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Malignant nerve sheath tumor

Gonzalo Lopez, Raphael E Pollock
Malignant peripheral nerve sheath tumor (MPNST) is a highly aggressive disease with a dismal prognosis. The disease can occur sporadically or in patients with inherited neurofibromatosis (NF-1). MPNST is typically resistant to therapeutic intervention. Hence, the need for improved therapies is warranted. Several broad spectrum histone deacetylase (HDAC) inhibitors have a high affinity for class I HDAC isoforms. Inhibition of multiple HDAC isoforms often results in undesirable side effects, while inhibiting a single isoform could possibly improve the therapeutic window and limit toxicity...
2017: Methods in Molecular Biology
O Kalita, K Cwiertka, D Vrána, M Vaverka, L Tučková, M Megová
BACKGROUND: Malignant peripheral nerve sheath tumor schwannoma (MPNST), also known as malignant schwannoma, is a very rare tumor accounting for only 2% of all sarcomas. The prognosis is relatively poor, with a 5-year survival rate of 46-69%. The treatment of MPNST has not been standardized yet. Mainstay treatment is radical resection. Oncological adjuvant or neoadjuvant treatment has equivocal indications with unclear effects. CASE: The case report presents a 55-year-old patient who showed resistance in the medial-ventral area of the left lower limb...
2016: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
Ayca Ersen, Melike Pekmezci, Andrew L Folpe, Tarik Tihan
No abstract text is available yet for this article.
October 12, 2016: Pathology Oncology Research: POR
Kunihiro Ikuta, Takehiro Ota, Lisheng Zhuo, Hiroshi Urakawa, Eiji Kozawa, Shunsuke Hamada, Koji Kimata, Naoki Ishiguro, Yoshihiro Nishida
Hyaluronan (HA) has been shown to play important roles in the growth, invasion and metastasis of malignant tumors. Our previous study showing that high HA expression in malignant peripheral nerve sheath tumors (MPNST) is predictive of poor patient prognosis, prompted us to speculate that inhibition of HA synthesis in MPNST might suppress the tumorigenicity. The aim of our study was to investigate the antitumor effects of 4-methylumbelliferone (MU), an HA synthesis inhibitor, on human MPNST cells and tissues...
October 5, 2016: International Journal of Cancer. Journal International du Cancer
Corrine R Davis, Beth A Valentine, Emma Gordon, Sean P McDonough, Paula A Schaffer, Andrew L Allen, Patricia Pesavento
A diagnosis of neoplasia was noted in 125 of 357 donkeys (35%) in our review of medical records from 5 veterinary schools in the United States and Canada. Equine sarcoid was the most common tumor in our study, accounting for 72% of all tumors and 82% of cutaneous tumors. Soft-tissue sarcomas were the second most common skin tumors. All other types of neoplasia were rare. Important differences in the occurrence of neoplasia in donkeys compared to horses included the rarity or absence of squamous cell carcinoma in any organ system and gray horse melanoma...
November 2016: Journal of Veterinary Diagnostic Investigation
Christopher R Jackson, Eugen C Minca, Jyoti P Kapil, Steven Christopher Smith, Steven D Billings
Malignant peripheral nerve sheath tumors are rare soft tissue sarcomas with histological and immunohistochemical similarities to spindle cell melanoma. Although spindle cell melanoma is significantly more common, both tumors may express S100 and lack staining for HMB-45, Melan-A, or MITF. Here we present a case of superficial malignant peripheral nerve sheath tumor with diffuse S100 positivity arising in a subtle neurofibroma in close proximity to an intradermal melanocytic nevus. This configuration had led to prior misdiagnosis as a desmoplastic melanoma arising in the nevus and to sentinel lymph node biopsy...
September 30, 2016: Journal of Cutaneous Pathology
Weibo Pan, Bin Feng, Zhan Wang, Nong Lin, Zhaoming Ye
PURPOSE: Malignant peripheral nerve sheath tumors are extremely rare in the general population and display a predilection for metastasis to the lungs. Here, we present a rare case of a malignant peripheral nerve sheath tumor located in the paraspinal region and highlight the importance of preoperative biopsy in diagnosis of spinal epidural peripheral nerve sheath tumors. METHODS: We describe the clinical course of the patient as well as the radiological and pathological findings of the tumor...
