Nerve Sheath Myxoma: A Clinicopathologic and Immunohistochemical Analysis of 57 Morphologically Distinctive, S-100 Protein- and GFAP-Positive, Myxoid Peripheral Nerve Sheath Tumors With a Predilection for the Extremities and a High Local Recurrence Rate

2007 ◽  
Vol 2007 ◽  
pp. 139-140
Author(s):  
D.M. Jukic
2015 ◽  
Vol 35 (12) ◽  
pp. 965-974 ◽  
Author(s):  
Gisele S. Boos ◽  
Daniele M. Bassuino ◽  
Fabiana Wurster ◽  
Neusa B. Castro ◽  
Tatiane T.N. Watanabe ◽  
...  

Abstract: In this retrospective study was determined the frequency of canine skin peripheral nerve sheath tumors (PNST) in cases diagnosed by the Setor de Patologia Veterinária of the Universidade Federal do Rio Grande do Sul (SPV-UFRGS), Brazil, between the years 2000 and 2012. The canine profiles, as well as histological, immunohistochemical and prognostic aspects of the tumors were based on 70 samples, comprising 40 females, 29 males and one unspecified sample. Between 2000 and 2012, 2,984 skin tumors of dogs were diagnosed in the SPV-UFRGS, totaling 2.34% of skin neoplasms in dogs. Animals that comprised the largest amount of samples (43%) were those with no breed (SRD), followed by German Shepherds (10%). Females were more affected than males (40/70 - 57% and 29/70 - 41% respectively). Skin PNST of this research showed predominant localization on the limbs (40% in the forelimbs and 29% in the hindlimbs); affecting adult dogs, mostly aged between 8 and 11 years (54%). The samples were routinely processed for hematoxylin and eosin, and were also evaluated by toluidine blue and Masson's trichrome staining, and immunohistochemistry (IHC) anti-vimentin, -S-100, -GFAP, -actin, von Willebrand factor and neurofilament. Anisocytosis and anisokaryosis, mitotic index, intratumoral necrosis, invasion of adjacent tissues, tumor location, local recurrence and metastasis were related to the diagnosis of benign (49/70) or malignant tumor (21/70). The Antoni A histological pattern was observed more frequently in benign tumors. The immunohistochemistry helped to diagnose PNST, and anti-vimentin and anti-protein S-100 showed the highest rates of immunostaining. Throughout statistical analysis of animals with tumor recurrence, it was found that the chance of an animal with a malignant peripheral nerve sheath tumor to develop recurrence is 4.61 times higher than in an animal that had a benign tumor.


2000 ◽  
Vol 122 (5) ◽  
pp. 667-672 ◽  
Author(s):  
Thom R. Loree ◽  
James H. North ◽  
Bruce A. Werness ◽  
Rina Nangia ◽  
Alan P. Mullins ◽  
...  

Malignant peripheral nerve sheath tumors of the head and neck region are uncommon and may be associated with neurofibromatosis or occur in a sporadic manner. This is a retrospective review of 17 patients with these tumors who were treated at a single institution. Analysis of clinical and pathologic factors that influenced outcome was performed. There were 9 men and 8 women. Seven patients had a history of neurofibromatosis. Radiotherapy was implicated as a possible etiologic factor in 4 patients. The neck was the most frequently involved site. Overall survival at 5 years was 52%. Survival was improved for women and for patients with low-grade tumors. Age, tumor site, and size had no impact on survival. Survival was worse for patients with neurofibromatosis than for those with the sporadic form of the disease ( P = 0.02). Survival was calculated by the method of Kaplan and Meier. The significance of such results was based on results of the log rank test. Local recurrence correlated with tumor size and resection margin status. No local recurrences occurred in those patients who had negative margins of resection and received adjuvant radiotherapy. Tumor grade was predictive of the development of distant metastases. Negative margins of resection are essential for obtaining local control, and the addition of adjuvant radiotherapy may be beneficial in this group. Salvage surgery for local recurrence is possible in some patients.


