A Case Report of Craniofacial Intraosseous Xanthoma in a Patient Seeking Facial Feminization Surgery.

BACKGROUND: Fibrous dysplasia (FD) is a benign developmental disorder of the bone that causes normal skeletal tissue to be replaced by excess fibrous tissue and poorly differentiated osteoblasts. Intraosseous xanthomas are benign intraosseous tumor growths characterized microscopically by the presence of lipid-laden foamy histiocytes, often with cortical expansion or disruption. Although FD commonly occurs in craniofacial bones, primary intraosseous xanthomas of the skull or facial skeleton are extremely rare. Although 2 distinct conditions, each may be difficult to differentiate on CT imaging when occurring in the facial skeleton. METHODS: We report a case of an incidental finding on craniofacial CT of a frontal bone lesion originally thought to be FD. The finding was in a 55-year-old transgender woman who was assigned male at birth before receiving multiprocedural facial feminization surgery. RESULTS: The clinical features, radiological findings, and treatment are discussed. Postoperatively, the patient had no sequelae secondary to facial feminization surgery or to the orbital lesion biopsy procedure. Bone graft appeared stable on CT imaging, although FD did not appear to resolve completely. CONCLUSIONS: Diagnosis of such lesions is challenging and may require both radiographic and histopathologic assessment. As in the case of this patient, intraosseous xanthomas may also be misdiagnosed as other benign lesions such as FD. In most known cases, surgical intervention leads to complete resolution without recurrence of the lesion.

Isolated infantile myofibroma of the calvarium: Report of a case with a literature review.

OBJECTIVE: Infantile myofibromatosis is a rare entity of childhood characterized by benign myofibroblastic tumors in the soft tissues, the bones, and occasionally the viscera. Solitary skeletal lesions are relatively uncommon. Calvarial involvement should be distinguished from more aggressive tumors for appropriate treatment. METHODS: We reviewed solitary infantile myofibroma of the calvarium and discussed the relevant computed tomography and magnetic resonance imaging findings along with differential diagnosis. A case study of the frontal bone in a 5-month-old girl was also presented. RESULTS: Fourteen cases were reviewed, including the current case. Of the 13 cases with known sex, eight were male and five female. The mean age was 3.03 with an age range of 0.41-9 years. Nine of the 14 tumors were in the frontal bone. The lesions were intradiploic with tabula interna and/or externa of the calvaria involvement. The mean largest diameter was 22.3 mm. Upon computed tomography, all the lesions were expansile and lytic, and hypoattenuated, isoattenuated or occasionally hyperatenuated. Calcification was not seen. On magnetic resonance imaging, most neoplasms were hypointense on T1-weighted and T2-weighted images. Neoplasms showed hypointense signal on diffusion-weighted imaging and hyperintense on apparent diffusion coefficient, without restricted diffusion in three cases. All lesions were intensely enhanced after gadolinium administration. Treatment was total surgical resection and recurrence was not observed during follow-up. CONCLUSIONS: Infantile myofibromas are rare, typically intradiploic expansile lytic lesions with tabula interna and/or externa involvement. Distinctive imaging features include the presence of hipointense signals on T2-weighted magnetic resonance images without restricted diffusion on diffusion-weighted imaging. A slow-growing, firm, painless, and nontender mass with supportive imaging findings should raise suspicion of the disease.

Secondary aneurysmal bone cyst of the frontal bone with fibrous dysplasia showing rapid expansion: a case report.

A 19-year-old woman presented with swelling of the left forehead without pain. She did not have any relevant past or family history. Computed tomography showed destruction of the outer cortex of the frontal bone. A solitary mass lesion with a fluid collection was detected with magnetic resonance imaging. Because the swelling of the left forehead had enlarged rapidly with osteolytic changes, surgical removal of the lesion was performed. The lesion appeared to be enveloped in a fibrous capsule. The soft lesion was removed from the frontal bone. The outer frontal bone was absent, although the inner frontal bone was preserved. Then, the frontal bone was resected with margins from the edge of the erosion. The dura mater under the lesion was intact. A cranioplasty was performed using titanium mesh. On histological examination, the trabecular bones revealed irregular shapes and arrangements, indicating fibrous dysplasia. There was a continuous high-cell-concentration pathological lesion outside the fibrous dysplasia. There were numerous cells, such as mononuclear cells, osteoclast-like multinucleated giant cells, foam cells, and red blood cells. The osteoclast-like multinucleated giant cells and other cells did not show significant nuclear atypia. Immunostaining with H3.3G34W was negative, and the ubiquitin-specific peptidase 6/Tre-2 gene showed no rearrangements. The histopathological diagnosis was secondary aneurysmal bone cyst with fibrous dysplasia. Additional postsurgical therapy was not performed. There has been no evidence of recurrence of the lesion for two years.

