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PubMed1986

[BAEPs of children with malignant tumor undergoing cisplatin treatment].

The effect of cisplatin on the auditory nervous system was examined in five children using brainstem auditory evoked potentials (BAEPs). The cases comprized of two neuroblastoma, one yolk sac tumor one neurofibrosarcoma and one Hodgkin's disease. All patients except for one infant aged eight months showed a normal audiogram in the pre-treatment examination. Cisplatin was administered at a dose of 75-105 mg/m2 (body surface area). BAEP tests were performed after cisplatin treatment from two weeks to 20 months. The patients were tested at rest in bed using stimulation with a 3 KHz, 80-dBHL click at 150-msec intervals 2,000 times. Four cases showed an abnormal BAEP pattern in the post-treatment examination. Two of them showed not only delayed conduction velocity of the first wave but also auditory disturbance, but these findings were improved after discontinuation of drug administration. We concluded that cisplatin frequently affects the auditory nervous system, and that this disturbance might be transient in the early stage.

Adolescent↗

PubMedDate not supplied

Neurofibromatosis of lid and orbit in early childhood.

Fifty cases of neurofibromatosis of lid and orbit that were followed up from early childhood for about 20 years showed: 1. Age at which cases firstly seen by ophthalmologist in 10 percent at first, in 20 percent between two and four; in 30 percent betweeen five and 12; in 30 percent between 13 and 17; and in 10 percent after 17 years. 2. The most common lesion in a unilateral orbital and upper lid swelling with ptosis in 30 percent; with added swelling of temporal region and eyebrow in 30 percent; with added swelling of cheek in 30 percent. Only unilateral swelling at root of nose in four percent; eyebrow in two percent; or temporal atrophy in two percent. 3. Associated cafe au lait patches are found in 70 percent, and multiple neurofibromata of skin in 50 percent. 4. Malignant transformation to neurofibrosarcoma occurs in six percent. 5. X-ray changes of the bony orbit: absent in 60 percent; congenital since birth in 20 percent-the lesser and greater wings of the spehenoid bone are most commonly absent; and due to pressure of growing neurofibromatosis of orbit in 20 percent.

Adolescent↗

PubMedDate not supplied

Immunochemical demonstration of keratin and vimentin in cytologic aspirates.

Antibodies to intermediate filament proteins were used to characterize tumor cells present in peritoneal and pleural effusions and in thin needle aspirates from palpable lymph nodes. Metastatic adenocarcinoma cells (breast, ovary, endometrium, cervix, colon and stomach) as well as squamous-cell carcinomas and mesotheliomas stained specifically with antibodies to keratin while mesenchymally derived tumor cells (lymphomas, melanoma, fibrosarcoma and neurofibrosarcoma) were positive only for vimentin. Especially in cases of lymph node aspirates, keratin staining in cells was a direct indication of metastatic carcinoma. Antibodies to these different components of the cytoskeleton can thus be used in cytopathologic diagnosis when a definitive diagnosis cannot be made on the basis of conventional cytologic features.

Ascitic Fluid↗

PubMed1984

Chromosome changes in soft tissue sarcomas.

An analysis of chromosome aberrations in human tumors was performed in 29 cases of soft tissue sarcoma. The tumor tissues were disaggregated with collagenase and the cells cultured for 2-3 days. Analyzable metaphases were obtained in 15 cases, 4 of which showed only normal karyotypes. The remaining 11 tumors showed various numerical and structural abnormalities in their karyotypes: 8 tumors were near-diploid and the remaining 3 were near-triploid. G- and Q-banding analyses revealed clonal abnormalities in the 11 cases with the presence of marker chromosomes; 15 different chromosomes were involved in chromosome rearrangements, chromosomes 1 and 2 being the most frequently affected. Because of the heterogeneity of the tumor group investigated (neurogenic sarcoma, 2 liposarcomas, neurofibrosarcoma, synovial cell sarcoma, fibrosarcoma, mesothelioma, leiomyosarcoma, rhabdomyosarcoma, Ewing's sarcoma, and hemangiopericytoma), it was impossible to reach any conclusion on the specificity of the cytogenetic abnormalities for a particular tumor type.

