Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Loss of negative growth regulation and high invasive potential are neoplastic traits often associated with abnormal expression of matrix metalloproteinases (MMPs). We previously found MMP-3 (stromelysin/transin) was secreted by quiescent rat Schwann cell cultures and expressed potent antiproliferative activity. In the present study we observed that human Schwann cells and cutaneous neurofibroma Schwann cell cultures secreted abundant MMP-3 and their proliferation was inhibited by autologous and rat Schwann cell conditioned media. Antiproliferative activities were depleted by immunoadsorption with anti-stromelysin antibodies. In contrast, plexiform neurofibroma cultures did not secrete MMP-3 and failed to respond to Schwann cell antiproliferative activities associated with MMP-3. Quiescent Schwann cells constitutively secreted low levels of MMP-2 (gelatinase A) and showed a low invasion potential in filter-based assays of basement membrane invasion. Cyclic AMP elevation, which profoundly influences cell differentiation, increased the invasion potential of rat Schwann cells and caused a corresponding increase in secretion of MMP-2. Schwann cells immortalized by protracted elevation of cAMP, as well as a schwannoma cell line (D6P2T), also rapidly invaded a reconstituted basement membrane and over-expressed MMP-2. Similarly, neurofibroma Schwann cells were highly invasive and secreted up to 10-fold more MMP-2 than normal human Schwann cells. Additionally, only cutaneous neurofibroma Schwann cell cultures secreted MMP-9 (gelatinase B) and MMP-1 (interstitial collagenase) and also invaded native type I collagen barriers. Cultures of normal Schwann cells and plexiform neurofibroma tumor expressed little or no MMP-1 and did not invade type I collagen barriers. These results suggest a role for MMPs in the control of proliferation and invasion by Schwann cells and in the formation of peripheral nerve sheath tumors.
Clin Exp Metastasis 1995 Jul
PMID:Differences in proliferation and invasion by normal, transformed and NF1 Schwann cell cultures are influenced by matrix metalloproteinase expression. 760 93

This is a clinicopathologic study of 115 lacrimal sac neoplasms in adults (mean 52 years). The most common presenting signs and symptoms were epiphora (53%), recurrent dacryocystitis (38%), and/or lacrimal sac mass (36%). The tumors were divided into epithelial (82 cases) and nonepithelial (33 cases) neoplasms. Benign epithelial tumors included squamous and transitional cell papillomas (32), oncocytomas (4), and benign mixed tumors (2). The malignant epithelial neoplasms included squamous cell carcinoma (22), transitional cell carcinoma (5), adenocarcinoma (4), mucoepidermoid (3), adenoid cystic (3), and poorly differentiated carcinoma (1). The nonepithelial tumors consisted of fibrous histiocytoma (13), lymphoid lesions (10), malignant melanoma (6), hemangiopericytoma (1), lipoma (1), granulocytic sarcoma (1), and neurofibroma (1). Review of the literature, including our own series, discloses a 55% malignancy rate for tumors originating in the lacrimal sac. Malignant epithelial neoplasms, especially invasive transitional cell carcinoma, often recur locally and can metastasize and be fatal. Epithelial malignancies tend to grow along the epithelium of the lacrimal drainage system, and thus cure is dependent on a wide surgical excision of the tumor and of the entire lacrimal drainage system (canaliculi, sac, and nasolacrimal duct) combined with a lateral rhinostomy and radiation therapy.
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PMID:Lacrimal sac tumors. 794 44

