Cases reported "Neoplasm Invasiveness"

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1/256. Pediatric eighth cranial nerve schwannoma without evidence of neurofibromatosis.

    Schwannomas of the eighth cranial nerve are rare in children. We report a 4 10/12 - year-old girl with no evidence of neurofibromatosis who presented with facial droop. Radiographic studies revealed a large cerebellopontine angle tumor. At surgery, the tumor was attached to the eighth cranial nerve and histologically was a schwannoma. This is the youngest reported case of unilateral eighth cranial nerve schwannoma in a patient without the stigmata of neurofibromatosis.
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2/256. Immature teratoma originating from the pituitary gland: case report.

    OBJECTIVE AND IMPORTANCE: Recently, it has been suggested that the primary site of suprasellar germinomas is the neurohypophysis, but nongerminomatous germ cell tumors originating from the pituitary gland have been rarely reported. CLINICAL PRESENTATION: A 27-year-old man presented with panhypopituitarism, bitemporal hemianopsia, and mild right oculomotor nerve paralysis. diabetes insipidus was not observed. The tumor was shown by magnetic resonance imaging to extend and invade bilateral cavernous sinuses, sellar and clival dura mater, and the sphenoid sinus mucosa. INTERVENTION: Transsphenoidal removal yielded a diagnosis of immature teratoma. serum alpha-fetoprotein was prominently elevated. Magnetic resonance imaging and surgical findings of the superiorly displaced residual pituitary gland strongly suggest the pituitary origin of this rare tumor. CONCLUSION: In contrast to the neurohypophyseal germinomas, nongerminomatous malignant germ cell tumors originating from the pituitary gland tend not to be associated with diabetes insipidus and thus mimic adenomas. Evaluation of the tumor markers is necessary in young patients with cavernous sinus syndrome and invasive pituitary tumors with heterogeneous intensity revealed by magnetic resonance imaging.
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3/256. Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis.

    OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent.
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4/256. Progression of a Nelson's adenoma to pituitary carcinoma; a case report and review of the literature.

    A 25-year-old woman developed Nelson's syndrome, 3 years after successful bilateral adrenalectomy for Cushing's disease. Despite pituitary surgery and radiotherapy the tumour showed invasive growth, leading to visual disturbance, paresis of the oculomotor nerve and, 34 years after adrenalectomy, to death by widespread purulent leptomeningitis. autopsy revealed a large adenohypophyseal carcinoma with a metastasis attached to the dura, both tumours showing immunocytochemical staining for ACTH and TSH. We review the literature on metastatic adenohypophyseal carcinoma in Cushing's disease and Nelson's syndrome and discuss the role of proliferation markers as indicators of malignant progression.
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5/256. Extraocular extension of unrecognized choroidal melanoma simulating a primary optic nerve tumor: report of two cases.

    BACKGROUND: Orbital extraocular extension of choroidal melanoma is well known and is usually detected in eyes with medium and large tumors, but it is very rare with small melanomas. It is particularly unusual for choroidal melanomas of any size to invade the optic nerve or its meninges. DESIGN: Two case reports. PARTICIPANTS: Two patients with small, relatively inconspicuous juxtapapillary pigmented choroidal lesions were referred with the diagnosis of primary optic nerve tumor. Both demonstrated a large nodular tumor in the meninges of the optic nerve, immediately posterior to the globe. methods: Retrospective review of clinical records and histopathology. RESULTS: In both cases, orbital magnetic resonance imaging confirmed the presence of a hyperintense enhancing nodular mass near the anterior portion of the optic nerve, prompting optic nerve biopsy in one case. Subsequent fundus examination disclosed a small juxtapapillary pigmented choroidal lesion measuring 1.0 mm or less in thickness. These observations suggested that the optic nerve tumor might be nodular extraocular extension of a small choroidal melanoma. Modified enucleation was performed in both cases, and histopathologic examination revealed a nodule of malignant melanoma within the meninges that compressed the optic nerve and extended extraocularly from a small, relatively inapparent juxtapapillary choroidal melanoma. In both cases, the extraocular component was large and symptomatic, whereas the intraocular component was inconspicuous. CONCLUSIONS: Small juxtapapillary choroidal melanomas can exhibit prominent extension into the orbit. All patients with orbital tumors should have careful ophthalmoscopy.
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6/256. Invasion of the recurrent laryngeal nerve by adenoid cystic carcinoma. An unusual cause of true vocal fold paralysis.

