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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of histologically verified neurinomas of the oculomotor nerve are reported. The preoperative diagnosis in all three cases was not made; on the contrary all cases were diagnosed as sphenoid ridge meningiomas. The oculomotor neurinoma manifests itself in the beginning with a discrete progressive palsy of the oculomotor nerve leading in later stages to complete ophthalmoplegia. Proceeding the oculomotor nerve paresis or going parallel to it is a functional loss of the homolateral optic nerve sometimes progressing to amaurosis. Unilateral exophthalmos as well as frontal or orbital neuralgic pain with or without sensory disorders in the area of trigeminus-I are characteristic for the clinical picture in later stages, all symptoms characteristic for the syndrome of the apex orbitae, resp. the superior orbital fissure, resp. the anterior cavernous sinus. The differential diagnosis has to consider above all the sphenoid ridge meningioma, the trigeminal neurinoma and the numerous tumors within the cavernous sinus (aneurysmas, meningiomas, chondromas, metastases of carcinomas, pituitary adenomas etc.) or the middle cranial fossa. Plain X-ray, carotid angiogram and computer tomogram are essential diagnostic means for localization and extension of the tumor but not for histological diagnosis. Oculomotor neurinomas are very seldom. The three observed cases did not have any relation to a possible generalized neurofibromatosis.
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PMID:[Neurinoma of the oculomotor nerve (author's transl)]. 65 Dec 44

This is a review of primary and secondary tumors of the optic nerve. The emphasis is an optic nerve gliomas and meningiomas. Optic nerve gliomas are slowly growing astrocytic neoplasms of the anterior visual pathways, the majority of which occur within the first two decades of life, with equal sex incidence in about 1 of 200,000 patients presenting with eye complaints. The incidence is greater in neurofibromatosis. The typical presentation is visual impairment in a verbal pre-school child with optic canal enlargement and optic atrophy. An intraorbital location of the tumor leads to axial, irreducible, non-pulsatile proptosis. An intracranial location may disturb hypothalamic and pituitary function and produce hydrocephalus. Ocular findings may also include limited motility on a mechanical-restrictive basis, a pupillary relative afferent defect, nystagmus, and variable, non-specific visual field defects. Roentgenographic studies may show concentric unilateral enlargement of the optic canal with preservation of a well corticated margin, a fossa under the anterior clinoid process in continuity with the optic canal ('J'-shaped sella), and findings of increased intracranial pressure. On pathologic examination the tumor is a smooth, fusiform, intradural enlargement of the optic nerve. Histologically there is proliferation of elongated (pilocytic) astrocytes in reticulated patterns with intervening microcystic spaces containing mucosubstance and surrounding reactive hyperplasia of the arachnoid. Mitoses are not found. The diagnosis is clinical X-ray studies and brain scan should be performed. The differential diagnosis is that of unilateral proptosis in a child and includes acute ethmoiditis, hyperthyrobidism, craniostenosis, other neoplasms, Hand-Schuller-Christian disease, and orbital hemorrhage due to trauma. Surgical resection is performed in cases with unilateral optic nerve involvement, the surgical approach being determined by tumor location. Bilateral or chiasmal cases are treated with radiotherapy when progression occurs. Malignant optic nerve gliomas and optic nerve hyperplasia are also discussed. Optic nerve meningiomas arise from the nerve sheath and are to be distinguished from orbital meningiomas arising from ectopic arachnoidal cells or those secondarily involving the orbit by extension from adjacent sites. Up to 80% of orbital meningiomas occur in females, in two age peaks, 25% in the first decade, and the rest in the 5th decade. Meningiomas present with visual loss and may produce proptosis, papilledema and/or optic atrophy, retinal striae, opticociliary shunts, limitation of extra-ocular movements, and lid edema, Signs of von Recklinghausen's disease should be sought. X-rays are the mainstay of diagnosis. Orbital meningiomas are composed of cells in sheets or in whorls with some spindle shaped cells. Calcifications are typical. Usually the dura is penetrated and the orbit invaded. Primary orbital meningiomas are locally infiltrating but do not metastasize. Complete local excision en bloc is recommended...
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PMID:Tumors of the optic nerve. 77 17

