Cases reported "Neurofibroma"

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1/13. thyroid gland neurofibroma in a NF1 patient.

    Neurofibromas are a hallmark of neurofibromatosis type 1 (NF1). They are usually benign and rarely present in the thyroid gland region. There is a suspected association between NF1 and intramedullary thyroid carcinoma and there is a well-known association between NF1 and pheochromocytoma. Here, we present a 55-year-old man with typical symptoms of NF1, whose course was complicated by a neurofibroma of the thyroid gland. His clinical spectrum of symptoms included bilateral cataract established before the age of 35 years, quadriparesis and an intrathoracic mass. The patient died because of abdominal carcinomatosis of unknown origin. The rarity of thyroid gland neurofibroma is discussed here, emphasizing the importance of early detection of these and other NF1 complications, also including the risk of malignant transformation with lethal outcome.
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2/13. A rare case of a facial-nerve neurofibroma in the parotid gland.

    The incidence of solitary neurofibroma of the facial nerve originating in the parotid region is extremely low. We report a case of a solitary neurofibroma in a 30-year-old male, who initially presented with a parotid mass without facial paresis or paralysis. A chain of small nodules had been palpable in the right parotid region for the previous 2-3 years. MRI and CT scans revealed several small ovoid lesions extending from the frontal margin of the parotid gland to the retromandibular region. The lesions were surgically removed. The main trunk of the facial nerve was adherent to the dorsal side of the largest nodule; however, this mass was resected atraumatically. Histopathological examination indicated neurofibroma. The incidence, presentation, diagnosis and surgical treatment of intraparotid neurofibroma are discussed and compared with those of Schwannoma.
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3/13. facial nerve neuromas: MR imaging. Report of four cases.

    Four cases of facial nerve neuroma were evaluated by computed tomographic (CT) scan and magnetic resonance imaging (MRI). The extension of the tumor in the petrous bone or the parotid gland was well defined by MRI in all cases. CT scan was useful to demonstrate bone erosions and the relation of the tumor to inner ear structures. In cases of progressive facial palsy, CT and MRI should be combined to detect a facial neuroma and to plan the surgical approach for tumor removal and nerve grafting.
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4/13. Metastatic acinic cell carcinoma in a neurofibroma mistaken for carcinosarcoma.

    BACKGROUND: Tumor-to-tumor metastasis is a rare, but well-recognized, entity most commonly involving metastatic carcinoma to a mesenchymal neoplasm. We report a case of acinic cell carcinoma of the parotid gland metastatic to a neurofibroma. methods AND RESULTS: A 55-year-old man with a history of a high-grade acinic cell carcinoma of the parotid was seen with a mass at the surgical site and metastatic foci in the scalp 10 months postoperatively. The resection specimen revealed a spindle cell lesion with metastatic foci of high-grade adenocarcinoma, initially diagnosed as a carcinosarcoma. The bland morphology and S-100-positive expression of the spindle cell lesion confirmed the diagnosis of neurofibroma. The high-grade features of the carcinomatous foci and their similarity to the primary tumor confirmed the presence of a tumor-to-tumor metastasis. CONCLUSION: To our knowledge, this is the first reported case of acinic cell carcinoma metastatic to a neurofibroma, an important entity in the differential diagnosis of biphasic tumors of the head and neck.
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5/13. neurofibroma of the lingual nerve: a case report.

    A neurofibroma of the lingual nerve is a rare clinical finding, the most common lesion site of a lingual neurofibroma being the tongue. In most situations, it is difficult to determine the precise nerve origins. Herein, we report a case of lingual nerve neurofibroma that presented as a submandibular mass, mimicking a submandibular gland tumor or solitary lymphadenopathy. Complete surgical excision of such a lesion for histopathologic examination provides a better treatment and final diagnosis. For a patient presenting with neurofibromatosis and a submandibular mass, a neurofibroma of nerve origin should be considered in the differential diagnosis.
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6/13. Unusual association of adrenal pheochromocytoma and para-aortic neurofibroma in pregnancy.

