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At least 343 records · Page 19Linked to original sources
PubMed1997

[Neurofibromatosis type 1: a survey of 195 patients].

OBJECTIVE: To analyse symptoms and complications in patients with neurofibromatosis type 1 (NF1). All patients were examined in a multidisciplinary outpatient neurofibromatosis clinic during a period of 10 years. DESIGN: Retrospective. SETTING: Academic Medical Center, University Hospital Amsterdam, the Netherlands. METHOD: All data on 450 persons visiting the neurofibromatosis clinic were stored in a database. Data were collected on the results of dermatological, neurological, ophthalmological and general examinations and on family history. For this study the follow-up data of 196 patients with a definite diagnosis of 'NF1' were analysed. RESULTS: In childhood diagnosis NF1 is predominantly based on specific dermatological symptoms such as > 6 café-au-lait (CAL) spots and freckling and on the presence of characteristic ophthalmological signs as two or more Lisch nodules. In this study the frequencies of these symptoms were 98% (CAL). 92% (freckles), and 93% (Lisch nodules) respectively. The frequencies of well-known complications of this disorder are comparable with the literature findings. In this study we found optic pathway glioma (OPG) in 10%, macrocephaly in 36%, hydrocephalus in 5%, retardation in 14%, brain tumours in 5%, kyphoscoliosis in 13%. renal artery stenosis in 0.5% and neurofibrosarcoma in 0.5% of NF1 patients. In children the degree of severity of this disorder is less than in adults, demonstrating the progressive character of the disease. CONCLUSION: The diagnosis of 'NF1' can usually be made by dermatological and ophthalmological examination. In case of a definite diagnosis in childhood regular follow-up is recommended since severe complications, such as OPG and kyphoscoliosis, may occur specifically in childhood and adolescence. For adult patients determination of the degree of severity is essential for the decision whether or not they need regular follow-up; they should have their blood pressure measured annually.

Adolescent↗
PubMed1997

Intraoperative electron irradiation in the management of pediatric malignancies.

BACKGROUND: External beam irradiation (PBRT), especially in children, is limited by the radiosensitivity of normal tissues. Local control remains a problem in abdominopelvic childhood malignancies. Intraoperative electron irradiation (IOERT) has the potential to increase the dose to the tumor, thereby improving local control, without increasing treatment morbidity. METHODS: Between February 1983 and October 1990, 11 children received IOERT as part of a multidisciplinary treatment program for locally advanced primary or recurrent abdominopelvic malignancies. The 7 boys and 4 girls ranged in age from 2-18 years. Tumor histologies included four neuroblastomas, two desmoid tumors, and one each of the following: embryonal rhabdomyosarcoma, synovial cell sarcoma, neurofibrosarcoma, malignant fibrous histiocytoma, and paraganglioma. Single radiation doses of 10-25 grays were delivered using 6-15-megaelectron volt electron beams to 1-5 IOERT fields. All patients also underwent EBRT and six received chemotherapy. RESULTS: Eight patients (73%) were alive and disease free at a median follow-up of 99 months (range, 37-126 months). All eight patients who underwent gross total resection were locally controlled. Three patients required surgical intervention for IOERT-related complications and two patients developed neuropathies. CONCLUSIONS: IOERT as part of a multidisciplinary treatment approach in patients with locally advanced pediatric malignancies appears to enhance local control in those patients in whom a gross total resection is possible. The long term survival rate was encouraging. Further study, with a larger number of patients, appears warranted to more carefully delineate the efficacy and tolerance of IOERT in the pediatric population.

Abdominal Neoplasms↗
PubMed1997

Purification and characterization of human lung fibroblast motility-stimulating factor for human soft tissue sarcoma cells: identification as an NH2-terminal fragment of human fibronectin.

