Fully Endoscopic Resection of Frontal Osteomas.

BACKGROUND: Osteomas are the most common primary bone tumors of the calvaria, with an incidence of less than 0.5%. In skull vault osteomas, the exostotic form that grows from the outer table is more common than the enostotic ones which arise from the inner table and grow intracranially. Osteomas of the forehead are very noticeable and disfiguring; patients usually seek medical advice for cosmetic reasons. Forehead osteomas were traditionally excised via either a direct incision over the lesion using the naturally occurring creases or a conventional bicoronal flap. More recently, endoscopic approaches for excision of forehead osteomas were introduced. The results were very encouraging and the technique was adopted by many groups worldwide yet with many technical variations. In this chapter we elaborate on the surgical technique and nuances of the fully endoscopic resection of frontal osteomas. METHODS: From a prospective database of endoscopic procedures maintained by the senior author, clinical data, imaging studies, operative charts, and videos of cases of forehead osteomas were retrieved and analyzed. The pertinent literature was also reviewed. RESULTS: The surgical technique of the fully endoscopic resection of frontal osteomas was formulated. CONCLUSION: The endoscopic technique has many advantages over the conventional procedures. In our hands, the technique has proven to be less time-consuming, efficient, and minimally invasive with excellent cosmetic results.

Questioning the value of stalk drilling after external auditory canal osteoma excision: case series, literature review, and meta-analysis.

OBJECTIVES: To question the value of drilling the site of the stalk ("insertion site" or "stalk" drilling) of a pedunculated external auditory canal osteoma (EACO) in reducing recurrence. DATA SOURCES: A retrospective medical chart review of all patients treated for EACO in one tertiary medical center, a systematic literature review using Medline via "PubMed", "Embase", and "Google scholar" search, and a meta-analysis of the proportion for recurrence of EACO with and without drilling. RESULTS: The local cohort included 19 patients and the EACO origin was the anterior EAC wall in 42% and the superior EAC wall in 26%. The most common presenting symptoms were aural fullness and impacted cerumen (53% each), followed by conductive hearing loss (42%). All patients underwent post-excision canaloplasty, and one sustained EACO recurrence. Six studies suitable for analysis were identified (63 EACOs). Hearing loss, aural fullness, otalgia, and cerumen impaction were the most common clinical presentations. The most common EACO insertion site was the anterior EAC wall (37.5%), followed by the superior EAC and posterior walls (25% each). The inferior EAC wall was least affected (12.5%). There was no significant difference in recurrence between EACOs whose stalk insertions were drilled (proportion 0.09, 95% confidence interval [CI] 0.01-0.22) to the ones whose insertion was not drilled (proportion 0.05, 95% CI 0.00-0.17). The overall recurrence proportion was 0.07 (95% confidence interval 0.02-0.15). CONCLUSION: EACO insertion site drilling does not reduce recurrence and should be avoided in the absence of a definite pedicle projecting to the EAC lumen.

Large Peripheral Osteomas and Dental Implants: A Case Report.

Peripheral osteoma of the jaw is a rare, benign, slow-growing lesion, which usually appears as a unilateral, pedunculated, radiopaque mass protruding from the periphery and is generally solitary. Multiple osteomas without any syndromic involvement are rare. In the present case, a 75-year-old male patient underwent implant placement in the edentulous posterior ridges of the maxilla and mandible. Over 7 years, multiple masses gradually proliferated in the buccal bone of the implant in three different sextants of the posterior region, reaching a size of 2.0 cm. Clinically and radiologically, these lesions were presumed to be peripheral osteomas and were surgically removed because the large mass made self-performed oral hygiene and maintenance of peri-implant health difficult. The histopathological evaluation confirmed that peripheral osteomas were both compact and cancellous. The patient did not exhibit any other clinical manifestations of Gardner syndrome. Whether dental implant placement and loading are involved in the occurrence of peripheral osteomas is unclear, but they might have affected the consistent growth of the mass as a reactive mechanism. After resection, the functional abilities of chewing and self-cleansing significantly improved. No recurrence of peripheral osteoma was observed after 1 year of follow-up, and peri-implant health was well maintained. Within the limitations of the present case report, multiple peripheral osteomas can occur adjacent to dental implants without any syndromic issues, and a large mass of PO can harm peri-implant health which requires surgical removal. It is speculated that dental implants may be associated with the slow and consistent growth of PO.

The role of choriocapillaris vessel density in the pathogenesis of macular neovascularization associated with choroidal osteoma.

