Cavernous Hemangioma of the Mastoid Antrum.

Hemangioma is a common vascular neoplasm that arises in the head and neck regions but is rare in the petrous bone. We report the first case of a solitary cavernous hemangioma in the mastoid antrum. A 68-year-old woman visited our hospital with a complaint of tinnitus without any other symptoms. Tinnitus of the right ear occurred especially when the patient yawned or swallowed. Both tympanic membranes appeared normal on otoscopic examination. On pure-tone audiometry, mild hearing loss up to 25 dB was detected in the right ear. Temporal bone computed tomography revealed a 7.0 mm × 4.5 mm × 5 mm, solitary soft tissue mass in the aditus ad antrum. Excisional biopsy was performed under general anesthesia through the canal wall as in a mastoidectomy. The mass was completely removed without any bleeding or ossicular chain damage. The mass was confirmed as a cavernous hemangioma. During follow-up, the patient's tinnitus and right low-tone hearing loss improved. No solitary hemangioma of the mastoid antrum has been reported previously. Surgical excision of the lesion appears to be proper treatment to achieve pathologic confirmation along with resolution of symptoms.

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.

DNA Mutational and Copy Number Variation Profiling of Primary Craniofacial Osteosarcomas by Next-Generation Sequencing.

BACKGROUND: Craniofacial osteosarcomas (CFOS) are uncommon malignant neoplasms of the head and neck with different clinical presentation, biological behavior and prognosis from conventional osteosarcomas of long bones. Very limited genetic data have been published on CFOS. METHODS: In the current study, we performed comprehensive genomic studies in 15 cases of high-grade CFOS by SNP array and targeted next generation sequencing. RESULT: Our study shows high-grade CFOS demonstrate highly complex and heterogenous genomic alterations and harbor frequently mutated tumor suppressor genes TP53, CDKN2A/B, and PTEN, similar to conventional osteosarcomas. Potentially actionable gene amplifications involving CCNE1, AKT2, MET, NTRK1, PDGFRA, KDR, KIT, MAP3K14, FGFR1, and AURKA were seen in 43% of cases. GNAS hotspot activating mutations were also identified in a subset of CFOS cases, with one case representing malignant transformation from fibrous dysplasia, suggesting a role for GNAS mutation in the development of CFOS. CONCLUSION: High-grade CFOS demonstrate highly complex and heterogenous genomic alterations, with amplification involving receptor tyrosine kinase genes, and frequent mutations involving tumor suppressor genes.

Association of imaging classification and histopathological grading in primary intraosseous meningioma of the skull.

BACKGROUND: Primary intraosseous meningioma of the skull (PIMS) is a rare type of primary extradural meningioma (PEM) involving cranial bone. The existing literature strongly suggest the importance of radiological feacures in pathological diagnosis of PIMS. Thereby, the aim of this study is to investigate the association between imaging classification and histopathological grading in PIMS. METHODS: In this retrospective study, we retrospectively analyzed the computed tomography scan/magnetic resonance imaging and pathological data pertaining to patients with pathologically proven PIMS. The association between radiological features, imaging classification, and histopathological grading was analyzed using logistic regression analysis. RESULTS: In this study, data of 25 patients with PIMS were assessed. The univariate logistic regression analysis results showed significant correlation between histopathological grading and imaging classification (OR: 22.5; 95% CI: 2.552-198.378; p = 0.005), intra- and extracalvarial extension (OR: 7.2; 95% CI: 1.066-48.639; p = 0.043), and tumor margin (OR: 7.19; 95% CI: 1.06-47.61; p = 0.043). According to the results of multivariate logistic regression analysis, imaging classification was the strongest independent risk factor for high-grade PIMS, and the risk of aggressiveness of osteoblastic type of PIMS was 16.664 times higher than that of osteolytic type of PIMS (OR: 16.664; 95% CI: 1.15-241.508; p = 0.039). CONCLUSIONS: Imaging classification is an independent risk factor for high-grade PIMS.

Craniomaxillofacial Fibro-osseous Lesions in Children.

Craniofacial fibro-osseous lesions represent a diverse spectrum of pathologic conditions where fibrous tissue replaces healthy bone, resulting in the formation of irregular, woven bone. They are more commonly diagnosed in young people, with treatment strategies dependent on clinical behavior and skeletal maturity. This article discusses the examples of craniofacial fibro-osseous lesions, based on the latest classifications, along with their diagnostic criteria and management.