Adult↗

PubMed1983

[Tumors of the central nervous system in biopsy and autopsy material. 7th communication: neurinomas and neurofibromatoses with CNS involvement].

In 1,491 autopsy cases with CNS tumors observed at the Pathological Institute of the Medical Academy of Erfurt in the period from 1953 to 1976 (54,946 autopsies) 72 cases (4.8%) with neurinomas were found. They comprise 67 solitary neurinomas, 1 bilateral acoustic neurinoma without other signs of neurofibromatosis, and 4 cases of neurofibromatosis with neurinomas of the CNS. Among the 68 cases with CNS neurinomas (neurofibromatoses excluded) 87% were acoustic neurinomas, 12% spinal tumors, and 1 case was located in the trigeminal nerve. In 60 (88%) of these 68 cases, the neurinoma was operated upon or clinically diagnosed, resp. The diameter of 18 (26%) neurinomas of the autopsy material was larger than 5 cm. Patients in the 6th decennium predominated in this series. The sex distribution revealed a preponderance of females over males (3:1). In 3 cases further CNS tumors (ependymoma, glioblastoma, meningioma) were found. Additionally, 3 cases had carcinomas of different localization (Table 5). Following tumors were seen in 9 cases of Morbus Recklinghausen with CNS involvement: 4 cases with multiple neurinomas, 3 meningiomas, 1 astrocytoma, 2 glioses and 1 angiomatous malformation (Table 6). Among 1,670 CNS tumors in biopsy material, 144 (8.6%) were neurinomas. 60% of them were located in the nervus acusticus, 40% spinally, mainly in the thoracic region. The 6th decennium was most affected, and females were more frequent than males (2:1) in our material. Nearly all CNS neurinomas were benign. Only 1 spinal tumors was classified as a malignant neurinoma. 2 of the 9 cases with Morbus Recklinghausen had malignant neurogenic tumors (neurofibrosarcomas).

Adolescent↗

PubMed1983

Neurogenic tumors of the sciatic nerve. A clinicopathologic study of 35 cases.

Thirty-five cases of neurogenic sciatic nerve tumors are described. Pain is the major clinical manifestation but may give rise to diagnostic confusion because of its often prominent site far distant from the neoplasm. Nonpalpable tumors are best identified by computed tomographic scan of the pelvis and thighs. Early surgical therapy is advisable. Neurilemomas are treated by simple enucleation with preservation of the sciatic nerve. They rarely recur. For neurofibromas, total excision is often not possible. As long as they are small and neurologically nondestructive, subtotal excision with preservation of the nerve trunk is recommended. When they are large or have caused marked neurologic dysfunction, radical tumor and nerve resection is required. In the usually highly malignant neurofibrosarcomas, disarticulation of the hip joint or hindquarter amputation affords the best chance for cure and should not be delayed once the diagnosis has been established.

Adolescent↗

PubMed1981

Soft tissue sarcomas (excluding rhabdomyosarcoma): radiotherapy applications.

Radiation therapy (RT) is a highly effective modality in the treatment of sarcomas of soft tissue when combined with conservative surgery. RT followed by surgery is judged to have important clinical advantages over the reverse sequence. Of special significance to the treatment of the pediatric patient is the observation that the treatment volume in preoperative RT is nearly always smaller than in postoperative RT. Precise definition of the target volume and utilization of minimum treatment volumes (i.e., reduction of the treatment volume toward the target volume to the greatest extent technically feasible) are particularly important to the improvement of functional results. Data are described for 21 patients who were 21 years old or younger and were treated by RT and surgery and RT alone and who have been followed for 3 or more years. Local control of the treated tumor was achieved in all 21. Absolute figures for disease-free survival were 5 of 5 and 10 of 16 patients in the 6- to 14- and 15- to 21-year age groups, respectively. Survival results according to stages for the latter group were as follows: stage I, 4 of 4; stage II, 4 of 7; and stage III, 2 of 5. All 9 patients with fibrosarcoma or neurofibrosarcoma survived free of disease. The need for an effective chemotherapy regimen for patients in stages IIB--III is emphasized.

