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Malignant Peripheral Nerve Sheath Tumor

Suguru Fukushima, Makoto Endo, Yoshihiro Matsumoto, Jun-Ichi Fukushi, Tomoya Matsunobu, Ken-Ichi Kawaguchi, Nokitaka Setsu, Keiichiro IIda, Nobuhiko Yokoyama, Makoto Nakagawa, Kenichiro Yahiro, Yoshinao Oda, Yukihide Iwamoto, Yasuharu Nakashima
[This corrects the article DOI: 10.1371/journal.pone.0178064.].
2018: PloS One
Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
Max Shutran, David Mosbach, Zachary Tataryn, Knarik Arkun, Julian K Wu
BACKGROUND AND IMPORTANCE: Malignant peripheral nerve sheath tumors (MPNST) are relatively rare tumors of peripheral nerves that are notable for their locally aggressive nature, ability to metastasize, poor prognosis, and association with Neurofibromatosis type I. We present the case of a patient with a trigeminal nerve MPNST who developed an unusual metastasis to the corpus callosum, in the absence of any other central nervous system or systemic metastatic disease. We review the pathology and presentation of MPNST...
March 5, 2018: Neurosurgery
Aurore Bouty, Eric Dobremez, Luke Harper, Jérôme Harambat, Cécile Bouteiller, Brigitte Zaghet, Pierre Wolkenstein, Stéphane Ducassou, Yan Lefevre
AIM: Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disorder. Malignant transformation into malignant peripheral nerve sheath tumors (MPNST) can occur. However, urinary tract involvement is rare. We report 4 cases of NF1 with bladder dysfunction. METHODS: A retrospective single center analysis of 4 patients was conducted over a 17-year period, focusing on urinary tract involvement. RESULTS: NF1 was diagnosed at a median of 16...
March 7, 2018: Urologia Internationalis
Santhosh A Upadhyaya, Rose B McGee, Breelyn A Wilky, Alberto Broniscer
Malignant progression of a benign or low-grade tumor in individuals with germline alteration of SMARCB1 gene is not well characterized. In a family in which two carrier children had germline SMARCB1 mutations and atypical teratoid rhabdoid tumor, we report malignant progression of a nerve sheath tumor over a 7-year period in an affected adult family member. Prompt identification of the germline SMARCB1 alteration and the resultant rhabdoid tumor predisposition syndrome can help guide genetic counseling and surveillance in affected family members...
March 7, 2018: Pediatric Blood & Cancer
Jonathan Chica, Isaac Yepes, S Shelby Burks, Ricardo Komotar, Roger Carrillo
Intracranial malignant peripheral nerve sheath tumors (MPNSTs) are an extremely rare entity with only a handful of cases reported in the literature. MPNSTs typically occur in the extremities and the trunk. The treatment algorithm includes, when possible, gross-total resection as these tumors are extremely aggressive. When these tumors occur intracranially, they are termed malignant intracerebral nerve sheath tumors. The diagnosis hinges on immunohistochemistry and pathological features and often the diagnosis can be delayed for this reason...
January 2018: Asian Journal of Neurosurgery
Naohiro Makise, Masaya Sekimizu, Takashi Kubo, Susumu Wakai, Shun-Ichi Watanabe, Tomoyasu Kato, Takayuki Kinoshita, Nobuyoshi Hiraoka, Masashi Fukayama, Akira Kawai, Hitoshi Ichikawa, Akihiko Yoshida
AIMS: Extraskeletal osteosarcoma (ESOS) is a sarcoma in the non-skeletal tissue that directly produces neoplastic osteoid or bone. Dedifferentiated liposarcoma (DDLPS) and malignant peripheral nerve sheath tumor (MPNST) are the two most common types of sarcoma that can harbor heterologous osteosarcomatous differentiation. We aimed to determine the potential relationship of ESOS to DDLPS and MPNST. METHODS AND RESULTS: We investigated MDM2 and H3K27me3 status in 19 cases of ESOS, 2 of which contained a low-grade component...
