Extracranial temporal bone paragangliomas: Re-defining the role of otologic surgery within the scope of function-preserving multimodal concepts.

INTRODUCTION: The aim of this study was to present our concept in the management of extracranial temporal bone paragangliomas and demonstrate the outcome after primary surgical management of the middle ear component, with an individualized indication for adjuvant radiotherapy. MATERIALS AND METHODS: The records of all patients treated for extracranial jugulotympanic paragangliomas by means of primary surgical management between 2010 and 2021 were studied retrospectively. RESULTS: Twenty-nine patients made up our study sample (mean age 58.8 years). 15 cases were managed solely by means of surgery. Out of the remaining 14 cases with reduction of the middle ear component, adjuvant irradiation was performed in 11 cases, whereas a wait-and-scan strategy was adopted at the patient's request in three cases. No further growth was detected in our study cases. CONCLUSION: Our protocol seems to be associated with an acceptable quality of life and a satisfactory oncologic outcome.

"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.

Primary yolk sac tumor of pterygopalatine fossa with loss of vision: A case report.

INTRODUCTION: Primary yolk sac tumor (YST) is an infrequently-diagnosed malignant extragonadal germ cell tumors. It is likely to recur locally and may present with widespread metastases once diagnosed. Primary YST of the head is uncommon but can cause severe complications, such as loss of vision once the tumor mass invades the optic nerve. PATIENT CONCERNS: A 20-month-old boy presented to the general clinic of the local children's hospital with a complaint of swelling of left face for 1 year and proptosis of the left eye for over 2 weeks as stated by his parents. Initially, he did have some vision, as he could walk by himself, but a special ophthalmologic examination was not performed. DIAGNOSES: Cranial computed tomography and magnetic resonance imaging revealed a large tumor accompanied by peripheral bone destruction in the left pterygopalatine fossa that extended to sphenoid, ethmoid, left maxillary sinuses, left nasoethmoid, and left orbit. The optic nerve was invaded on both sides. Chest and abdominal imaging were normal. A primary diagnosis of Langerhans cell hyperplasia was made. However, blood tests on the second day of hospitalization revealed significantly elevated serum alpha-fetoprotein levels. On the third day, the boy lost his eyesight, with loss of pupillary and no light sensation during flashlight stimulation on both sides. INTERVENTIONS: Nasal endoscopy was performed on the fourth day, the vast majority of soft tissue mass was resected for biopsy. Histopathological examination revealed features of endodermal sinus tumor. A final diagnosis of primary YST of pterygopalatine fossa was made. Because the mass could not be resected completely, he received combined chemotherapy with bleomycin, etoposide, and carboplatin for 6 cycles over six months. OUTCOMES: The patient recovered with significant tumor shrinkage and without secondary metastasis after 18 months but left permanently blind. CONCLUSION: The worst complication of loss of vision after Primary YST of pterygopalatine fossa alerts us that close physical examination during the initial investigation should be performed, which is especially important in young children who cannot express complaints well. Early detection and treatment with surgical resection and chemotherapy may contribute to satisfactory outcomes and avoidance of visual impairment.

Spontaneous CSF rhinorrhea as a presenting symptom of a clival chordoma / Fístula espontánea de LCR como síntoma de presentación de un cordoma de clivus

CSF rhinorrhea is a rare presenting symptom of a clival chordoma as these tumors do not tend to show dural invasion as a histological feature. Here we discuss the case of a 61 years old female with spontaneous rhinorrhea secondary to a clival chordoma, and the value of some specific MRI sequences in accurate identification of a tumor with such atypical presentation, enabling the surgeon to plan accordingly (AU)

Los cordomas de clivus en muy raras ocasiones se presentan como fístulas espontáneas del líquido cefalorraquídeo, ya que estos tumores no suelen invadir el plano dural. Se presenta el caso de una paciente de 61 años con rinolicuorrea espontánea secundaria a un cordoma de clivus, haciendo énfasis en secuencias específicas de resonancia magnética para la correcta identificación de un tumor con una manifestación inicial tan atípica, permitiendo una planificación quirúrgica adecuada (AU)

Sphenoid Wing Meningioma Presenting as Sudden Sensorineural Hearing Loss: A Case Report and Literature Review.