Adolescent↗

PubMed1980

Cyclophosphamide, vincristine, adriamycin, and DTIC (CYVADIC) combination chemotherapy for the treatment of advanced sarcomas.

One hundred and forty adult patients with advanced sarcomas (125 soft tissue and 15 bone) were treated with a combination chemotherapy regimen consisting of cyclophosphamide, vincristine, Adriamycin, and DTIC (CYVADIC). There were 21 (15%) complete and 45 (32%) partial responders, with an overall response rate of 47%. The response rate was 50% (17% complete) for patients with soft tissue sarcomas compared with 20% (none complete) for patients with bone sarcomas. The median duration of response was 9.5 months (range, 1-40+ months) for complete responders and 7 months (range, 1-31 months) for partial responders. The median time to achieve response was 9 weeks and the median number of courses of therapy to response was three. The median survival time was 16 months for responders compared with 7 months for nonresponders (P = 0.001). The most responsive tumor types were neurofibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, fibrosarcoma, and angiosarcoma. Pulmonary and soft tissue metastases were more responsive than bone and liver metastases. CYVADIC toxicity was predominantly limited to myelosuppression, vomiting, fever of unknown origin, and neuropathy. CYVADIC is an effective combination chemotherapy regimen in the treatment of advanced sarcomas.

Antineoplastic Agents↗

PubMed1995

Thallium-201 scintigraphy in pediatric soft-tissue tumors.

UNLABELLED: Thallium-201 has been used as a tumor imaging agent in adults with lymphoma, brain tumors, thyroid carcinoma and bone sarcomas. The application of 201Tl in pediatric oncology has been very limited. METHODS: Whole-body 201Tl studies were incorporated into the imaging protocols of 45 children with solid soft-tissue tumors. Bone and 67Ga scans as well as 131I-MIBG scans for patients with neuroblastoma were obtained. RESULTS: Seventeen children with lymphoma, five with Hodgkin's disease and twelve with non-Hodgkin's lymphoma (NHL) were studied. All of the patients with Hodgkin's disease showed avid uptake of 201Tl and 67Ga. The six patients with B-cell NHL had positive 67Ga scans and four had positive 201Tl scans. There was variable uptake in T-cell and undifferentiated lymphoma with an overall sensitivity of 69% for 201Tl and 85% for 67Ga. In 14 patients with rhabdomyosarcoma, 10 (71%) had positive 201Tl scans and 11 (79%) had positive 67Ga images. Gallium and thallium scans detected lesions equally. There was poor uptake of 201Tl in the six children with neuroblastoma. Primary tumor and metastases were detected more readily with the bone and MIBG scans. Three children with Wilms' tumor had poor or no uptake on 201Tl images, two of three patients with histocytosis had no significant uptake and one patient had mild uptake of 201Tl. One patient with primitive neuroectodermal tumor had positive 201Tl and 67Ga images and one patient with a neurofibrosarcoma had negative 201Tl and 67Ga images. There was no correlation with histological types of tumor or disease staging. CONCLUSION: This study shows significant uptake of 201Tl in many pediatric solid soft-tissue tumors. Additional study is needed to determine 201Tl effects on treatment response and tumor viability. Also, more studies are needed to assess 201Tl delayed images to determine if there is more tracer concentration in the tumors.

3-Iodobenzylguanidine↗

PubMed1994

Genetic basis of neurological tumours.

