Cases reported "Skull Base Neoplasms"

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1/10. Primary orbito-cranial adenoid cystic carcinoma with torcular metastasis: a case report and review of the literature.

    Adenoid cystic carcinoma is a rare neoplasm arising from the exocrine glands such as salivary glands, lachrymal glands, upper respiratory tract, breast or uterine cervix. Intracranial involvement is commonly from direct skull base involvement, although metastasis may rarely be seen. The predisposition of the adenoid cystic carcinoma for perineural and perivascular invasion is the prime reason for the locally invasive character of the tumour, often extending into the cranium via foramina at the skull base. The authors report a case of primary orbito-cranial extradural adenoid cystic carcinoma and cranial metastasis away from the primary site. This patient initially had a local excision, and later an exenteration of the right eye followed by with radiotherapy. Within months she presented with an extradural cranial recurrence, distant torcular metastasis without any neurological deficit. craniotomy and radical excision was undertaken as these tumours have slow growth rates, and long term survival of the se patients even in the presence of local recurrence and metastasis has been well documented.
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2/10. Hepatocellular carcinoma with metastasis to the skull base, pituitary gland, sphenoid sinus, and cavernous sinus.

    Two cases of hepatocellular carcinoma, with metastases to the skull base, pituitary gland, sphenoid sinus, and cavernous sinus are reported. patients presented with diplopia, retro-orbital headache, and multiple cranial nerves palsies. Pituitary metastases may require surgery as palliative treatment, and for the confirmation of histology. One of the current cases was diagnosed with hepatocellular carcinoma prior to transphenoidal resection of the pituitary metastasis. The second patient was found to have hepatocellular carcinoma after review of histology, and the development of signs and symptoms relating to the primary tumour.
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3/10. chordoma in the sella turcica.

    A 75-year-old man presented with a rare case of chordoma in the sella turcica of the skull base. He had been treated for hypertension and chronic renal failure since 1990. Computed tomography detected a tumor in the sella turcica in 1994, but the patient had no clinical complaints and the serum pituitary hormone levels were normal. He died of disseminated intravascular coagulation, myocardial infarction, pulmonary infection, and multiple cerebral infarctions in 2000. At autopsy, the tumor in the sella turcica was 3.1 cm in greatest diameter and had compressed the pituitary gland posteriorly. Histological examination found oval cells and vacuolated short spindle-shaped cells which showed morphological changes similar to myxoma cells. The tumor was lobulated by narrow connective tissues. The tumor did not contain any cartilaginous tissue components, and was stained positively for epithelial membrane antigen but negatively for S-100 protein. The final diagnosis was chordoma. There was no association between the tumor and the cause of death.
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4/10. Regional spread of nonneurogenic tumors to the skull base via the facial nerve.

    OBJECTIVE: This study examined the clinical and pathologic features of regional spread of nonneurogenic neoplastic disease to the intratemporal segments of the facial nerve. STUDY DESIGN: Retrospective review.SETTING Three tertiary referral centers. patients: Six patients with neoplastic disease of nonneurogenic origin involving segments of the facial nerve within the temporal bone. INTERVENTIONS: All patients underwent surgery with curative intent. Five patients received adjuvant radiation, and two received adjuvant radiation and chemotherapy. MAIN OUTCOME MEASURES: Histopathology, site of primary tumor, intratemporal location of regional spread along the facial nerve, degree of facial paralysis, and presence of residual disease. RESULTS: Five cases of malignancy were reported: one case of parotid adenoid cystic carcinoma, one case of parotid mucoepidermoid carcinoma, two cases of squamous cell carcinoma of the skin, and one case of an unidentified carcinoma. Perineural spread was histologically found in all cases of malignant disease. In addition, one case of benign pleomorphic adenoma of the parotid gland that circumferentially involved an intratemporal segment of the facial nerve was reported. facial paralysis was present in five of six (83%) of cases. Four patients had unresectable malignant disease, and two died despite multimodality therapy. CONCLUSIONS: The facial nerve provides a route for the spread of neoplastic disease into the temporal bone, and perineural invasion is an important mechanism of invasion and motility of malignant disease. Nonneurogenic intratemporal tumors of the facial nerve are a rare but significant cause of facial paralysis.
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5/10. sella turcica metastasis from follicular carcinoma of thyroid.

