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      Ulnar malignant peripheral nerve sheath tumour diagnosis in a mixed-breed dog as a model to study human: histologic, immunohistochemical, and clinicopathologic study

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          Abstract

          Canine Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are uncommonly reported in the ulnar, since they are underestimated relative to the more common spindle cell tumours of soft tissue. In dogs, MPNST accounts for 27% of nervous system tumours. In man, MPNST represents 5-10% of all soft tissue sarcomas and is often associated with neurofibromatosis type 1 (NF-1).An 8-year-old, 9 kg, female mixed-breed dog with a subcutaneous mass on the upper right side of the ulnar region was presented to the small animal research and teaching hospital of Tehran University. The dog was anorexic with general weakness. The mass (7 × 4 cm) was removed surgically and processed routinely. Microscopically, the mass was composed of highly cellular areas with a homogeneous population of round or spindle cells, high cellular pleomorphism, high mitotic index and various morphologic patterns. Furthermore, spindle cells arranged in densely or loosely sweeping fascicles, interlacing whorls, or storiform patterns together with wavy cytoplasm, nuclear palisades, and round cells were arranged in sheets or cords with a meshwork of intratumoral nerve fibers. In addition, in this case the presence of neoplastic cells within the blood vessels was observed. Immunohistochemically, tumor was positive for vimentin and S-100 protein. The histopathologic features coupled with the S-100 and vimentin immunoreactivity led to a diagnosis of malignant neurofibroma.

          To the best of our knowledge, primary ulnar MPNST has not been reported in animals. This is the first documentation of an ulnar malignant peripheral nerve sheath tumour in a dog.

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          Most cited references39

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          Immunohistochemical evaluation of canine peripheral nerve sheath tumors and other soft tissue sarcomas.

          Seventeen cases of canine peripheral nerve sheath tumors (PNSTs), 11 malignant PNSTs (MPNSTs), and six benign PNSTs (BPNSTs) were examined. The prognosis in five of six dogs with BPNSTs was excellent, whereas all dogs with MPNSTs died within 2 years after the last surgical resection. One BPNST formed a recurrent mass with features of a MPNST. Histopathologically, the predominant tumor cell of MPNSTs was either spindle or round in shape with epithelioid characteristics. Other atypical cells had abundant granular cytoplasm or were multinucleated giant cells with periodic acid-Schiff-positive cytoplasmic globules. Furthermore, two MPNSTs contained cartilaginous and osseous metaplasia. On the contrary, most BPNSTs exhibited typical features of schwannoma or neurofibroma, whereas two BPNSTs had atypical morphology. One BPNST consisted of epithelioid cell proliferation with some tumor cells revealing nuclear atypia. Immunohistochemically, the expression of vimentin (100%), S-100 (73%), nerve growth factor receptor (NGFR, 64%), and myoglobin (64%) was commonly found in MPNSTs. The two BPNSTs with atypical histologic appearances were positive for vimentin, S-100, NGFR, and neuron-specific enolase, and one of these had moderate immunoreactivity for cytokeratin. Most BPNSTs were positive for glial fibrillary acidic protein, as well as S-100 and NGFR. Although most rhabdomyosarcomas (RMSs) and canine hemangiopericytomas (CHPs) also showed focal immunoreactivity for S-100, most RMSs were intensely positive for myoglobin and negative for NGFR. Most CHPs (80%) exhibited focal alpha-smooth muscle actin (alpha-SMA) expression, whereas all PNSTs were negative. These results indicate that immunohistochemistry for NGFR and alpha-SMA might be useful for differentiating canine PNSTs from RMSs or CHPs, respectively.
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            Schwannoma (neurilemoma) with malignant transformation. A rare, distinctive peripheral nerve tumor.

            Malignant transformation of a schwannoma (neurilemoma) is an exceedingly rare event. We describe two cases with such change and review the reported purported examples. The tumors in our patients involved a finger and pelvis. Sex, age, and clinical follow-up were available for only the second case, involving a 31-year-old man who died with recurrent and metastatic tumor. Seven acceptable cases were found in the literature. Analysis of the nine cases of schwannoma with malignant transformation showed no sex predilection, but revealed a tumor differing significantly from conventional malignant peripheral nerve sheath tumors. The mean age (56 years) was two decades older, no patient had neurofibromatosis, in four cases there was a years-long history of an antecedent mass, and in none of the cases was the malignant component an interlacing, fasciculated spindle-cell tumor. Rather, the malignant component was commonly purely epithelioid (seven of nine cases). In the two other cases, cells of the malignant component had neuroepithelial features. The prognosis for patients with schwannomas undergoing malignant change is poor. Five of eight patients with follow-up (62%) died of disease with either residual (one patient) or metastatic tumor (four patients).
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              Malignant "Triton" tumors. Natural history and immunohistochemistry of nine new cases with literature review.

              Malignant schwannomas with rhabdomyoblastic differentiation have been termed malignant "Triton" tumors (MTT). To define the natural history of MTT, we have analyzed our experience (9 cases, the largest series reported) in combination with the 27 previously described in the literature (total 36 cases). This study was initiated due to the unusual presentation of MTT as a polypoid esophageal mass. Rhabdomyoblastic differentiation in these tumors was confirmed using myoglobin immunohistochemistry. Two groups of patients were identified: those with Von Recklinghausen's Neurofibromatosis (Group I, VRN cases); and those without (Group II, sporadic, non-VRN cases). Group I patients accounted for over 70% of cases and displayed a marked male predominance, young age, and common head and neck presentation. By contrast, Group II patients were older, had a female predominance, and tumors frequently located on the trunk. Both groups fared equally poorly: local recurrence was common and the 5-year survival rate for all cases was 12%. Our data support the view that the natural history of MTT, whether in VRN patients or not, is much more aggressive than sporadic malignant schwannoma and similar to VRN sarcomas in general. This poor outlook could not be attributed to site; rather, it appeared to reflect the high frequency of Grade III histology in this disease.
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                Author and article information

                Journal
                Diagn Pathol
                Diagn Pathol
                Diagnostic Pathology
                BioMed Central
                1746-1596
                2013
                20 May 2013
                : 8
                : 86
                Affiliations
                [1 ]Department of Pathology, Faculty of Veterinary Medicines, Tehran University, Tehran, Iran
                [2 ]Paraveterinary Faculty of Ilam, University of Ilam, Ilam, Iran
                [3 ]Department of Clinical Science, Faculty of Veterinary Medicine, Tehran University, Tehran, Iran
                Article
                1746-1596-8-86
                10.1186/1746-1596-8-86
                3699426
                23688209
                dca72502-d4d7-47eb-bbc3-c0b27abc84ba
                Copyright ©2013 Tavasoly et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 3 May 2013
                : 16 May 2013
                Categories
                Case Report

                Pathology
                dog,pathology,immunohistochemistry,tumor,markers
                Pathology
                dog, pathology, immunohistochemistry, tumor, markers

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