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At least 433 records · Page 24Linked to original sources

Broad phase II study of vindesine.

A broad phase II study of vindesine was conducted in 79 adults with advanced malignancies. Most patients had solid tumors, particularly adenocarcinomas (59%), and 67 patients had received prior chemotherapy which included vincristine in 24 patients and vinblastine in one. Depending on tolerance to previous therapy, patients received vindesine at doses of 3-4 mg/m2 administered iv at 2-week intervals. Objective tumor regressions were seen in eight of 36 patients (one complete response, one partial response [PR], and six minor responses) with colorectal cancer, one of four patients (PR) with squamous cell cancer of the esophagus, one of four patients (PR) with malignant melanoma, and one of two patients (PR) with lymphoma. Five of 11 responding patients had received vincristine previously. Toxic effects were manageable. The results of this study justify in-depth, disease-oriented phase II evaluations of vindesine, regardless of prior therapy with other vinca alkaloids.

Adolescent↗

Malignant melanoma at unusual sites.

The author deals with natural history an clinical appearance of malignant melanomas occurring at unusual sites. Diagnostic problems, treatment and prognosis are discussed on base of pertinent literary data and own experiences described in case reports of the related clientel (1 case with primary melanoma of the breast region; 1 case with melanoma of the tongue; 4 anorectal melanomas; 1 case with a neurotropic melanoma). Finally, the neurotropic variant of malignant melanoma is described, not for its unusual location but rather for its special histological picture and differential-diagnostic importance.

Adult↗

Diagnosis and management of brachial plexus lesions in cancer patients.

Brachial plexus dysfunction is a well-known complication of cancer. Metastatic brachial plexus (RBP) are the most common causes. The distinction between MBP and RBP is very important but is not easy to make. This article presents in detail the distinguishing features of these types of brachial dysfunction. In regard to treatment, radiation, chemotherapy, narcotic analgesics, paravertebral nerve blocks, dorsal rhizotomy, dorsal root entry zone procedure, and high contralateral cordotomy are helpful in managing the symptoms of MBP. Transdermal electrical nerve stimulation, dorsal column stimulators, neurolysis, and neurolysis with omentoplasty have been tried in RBP. Good physical therapy, tricyclics, antiarrhythmics, anti-convulsants, nonsteroidal anti-inflammatory drugs and steroids are helpful in both conditions.

Brachial Plexus↗

[Tumor of the peripheral nerve sheath with rhabdomyoblastic differentiation arising in a ganglioneuroma: neurilemmoma? A fortuitously detected case].

An otherwise healthy 21-year-old man with no evidence or family history of Von Recklinghausen's neurofibromatosis presented a posterior mediastinal mass detected on routine chest radiographs. The findings of standard light microscopy, ultrastructural examination and immunohistochemical studies suggested the working hypothesis of an unusual malignant "Triton" tumour: -arising in a pre-existing de novo ganglioneuroma; -fortuitously detected; -predominantly made of multinucleated spindle cells with only ultrastructural and immunohistochemical rhabdomyoblastic differentiation.

Adult↗

Risk of malignancy and death in neurofibromatosis.

OBJECTIVE AND METHODS: Neurofibromatoses are cancer-prone hamartomatoses that involve a variety of tissue and cell types. As part of a population-based clinical and genetic study of neurofibromatosis in northern Finland, all surgical and autopsy specimens of neurofibromatosis patients were retrieved and histologic slides were reviewed. RESULTS: Specimens were available for 69 of the 197 neurofibromatosis type 1 patients identified. Six malignant peripheral nerve sheath tumors and nine other malignant tumors were detected. In this study, the risk for neurofibromatosis-related malignancy was 8%. Nine neurofibromatosis type 1 patients died, at a mean age of 37 years. The cause of death was related to neurofibromatosis in eight. CONCLUSIONS: The risk of developing malignant tumors and early death is increased in patients with neurofibromatosis, the most common malignancy being malignant peripheral nerve sheath tumors. These risks need to be recognized, and the families should be advised to seek genetic counseling and proper follow-up.

Adolescent↗

[Tumors of the phrenic nerve].

A schwannoma of the phrenic nerve is a rare disorder which presents as a tumour of the anterior mediastinum. It is seen in adults and is usually latent. We report two cases in elderly subjects in whom the phrenic nerve tumour had achieved a significant size. One of these schwannomas had degenerated into sarcomatous change which is the first case reported to the present time.

Aged↗

Expression of Apo-3 and Apo-3L in primitive neuroectodermal tumours of the central and peripheral nervous system.

Deregulation of apoptosis has been implicated in the pathogenesis, spontaneous regression and treatment resistance of neuroblastoma. A newly recognised member of the tumour necrosis factor (TNF)-family of death receptors known as Apo-3 has been mapped to human chromosome 1p36.3, a region commonly deleted in aggressive neuroblastoma. Based on its localisation and function, Apo-3 is a candidate for the putative neuroblastoma tumour suppressor gene. Therefore we analysed mRNA expression of the Apo-3 receptor/ligand (Apo-3/Apo-3L) system in a representative panel of 18 neuroblastoma cell lines, 41 primary neuroblastoma and 13 ganglioneuromas/ganglioneuroblastomas by semi-quantitative RT-PCR. We compared the level of expression with the well-established prognostic factors age, stage, histology, MYCN-amplification and TrkA expression, as well as outcome. For comparison, we studied Apo-3/Apo-3L expression in 27 central nervous system (CNS) primitive neuroectodermal tumours/medulloblastomas (PNET/medulloblastoma) and in six normal brain samples. Neuroblastoma cell lines with 1p deletion and MYCN-amplification expressed significantly lower levels of Apo-3 (P=0.009 and P=0.03, respectively) compared with neuroblastoma cell lines without 1p deletion or MYCN-amplification. The mean expression level of Apo-3L was significantly higher in ganglioneuromas/ganglioneuroblastomas compared with neuroblastomas (P=0.001) and in normal brain compared with PNET/medulloblastoma (P<0.0001). Expression of Apo-3L was significantly associated with survival in neuroblastomas (P<0.049) and in PNET/medulloblastomas (P=0.01). Expression of Apo-3 was significantly associated with survival in PNET/medulloblastomas (P=0.03). Thus, the Apo-3 receptor/ligand system might be involved in the regulation of apoptosis in neuroblastomas and PNET.

