Intra-operative extracorporeal irradiation of tumour-invaded craniotomy bone flap in meningioma: a case series.

BACKGROUND: Extracorporeal irradiation of tumorous calvaria (EITC) can be performed to restore function and form of the skull after resection of bone-invasive meningioma. We sought to examine the rate of tumour recurrence and other selected outcomes in patients undergoing meningioma resection and EITC. METHODS: Retrospective single-centre study of adult patients undergoing meningioma resection and EITC between January 2015 and November 2022 at a tertiary neurosurgical centre. Patient demographics, surgery data, tumour data, use of adjuvant therapy, surgical complications, and tumour recurrences were collected. RESULTS: Eighteen patients with 11 (61%) CNS WHO grade 1, 6 (33%) grade 2, and 1 (6%) grade 3 meningiomas were included. Median follow-up was 42 months (range 3-88). Five (28%) patients had a recurrence, but none were associated with the bone flap. Two (11%) wound infections requiring explant surgery occurred. Six (33%) patients required a further operation. Two operations were for recurrences, one was for infection, one was a washout and wound exploration but no evidence of infection was found, one patient requested the removal of a small titanium implant, and one patient required a ventriculoperitoneal shunt for a persistent CSF collection. There were no cases of bone flap resorption and cosmetic outcome was not routinely recorded. CONCLUSION: EITC is feasible and fast to perform with good outcomes and cost-effectiveness compared to other reconstructive methods. We observed similar recurrence rates and lower infection rates requiring explant compared to the largest series of cranioplasty in meningioma. Cosmetic outcome is universally under-reported and should be reported in future studies.

Practical Guideline for Prevention of Patchy Hair Loss following CyberKnife Stereotactic Radiosurgery for Calvarial or Scalp Tumors: Retrospective Analysis of a Single Institution Experience.

INTRODUCTION: Patchy alopecia is a common adverse effect of stereotactic radiosurgery (SRS) on the calvarium and/or scalp, yet no guidelines exist for its prevention. This study aims to investigate the incidence and outcomes of patchy alopecia following SRS for patients with calvarial or scalp lesions and establish preventive guidelines. METHODS: The study included 20 patients who underwent CyberKnife SRS for calvarial or scalp lesions, resulting in a total of 30 treated lesions. SRS was administered as a single fraction for 8 lesions and hypofractionated for 22 lesions. The median SRS target volume was 9.85 cc (range: 0.81-110.7 cc), and the median prescription dose was 27 Gy (range: 16-40 Gy), delivered in 1-5 fractions (median: 3). The median follow-up was 15 months. RESULTS: Among the 30 treated lesions, 11 led to patchy alopecia, while 19 did not. All cases of alopecia resolved within 12 months, and no patients experienced other adverse radiation effects. Lesions resulting in alopecia exhibited significantly higher biologically effective dose (BED) and single-fraction equivalent dose (SFED) on the overlying scalp compared to those without alopecia. Patients with BED and SFED exceeding 60 Gy and 20 Gy, respectively, were 9.3 times more likely to experience patchy alopecia than those with lower doses. The 1-year local tumor control rate for the treated lesions was 93.3%. Chemotherapy was administered for 26 lesions, with 11 lesions receiving radiosensitizing agents. However, no statistically significant difference was found. CONCLUSION: In summary, SRS is a safe and effective treatment for patients with calvarial/scalp masses regarding patchy alopecia near the treated area. Limiting the BED under 60 Gy and SFED under 20 Gy for the overlying scalp can help prevent patchy alopecia during SRS treatment of the calvarial/scalp mass. Clinicians can use this information to inform patients about the risk of alopecia and the contributing factors.

Infección por bartonella henselae en pacientes pediátricos. Lesión pseudotumoral de calota. Reporte de caso

Los tumores de calota en pacientes pediátricos poseen múltiples etiologías. Dentro de las causas pseudotumorales, las infecciones juegan un rol importante, siendo la osteomielitis por Bartonella henselae (Enfermedad por Arañazo de Gato) una posibilidad diagnóstica rara, pero que debe ser estudiada y descartada. Se presenta el caso de una lactante de 1 año, con lesión expansiva de calota, a nivel frontal derecho, hipervascularizada e infiltrativa. Se realizó estudio con ultrasonido, tomografía cerebral y cintigrama óseo. Se realizó resección quirúrgica completa de la lesión, con preservación de la duramadre y zona fontanelar, además de un cuidadoso trato con el seno sagital superior. Evolucionó sin complicaciones perioperatorias. El resultado de la biopsia fue compatible con proceso inflamatorio crónico, osteomielitis supurada. Tinción de Warthin Starry positiva sugerente de Bartonella henselae. Se descartó etiología tuberculosa y fúngica. Serología positiva para Bartonella henselae. La paciente completó antibioticoterapia, azitromicina y cotrimoxazol, con evolución clínica favorable.

