Rinosinusitis micótica alérgica con destrucción de base de cráneo / Allergic fungal rhinosinusitis with skull base destruction

La sinusitis micótica alérgica es una enfermedad inflamatoria de la mucosa rinosinusal producida por hongos que pueden aislarse de la cavidad de nasal de individuos sanos. Se produce indirectamente por los hongos que actúan como antígeno y desencadenan una reacción inmunológica mediada por IgE que origina pólipos y una secreción mucosa espesa con detritus e hifas denominada mucina. Su presentación clínica más frecuente es una sinusitis crónica unilateral o bilateral con pólipos. Con menos frecuencia, las sustancias originadas por la desgranulación de los eosinófilos producen remodelación o destrucción ósea y la sinusitis puede simular una neoplasia. Se describe el caso clínico de un paciente que padeció una sinusitis micótica alérgica con destrucción ósea masiva de la base del cráneo y que tuvo extensión intracraneal extradural e intraorbitaria de la enfermedad. Fue tratado con éxito mediante cirugía y corticoides. (AU)

Allergic fungal sinusitis is an inflammatory disease of the rhinosinusal mucosa caused by fungi that can be isolated from the nasal cavity of healthy individuals. The pathology is produced indirectly by the fungus that acts as an antigen and triggers an IgE-mediated allergic reaction that causes polyps and a thick mucous discharge with detritus and hyphae called mucin. Its most common clinical presentation is unilateral or bilateral chronic sinusitis with polyps. Less commonly, substances originated by the degranulation of eosinophils cause bone remodeling or destruction, and sinusitis can simulate a neoplasia. We describe the clinical case of a patient who suffered from allergic fungal sinusitis with massive bone destruction of the skull base and who had intracranial, extradural and intraorbital extension of the disease. He was successfully treated with surgery and corticosteroids.Key words: allergic fungal sinusitis, intracranial extension, endoscopic surgery, transorbital transpalpebral approach. (AU)

An olfactory neuroblastoma with intracranial extension treated with chemotherapy and radiotherapy: A case report

Background@#Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a rare malignant tumor that presents as a locally aggressive disease. It accounts for an incidence of 0.4 per million population affecting men and women equally in all ages. As olfactory neuroblastoma is rare, having an intracranial extension is even more unusual. Only a few cases have been reported in literature, hence, there is no widely accepted standard of care. @*Case Presentation@#This is a case of a 33-year-old female who presented with a 4-month history of nasal congestion which progressed to a rapidly growing nasal mass and bilateral proptosis. She soon became bedridden and exhibited signs of increased intracranial pressure. Imaging revealed a heterogeneously enhancing nasal cavity mass with intracranial extension. Biopsy and immunohistochemistry confirmed the olfactory neuroblastoma. She subsequently underwent chemotherapy and radiotherapy since the tumor was unresectable. In a span of three months, after only three cycles of chemotherapy with cisplatin and etoposide and thirty fractions of radiation therapy, the patient significantly improved from being completely bedridden to an ambulatory individual capable of self-care. We completed eight cycles of chemotherapy and referred to surgical specialists for possible resectability. However, the patient did not consent for surgery and opted to have palliative care.@*Conclusion@#Most cases of olfactory neuroblastoma are treated through combination therapy. The patient significantly improved from the administration of chemotherapy with cisplatin and etoposide plus radiotherapy. This case report shows the significance of chemotherapy with radiotherapy as the treatment of choice for late-stage olfactory neuroblastoma in which surgery is not amenable.

Rhinoscleroma Revisited

ABSTRACT Rhinoscleroma (more appropriately 'scleroma') is a chronic, disfiguring inflammatory lesion that is rarely encountered in the present-day otolaryngology practice. The diagnosis often becomes difficult especially when it recurs and presents late with complications. This report illustrates the clinical and histologic features of rhinoscleroma in a defaulter patient who represents advanced-stage disease with orbital and intracranial extensions. Special emphasis has been provided on the computed tomography impressions such that the evolution of scleroma with time can be studied.

