Misdiagnosed long-standing unilateral nasal obstruction: ossifying fibroma of the inferior turbinate.

A man in his 20s presented with complaints of unilateral nasal obstruction for the past 6 years that progressively worsened leading to irrational use of over-the-counter nasal decongestants. With the worsening of symptoms, a non-contrast CT was done. It showed a dense expansile sclerotic lesion of the right inferior turbinate, which was excised endoscopically. Cemento-ossifying fibromas of the inferior turbinate are rare and require assessment and surgical excision to relieve the symptom of nasal obstruction. It derives its name from the variable proportions of fibrous and mineralised tissue present in it and exclusively develops in the craniofacial region. It can be surgically managed by an endoscopic, an endonasal non-endoscopic (with a speculum) or an open approach (lateral rhinotomy, sublabial approach or mid-facial degloving). Here, we present how such a case was detected and managed surgically by the endoscopic approach, which is a minimally invasive option with shorter hospital stay and early recovery.

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.

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension.

BACKGROUND AND OBJECTIVE: Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor. CLINICAL PRESENTATION: This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course. CONCLUSION: The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.

[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.

Kaposiform hemangioendothelioma of skull base with dura invasion in a pediatric patient: a case report.

Kaposiform hemangioendothelioma is an extremely rare vascular tumor which shows aggressive local growth. We present a case of rapid growing vascular skull tumor with dura invasion in a pediatric patient with neurofibromatosis type 1. A 14-year-old male complained of headache and dizziness for 1 month after minor head trauma. Brain magnetic resonance imaging (MRI) revealed a 5-cm-sized tumor in the left frontotemporal bone with internal hemorrhage and cystic changes. The gross total resection of tumor was done. At the 7-month follow-up, brain MRI revealed a recurrent skull tumor with intracranial dura mass. He underwent second surgery, and the pathologic diagnosis was suggestive of Kaposiform hemangioendothelioma. For this vascular proliferative tumor, mTOR inhibitor was treated for 6 months, and there was the recurred nodular-enhancing mass along the sphenoid ridge. After additional 2 months of medication, the following MRI revealed a decreased nodular-enhancing mass.

Endoscopic Approach for Resection of Frontal Forehead Osteoma: Technical Case Report Instruction.

Forehead osteomas are benign bone tumors. They are frequently associated with exophytic growth in the outer table of the skull, causing cosmetic disfigurement of the face.1-9 The objective of this study was to present the efficacy and feasibility of the endoscopic treatment of forehead osteomas by presenting a case report with details of the surgical technique. A 40-year-old female patient presented with aesthetic complains of a progressing bulge in the forehead. A computed tomography scan with 3-dimensional reconstruction showed bone lesions on the right side of the forehead. The patient underwent surgery under general anesthesia with no noticeable incision, which was planned 2 cm behind the hairline in the midline because the osteoma was close to the midline plane on the forehead (Video 1). A retractor coupled with a 4-mm channel for endoscopy and a 30-degree optic was used to dissect, elevate the pericranium, and locate the 2 bone lesions in the forehead. The lesions were removed using a chisel, endoscopic facelifting raspatory, and a 3-mm burr drill. The tumors were resected completely, resulting in good cosmetic outcomes. The endoscopic approach for treating forehead osteomas is less invasive and facilitates complete removal of tumors, which results in good cosmetic outcomes. Neurosurgeons should consider and add this feasible approach to enhance their surgical armamentarium.

Genomic Profiling of the Craniofacial Ossifying Fibroma by Next-Generation Sequencing.