Sphenoid wing meningiomas are tumors that typically present with vision deterioration and neurological changes due to their proximity to the sella, cavernous sinus, and other vital structures. Some unusual symptoms have also been described in the literature, such as cognitive dysfunction, parkinsonism, and intracerebral hemorrhage. In this report, we detail another unusual case of sphenoid wing meningioma in a 63-year-old female who presented with left sudden sensorineural hearing loss. A brief review of the literature is also included.

Outcome of Epidural Hematoma: Lessons from Solitary Fibrous Tumor/Hemangiopericytoma.

BACKGROUND: Intracranial solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is rare. In this report, a case of epidural hematoma (EDH) that eventually evolved into SFT/HPC is presented. We describe the possible association between the 2 diseases, which has not been previously reported. CASE DESCRIPTION: A 40-year-old man suffered from an EDH in the right parietal area 12 years ago and accepted conservative treatment. Follow-up computed tomography (CT) scan shows that the density of the right EDH gradually changed from uniform slightly lower density to mixed density. A new CT scan revealed an epidural mass extending to the subcutaneous with local bone destruction. An operation was performed via a large right parietal craniotomy, and the final diagnosis was World Health Organization grade III SFT/HPC after histopathologic examination and immunohistochemical verification. The patient died of deterioration of brain disease 3 months after the final diagnosis. CONCLUSIONS: To our knowledge, this is the first report that HPC occurred in the epidural cavity. We are the first time to describe the possible association between EDH and HPC.

Le Fort I Down-Fracture for Management of Midface Tumors.

OBJECTIVE: To perform an integrative review associating current literature with a clinical series regarding the use of Le Fort I osteotomy for the removal of tumors located in the midface and central region of the skull base. METHODS: A systematic review was performed through the PubMed, SCOPUS, and Cochrane databases. In addition, 4 different patients operated using the above-cited technique are described in this study. RESULTS: Initially, 123 articles were found. After the removal of duplicates, and title and abstract reading, 27 articles were selected for data extraction. The Le Fort I surgical approach of tumors was performed in 183 patients. CONCLUSION: The Le Fort I surgical approach allows lesion exeresis with good visualization, low rates of recurrences and complications, and without aesthetic compromises for the patient.

Protruding Meningioma of the Forehead With Extensive Hyperostosis Mimicking Skull Osteoma.

Meningioma is the most common intracranial benign tumor in adults. Hyperostosis accompanies about 4.5% of meningiomas. The authors report a rare case of hyperostotic meningioma that may have been misdiagnosed as giant osteoma.A 42-year male visited our clinic due to an egg-sized, hard mass on his left forehead. The mass suspected to be giant osteoma was about 4.2 × 4.0 cm sized, hard, non-movable, and non-tender. But based on radiologic findings, the mass was diagnosed as meningioma with extensive hyperostosis.Without neurologic symptoms, the diagnosis of meningioma associated with hyperostosis can be challenging and be misdiagnosed as fibrous dysplasia and osteoma by simple examination without enhanced CT and MRI.Therefore, although osseous lesions are strongly suspected to be osteomas, surgeons should consider other diagnoses, and if necessary, use contrast enhanced CT or MRI to differentiate these bony lesions.

Chondromyxoid fibroma of the temporal bone: A rare case report.

