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PubMed1994

Subcutaneous sarcomas of probable neuronal origin in a transgenic mouse strain containing an albumin promoter-fused simian virus 40 large T antigen gene.

Frequent development of subcutaneous neurogenic sarcomas was observed in a hepatocellular carcinoma-producing transgenic mouse strain harboring an albumin-promoted simian virus 40 (SV40) large T antigen gene. Found unexpectedly in 19 out of 306 mice (6.2%) by 6 months of age, all the sarcomas were similar and were characterized as neurogenic on the basis of histological features including Homer-Wright type rosette formation, the presence of dense core granules of 100-200 nm diameter under the electron microscope, expression of neuron specific enolase, S-100 protein, and catecholamines, and nerve cell-like differentiation in culture in response to But2cAMP. Immunohistochemical study revealed tiny clusters of SV40 T antigen-expressing cells with neurogenic character in normal-appearing adult mouse subcutis as candidate progenitors of the sarcomas. The tumor cells strongly expressed large T antigen but did not express albumin or albumin mRNA at the detection sensitivity used. Transient transfection assay (CAT assay), however, revealed the presence of transcriptional factor(s) acting on the albumin promoter in tumor cells. Thus, the present investigation suggested the presence of specifically differentiated neurogenic cells in the mouse subcutis with aberrant expression of the transgene.

Albumins↗
PubMedDate not supplied

Diagnostic and clinical outcome of neurogenic tumours in the head and neck area.

A retrospective analysis of diagnostic procedures and clinical outcome of patients with neurogenic tumours of the head and neck region was performed. There were 25 patients - 16 with neurinoma, 5 with neurofibroma and 4 with neurogenic sarcoma - who presented with a mass in the head and neck area. In 13 patients, the neurinoma originated from the facial nerve, 2 of them were located in the middle ear and mastoid and showed facial palsy. Four patients presented with neurofibromatosis type 1. Twenty-three patients were operated on to remove the tumorous masses. Eighteen tumours were assigned intraoperatively to a cranial nerve main trunk, whereas 7 tumours originated from small branches or showed diffuse growth in neurofibromatosis. All benign neurogenic tumours were removed totally without relapse. One of 2 auricular nerve autografts in middle-ear facial nerve neurinoma resulted in a sufficient muscle tonus of the face. Three patients with malignancies died within 12-24 months showing local recurrencies (n = 1) and/or pulmonary metastasis (n = 2). As expected, neurogenic tumours are unusual neoplasms of the head and neck regions. On the other hand, neurogenic malignancies exhibit diagnostic problems as well as uncertain chances for long-term survival. Tumour staging, determined by extension and critical for treatment decisions, is well evaluated by CT scan and MRI. A multimodal therapy regimen with operation and radiation is recommended for neurogenic sarcoma also when developing in neurofibromatosis. However, in main trunk neurinomas, nerve autografting with interposition is the therapy of choice with a good chance of functional recuperation.

Combined Modality Therapy↗
PubMedDate not supplied

Rectal neurogenic sarcoma: case report and review of the literature.

A neurogenic sarcoma without NF-1 was discovered in a 73-year-old woman in the anorectal region, an unusual site for these tumors. The tumor was of high-grade malignancy and deeply located with mesorectal infiltration; it did not originate from a major nerve. We presume an origin from less differentiated neural crest cells and present a review of the literature on the best treatment for these neoplasms.

Aged↗
PubMed2026

Personalized medicine strategy for MPNSTs: using precision oncology on PDOX models to inform tumor boards.

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are a heterogeneous group of aggressive soft tissue sarcomas with poor prognosis. Currently there is a lack of effective treatments for MPNSTs. Here, we propose a personalized medicine approach that integrates a precision oncology strategy guided by MPNST genomic analysis, with a functional validation of treatment response in an orthotopic xenograft model (PDOX) derived from the same MPNST. METHODS: Comprehensive whole genome sequencing analysis was performed in primary MPNSTs, relapses and (in one case) metastases, following disease progression in two independent individuals. Matched MPNST PDOX models were generated by orthotopically implanting tumor fragments near the sciatic nerve of immunodeficient mice. Candidate targeted combination therapies were prioritized based on genomic alterations and tested in vivo in the PDOX models. RESULTS: The feasibility of the developed strategy is illustrated for two MPNST patients, one Neurofibromatosis type 1 (NF1) individual that developed two independent MPNSTs and another sporadic MPNST case with multiple metastatic relapses. Genomic analysis revealed a remarkable degree of genomic stability across primary MPNSTs and their successive relapses in each patient, and even metastases in one individual. While based on a small number of cases requiring additional analyses, this finding aligns with previous evidence suggesting a fair genomic conservation throughout tumor evolution. This stability supports the identification of consistent therapeutic vulnerabilities throughout disease progression. Among the therapies tested, co-treatment of MEK inhibitor (MEKi) plus bromodomain inhibitor (BETi) elicited the highest antitumor activity, resulting in approximately 60% tumor volume reduction in the sporadic MPNST PDX model, whose patient has been receiving this therapy for eight months with sustained remission. CONCLUSIONS: This study demonstrates the feasibility and clinical utility of integrating genomic-driven precision oncology with PDOX-based functional testing for MPNSTs. This strategy may support molecular tumor boards (MTBs) in their treatment decisions. The observed genomic stability supports the use of longitudinal tumor profiling to guide treatment, and the success of MEKi+BETi highlights its potential as a combination therapy for MPNSTs.