Calvarial tumors in pediatric patients have multiple etiologies. Among the pseudotumoral causes, infections play an important role, being Bartonella henselae osteomyelitis (Cat Scratch Disease) a rare diagnostic possibility, but it should be studied and ruled out. We present the case of a 1 year old infant, with an expansive lesion of the calvaria, at right frontal level, hypervascularized and infiltrative. Ultrasound, brain tomography and bone scintigram were performed. Complete surgical resection of the lesion was performed, with preservation of the dura mater and fontanel area, in addition to a careful treatment with the superior sagittal sinus. The patient evolved without perioperative complications. The biopsy result was compatible with a chronic inflammatory process, suppurative osteomyelitis. Positive Warthin Starry stain suggestive of Bartonella henselae. Tuberculous and fungal etiology was ruled out. Positive serology for Bartonella henselae. The patient completed antibiotic therapy, azithromycin and cotrimoxazole, with favorable clinical evolution.

Protuberant fibro-osseous lesion of the temporal bone: report of four cases and review of the literature.

'Bullough lesions', also referred to as protuberant fibro-osseous lesions (PFOL), are rare temporal bone lesions initially described in 1999. Since only 12 cases have been reported, several key issues, such as their origin and recommended management strategies, remain unresolved. This article reports the largest cohort included in the literature to date, comprising four patients with PFOL. PFOL appears to be characterized by female and right-side predominance. These lesions were consistently located regarding the mastoid, generally diagnosed in early adulthood, without functional symptoms, and were always fibro-osseous. Invasive/malignant features were not found on imaging or histology. The main differential diagnosis was malignant low-grade parosteal osteosarcoma. Clinical examination and computed tomography images provided strong elements supporting the diagnosis of PFOL. Biopsy allowed molecular biology investigations (MDM2 and CDK4 amplification), in order to rule out low-grade parosteal osteosarcoma.

A "Wait-and-See" Approach to Quiescent Single-System Langerhans Cell Histiocytosis to Spare Children From Chemotherapy.

Background: Langerhans Cell Histiocytosis (LCH) is a childhood disorder of histiocytes that is generally treated with systemic chemotherapy. Spontaneous resolution has been previously reported in Single System LCH (SS-LCH), which is less aggressive than multisystem disease. However, there are no clear guidelines on which patients can be safely spared from systemic chemotherapy. Here, we propose a risk stratification framework based on disease quiescence as determined by clinical and biochemical features of inflammation, to identify low risk patients who may be potentially spared from chemotherapy through a conservative "wait-and-see" approach. Methods: Retrospective analysis in a single institution was conducted in children with SS-LCH, comparing features of inflammation and outcomes of those who received chemotherapy vs. those with quiescent disease, who were managed conservatively. Results: Of 44 children with SS-LCH, only patients without risk-organ involvement were considered for conservative management. A "wait-and-see" approach was adopted for patients with quiescent disease as defined by clinical and biochemical evidence of disease activity. Following 2 weeks of watchful observation, decisions were made to either start treatment or continue conservative management. Based on data collected at diagnosis, patients with quiescent disease had a lower mean platelet count 339 × 109/L (95%C.I: 285-393) vs. 482 × 109/L (95% C.I: 420-544) p < 0.01, a lower mean white cell count 9.3 × 109/L (95%C.I: 7.5-11.1) vs. 13.1 × 109/L (95%C.I: 11-15.2) p < 0.01 and lower Erythrocyte-Sedimentation-Rate (ESR) 8.2 mm/h (95%C.I: 5.4-11) vs. 53.7 mm/h (95%C.I: 11-96.3) p = 0.04, suggesting that these are potential biochemical markers of disease activity. Other features of disease quiescence noted were rapid progression, functional disability, presence of a skull depression rather a lump and the lack of fever. Conclusions: Further studies are required to validate our proposed framework to determine disease activity in SS-LCH. Within the limits of this current analysis, it appears that low-risk patients with clinically and biochemically quiescent SS-LCH, may potentially be spared from chemotherapy with good long-term outcomes.