RATIONALE: Chondromyxoid fibroma (CMF) is a rare form of benign bone tumor and easily misdiagnosed as fibrosarcoma. Hence, to explore the clinical manifestations, diagnostic tests, and therapeutic procedures for temporal bone cartilage myxoid fibroma, it is important to optimize patient treatment and avoid overtreatment. Previous research has discussed cases of CMF, but this paper presents a systematic, complete, and comprehensive introduction of this disease based on this case and related literature. PATIENT CONCERNS: A 52-year-old male patient presented with pain in his right ear for 2 years and hearing loss in his right ear with tinnitus for 1 year. The patient had a history of hypertension for 9 years and it was well-controlled. DIAGNOSIS: A computed tomography (CT) scan of the temporal bone showed an expansive growth on the right temporal bone plate and tympanic plate, presenting as a cloud-like ground glass opaque shadow involving the temporom and ibular joint, middle skull base, and small auditory bones. A magnetic resonance imaging (MRI) of the temporal bone showed a large and irregular soft tissue mass shadow on the right temporal bone plate. The right temporal bone plate was occupied by the lesion, consistent with a bone origin. From the results of the imaging examination of the patient, a lesion occupying the temporal bone in the right ear and mastoiditis in the right middle ear was initially diagnosed. INTERVENTIONS: Right ear temporal bone tumor resection and abdominal fat extraction were conducted. OUTCOMES: Postoperative pathological results demonstrated myxoid fibroma of the temporal bone cartilage. No recurrence or severe complications were observed in 8 months of follow-up. LESSONS: A finding of myxoid fibroma of the temporal bone cartilage is rare in the clinic. The growth of such tumors is slow. The temporal bone CT and inner ear MRI were helpful in diagnosis. Surgery was the principal treatment.

Minimally Invasive Tubular Approach for Occipital Condylar Biopsy and Resection: Case Report and Review of Literature.

BACKGROUND: Patients with isolated occipital condyle lesions concerning for metastases rarely undergo surgical biopsies and are more commonly treated with empirical radiation with or without chemotherapy. This is likely related to the morbidity associated with open condylar approaches and the importance of surrounding structures. We present a minimally invasive technique to approach the occipital condyle using a tubular dilating retractor system. CASE DESCRIPTION: A 38-year-old woman with medical history of breast cancer presented with a 2-month history of headache localizing to the right occipital area and right tongue deviation. Magnetic resonance imaging revealed a heterogeneously enhancing mass within the right occipital condyle. The patient was brought into the operating room and placed in prone position. A 2-cm paramedian incision was made on the right approximately 2.5 cm off midline at the level of C1. Sequential dilation using a tubular retractor system was performed under fluoroscopic guidance. Once the condylar cortex was identified and entered, the tumor was immediately visualized and resected. Operative time was 65 minutes and estimated blood loss was 5 mL. The patient was discharged to home on postoperative day 1. CONCLUSIONS: This report, to our knowledge, presents the first case of a minimally invasive tubular retractor system-based approach for biopsy and resection of an occipital condylar metastasis causing occipital condyle syndrome. This approach allows for tissue diagnosis to precisely dictate medical management and minimizes the morbidity associated with traditional open surgical approaches.

Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia.

A possible cause of hypophosphatemia is paraneoplastic secretion of fibroblast growth factor 23 (FGF-23). Tumors secreting FGF-23 are rare, mostly of mesenchymal origin, usually benign, and may be located anywhere in the body, including hands and feet, which are often not represented in conventional imaging. A 50-year-old woman presented with diffuse musculoskeletal pain and several fractures. Secondary causes of osteoporosis were excluded. Laboratory analysis revealed hypophosphatemia and elevated alkaline phosphatase, parathyroid hormone, and FGF-23. Thus, oncogenic osteomalacia due to neoplastic FGF-23 secretion was suspected. FDG-PET-CT and DOTATATE-PET-CT imaging demonstrated no tumor. Cranial MRI revealed a tumorous mass in the left cellulae ethmoidales. The tumor was resected and histopathological examination showed a cell-rich tumor with round to ovoid nuclei, sparse cytoplasm, and sparse matrix, resembling an olfactory neuroblastoma. Immunohistochemical analysis first led to diagnosis of olfactory neuroblastoma, which was later revised to phosphaturic mesenchymal tumor. Following the resection, FGF-23 and phosphate levels normalized. In conclusion, we here describe a patient with an FGF-23-secreting phosphaturic mesenchymal tumor with an unusual morphology. Furthermore, we emphasize diagnostic pitfalls when dealing with FGF-23-induced hypophosphatemia.

Imaging Features of isolated hypoglossal nerve palsy.

The hypoglossal nerve gives motor innervation to the intrinsic and extrinsic muscles of the tongue. Pathology of this nerve affects the balanced action of the genioglossus muscle causing tongue deviation toward the weak side. Clinically, hypoglossal nerve palsy manifests with difficulty chewing, swallowing and with dysarthric speech herein, we review the anatomy of the hypoglossal nerve as well as common and infrequent lesions that can affect this nerve along its course.