1996 ◽  
Vol 33 (6) ◽  
pp. 727-730 ◽  
Author(s):  
N. Kirchhof ◽  
W. Scheidemann ◽  
W. Baumgärtner

Multiple neurofibromas, schwannomas, and hyperplastic enteric plexuses were observed in the distal jejunum and ileum of a 6-year-old pinto gelding. The animal was presented because of an acute episode of colic. Three meters of distal small intestine, partially incarcerated in the epiploic foramen, were surgically removed. Numerous tumor nodules up to 10 mm in diameter were found adjacent to a Meckel's diverticulum, predominantly located in the subserosa of a hypertrophic segment. Histologically, tumors were well demarcated and composed of interlacing fascicles formed by spindloid cells. Adjacent enteric plexuses were hyperplastic. Immunohistochemically, all tumors were positive for vimentin and S-100. Desmin immunoreactivity was only observed in larger tumors (>500 μm). Glial fibrillary acid protein was demonstrated nearly exclusively in smaller ones. Immunostaining for neurofilament was restricted to entrapped ganglion cells. Based on conventional light microscopic examination and immunohistochemical evaluation, the lesion was diagnosed as multiple benign peripheral nerve sheath tumors in the small intestine.


2001 ◽  
Vol 38 (6) ◽  
pp. 679-688 ◽  
Author(s):  
G. Stoica ◽  
S. I. Tasca ◽  
H.-T. Kim

Thirty-four peripheral nerve sheath tumors of four domesticated animal species were characterized and assayed for point mutation of the neu oncogene. Based on their morphoimmunophenotype, 32 tumors were classified as schwannomas. Schwannoma morphology was characterized by the presence of Antoni type A and B pattern and immunoreactivity for S-100 protein and vimentin. Two anaplastic and metastatic tumors originating from spinal cord root, immunonegative for S-100 protein and positive for vimentin, were classified as malignant peripheral nerve sheath tumors (MPNSTs). Four malignant schwannomas and two MPNSTs expressed a point mutation of the neu oncogene by the polymerase chain reaction-restriction fragment length polymorphism method. The finding of neu oncogene mutation could be a useful diagnostic genetic marker in the malignant form of peripheral nerve sheath tumors in animals.


2019 ◽  
Vol 2 (3) ◽  
pp. 82
Author(s):  
Daniel Maranatha ◽  
Shinta Karina Yuniati

Background: Malignant tumors arising from peripheral nerves or displaying differentiation along the lines of the various elements of the nerve sheath are referred to Malignant peripheral nerve sheath tumors (MPNST) and also called malignant schwannomas or neurofibrosarcomas. The common sites of involvement are head, neck, extremities and thorax. This case was discussed due to its rare incidence, namely less than 5% of soft tissue malignant tumor. The overall five year survival rate is approximately 50% in patients with resectable tumor. Case: We report a case of MPNST arising from mediastinum, the patient was a male, 41-years-old, with chief complaint are chest pain and mass in the back. Contrast enhanced chest CT Scan showed a solid mass (10.02 × 6.97 × 10.53 cm) in the right side of the posterior mediastinum. On microscopic examination with hematoxylin eosin imunostaining, of the mass on mediastinum showed typical features of MPNST, which were positive for S-100 imunostaining. Based on convensional histopathologic and imunostaining, this case was concluded as Malignant peripheral nerve sheath tumors (MPNST). Conclusion: The treatment of chest wall MPNSTs is multimodality, including wide local excision, adjuvant radiotherapy, and chemotherapy. In our case, we performed direct excision due to the infiltrating mass to the surrounding tissue, so the prognosis for this case was good. This case indicates the role of chemotherapy in treatment of advanced MPNST. Studies shows the superiority of the doxorubicin–ifosfamide regimen. As in this case the patient showed complete respons after surgery and adjuvant chemotherapy.


2017 ◽  
Vol 26 (3) ◽  
pp. 291-298 ◽  
Author(s):  
Dean Chou ◽  
Mark H. Bilsky ◽  
Alessandro Luzzati ◽  
Charles G. Fisher ◽  
Ziya L. Gokaslan ◽  
...  

OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft-tissue sarcomas. Resection is the mainstay of treatment and the most important prognostic factor. However, complete resection of spinal MPNSTs with tumor-free margins is challenging due to the likelihood of residual tumor cells. The objective of this study was to describe whether the type of Enneking resection in the management of spinal MPNSTs had an effect on local recurrence and survival. METHODS The AOSpine Knowledge Forum Tumor developed a multicenter database that includes demographic, diagnostic, therapeutic, local recurrence, and survival data on patients with primary spinal column tumors. Patients who had undergone surgery for a primary spinal MPNST were included and were analyzed in 2 groups: 1) those undergoing Enneking appropriate (EA) resections and 2) those undergoing Enneking inappropriate (EI) resections. EA surgery was performed if there was histopathological evidence of an intact tumor pseudocapsule and at least a marginal resection on a vital structure. EI surgery was performed if there was an intentional or inadvertent transgression of the margin. RESULTS Between 1993 and 2012, 29 primary spine MPNSTs were identified in 12 (41%) females and 17 (59%) males with a mean age at diagnosis of 40 ± 17 years (range 5–74 years). The median patient follow-up was 1.3 years (range 42 days to 11.2 years). In total, 14 (48%) patients died and 14 (48%) patients suffered a local recurrence, 10 (71%) of whom died. Within 2 years after surgery, the median survival and local recurrence were not achieved. Data about Enneking appropriateness of surgery were available for 27 patients; 9 (33%) underwent an EA procedure and 18 (67%) underwent an EI procedure. Enneking appropriateness did not have a significant influence on local recurrence or survival. Twenty-two patients underwent adjuvant treatment with combined chemo- and radiotherapy (n = 7), chemotherapy alone (n = 3), or radiotherapy alone (n = 12). Adjuvant therapy had no significant influence on recurrence or survival. CONCLUSIONS The rates of recurrence and survival were similar for spinal MPNSTs regardless of whether patients had an EA or EI resection or received adjuvant therapy. Other factors such as variability of pathologist interpretation, PET CT correlation, or neurofibromatosis Type 1 status may play a role in patient outcome. Nonetheless, MPNSTs should still be treated as sarcomas until further evidence is known. The authors recommend an individualized approach with careful multidisciplinary decision making, and the patient should be informed about the morbidity of en bloc surgery when considering MPNST resection.


2016 ◽  
Vol 66 (4) ◽  
pp. 455-465 ◽  
Author(s):  
Rubina Sirri ◽  
Silvia Sabattini ◽  
Giuliano Bettini ◽  
Luciana Mandrioli

Abstract The aim of this study was to re-evaluate archived samples of canine soft tissue sarcomas (STSs) morphologically consistent with peripheral nerve sheath tumors (PNSTs). In each case, an immunohistochemical panel was applied, including α-SMA, calponin, desmin, S-100, GFAP, NSE and Olig2, in order to assess whether the phenotype was consistent with the tumor histological appearance. Additionally, the expression of EGFR, a marker with potential therapeutic implications in malignant PNSTs, was evaluated. Twenty-one tumors were included. Fourteen cases (66.7%) were positive for one or more muscular markers and were reclassified as perivascular tumors (PWTs). A positive labeling for S-100 was observed in one tumor (4.8%), thus classifi ed as PNST. The other 6 tumors were generically classified as poorly differentiated STSs. No unique histopathological feature was observed within the three groups. NSE and Olig2 labeling was aspecific and not useful for diagnostic purposes. GFAP was negative in all cases. Six cases (28.6%) were positive for EGFR, including the PNST. Even after the application of a wide immunohistochemical panel, distinguishing between PNSTs and PWTs remains a challenge. Finally, a subgroup of cases cannot be classified based on light microscopy alone.


2021 ◽  
Vol 49 ◽  
Author(s):  
Ana Caroline da Silva Néto Souza ◽  
Carlos Humberto da Costa Vieira Filho ◽  
Elainne Maria Beanes Da Silva ◽  
Caterina Muramoto ◽  
Icaro Farias Correia ◽  
...  