Desmoplastic fibroma of the pediatric cranium with CTNNB1 mutation: case report and literature review.

PURPOSE: Desmoplastic fibroma (DF) is an uncommon intermediate bone tumor rarely involving the skull with unidentified pathogenesis. We report the first case of pediatric temporoparietal cranial desmoplastic fibroma (DF) with a CTNNB1 gene mutation and review the previous literature. CASE PRESENTATION: A 3-year-old boy had a firm, painless mass on the right temporoparietal region for 22 months. The cranial CT scan showed isolated osteolytic destruction in the outer plate and diploe of the right temporoparietal bone. Gross total resection of the lesion and cranioplasty were performed. After that, a growing epidural hematoma was observed so another operation was performed to remove the artificial titanium plate. Postoperative pathology indicated a DF diagnosis and molecular pathology suggested a missense mutation in exon 3 of the CTNNB1 gene (c.100G > A,p.Gly34Arg). CONCLUSION: Pediatric cranial DF is rare and easy to be misdiagnosed before operation. For cranial DF, lesion resection can be performed and perioperative management should be strengthened. Mutations in the CTNNB1 gene might be one of the molecular pathologic features of DF.

Melanotic neuroectodermal tumor of infancy arising in the skull: a case report.

Melanotic neuroectodermal tumor of infancy is a rare and usually benign neoplasm occurring in children of young age. This pigmented tumor typically presents in the head and neck region, but other locations may be involved. We report in this article a rare case of a 3-month-old girl presenting with a slowly growing mass localized in the anterior fontanelle. The patient's magnetic resonance imaging (MRI) showed a mass extending both extracranial and intracranial, and compressing the adjacent structures. The patient underwent subtotal resection of the mass and a histological study confirmed the diagnosis of melanotic neuroectodermal tumor of infancy. The patient presented later on with a recurrence. An early diagnosis and surgical management for these tumors remain the only guarantees to limit the progression and prevent their recurrence and metastasis.

Cemento-ossifying fibroma transforming to osteosarcoma.

Ossifying fibroma is a type of fibro-osseous lesion categorised into cemento-ossifying fibroma and juvenile ossifying fibroma. Malignant transformation of fibro-osseous lesions is documented especially for fibrous dysplasia, but scarcity is seen when we search for malignant transformation of ossifying fibroma. Thus, we are presenting an extremely rare case of cemento-ossifying fibroma transforming into osteosarcoma with long sequential radiographic details.

Single-port Endoscopic Removal of Forehead Osteoma: An Otolaryngologist's Procedure.

For otolaryngologists, single-port endoscopic removal of forehead osteoma draws upon a familiar skill set and is a robust technique for complete tumor removal with excellent cosmesis. Laryngoscope, 134:2194-2197, 2024.

[Primary intraosseous cavernous hemangioma of the cranium: a case report]. / Pervichnaya vnutrikostnaya kavernoznaya gemangioma kostei cherepa: obzor literatury i opisanie klinicheskogo nablyudeniya khirurgicheskogo lecheniya.

Primary intraosseous cavernous hemangioma (PICH) is a rare benign vascular tumor. This neoplasm is common in the spine and less common in skull. Toynbee J. first described this tumor in 1845. PICH of the cranium does not always have typical X-ray features and should be always differentiated with other more common skull lesions. Surgical resection is preferable since total resection is followed by favorable prognosis. We present a 65-year-old patient with asymptomatic tumor of the right parietal bone. CT revealed osteolytic lesion that required total resection and skull repair. Histopathological analysis revealed intraosseous cavernous hemangioma.

Spontaneous Regression of Ossifying Fibroma in the Oral and Maxillofacial Region: A Rare Case With 4-Year Follow-Up.

The case presented in this study is a rare instance of an ossifying fibroma (OF), a type of benign fibro-osseous lesions, spontaneously regressed without surgical intervention. A 9-year-old boy with an intraosseous lesion in the left maxilla was diagnosed as OF. The surgeon suggested surgical excision, but due to personal reasons, the patient and his parents deferred the surgery and opted for regular follow-up. During a 4-year follow-up, the surgeon found that the lesion had significantly decreased in size and the facial deformity had remitted. At the latest follow-up, the deformity of his left face became almost unnoticeable, and the lesion seemed to be subtle on cone-beam computed tomography images. This case highlights the possibility of spontaneous regression of OF. This phenomenon may occur due to the teeth eruption, which can lead to the formation of periodontal ligaments and engage the regression of OF.