Unilateral cochleovestibular nerve compression syndrome in a patient with bilateral IAC osteoma.

INTRODUCTION: Osteomas of the internal auditory canal are rarely reported in the literature. Patients may complain of disabling symptoms of dizziness, hearing loss, and vestibular dysfunction. We report the case of a patient with bilateral osteomas of the internal auditory canal (IAC) associated with bilateral neurovascular compression mainly affecting the right cochleovestibular nerve (VIII) and right anterior inferior cerebellar artery (AICA). OBSERVATION: This 75-year-old woman patient complained of disabling paroxysmal vertigo, typewriter tinnitus and hearing loss of the right ear. Temporal bone computed tomography showed bilateral osteoma arising from the posterior superior wall of the IAC. MRI sequences of the right VIII demonstrated compression by the right AICA against the inferior wall of the narrowed IAC. Treatment with oxcarbazepine allowed marked and lasting improvement of the patient's symptoms. CONCLUSION: To our knowledge, this is the first description of an ipsilateral neurovascular compression syndrome of the VIII secondary to the presence of an osteoma narrowing the IAC.

Primary Extracranial Meningioma of Mastoid in a Patient With History of Skin Squamous Cell Carcinoma, Lung Adenocarcinoma and Prostatic Carcinoma.

BACKGROUND: Meningiomas are the most common benign intracranial tumors and frequently develop in the parasagittal region, but can also present extracranially. Rarely, meningiomas may involve the middle ear and mastoid, resulting from contiguous spread of adjacent intracranial tumor, or extremely rarely, as an isolated primary tumor, which is frequently misdiagnosed and unrecognized, resulting in inappropriate clinical management. CASE REPORT: Herein we report such a case of an 80-year-old man with history of multiple cancer who presented with ear pain, vertigo and hearing loss. Audiometry demonstrated bilateral sensorineural hearing loss. Contrast-enhanced temporal bone computed tomography revealed a soft-tissue mass in the right epitympanum without bone erosion or any intracranial involvement. Radiological and operative findings were suspicious for cholesteatoma. Histological examination showed an epithelial neoplasm arranged in nests and whorls with intranuclear inclusions. No psammoma bodies, mitotic figures, or tumor necrosis were identified. The tumor cells were positive for epithelial membrane antigen, vimentin, progesterone receptor and CD56; and negative for synaptophysin, chromogranin, pancytokeratin (AE1/AE3), cytokeratin 7, prostate-specific antigen, inhibin, S100, P63, and P40. Ki67 highlighted about 2% of the tumor cells. Based on the morphological features and immunohistochemical profile, the tumor was diagnosed as primary extracranial meningioma of the mastoid, meningothelial subtype, World Health Organization grade 1. CONCLUSION: To the best of our knowledge, primary mastoid meningioma clinically mimicking a cholesteatoma presenting in a patient with a history of multiple primary carcinomas has not been previously reported. The pathogenesis, diagnosis and treatment of this entity are discussed.

A Case of Aggressive Recurrent Intracranial Subdural Hematoma Associated with Angiosarcoma Originating from the Skull.

BACKGROUND: Angiosarcoma often arises as a cutaneous disease in the scalp and the face; however, subdural hematoma (SDH) associated with angiosarcoma is extremely rare. CASE DESCRIPTION: A 72-year-old woman visited our hospital with gait disorder and progressive consciousness disturbance approximately 3 months after a minor head injury. Initially, on reviewing the results of imaging studies, she was diagnosed with traumatic chronic SDH. Despite repeated operations thereafter, including the embolization of the middle meningeal artery, her general condition progressively worsened, and computed tomography of head repeatedly showed the recurrence of SDH. Based on histopathologic and intraoperative findings, she was finally diagnosed with angiosarcoma originating from the skull. She died shortly thereafter because of aggressive recurrent intracranial SDH caused by leptomeningeal dissemination. CONCLUSIONS: In addition to cancers metastatic to the skull or dura mater, angiosarcoma should be included in the differential diagnosis for patients with repeated SDH and bone defect. An effective treatment for angiosarcoma with SDH that shows an unfavorable prognosis has not been established; however, an early diagnosis might be useful for a novel treatment.