Intraosseous malignant peripheral nerve sheath tumor at the frontal bone in a child: a rare cause of proptosis.

We report a nine-year-old male having malignant peripheral nerve sheath tumor (MPNST) of the frontal bone, represented with a twelve-month history of ptosis and proptosis in his right eye and enlarged rapidly in the last three months. Except for slight numbness at his one-third of the right forehead, he had no neurological deficit. The patient's both eyes were having normal eye movements, and he had no visual acuity or field loss. After surgery, we observed the patient with no recurrence for 4 years.

Heterotopic Ossification Bone Formation in the Frontal Bones of an African Grey Parrot (Psittacus erithacus).

A 5-year-old, male African grey parrot (Psittacus erithacus) was presented with multiple, slow-growing, firm, bilateral masses around the dorsal orbital rims. Computer tomographic imaging revealed mild, incomplete bridging bone formation on the rostrodorsal aspects of the head. A moderate amount of smooth bone formation was identified at the rostrodorsal aspect to the left orbit, with minimal associated soft tissue swelling. Surgical biopsies were collected from the masses and histopathological analysis of the most rostral right mass showed well-differentiated bone, surrounded by dense fibrous connective tissue. Scattered, well-differentiated osteocytes were present within the bone. No evidence of neoplastic changes or infectious agents were identified. The histopathological changes were consistent with metaplastic bone formation. History obtained from the owner revealed recent head trauma, which likely induced the cranial heterotopic ossification in the African grey parrot.

Frequency of hyperostosis frontalis interna in patients with active acromegaly: is there a possible role of GH excess or hyperprolactinemia in its etiopathogenesis?

PURPOSE: Acromegaly is characterized by bone changes due to excessive growth hormone (GH) secretion. Hyperostosis frontalis interna (HFI) is described as an overgrowth in the inner plate of the frontal bone. An increased incidence of HFI has been reported in patients with acromegaly. Since the etiology of HFI is poorly understood, we have analyzed whether there is a relationship between the hormonal and metabolic status of patients with acromegaly (with or without hyperprolactinemia) and the pathogenesis of HFI. METHODS: Forty-five patients with acromegaly and two control groups consisting of 25 patients with prolactinoma (group 1) and 47 healthy subjects (group 2) were included in this retrospective study. Baseline hormonal data and cranial imaging were obtained from medical records and analyzed. RESULTS: Mean frontal bone thickness was 6.75 mm in acromegaly, 4.85 mm in group 1, and 5.1 mm in group 2 of controls (p < 0.001). The frequency of HFI was higher in acromegalic patients than in the controls (22%, 0%, and 2.2%, respectively). There was no difference between the HFI positive and negative acromegalic patients in basal GH, IGF-1, and PRL levels, IGF-1 index, diagnosis lag time, and insulin resistance. There was no difference between groups regarding parietal and occipital bone thickness. CONCLUSION: Although the frequency of HFI is 22% in patients with acromegaly, neither excess GH nor hyperprolactinemia plays a role in its etiopathogenesis. Various genetic or epigenetic factors may contribute to its etiology.

Frontal Bone Osteomyelitis in Adult.

Frontal bone osteomyelitis is a rare clinical disease which occurs as a result of frontal sinusitis, penetrating injury on head, post-operative complication after sinus surgery and hematogenous spread from distant site. Early diagnosis, appropriate surgical debridement and antibiotic are keys to prevent from life threatening intracranial complications. We report a 63 years old male patient with osteomyelitis of outer cortex of frontal bone. The wound was thoroughly debrided after computer tomography scan showed an osteolytic lesion on outer table of fontal bone and antibiotic was continued for 2 months until inflammatory markers become normal. Keywords: Frontal bone; Intracranial complications; osteomyelitis; pott's puffy tumour.

Resolution of persistent traumatic supraorbital pain after neuroma excision.

We describe a case of an 18-year-old male who developed a supraorbital neuroma following facial trauma that occurred 2 years earlier. He presented with complaints of persistent facial pain and migraines despite successful laceration repair and removal of foreign bodies at the time of injury. A non-contrast computed tomography (CT) scan of the orbits revealed an enlarged supraorbital nerve with remodeling and expansion of the supraorbital notch, suggesting a neuroma. The patient underwent orbitotomy with excision of neuroma (confirmed histologically) and experienced a complete resolution of periorbital pain.

Computer-Aided Design and Manufacturing versus Conventional Surgical Planning for Head and Neck Reconstruction: A Systematic Review and Meta-Analysis.