A series of six cases of a previously unrecognized variant of liposarcoma characterized by a prominent spindle cell component is reported herein. Clinically, all of the tumors arose in adults and developed around the shoulder girdle or upper limbs; all but one arose in subcutaneous tissue. Three patients developed multiple local recurrences over a period of 4-20 years. Recurrences in one case were purely lipoma-like. Following dedifferentiation in a recurrence, one patient developed distant metastases and eventually died, 46 months after the primary excision. Grossly, these lesions are characterized by multinodularity, and microscopically they show a relatively bland spindle cell proliferation arranged in fascicles and whorls and set in a variably myxoid stroma. The spindle cell areas are accompanied by an adipocytic component, which exhibits the morphologic features required for inclusion in the well-differentiated liposarcoma-atypical lipoma group, including definite lipoblasts. Main differential diagnoses include benign lesions such as spindle cell lipoma and diffuse neurofibroma, as well as dermatofibrosarcoma protuberans and other malignancies such as sclerosing liposarcoma, low-grade myxofibrosarcoma, low-grade malignant peripheral nerve sheath tumor, and low-grade fibromyxoid sarcoma. In view of their distinctive histologic appearance, and because aggressive clinical behavior was observed despite their superficial location, we propose that these lesions be regarded as spindle cell variants of well-differentiated liposarcoma.
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PMID:Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. 777 80

A 20-year-old caucasian woman with a 5-year history of right arm, neck, and back pain sought treatment when an automobile accident (4 months before admission) exacerbated her pain. Magnetic resonance imaging revealed an intra- and extradural mass compressing the spinal cord at the C5-C6 level. It also extended into and widened the neural foramen, mimicking a neurofibroma. A single cafe-au-lait spot was discovered in the inguinal region. A two-staged surgical resection was performed on an apparent hemorrhagic C6 nerve root mass. The mass exhibited diagnostic features of a malignant melanoma histologically, immunocytochemically, and ultrastructurally. A search for a primary lesion outside the nervous system or other metastases during an 8-year period from the onset of symptoms has been negative. The patient's chronic history, evidence of neural foraminal enlargement, and the absence of other malignant melanoma lesions or subsequent metastases indicates that this lesion may be a primary melanoma of the nerve root with a benign course.
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PMID:Melanoma arising in a cervical spinal nerve root: report of a case with a benign course and malignant features. 819 Feb 31

We described two cases of malignant schwannoma arising in patients with von Recklinghausen's disease and examined the mast cells infiltrated into histologic sections. One of the two cases histologically revealed apparent mast cell infiltration in some areas of malignant schwannoma as well as in the benign neurofibroma. The malignant lesion demonstrated significantly increased percentages of degranulated mast cells over the benign lesion using FITC-avidin staining. In an electron microscopic study, mast cells in the malignant lesion displayed empty granules, piecemeal degranulation, and canaliculi structures suggesting activation. These findings were not observed in the benign lesion. The other patient histologically showed no mast cells in the malignant lesion, although the benign neurofibroma in the patient disclosed numerous mast cells. The first patient had neither recurrence nor distant metastasis. On the other hand, the second patient without mast cells in the histology had multiple distant metastases.
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PMID:Malignant schwannoma arising in patients with von Recklinghausen's disease: report of two cases and the comparison of mast cells between benign and malignant portions. 858 54

Cutaneous ganglioneuroma is rare. Only five cases have been reported, and in all patients the lesions developed after birth. We describe a congenital cutaneous ganglioneuroma. The differential diagnosis includes well-differentiated cutaneous metastases of neuroblastoma and ganglion cells entrapped by a neurofibroma in patients with neurofibromatosis.
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PMID:Primary congenital cutaneous ganglioneuroma. 869 26

In the period 1982-96, 18 bronchial resections were performed. Simple resection was carried out in eight patients, and in ten lung tissue was also removed; six upper lobes and three middle lobes were involved, and in one patient bilobectomy was necessary. The histologic diagnosis was carcinoid in 15 cases (83%), mucoepidermoid carcinoma in two, and in one case neurofibroma. Two patients had postoperative complications, but both recovered completely. All patients were followed up regularly by bronchoscopy. One patient had carcinoid with liver and bone metastases at the time of operation in 1989, but is still alive. Another died from metastases, the rest were in good condition at the most recent follow up. Bronchial resection is a safe procedure which should be preferred to standard pneumonectomy, in order to save as much lung tissue as possible. The frequency of bronchial resections for carcinoids (compared with standard pneumonectomy) was 60%.
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PMID:[Bronchial resections]. 923 6