PURPOSE: To detect the vessel density of choriocapillaris (CC) vascular network in eyes affected by choroidal osteoma and in eyes complicated by macular neovascularization (MNV), using optical coherence tomography angiography (OCTA). METHODS: In this retrospective study, twenty-eight eyes of 28 patients were divided into three groups: group 1 including patients with calcified choroidal osteoma, group 2 including patients with decalcified choroidal osteoma, and group 3 including patients with decalcified choroidal osteoma complicated by MNV. OCTA analyzed the vessel density of CC in these lesions localized in the peripapillary region. RESULTS: We enrolled 12 eyes with calcified choroidal osteoma, 11 eyes with decalcified choroidal osteoma, and 5 eyes with decalcified choroidal osteoma complicated by MNV. The eyes with decalcified choroidal osteoma and MNV revealed a statistically significant reduction in vessel density of the CC respect to the other groups (p < 0.001). Moreover, the vessel density of CC in decalcified choroidal osteoma was significantly reduced compared to calcified choroidal osteoma (p < 0.001). CONCLUSIONS: OCTA allowed a quantitative evaluation of choriocapillaris vessel density in choroidal osteoma, in order to detect the changes of this vascular network, which could lead to the development of MNV. Therefore, OCTA could be a new diagnostic tool in the clinical management of the choroidal osteoma. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05342324.

Acquired Perforating Osteoma Cutis: A Rare Histopathological Diagnosis.

ABSTRACT: Perforating osteoma cutis is a benign proliferation of mature bone within the dermis and subcutaneous tissue of the skin with transepidermal elimination. Transepidermal elimination of bone is the hallmark of perforating osteoma cutis and is defined by the breaching of bone through the epidermis. Perforating osteoma cutis is exceptionally rare because only 6 cases have been recorded in the literature at the time of preparation of this report. In this report, we present the case of a 65-year-old female patient with a medical history of nonmelanoma skin cancer, hypertension, hyperlipidemia, and type II diabetes mellitus presented for evaluation of a skin lesion of the posterior lower left leg, which had been present for 1 year. Clinical and histopathologic findings were consistent with the diagnosis of acquired perforating osteoma cutis. Treatment with surgical removal by tangential biopsy has thus far proven to be both diagnostic and therapeutic because no recurrence has been noted as of 6 months.

Subdural osteoma in an adolescent patient with epilepsy: an unusual case report and literature review.

OBJECTIVE: Subdural osteoma (SO) is a rarely reported benign tumor, and there is no report of SO manifested with epileptic seizures. We aim to further the understanding of SO-related epilepsy. METHODS: Here, we report a meaningful case of epilepsy secondary to SO. A systematic review of the literature about SO using the electronic database PubMed and Web of science up to December 2022 was conducted. RESULTS: A 15-year-old girl presented with epileptic seizures for 8 years. Magnetic resonance imaging revealed an irregular lesion with heterogeneous signal in the right frontal convexity. Right frontal craniotomy was performed to remove the lesion. The pathological diagnosis was SO. Histological analysis revealed that the mechanosensitive ion channels Piezo 1/2 were upregulated in the brain tissue compressed by the osteoma, compared with the levels in the osteoma-free region. Seizure freedom was obtained during the 6-month follow-up after the surgery. We identified 24 cases of SO in 23 articles. With our case, a total of 25 cases with 32 SOs was included. Of 25 cases, 24 are adults, and 1 is a child. Seizure has been reported only in our case. Frontal osteoma was found in 76% of the patients. Symptoms were cured in 56% of the patients after surgery. CONCLUSION: Surgery is a safe and effective approach to the treatment of symptomatic osteoma. Mechanical compression on cerebral cortex may be a predisposing factor of the epileptogenesis caused by the SO.

Diagnosis and Management of Craniofacial Osteomas.

IMPORTANCE: Osteoid osteomas are benign bony overgrowths that can occur in any region of the body. However, they have a predilection to occur in the craniofacial region. Because of the rarity of this entity, there is a lack of literature detailing the management and prognosis of craniofacial osteoid osteomas. OBSERVATIONS: Craniofacial osteomas have a predilection to involve the paranasal sinuses, but can also be found within the jaw, skull base, and facial bones. Because of their slow-growing nature, craniofacial osteomas are often incidentally discovered on routine imaging or after they compress nearby structures or distort nearby anatomy. Osteoid osteomas of the face can be treated with resection via various approaches. Recent advancements describe minimally invasive endoscopic techniques and adjuvant therapy with radiofrequency ablation guided by cone biopsy computed tomography. Osteoid osteomas have an excellent prognosis with complete resection. They demonstrate a low incidence of recurrence when compared with other osteoblastic lesions of the craniofacial structures. CONCLUSIONS AND RELEVANCE: Craniofacial osteoid osteomas remain a developing topic within the field of craniofacial surgery. Their removal may be trending toward minimally invasive techniques. However, all treatment modalities appear to result in improved cosmetic outcomes and low recurrence rates.

