脊髓圆锥髓内表皮样囊肿的手术治疗

目的 探讨脊髓圆锥髓内表皮样囊肿的手术方式及其疗效。方法 对2001~2014年收治的27例不同形态的圆锥髓内表皮样囊肿进行分类,采取不同的手术方式进行治疗,并对预后予以随访评估。结果 所有病例均行手术治疗,其中单发囊肿23例,多发囊肿4例;25例行内容物全切,2例多发囊肿病例中有个别小囊肿未行切除;21例行囊壁电灼全切,5例行囊壁部分电灼切除。术前大小便功能障碍23例,脚部麻木不适9例,小腿或脚部无力7例;术后症状明显改善21例,3例小便功能障碍较术前略加重,4例脚部麻木较术前明显加重,1例脚部无力稍加重。术后随访19例,随访期限9个月~11年,平均4.2年;17例症状明显减轻;3例小便功能障碍加重者加重,2例恢复到术前水平;复发2例再次手术,术后恢复良好。结论 对于脊髓圆锥髓内表皮样囊肿,根据不同的肿瘤形态采用不同的手术方式能明显提高疗效,减少并发症,防止复发。     

Intracranial epidermoid cyst with proliferative folliculosebaceous epithelium: Report of a rare case and discussion on pathogenesis

Intracranial epidermoid cysts are rarely known to increase in size over a brief period. While malignant transformations of epidermoid cyst have been previously described, no reports to date have described rapid proliferation accompanied by mature hair follicles and sebaceous glands without malignant transformations. The present case involved a 71‐year‐old man who visited a local physician with disturbance of equilibrium. A mass lesion was detected at the left cerebellopontine angle and was subsequently removed. Histopathological diagnosis of this lesion was as an epidermoid cyst. Seventeen years after this initial surgery, worsened left hypoacusis, disorientation, eating disorder and gait disturbance appeared, and the patient visited the physician again. Local recurrence of the lesion was observed, and the patient was referred to our hospital for its removal. Intraoperative findings revealed a pearly white tumor, histopathologically diagnosed as an epidermoid cyst. Three years later, local recurrence was observed, and the cyst was again removed. Intraoperative findings revealed a partially pearly white tumor similar to the tumor observed during the second surgery, but the majority of the tumor was non‐shiny, ochre‐colored and suckable. Histopathological diagnosis was an epidermoid cyst with an epidermis‐like structure. Postoperative activities of daily living were independent thereafter, but from March of the following year, he began to experience increasing difficulty in walking and subsequently visited our hospital again. Tumor recurrence was observed and was removed again in November of the same year. Intraoperative findings revealed a tumor that was primarily ochre‐colored, non‐shiny, brittle and suckable. The histopathological diagnosis was folliculosebaceous epithelial proliferative lesion accompanied by an epidermis‐like epithelium and a differentiation into hair follicles and sebaceous gland,s and the tumor was determined to be an epidermoid cyst with proliferative folliculosebaceous epithelium.     

Giant intramedullary epidermoid extending from the brain stem to the upper thoracic spinal cord

Epidermoid cysts are benign tumors originating from ectoderm remnants. Most epidermoid cyst cases are intracranial. Spinal epidermoid cysts are uncommon and most of the reported cases are in the thoracic and lumbar spine. Occurrence of intramedullary epidermoid cysts in the cervical spine is extremely rare. An 18-year-old male with a giant intramedullary tumor extending from the brain stem to the upper thoracic spinal cord presented at our outpatient department. The patient underwent total excision of a silvery white lesion through a midline myelotomy. Histopathological examination was suggestive of an epidermoid cyst. We present a brief report of the case and discuss the relevant literature.     

Recurrent intrinsic brain stem epidermoid cyst

The authors report the case of a 14-month-old baby boy with an epidermoid cyst located entirely within the pons and medulla, without an exophytic component. The lesion was examined by computed tomography and magnetic resonance imaging. The child was operated upon three times after two recurrences of the lesion. A suboccipital, subtonsillar approach was used for the first and second procedures and a transtemporal approach for the last one. Excision was thought to be complete the first time, since a solid tumor was found and removed in a large cyst. The cyst wall was not identified. No tumor was found during the second procedure despite recurrence of the cyst, which was drained without an attempt to remove the cyst wall. Finally the cyst recurred with a large tumor in the cyst wall which was again totally removed. Consistent with the high mortality of brain stem epidermoid cysts in the literature, the child eventually died. The therapeutic problems, surgical options, and consequences are discussed.     

