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.     

Microsurgical treatment for 55 patients with hemifacial spasm due to cerebellopontine angle tumors

Tumor-related hemifacial spasm (HFS) has been found to be rare. During the period from October 1984 to October 2008, we treated 6,910 HFS patients using a microsurgical procedure. Of these HFS patients, 55 cases were associated with cerebellopontine angle tumors. A small craniectomy was performed in order to excise the tumor. All tumors were found to compress the root exit zone (REZ) of the facial nerve to different extents, but concomitant vascular compression of the facial nerve was observed in a majority of cases, and microvascular decompression of the facial nerve at REZ was conducted in 43 of 55 patients (78.2%) by displacing the co-compressing vasculature away from the REZ and retaining it using a Teflon pad. Intraoperative findings and postoperative pathological examinations suggested that the tumors were epidermoid cysts, meningiomas, and Schwannomas. Follow-up in 48 of 55 patients for 4–230 months after surgery showed that the clinical symptoms of HFS disappeared in 43 cases, improved in two cases, and recurred in three cases. Ten patients had sequelae associated with the operation. We concluded from this study that the majority of cases of tumor-related HFS are caused by combined tumor and vascular co-compression at the REZ, and tumor removal and microvascular decompression are required in order to relieve the symptoms.     

Giant intradiploic epidermoid cysts of the skull. A report of eight cases

Giant intradiploic epidermoid cyst of the skull is a rare tumour. Only a small number of cases have been reported. Over a 16-year period, we treated eight cases of giant intradiploic epidermoid cyst of the skull. All cases were between 19 and 45 years of age, except one aged of 61 years. Five cases presented with local swellings, three patients came with infection and one had an extradural abscess. All the patients were operated upon after adequate preoperative care. Patients with infection required appropriate antibiotics. Total or near total excision was carried out in all. One patient developed postoperative infection. So for those have been no recurrences. This appears to be the largest series of giant intradiploic epidermoid cyst of the skull so far reported.     

Secondary trigeminal neuralgia in cerebellopontine angle tumors

The analysis of the treatment results in patients with cerebellopontine angle (CPA) tumors, manifested as trigeminal neuralgia (TN). During the 10-year period from 1998 to 2008, 14 patients with verified CPA tumors that had the typical manifestations of TN were operated on at our hospital (5.8% from all patients with TN who underwent surgery). In nine cases the epidermoid was identified; three patients had meningioma, one patient had acoustic neurinoma, and one patient had lipoma. The follow-up of all patients lasted at least 12 months. The intraoperative assessment identified the three variants of relationship between the tumors and neurovascular structures: (1) tumor grows around the trigeminal nerve; (2) the tumor causes compression and displacement of the trigeminal nerve; and (3) tumor presses the arterial vessel to the trigeminal nerve by moving the vessel or nerve. For six patients, with removal of tumor a microvascular decompression of the trigeminal nerve was performed. Complete pain relief was achieved in 12 patients (86%). TN is an expectative symptom of CPA tumors. The most frequent cause of secondary TN of CPA tumors is epidermoid. All patients with manifestations of TN should undergo the magnetic resonance imaging for early diagnosis of CPA tumor.     

Differenzialdiagnose einer Mundbodenschwellung

Epidermoid cysts are malformations that are rarely observed in the oral cavity. Histologically, these cysts may be further classified as epidermoid, dermoid, or teratoid depending on the stratified epithelium. We report the case of an 11-year-old girl who had developed an epidermoid cyst over a period of 4 years, which presented as a soft, painless left-sided sublingual swelling without mucosal lesions. Imaging revealed a cystic formation with a central concrement. The lesion was surgically excised via an intraoral approach and after histological examination was diagnosed as an epidermoid cyst. Differential diagnoses for epidermoid cysts include infections, tumors, mucous extravasation, and sialolithiasis or sialoadenitis of the sublingual gland. If tumors are excised completely recurrence is very rare. In cases of postoperative saliva retention with recurrent inflammation of the salivary gland, submandibulectomy is indicated.     