Rare Recurrent Giant Frontal Osteoma With Skin Multiple Keratinous Cysts and Multinucleated Giant Cell Granulomas.

BACKGROUND: Osteoma is the most common benign tumor of the craniomaxillofacial region. Its etiology remains unclear, and the computed tomography and histopathologic examination contribute to its diagnosis. There are very rare reports of recurrence and malignant transformation after surgical resection. Furthermore, giant frontal osteomas that occurred repeatedly and were accompanied by skin multiple keratinous cysts and multinucleated giant cell granulomas have not been reported in previous literature. METHODS: The previous cases of recurrent frontal osteoma in the literature and all cases of frontal osteoma in our department in the last 5 years were reviewed. RESULTS: A total of 17 cases of frontal osteoma (mean age 40 y, all female) were reviewed in our department. All patients underwent open surgery to remove the frontal osteoma, and no evidence of complications was found during postoperative follow-up. Two patients underwent 2 or more operations due to the recurrence of osteoma. CONCLUSIONS: Two cases of recurrent giant frontal osteoma were reviewed emphatically in this study, including 1 case of giant frontal osteoma with skin multiple keratinous cysts and multinucleated giant cell granulomas. As far as we know, this is the first giant frontal osteoma that occurred repeatedly and was accompanied by skin multiple keratinous cysts and multinucleated giant cell granulomas.

Choroidal Neovascular Membranes in Retinal and Choroidal Tumors: Origins, Mechanisms, and Effects.

Choroidal neovascularizations are historically associated with exudative macular degeneration, nonetheless, they have been observed in nevus, melanoma, osteoma, and hemangioma involving the choroid and retina. This review aimed to elucidate the possible origins of neovascular membranes by examining in vivo and in vitro models compared to real clinical cases. Among the several potential mechanisms examined, particular attention was paid to histologic alterations and molecular cascades. Physical or biochemical resistance to vascular invasion from the choroid offered by Bruch's membrane, the role of fibroblast growth factor 2 and vascular endothelial growth factor, resident or recruited stem-like/progenitor cells, and other angiogenic promoters were taken into account. Even if the exact mechanisms are still partially obscure, experimental models are progressively enhancing our understanding of neovascularization etiology. Choroidal neovascularization (CNV) over melanoma, osteoma, and other tumors is not rare and is not contraindicative of malignancy as previously believed. In addition, CNV may represent a late complication of either benign or malignant choroidal tumors, stressing the importance of a long follow-up.

Computed tomography and magnetic resonance imaging of an unusual intraconal orbital osteoma.

We report the case of a 64-year-old male patient with a 5 month history of proptosis, motility limitation and vision loss in OD. Visual acuity (VA) was 20/200 in OD and 20/20 in OS. CT showed a large, round, intraconal lesion, with bony density and no apparent connection to adjacent orbital walls. MRI showed a T1-weighted hypointense lesion surrounded by a contrast enhancing capsule. The orbital tumor was excised through a lateral orbitotomy revealing a nodular, round, osseous structure. Histological examination disclosed well-formed lamellar bone trabeculae, with no necrosis or mitosis figures. Immunohistochemical staining was negative for MDM2 and CDK4. After 3 years, there was no evidence of tumor recurrence and VA had improved to 20/30. Intraconal osteomas with no clear attachment to orbital walls are extremely rare. We are aware of a few reported cases in the lid, hand, thigh, tongue, pterygopalatine fossa and brain. To the authors' knowledge, this is the first report in English literature of an orbital intraconal osteoma without any visible relation to the orbital walls.

[Mastoid osteoma]. / Osteoma sostsevidnogo otrostka.

Osteomas of the temporal bone are rare, especially osteomas originating from the mastoid process. Most often occur in women aged 20-30 years. The maximum growth rate is observed during puberty. Usually, the neoplasm becomes an accidental finding on X-rays or CT scans. The clinic of osteoma depends on its location and size. The patient may complain of a cosmetic defect, headache, discomfort and a feeling of heaviness in the area of the neoplasm. In this clinical case, a long-term asymptomatic course of osteoma of the temporal bone is described. CT examination was used for its visualization, and treatment was carried out surgically using a drill.

Recurrent CTNNB1 mutations in craniofacial osteomas.