Imaging of skull vault tumors in adults.

The skull vault, formed by the flat bones of the skull, has a limited spectrum of disease that lies between the fields of neuro- and musculoskeletal radiology. Its unique abnormalities, as well as other ubiquitous ones, present particular features in this location. Moreover, some benign entities in this region may mimic malignancy if analyzed using classical bone-tumor criteria, and proper patient management requires being familiar with these presentations. This article is structured as a practical review offering a systematic diagnostic approach to focal calvarial lesions, broadly organized into four categories: (1) pseudolesions: arachnoid granulations, meningo-/encephaloceles, vascular canals, frontal hyperostosis, parietal thinning, parietal foramina, and sinus pericrani; (2) lytic: fibrous dysplasia, epidermal inclusion and dermoid cysts, eosinophilic granuloma, hemangioma, aneurysmal bone cyst, giant cell tumor, metastasis, and myeloma; (3) sclerotic: osteomas, osteosarcoma, and metastasis; (4) transdiploic: meningioma, hemangiopericytoma, lymphoma, and metastasis, along with other less common entities. Tips on the potential usefulness of functional imaging techniques such as MR dynamic susceptibility (T2*) perfusion, MR spectroscopy, diffusion-weighted imaging, and PET imaging are provided.

[The infracochlear approach for diagnostic petrous apicotomy]. / Der infracochleäre Zugang zur Felsenbeinspitze aus diagnostischer Indikation.

Diagnostic and therapeutic approaches to the petrous apex involve sub-/transtemporal, retrosigmoidal, infratemporal and-lesser invasive-e. g. infracochlear, infralabyrinthine or under favourable anatomic conditions, transsphenoidal surgical pathways. For diagnostic purposes, minimally invasive approaches should be preferred due to their lesser morbidity. This article illustrates the infracochlear approach to the petrous apex in the case of a diagnostic indication in a patient with an incidental, asymptomatic tumorous lesion of the right petrous apex with bony erosion. After the bone of the floor of the ear canal and the hypotympanum was removed, the carotid artery and the jugular bulb were identified using a diamond burr. The route to the petrous apex is triangled by the cochlea superiorly, the jugular bulb posteriorly, and the carotid artery anteriorly. After opening the petrous apex lesion, biopsies were taken. The defect in the floor of the ear canal and the hypotympanum was reconstructed with cartilage and temporalis fascia. The patient recovered quickly from surgery without vertigo or hearing loss. Histological evaluation showed a chondrosarcoma. The patient opted for primary radiation therapy (C12, 63 Gy). The infracochlear approach is minimally invasive and can offer access to the petrous apex with minimal morbidity. The pathway, however, is narrow and deep and bounded by the jugular bulb and the carotid artery. The available space can be estimated from preoperative CT scans and, if possible, with 3D reconstructions. Navigation can additionally enhance safety.

Reconstrucción primaria con implantes personalizados después de resección de gran tumoración craneofacial / Primary reconstruction with customized implants after resection of a large craniofacial tumor

Reportamos la reconstrucción inmediata con implantes personalizados en la región craneofacial en una paciente mujer de 22 años con tumoración craneofacial de 12x15 cm en la región fronto orbito cigomático izquierdo que correspondió a fibroma oscificante. Se realizó la reconstrucción con un implante hecho a medida basado en imágenes tomográficas. El implante de polieteretercetona (PEEK) fue seleccionado para reemplazar la pérdida ósea y para restaurar la anatomía facial. No hubo necesidad de ajuste transquirúrgico del implante; ni se presentaron reacciones de rechazo, infección o exposición del implante en el periodo posoperatorio. Aunque el hueso autólogo es preferido para implantes, resulta difícil adecuarlos en situaciones de un compromiso extenso como en el caso presentado; por ello los implantes de PEEK son actualmente una opción en términos de biocompatibilidad, resistencia, duración, conductividad térmica y traslucencia radiográfica.