Multiple primary intraosseous meningioma mimicking fibrous dysplasia: a case report.

Primary intraosseous meningioma (PIM) is a rare subtype of primary extradural meningiomas. These rare ectopic meningiomas have been usually reported in the frontotemporal regions of the calvarium, orbits, and anterior cranial fossa. We report a case with bilateral tumors located in frontoparietal regions of calvarium. Our initial diagnosis was fibrous dysplasia but the lesions were seen to expand under follow-up. One was resected and the histopathological diagnosis was PIM. This is the second reported case of multiple PIM.

Concomitant giant purely intradiploic cavernous haemangioma and multifocal neurotoxoplasmosis.

Purely calvarial or intradiploic cavernous haemangiomas (PICHs) are rare benign tumours accounting for 0.2% of all bone tumours and 10% of benign skull tumours. They are generally small, slow-growing and asymptomatic lesions. Here the authors described an immunocompromised patient with concomitant giant intradiploic ossified globular cavernous angioma and multifocal neurotoxoplasmosis that underwent a combined approach to treat both lesions with an en-bloc resection of the right parietal intradiploic lesion and biopsy of the left occipital subcortical lesion.Indeed, it is essential to exclude the presence of metastases by making a timely differential diagnosis. En-bloc surgical resection of purely intradiploic ossified cavernous angioma is the gold standard treatment and the prognosis after a complete excision is usually excellent with rarer recurrence rate.

Primary Intraosseous Cavernous Hemangioma of the Cranium: A Systematic Review of the Literature.

Primary intraosseous cavernous hemangioma (PICH) is a rare, benign tumor of vascular origin, typically arising in the vertebral body. Its presence in the skull is exceedingly rare, with only a few cases being reported worldwide. We carried out the first systematic review of the literature, covering the epidemiology, clinical and imaging features, management, and prognosis of cranial PICH. The literature search revealed 51 studies with 77 patients; the mean age of the patients was 32.7 years with a female predominance of 1.4:1. The majority of cranial PICHs were located in the calvarium, primarily in the frontal and parietal regions, with only a few located in the skull base. The most common initial clinical manifestation was local growth or swelling, followed by a headache. Radiographically, PICHs represented osteolytic, intradiploic masses, which in many cases displayed trabeculations, leading to the so-called "honeycomb" or "starburst" pattern. After contrast administration, PICHs typically enhance. Tumor removal, with craniectomy or en bloc resection and subsequent skull reconstruction, was selected for calvarial PICHs, whereas a transsphenoidal approach, with only partial resection, was applied for clival/sella PICHs. Preoperative embolization, aiming to minimize intraoperative blood loss, was performed in the case of large tumors. At a mean follow-up of 39 months, no patient experienced tumor recurrence, even after subtotal resection. Owing to the benign nature of the tumor, maximal safe resection is recommended as the treatment of choice for patients with cranial PICH.

Giant Skull Tumor: A Benign Meningioma.

ABSTRACT: A 37-year-old male patient was complaining from painless forehead swelling, which started 5 years ago. Brain computed tomography scan and magnetic resonance imaging showed a large extradural mass compressing the both frontal lobes with skull bone infiltration, hyperostosis and enlargement. The patient was operated in 2 stages. In the first stage, the authors achieved separation for the tumor from the scalp and skull. In the second stage after 1 week, the authors continued with circumferential dural opening around the tumor and separation of the tumor from brain tissue. Duroplasty was performed by autologous fascia latta and skin flap was closed primarily. The specimen was received grossly as huge mass weighing 1530 g and measuring 39 × 16 × 12 cm. Histopathologic examination showed grade I meningioma extending beyond the skull to the surrounding soft tissue.

Two-step treatment of a giant skull vault hemangioma: A rare case report and literature review.

Skull vault hemangiomas are benign vascular tumours of the calvaria that are usually asymptomatic or present as firm, painless lumps. We present a case of a 59-year-old female with a giant intraosseous calvarial hemangioma that was admitted in our department with a palpable mass over the left frontoparietal region, personality changes and impaired emotional and cognitive functions. The patient was treated with a two-step approach involving endovascular and surgical treatment, and suffered two rare, but recognized complications, a contrecoup intracerebral haemorrhage and valproate-induced stupor and parkinsonism. At the 6-month follow-up, the patient had complete recovery with a good neurological outcome.