[Acute visual loss related to sphenoid meningioma]. / Troubles visuels aigus associés aux méningiomes sphénoïdaux.

Meningiomas represent about 20% of intracranial tumors. Involvement of the medial sphenoid wing includes anterior clinoid, cavernous sinus and superior orbital fissure meningiomas. Due to the proximity of these tumors to the optic nerve, typically progressive unilateral vision loss, over several months to years, is the classic clinical presentation. We report three cases of acute monocular vision loss, two transient and one permanent, ipsilateral to a sphenoid meningioma. Ophthalmological involvement with sphenoid meningiomas is most often chronic, due to interruption of axoplasmic flow and demyelination of the optic nerve by local compression. However, vascular involvement with ischemia of the optic nerve or transient low blood flow secondary to compression of the carotid branches vascularizing these structures is another possible mechanism. In our series, two patients had amaurosis fugax, and one patient had sudden, persistent visual loss in relation to acute anterior ischemic optic neuropathy on the side of the meningioma. The mean age of patients with acute visual manifestations was 62 years. These ischemic and non-compressive visual symptoms, ipsilateral to sphenoid meningiomas, are difficult to interpret. Whether these temporary visual disturbances of vascular origin should be considered an early sign of future severe or permanent visual impairment when no optic nerve compression is observed is not certain. The place of these acute visual disturbances in the therapeutic decision, particularly surgical, remains to be defined. Larger multicentric prospective studies are needed to better understand the role of local circulatory factors attributable to meningioma in the occurrence of these acute visual signs.

Otopathology in Angiosarcoma of the Temporal Bone.

OBJECTIVES/HYPOTHESIS: Investigate the otopathology of angiosarcoma of the temporal bone, which has not been previously described in the literature. STUDY DESIGN: Postmortem evaluation and literature review. METHODS: Postmortem histological evaluation of the temporal bones and review of the literature for the treatment and prognosis of this rare disease were performed. RESULTS: A 50-year-old male with right chronic otitis media presented with progressive hearing loss, disequilibrium, otalgia, and acute facial paresis. Biopsy of the external auditory canal was unrevealing, but specimens from a canal wall down tympanomastoidectomy later showed high-grade angiosarcoma. Magnetic resonance imaging demonstrated an unresectable middle ear and mastoid mass extending superiorly into the temporal lobe. The patient received induction chemotherapy followed by proton beam radiation therapy and concurrent paclitaxel and bevacizumab. His course was complicated by a cerebrospinal fluid leak and cauda equina syndrome from leptomeningeal sarcomatosis. The patient died after developing meningitis and a temporal lobe abscess. Postmortem otopathology revealed persistent angiosarcoma in the internal auditory canal, although none was found in the middle ear or mastoid. There was inflammatory infiltrate throughout the mastoid, with direct extension of neutrophils and bacteria into the cochlea and through the tegmen into the middle cranial fossa. CONCLUSIONS: Angiosarcoma of the temporal bone can arise in the setting of chronic otitis media. In this case, postmortem temporal bone sections demonstrated viable cancer despite chemoradiation. Inflammatory infiltrates crossing from the middle ear/mastoid into the labyrinth and central nervous system illustrate pathways for the development of otogenic meningitis. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:737-742, 2019.

Metastatic craniocervical hemangiopericytoma presenting with hydrocephalus and papilledema - Case review.

Hemangiopericytomas are rare mesenchymal tumors with propensity to recur locally and metastasize. We report the unique case of a fifty-five-year-old male with recurrence of a previously resected craniocervical hemangiopericytoma presenting with obstructive hydrocephalus secondary to new metastatic cerebellar deposits. Emergent surgical resection of the cerebellar hemangiopericytomas was performed prior to adjuvant radiotherapy. Hemangiopericytomas are rare but important differentials for craniocervical junction lesions. Gross total resection remains the cornerstone of management with post-operative radiotherapy and chemotherapy as potential adjuncts. Tumors located in deep regions pose complex management challenges as safe maximal excision may be limited by proximal eloquent structures.