Osteoma is a benign bone forming tumor predominantly arising on the surface of craniofacial bones. While the vast majority of osteomas develops sporadically, a small subset of cases is associated with Gardner syndrome, a phenotypic variant of familial adenomatous polyposis caused by mutations in the APC gene resulting in aberrant activation of WNT/ß-catenin signaling. In a sequencing analysis on a cohort of sporadic, non-syndromal osteomas, we identified hotspot mutations in the CTNNB1 gene (encoding ß-catenin) in 22 of 36 cases (61.1%), harbouring allelic frequencies ranging from 0.04 to 0.53, with the known S45P variant representing the most frequent alteration. Based on NanoString multiplex expression profiling performed in a subset of cases, CTNNB1-mutated osteomas segregated in a defined "WNT-cluster", substantiating functionality of CTNNB1 mutations which are associated with ß-catenin stabilization. Our findings for the first time convincingly show that osteomas represent genetically-driven neoplasms and provide evidence that aberrant WNT/ß-catenin signaling plays a fundamental role in their pathogenesis, in line with the well-known function of WNT/ß-catenin in osteogenesis. Our study contributes to a better understanding of the molecular pathogenesis underlying osteoma development and establishes a helpful diagnostic molecular marker for morphologically challenging cases.

Complications, treatments, and visual prognosis of choroidal osteomas.

PURPOSE: This study aims to report complications, treatments, and visual prognosis of choroidal osteoma. METHODS: We retrospectively reviewed electronic medical records and multimodal images of 41 patients with choroidal osteoma. RESULTS: Visually significant complications included choroidal neovascularization (CNV) in 21 (47.7%) eyes and subretinal fluid (SRF) without CNV in 14 (31.8%) eyes. The most common treatment was intravitreal anti-vascular endothelial growth factor (VEGF) injection: 13 (61.9%) eyes with CNV received an average of 6.3 injections, and 6 (42.9%) eyes with SRF but without CNV received 1.8 injections. As the first-line treatment, intravitreal anti-VEGF injection induced complete or partial remission in 93.4% of eyes with CNV and 57.1% of eyes with SRF. The probability of legally low vision estimated at 3 and at 5 years was 29.1% and 34.2%, respectively. The presence of CNV and outer retinal tubulation (ORT) was independent risk factors for vision loss (adjusted odds ratio, 8.08 and 6.94, respectively). CONCLUSIONS: The development of CNV and ORT was strong risk factors for visual impairment. Due to the frequent recurrence of complications and poor visual prognosis, regular check-ups and appropriate treatment choices are warranted.

Benign bony tumors of the paranasal sinuses, orbit, and skull base.

Benign bony tumors of the skull base and paranasal sinuses are uncommon entities, with an overall higher incidence in males. Benign bony tumors may lead to local expansion with resultant mass effect of potentially critical structures. Some benign bony tumors may undergo malignant transformation. This article reviews the presentation and management of benign bone tumors of the skull base and paranasal sinuses with special consideration to involvement of the adjacent orbit, intracranial and critical neurovascular structures. This review covers tumor incidence, location, gross and histologic appearance as well as radiographic findings, treatment, and recurrence rates. Tumors discussed in this article include osteochondromas, osteomas, osteoid osteomas, aneurysmal bone cysts, fibrous dysplasia, giant cell tumors, cemento-ossifying fibroma, ameloblastic fibro-odontoma, ecchordosis physaliphora, chondromyxoid fibroma, primary chronic osteomyelitis, primary chronic osteomyelitis, osteochondromyxoma, and dense bone islands.

Peripheral Osteoma of the Zygoma.

ABSTRACT: Osteoma is a benign, slow growing lesion that consists of compact or cancellous bone. Three types of osteomas could be classified: the central osteoma arising from the endosteum, the peripheral osteoma from the periosteum, and the extraskeletal soft tissue osteoma. in the craniofacial region, peripheral osteomas of the zygoma are quite rare. A literature review identified 7 cases of zygomatic arch and 3 cases of zygomatic body. This is the first report of zygomatic osteoma that was endoscopically removed. This report presents a rare case of osteoma of the zygoma and its endoscopic approach. The authors were able to confirm that endo-scopic approach of this zygomatic osteoma was safe and effective surgical choice.

Pediatric Cases of Recurrent Skull Giant Osteoma Misdiagnosed as Fibrous Dysplasia.

ABSTRACT: Osteomas are benign mature bone tumors that typically arise in the skull. Osteomas larger than 3 cm in diameter are considered giant osteomas. Giant osteomas of the skull vault are very rare, especially in children; therefore, only a few cases have been reported in the literature. Although osteomas are usually asymptomatic, a large skull mass can cause headache, as well as esthetic disfigurement of the forehead. it can be misdiagnosed as other conditions, such as fibrous dysplasia, ossifying cephalhematoma, or other malignant bone tumors. Herein, the authors report 2 rare pediatric cases of giant osteomas mimicking fibrous dysplasia and their successful surgical excision. These cases showed good results without recurrence or complications on long-term follow-up after complete excision.