We report the immediate reconstruction with personalized implants in the craniofacial region in a 22-year old female patient with a craniofacial tumor of 12x15 cm in the left zygomatic orbital frontal region that corresponded to an ossifying fibroma. The reconstruction was performed with a custom-made implant based on tomographic images. The polyetheretherketone (PEEK) implant was selected to replace bone loss and restore facial anatomy. There was no need for transsurgical adjustment of the implant, no rejection, infection or implant exposure in the postoperative period. Although autologous bone is preferred for implants, it is often limited in the morbidity of the donor site, the difficulty of giving shape mainly to the borders and the limitation of the area to be donated; therefore the PEEK implants are currently an option in terms of biocompatibility resistance, duration, thermal conductivity and radiographic translucency.

[Tumors of the paranasal sinus invading the orbit]. / Tumoren der Nasennebenhöhlen mit Übergreifen auf die Orbita.

Invasion of the orbit by tumors of the paranasal sinus represents an advanced state of disease. Prognosis has hardly improved in recent decades, despite multimodal therapeutic approaches. Complete resection of the tumor still remains the most promising therapy. Orbital exenteration implies a dramatic event for the patient's psyche and quality of life. If the eye that would have to be exenterated is the only one remaining with sufficient vision, exenteration should be abandoned in consensus with the patient. Aesthetic improvements following exenteration can be achieved by surgical and prosthetic techniques. The method of prosthesis fixation needs to be determined with respect to the patients' individual expectations and manual abilities. The main aspects of functional rehabilitation are good local cleanability and sufficient nasal breathing without encrusted mucus, which can be achieved by restoring the natural laminar airflow. Tumors of the paranasal sinus with orbital invasion represent a diagnostic and therapeutic challenge. The information regarding prognosis, therapeutic approaches, post-therapeutic quality of life, and rehabilitation options should be thoroughly discussed by patients and therapists prior to treatment.

Chronic temporomandibular joint pain: two cases of osteoid osteoma and a review of the literature.

Osteoid osteoma is a benign bone tumour with self-limiting growth potential occurring in any part of the body. Two rare cases of a pathologically proven osteoid osteoma invading the temporomandibular joint (TMJ) are reported herein. This article also reviews the cases of osteoid osteoma of the craniofacial complex reported in the English-language literature to date. Although the clinical presentation of osteoid osteoma in the jaw differs from that of osteoid osteoma in the more common locations, the radiographic features are similar. In both cases presented, computed tomography revealed a small round osseous lesion with sharp margins in the TMJ. Bone scintigraphy was performed in order to differentiate the lesions from other osseous lesions. Both patients underwent surgical excision of the lesion with immediate relief of the pain. The importance of early recognition of the clinical and imaging characteristics of an osteoid osteoma of the TMJ is emphasized, in order to prevent misdiagnosis and avoid discouraging therapies.

Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography (FDG PET/CT) Findings in an Unusual Case of Multiple Myeloma Presenting with a Large Extra-Axial Intracranial Mass.

BACKGROUND: We aimed to present unusual cranial FDG PET/CT findings of a 56-year-old female with multiple myeloma (MM). CASE REPORT: Plain CT images revealed a lytic lesion in the right parietal bone, filled with an oval-shaped, large, extra-axial, extradural, intracranial mass which measured 75×75×40 mm and had smooth borders. The right parietal lobe was compressed by the mass. The maximum standardized uptake value (SUVmax) of the mass lesion was 8.94 on FDG PET/CT images. Multiple lytic lesions with an increased uptake were also detected in other calvarial bones, in several vertebras and in the proximal left femur. After seven months, a control FDG PET/CT following radiotherapy and chemotherapy revealed almost complete regression of the right parietal extra-axial mass lesion. The number, size and metabolism of lytic lesions in other bones also decreased. CONCLUSIONS: FDG PET/CT was useful for an initial evaluation of MM lesions and was effective in monitoring the response of these lesions to therapy.

