Concomitant Malignant Pulmonary Peripheral Nerve Sheath Tumour and Benign Cutaneous Peripheral Nerve Sheath Tumour in a Dog

Silva, E.O.; Goiozo, Paulo Felipe Izique; Pereira, Ludimilla; Headley, Selwyn Arlington; Bracarense, A.F.R.L.

doi:10.1016/j.jcpa.2017.05.002

Cited by 9 publications

(16 citation statements)

References 28 publications

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“…Both Antoni A and Antoni B histological growth patterns were observed. A similar pattern was also reported by Chijiwa et al (2004) [3] and Silva et al (2017) [14]. The concentric whorls were present in PNSTs, but more often were observed in PWTs as neoplastic cells surrounding blood vessels rather than collagen fi bers.…”

Section: Discussionsupporting

confidence: 84%

“…Histologically, benign tumors of peripheral nerve sheath are usually well circumscribed, encapsulated and composed predominantly of spindle cells arranged in bundles and sheets [12]. The slightly polymorphic neoplastic cells could be separated by a collagenous matrix and form concentric whorls (Antoni type A pattern) or the cellular density could be reduced and cells surrounded by loose fi brous to mucinous stroma (Antoni type B pattern) [13,14]. The double row of palisading tumor cells, known as Verocay bodies, is rarely observed [15].…”

Section: Introductionmentioning

confidence: 99%

“…Neoplastic cells are arranged in fascicles, whorls or sheets and embedded in a fi brillar collagenous stroma. MPNSTs have an infi ltrative growth pattern and metastasize to distant organs [13,14]. Areas of glandular, rhabdomyoblastic squamous, cartilaginous or osteoid differentiation may be seen occasionally [12,13].…”

Section: Introductionmentioning

confidence: 99%

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Immunohistochemical Distinguishing between Canine Peripheral Nerve Sheath Tumors and Perivascular Wall Tumors

et al. 2019

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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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Section: Discussionsupporting

confidence: 84%

Section: Introductionmentioning

confidence: 99%

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Immunohistochemical Distinguishing between Canine Peripheral Nerve Sheath Tumors and Perivascular Wall Tumors

et al. 2019

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“…In dogs, neurofibromas are typically associated with the skin, peripheral nerves, tongue, and intestine [32,60,61]. Although the generic diagnosis of benign PNST is often used, the existence of neurofibromas in dogs with similarities to humans has been described with regard to signalment, histology, immunohistochemistry, and electron microscopic findings [32].…”

Section: Dogsmentioning

confidence: 99%

Spontaneous and Engineered Large Animal Models of Neurofibromatosis Type 1

Osum

Watson

Largaespada

2021

IJMS

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Animal models are crucial to understanding human disease biology and developing new therapies. By far the most common animal used to investigate prevailing questions about human disease is the mouse. Mouse models are powerful tools for research as their small size, limited lifespan, and defined genetic background allow researchers to easily manipulate their genome and maintain large numbers of animals in general laboratory spaces. However, it is precisely these attributes that make them so different from humans and explains, in part, why these models do not accurately predict drug responses in human patients. This is particularly true of the neurofibromatoses (NFs), a group of genetic diseases that predispose individuals to tumors of the nervous system, the most common of which is Neurofibromatosis type 1 (NF1). Despite years of research, there are still many unanswered questions and few effective treatments for NF1. Genetically engineered mice have drastically improved our understanding of many aspects of NF1, but they do not exemplify the overall complexity of the disease and some findings do not translate well to humans due to differences in body size and physiology. Moreover, NF1 mouse models are heavily reliant on the Cre-Lox system, which does not accurately reflect the molecular mechanism of spontaneous loss of heterozygosity that accompanies human tumor development. Spontaneous and genetically engineered large animal models may provide a valuable supplement to rodent studies for NF1. Naturally occurring comparative models of disease are an attractive prospect because they occur on heterogeneous genetic backgrounds and are due to spontaneous rather than engineered mutations. The use of animals with naturally occurring disease has been effective for studying osteosarcoma, lymphoma, and diabetes. Spontaneous NF-like symptoms including neurofibromas and malignant peripheral nerve sheath tumors (MPNST) have been documented in several large animal species and share biological and clinical similarities with human NF1. These animals could provide additional insight into the complex biology of NF1 and potentially provide a platform for pre-clinical trials. Additionally, genetically engineered porcine models of NF1 have recently been developed and display a variety of clinical features similar to those seen in NF1 patients. Their large size and relatively long lifespan allow for longitudinal imaging studies and evaluation of innovative surgical techniques using human equipment. Greater genetic, anatomic, and physiologic similarities to humans enable the engineering of precise disease alleles found in human patients and make them ideal for preclinical pharmacokinetic and pharmacodynamic studies of small molecule, cellular, and gene therapies prior to clinical trials in patients. Comparative genomic studies between humans and animals with naturally occurring disease, as well as preclinical studies in large animal disease models, may help identify new targets for therapeutic intervention and expedite the translation of new therapies. In this review, we discuss new genetically engineered large animal models of NF1 and cases of spontaneous NF-like manifestations in large animals, with a special emphasis on how these comparative models could act as a crucial translational intermediary between specialized murine models and NF1 patients.

