Proton beam therapy for malignant transformation of intracranial epidermoid cyst.

We report the first clinical case on the successful use of proton beam therapy in the management of malignant transformation of intracranial epidermoid cyst. A 43-year-old man was initially diagnosed as this disease with left facial paresis, hypesthesia and hypoalgesia in the territories of the trigeminal nerve. After failure of surgical interventions, he was referred to our radiation centre. We performed a postoperative proton beam therapy for treatment. We delivered a total dose of 57 GyE in 31 fractions. He tolerated the treatment well with mild acute toxicities and remained healthy and functional by 2-year follow-up postradiotherapy. No evidence of delayed radiation-induced neurotoxicity was observed.

Hemifacial Spasm Caused by Small Epidermoid Tumor, Misinterpreted as Delayed Secondary Hemifacial Spasm Caused by Vestibular Schwannoma Treated with Gamma Knife Surgery.

BACKGROUND: Hemifacial spasm (HFS), one of the most common hyperactive cranial rhizopathies, is a disorder characterized by spontaneous, intermittent, and repetitive contraction of unilateral facial muscle. The most common cause of HFS is a mechanical compression of the facial nerve at the root exit zone (REZ) by blood vessels located in the brainstem. The incidence of cerebellopontine angle (CPA) tumor-induced HFS ranges from 0.3%-2.5% of all patients with HFS, and the incidence of epidermoid cyst among such tumors is extremely rare (0.2% to 0.25%). We present a patient manifesting HFS induced by a small epidermoid cyst who underwent Gamma Knife surgery (GKS) for ipsilateral vestibular schwannoma (VS) more than 10 years ago. CLINICAL DESCRIPTION: A 59-year-old female was admitted to our hospital with HFS. Ten years before admission, the patient underwent GKS for ipsilateral VS. Enhanced brain magnetic resonance imaging demonstrated that the volume of VS was not increased. Also, no definitive mechanical compression of the facial nerve REZ by blood vessels was detected. We concluded that HFS was secondary to the VS treatment by GKS and decided to operate to remove the tumor and identify REZ. A standardized lateral suboccipital retrosigmoid approach was performed. The cochlear nerve was encased by a tumor mass, and the posterior inferior cerebellar artery (PICA) was displaced by the mass. During tumor excision, we ensured that the VS and displaced PICA did not directly compress the REZ. Interestingly, an unexpected lesion was found with a yellowish viscous mass located between the sixth nerve and ventral side of the seventh nerve REZ. After the tumor removal, a small piece of Teflon was inserted between the PICA and REZ of the facial nerve. Postoperatively, the patient experienced no HFS or facial nerve dysfunction. Histopathologic examination of the lesion revealed an epidermoid cyst. CONCLUSIONS: We should consider various possibilities for the cause of HFS and review brain imaging meticulously if patients were treated for CPA tumor with GKS a long time ago and the delayed HFS occurred without changes in tumor volume. HFS induced by epidermoid cyst is rare. Therefore it is critical for the neurosurgeon to ensure the absence of any abnormal contact with the REZ of the facial nerve during surgery for HFS induced by CPA tumors.

Radiotherapy for recurrent intracranial epidermoid cysts without malignant transformation: a single-institution case series.

INTRODUCTION: Recurrent intracranial epidermoid cysts may be difficult to address surgically given their proximity to critical neurovascular structures of the skull base. There are emerging reports of using radiotherapy (RT) for the treatment of recurrent epidermoid cysts. Here, we report a case series of adjuvant fractionated external beam RT for recurrent intracranial epidermoid cysts. METHODS: A single-institution review of all recurrent epidermoid cysts treated with adjuvant therapy between 2000 and 2017 was performed. RESULTS: Eight patients with recurrent epidermoid cysts who underwent adjuvant external beam RT were identified. Average age at initial diagnosis was 42.2 years, and median clinical follow-up after initial diagnosis and RT was 16.4 and 2.9 years, respectively. The median number of surgical resections prior to RT was 3 (range 2-5). Rationale for RT included multiple recurrent disease, rapid recurrence following prior resection, increased risk of further surgical morbidity, and patient preference. Median dose was 50.4 delivered in 1.8 Gy fractions (median 28 fractions). By the date of last follow-up, no patient has demonstrated progression, and there have been no cases of malignant degeneration. CONCLUSION: Adjuvant RT should be considered in the context of recurrent epidermoid cysts to decrease the likelihood of further recurrences.