September 27, 2016: European Spine Journal
M Galukande, A Khingi
BACKGROUND: Chest wall schwannomas are rare tumors arising from the intercostals nerves. Schwannomas are lobulated, encapsulated spherical masses, different from neurofibromas in that matter. Men and women are equally affected in their third and fourth decades. CASE PRESENTATION: A 42 year old female presented with a 6 month history of progressively worsening pain over the right shoulder and chest wall, aggravated by movement and with associated right arm oedema and paraesthesia...
2016: SpringerPlus
Ushio Hanai, Tadashi Akamatsu, Megumi Kobayashi, Yotaro Tsunoda, Kenichi Hirabayashi, Tanehumi Baba, Hideki Atsumi, Mitsunori Matsumae
INTRODUCTION: The prognosis of malignant peripheral nerve sheath tumor (MPNST) with neurofibromatosis type 1 (NF-1) is worse than that of a solitary MPNST, because of the tumor size and location difficult to resect completely. We experienced a case of MPNST in the occipital region with NF-1. CASE REPORT: A 59-year-old woman presented with NF-1 and an MPNST of the occipital region. We performed wide excision involving the occipital bone, and reconstructed with a titanium plate and a free latissimus dorsi muscle flap...
2016: Tokai Journal of Experimental and Clinical Medicine
Masayuki Oki, Tomihisa Nanao, Takuma Shinoda, Ayumi Tsuda, Atsushi Yasuda, Toshiro Seki, Hideki Ozawa, Naoya Nakamura, Atsushi Takagi
To date, there are only 15 case reports of lymphoma in patients with neurofibromatosis type 1 (NF1), a common autosomal dominant tumor predisposition syndrome. Here, we present the first report of a primary effusion lymphoma (PEL)-like lymphoma (PEL-L), which is a human herpes virus 8/Kaposi sarcoma herpes virus-unrelated PEL, in a 73-year-old woman with NF1. The woman presented with pleural effusion following surgery for a small intestinal gastrointestinal stromal tumor and a malignant peripheral nerve sheath tumor...
2016: Tokai Journal of Experimental and Clinical Medicine
Philip R Cohen
BackgroundSegmental neurofibromatosis, referred to as mosaic neurofibromatosis 1, patients present with neurofibromas or café au lait macules or both in a unilateral segment of the body.PurposeA woman with segmental neurofibromatosis and triple cancer (renal cell carcinoma, mixed thyroid carcinoma, and lentigo maligna) is described and cancers observed in patients with segmental neurofibromatosis are reviewed.MethodsPubMed was used to search the following terms, separately and in combination: cancer, malignancy, mosaic, neoplasm, neurofibroma, neurofibromatosis, segment, segmental, tumor...
2016: Dermatology Online Journal
Hiroshi Urakawa, Eiji Kozawa, Kunihiro Ikuta, Shunsuke Hamada, Naoki Ishiguro, Yoshihiro Nishida
The aim of this study is to examine the impact of disease free (DF) status on the prognosis in patients with metastatic non-small round cell soft tissue sarcoma (STS). We retrospectively reviewed 51 metastatic STS patients who were treated in Nagoya University Hospital from 2005 to 2015. The relation between various clinical factors and overall survival (OS) was analyzed. The log rank test and Cox's proportional hazards test were used to evaluate the differences between groups. p-values of <0.05 were considered to indicate significance...
September 7, 2016: Clinical & Experimental Metastasis
G A Frank, Yu Yu Andreeva, L V Moskvina, G D Efremov, S I Samoilova
The paper reviews the 2016 WHO classification of prostate tumors, notes the alterations made, and describes approaches to the diagnosis of cancer types and grades. It also gives original photomicrographs from the authors' collection. The main alterations were as follows: - The types of prostate adenocarcinoma were added by pleomorphic giant-cell carcinoma; oncocytic (8290/3) and lymphoepithelial (8082/3) carcinomas were excluded. - Grade III prostatic intraepithelial neoplasia (PIN) was substituted for high grade PIN (8148/2)...
July 2016: Arkhiv Patologii
Humain Baharvahdat, Babak Ganjeifar, Nema Mohamadian Roshan, Aslan Baradaran
Spinal Intradural primary malignant peripheral nerve sheath tumors (MPNST) are rare in patients without neurofibromatosis. Here we represent a 3- year-old girl of primary intradural spinal malignant peripheral nerve sheath tumor. The tumor was removed partially and MPNST was diagnosed in the histopathological exam. Her condition deteriorated due to acute hydrocephalus in the following days. In this article we discuss the clinical presentation, imaging, treatment, and prognosis of our patient and the other 22 patients of primary intradural MPNST, found in the literature...