February 28, 2018: Histopathology
Angela C Hirbe, Jack Jennings, Nael Saad, Joseph D Giardina, Yu Tao, Jingqin Luo, Shellie Berry, Jacqui Toeniskoetter, Brian A Van Tine
LESSONS LEARNED: Ablation therapy appears to be a reasonably safe and effective approach to obtain a significant treatment-free interval for a subset of patients with limited sites of metastatic disease for which systemic control can be obtained with six cycles of chemotherapy. BACKGROUND: Metastatic sarcoma often becomes resistant to treatment by chemotherapy. There is sometimes prolonged stable disease from active chemotherapy that provides a window of opportunity for an intervention to prolong disease-free survival...
February 27, 2018: Oncologist
Hiroshi Otsuka, Kenichi Kohashi, Masato Yoshimoto, Shin Ishihara, Yu Toda, Yuichi Yamada, Hidetaka Yamamoto, Yasuharu Nakashima, Yoshinao Oda
The histological definitive diagnosis of malignant peripheral nerve sheath tumor (MPNST) is quite difficult because the morphological features are not specific and no useful immunohistochemical marker has been identified. Loss-of-function mutations in EED or SUZ12, which encode the core subunit of polycomb repressive complex 2 (PRC2), were reported in MPNSTs, and the mutations were shown to cause inactivation of PRC2, leading to loss of trimethylation of histone H3 at lysine 27 (H3K27me3). Immunohistochemistry of H3K27me3 is expected to be a specific marker for MPNSTs...
January 31, 2018: Pathology, Research and Practice
Valentina Sangiorgio, Vanna Zanagnolo, Giovanni Aletti, Luca Bocciolone, Simone Bruni, Fabio Landoni, Nicoletta Colombo, Angelo Maggioni, Enzo Ricciardi
Cervical sarcomas are rare neoplasms, accounting for <1% of all cervical malignancies and characterized by an aggressive course despite radical excision. We report the clinical and microscopic features of a spindle cell sarcoma arising as a polypoid endocervical mass in a 45-yr-old woman. The neoplasm was characterized by a monotonous, mildly atypical proliferation of spindle cells, displaying a fibrosarcoma-like parallel pattern of highly dense fascicles, growing under the cervical epithelium. Mitotic activity was conspicuous, with up to 40 mitoses per 10 HPF...
February 22, 2018: International Journal of Gynecological Pathology
Russell Payne, Oliver D Mrowczynski, Becky Slagle-Webb, Alexandre Bourcier, Christine Mau, Dawit Aregawi, Achuthamangalam B Madhankumar, Sang Y Lee, Kimberly Harbaugh, James Connor, Elias B Rizk
OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are soft-tissue sarcomas arising from peripheral nerves. MPNSTs have increased expression of the oncogene aurora kinase A, leading to enhanced cellular proliferation. This makes them extremely aggressive with high potential for metastasis and a devastating prognosis; 5-year survival estimates range from a dismal 15% to 60%. MPNSTs are currently treated with resection (sometimes requiring limb amputation) in combination with chemoradiation, both of which demonstrate limited effectiveness...
February 23, 2018: Journal of Neurosurgery
Gorka Zabalo, Daniel de Frutos, Juan Carlos García, Rodrigo Ortega, Juan José Guelbenzu, Idoya Zazpe
We report a case of a 41 years old patient complaining of chronic dorsalgia. MRI showed a well defined intradural extramedular dumbbell-shaped lesion, associated to a left paravertebral tumor at D5-D6 level. The tumor was embolizated prior to surgery. Following she underwent a D4-D6 laminotomy, left D5-D6 costotransversectomy and resection of the intracanal extradural part of the lesion with section of the left D5 nerve. Posteriorly, complete resection of the extracanal portion of the tumor was performed by a postero-lateral approach...
February 19, 2018: Neurocirugía
Mahdi Khajavi Md, Shahrokh Khoshsirat Md, Lida Ahangarnazari Md, Niloofar Majdinasab Md
Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas (NF). It develops as a result of proliferation in all parts of peripheral nervous system and can cause the functional damage, deformities, pain, considerable mortality, and morbidity and even the increasing risk of malignant transformation in some critical cases. Currently, the surgical intervention is the treatment of choice for PNF patients, which due to the tumor invasion, massive growth, and the chance of postoperative regrowth is not possible...