Two Cases of Cutaneous Squamous Cell Carcinoma with Intracranial Extension / 대한피부과학회지

Cutaneous squamous cell carcinoma (SCC) is the second most common skin malignant neoplasm. Cutaneous SCC shows a broad spectrum, ranging from easily managed superficial tumors to highly infiltrative, metastasizing ones that can cause death. We have experienced two patients with SCC with intracranial extension. One case was an 88-year-old man with a tumor on the forehead treated with Mohs micrographic surgery who presented with local recurrence at the perilesional region of the primary site after 3 years. Wide excision was performed, and histologic findings showed a SCC that extended to the dura mater. The other case was a 69-year-old woman who presented with an erythematous 2x4 cm-sized plaque on the right temple, and a biopsy examination revealed SCC. The patient refused surgery and 7 months later, presented with a 5x10 cm-sized oozing plaque with multiple ulcers. Radiologic evaluation demonstrated intracranial invasion and right retropharyngeal metastatic lymph nodes. She was treated with radiotherapy for 4 months.

Clinical Presentation and Management of Jugular Foramen Paraganglioma

OBJECTIVES: Jugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symptoms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal management of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma. METHODS: Retrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treatment outcomes and complications. RESULTS: Most common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radiosurgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications. CONCLUSION: Neurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiography with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can maintain the compliance of the patients.

Esthesioneuroblastoma with Intracranial Extension: Case Report

We report a case of esthesioneuroblastoma with intracranial extension treated with surgical resection and chemotherapy. A 5-year-old child presented with visual disturbance, and was subsequently found to have a huge sized mass in the anterior skull base, suprasellar region and lateral ventricle. A bifrontal craniotomy with excision of the tumor through subfrontal and interhemispheric approach was performed. The patient received postoperative chemotherapy according to Tandem protocol. A follow-up magnetic resonance(MR) image showed that the tumor showed partial regression, however, there was no change in tumor size in lateral ventricle. Second operation with excision of remnant mass through a transcallosal approach was performed. MR image, 9 months after first operation showed the postoperative change in frontal area without enhancing mass. By employing staged operation and chemotherapy, the authors obtained good results in the treatment of esthesioneuroblastoma with intracranial extension.

Orbital Cavernous Lymphangioma with Intracranial Extension: Case Report

Lymphangioma is a rare benign developmental vascular tumor that may be found in orbit, skull and elsewhere in head and neck. Few cases of extension of this benign but insidious tumor posteriorly out of the bony orbital cavity have been reported. The patient was 40-year-old man complaining of proptosis of right eye for one month. Physical examination revealed severe right exophthalmus, impairment of eyeball movement in all directions. Visual acuity was much impaired and he could percept only light with right eye. CT and MRI scans showed intraconal and extraconal involvement of ill-defined, heterogenous mass with extension of the tumor posteriorly beyond the orbital cavity involving right frontal and temporal lobe, skull and subcutaneous tissue. The tumor was subtotally removed via orbito-frontal approach without damaging vital neural and orbital component. Then, orbital roof reconstruction and cranioplasty were done with resin. Successful surgical removal of lymphangioma is very difficult due to its severe infiltration to surrounding tissue and tendency to bleed during debulking. We report a rare case of orbital cavernous lymphangioma with intracranial extension treated with surgical decompression, with review of literatures.

Intracranial Extension of Adenoid Cystic Carcinoma of the Palate / 대한방사선종양학회지

Intracranial involvement by adenoid cystic carcinoma (ACC) is very rare and there is no report of intracranial extension from the palate ACC in Korea. Intracranial involvement can occur in one of three ways : direct extension, perineural spread, and hematogenous spread. A case report of a 35-year-old woman with intracranial ACC is presented. Initially she had ACC of the right palate and was treated by surgery and postoperative radiation therapy. Three years and 10 months later, the paresthesia in the distribution of ophthalmic and maxillary branch of right trigeminal nerve developed without evidence of recurrence in CT scan. Ptos and total ophthalmoplegia developed sequentially and the second operation was performed. It was suggested that the tumor was spread perineurally along the trigeminal nerve into the Gasserian ganglion and then cavernous sinus and orbit. Seven years and 6 months after the first operation, direct intracranial extension into the right temporal lobe developed via sphenoid bone, sphenoid sinus and temporal bone and the third operation was done. And then lung metastasis was diagnosed. She is alive for 9 years 5 months after first operation.