Intramedullary epidermoid associated with an intramedullary spinal abscess secondary to a dermal sinus.

Intramedullary spinal abscesses are rare and potentially devastating lesions. A heightened clinical awareness of patients at risk is essential for an early diagnosis and intervention to improve the prognosis of these patients. The first case of spinal abscess was described in 1830, and only 63 cases have been reported subsequently. Intraspinal epidermoids also are unusual lesions. These tumors were reported first in 1829 and represent less than 1% of all intraspinal tumors. We present a case of a 2-year-old girl who had rapidly progressive paraplegia with urinary and bowel retention and was found to have an intramedullary epidermoid and an intramedullary abscess as a result of a dermal sinus. The association of an intramedullary abscess and epidermoid tumor, two rare lesions, has not been reported previously. We also review the literature related to both intramedullary epidermoids and intramedullary abscesses of the spinal cord. Advances in sectional imaging as described should help lead to an early and safe diagnosis of these rare but often debilitating lesions. This case illustrates the importance of a complete neurological assessment of all patients with a congenital dermal sinus because of the potential for intradural extension and the frequent association with other dysraphic abnormalities. Prophylactic surgery is indicated in many cases to prevent dangerous and recurrent infections of the central nervous system.     

Surgical treatment of intracranial epidermoid tumors

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.     

Intracranial epidermoid tumours: thirty-seven years of diagnosis and treatment

The clinical, operative and pathological characteristics of 102 consecutive cases of intracranial epidermoid tumours are reported. These cases constituted 1.1% of all intracranial tumours and 13.5% of congenital neoplasma admitted to our service in the period 1951-88. Of 91 (89.2%) intradural tumours, 74 (72.5%) were in the subarachnoid cisterns, especially in the cerebellopontine angle cistern (31 cases). The tumours were solid in 69 cases (67.6%) and cystic in 26 (25.5%); the remaining 7 cases were not recorded. There were no specific clinical features with which to identify the nature and extent of the tumour. Diagnosis and treatment are described, with an evaluation of CT and MRI. Before 1981, total removal rate of tumour was 29.3%; after that date it was 72.7% with the aid of microsurgery. The overall surgical mortality rate was 4.9% (5 cases), but there were no deaths in the microsurgical group. Follow-up for a period of 0.5-37 years (mean 13 years) was carried out in 68 patients (70.1%). Among 24 patients with incomplete removal, recurrence of tumour was verified in 4 cases; 3 underwent reoperation with excellent or good results. One patient refused operation and died. All the remaining 20 cases, save 3 who died of other diseases, returned to their normal activities without recurrent manifestations. The results suggest that the primary treatment of epidermoid tumours should be surgical removal including the contents and capsule of a tumour. With the aid of microsurgical technique, complete removal is possible. Patients with incomplete removal can also obtain a long-term favourable result.     

Microneurosurgical treatment of intracranial dermoid and epidermoid tumors

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)     

Benign testicular tumors

Testicular tumor is considered rare in Oriental people and benign testicular tumor has never been reported from this area. From 1969 to 1985 70 cases of intratesticular tumors were treated at Tri-Service General Hospital. 12 of them were benign in nature including 8 epidermoid cysts, 1 cavernous hemangioma, 1 foreign body granuloma with pseudocyst, 1 Sertoli cell tumor and 1 intratesticular simple cyst. A testis-sparing procedure was performed for 5 epidermoid cysts, the Sertoli cell tumor and the simple intratesticular cyst. The tumors were excised completely and the testes were preserved after careful and thorough frozen section pathological studies. One of the epidermoid cysts and the case of simple testicular cyst were diagnosed correctly before surgery by using scrotal ultrasound.     

Choroid plexus papilloma: a clinicopathological study of 23 cases.