Paracrine motogenic factors, including motility cytokines and extracellular matrix molecules secreted by normal cells, can stimulate metastatic cell invasion. Both intact extracellular matrix molecules and their degradative products may exhibit these activities. We have found that human lung fibroblasts produce paracrine motility-stimulating factors for recently established human sarcoma cell strains. We purified the major fibroblast motility-stimulating factor (FMSF) from human lung fibroblast-conditioned medium by sequential heparin affinity chromatography and DEAE anion exchange chromatography. Lysylendopeptidase C digestion of FMSF and sequencing of peptides purified by reverse-phase high-pressure liquid chromatography identified FMSF as an NH2-terminal fragment of human fibronectin. Using SYN-1 sarcoma cells, FMSF predominantly stimulated chemotaxis and some chemokinesis, and it was chemotactic for a variety of human sarcoma cells, including fibrosarcoma, leiomyosarcoma, liposarcoma, synovial sarcoma, and neurofibrosarcoma cells. The FMSF activity present in human lung fibroblast-conditioned medium was completely eliminated by either neutralization or immunodepletion with a rabbit antihuman-fibronectin antibody, thus further confirming that the NH2-terminal fibronectin fragment was the FMSF responsible for the motility stimulation of human soft tissue sarcoma cells. Because human soft tissue sarcomas have a distinctive hematogenous metastatic pattern (predominantly lung), and lung-derived fibroblasts secrete large amounts of FMSF, FMSF and fibronectin may play a role in stimulating sarcoma invasion into lung tissue.

Amino Acid Sequence↗
PubMed1997

[A case of multifocal inflammatory pseudotumor of the liver].

INTRODUCTION: The inflammatory pseudotumor (IPT) of the liver is a tumor-like lesion of unknown etiology and pathogenesis. Non infectious by definition the lesion is considered to be a long-lasting inflammatory process. CASE: In this report we describe a multifocal liver pseudotumor of a 52 year man who underwent a selective hepatectomy for a clinical and radiological suspicion of a liver cell carcinoma. RESULTS: Grossly the masses consisted of rubbery grey-yellowish nodules well-circumscribed from the liver parenchyma. Histologic examination revealed a double shaped cellular population surrounded by a thin rim of collagen fibres. The main population consisted of fibroblasts and histiocytes with no atypias or mitoses, arranged in a storiform or fascicular pattern; the second population consisted of a mixed population of lymphocytes, plasma cells, neutrophils and monocytes. Fibroblasts and histiocytes revealed diffuse positive immunostaining for vimentin and focal immunostaining for cytokeratin and desmin. The histiocytes were also CD68, CD18, CD14 and CD4 positive. CONCLUSIONS: Our case reveals same histological and immunohistochemical features already reported in literature. Primary and metastatic tumors involving the liver need to enter in the differential diagnosis but IPT lacks features of malignancy and the cell population is mixed. Although there is no specific marker for this lesion in cases featuring malignancy immunohistochemistry may be useful for a differential diagnosis: S-100 in cases of neurofibrosarcomas, actin and myosin for rhabdomyosarcomas, cytokeratin for epithelial tumors.

Biomarkers↗
PubMedDate not supplied

A diffuse form of neurofibroma of bladder in a child with von Recklinghausen disease.

Neurofibromatosis in infants is uncommon and involvement of the bladder is rare. The reported bladder lesions are rare, and in such patients include neurofibromas, neurofibrosarcomas and rhabdomyosarcomas. Neurofibromas can be of different histologic types. The histologic type of the about 20 reported bladder neurofibromas in children is not clarified or is stated to be of plexiform type. We describe herein an unusual case of a neurofibroma of bladder in a child with von Recklinghausen disease. The therapeutic management and the possible prognostic implication of the type of the bladder neurofibroma and bladder lesions other than neurofibroma are discussed.

Cystectomy↗
PubMedDate not supplied

[Oropharyngeal tumors of dogs--a clinical study of 79 cases].

This study presents the data on incidence, TNM-classification and therapy outcome of 79 dogs with oropharyngeal tumors, which were admitted to the Clinic of Small Animals, Hannover School of Veterinary Medicine. 52 neoplasms were examined histologically. The most common tumors were malignant melanoma (n = 17), fibrosarcoma (n = 5), squamous cell carcinoma and peripheral odontogenic fibroma (n = 4). It could be determined that dogs treated by surgery, regardless of tumor type and type of surgery, had longer survival times than untreated dogs. With regard to survival time and the rate of local tumor recurrence, radical surgery (partial mandibulectomy/maxillectomy) led to good results in squamous cell carcinomas and invasive odontogenic tumors, but, keeping in mind the small number of cases, showed no advantage over conservative surgery in malignant melanomas, fibrosarcomas, neurofibrosarcomas and non invasive odontogenic tumors. It could be shown that the clinical staging of the patients was of prognostic relevance.