Neurological tumours are common neoplasms of both adults and children. Recent studies have begun to delineate the genetic abnormalities that underlie such tumours, and have implicated two classes of genes, oncogenes and tumour suppressor genes. Most investigations have focused on those astrocytomas that affect the cerebral hemispheres of adults, since these are the most common and malignant brain tumours. The high-grade astrocytomas that affect adults, such as glioblastoma multiforme, often have amplification of the epidermal growth factor receptor (EGFR) oncogene and loss of a variety of chromosomal loci that probably harbour tumour suppressor genes. Of the various tumour suppressor gene loci, the p53 gene on chromosome 17p has been studied most closely and has been shown to be mutated in both low- and high-grade astrocytomas. These genetic alterations may provide a means for subdividing astrocytomas into diagnostic categories. For instance, p53 gene mutations occur more commonly in glioblastomas from young adults and women, while EGFR gene amplification is more common in glioblastomas from older adults and men. For the other primary CNS tumours, genetic studies remain in their infancy. The neurocutaneous syndromes, such as neurofibromatosis types 1 and 2, have provided unique insights into neurological oncogenesis. The NF1 gene on chromosomes 17q and its product, neurofibromin, may be important in the formation of neurofibrosarcomas, while the NF2 gene on chromosome 22q and its product, merlin, are probably involved in the formation of schwannomas and other nervous system tumours. The further characterization of these and other neurological tumour genes will undoubtedly illuminate many other areas in neurooncology.

Genes, Tumor Suppressor↗

PubMed1994

Characterization of naturally occurring cutaneous neurofibromatosis in Holstein cattle. A disorder resembling neurofibromatosis type 1 in humans.

Neurofibromatosis in cattle is typically a noncutaneous disease. A small group of cows in a Holstein dairy herd developed cutaneous neurofibromatosis. This unique condition was investigated and compared with neurofibromatosis type 1 (NF1) in humans. All cutaneous lesions but one were consistent with neurofibromas in noncutaneous sites in cattle and neurofibromas in patients with NF1. One bovine lesion was classified as a neurofibrosarcoma. Immunohistochemistry and electron microscopy supported Schwannian differentiation in benign and malignant lesions. Linkage analysis with a polymorphism in the bovine NF1 gene confirmed that two affected animals from the same sire inherited the same paternal NF1 allele. Bovine cutaneous neurofibromatosis is a naturally occurring disease in this group of animals, characterized by skin tumors morphologically identical to those of NF1. An informative polymorphism at the NF1 locus of two animals and their sire suggests this disorder may be caused by hereditary mutations at the bovine NF1 locus.

Animals↗

PubMed1994

Distribution of type IV collagen in pancreatic adenocarcinoma and chronic pancreatitis.

Changes in the basement membrane are present in various neoplastic conditions such as neurofibrosarcoma, cervical carcinoma, colorectal cancers and hepatoblastoma. This study examines the expression of type IV collagen in the basement membrane, using an immunohistochemical method, in the normal pancreas (n = 10), chronic pancreatitis (n = 15) and pancreatic adenocarcinoma (n = 30). The formalin fixed, paraffin embedded tissue was sectioned and pretreated with protease prior to immunostaining for type IV collagen. There was a statistically significant difference in type IV collagen expression between pancreatic carcinoma and chronic pancreatitis (P = 0.0001; chi 2 test with continuity correction). In pancreatic adenocarcinoma, type IV collagen distribution in the basement membrane was discontinuous and irregular or absent around individual or groups of neoplastic cells (n = 30). Most cases of chronic pancreatitis showed continuous pattern of basement membrane type IV collagen around residual ducts (n = 10). In the normal pancreas, only one of the ten cases showed discontinuous basement membrane around pancreatic ducts, while in the rest of the cases, the pattern was continuous. This study suggests that there is abnormal distribution of type IV collagen in the basement membrane in pancreatic carcinoma, which may be related to either abnormal deposition or degradation of the collagen. Immunostaining for type IV collagen may be of some diagnostic use for distinguishing pancreatic adenocarcinoma from problematic cases of chronic pancreatitis.