BACKGROUND: Ossifying fibroma (OF) of the craniofacial skeleton is a fibro-osseous lesion characterized by various patterns of bone formation in a cellular fibroblastic stroma. The molecular landscape of OF remains mostly unknown. There are a few known pathogenic abnormalities in OF, including HRPT2 mutations in conventional OF and SATB2 translocations in juvenile psammomatoid OF. On the other hand, conflicting reports exist regarding MDM2 gene amplification and chromosomal copy number alterations (CNA) in OF. METHODS: Surgically removed biopsies and curettage specimens from OF patients were obtained. Clinical, radiographic, and pathologic features of tumors were reviewed. Genomic DNA was extracted from formalin-fixed, paraffin-embedded blocks of tumor tissue. Capture-based DNA next-generation sequencing targeting the coding regions 529 cancer genes and select introns was performed. RESULTS: We identified 17 OF cases from 8 male and 8 female patients with mean age of 22 years (range 1-58 years). Nine case occurred in the gnathic bones and 8 in the extragnathic craniofacial bones. These cases included 3 juvenile psammomatoid OF, 6 conventional OF and 8 juvenile trabecular OF. Large-scale CNAs were present in 6 of 17 cases. Seven cases (41%) had focal amplifications including FOSB (n = 2, 11%), FOS (n = 4, 23%), COL1A1 (n = 4, 23%) and TBX3 (n = 5, 29%). Three cases (17%) had pathogenic CDC73 mutations. No cases showed focal MDM2 amplification. CONCLUSIONS: Here, we provided a comprehensive molecular characterization of OF that reveals a heterogeneous genetic profile with occasional large-scale CNAs (n = 6, 35%). FOS, FOSB, and TBX3 genes that regulate AP-1 transcriptional complex are frequently altered in OF (n = 7, 41%), chiefly in juvenile trabecular OF. These genes encode transcription factors that act as downstream effectors of the MAP kinase signaling pathway. MDM2 amplification is an exceedingly rare event in OF, if present at all, so identification of this event should continue to raise concern for low-grade gnathic osteosarcoma. In summary, our findings suggest that OF represents a heterogeneous group of tumors at the genetic level but dysregulation of the AP-1 pathway may play a role in pathogenesis of juvenile trabecular OF.

A Clinical, Radiological and Histopathological Review of 74 Ossifying Fibromas.

BACKGROUND: Ossifying fibroma (OF) is a fibro-osseous lesion of the jaws and craniofacial bones. Accurate diagnosis can be challenging due to significant overlap of clinicopathological features. This study aimed to evaluate the clinical, radiological and histological features that can aid in diagnosis and identify characteristics that allow categorisation into the three subtypes: juvenile trabecular, psammomatoid and cemento-ossifying OF. METHODS: A total of 74 cases of OF were systematically reviewed for their principle features. Of these, 46 cases were evaluated for their radiographic features including size, location and relationship to the teeth. Histological assessment and stereological point counting were performed in 69 cases to assess the pattern, type and proportion of calcification, the nature of the stroma, the border of the lesion and the presence of secondary changes. Fisher's exact test and Chi-squared tests were used to determine associations between clinicopathological parameters and maxillary, mandibular, odontogenic, non-odontogenic and psammomatoid or trabecular lesions. RESULTS: OF showed a female predilection (F: M; 2:1) and a slight bimodal age distribution with peaks in the second (23%) and fourth decades (27%) (Mean age: 32.4 years). 83% of cases presented as an intra-oral swelling, with the mandible being the most common site (73%). Histologically, a range of morphological patterns were seen, with 50% of cases showing mixed trabecular and psammomatoid features. However, there were no significant differences between the variants of OF in terms of age, gender or histological features. CONCLUSION: Histological features of OF cannot be used to differentiate between the subtypes.

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.

Extradural hematoma from trauma to midline skull tumour.

We present a case of extradural hematoma resulting from a relatively minor closed injury over the vertex where a plasma cell tumour had invaded the superior sagittal sinus. The patient underwent an emergency craniotomy and evacuation of the hematoma. Hemostasis and prevention of recollection of the hematoma were hampered by the erosion of the sagittal sinus making its direct repair impossible. This was achieved by hitching up the dura lateral to the sinus to become its lateral wall reinforced by hemostatic agents. The patient made a full recovery. Malignant tumours invading the dural venous sinuses and eroding the skull can cause life-threatening intracranial bleeding after relatively minor trauma.

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.

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.

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.

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.

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.