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“…21,27,28 Rarely reported sites of origin include the lung, spleen, liver, diaphragm, vagus nerve, tongue, omentum, ocular origin, heart, brain, adrenal gland and testis. 22,29–50 Incomplete surgical resection leads to recurrence and metastatic disease is rarely reported. 22,24,39,43,44,46,47,49–52 Stereotactic radiation therapy for canine trigeminal and brachial plexus MPNSTs may prolong survival times when surgical resection is not possible.…”

Section: Discussionmentioning

confidence: 99%

Colonic malignant peripheral nerve sheath tumour in a cat

Boland

Setyo

Sangster

et al. 2019

Journal of Feline Medicine and Surgery Open Reports

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Case summary A 14-year-old male neutered domestic mediumhair cat presented with a 4 month history of inappetence and weight loss. Pertinent abnormalities on haematology and biochemistry included a mild microcytic regenerative anaemia (packed cell volume [PCV] 24% [reference interval (RI) 30–45%], mean cell volume 30.8 fl [RI 40–45 fl], absolute reticulocyte count 326.8 × 10 12 ) and increased alkaline phosphatase activity (76 IU/l; RI <50 IU/l). Abdominal ultrasound and CT scan revealed masses in the transverse colon (2.0 cm × 1.2 cm) and right medial liver lobe (5.0 cm diameter). Thoracic radiographs were unremarkable. Right medial liver lobe resection and colectomy were performed. Immunohistochemistry was positive for S-100 protein, vimentin and glial fibrillary acidic protein, very weakly positive for c-kit and negative for muscle-specific actin and CD18, consistent with a colonic malignant peripheral nerve sheath tumour (MPNST) with a hepatic metastasis. Postoperative treatment with metronomic cyclophosphamide was well tolerated. Eighteen months postoperatively the cat re-presented after 3 days of progressive lethargy and inappetence. Haematology revealed a marked non- or pre-regenerative anaemia (PCV 10%). Coagulation times were prolonged (prothrombin time 39 s [RI 15–22 s] and activated partial thromboplastin time >300 s [RI 65–119 s]). Abdominal ultrasound identified multiple renal and hepatic nodules. Euthanasia was performed and post-mortem examination confirmed metastasis of the MPNST. Relevance and novel information This report describes the treatment of a metastatic colonic peripheral nerve sheath tumour in a cat. Feline visceral MPNSTs are rare and little is known about prognosis or optimal treatment.

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Concomitant Malignant Pulmonary Peripheral Nerve Sheath Tumour and Benign Cutaneous Peripheral Nerve Sheath Tumour in a Dog

Cited by 9 publications

References 28 publications

Immunohistochemical Distinguishing between Canine Peripheral Nerve Sheath Tumors and Perivascular Wall Tumors

Immunohistochemical Distinguishing between Canine Peripheral Nerve Sheath Tumors and Perivascular Wall Tumors

Spontaneous and Engineered Large Animal Models of Neurofibromatosis Type 1

Colonic malignant peripheral nerve sheath tumour in a cat

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