Adenocarcinoma↗

PubMed1993

[Sarcomatous degeneration of a giant scrotal lipoma. Presentation of a clinical case].

This paper presents a case of giant scrotal lipoma with areas of sarcomatous degeneration which were evident on anatomico-pathological examination; the authors considered it worth-while reporting this case given that giant lipomas with sarcomatous degenerations are rare. It is important to underline that in comparison to other malignant tumours of the scrotum the frequency of liposarcomas is very low. In addition to the liposarcoma reported here, at the level of the scrotum it is worth recalling neurofibrosarcoma and fibrosarcoma and some rare sarcomas of the spermatic cord among the malignant sarcomas of sarcomatous origin reported in the literature. Difficulties may arise from a diagnostic point of view due to the scarcity of symptoms. Among the most frequent symptoms is the presence of a hard mass on palpation of the scrotum; the volume of this mass may increase very rapidly. It is important to note that this tumour may sometimes originate as malignant and then degenerate in toto or in some isolated areas. Instrumental tests include abdominal radiography without the use of contrast medium in order to exclude the presence of herniated abdominal viscera at the level of the scrotum. Ultrasonography may also be extremely useful since it is capable of providing information regarding the presence, inside the scrotal sack, of liquid retention or solid forms. At all events histological diagnosis is carried out in the majority of cases following the removal of the operated part. The exeresis of the tumour therefore becomes both diagnostic and therapeutic. Surgery consists in the simple exeresis of the neoplasia; in some cases orchiectomy is required in addition to exeresis. Some authors also recommend the dissection of inguinal lymph nodes following extemporary anatomo-pathological tests. In other cases when histological tests confirm the malignant degeneration with a high degree of biological aggressiveness, the patient has to undergo postoperative radiotherapy and/or chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1977

B-scan ultrasound in the diagnosis of popliteal aneurysms.

Sonography gives a characteristic picture of popliteal aneurysms, with an easily demonstrable connection of the sonolucent aneurysmal sac with the popliteal artery. It is a good screening procedure for suspected aneurysms and for patients with popliteal pain, mass, or sudden distal ischemia. Three case reports of five popliteal aneurysms diagnosed by ultrasound are presented. A case of neurofibrosarcoma occurring in the popliteal fossa is shown for comparison.

Aneurysm↗

PubMed1996

[Natural evolution of neurocutaneous syndrome in adults].

Neurofibromatoses (NF1 and NF2) are genetic diseases with an extremely wide range of manifestations, particularly NF1. In order to gain insight into their prognosis, we have conducted a follow-up study of 100 patients with neurofibromatoses, 89 NF1 patients and 11 NF2 patients all of them presenting neurological manifestations. Four deaths occurred in the NF1 group (3 neurofibrosarcomas) and two in the NF2 group (after neurosurgery). We describe a series of NF1 brainstem tumors as being pilocytic astrocytomas, much less aggressive than non-NF1 brainstem tumors but more symptomatic than brainstem UBOs ('unidentified bright objects') in NF1. The series of optic nerve tumors also demonstrate the poor and non-progressive evolution of tumors with the same histopatological estructure. Hydrocephalus related to aqueductal stenosis is a frequent and tratable manifestation in NF1 patients. Data are presented which suggest that a severe form of NF2 is much more frequent than a mild form but it is rule that number and progressivity of tumors render the prognosis always severe in NF2 patients. A long term follow-up study has been carried among 20 patients with tuberous sclerosis: patients who presented seizures before the age of 5 years have mental retardation in spite of disappearing, often spontaneously, the seizures. The initial symptoms in Von Hippel-Lindau patients depend often on the hemangioblastoma of the spinal cord and a strict control is proposed for patients with risk of suffering spinal-cerebello-retino hemangioblastomatosis. Other phakomatoses are also being considered in their general prognosis.