BACKGROUND: Choroid plexus papillomas (CPPs) are rare, accounting for less than 1% of all intracranial tumors in adults. However, they are relatively more common in childhood and constitute 1.5 to 4% of intracranial tumors. DESCRIPTION: They are most often located in the lateral ventricle, followed by the fourth and third ventricles and, rarely, in the cerebellopontine angle. The radiological appearance of a CPP as a cyst with a mural nodule is a curiosity. Bone formation is rare in CPPs and only 6 cases have been described in the literature. Neuromelanin production is also extremely rare and only 2 cases have been reported to date. CONCLUSION: In the present communication, 23 cases of CPP are analyzed and rare clinical, pathological, and radiological features are described.     

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.     

Malignant tumors at the site of total hip prosthesis. Analytic review of 46 cases

During the years 1974 to 2003, a total of 46 cases of malignant tumors at the site of total hip arthroplasty, 41 sarcomas, 4 lymphomas, and 1 epidermoid carcinoma, have been reported in the Western literature. Soft tissue sarcomas occurred in 31 and bone sarcomas in 10 cases. Malignant fibrous histiocytoma was the most common in 20 (65%) of all soft tissue sarcomas. The patients with malignant tumors had been operated on more often for secondary osteoarthritis and had more local complications than those in ordinary total hip arthroplasty series. Primary osteoarthritis was the indication in 63% and secondary osteoarthritis in 37% of the 38 reported cases. Tumor developed after 8 (17%) revisions. Deep infection complicated 4 (9%) of the cases. Four cases (9%) were preceded by a precondition for cancer, 2 postradiation necroses of the femoral head, 1 chronic sinus, and 1 Mafucci syndrome. Mean age of the patients at the time of first implantation was 59 years (range, 24-79 years). The mean latent period was 6 years (range, 0.5-20 years) from the first operation. Longer latency period did not correlate positively with the cancer risk. Sarcomas were highly aggressive. Of the 31 reported patients, 24 (77%) died within 1 year after the diagnosis. The number of reported sarcoma cases is low considering the total population behind the cases. In addition, one observed local sarcoma from Nordic cohorts was less than expected. Chronic, particle-induced inflammation around the prosthesis does not seem to increase the risk for carcinogenesis.     

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.     

Congenital inclusion cysts of the anterior fontanelle

BACKGROUND: Congenital inclusion cysts of the anterior fontanelle are rare lesions. Both dermoid and epidermoid cysts are located in the midline of the scalp and occupy the subgaleal space. METHODS: We report 7 cases, 4 boys (57.1%), and 3 girls (42.9%), with ages ranging from 3 months to 16 years (mean 40.85 +/- 68.56 months; median 10 months). Four patients (57.1%) were white and 3 (42.9%) were Afro-Brazilian. RESULTS: The cysts had manifested soon after birth in all patients. They gradually enlarged, with no intracranial extensions. Four patients had dermoid cysts and the others had epidermoid cysts. All of them underwent surgery with complete excision of the cyst and no capsular rupture. There were no complications and no deaths. CONCLUSIONS: Congenital inclusion cysts of the anterior fontanelle are rare lesions that usually manifest at birth. The diagnosis is usually easy and surgery is mandatory, with a good prognosis. Recurrence is rare.     

Prepubertal testicular tumors: A 20‐year experience with 40 cases

Testicular tumors are rare in children. In addition, prepubertal testicular tumors (PTT) are fundamentally different from their postpubertal counterparts. We reviewed our 20‐year experience with 40 cases to suggest an algorithm for the appropriate surgical approach for patients with PTT. All tumors ultrasonographically suspected of being benign were confirmed to be benign postoperatively. There was a significant difference between median preoperative α‐fetoprotein (AFP) levels of infants with yolk sac tumor and teratoma. The majority of our cases were benign, with the most common histopathological subtype being teratoma, as recently reported. Testis‐sparing surgery was carried out in five patients with teratoma and three patients with epidermoid cyst. Other than one patient with teratoma, none of the patients developed recurrence or testicular atrophy after testis‐sparing surgery. When testis‐sparing surgery is considered, our algorithm based on ultrasonographic findings and preoperative AFP levels might be helpful.     

