Cases reported "Skull Neoplasms"

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1/85. A de novo discharging sinus of the fronto-orbital suture: a rare presentation of a dermoid cyst.

    Dermoid cysts are developmental anomalies, resulting primarily from trapped germinal epithelium. In the neck and head region, the most common location is the fronto-orbital upper outer quadrant of the orbit. Because only a few cases of dermoid cysts with discharging sinus of the fronto-orbital area have been reported in the literature, the authors present an unusual case of a frontozygomatic suture dermoid cyst, presenting as a sinus, in an 56-year-old man. The histologic report confirmed that the cystic lesion was a dermoid cyst with a tract. diagnosis and management are discussed.
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2/85. Giant-cell tumor of the skull base.

    giant cell tumors are uncommon primary bone tumors. They primarily occur in the long bones. giant cell tumors are extremely rare in the skull and head and neck. When it does occur, the maxilla and mandible are the common sites to be involved. We described two cases of giant cell tumor in the temporal bone. In the noncontrast enhanced CT, the lesion presents as a soft tissue density mass with expansion of the bone. The bony cortex is usually intact. The adjacent soft tissues and cerebral parenchyma show no infiltration or edema. The post contrast scan reveals homogenous enhancement of the mass.
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3/85. head and neck infantile myofibromatosis--a report of three cases.

    Infantile myofibromatosis (IM) is a proliferative disorder characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular, bone or visceral structures. This proliferation may occur at any anatomical site, but in 30% of the cases it involves the head and neck. We report here three cases of head and neck IM occurring in young children and presenting as solitary lesions. The clinical heterogeneity and the misleading histopathological appearances can make the diagnosis difficult. The treatment is surgical but the low rate of recurrence and the possibility of spontaneous tumoral regression may lead to conservative surgery or therapeutic abstention.
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4/85. Aggressive basal cell carcinoma of the temporal region in a patient with Gorlin-Goltz syndrome.

    Gorlin-Goltz syndrome is an autosomal dominant disorder with variable penetration characterized primarily by five major findings: multiple basal cell carcinomas presenting at a young age, pits on the palms and soles, skeletal abnormalities, jaw cysts, and ectopic calcification of the falx cerebri and other structures. When the basal cell carcinomas are located in the head and neck there is a high risk of invasion of deep structures if early and radical treatment is not performed. The authors present a 59-year-old man affected by basal cell carcinoma in the context of Gorlin-Goltz syndrome. Although patients with this syndrome can present aggressive basal cell carcinomas, it is unusual to find them involving the craniofacial bones. In this patient the basal cell carcinoma involved the middle ear, the intrapetrous aspect of the facial nerve, and the dura mater. The reconstruction of a wide three-dimensional defect, in which the brain was exposed, was achieved with local flaps and a free musculocutaneous rectus abdominis flap. Factors affecting reconstruction in the lateral cranial base are discussed.
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5/85. Langerhans' cell histiocytosis: report of a case with temporal localization.

    The authors report a case of Langerhans' cell histiocytosis (LCH) with temporal localization and rapidly evolving initial clinical presentation in a 12-year-old boy. This disease of currently unknown etiology is actually considered a proliferative entity of cells with phenotypic characteristics of normal Langerhans' cells. An immunoregulation defect leading to abnormal maturation and migration of Langerhans' cells might be the basis for LCH. According to the Hystiocyte Society diagnostic criteria, ATPase, S 100 and D-mannoxidase positivity in addition to typical hystopathologic findings are sufficient for diagnosis of LCH. head and neck localization of LCH occurs in about 70% of cases; males are more frequently affected than females, age at presentation varies from a few months to 15 years. Presenting features, initial diagnostic evaluation, differential diagnosis and treatment protocol of a unifocal monosystemic temporal bone localization of LCH are presented.
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6/85. Squamous cell carcinoma of the temporal bone: diagnosis, treatment and prognosis.

    Squamous cell carcinoma of the external ear canal is an uncommon condition that is associated with a poor outcome. The development of an accepted staging system has not been forthcoming and this has inhibited the formation of an evidence-based therapeutic protocol. We report the findings in 14 patients with squamous cell carcinoma of the external ear canal treated in our institutions. The most common presenting symptoms were otorrhoea and otalgia. Four patients had a history of chronic ear discharge and one had previous radiotherapy for nasopharyngeal carcinoma. Five patients had facial palsy which was a poor prognostic sign. Only one patient had clinical neck disease. Pre-operative imaging with CT or MRI scans was accurate in determining the extent of tumour involvement. The initial T-staging relied heavily on these findings. With combination treatment involving surgery, radiotherapy and chemotherapy, disease free survival achieved was 69% (9 of 13) over a mean follow-up period of 24.7 months. One patient absconded treatment. patients with early stage tumours faired better than patients with advanced tumours (100% vs 33%). There was low incidence of involvement of the parotid gland (1 of 7 patients). patients with facial nerve involvement had a significantly poorer outcome (p = 0.035).
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7/85. Inflammatory myofibroblastic tumor involving the pterygopalatine fossa.