    True vocal fold paralysis and goitre are both common problems encountered in ENT practice. Their co-existence, however, should arouse suspicion of the presence of malignant thyroid disease. A rare case of true vocal fold paralysis caused by a clinically occult subglottic adenoid cystic carcinoma, in a 72-year-old, is described. The existence of multinodular goitre in this patient was co-incidental and confounded the diagnostic process.
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7/256. Malignant peripheral nerve sheath tumour in the maxilla associated with von Recklinghausen's disease.

    Malignant transformation of neurofibromatosis is one of the most serious complications of von Recklinghausen's disease (VRD). The most common associated malignancy is the malignant peripheral nerve sheath tumour (MPNST). Few cases of MPNST associated with VRD in the maxillary region have been reported. This report describes a rare case of MPNST in the maxilla and the aggressive nature of MPNST associated with VRD.
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8/256. Bilateral endolymphatic sac papillary carcinoma.

    We report a case of bilateral and almost symmetrical endolymphatic sac papillary adenocarcinoma. A 22-year-old male patient presented with bilateral sixth, seventh, eighth and lower cranial nerve paresis and ataxia. Radiological investigations revealed extensively vascular tumours in the region of both jugular bulbs. The literature on this rare entity is briefly discussed.
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9/256. Pyloric gland metaplasia with perineural invasion of the gallbladder: A lesion that can be confused with adenocarcinoma.

    BACKGROUND: Metaplastic pyloric glands have been described in a variety of organs including the gallbladder, in which they can extend into the muscular wall and serosa. methods: Clinical, histologic, and immunohistochemical features of four cases of gallbladder florid pyloric gland metaplasia with perineural and intraneural invasion are analyzed. RESULTS: The patients with pyloric gland metaplasia and perineural and intraneural invasion were all females ages 57-72 years. A preoperative diagnosis of chronic cholecystitis and cholelithiasis was made for all four patients, but a histologic diagnosis of adenocarcinoma was made for two patients and entertained in two others. Macroscopically the gallbladders showed changes usually associated with chronic cholecystitis. No intraluminal masses were observed in any of the gallbladders. The characteristic microscopic features included florid pyloric gland metaplasia, proliferation of medium-sized nerve trunks more prominent in the muscular layer and serosa, and perineural and intraneural invasion by the metaplastic glands lined by cytologically bland cuboidal or columnar mucin-containing cells. At last follow-up all patients were alive and symptom free 1-7 years after laparoscopic cholecystectomy. CONCLUSIONS: Pyloric gland metaplasia of the gallbladder should be added to the long and increasing list of benign epithelial proliferations that are associated with perineural and intraneural invasion. This lesion should not be mistaken for adenocarcinoma of the gallbladder, a misinterpretation that may have serious therapeutic implications. The pathogenesis of this phenomenon is unknown.
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keywords = nerve
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10/256. Sellar chondroma--case report.

    A 12-year-old boy presented with right visual disturbance. skull radiography and computed tomography (CT) showed an irregular deformity of the sella turcica, hypertrophic change of the dorsum sellae, and an inhomogeneously calcified mass in the sella turcica. Magnetic resonance (MR) imaging demonstrated the mass lesion filled the hypophyseal fossa, and extended to the dorsum sellae, right cavernous sinus, and right suprasellar region. The Dolenc pterional combined epidural and subdural approach was carried out. The histological diagnosis was chondroma. Sellar chondroma requires relief of the compression to the chiasm or optic nerve as soon as possible, so partial resection can still be beneficial. However, follow-up MR imaging or CT, visual examination, and control of pituitary dysfunction are required after the operation.
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