Dual biologic therapy for severe ulcerative colitis, concomitant extraintestinal manifestations and an orbital osteoma: a successful treatment.

It is difficult to manage UC patients with tumor and extraintestinal manifestations (EIMs). We present this case of acute severe UC patient with peripheral arthritis and orbital tumor could be successfully managed by the concomitant use of two biologics (Dual targeted therapy, DTT) including vedolizumab (VDZ) and ustekinumab (UST). VDZ is approved for the treatment of patients with moderate-to-severe UC, but might have limited efficacy in treating EIMs due to its gut specific mechanism. UST shows clinical efficacy in the treatment of EIMs and is used for UC treatment.

Secondary choroidal neovascularization due to choroidal osteoma after 9 years follow-up.

BACKGROUND: Choroidal osteoma is a benign intraocular tumor that can increase risk of developing choroidal neovascularization. The visual prognosis is influenced by the tumor location, decalcification status, overlying RPE atrophy, presence of choroidal neovascularization, persistence of subretinal fluid and occurrence of subretinal hemorrhages. CASE PRESENTATION: The authors present a 40-year-old woman diagnosed with choroidal osteoma of the right eye. Her best corrected visual acuity was 12/20 but decreased to 5/20 due to secondary choroidal neovascularization after 8 years follow up. Fundus examination revealed an enlarged choroidal osteoma in most margins at posterior pole with schistose hemorrhage beside macula. Optical coherence tomography angiography revealed unique features in the vascular changes of choroidal neovascularization in choroidal osteoma in the outer retinal layer and choroid capillary layers, and subretinal neovascularization. Indocyanine green fluorescence angiography showed there was hypo-fluorescence at the peripapillary with faint hyper-fluorescence at the macular, corresponding to the location on the fundus photograph. The patient received 3 injections of intravitreal ranibizumab. After 1 year follow up, her visual acuity of the right eye was 18/20 and the CNV had regressed. CONCLUSIONS: We present the findings and treatment of a case of choroidal osteoma with secondary choroidal neovascularization. Optical coherence tomography angiography combined with FFA and ICGA is used to analysis the characteristics of secondary choroidal neovascularization. Optical coherence tomography angiography can reveal some unique characteristics in the vascular changes compared to fundus fluorescein angiography.

Giant Osteomas: Optimizing Outcomes Through Virtual Planning; a Report of Two Cases and Review of the Literature.

Osteomas are benign slow-growing tumors of compact or cancellous bone. They are often asymptomatic. However, a rare variety of "giant" osteomas exists, which can reach an impressive size, causing both functional and cosmetic concerns. The location and size of these lesions can make surgical intervention challenging. Here we present 2 cases of giant osteomas in which 3-dimensional planning was of great aid in optimizing functional and esthetic outcomes, and review the literature related to giant osteomas of the mandible.

Management of paranasal sinus osteomas: A comprehensive narrative review of the literature and an up-to-date grading system.

BACKGROUND: Surgical excision represents the unequivocal treatment modality for symptomatic paranasal sinus osteomas. However, the optimal surgical approach and the extent of the surgery, as well as the management stance in the case of an asymptomatic tumor, remain controversial. METHODS: The MeSH terms 'Osteoma', 'Nasal Cavity', and 'Paranasal Sinuses' were used to retrieve articles concerning the management of paranasal sinus osteomas that were published in the last 30 years, the vast majority of which comprised case reports of one or two cases. Original articles or large series of more than six cases were prioritized. RESULTS: Our review summarizes previous findings and opinions relevant to the management of symptomatic and asymptomatic paranasal sinus osteomas. The recent shifts in trends of their management are thoroughly discussed. Currently, an extension of the lesion through the anterior frontal sinus wall; an erosion of the posterior wall of the frontal sinus; a far-anterior intraorbital extension; an attachment to the orbital roof beyond the midorbital point; and some patient-specific adverse anatomic variations that may restrict access, are considered strong contraindications to a purely endoscopic approach. On the grounds of this thorough review, a new grading system for frontal and frontoethmoidal osteomas is proposed to allow better conformity to recent advancements and current clinical, research, and educational needs. CONCLUSION: Over the past 30 years, endoscopic techniques have emerged as the new standard of care for favorably located paranasal sinus osteomas. Nonetheless, open approaches remain indispensable for the management of the more perplexing cases of frontal sinus osteomas.