Twenty patients, 16 males and 4 females, aged 11-76 yr, were treated for a metastatic pheochromocytoma at our institution between 1985 and 1990. A neurofibromatosis was associated in 4. Thirteen patients had a unilateral adrenal tumor, 3 had an extraadrenal retroperitoneal tumor, 2 had a bilateral adrenal pheochromocytoma, one had a unilateral tumor with a contralateral medullary hyperplasia and one an adrenal and an extraadrenal pheochromocytoma. Metastases occurred in all patients, at presentation in 11, 10 to 30 months later in 7, and 9 and 28 yr later, respectively in two. Histology did not afford conclusive evidence for malignancy. Catecholamine hyperproduction was present in all, predominantly affecting norepinephrine. Neuron Specific Enolase level was elevated in 11, Neuro-Peptide Y level in 9 and procalcitonin level in 11/18. High dopamine, methoxytyramine and homovanillic acid excretion levels seemed to correlate with large tumors or terminal stage. MIBG uptake was found in 16 after a diagnostic dose and in 1 only after a therapeutic dose. Surgery was performed on primary tumor in 18 and on distant metastase in 10. Iodine-131 MIBG therapy was performed in 11, among whom 9 were evaluable. Cumulative activity ranged from 100 to 711 mCi, in 1 to 6 courses. Symptomatic improvement occurred in 5 patients, stabilization was observed in 3 and tumor partial response in two, which lasted for 28 and 9 months, respectively terminating in a rapidly progressing disease with bone marrow involvement. Moderate myelosuppression occurred in 4 patients. Chemotherapy gave no response in 7 evaluable patients. Fourteen patients died with a median survival of 16 months from diagnosis of metastases (range 3-60). Response to therapy was poor and warrants further cooperative trials.
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PMID:Malignant pheochromocytoma: clinical, biological, histologic and therapeutic data in a series of 20 patients with distant metastases. 147 46

Major syndromes in which cutaneous and extracutaneous nervous neoplasms are frequently associated include: 1) dysgenetic syndromes or phacomatoses (tuberous sclerosis and neurofibromatosis), 2) multiple schwannoma syndromes (schwannomatosis and Carney's complex), 3) multiple mucosal neuromas syndrome, 4) neurocutaneous pigmentary syndromes (Peutz-Jeghers-Touraine syndrome and neurocutaneous melanosis), and 5) sundry associations (cutaneous meningiomas and cutaneous metastases of neuroblastoma or carcinoid tumors). The early clinical and pathological recognition of these cutaneous neural and pigmentary associated lesions should stimulate the search for centrally located neural or neuroendocrine neoplasms, some of which might be life-threatening.
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PMID:Cutaneous neuropathology: neurofibromas, schwannomas and other neural neoplasms with cutaneous and extracutaneous expressions. 195 52

This report presents a very rare case of a malignant "Triton" tumor developing in the thyroid. Although malignant "Triton" tumors have often been known to develop in cases of neurofibromatosis, Von Recklinghausen's disease, this patient showed no clinical evidence of that disease. Total thyroidectomy including left standard radical neck dissection was followed by postoperative irradiation to the neck. Despite this, local and distant metastases developed four months after treatment and the recurrent foci did not respond to chemotherapy or irradiation. The clinical course of this case mimicked that of anaplastic carcinoma of the thyroid and interestingly, a minimal lesion of papillary adenocarcinoma was found to exist concurrently in the thyroid gland of this patient.
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PMID:Malignant "triton" tumor in the thyroid--a case report. 196 Sep 8

Ninety-two cases of intrathoracic neurogenic tumors operated on between 1950 and 1982 are reviewed. The benign forms (86 cases, 93.5%) included 23 ganglioneuromas, 50 neurilemmomas and 13 neurofibromas. Of the latter, 4 occurred in patients with Von Recklinghausen's disease. A double local recurrence was observed after the removal of a neurilemmoma. One of the patients with generalized neurofibromatosis died 5 months after operation from local sarcomatous degeneration and distant metastases. In this group of benign lesions, no other death was observed which could be attributed to the endothoracic neural tumor. The malignant forms (6 cases, 6.5%) included 4 ganglioneuroblastomas and 2 neurofibrosarcomas. The surgical excisions were described as radical in every case and all the patients were given radiotherapy postoperatively. One patient with ganglioneuroblastoma died from metastases 2 years later, and one with neurofibrosarcoma from local recurrences 7 months later. One patient with neurofibrosarcoma and 3 with ganglioneuroblastomas are alive and well 4, 5, 6 and 11 years, respectively, later. Surgical excision remains the best method of diagnosing and treating endothoracic tumors of neural origin. Of special interest are the "dumbbell" or hourglass tumors and lesions which occur in Von Recklinghausen's disease.
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PMID:Neurogenic intrathoracic tumors. A clinicopathological review of 92 cases. 242 96

