presentan un crecimiento lento (19). El pronóstico a largo plazo es excelente si se realiza la resección total de la lesión y su cápsula (20). En nuestro caso se logró la resección total de la lesión sin complicaciones postquirúrgicas. ...
Los quistes epidermoides son lesiones congénitas benignas, de lento crecimiento, que representan el 1% de todos los tumores intracraneales. En un 25% tienen localización intradiploica y excepcionalmente infratentorial. Presentamos a un paciente masculino de 7 años de edad, en quien se observó aumento de volumen en región parietal izquierda, acompañado de cefalea de aparición insidiosa, holocraneana, de leve intensidad. Como antecedentes personales refiere diagnóstico de migraña con aura y epilepsia caracterizada por crisis parciales complejas. Los estudios imagenológicos reportaron una lesión ocupante de espacio en región parietal izquierda, de forma ovoidea, de característica lítica y un tamaño de 8,8x17mm de diámetro. Fue llevado a mesa operatoria para resolución quirúrgica de su patología. Se logró la excéresis total de la lesión y el estudio histopatológico reportó quiste epidermoide.
Intracranial epidermoid tumors are rare, potentially curable, benign lesions that are sometimes associated with perioperative complications, and tend to recur if not completely removed. Histologically benign epidermoid tumors may also develop into highly malignant tumors. This study evaluated on 28 cases of intracranial epidermoid tumor treated over a 13-year period by radical resection with microneurosurgical techniques. The majority of patients underwent computed tomography and/or magnetic resonance imaging within the first 24 hours postoperatively to confirm the results of surgery. Radical surgical resection was achieved in 21 of the 28 cases, and there was no operative mortality. The most common postoperative complication was transient paresis of various cranial nerves. During a mean follow up of 6 years, only one tumor became malignant. Radical surgical resection should be the goal in treating these benign lesions, but if not possible, every effort should be made to minimize the amount of tumor tissue that remains.
A 55-year-old woman presented with an epidermoid cyst extending to the cerebellum manifesting as headaches and pain in the left eye. Magnetic resonance imaging showed an intradiploic part with ring enhancement and an intracerebellar part. Intraoperative inspection revealed erosion of the occipital bone and defective dura mater. The tumor was located both epidurally and subdurally and the cyst consisted of pearly white keratin. The tumor was totally removed and the patient was discharged with no neurological deficit. The intradiploic part of the tumor formed the body and the intracerebellar part was caused by inflammatory reaction, which resulted in the atypical enhancement of the intradiploic part.
Forty-three patients with intracranial, intradural dermoid (8) and epidermoid (35) tumors underwent radical surgical resection utilizing strict microneurosurgical technique. The average age was 37.3 years for the patients with epidermoid tumors and 36.2 years for the patients with dermoid tumors. The male to female ratio was 3:2 for the epidermoid group and 3:1 for the dermoid group. Common clinical presentations included cerebellar dysfunction, cranial nerve impairment, and seizures. Typically, computed tomography scans revealed the epidermoid tumors (30 cases studied) as nonhomogeneous hypodense lesions with irregular borders and without contrast enhancement. The dermoid tumors (7 cases studied) had a similar appearance, but with a wider range of attenuation values. Magnetic resonance imaging findings for the epidermoid tumors (6 cases studied) consisted of increased T1 and increased T2 relaxation times. Supratentorial tumors were excised by the pterional (frontosphenotemporal) approach, mesencephalic tumors by either a supratentorial posterior interhemispheric transtentorial approach or an infratentorial/supracerebellar method, and posterior fossa tumors by either a medially or laterally positioned suboccipital osteoplastic craniotomy. One epidermoid tumor and one dermoid tumor were considered to be subtotally resected because of dense adherences left attached to vital structures; the remaining 41 tumors were completely excises. The most frequent complications were aseptic/chemical meningitis and transient cranial nerve palsies. There were no perioperative deaths. Mean follow-up was 5.2 years. Eighty-six percent of patients reported good to excellent results. No patient had experienced symptomatic or radiographic evidence of recurrence. These results suggest that although dermoid and epidermoid tumors tend to cross anatomical boundaries via the subarachnoid system, in the majority of patients tumors can be resected in their entirety with relatively low morbidity by utilizing microneurosurgical techniques.