    A case of metastasis to the sella turcica from a follicular adenocarcinoma of the thyroid gland is presented. Metastasis to this site is rare and review of the literature reveals only 12 cases of metastatic thyroid carcinoma involving the sella turcica and pituitary gland. The optimal treatment strategy is still to be determined. A 43-year-old woman presented with headache, nausea, visual impairment and galactorrhea. An MRI scan of the cranium revealed an enhancing destructive sellar lesion. The patient underwent transsphenoidal removal of the lesion to alleviate visual loss. The histological features of the sellar tumor were identical to those of a follicular adenocarcinoma partially removed from the thyroid gland 22 months earlier. Total thyroidectomy followed by three courses of iodine-131 ablation enhanced with synthetic thyrotropin and thyroid hormone suppression therapy was instituted. The post-operative course was satisfactory with improved vision and ceased galactorrhea. This case was successfully treated with a combination of surgical removal, iodine-131 ablation and hormone suppression therapy, which resulted in disease control duration of four years. sella turcica metastases of thyroid carcinoma are exceedingly rare and currently there are no established therapeutic guidelines.
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6/10. Stereotactic radiosurgery for recurrent pleomorphic adenoma invading the skull base--case report--.

    A 38-year-old man presented with a recurrent pleomorphic adenoma in the parapharyngeal space invading the skull base 19 years after the first operation for a parotid gland tumor. Stereotactic radiotherapy was performed to control the tumor growth using a marginal dose of 8 Gy and maximum dose of 18 Gy with care taken to minimize the dose to nearby structures. The symptoms were reduced within a few months. magnetic resonance imaging over 5 years showed that the tumor was controlled with no regrowth. Stereotactic radiotherapy is a therapeutic option for the treatment of pleomorphic adenomas.
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7/10. Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement.

    Rosai-Dorfman disease is associated with a histiocytic infiltration of lymphoid tissue, but may also involve the orbit, nasopharynx, respiratory pathways, gastrointestinal tract, endocrine glands (particularly the thyroid), bone, and skin. neurologic manifestations are rare, occurring in 4% of one series, and the diagnosis is often not made until pathology is available. Using data from two cases, we present the clinical manifestations, pathology, and treatment, and review the literature regarding the ocular and neurologic manifestations. Finally, we discuss the optimum management of this disorder.
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8/10. Pituitary prolactinoma mimicking tumor originating from the sphenoid sinus or clivus.

    We report a case of a pituitary prolactinoma mimicking a sphenoid sinus or clival tumor. On magnetic resonance (MR) images, a soft tissue mass located inferior to the apparently normal pituitary gland, and occupied the posterior third of the sphenoid sinus and eroded the upper portion of the clivus. It had iso- to low intensity on the T1-weighted image, and slightly high intensity on the T2-weighted image. Careful observation revealed that the floor of the sella turcica was not identified and there was sulcus formation between the tumor and clivus, probably suggesting extraclival tumor, pituitary tumor. Transsphenoidal tumor resection and histopathological study revealed that it was a prolactinoma.
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9/10. Salivary gland-like tumors of the sellar region.

    Herein we present a group of rare tumors of the sella region that have not been previously recognized. Although clinically and radiographically the tumors resemble nonfunctioning pituitary adenomas, their histologic, immunohistochemical, and ultrastructural features differ and indicate a salivary gland origin. The lesions cover a morphologic spectrum that includes cellular pleomorphic adenoma, monomorphic adenoma, oncocytoma, and low-grade adenocarcinoma of the salivary gland. All tumors except the oncocytoma were immunoreactive for cytokeratin and were negative for pituitary hormones and synaptophysin. Ultrastructural characteristics in the cases examined include hypodense stromal material, basal lamina, and tonofilament bundles. The single oncocytoma was packed with mitochondria and lacked membrane-bound secretory granules. dna ploidy based on image analysis and MIB-1 labeling indices showed diversity within this group of tumors, with labeling indices ranging from 0.06% to 15%. The presumed origin of these rare neoplasms is from salivary gland rests related to the normal pituitary gland. Despite their varied morphology, such tumors are easily confused with pituitary adenoma. Although rare, tumors of salivary gland origin should be considered in the differential diagnosis of unusual adenohypophyseal tumors.
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10/10. Dumbbell tumor of the anterior skull base. meningioma? No, adenoid cystic carcinoma!

    BACKGROUND: Adenoid cystic carcinoma (ACC) is a rare neoplasm of the exocrine glands. Because of its tendency for skull base involvement and intracranial spread, ACC should be diagnosed promptly by the neurosurgeon, although discrimination from meningioma is often difficult. Radical resection of such tumors requires familiarity with complex craniofacial approaches. CASE DESCRIPTION: An unusual case of dumbbell-shaped ACC centered over the planum sphenoidale is presented. Regular margins and neuroimaging features suggested a preoperative diagnosis of meningioma. The lesion was gross, totally resected in a two-staged procedure through frontobasal and transfacial approaches, with good functional and aesthetic result. CONCLUSION: The epidemiologic, histologic, and clinical features of ACC are reviewed. ACC is rarely encountered by the neurosurgeon; however it should always be considered in the differential diagnosis of skull base tumors. Interdisciplinary surgical approaches represent the major advance in the treatment of these complex neoplasms.
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