Spontaneously hyperdense intracranial epidermoid cyst

Intracranial epidermoid cysts are relatively rare tumors. These cysts are generally nearly isodense to CSF at CT and nearly isointense to CSF on T1W and T2W MR imaging. Spontaneously hyperdense epidermoid cysts are exceptional. We report the case of a 12-year-old girl with a spontaneously hyperdense epidermoid cyst of the posterior fossa presenting with focal neurological symptoms. The diagnosis was confirmed at histological examination.     

Hemorrhagic epidermoid cyst in a patient with generalized tonic clonic seizure

Epidermoid cysts account for approximately 1% of all intracranial tumors. Hemorrhage into an epidermoid cyst is extremely rare, and has only been reported a few times. To our knowledge, there are no reports of a hemorrhagic epidermoid cyst presenting with a first generalized tonic clonic seizure. We present a 68-year old female with an epidermoid cyst with intracystic hemorrhage who presented with a first time generalized tonic clonic seizure. When complicated with hemorrhage, the diagnosis of an epidermoid cyst is challenging and there is a potential for misdiagnosis.     

Epidermoid cyst mimicking an intrinsic brainstem tumor

ObjectiveTo describe an atypical clinical and radiological presentation of a brainstem epidermoid cyst in a child and to provide a review of the medical literature on brainstem epidermoid cysts in children.Material and methodReview of medical records and operative notes of an unusual case of a patient with a brainstem epidermoid cyst. MEDLINE literature search using the terms brainstem, epidermoid cyst and children.ResultsGross total resection of the cyst was achieved. The patient had an uneventful recovery.ConclusionEpidermoid cysts are rare tumors of the brain and children. The management of these tumors can be quite challenging. A good clinical and neuroradiological evaluation pre-operatively is fundamental for a successful surgical treatment. Surgical resection should be as radical as possible without putting the patient's neurological status into risk.     

Ectopic ventricular recurrence of an epidermoid cyst in the middle fossa.

We present the first case of ectopic ventricular recurrence of an epidermoid cyst in the middle fossa with confirming histological characteristics. A 51-year-old woman presented with an epidermoid cyst in the middle fossa and underwent complete resection. On the 6-month surgical follow-up MRI, a nodular lesion was detected in the frontal horn of the left lateral ventricle without evidence of recurrence at the primary site. The patient underwent transcortial tumor resection. Results of histological studies confirmed that the lesion was an epidermoid cyst similar to the primary lesion. This is a case report of the cerebrospinal fluid spread of an epidermoid cyst, which strengthens the case for special care at the time of surgery to prevent spread of the lesion.     

Spinal cord congenital epidermoid cyst (a dorsal case)

A 18 year-old man had a progressive paraparesis over a two months period due to a thoracic intramedullary epidermoid cyst. Surgical removal of the cyst was followed by a dramatic improvement. The frequency, the clinical manifestations and the congenital or acquired nature of the dermoid and epidermoid intraspinal cysts are reviewed. The possibilities and the limits of the surgical treatment of this rare intraspinal benign tumors are discussed.     

Intrasellar epidermoid cyst presenting as pituitary apoplexy

We herein present a patient with intrasellar epidermoid cyst presenting as pituitary apoplexy, who was operated upon with a trans-sphenoidal approach. The clinical presentation, radiological and histological differential diagnosis and type of surgical intervention for intrasellar epidermoid cysts are discussed.     