IL-1 Receptor-Knockout Mice Develop Epidermal Cysts and Show an Altered Innate Immune Response after Exposure to UVB Radiation.

In this study, we observed that mice lacking the IL-1 receptor (IL-1R) (IL1r-/-) or deficient in IL1-ß developed multiple epidermal cysts after chronic UVB exposure. Cysts that developed in IL1r-/- mice were characterized by the presence of the hair follicle marker Sox 9, keratins 10 and 14, and normal melanocyte distribution and retinoid X receptor-α expression. The increased incidence of cysts in IL1r-/- mice was associated with less skin inflammation as characterized by decreased recruitment of macrophages, and their skin also maintained epidermal barrier function compared with wild-type mice. Transcriptional analysis of the skin of IL1r-/- mice after UVB exposure showed decreased gene expression of proinflammatory cytokines such as tumor necrosis factor-α and IL-6. In vitro, primary keratinocytes derived from IL1r-/- mice were more resistant to UVB-triggered cell death compared with wild-type cells, and tumor necrosis factor-α release was completely blocked in the absence of IL-1R. These observations illustrate an unexpected yet prominent phenotype associated with the lack of IL-1R signaling in mice and support further investigation into the role of IL-1 ligands in epidermal repair and innate immune response after damaging UVB exposure.

Milia en placa en la cara posterior de ambos pabellones auriculares secundaria a radioterapia / Milia en plaque on the posterior surface of both auricules following radiation therapy

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Management algorithm for HIV-associated parotid lymphoepithelial cysts.

We report an evidence-based management algorithm for benign lymphoepithelial cysts (BLEC) of the parotid glands in HIV patients based on long-term outcomes after radiation therapy. From 1987 to 2013, 72 HIV-positive patients with BLEC of the parotid glands treated at our institutions were identified and their medical records were reviewed and analyzed. The primary endpoint of our study was to determine a dose response in HIV patients with BLEC. In group A (≤18 Gy), which received a median dose of 10 Gy (8-18), overall response (OvR), complete response (CR), partial response (PR), and local failure (LF) was experienced by 7, 7, 0, and 93 %, respectively. In group B (≥22.5 Gy), which received a median dose of 24 Gy (22.5-30), OvR, CR, PR, and LF was experienced by 88, 65, 23, and 12 %. Logistic regression revealed that higher dose (≥22.5 Gy) predicted for cosmetic control (p = 0.0003). Multiple regression analysis revealed higher dose predicted for cosmetic control (p = 0.0001) after adjusting for confounding variables (age, gender, race, HAART use, BLEC duration, and fractionation size). No patients in either group experienced RTOG grade ≥3 toxicities. A radiation dose of 24 Gy delivered in 12-16 fractions of 1.5-2 Gy per fraction provides long-term cosmetic control in HIV-positive patients with BLEC of the parotid glands.

Radiotherapy for recurrent epidermoid cyst.

Intracranial epidermoid cysts are rare benign congenital lesions for which the mainstay of treatment has been surgical resection. Due to a propensity to grow along the skull base, subtotal resection is often elected to avoid excessive surgical morbidity, but it comes with an increased risk of recurrence with its associated treatment difficulties. We here present the cases of three patients with recurrent epidermoid cyst who underwent multiple surgical resections followed by external beam radiation therapy with excellent results to date.

Survival outcomes for radiotherapy treatment of epidermoid tumors with malignant transformation.

Epidermoid tumors are intracranial lesions that may occasionally undergo malignant transformation. Although surgical resection is the first-line treatment for malignant epidermoids, postoperative radiotherapy has been intermittently reported with favorable findings. Our analysis identified all previously reported patients with malignant epidermoids treated with surgical resection alone or surgery plus radiotherapy to examine the potential role for this adjuvant therapy. Whereas patients treated with surgery only had an overall survival of 6.6 months, those treated with postoperative radiotherapy demonstrated a statistically significant increase in survival to 12.7 months (log-rank test, p<0.003). Furthermore, the mean dosage of radiation given to this patient population was 52.2 Gy, with no appreciable survival benefit for the utilization of levels of radiation greater than 50 Gy. When determining the management for malignant transformation of epidermoid tumors, the combination of surgical resection and radiotherapy may be associated with improved short-term survival.