BACKGROUND: Virtual surgical planning and computer-aided design/computer-aided manufacturing (CAD/CAM) for complex head and neck reconstruction has a number of cited advantages over conventional surgical planning, such as increased operative efficiency, fewer complications, improved osseous flap union, immediate osseointegrated dental implant placement, and superior functional and aesthetic outcomes. The authors performed a systematic review and meta-analysis of the available evidence on CAD/CAM maxillofacial reconstruction with the primary purpose of determining which approach is more efficacious. METHODS: In accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a PubMed and Embase database search was performed to identify English-language, human-subject studies of CAD/CAM-assisted head and neck reconstruction. All comparative studies were included in a meta-analysis to identify differences in operative time, ischemia time, surgical-site occurrence, microvascular complication, and partial or total flap loss between the two groups. All included studies (comparative and noncomparative) were used in the systematic review, summarizing the various flap characteristics, technical nuances, and functional and aesthetic outcomes. RESULTS: Twelve articles were included in the meta-analysis, representing 277 patients in the CAD/CAM group and 419 patients in the conventional group. CAD/CAM was associated with 65.3 fewer minutes of operating room time (95 percent CI, -72.7 to -57.9 minutes; p < 0.0001) and 34.8 fewer minutes of ischemia time (95 percent CI, -38 to -31.5 minutes; p < 0.0001). There were no significant differences in surgical-site occurrence, nonunion, flap loss, microvascular complications, or hardware-related complications. CONCLUSIONS: CAD/CAM is associated with shorter operating room and ischemia times. There are no significant differences in flap or hardware-related complications between CAD/CAM and conventional surgical planning.

Autopsy or anatomical dissection: evidence of a craniotomy in a 17th-eighteenth century burial site (Ravenna, Italy).

Surgical procedures undergone in life, autopsies and anatomical preparations can all leave clearly identifiable traces on human skeletal remains. Several studies on skeletons from archeological contexts have identified traces of these practices. However, the distinction between medical/forensic autopsy and anatomical dissections for scientific research can be challenging. We report the case of a middle-aged female skeleton from the cemetery of the church of San Biagio (Ravenna, Italy), dating back to the 17th-19th centuries, that shows signs of a complete craniotomy. In an attempt to clarify the reason for this practice, we analyzed all pathological and non-pathological markers on the skeleton. We carried out anthropological analyses and osteometric measurements to determine the biological profile and the cranial capacity of the individual. Paleopathological investigation and analyses of traumatic injury patterns were carried out using both a morphological and a microscopic approach. While we observed that the craniotomy was performed with a rip saw, we identified perimortem blunt force trauma to the frontal bone and an osteolytic lesion on the inner surface of the frontal bone. No other pathology was recognizable on the skeleton. Our differential diagnosis confidently proved that the craniotomy was due to an autoptsy procedure and was not the result of an anatomical dissection. We believe that, among other possible reasons, failed surgery could likely be the motive behind the ordering of the autopsy.

Multilobular tumor of bone on the forehead of a guinea pig.

Multilobular tumor of bone (MLTB) is an infrequent, slow-growing, bone neoplasm formed predominantly on the head. These tumors can behave as malignant neoplasms clinically and pathologically and can metastasize occasionally. No cases of MLTB in rodents have been reported, to our knowledge. We describe a novel case of an MLTB in a guinea pig. An adult guinea pig had an exophytic mass fixed on the frontal bone, maxilla, and nasal bone. On radiography, the mass had a spherical contour and variable density and was formed on the surface of the cranial bones. The mass was excised surgically. The cut surface was light-yellow to milky-white and had a granular texture with fine fibrous septa. Histologically, the neoplasm had a multilobular pattern, which consisted of many islands of bone and/or cartilage matrix surrounded by small cells and separated by fibrous septa, which closely resembles the equivalent neoplasm in dogs.

Epithelioid and spindle cell rhabdomyosarcoma with FUS-TFCP2 or EWSR1-TFCP2 fusion: report of two cases.

The WHO Classification of Tumors of Soft Tissue and Bone divides rhabdomyosarcoma (RMS) into alveolar, embryonal, pleomorphic, and spindle cell/sclerosing types. Advances in molecular diagnostics have allowed for further refinement of RMS classification including the identification of new subtypes. Very rare RMS with epithelioid and spindle cell morphology, female predominance, marked osseous predilection, ALK expression, EWSR1/FUS-TFCP2 gene fusions, and highly aggressive clinical behavior have recently been recognized with only 23 cases reported in the English language literature. Herein, we report two additional cases with detailed clinicopathologic description and molecular confirmation. In brief, two young women presented each with a primary bone tumor-one with a frontal bone tumor and another with an osseous pelvic tumor. Both tumors showed epithelioid to spindle cell morphology, ALK expression, and EWSR1/FUS-TFCP2 gene fusions. Both patients died of disease less than 17 months from diagnosis despite administration of multiple lines of aggressive treatment. In addition, we review the literature and discuss differential diagnostic and potential treatment considerations.

Radiological evaluation of Hyperostosis frontalis interna: is it of clinical importance?