Animals↗
PubMed1978

[The problem of malignant degeneration in case of multiple neurofibromatosis (author's transl)].

Within less than one year, our hospital received three cases of malignant manifestation in case of multiple neurofibromatosis. If the histological findings and the neurogenic origin of the malignant tumor are evaluated very critically, the malignant suppression of the differentiation of one or more neurofibromas in case of multiple neurofibromatosis must be considered as occuring very rarely. If in case of a neurofibromatosis a malignant manifestation is found, it has to be examined very carefully by tissue specimens of numerous spots (Loehr and Willebrand), furthermore the neurogenic origin must be ensured (Zuelch). There are three clinical possibilities: 1. Sarcomatous degeneration of a neurofibroma with demonstrable neurogenic origin. 2. Histological appearence of a malignant suppression of the differntiation of a neurofibroma with benign clinical course. 3. Independent sarcoma as second disease without original connection to an existing multiple neurofibromatosis.--A critical evaluation of our cases showed that there was only one case of malignant tumor with neurogenic origin, namely a neurofibrosarcoma originating from a multiple neurofibromatosis.

Adult↗
PubMed2004

Salvage surgical resection after high-dose ifosfamide (HDIF) based regimens in advanced soft tissue sarcoma (ASTS): a potential positive selection bias--a study of the Spanish group for research on sarcomas (GEIS).

PURPOSE: To assess the impact of different factors on response rate (RR), time to tumor progression (TTP), and overall survival time (OS) in patients with locally advanced or metastatic soft tissue sarcoma (ASTS), included in three protocols with high-dose ifosfamide (HDIF). PATIENTS AND METHODS: One hundred fifty six ASTS patients included in three consecutive phase II trials with HDIF (>10 g/m(2)), alone or in combination with doxorubicin (DX), were analyzed. Cofactors were institution, trial, gender, age, performance status, histologic type, grade of malignancy, prior radiotherapy, presence of locoregional disease, metastatic site, salvage surgery, number of organs involved, and disease-free interval. RESULTS: By multivariate analysis performance status >0 and lack of salvage surgery correlated with a poorer survival. A good-risk and a poor-risk group were identified, with median survival time (OS) of 29, 5, and 10 months, respectively (P = 0.00001). The 1-, 2-, and 3-year OS for 83 good-risk patients (either with PS = 0 or receiving salvage surgery) was 83, 44, and 29%, respectively, those figures being 37, 7, and 3% for 73 poor-risk patients. CONCLUSION: The design of randomized trials in ASTS including HDIF should consider those prognostic factors as stratification variables.

Adult↗
PubMed1995

Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): a 10-year experience.

BACKGROUND: Prior studies have shown a high rate of local recurrence and a dismal overall prognosis in malignant peripheral nerve sheath tumors (MPNSTs). METHODS: Thirty-three patients who had undergone primary treatment for localized extremity MPNSTs between 1982 and 1992 were reviewed. These cases were derived from a prospective database of 890 adult extremity soft-tissue sarcomas (STS). MPNSTs were compared with other extremity STS. RESULTS: MPNSTs were more often high grade and deep compared with other extremity STS (94% vs. 72% [p = 0.009] and 97% vs. 76% [p = 0.01], respectively). Location (upper or lower extremity), size (> 5 cm vs. < or = 5 cm), and status of margins after surgical resection (positive or negative for disease) did not differ between the two groups. When deep and high-grade MPNSTs were compared with deep and high-grade STS, a more aggressive local treatment was applied to MPNSTs with a higher number of amputations for MPNSTs (32%) compared with STS (9%; p < 0.001). In order to obtain adequate margins, 16 of 21 MPNSTs arising from major nerves required either amputation (n = 8) or nerve resection (n = 8). Adjuvant radiotherapy was used in 48% of deep and high-grade MPNSTs, and 3-year local disease-free survival was 70%. Survival of deep and high-grade MPNSTs was comparable with other deep and high-grade STS (3-year survival 50% vs. 69%, respectively; p = 0.1). CONCLUSION: MPNSTs show adverse clinicopathologic features compared with other STS. However, when treated aggressively, MPNSTs have an outcome similar to other deep and high-grade extremity STS.