A Case of Fibrous Dysplasia of the Ethmoid Bone Forming Intracranial Cyst / 대한이비인후과학회지

Fibrous dysplasia is a benign bone lesion characterized by the replacement of the normal substances of the interior of the bone by fibroosseous connective tissue histologically exhibiting varying degrees of osseous metaplasia. Craniofacial dysplasia represents approximately 2.5% of all bone tumors and 7.5% of benign bone tumors. Craniofacial dysplasia frequently affects the maxilla and frontal bone and induces local facial swelling and exophthalmos. The dysplasia has predilection for membranous bone such as femur or tibia, so its origin in the ethmoid is very rare. Recently, we experienced a case of fibrous dysplasia originating in the ethmoid bone extending to intracranial region. Our case demonstrated a huge bony lesion involving right ethmoid, frontal and sphenoid bone and extending to the left frontal area extensively forming a large dumbbell shaped cyst with thin calcified wall. There are no reported cases of fibrous dysplasia forming huge cyst in the intracranial region internationally. Hence, we report a case of fibrous dysplasia of ethmoid sinus forming the intracranial huge cyst.

A Case of Huge Cholesterol Granuloma Cyst in Temporal Bone with Intracranial Extension / 대한이비인후과학회지

Cholesterol granuloma is thought to arise as the consequence of obstruction of the air cell system. Once the ventilation is blocked, negative pressure builds with mucosal hemorrhage and blood extravasation into the air cell system. And the catabolism of blood releases cholesterol, hemosiderin, lipids and fibrin. It is the foreign body reaction of these products that leads to cholesterol granuloma and cholesterol granuloma cyst formation. The cholesterol granuloma and cholesterol granuloma cyst may develop in any portion of the pneumatic system of the temporal bone and small lesions are commonly encountered in the middle ear and mastoid air cells. But, huge cholesterol granuloma cyst is a unusual lesion and cholesterol granuloma cyst with extensive temporal bone destruction and intracranial extension has been rarely reported. Recently we experienced a case of huge cholesterol granuloma cyst with intracranial extension in a 38-year-old female, and report this case with review of literatures.

Malignant Fibrous Histiocytoma of the Pericranium with Intracranial Extension: A Case Report

A case of malignant fibrous histiocytoma(MFH) arising from the pericranium which extend to the calvaria and the dura mater in a 32-year-old man is reported. This tumor presented as scalp induration and headache without any other neurologic abnormalities. Radical excision presented a surgical challenge because of the extensiveness of the lesion. In this case radical excision of pericranial and calvarial lesions were possible, however it is impossible to remove the lesion in the dura mater radically because of its extensiveness. Thus the radiation therapy was necessary after operation. Histologically, pericranial, calvarial and dura mater tumor tissues were same as that of the typical malignant fibrous histiocytoma of the soft tissue. The authors review the literatures of the MFH involving central nerve systems and discuss the management and the radiologic findings of this rare tumor.

Sphenoid Sinus Mucocele with Intracranial Extension: Case Report

A case of mucocele of the sphenoid sinus with blurred vision and headache is presented. Mucocele of the sphenoid sinus is relatively rare and benign. Accurate diagnosis is possible through Brain CT scan and MRI. But it should be included in the differential diagnosis of malignancy or pituitary tumor, and unnecessary treatment such as radiotherapy or craniotomy should not be performed. In this case, mucocele is extended into sella turcica which is markedly destroyed and eroded. We removed it by sublabial transseptal sphenoidal sinusotomy and drainage without difficulties and postoperative complications.

Diagnosed and Treatment of Sphenoid Sinus Mucoceles with Intracranial Extension

Sphenoid sinus mucocele with the intracranial extension is a rare disease but should be included in the differential diagnosis of destructive changes of the sella turcica, since a seemingly harmful intracranial procedure may be attempted. With the use of CT scan and MRI, it can be diagnosed preoperatively with a much better accuracy. Three histologically-verified cases of sphenoid mucocele with intracranial extension developing headache, visual disturbance and/or amenorrhea are described with a review of the literatures. All cases were successfully operated upon by the intranasal procedure.