Intracranial germinoma in the pineal region arising after subtotal resection of epidermoid cyst: case report

We present an unusual case of a germinoma of the pineal region arising adjacent to an epidermoid cyst in a 16-year-old male. Initial imaging findings were classic for epidermoid cyst. The patient underwent two partial resections at an outside institution, each specimen demonstrating pure epidermoid cyst. Follow-up imaging over a period of 24 months showed an area of progressive contrast enhancement adjacent to the initial lesion, suggesting the development of a neoplasm. Given the area of contrast enhancement in addition to worsening headaches and visual changes, he underwent a third and final resection at our institution. Pathology revealed a mixed germ cell tumor with prominent germinoma component in addition to a well-differentiated epidermoid cyst. Details of his imaging and pathologic findings are presented, and possible explanations for these findings are explored, the most likely of which is lack of complete resection at the onset failed to identify the whole of the neoplasm. We conclude that pediatric epidermoid cysts of the pineal region should always receive close follow-up, particularly when total resection is not performed.     

Malignant transformation of epidermoid cyst

The paper presents a rare case of malignant transformation of an epidermoid cyst to the keratinizing type of squamous cell carcinoma. In a 47-year-old female patient with severe visual disturbances, MRI scans revealed a large supracellular tumor spreading to the third ventricle and medial portions of temporal lobes. The tumor was removed. A histological study of its biopsy specimen showed signs of both a benign process (epidermoid cysts) and its malignant epidermoid degeneration to the keratinizing type of squamous cell carcinoma. After 12-month combined therapy there was a continuous tumor growth with a significant dissemination within the base of the skull. The pathogenesis of epidermoid cysts, possible causes of malignant transformation, modalities of treatment and prognosis are briefly discussed.     

Electron-microscopical study of a Rathke's cleft cyst

The electron-microscopical appearance of a Rathke's cleft cyst is described. The cyst lining is seen to consist predominantly of secretory columnar epithelium, both ciliated and non-ciliated. There are also epidermoid cells occurring singly or in squamae. The histogenesis of Rathke's cleft cysts, in relation to craniopharyngiomas, is discussed.     

Malignant transformation of intracranial epidermoid cyst with leptomeningeal carcinomatosis: case report

INTRODUCTION: Although rare, the malignant degeneration of epidermoid cysts is well documented. Its occurrence with leptomeningeal dissemination and malignant cells in CSF is scarce. To the best of our knowledge only four cases have been reported. CASE REPORT: The authors report the case of malignant transformation of an epidermoid cyst with leptomeningeal carcinomatosis (which simulates aseptic meningitis) in a 54 year-old woman. Changes in CSF and radiological features are not correlated with the clinical course. Despite improvement of both the lesions on the scan images and the malignant cells in the CSF under chemotherapy the patient died. CONCLUSION: The differential diagnosis of malignant degeneration of epidermoid cyst, and leptomeningeal carcinomatosis are discussed. The diagnosis of malignant transformation should be retained when MRI (or CT scan) shows contrast enhancement at the epidermoid site, and malignant cells are detected in CSF The prognosis is generally poor.     

A case of hyperdense diploic epidermoid cyst on CT scan mimicking meningioma

Epidermoid cysts typically have images characteristic similar to cerebrospinal fluid (CSF). A case of a hyperdense epidermoid cyst on computed tomography (CT) scan mimicking meningioma is described and the possibility of unusual imaging characteristics of epidermoids is highlighted as a diagnostic problem.     

Intracranial neuroenteric cysts: a concise review including an illustrative patient

Neuroenteric cysts (NC) are rare, benign lesions lined by mucin-secreting cuboidal or columnar epithelium of an intestinal or respiratory type. They are regarded as ectopic endodermal cysts, and tend to be found in the spine rather than an intracranial location. Advances in neuroimaging have led to an increased frequency of diagnosis of NC, especially as an incidental finding, although such cysts may be confused radiologically with other lesions such as epidermoid and arachnoid cysts. We undertook a PubMed search of the literature using the search terms "neuroenteric cyst" and its many pseudonyms, including "endodermal cyst", "enterogenous cyst", "neurenteric cyst", "epithelial cyst", "intestinome", "teratomatous cyst", "gastrocytoma", and also "enterogenic", "foregut", "respiratory", and "bronchogenic cyst". Only reports in English and those containing histopathologically-confirmed NC were considered for this review. In total, 140 patients with intracranial NC were found, including the patient reported in the present review. This review describes the classification, epidemiology, embryology, clinical presentation, radiology, histopathology, and surgical treatment of NC, and includes an illustrative patient.     