Osteoma craniofacial: estudo de 35 casos / Craniofacial osteoma: study of 35 cases

Introdução: O osteoma é um tumor benigno de crescimento lento, composto de osso esponjoso ou compacto, originado no periósteo dos ossos craniofaciais. Objetivo: Relatar o perfil epidemiológico de pacientes portadores de osteomas craniofaciais. Métodos: Foi realizado um estudo multicêntrico retrospectivo dos casos diagnosticados no Serviço de Neurocirurgia do Hospital Governador João Alves Filho (Aracaju/SE), durante o período entre janeiro de 1999 a junho de 2008 e no Laboratório da Disciplina de Patologia Bucal da Faculdade de Odontologia de Pernambuco - FOP, da Universidade de Pernambuco - UPE, no período de julho de 1992 a julho de 2008. Foram analisados os indicadores gênero, faixa etária, localização topográfica, sintomatologia, tratamento, a partir de ficha própria de coleta de dados. Resultados: Foi observado que 14 casos (40%) possuíam localização craniana, enquanto em 21 casos (60%) o acometimento era facial. A localização topográfica craniana mais freqüente foi o osso frontal (57%), seguido pelo temporal (14%). Na face, o osso mandibular predominou com 67% dos casos. O gênero feminino mostrou-se predominante, com 60% dos casos. As lesões foram mais freqüentes na segunda década de vida (40%), independentemente se o acometimento foi craniano ou facial. Apresentaram-se comumente assintomáticas (62%), sendo os casos sintomáticos em sua maioria com localização craniana (57%). Conclusões: Os osteomas cranianos e faciais são mais freqüentes no osso frontal e mandibular, respectivamente. O gênero feminino e a segunda década de vida mostram mais acometimento. A maioria dos casos apresentou-se assintomática, porém, sintomáticos quando cranianos.

Introduction: Osteoma is a benign slow growth tumor, constituted by compacted or sponged bone with the origin in the skull and face periosteum. Objective: To report the epidemiologic pattern of patients with craniofacial osteomas. Methods: A multicentric retrospective study was done, regarding the cases diagnosed at Governador João Alves Filho Hospital Neurosurgery Service (Aracaju/SE) between January, 1999 and June, 2008 and at the Pernambuco School of Dentistry Oral Pathology Laboratory (FOP/UPE), between July, 1992 and July, 2008. It was analyzed gender, age, topographic site, symptomatology and treatment, through an own paper for data collection. Results: It was observed that 14 cases (40%) were in the skull and 21 cases (60%) in the face. At the skull, the frontal bone was most prevalent (57%), followed by temporal bone (14%). In the face, the mandible represented 67% of all cases. Lesions in females were predominant, with 60% and in the second decade of life (40%). They were mostly asymptomatic (62%), with the symptomatic cases frequently in the skull (57%). Conclusion: The skull and facial osteomas occur frequently in the frontal bone and mandible, respectively. The women and second decade of life show more involvement. Most cases appeared to be asymptomatic, being symptomatic when at the skull.

Ewing's Sarcoma Mimicking a Meningioma in Radiological Findings: A Case Report

Ewing's sarcoma is an uncommon primary bone tumor. Primary Ewing's sarcoma of the cranium is extremely rare and constitutes only 1% of all Ewing's sarcoma cases. Usually, primary Ewing's sarcoma of the carnium manifests as an expansile osteolytic malignant bone tumor with or without intracranial extension. We report here the radiological findings of a case of Ewing's sarcoma mimicking a meningioma in an 18-year-old man.

Primary Intraosseous Malignant Fibrous Histiocytoma of the Skull: A Case Report

Malignant fibrous histiocytoma(MFH) is a rare primary neoplasm that constitutes less than 1% of the malignant tumors of bone, and involvement of the skull is very rare. We present a case of malignant fibrous histiocytoma of the skull, presenting an intraosseous lesion in a 43-yr-old woman. She had a rapidly growing, tender mass in the right parietal region. A plain radiograph showed an osteolytic lesion of the right parietal bone. Magnetic resonance imaging revealed that the lesion showed heterogeneous low signal intensity on T1-weighted images and slightly high signal intensity on T2-weighted images. No evidence of an extraosseous extension to the adjacent dura and soft tissue was found, and a wide excision of the parietal bone was performed. Histologically, the tumor was a typical MFH displaying pleomorphic spindle cells in a storiform pattern. The results of immunohistochemical stainings revealed that the tumor cells were positive for vimentin, alpha-1-antitryp-sin, and p53, and negative for smooth muscle actin, S100 protein, desmin, and MyoD1. Three months later, a mainly cystic, recurrent mass was developed at the previously operated site. Before the resection, we first performed the percutaneous aspiration cytology, revealing diagnostic multinucleated pleomorphic cells. There-after, she had to receive repetitive resections of recurrent or residual lesions, and she died of postoperative meningoencephalitis two years after the first operation.