: An unusual case of intradiploic epidermoid tumor is reported. In this case, a soft mass located over the parietal bone was noted shortly after birth. The mass gradually enlarged. The tumor underwent malignant change after several excisions and repeated inflammations. The patient was successfully treated with extensive excision, radiotherapy, and chemotherapy. Compiling a summary of reported cases with a tabulation of pertinent information, we discuss the incidence, pathogenesis, prognosis, and therapy of malignant intradiploic epidermoid. (Neurosurgery 16:252-256, 1985)
Copyright (C) by the Congress of Neurological Surgeons
Extra-axial posterior fossa masses can be diagnosed reliably by computed tomography (CT) in most cases. Acoustic and trigeminal neurinomas, meningiomas, cholesteatomas, and other extra-axial masses can usually be distinguished from intra-axial masses by asymmetric widening of the basal subarachnoid spaces, bone destruction, continuity of the tumor mass with the tentorium or bone, and more sharply defined margins. Multiple-cut study of the posterior fossa improved visualization of the fourth ventricle and basal cisterns. Interpretation of cisternal changes in association with changes in the fourth ventricle and abnormal attenuation coefficients permits accurate diagnosis of posterior fossa masses.
The authors report on the clinical data, operating technique, postoperative complications, and late results in a series of 31 epidermoid and 21 dermoid cysts of the central nervous system.
Forty-three patients with intracranial, intradural dermoid (8) and epidermoid (35) tumors underwent radical surgical resection utilizing strict microneurosurgical technique. The average age was 37.3 years for the patients with epidermoid tumors and 36.2 years for the patients with dermoid tumors. The male to female ratio was 3:2 for the epidermoid group and 3:1 for the dermoid group. Common clinical presentations included cerebellar dysfunction, cranial nerve impairment, and seizures. Typically, computed tomography scans revealed the epidermoid tumors (30 cases studied) as nonhomogeneous hypodense lesions with irregular borders and without contrast enhancement. The dermoid tumors (7 cases studied) had a similar appearance, but with a wider range of attenuation values. Magnetic resonance imaging findings for the epidermoid tumors (6 cases studied) consisted of increased T1 and increased T2 relaxation times. Supratentorial tumors were excised by the pterional (frontosphenotemporal) approach, mesencephalic tumors by either a supratentorial posterior interhemispheric transtentorial approach or an infratentorial/supracerebellar method, and posterior fossa tumors by either a medially or laterally positioned suboccipital osteoplastic craniotomy. One epidermoid tumor and one dermoid tumor were considered to be subtotally resected because of dense adherences left attached to vital structures; the remaining 41 tumors were completely excised. The most frequent complications were aseptic/chemical meningitis and transient cranial nerve palsies. There were no perioperative deaths. Mean follow-up was 5.2 years. Eighty-six percent of patients reported good to excellent results. No patient had experienced symptomatic or radiographic evidence of recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)
Epidermoid and dermoid sequestration cysts are a form of heterotopia due to displaced or implanted ectoderm. They are unrelated to benign cystic teratomas. Congenital cysts occur following inclusion of epidermis at time of embryonic closure of the neural groove or of epithelial fusion lines in the head and neck and about the anus. If only epidermis is included, the cyst is an epidermoid. A dermoid cyst is formed if dermal elements are also present. If either communicates with the skin, a dermal sinus is created.Acquired cysts may be post-traumatic or iatrogenic. The former generally occur in the phalanges while iatrogenic cysts follow surgery or spinal puncture.
An unusual type of congenital subgaleal cyst, either dermoid or epidermoid, was found in 21 Nigerian infants. This entity was located in the midline of the scalp, anywhere from above the nasion to the inion, and occupied the subgaleal space. These cysts were noted soon after birth and gradually enlarged. They had no intracranial extension and were easily excised intact. Clinically, radiologically, and histologically they were similar to congenital inclusion dermoid cysts of the anterior fontanel. The histology and some of the peculiarities of these cysts are described. Although most of these cysts occur at the anterior fontanel, they can occur anywhere in the midline, and the subgaleal space of the anterior fontanel is not the exclusive site, as has been claimed by previous authors.
A case of epidermoid cyst of the skull was investigated by computed tomography. In toto excision permitted a correlation with anatomical specimen findings and demonstrated a good correspondence.
We report a case of a 65-year-old man with intradipolic epidermoid cyst of the frontal bone. The patient presented with headache and swelling of the forehead. Skull x-ray showed massive destruction of the frontal bone. Magnetic resonance imaging (MRI) revealed an epidural mass without any encroachment of the dura mater. The cystic tumor was removed followed by cranipolasty with methylmethacrylate. Four years later, the tumor recurred in the same region. At the second surgery the bony defect was again replaced with methylemthacrylate plate. Four years after the second operation, the tumor reemerged with some subcutaneous fluid retention. At the third surgery, the remaining tumor capsule was removed as much as possible except for that part which adhered to the superior sagittal sinus. The authors discuss the relationship of tumor recurrence with utilization of methylmethacrylate for cranioplasty in this type of tumor.