Adolescent↗

PubMed1996

Argyrophilic nucleolar organizer regions in soft tissue tumors.

Argyrophilic nucleolar organizer region (AgNOR) staining was employed on 51 apparently normal representative soft tissues, 53 benign soft tissues tumors and 52 malignant soft tissue tumors with an aim to study the sensitivity and specificity of method in differentiating between the benign and malignant soft tissue tumors. The mean AgNORs count in apparently normal fibrous tissue was 1.02, whereas it was 0.94 in adipose tissue, 1.14 in smooth muscle tissue, 1.115 in skeletal muscle tissue, 1.025 in blood vessels endothelial lining cells and 1.04 in nerve tissue. The mean AgNOR count was found to be higher in benign soft tissue tumors as compared to respective apparently normal soft tissue and was found to be statistically significant. The mean AgNOR count in soft tissue sarcomas was found to be higher as compared to both apparently normal soft tissue and benign soft tissue tumors. An increase AgNOR score in both benign and malignant soft tissue tumors as compared to apparently normal soft tissue indicates high proliferative activity. The neurofibrosarcoma showed low AgNOR count as compared to other soft tissues sarcomas. The fibrohistiocytic sarcoma, leiomyosarcoma and angiosarcoma showed a mean AgNOR score of 4 or more than four. The mean AgNOR score was found to increase with high grade of the tumor. The AgNOR staining is simple and useful method in estimating tumor cell proliferation thereby differentiating normal soft tissue from non-neoplastic proliferative growth, benign and malignant soft tissue tumors. It may help in differentiating fibromatosis from fibrosarcoma, dermatofibrosarcoma protuberans of low grade malignancy from high grade malignant fibrous histiocytoma and benign hemangiopericytoma from malignant hemangiopericytoma.

Adult↗

PubMed1997

The role of magnetic resonance imaging. When to use it and what to look for.

There are no specific MRI features which are diagnostic in bone and soft tissue sarcomas, but a combination of certain findings allows an accurate diagnosis in some cases including giant cell tumour, chondrosarcoma, liposarcoma, neurofibrosarcoma, aggressive fibromatosis and pigmented villonodular synovitis. MRI is the method of choice for staging bone and soft tissue sarcomas. It is of particular use for identifying satellite nodules and skip lesions within the same bone or anatomical compartment. CT scan is important for screening for pulmonary metastases, and bone scan remains useful for screening for distant skeletal disease. The biopsy should be planned and performed after any MRI examination. MRI is the most sensitive post-therapy evaluation for local recurrence of bone and soft tissue sarcoma.

Bone Neoplasms↗

PubMed1996

Neurocristopathy resembling neurofibromatosis type 1 in an NGF-SV40 transgenic line.

BACKGROUND: Animal models of carcinogenesis have been produced in transgenic mice by directing the expression of oncogenes such as SV40 T antigen and myc to different tissues by creating fusions with promoter/enhancer elements of various mammalian or viral genes. RESULTS: A transgenic mouse line was created in which SV40 T antigen is under the control of the mouse nerve growth factor (NGF) promoter. While the oncogene is expressed in a wide range of NGF producing tissues, it specifically causes the development of either neurofibromas or neurofibrosarcomas similar to those found in the human disease neurofibromatosis type 1 (NF1). These tumours are completely penetrant and appear after a mean latency of about 8 months. In contrast to the previously reported neurofibromatosis mouse model HTLV-1 tax, the tumours in these transgenic mice arise in Schwann cells rather than perineural fibroblasts and have a very restricted tissue distribution. In a cell line cloned from a neurofibroma from these mice, NGF was detected in the culture medium at levels similar to those produced by cultured primary Schwann cells. CONCLUSION: As all animal model for a heritable neurocristopathy resembling NF1, this mouse should allow study of the pathology and treatment of this disease.

Animals↗