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.     

Anterior craniofacial resection for malignant ethmoid tumors--a series of 91 patients

BACKGROUND: Anterior craniofacial resection is now recognized as the best treatment for ethmoid tumors involving the cribriform plate with or without invasion of anterior cranial fossa. METHODS: Ninety-one patients underwent an anterior craniofacial resection for ethmoid malignant tumors at the Milan Cancer Institute between 1987 and 1994. The patient population was divided into two sections (30 and 61 patients) based upon some important variants (type of craniotomy, antibiotic treatment, postoperative care). RESULTS: The mean age was 53.4 years (range, 24 to 78 years). There were 62 men and 29 women. Forty-nine patients had a recurrence after previous treatments (surgery and/or radiotherapy). The subdivision by histology was as follows: 50 cases of adenocarcinoma, 16 cases of epidermoid and undifferentiated carcinoma, 8 cases of esthesioneuroblastoma, 5 cases of adenoid cystic carcinoma, 5 cases of melanoma, and 6 rare tumors. The stages (according to our new staging) were as follows: 37 cases with T2, 27 cases with T3, and 27 cases with T4. The mean follow-up was 47 months. Seven patients died after surgery (6 in the first series). The survival at 3 and 5 years was, respectively, 52% and 47%, and the disease-free survival (DFS) was 30% and 24%, with a statistically significant difference at multivariate analysis in favor of patients without prior treatment (p = .033) or T2 versus T3 and T4 (p<.007). CONCLUSIONS: An anterior craniofacial resection should be performed in cases of ethmoid tumors reaching or eroding the cribriform plate. A scrupulous intra- and postoperative approach is necessary to avoid severe complications. The patients often survive for a long time with recurrence ongoing. Our new staging identifies the critical extensions of ethmoid tumors.     

Clinical characteristics,treatment, and outcome of pancreatic schwannomas

This article involves the study of a patient with a rare benign schwannoma in the body of the pancreas. After reviewing 39 patient cases previously reported in the literature, a discussion of the schwannoma with regard to clinical presentation, diagnosis, and treatment is examined. A review of the patient’s chart was performed along with a review of the literature using a Medline search. Translations were performed whenever necessary. There are 23 reports of 29 patient cases of pancreatic schwannomas in English and European literature and one report of 10 patient cases in the Japanese literature. The mean age was 57.75 years (range 32–89) and the male-to-female (M:F) ratio was 17:23. The mean reported size was 8.79 cm. The lesion was located in the head in 16 patients (40%), the body in 8 patients (20%), the body and tail in 8 patients (20%), the tail in 6 patients (15%), the head and body in 1 patient (2.5%), and the location was not specified in 1 patient (2.5%). Of the English and European patients, 11 out of 30 patients (36.7%) exhibited solid tumors and 14 out of 30 patients (46.7%) exhibited cystic tumors. The majority of the tumors (35 out of 40) were benign, but there were.ve reported malignancies. There were no deaths or recurrences reported with a follow-up of 18.68 months ± 24.09 (range 3–108 months). Pancreatic schwannomas are rare, and the preoperative diagnosis is difficult. Intraoperative frozen section can confirm the diagnosis of a benign schwannoma. Enucleation of the tumor from the surrounding parenchyma is recommended, if possible. Patients undergoing resection indicate an excellent long-term prognosis.     

Carcinoma changes in cysts of skin

Summary Carcinomatous change of commonly encountered, benign epidermoid cysts, is a very rare event. Two cases of squamous cell and one case of basal cell carcinoma arising in the cyst wall and invading adjacent dermis were diagnosed in three epidermoid cysts in a review of 386 cases. The frequency of carcinomatous change has been reported to be as much as 9%. In our series it was 0.77%.