Adolescent↗

Study of the post-natal effects of chemopreventive agents on ethylnitrosourea-induced transplacental carcinogenesis in rats. II. Influence of low-molecular-weight polypeptide factors from the thymus, pineal gland, bone marrow, anterior hypothalamus, brain cortex and brain white substance.

The influence of the polypeptide factors extracted from thymus, pineal gland, bone marrow, anterior hypothalamus, brain cortex or brain white substance on N-ethyl-N-nitrosourea (ENU)-induced transplacental carcinogenesis was studied in rats. ENU was given to pregnant rats as a single i.v. exposure at a dose of 75 mg/kg body weight on the 21st day of gestation. The polypeptide factors were given to the offspring as a series of s.c. injections, at a dose of 0.5 mg/rat/day, starting at one or 2.5 months of age and continuing throughout the whole of post-natal life. ENU induced tumors of the brain, spinal cord, peripheral nerves and kidneys in 94-98% of the offspring exposed to the carcinogen, with an average number of 2.3-2.6 tumors per rat, and an average survival time of 294 days. Post-natal thymus factor or pineal gland factor administration was followed by an increase in mean lifespan of approximately 2 months and a significant decrease (P < 0.05) in the total tumor number per tumor-bearing rat, as well as the incidence and multiplicity of spinal cord tumors. Pineal gland factor also decreased the incidence of peripheral nerve and kidney tumors and their number per tumor-bearing rat. Brain cortex factor and brain white substance factor treatment was followed by a decrease in total tumor multiplicity of 1.2- to 3.3-fold, and a decrease in incidence of brain tumors of 10 to 33% per rat in comparison to the controls. Brain cortex factor also decreased the total tumor incidence. At the same time, brain white substance factor administration increased the incidence of peripheral nerve tumors and decreased the mean lifespan. Both bone marrow factor and anterior hypothalamus factor did not have any modifying effects on any of the ENU-induced tumors and mean lifespan. Thus, our results show the possibility of attenuation of transplacental ENU-induced carcinogenesis with post-natal administration of some polypeptide substances.

Animals↗

A case of von Recklinghausen's disease with bilateral pheochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumors.

A 48-year-old man with neurofibromatosis type 1 presented with chest pain, paroxysmal hypertension, tachycardia, and progressive respiratory insufficiency. Clinical investigation displayed calcified tumors in the anterior mediastinum and pararenal region. Histological examination at autopsy revealed composite tumors consisting of pheochromocytoma and malignant peripheral nerve sheath tumor (MPNST) at two sites: the left adrenal gland and the region surrounding the inferior vena cava, probably corresponding to the right adrenal gland. The MPNST component showed a varied histological appearance, including hyalinized bands with polygonal cells, a cartilaginous and myxoid stroma, a hemangiopericytomatous architecture, and a fibrosarcomatous structure, which suggested osteosarcoma, chondrosarcoma, angiosarcoma, and fibrosarcoma, respectively. In addition, based on the ultrastructural findings, the gastrointestinal tract was involved with mesenchymal tumors showing neurogenic differentiation. These lesions suggest the divergent cellular differentiation of neural crest-derived cells to mesenchymal elements as well as neuroectodermal neoplasms.

Adrenal Gland Neoplasms↗

Malignant autonomic nerve tumor of the duodenum.

GAN tumors are rare and their diagnosis and distinction from other gastrointestinal stromal tumors is based on electron microscopy findings. Although no specific distinctive diagnostic imaging features are seen, this diagnosis should be considered when a large solid mass adjacent to a bowel loop shows the presence of air.

Autonomic Nervous System Diseases↗

A patient with a VEGF and endostatin producing gastrointestinal autonomic nerve tumour.

Tumour associated neovascularisation has been characterised as chaotic and insufficient. This report details the results of the analysis of angiogenic factors in tumour cyst fluid, pleural fluid, and blood from a patient with a gastrointestinal autonomic nerve tumour. The tumour produced vascular endothelial growth factor and endostatin in large quantities, which may explain the dysfunctional angiogenesis and tendency to bleeding seen in this tumour type.

Adult↗

[Gastrointestinal autonomic nerve tumor].

Gastrointestinal autonomic nerve tumors (GAN-tumor) are rare malignant neurogenic stromal tumors of the intestinal tract. The origin is suspected in the autonomic nerve plexus Meissner or Auerbach with the interstitial cells of Cajal as precursors. We report on a 53-year-old patient with a clinical apparent and radiological 5 cm measuring tumor of the jejunum, which was resected and immunohistochemically verified as GAN-tumor. Within the follow-up of 29 months metastases appeared within the omentum majus with a diffuse peritoneal spreading. Several trials of adjuvant chemotherapy (adriamycine/ifosamide, taxotere, gemcitabine/xyloda) were ineffective. 15 months after the second operation the patient died. Since the first description of the GAN-tumor in 1984 87 patients were reported in the literature. No recurrences or metastasis were seen in tumors with a seize less than 5 cm. A tumor seize of more than 10 cm is associated with recurrences in 64% of the cases within 2 years. Since there is no option for medical treatment, surgical resection is the treatment of choice and has to be considered also in the case of recurrence.

Autonomic Nervous System↗