Quistes dermoides y epidermoides intracraneales / Dermoid and epidermoid itracranial cysts

Objective. To present the clinical and radiologic features and surgical results in the management of dermal and epidermal cysts treated in our institution between 1997 and 2006. Material a method. Age, sex, way of presentation, location, radiologic features, surgical technique and compications of 15 epidermal and 1 dermal tumors diagnosed between may 1997 and October 2006, were retrospectively assessed. Results. Mean age: 43 yrs. Most frequent clinical manifestation: headache. Most tumors were at CPA. All resections were done with microsurgical technique. There were 3 cases of chemical meningitis that resolve without sequel. Discussion. Dermal and epidermal cysts are originated by inclusion of ectodermic material at the moment of occlusion of the neural tube. They represent the 0.3-1.5 of intracranial tumors beeng dermal 5-9 times less frequent. The most frequent location of epidermal cysts is at the CPA while dermal are more frequent in the midline. Clinical fatures depend on location. MRI is the gold standard for diagnosis, epecially the DWI sequence. CT is important for the detection of bone invasion. Treatement is so extensive as possible surgical resection, difficult, sometimes because of strong adhesions to eloquent structures. Conclusion. Dermal and epidermal cysts are high fat content lesions, that produce edherence to neural structures. Surgical excision is the election tratement.

Intraspinal epidermoid cyst successfully treated with radiotherapy: case report.

OBJECTIVE AND IMPORTANCE: Epidermoid cysts are benign lesions that account for 0.7% of all intraspinal tumors. Standard treatment is complete resection. The recurrence rate after surgery cannot be estimated from the scarce literature, but it has been acknowledged that, in some patients, curative surgery may be impossible and temporary relief of symptoms is the only aim. CLINICAL PRESENTATION: In 1996, a 59-year-old woman presented with a 30-year history of a spinal epidermoid cyst, for which she had previously undergone eight operations. Spastic paraplegia of the lower limbs had been present from initial diagnosis, and the neurological level had moved upward to C6. At the time of presentation, paresthesia in her arms and hands had developed. A magnetic resonance imaging scan revealed typical sequelae of previous surgery and a fibrotic region starting at the level of C5-C6 with formation of a cyst that extended to the conus terminalis. The spinal cord could not be visualized caudally at C7. INTERVENTION: Because both patient and surgeons did not want further surgical interventions that might comprise upper limb function, radiotherapy was performed. A dose of 46 Gy was delivered to the cervicothoracic spine (C4-T1) by opposing anteroposterior high-energy photon beams. The treatment was well tolerated, and after treatment, the patient's clinical symptoms improved slightly, without evolution on magnetic resonance imaging. CONCLUSION: Irradiation was effective in stabilizing the patient's disease. Although radiotherapy is not established in the treatment of epidermoid cysts, it should be considered as an alternative to palliative surgery and in patients who cannot undergo operations.

Recurrent intracranial epidermoid cyst treated with radiotherapy.

We report the case of a 58 year old woman with an epidermoid cyst of the right cerebello-pontine angle that was subtotally excised. She recurred 2 years later and received radiotherapy with resolution of symptoms and shrinkage of the cyst. The natural history of epidermoid cysts and the role of radiotherapy in their management is discussed.

Contribution of MRI to the diagnosis and post-therapeutic monitoring of brain stem tumours.

MRI has become indispensable to the positive diagnosis of brain stem tumours which develop preferentially in young people. The prognosis of these tumours, especially infiltrating gliomas, being relatively sombre, it is important to make a precise morphological diagnosis of the lesions (increase in size, signal abnormalities) which will help both neurosurgeons and radiotherapists. As regards post-therapeutic monitoring, MRI with gadolinium contrast injection is harmless and will make it possible to follow these patients regularly and to detect recurrences.