Hyperostosis frontalis interna (HFI) presents irregular thickening of the frontal bone. Even though HFI is frequently seen during routine radiological imaging, it usually remains unrecorded owing to a common belief that it just represents an incidental finding or anatomical variant. Recent studies implied that HFI may be clinically relevant. Etiology of HFI is still debated, while presumptions are mainly based on altered sex steroids impact on skull bone growth. Some authors implied that frontal bone might be particularly affected by this condition due to specificity of its underlying dura. In this paper we present a 27-years old female patient with a treatment resistant headache. Head CT showed massive, irregular bony mass, with lobulated contours arising from the right frontal bone, but did not cross the fronto-parietal suture, spearing the superior sagittal sinus and skull midline. After surgery, histopathological analysis of the frontal bone sample in our patient showed thickening pattern similar to those described in micro-CT studies of HFI. Furthermore, in an attempt to test speculation of the possible role of estrogen in pathogenesis of HFI, we investigated the expression of α-estrogen receptors on dura of the frontal region. These analyses confirmed nuclear expression of estrogen on frontal region dural tissue, supporting previous speculation of the development mechanisms of HFI and contributing to a better understanding of this common condition of the frontal bone. Additionally, the presence of HFI may result in severe symptomatology, which could be misinterpreted and related to other disorders if HFI is not radiologicaly recognized and reported.

A rare case of orbital tuberculosis with cold abscess and frontal bone lesion.

Tuberculosis is a multisystem infectious disease caused by Mycobacterium tuberculosis and a leading infectious cause of morbidity and mortality worldwide. Orbital tuberculosis is a rare form of extra pulmonary tuberculosis, even in endemic areas. It may involve the soft tissue, lacrymal gland, periosteum, or bones of the orbital wall and can extend to adjacent paranasal sinuses or intracranial cavities. The delay in diagnosis can be due to the fact that the clinical signs simulate any inflammatory disease. The diagnosis is usually based on tissue examination in histopathological evidence presenting as granulomatous lesion or presence of acid fast bacilli (AFB). The long term anti tuberculosis therapy is the effective treatment. Here we present the case of orbital tuberculosis on a young man operated in our department and who had a good outcome under anti tuberculosis drugs for 12 months.

Osteoblastoma-Like Osteosarcoma of the Cuboid and Skull: A Case Report and Review of the Literature.

Osteoblastoma-like osteosarcoma is a rare form of osteosarcoma that shares similar clinical and pathological characteristics with osteoblastoma. We describe a 12-year-old boy with osteoblastoma-like osteosarcoma of the cuboid and skull without lung involvement. Despite inadequate primary surgical treatment, the patient recovered well after wide excision and postoperative chemotherapy. We report the case for the rarity of the sites and the misleading clinical and pathological manifestation.

Multiple Extraaxial Cavernous Hemangiomas: Rare Entity.

BACKGROUND: Cavernous hemangiomas arising in the extraaxial space are rarely encountered, often mimicking other more common pathologies. Furthermore, multiple coexisting lesions and posterior fossa involvement are scarcely reported. Herein, we present the case of a patient with concurrent frontal bone and posterior fossa extraaxial cavernous hemangiomas. We also review the challenges associated with the diagnosis and management of these entities. CASE DESCRIPTION: An otherwise healthy 41-year-old gentleman presented with a 7-month history of a progressive right forehead mass. Imaging demonstrated a right frontal bone mass and an incidentally noted transverse sinus-based mass. The patient opted for surgical resection of both lesions. A right frontal craniotomy was performed to remove the bony lesion, followed by a suboccipital approach for the dural-based mass. There were no significant complications intraoperatively, and gross total resection was achieved for both lesions. Final pathology for each was consistent with cavernous hemangioma. CONCLUSIONS: Extraaxial cavernous hemangiomas are uncommon clinical entities that are challenging to distinguish from other diseases. If intraoperative complications can be avoided, treatment with surgical resection often offers excellent patient outcomes.

Brain and frontal-bone metastasis from nasopharyngeal carcinoma: Case report and literature review.

BACKGROUND: Metastases to the bones, lungs, and liver are common in patients with nasopharyngeal carcinoma (NPC) but not to the brain and frontal bone (B + FB). METHODS: We describe a patient with NPC with B + FB metastasis. He received two cycles of palliative chemotherapy (gemcitabine and cisplatin) and then radiotherapy (60 Gy) for B + FB metastasis. A literature review of previous cases was also undertaken. RESULTS: Follow-up 6 months after completion of chemotherapy and radiotherapy showed that our patient experienced a complete response without signs of NPC progression. CONCLUSIONS: B + FB metastases from NPC are uncommon. Our case highlights the diagnostic and treatment difficulties clinicians face when dealing with patients with uncommon sites of metastasis. Optimal adjuvant therapy followed by local radiotherapy might elicit long survival in patients with NPC with uncommon sites of metastasis.