Background: The most common location of malignant tumors of the peripheral nerve sheath in the spinal cord is the intradural-extramedullary region, and is rare in the spinal nerve roots in the lumbar region. They mainly affect large female dogs over six years of age. Imaging tests assist in the presumptive diagnosis, but confirmation requires histopathological and immunohistochemical examination. The prognosis is guarded. Diagnostic imaging, anatomopathological and immunohistochemical findings of a malignant tumor of the intradural-extramedullary peripheral nerve sheath with medullary infiltration in the lumbar region in a young dog are reported.Case: A body of a 6-year-old Poodle dog was donated for necropsy and diagnostic clarification. In the history, there was a suspicion of lumbar intramedullary neoplasia, detected by computed tomography (CT), with a 4 years progressive chronic evolution. Additionally, the dog had hidden spina bifida (L7 to S3), as detected by radiography and CT. On post mortem radiographic examination (X-ray), there was an enlargement of the vertebral canal (T10 to S2), intense osteolysis (L1 to S2), spinous processes (L5 to L7), and ankylosis (L3 to L7). Necropsy revealed ankylosis (L3 to L7) and intradural-extramedullary mass (9.5 × 2.6 × 2.3 cm) (L2 to L6). No metastases were identified. On microscopy, there was neoplastic proliferation of cells with intense pleomorphism, arranged in bundles interlaced in palisades and sometimes solid mantles. The mitotic index was high, ranging from 10 to 12 mitoses per field. There was also necrosis, hemorrhage, edema, and focal axonal demyelination of the adjacent white matter in the spinal cord. Masson Trichrome staining highlighted an intense diffuse conjunctive stroma. There was a suspicion of a malignant tumor of the peripheral nerve sheath and an immunohistochemical panel was performed for confirmation. There was strong and diffuse positivity for vimentin and S-100 and partial positivity for neuron-specific enolase (NSE), negative for anti-factor VIII, glial fibrillary acidic protein (GFAP), α-actin for smooth muscle, cytokeratin, neurofilament, and desmin. Thus, the diagnosis of malignant neoplasm of the peripheral nerve sheath was confirmed.Discussion: Peripheral nerve sheath tumors are classified as benign or malignant. In dogs, they are frequent in elderly, females, and large breeds. In this case report, the animal was young, female, and small breed. The location of the spinal nerve roots is uncommon, and is more commonly found in the brachial plexus. In the animal reported, the tumor was observed as lumbar swelling. Clinical signs vary with the affected region, however, neurogenic claudication and muscle atrophy are more frequent, as observed in this report. Imaging examinations such as X-rays and CT assist in the presumptive diagnosis. In this case report, spina bifida was identified on radiography, and CT suggested the presence of intramedullary neoplasia and allowed to monitor tumor growth. Post mortem X-ray imaging revealed intense osteolysis and ankylosis, which were confirmed at necropsy, which also elucidated its intradural-extramedullary location with infiltration into the spinal cord. The confirmation of the neoplasm was made by histopathological and immunohistochemical examination; the latter should be made a panel, not restricted to the use of antibodies S-100 and vimentin only. The prognosis of malignant peripheral nerve sheath tumors (MPNST) of the spinal cord is poor, and although there are palliative methods, there is no curative treatment, as complications can interfere with the quality of life of the animal. MPNST should be included in the differential diagnosis of spinal disorders, even in young dogs and small breeds. CT helps in early diagnosis to make decisions aimed at the animal's well-being.


2019 ◽  
Vol 69 (3) ◽  
pp. 290-299
Author(s):  
Ivana Vučićević ◽  
Darko Marinković ◽  
Vladimir Kukolj ◽  
Slađan Nešić ◽  
Milan Aničić ◽  
...  

Abstract Peripheral nerve sheath tumors (PNSTs) comprise a heterogeneous group of neoplasms originating from the elements of the nerve sheath. They are divided into two forms: benign and malignant PNST. Both benign and malignant PNSTs are not very common in domestic animals but they are reported in different animal species. Histologically, PNSTs are composed predominantly of spindle cells arranged in bundles, whorls and sheets, with a different number of pleomorphic cells and mitotic figures. The aim of this study was a reclassification of 17 dog tumor samples initially diagnosed with peripheral nerve sheath tumors using histopathological analysis. The main criterion for reclassification was immunohistochemical positivity for various antigens. PNSTs are often histologically very similar to other spindle cell tumors and immunohistochemistry is required for differential diagnosis. PNSTs generally express vimentin, S-100 protein, glial fibrillary acidic protein (GFAP), collagen IV and laminin. Four tumor samples were positive to muscular marker α-SMA and vimentin and negative for S-100 protein and desmin. The spindle cells whirling around some blood vessels were observed in these tumors so they were reclassified as perivascular wall tumors (PWTs). The other 13 tumors were S-100 protein and vimentin positive and α-SMA and desmin negative, thus classified as PNST. The use of the immunohistochemical panel is necessary for distinguishing PNSTs from PWTs in routine diagnostics.


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