Primary Intracranial Squamous Cell Carcinoma in the Brain Stem with a Cerebellopontine Angle Epidermoid Cyst

Primary intracranial squamous cell carcinoma is extremely rare, with most cases arising from a preexisting benign epidermoid cyst. We report a rare case of primary intracranial squamous cell carcinoma in the brain stem with a cerebellopontine angle (CPA) epidermoid cyst. A 72-year-old female suffered from progressive left hemiparesis, difficulty in swallowing, and right hemifacial numbness. Diffusion-weighted magnetic resonance imaging revealed a high signal intensity (SI) lesion in the CPA region and an intra-axially ring-enhanced cystic mass in the right brain stem with low SI. Whole-body positron emission tomography showed no evidence of metastatic disease. The histological findings revealed a typical epidermoid cyst in the CPA region and a squamous cell carcinoma in the brain stem. We speculate that the squamous cell carcinoma may have been developed due to a chronic inflammatory response by the adjacent epidermoid cyst. The patient underwent a surgical resection and radiotherapy. After 12 months, she had no evidence of recurrence.     

Giant intradiploic infratentorial epidermoid cyst

Epidermoid cysts are benign, uncommon lesions (1% of all intracranial tumors). Their localization is intradiploic in 25% of cases, and exceptionally subtentorial. We report here a rare case of giant intradiploic infratentorial epidermoid cyst. A 74-year old patient presented with recent diplopia and sindrome cerebellar. CT scan and MR imaging revealed a giant osteolytic extradural lesion of the posterior fossa (5.2 cm x 3.8 cm) with a small area of peripheral enhancement after contrast injection. Retrosigmoid suboccipital craniectomy allowed a satisfactory removal of the tumor, followed by an acrylic cranioplasty. The outcome was good. Neuropathological examination confirmed an epidermoid cyst. We review the literature and discuss our case.     

神经内窥镜辅助显微手术治疗桥脑小脑角区胆脂瘤

目的:探讨神经内窥镜辅助显微手术治疗桥脑小脑角区胆脂瘤的治疗效果及应用价值。方法:应用神经内窥镜辅助显微外科手术治疗桥脑小脑角区胆脂瘤20例,均采用乙状窦后入路。结果:显微镜下手术后应用0°、30°内窥镜观察,20例均有不同程度的肿瘤残留。神经内窥镜辅助下继续行残余肿瘤切除,全切18例,次全切2例。术后无死亡及无菌性脑膜炎表现。术后3个月,随访16例患者,均恢复正常生活。结论:经乙状窦后入路的神经内窥镜辅助显微手术治疗桥脑小脑角区胆脂瘤,可提高肿瘤切除率,减少对脑干及颅神经的牵拉和对重要血管的损伤,降低手术危险性及术后并发症,提高手术疗效,缩短住院时间。     

Clinical characteristics and surgical outcomes of patients with interdural epidermoid cyst of the cavernous sinus

Interdural epidermoid cysts of the cavernous sinus originate within the lateral dural wall of the cavernous sinus. Few data are available on the diagnosis and treatment of these tumors. In this study, four patients with interdural epidermoid cyst of the cavernous sinus are reported and data from six patients reported in the English literature are summarized. Trigeminal nerve dysfunction, ophthalmoplegia, and headache were the common symptoms and signs. MRI was the primary diagnostic tool. Contrast enhanced MRI showed slight or strong rim enhancement in six of the 10 patients. All patients were treated by surgical resection using frontotemporal or pterional craniotomy via the intradural or interdural approach. Total removal was achieved in three of the four patients, and four of 10 patients in the whole series. Postoperative neurological function improved in all patients. During follow-up, there were three known clinical recurrences in the total group, but no recurrence in the four patients treated at Qilu Hospital of Shandong University. The findings suggest that aggressive surgical management is associated with good results and with low complication and recurrence rates, but radical resection at the risk of impairing the cranial nerves is not recommended.