Eighty patients with intramedullary spinal cord tumors were treated by microsurgical methods between 1988 and 1996. Twenty-nine patients were diagnosed with astrocytoma, 36 with ependymoma, five with metastasis, four with lipoma, three with dermoid or epidermoid tumor, two with neurofibroma, and one with neuroma. Vascular and infectious lesions (such as abscesses and hemangioblastomas) were excluded. After laminectomy, total removal of the lesion was achieved in 68 of 80 patients and subtotal removal in 12. Postoperative radiation therapy was performed in 13 of 80 patients. The follow-up period ranged from 12 to 92 months (mean 42.2 months). All patients underwent preoperative and postoperative magnetic resonance imaging at intervals ranging from 3 months to 5 years postoperatively. Four patients showed clinical and radiological evidence of local tumor recurrence during the follow-up period. Four patients died 5 months to 15 months postoperatively from the re-expansion of their primary metastatic disease. The operative results at long term (after the 6th postoperative month) were better than the results at short term (before the 6th postoperative month) and revealed clinical improvement in 63, no change in 10, and deterioration in seven patients. We recommend early radical surgery, whenever possible, to be performed when the patient's neurological status is still good. Subtotal removal and irradiation are better for malignant or metastatic tumors. Partial decompressive removal is best for large intramedullary lipomas. Plastic laminotomy with preservation of the intervertebral joints is especially recommended in young or middle-aged patients.
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PMID:Microsurgical treatment of intramedullary spinal cord tumors. 964 Sep 61

We report a case of a 17-year-old girl with multiple intracerebral tumors. Previously, a neurofibroma in the posterior mediastinum and neurofibromatosis had been diagnosed. She developed a spastic tetraparesis with a prominent hemiparesis of the right side within several weeks. On admission we found clinical signs of elevated intracranial pressure. Cranial CT and MRI scans showed multiple space-occupying intracerebral tumors, thought to be multiple meningeoma. The patient was referred to the neurosurgical department, where two of the intracerebral tumors were excised. The histological examination revealed metastases of a neurosarcoma.
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PMID:Multiple intracerebral metastases of a 17-year-old girl with previously diagnosed neurofibromatosis type I. 998 38

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon soft tissue tumors. In children with neurofibromatosis 1 (NF1), a MPNST often arises in a pre-existing neurofibroma, or may represent an initial manifestation without other obvious stigmata of the disease. The development of MPNSTs may be associated with instability of the p53 tumor suppressor gene since it is the most frequent genetic abnormality in soft tissue sarcomas. To assess the presence of p53 accumulation in MPNSTs and its correlation with clinical and pathologic features, we studied 12 neurofibromas (NFs), including 4 tumors with cellular features (one congenital) and 10 MPNSTs. Six MPNSTs were associated with NF1, all of which developed within a plexiform neurofibroma. Cell proliferation evaluated with an antibody to Ki-67 and nuclear p53 staining were both detected by immunohistochemistry. We found p53 positivity in 60% of MPNSTs. All NFs except the congenital tumor were p53 immunonegative (P < 0.01). Rare p53-positive nuclei were detected in the transitional zone in two of six MPNSTs arising in plexiform NFs. Ki-67 distinguished the NFs from MPNSTs (P < 0.005). Half of the NF1 patients with p53-positive MPNSTs developed recurrence or metastases or developed a second malignancy within 2 years of diagnosis, whereas patients with p53-positive sporadic MPNSTs were free of disease 1 to 7 years later. We found p53 accumulation more frequently in NF1-associated MPNSTs. p53 mutations may be an additional biologic factor to account for the poor prognosis in these tumors.
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PMID:p53 and Ki-67 proliferating cell nuclear antigen in benign and malignant peripheral nerve sheath tumors in children. 1034 83


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