A case of schwannosarcoma associated with a pleural neurofibroma and a parathyroid adenoma is presented. The neuroectodermal origin and the relationship with neurofibromatosis are discussed. The sarcomatous relapse of a schwannoma previously classified as benign confirms the difficulty of microscopic analysis and the malignancy power of the tumor. A long survival is obtained by a large intestinal and mesenteric resection as long as hepatic metastases are not present or can be resected. The pleural neurofibroma needs symptomatic treatment while parathyroid adenoma must be early removed as soon as malignant hypercalcemia is detected.
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PMID:[A malignant schwannoma of the jejunum associated with multiple neurofibromas and a primary adenoma of the parathyroid]. 249 38

Liver involvement by neurofibromatosis is rare. This report describes a young man with von Recklinghausen's disease and hepatic neurofibromas who developed a large right hepatic lobe malignancy and died of massive intratumor hemorrhage. Postmortem examination showed the tumor to be composed of both malignant schwannoma and angiosarcoma and to have arisen from contiguous neurofibromas in portal tracts. Widespread pulmonary metastases consisted of the angiosarcomatous elements alone. The expression of malignant schwannoma and angiosarcoma phenotypes in this tumor may be related to a common histogenesis from cells of the neural crest.
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PMID:Hepatic neurofibromatosis, malignant schwannoma, and angiosarcoma in von Recklinghausen's disease. 309 7

Neoplastic proliferations of neuroendocrine cells (NE) may occur throughout the entire GI tract but affect particularly appendix and ileum ("midgut carcinoids"), rectum ("hindgut carcinoids"), as well as stomach and the duodenum ("foregut carcinoids"). Only more exceptionally, they arise in the esophagus, jejunum and colon. The NE tumors encompass a heterogeneous gross and microscopic structural spectrum, ranging from inconspicuous microproliferations ("mucous membrane nevi") to bulky tumor masses. Their growth patterns are usually characteristic and easily recognized. In doubtful cases their NE differentiation becomes established by a characteristic silver affinity, by the ultrastructurally observed presence of characteristic "endocrine" secretion granules, and by immunohistochemically detectable occurrence of "pan-NE markers" (neuron-specific enolase, chromogranins, and synaptophysin), biogenic amines (mainly serotonin), and neurohormonal peptides. Foregut carcinoids usually contain serotonin, gastrin, and somatostatin, midgut carcinoids often only serotonin and tachykinins, whereas the hindgut carcinoids as a rule are multihormonal with a wide spectrum of hormonal peptides, including even insulin. Most GI NE tumors are found in the appendix (50%) and the ileum (30%). Practically all (98%) of the appendiceal NE tumors are benign. They have recently been proposed as arising from apparently Schwann-cell-related NE cells in the submucosa, whereas the ileal--and probably also all the other non-appendiceal NE tumors--are derived from the totipotential cells in epithelial crypts of the mucosa. Among the ileal NE neoplasms a large number can metastasize and result in a fatal outcome. The ability to metastasize is related to the size and to the multiplicity of the primary tumors at the time of initial diagnosis and, to some extent, to their histopathologic growth pattern. Now, some relationship between the prognosis and the cytochemically assessed nuclear DNA content of the NE tumor cells has also been established; not less than about 1/4 to 1/3 seem to be aneuploid. Almost 90% of the rectal carcinoids are benign. Exceptionally, a highly malignant NE neoplasms can arise from the colon/rectum--as well as from the esophagus--composed of NE cells of small and intermediate size. The NE tumors of the stomach are often composed of ECL (enterochromaffin-cell-like) cells; such ECL cell carcinoids are related to atrophic gastritis with pernicious anemia; experimentally, they can be induced by hypergastrinemia in rats. Duodenal carcinoids often contain psammoma bodies and can be associated with neurofibromatosis.
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PMID:Neuroendocrine tumors of the gastrointestinal tract. 329 Aug 66

The first case of malignant schwannoma of the submandibular gland in a 23-year-old man with neurofibromatosis is reported. Light microscopy showed typical features of neurogenic sarcoma and an immunocytochemical study confirmed the schwannian differentiation of the neoplasm. The patient is still well without evidence of metastases or local recurrence of the tumor 80 months after the excision of the neoplasm.
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PMID:Malignant schwannoma of the submandibular gland. A case report. 371 98


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