Adolescent↗
PubMed1994

On clinical usefulness of Tl-201 scintigraphy for the management of malignant soft tissue tumors.

The purpose of this study was to investigate Tl-201 as a tumor scanning agent in patients with malignant soft tissue sarcomas and to establish the sensitivity of this type of scintigraphy concerning local recurrences or metastases that may remain clinically suspected. Seventy-eight patients with malignant soft tissue sarcomas and 22 with benign soft tissue tumors were studied. Of these 78 malignant soft tissue sarcomas patients, the sensitivity of Tl-201 (81.2%) was higher than that of Ga-67 (68.8%). Thirty-three out of 78 patients received a total of 95 consecutive scintigraphic follow-up examinations. Therapeutic effects was assessed by comparing the results of Tl-201 examinations with the clinical findings. Of these 33 patients, the therapeutic effects observed were as follows: complete remission 1, partial remission 8, progress of disease 1, and no remarkable change 23. Tl-201 scintigraphy has proved itself very useful not only in clinically detecting the malignant soft tissue sarcomas and in assessing therapeutic effects on these diseases, but also in assessing the follow-up patients with malignant soft tissue sarcomas.

Adolescent↗
PubMed1997

Neurosarcoma of the face: MRI.

Neurosarcoma is a rare tumour originating from the sheath of peripheral nerves. Facial lesions have been reported in about 20 patients. We describe the MRI appearances of neurosarcoma with histological correlation in three patients. The lesions lay in the submandibular region, the left parapharyngeal space and the right orbit. MRI showed a well-defined mass with mixed components. The lesions were moderately heterogeneous on T1-weighted images in two cases and on T2-weighted images in all cases. Gadolinium enhancement occurred in all cases to variable degrees. In two cases, small high signal foci were seen on T2-weighted sequences. MRI appearances of neurosarcoma are not specific.

Adolescent↗
PubMed2005

Inflammatory myofibroblastic tumor of the posterior mediastinum: an older adult case with anaplastic lymphoma kinase abnormalities determined using immunohistochemistry and fluorescence in situ hybridization.

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm that usually occurs in children and young adults. Anaplastic lymphoma kinase (ALK) abnormalities in IMT, determined using immunohistochemistry and/or molecular genetic studies, including fluorescence in situ hybridization (FISH), have almost been limited to children and young adults. In elderly cases of IMT, these ALK abnormalities are very rare. We report on a case of IMT arising in the posterior mediastinum of a 59-year-old Japanese man that showed ALK abnormalities determined using immunohistochemistry and FISH, suggesting the neoplastic nature of a subset of IMTs in older patients similar to those in younger ones and the presence of an additional mechanism(s) that allows them to start to grow late.

Anaplastic Lymphoma Kinase↗
PubMed2001

Malignant peripheral nerve sheath tumor of the cauda equina.

Only one case of malignant peripheral nerve sheath tumor (MPNST) affecting the cauda equina region has been reported earlier. A 32-year-old male with congenital multiple subcutaneous swellings presented with low back pain, progressive paraparesis and bladder-bowel dysfunction. Magnetic resonance imaging (MRI) demonstrated a heterogeneously enhancing intradural lesion at L2-L4. At operation, on opening the dura, multiple nodular, firm matted masses attached to the lumbosacral nerve roots were encountered. Peripheral lesions were partially excised. Histopathological exam revealed varied cellularity with necrosis and pleomorphic nuclei suggestive of MPNST. MRI features, pathophysiological characteristics and the literature are reviewed.

Adult↗