    SUMMARY: Inflammatory myofibroblastic tumors (IMT) comprise a rare group of lesions characterized histologically by acute and chronic inflammatory cells with a variable degree of fibrous stroma. Occurrence in the extracranial head and neck in children is unusual, and involvement in the pterygopalatine fossa has not, to our knowledge, been reported as occurring in this age group. We present the CT findings of an IMT of the pterygopalatine fossa in a 6-year-old female patient with a 2-week history of fever and a painless swelling of the left cheek. The diagnosis of IMT should be included in the differential diagnosis of a child presenting with an aggressive mass associated with systemic features such as fever, elevated sedimentation rate, and leukocytosis.
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8/85. Drop metastasis from sinonasal undifferentiated carcinoma: clinical implications.

    STUDY DESIGN: The first reported case of multiple intradural, extramedullary spinal metastasis from sinonasal undifferentiated carcinoma is presented. OBJECTIVES: To elucidate the mechanisms by which metastatic disease invades the spinal axis, and to discuss the possibility of spinal drop metastasis from head and neck tumors that invade the dura. SUMMARY OF BACKGROUND DATA: Sinonasal undifferentiated carcinoma is a rare yet aggressive neoplasm of the upper airways and anterior skull base. This neoplasm is known to invade the cranial vault and brain locally. However, it has not previously been reported to seed the cerebrospinal fluid or result in drop metastasis. Such drop metastasis may result in significant neurologic deficit if not diagnosed and treated in a timely manner. methods: This report is based on a single patient treated by a multidisciplinary team from the departments of neurosurgery, otolaryngology, and radiation oncology at the University of Southern california School of medicine. RESULTS: This patient initially underwent resection and local radiation therapy for sinonasal undifferentiated carcinoma of the anterior skull base. At the time of surgery, the tumor was noted to violate the dura and arachnoid along the subfrontal plane. At 11/2 years after the initial treatment, a bandlike distribution developed at T2 as well as paresthesias and numbness below that level. Imaging of the spine showed an intradural, extramedullary tumor at T2 consistent with a schwannoma or meningioma. The patient underwent a laminectomy and tumor resection, which showed poorly differentiated sinonasal carcinoma. Local radiation therapy was administered, and the patient experienced complete recovery of neurologic function. Bilateral leg pain and weakness developed 14 months later. magnetic resonance imaging of the spine showed a new intradural, extramedullary lesion at T12, remote from the first lesion. This second metastasis was managed with surgical resection and adjuvant radiation therapy. CONCLUSIONS: This is the first reported case of a sinonasal carcinoma leading to intradural extramedullary metastasis. The primary tumor likely seeded the cerebrospinal fluid, thus resulting in drop metastasis. patients with sinonasal undifferentiated carcinoma that invades the dura should be monitored closely for evidence of metastasis before symptoms develop.
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9/85. Cranial infantile myofibromatosis: report of three cases.

    BACKGROUND: Infantile myofibromatosis is a proliferative disorder of infancy and early childhood characterized by the development of single or multiple nodular lesions arising from cutaneous or subcutaneous tissue, muscle, bone or visceral organs. In approximately one-third of cases, this myofibroblastic proliferation involves the head and neck region. CASE REPORT: In this paper we report on three cases of cranial infantile myofibromatosis in infants. The clinical presentation and the deceptive histopathological features can make diagnosis difficult. CONCLUSION: The significance of recognizing this entity is stressed, since its indolent clinical behavior might prevent diagnosis.
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10/85. Symptomatic cavernous hemangioma of the occipital condyle treated with methacrylate embolization.

    BACKGROUND: Cavernous hemangiomas of the cranial base are rare tumors. No case of symptomatic intraosseous angioma affecting the occipital condyle has been reported. This particular case was treated with surgical embolization using acrylic resin.CASE DESCRIPTION: A 20-year-old man with a 1-year history of neck pain and torticollis was referred to our hospital. Neuroradiological examination revealed the typical picture of an intraosseous cavernous hemangioma located in the right occipital condyle. The patient was operated through a suboccipital approach. biopsy and direct embolization with methacrylate was performed. The definitive pathological diagnosis confirms the neuro-radiological suspicion of intraosseous cavernous hemangioma. The follow-up of the patient (4 years) revealed no recurrence of pain or abnormal posture.CONCLUSIONS: A rare case of cranial base cavernous hemangioma is reported. Methacrylate embolization can be a good option for the treatment of this uncommon lesion.
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