Precision Medicine↗
PubMed2000

Cardiac tumor biopsy under the guidance of intracardiac echocardiography.

Transthoracic echocardiography or transesophageal echocardiography is sometimes useful in intracardiac tumor biopsy. Intracardiac echocardiography was used as an alternative to either of these for performing a biopsy of a right cardiac tumor in a 79-year-old woman. The procedure was well tolerated and no complications occurred. Histopathological findings and immunohistological staining were compatible with the diagnosis of neurogenic sarcoma.

Aged↗
PubMed2001

Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.

A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.

Adult↗
PubMedDate not supplied

Neurosarcoma duodeni.

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Duodenal Neoplasms↗
PubMed2005

A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center.

OBJECT: This is a retrospective review of 397 benign and malignant peripheral neural sheath tumors (PNSTs) that were surgically treated between 1969 and 1999 at the Louisiana State University Health Sciences Center (LSUHSC). The surgical techniques and adjunctive treatments are presented, the tumors are classified with respect to type and prevalence at each neuroanatomical location, and the management of malignant PNSTs is reviewed. METHODS: There were 361 benign PNSTs (91%). One hundred forty-one benign lesions were brachial plexus tumors: 54 schwannomas (38%) and 87 neurofibromas (62%), of which 55 (63%) were solitary neurofibromas and 32 (37%) were neurofibromatosis Type 1 (NF1)-associated neurofibromas. Among the brachial plexus lesions supraclavicular tumors predominated with 37 (69%) of 54 schwannomas; 34 (62%) of 55 solitary neurofibromas; and 19 (59%) of 32 NF1-associated neurofibromas. One hundred ten upper-extremity benign PNSTs consisted of 32 schwannomas (29%) and 78 neurofibromas (71%), of which 45 (58%) were sporadic neurofibromas and 33 (42%) were NF1-associated neurofibromas. Twenty-five benign PNSTs were removed from the pelvic plexus. Lower-extremity PNSTs included 32 schwannomas (38%) and 53 neurofibromas (62%), of which 31 were solitary neurofibromas and 22 were NF1-associated neurofibromas. There were 36 malignant PNSTs: 28 neurogenic sarcomas and eight other sarcomas (fibro-, spindle cell, synovial, and perineurial sarcomas). CONCLUSIONS: The majority of tumors were benign PNSTs from the brachial plexus region. Most of the benign PNSTs in all locations were neurofibromas, with sporadic neurofibromas predominating. Similar numbers of schwannomas were found in the upper and lower extremities, whereas neurofibromas were more prevalent in the upper extremities. Despite aggressive limb-ablation or limb-sparing surgery plus adjunctive therapy, malignant PNSTs continue to be associated with high morbidity and mortality rates.

Academic Medical Centers↗
PubMed1997

Intraspinal tumours in children--clinical presentation.

Intraspinal tumours in children are rare, the estimated average ratio of spinal to intracranial tumour in the paediatric population is 1:10. We reviewed our experience of paediatric spinal tumours over the period 1992-96. Nineteen patients presented during this time, 12 males and 7 females with the mean age of 7.8 years. The main presenting symptoms were pain, limb weakness, ataxia, sensory disturbance and spinal curvature abnormalities with a mean duration of 10 months. There was a wide variety of tumour types. All underwent a laminectomy with 8 having total tumour excision, 5 partial excision, and 6 had biopsies only. Five patients had extradural lesions and fourteen were intra-dural, four of which were extramedullary and six were intramedullary tumours. There were no major complications of surgery and only one patient had a CSF leak which was repaired. The average hospital stay was 15 days. Seven patients underwent radiotherapy and four had chemotherapy. Four patients are disease free and seven are symptom free after a mean follow-up of 2 years. Four patients died in this series with extensive diffuse tumours.

Adolescent↗
PubMed1999

[Neurosarcoma of the submandibular gland arising in a pleomorphic adenoma. Apropos of a case].

A 71-year-old woman consulted for a tumor of the right submandibular gland that had been present for 4 years and had rapidly increased in size during the last two months. Histological examination of frozen sections and immunohistochemistry concluded to a poor differentiated sarcoma probably a neurosarcoma with residual pleomorphic adenoma component. In spite of wide resection, local recurrences with nodes and lung metastases occurred leading to the death of the patient. We think interesting to report this unusual salivary tumor and to discuss its pathological features.