Clinical and Radiological Analysis of Scalp Masses

OBJECTIVE: The authors report an analysis of clinical data including incidence, clinical manifestations, diagnostic tools and surgical treatment of neoplasms of scalp and calvarium in adults. METHODS: The clinical, radiological, and pathological records of 67 patients who underwent mass excision in scalp or skull at our department during recent five years were reviewed. RESULTS: The mean age was 43 years(range 16 to 85 years) and male to female ratio was 42 : 25. There were 62 scalp and five calvarial masses. Among scalp masses, there were 52 tumors, three vascular lesions, three inflammatory lesions and four miscellaneous lesions. The scalp tumors were composed of 44 benign lesions and nine malignant lesions. The most common type of scalp tumor was lipoma, followed by epidermoid and dermoid. The calvarial masses consisted of four osteomas and one eosinophilic granuloma. Surgical excision was carried out in all cases. CONCLUSION: Most scalp and calvarial masses are benign lesions, however, surgical excision seemed to be indicated in most cases for diagnosis and treatment.

Imaging diagnosis of skull epidermoid cyst:analysis of four cases / 中华放射学杂志

Objective To study the CT and MR imaging features of skull epidermoid cyst. Methods CT and MR findings of 4 cases of pathologically proved skull epidermoid cyst were analyzed and correlated with operative and pathologic reports.Results CT and MR appearances of skull epidermoid cyst were as follows:(1)crater-form or scalloping deossification with sclerotic border and fatty density focus was detected on CT.(2)The lesions were isointense to hypointense relative to normal white matter in T1-weighted image,prominent hyperintense in T_2-weighted image,mild to moderate hyperintense in diffusion-weighted imaging(DWI),and isointense to hypointense in apparent diffusion coefficient(ADC) map on MR.Proton MRS revealed lactate(Lac)peaks at 1.3 ppm and amino acids(AAs)at 0.9 ppm. (3)No enhancement was found in substance region of all tumors on both enhanced CT and enhanced MR examinations.Conclusion CT and MR appearances of skull epidermoid cyst have unique features which are very helpful in the diagnosis of skull epidermoid cyst.

Intracranial Inflammatory Pseudotumors: MRI and CT Findings

PURPOSE: The purpose of this study was to describe the MR imaging and CT findings of intracranial inflammatory pseudotumors. MATERIALS AND METHODS: We reviewed the MR imaging (n=8) and CT (n=4) studies of eight patients (M:F = 4:4, mean age: 41 years) with pathologically proven intracranial inflammatory pseudotumor. The findings were then evaluated with regard to location, shape, MR signal intensity, CT density and degree of contrast enhancement of the lesion, surrounding parenchymal edema, adjacent bone change and the location of accompanying extracranial lesion. RESULTS: In two patients, the parietal convexity was involved unilaterally, with no extracranial mass, and in the other six, the middle cranial fossa was involved unilaterally and extracranial mass was present. The lesion also involved the tentorium in four cases, the cavernous sinus in four, the anterior cranial fossa in one, and the posterior cranial fossa in one. The location of extracranial mass was the mastoid and middle ear cavity in two cases, the infratemporal fossa in two, both the infratemporal fossa and paranasal sinuses in one, and the orbit in one. MR images showed diffuse dural thickening in all eight cases, leptomeningeal thickening in four, and focal meningeal-based mass in two. As seen on T1-weighted images, the lesions were isointense to gray matter in eight cases, and on T2-weighted images were hypointense in seven cases and isointense in one. Marked homogeneous contrast enhancement was seen in all eight cases. The lesions seen on brain CT, performed in two cases, were isodense. Adjacent brain parenchymal edema and the destruction of adjacent bones were each seen in five cases. CONCLUSION: The characteristic MR findings of intracranial inflammatory pseudotumors are(1) diffuse dural thickening;(2) a focal meningeal-based mass which on T2-weighted images is seen as hypointense; and marked (3) contrast enhancement : these findings are, however, nonspecific. In order to differentiate these tumors, an awareness of these findings is, however, useful.