March 4, 2016: Turkish Neurosurgery
Erhan Emel, Anas Abdallah, Özden Erhan Sofuoğlu, Ali Ender Ofluoğlu, Müslüm Güneş, Betül Güler, Bilge Bilgiç
AIM: Spinal schwannomas (SS) represent the most common intradural extramedullary lesions, accounting for approximately 24% of all nerve sheath tumors in adults. In this study, long-term outcomes of 49 consecutive SS have been presented. MATERIAL AND METHODS: Medical records were retrospectively reviewed in 371 cases of spinal tumors who underwent surgery between the years 2005 and 2014. Cases which confirmed as schwannoma histopathologically were included in this study...
October 22, 2015: Turkish Neurosurgery
Ralph J Mobbs, Kevin Phan, Monish M Maharaj, Joga Chaganti, Neil Simon
BACKGROUND: Ulnar neuropathy at the elbow (UNE) is the second most common mononeuropathy of the upper extremity. One rare cause of UNE is nerve mass lesions, including intraneural ganglion cysts (IGCs). IGC imaging studies provide important information that may determine the nature of a peripheral nerve mass lesion. CASE DESCRIPTION: We present the case of a 73-year-old woman who presented with rapid deterioration of left hand function over 2 months with weakness of fine motor control, grip strength, and dysesthesia in the ulnar nerve distribution...
September 1, 2016: World Neurosurgery
Aaron W James, Elizabeth Shurell, Arun Singh, Sarah M Dry, Fritz C Eilber
Malignant peripheral nerve sheath tumor (MPNST) is the sixth most common type of soft tissue sarcoma. Most MPNSTs arise in association with a peripheral nerve or preexisting neurofibroma. Neurofibromatosis type is the most important risk factor for MPNST. Tumor size and fludeoxyglucose F 18 avidity are among the most helpful parameters to distinguish MPNST from a benign peripheral nerve sheath tumor. The histopathologic diagnosis is predominantly a diagnosis of light microscopy. Immunohistochemical stains are most helpful to distinguish high-grade MPNST from its histologic mimics...
October 2016: Surgical Oncology Clinics of North America
M M Dolmans, Y Iwahara, J Donnez, M Soares, J L Vaerman, C A Amorim, H Poirel
STUDY QUESTION: What is the risk of finding malignant cells in cryopreserved ovarian tissue from sarcoma patients? SUMMARY ANSWER: Minimal disseminated disease (MDD) was not detected in frozen-thawed ovarian tissue from 26 patients by any of the sensitive methods applied. WHAT IS KNOWN ALREADY: In case of leukemia, the risk of malignant cell transmission through the graft is well known and widely documented. However, for bone cancer, like Ewing sarcoma or osteosarcoma, only a small number of case reports, have been published...
October 2016: Human Reproduction
Elizabeth Shurell, Arun S Singh, Joseph G Crompton, Sarah Jensen, Yunfeng Li, Sarah Dry, Scott Nelson, Bartosz Chmielowski, Nicholas Bernthal, Noah Federman, Paul Tumeh, Fritz C Eilber
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an aggressive sarcoma with few treatment options. Tumor immune state has not been characterized in MPNST, and is important in determining response to immune checkpoint blockade. Our aim was to evaluate the expression of programmed death-ligand 1 (PD-L1), programmed cell death protein 1 (PD-1), and presence of CD8+ tumor infiltrating lymphocytes (TILs) in MPNST, and correlate these findings with clinical behavior and outcome...
August 31, 2016: Oncotarget
Tomohito Hagi, Tomoki Nakamura, Ayumu Yokoji, Akihiko Matsumine, Akihiro Sudo
The present study reports a case of medullary metastasis without lung metastasis that occurred as a result of a malignant peripheral nerve sheath tumor (MPNST). An 81-year-old woman presented with a MPNST in the left brachial plexus, arising from the cervical nerve root. The patient underwent carbon ion radiotherapy; however, tumor recurrence was identified in the left shoulder. Subsequently, the patient underwent wide excision. Three weeks subsequent to surgery, imbalance and dysarthria developed suddenly...
September 2016: Oncology Letters
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