Adenoma, Pleomorphic↗
PubMed2000

[A case of maxillary neurosarcoma].

A case of neurosarcoma located in maxillary sinus is presented. The patient was 62 years old women who complained of nasal obstruction, epistaxis and headache. Results of physical examination showed a giant mass filling the nasal cavity and maxillary sinus. The lesion was completely resected by lateral rhinotomy way. After the operation histopathological diagnosis was difficult, one pathologist recognised schwannoma benignum other one sarcoma. Finally postoperative diagnosis was schwannoma benignum, it was misdiagnosis. Two years later the patient was surgically treated again because of tumor recurrence. These time pathological diagnosis was neurosarcoma. Radiotherapy was done as well. The patient died 18 months after treatment, tumor regrowth and generalization metastases was reason of the death.

Diagnosis, Differential↗
PubMedDate not supplied

Case report of schwannoma of the rectum--clinical and pathological contribution.

The case of benign schwannoma of the rectum primarily misdiagnosed as myogenic (neurogenic?) sarcoma is presented. A large tumor of 8 cm in diameter of the anterior rectal wall was removed with wide margins and an artificial anus was constructed. During 12 years of follow-up neither local recurrence nor distant metastases were observed. The patient is still alive and free of the disease. For that reason a surgery specimen of the tumor was pathologically reanalyzed and it showed features of type Antoni A and B tissues. These findings, together with strong reactivity for S-100 protein, Vimentin and negative for Actin supported the diagnosis of benign schwannoma. Because the localization of the tumor in the rectum is extremely rare, clinical and pathological features are presented and discussed.

Actins↗
PubMed2001

Paradoxical vestibular disease with trigeminal nerve-sheath tumor in a dog.

A thirteen-year old spayed female poodle was referred because of atrophy of temporal and masseter muscles on the left and head tilt and episodical circling to the right side. Additionally, decreased facial sensation, absent menace reaction, palpebral and corneal reflexes on the left side, as well as ipsilateral hemiparesis and tongue palsy were noticed. Generalised vestibular ataxia and hypermetria in the front limbs were present. Based on the clinical signs, the presumptive anatomical localization of the lesion was the cerebellopontine angle including parts of the caudal brainstem with involvement of the trigeminal, facial and hypoglossal nerves. Involvement of either flocculonodular lobe or the caudal cerebellar peduncle on the left side causing paradoxical vestibular disease was suspected. On magnetic resonance imaging a large enhancing lesion in the area of the left cerebellopontine angle involving the trigeminal nerve and compressing cerebellum and brainstem was seen. Because of the poor prognosis the dog was euthanized on the owner's request. This space occupying lesion could be identified as a trigeminal neurofibrosarcoma/schwannoma on post mortem histopathological examination.

Animals↗
PubMed2002

Sciatic nerve resection: is that truly an indication for amputation?

En bloc resection of the sciatic nerve with an adjacent bone or soft tissue tumor has been assumed to be associated with a poor functional outcome and, therefore, was considered an indication for amputation. Although many surgical oncologists today challenge this assumption and do limb-sparing resection in these patients, a report of the functional outcome of a series of patients who had this procedure has not been published. Between 1991 and 1999, the authors treated 15 patients who had resection of the sciatic nerve. There were 10 females and five males, ranging in age from 2 to 73 years. Diagnoses included 11 high-grade soft tissue sarcomas, one primary bone sarcoma, and three metastatic bone tumors. Four lesions were located in the pelvis, one in the buttock, and 10 in the posterior thigh. At the most recent followup, 14 patients were ambulatory, seven of whom required a walking aid. Because of peroneal nerve palsy, all patients required a short-leg brace. However, overall function was determined to be good in 11, moderate in three, and poor in one patient. None had a pressure sore of the foot and none required a secondary amputation. Good function is achieved in most patients who have sciatic nerve resection. Therefore, the necessity to resect the sciatic nerve is not an indication for amputation.

Adolescent↗
PubMed2002

[Diagnosis and results of surgical treatment of intrathoracic neurogenic tumors].

The aim of the study was to study efficiency of diagnosis and surgical treatment of intrathoracic neurogenic tumors. Since 1990 to 2002, 28 patients with neurogenic tumors underwent surgical treatment in National Cancer Center and various clinics of Tbilisi. Age of the patients ranged from 14 to 65 years. The tumors located in the mediastinum in 20 patients, in the chest wall with intrathoracic growth--6 patients, and in the lung--2 patients. Radiology (X-ray, CT), endoscopy and cytology were used for diagnosis. It was demonstrated that CT, CT-guided fine-needle biopsy and videothoracoscopy improve diagnosis of intrathoracic neurogenic tumors. Radical surgery is the method of choice in surgical treatment of intrathoracic neurogenic tumors.

Adolescent↗
PubMedDate not supplied

NEUROGENIC sarcoma.

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Fibrosarcoma↗