    The association of pheochromocytoma (PHEO) and pregnancy is uncommon and life threatening for both the fetus and the mother. early diagnosis and treatment is essential to decrease maternal and fetal mortality and to differentiate the disease from the more common pre-eclampsia. While medical treatment should be started immediately after diagnosis, the timing of surgical treatment is still debated. We describe the case of a 27-yr-old woman in the 18th week of pregnancy who showed a biochemical pattern typical of PHEO and, by imaging studies, 2 tumors with the same characteristics: the first localized on the right adrenal gland, the second at the right renal hilum. The patient underwent surgery because of suspicion of malignant PHEO with local metastasis, while histology revealed a rare association of a solitary PHEO and para-aortic neurofibroma, both tumors embryologically deriving from a common cell precursor.
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7/13. Pleomorphic adenomas of the parotid gland resembling mesenchymal tumors.

    Two cases of unusual pleomorphic adenoma of the parotid gland are described. Each showed marked palisaded zones. Distinction from smooth muscle tumors, neurofibromas, and other spindle-cell neoplasms is described.
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8/13. neurofibroma of the ear.

    Neurofibromas cause unsightly and crippling deformities. These locally destructive tumors arise along the distribution of peripheral and cranial nerves. The 2 patients presented here had neurofibromas of the external ear. In one patient, the lesion was limited to the auricle. In the other, the tumor extended into the facial nerve and parotid gland. Preoperative computed tomographic scanning, a postauricular approach to the tumor, and careful dissection produced an aesthetic debulking of the auricular neurofibromas with preservation of facial nerve function and improvement in hearing.
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9/13. Synchronous malignant glandular schwannomas in congenital neurofibromatosis.

    An eight-year-old child with congenital neurofibromatosis developed rapidly growing synchronous malignant schwannomas in the neck and mediastinum. These tumors, displaying a biphasic pattern of spindle cell sarcoma admixed with mucinous epithelium, represent the eighth and ninth known examples of glandular schwannoma. The clinical and pathological features of this case are detailed and the histogenesis of the tumors discussed.
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10/13. Benign peripheral nerve sheath tumors (neurofibromas) of the lacrimal gland.

    Two patients, a 43-year-old woman and a 62-year-old man, developed benign peripheral nerve sheath tumors (neurofibromas) of the lacrimal gland, in the former case of the deep orbital lobe, and in the latter of the palpebral lobe. A neurogenic tumor was not seriously considered clinically in either case, because of the paucity of previous reports on the occurrence of neural tumors in the lacrimal fossa region. Instead, in the first case a benign mixed tumor was considered the most likely clinical diagnosis, whereas in the second the coexistence of a swelling in the parotid gland raised the possibilities of a lymphoid tumor or a leukemia. Due to unusual light microscopic features, transmission electron microscopy was required to secure unequivocal diagnoses. In the orbital lobe tumor, an encapsulated spindle cell proliferation without nuclear palisading but with focal areas of myxoid change suggested either a cellular benign mixed tumor or a schwannoma. The palpebral lobe lesion displayed a myxoid spindle cell proliferation that had splayed apart the ducts and acinae of the lacrimal gland, to impart the overall appearance of a benign mixed tumor. The ducts, however, were multilaminar rather than possessing the usual double cellular layer seen in classical benign mixed tumors, and the glandular units contained zymogen granules, which are typically not seen in benign mixed tumors. Electron microscopy disclosed in both lesions the presence of interrupted basement membrane material partially surrounding the tumor cells, long spacing collagen (banded basement membrane material), and poorly developed desmosomes, features compatible with a neural tumor. Neither myofilaments, tonofilaments, nor ductal-type granules sometimes observed in benign mixed tumors were discovered. The tumor cells in these cases therefore failed to demonstrate clear-cut Schwann cell characteristics (eg, continuous basement membranes, pseudo-mesaxons, tangles of cell processes), and more closely resembled perineural cells that have been described in ultrastructural studies of peripheral nerve tumors including neurofibromas of other sites.
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