A case of diploic epidermoid in the right parietal bone was presented. A 66-year-old male was admitted to our hospital because of aphasia. Plain skull radiogram showed an osteolytic lesion in the right parietal bone. Computed tomography revealed a mass with destruction of the parietal bone extending from an inner to an outer table. The mass was shown by MR imaging as hypointensity on the T1-weighted image and as hyperintensity on the T2-weighted image. The hyperintensity became more remarkable by using a diffusion technique. Based on these findings, a preoperative diagnosis of right parietal epidermoid was made. The mass was totally removed and diagnosed pathologically as epidermoid. It was concluded that diploic epidermoid invading intradurally could be differentiated from other osteolytic tumors by using MR diffusion-weighted image.
Cerebellopontine angle epidermoid tumour generally has a typical appearance with conventional MRI sequences. The lesion is irregular in shape and gives slightly higher signal than cerebrospinal fluid on T1- and T2-weighted images, with a characteristic marbled inner pattern on T1-weighted images. Diffusion-weighted imaging (DWI) can be useful for the diagnosis of an atypical epidermoid tumour. Our case report illustrates the usefulness of DWI for postoperative assessment of residual foci of tumour. The specific appearance of an epidermoid tumour is illustrated, with emphasis on apparent diffusion coefficient (ADC) measurements.
Epidermoid cysts are uncommon, benign and slow-growing lesions. They may often reach an enormous size without producing neurological symptoms. Intradiploic epidermoid cysts are nearly 25% of all epidermoids. They are derived from ectodermal remnants that stay within the cranial bones during embryonic development. Intradiploic epidermoid cysts can be located in any part of the skull. Two of our four cases were located in left occipital bone and the others were in the frontal bone. These tumours can occur at any age from the first to the seventh decade of life. Our cases had a mean age of 38.75 years (19-55 years). They may reach great sizes before the initial diagnosis and may produce major neurological signs. For good long-term prognosis, correct radiological assessment and complete removal of the tumour with its capsule are essential. We report four cases of intradiploic epidermoid cysts of the skull and analyse the clinical, radiological features and treatment of these lesions in the light of relevant literature.
Epidermoid cysts are the most common intracranial embryonal tumor, although they account for only 1% of all intracranial tumors. Epidermoids often spread into several intracranial compartments. Thorough preoperative surgical planning is imperative for safe epidermoid removal. This paper discusses the neurotologic management of intracranial epidermoid cysts
Retrospective chart review.
A database search revealed 10 patients with diagnosis of intracranial epidermoid cysts between January 1, 1971 and December 31, 2003 at our institution.
Six males and four females with ages ranging from 18 to 54 years of age underwent surgery between September 1, 1971 and November 4, 2003. The average tumor size was 3.9 cm; six originated in the cerebellopontine angle and four in the petrous apex. Six patients had a translabyrinthine approach to the tumor, two with additional transcochlear exposure. Two patients had tumors removed by way of the middle fossa approach and one through the suboccipital approach. Multiple cranial nerves were involved by tumor in all patients, including nerves III through XI. The internal carotid artery was involved by tumor in four patients. Multiple cranial nerve deficits were seen preoperatively, and facial weakness was the most common new deficit postoperatively. Eight patients required intradural access for complete tumor removal. Seven had complete tumor removal. Headaches were the most common complication. One patient had seizures postoperatively, and another had a malignant epidermoid, which resulted in death.
Intracranial epidermoid cysts require complex surgical planning. They involve multiple cranial nerves and vascular structures. Complete resection is frequently possible with minimal new cranial nerve deficits.
Epidermoid cysts are rare disontogenetic tumoral lesions in the cranial compartment (1% of all intracranial tumors) and are usually located in relation to the cranial sutures; the intradiploic location is the less frequent. We report a clinical case in which the post-traumatic etiology of a frontal intradiploic epidermoid is considered. The histological findings revealed the presence of an epidermoid tumor associated with a typical foreign body inflammatory reaction, after repeated head traumas at the site of the tumour. This is the first case in whom the association between this tumoral lesion and a tissue inflammatory reaction due to foreign bodies is demonstrated.
Diagnostic neuroradiology. Williams & Wilkins
- Taveras J M Wood
Taveras J.M., Wood E.H. Diagnostic neuroradiology. Williams & Wilkins, Baltimore 1976, pp. 122-129.
Epidermoid, dermoid and neuroenteric cysts
- M K Gumerlock
Gumerlock M.K. Epidermoid, dermoid and neuroenteric cysts.
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Elsevier B.V. 2004, pp. 1123.
Diagnostic neuroradiology
- J M Taveras
- E H Wood
Taveras J.M., Wood E.H. Diagnostic neuroradiology. Williams
& Wilkins, Baltimore 1976, pp. 122-129.