Ossifying Fibroma of Non-odontogenic Origin: A Fibro-osseous Lesion in the Craniofacial Skeleton to be (Re-)considered.

In the cranio-facial skeleton, a heterogeneous group of well characterized fibro-osseous lesions can be distinguished. Whereas fibrous dysplasia can affect any skeletal bone, ossifying fibroma and cemento-osseous dysplasia exclusively develop in the cranio-facial region, with most subtypes restricted to the tooth bearing areas of the jaws. Herein we present a series of 20 fibro-osseous lesions that developed mostly in the frontal bone and in the mandible, presenting as expansile intramedullary tumors with a unique histologic appearance and an indolent clinical course. We provide evidence that these tumors are distinct from the categories included in the WHO classification and are therefore currently unclassifiable. The definition of cemento-ossifying fibroma as an odontogenic neoplasm developing only in close proximity to teeth should be re-considered and incorporate also extragnathic lesions as shown here.

"Slipped capital femoral epiphysis in a 25-year-old hypogonadic man with a large cranial chondroma: causality or coincidence? ".

BACKGROUND: Slipped capital femoral epiphysis (SCFE) is a hip disorder frequently occurring in adolescence. In adults it is rare and so far very few cases have been documented. CASE PRESENTATION: This report presents a 25-year-old patient diagnosed with an anterior fossa giant chondroma, hypogonadotropic hypogonadism, and SCFE. The patient underwent surgical and hormonal therapy. His symptoms revealed, and he became a father. CONCLUSIONS: Every patient diagnosed with SCFE in adulthood should undergo endocrinological assessment based on physical examination and laboratory tests.

Multiple myeloma complicated by skull plasmacytoma discovered after head injury.

Plasmacytoma is a malignant tumor originating from the plasma cells of the bone marrow. Those discovered after a head injury is rare. We report a case of a 48-year-old female who complained of scalp mass without other symptoms after head injury. Meningioma was considered preoperatively based on imaging findings, and surgical resection was performed. Postoperatively, multiple myeloma complicated by skull plasmacytoma was diagnosed by histopathology and systematic examinations in succession. When evaluating a head mass that appeared after a head injury, plasmacytoma should be considered at times. Osteolytic changes and biconvex form on imaging are beneficial to differentiation.

Programmed Death-Ligand 1 Expression and Tumor-Infiltrating Lymphocytes in Temporal Bone Squamous Cell Carcinoma.

OBJECTIVES/HYPOTHESIS: The tumor immune microenvironment in temporal bone squamous cell carcinoma (TBSCC), including the programmed death-ligand 1 (PD-L1) expression and tumor-infiltrating lymphocytes (TILs), has not been established. STUDY DESIGN: Retrospective cohort study. METHODS: We performed immunohistochemistry analyses to retrospectively analyze 123 TBSCC cases for PD-L1 expression and TILs and their prognostic significance. We also evaluated the prognostic correlations between these immunomarkers and the therapeutic responses to chemoradiotherapy (CRT). RESULTS: PD-L1 expression (≥1%) was detected in 62 (50.4%) TBSCC cases and was significantly associated with worse prognosis: progression-free survival (PFS), P < .0001; overall survival (OS), P = .0009. A high density of CD8+ TILs was significantly associated with better prognosis (PFS, P = .0012; OS, P = .0120). In contrast, a high density of Foxp3+ TILs tended to be associated with an unfavorable prognosis (PFS, P = .0148; OS, P = .0850). With regard to the tumor microenvironment subtypes defined by CD8+ TILs and PD-L1 expression, the CD8low /PD-L1+ group showed significantly worse prognosis. Among the 36 neoadjuvant CRT-treated cases, PD-L1 expression was significantly associated with worse OS (P = .0132). Among the 32 CRT-treated cases without surgery, a high density of CD8+ TILs tended to be more highly associated with complete response to CRT compared to a low density of CD8+ TILs (P = .0702). CONCLUSIONS: These results indicate that the evaluation of the tumor immune microenvironment may contribute to the prediction of prognoses and the selection of an individualized therapeutic strategy for patients with TBSCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2674-2683, 2021.