January 10, 2018: Current Problems in Cancer
Manas Bajpai, Nilesh Pardhe
Oral schwannomas (OSs) are rare benign tumors of oral cavity derived from peripheral nerve sheath, composed of proliferation of schwann cells in a characteristic pattern. AS are long-standing lesions which exhibit degenerative changes and atypia due to which they could be mistaken for malignancy. The mean age of occurrence of AS in oral cavity is 43 years, and no case of oral AS has been reported in a pediatric patient so far. Here, we present a rare case of AS of oral cavity in a 10-year-old male child.
May 2017: Journal of Indian Society of Periodontology
Lai Man Natalie Wu, Yaqi Deng, Jincheng Wang, Chuntao Zhao, Jiajia Wang, Rohit Rao, Lingli Xu, Wenhao Zhou, Kwangmin Choi, Tilat A Rizvi, Marc Remke, Joshua B Rubin, Randy L Johnson, Thomas J Carroll, Anat O Stemmer-Rachamimov, Jianqiang Wu, Yi Zheng, Mei Xin, Nancy Ratner, Q Richard Lu
Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive Schwann cell (SC)-lineage-derived sarcomas. Molecular events driving SC-to-MPNST transformation are incompletely understood. Here, we show that human MPNSTs exhibit elevated HIPPO-TAZ/YAP expression, and that TAZ/YAP hyperactivity in SCs caused by Lats1/2 loss potently induces high-grade nerve-associated tumors with full penetrance. Lats1/2 deficiency reprograms SCs to a cancerous, progenitor-like phenotype and promotes hyperproliferation...
February 12, 2018: Cancer Cell
M Laura Feltri, Yannick Poitelon
Current therapies for malignant peripheral nerve sheath tumors (MPNSTs) are ineffective. The study by Wu et al. in this issue of Cancer Cell provides evidence that the HIPPO pathway is overactive in human MPNSTs and that combined modulation of LATS1/2-YAP/TAZ and PDGFR signaling in Schwann cells reduces MPNST growth.
February 12, 2018: Cancer Cell
Christine S Higham, Eva Dombi, Aljosja Rogiers, Sucharita Bhaumik, Steven Pans, Steve E J Connor, Markku Miettinen, Raf Sciot, Roberto Tirabosco, Hilde Brems, Andrea Baldwin, Eric Legius, Brigitte C Widemann, Rosalie E Ferner
Background: Neurofibromatosis 1 (NF1) leads to the development of benign and malignant peripheral nerve sheath tumors (MPNST). MPNST have been described to develop in pre-existing benign plexiform neurofibromas (PN) and have a poor prognosis. Atypical neurofibromas (ANF) were recently described as precursor lesions for MPNST, making early detection and management of ANF a possible strategy to prevent MPNST. We aimed to clinically characterize ANF and identify management approaches. Methods: We analyzed clinical, imaging, and pathology findings of all patients with NF1 and ANF at three institutions...
February 2, 2018: Neuro-oncology
Aijun Liang, Bin Xi, Chaoyang Zhou, Yu Yang, Jianzhong Zhang, Shaogao Gui, Fanghua Xu, Dengfeng Wan
Although benign trigeminal schwannomas are uncommon, malignant peripheral nerve sheath tumors (MPNSTs) of the trigeminal nerve are extraordinarily rare. This study aimed to describe an exceedingly rare case of MPNST of the trigeminal nerve involving the middle and posterior cranial fossa. Only 20 similar cases of MPNSTs of the trigeminal nerve have been reported so far. The clinical characteristics and management of these extremely rare tumors have also been explored in this study.
February 2, 2018: World Neurosurgery
Vincenzo Dario Mandato, Federica Torricelli, Valentina Mastrofilippo, Riccardo Valli, Lorenzo Aguzzoli, Giovanni Battista La Sala
BACKGROUND: Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours' histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes...
February 5, 2018: BMC Cancer
A Agaimy, F Haller, A Hartmann
The last two decades have seen significant advances in the pathology of sinonasal tract neoplasms. This was the consequence of the availability of several innovative diagnostic tools, which resulted in a dynamic evolution of entities and splitting of newly defined or conceptualized entities and subtypes that have been included in the spectrum of old heterogeneous diseases. Most of these new tumor subtypes have distinctive demographic, clinicopathologic, and biological characteristics with prognostic and therapeutic implications for individual patients...
February 2, 2018: Der Pathologe
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