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Plexiform schwannoma

Satoru Kudose, Michael Kyriakos, Michael Magdi Awad
: Plexiform schwannoma (PS) is an uncommon variant of schwannoma characterized by a multinodular (plexiform) growth pattern. It comprises up to 5 % of all schwannomas. The association between PS and neurofibromatosis type 1 or type 2 (NF1/NF2) is only rarely reported. Most cases of PS occur in the skin and subcutaneous soft tissue, with only a few reports of digestive tract involvement. We describe an 18-year-old male with NF2 who had bilateral vestibular schwannomas and multiple cutaneous PSs, and a 3-year history of abdominal pain...
September 30, 2016: Clinical Journal of Gastroenterology
Sahejmeet S Guraya, Richard A Prayson
Primary salivary gland peripheral nerve sheath tumors (PNST) are uncommon. This study is a retrospective, clinicopathologic review of 9 cases of PNST (5 neurofibromas, 3 schwannomas and 1 malignant peripheral nerve sheath tumor (MPNST)) arising from the salivary glands, encountered between 1990 and 2015. All patients with neurofibromas were male (ages 1-62 years) and had a single parotid lesion of which 2 were diffuse, 2 plexiform and one mixed diffuse/plexiform. Four had a history of neurofibromatosis I. Four of 5 presented with symptoms related to mass effect including facial swelling, facial drooping, and dysphagia...
August 2016: Annals of Diagnostic Pathology
Panduranga Chikkannaiah, Mythri M Boovalli, Velusamy Nathiyal, Srinivasamurthy Venkataramappa
INTRODUCTION: Peripheral nerve sheath tumors (PNSTs) are neuroectodermal in origin. Now these tumors are classified under World Health Organization (WHO) classification of tumors of soft tissue and bone 2013. OBJECTIVE: To study the morphological spectrum of PNST and to study the secondary degenerative changes associated with it. MATERIALS AND METHODS: This study was conducted from January 2010 to June 2015. The gross details of tumor and patient's demographic profiles were reviewed...
July 2016: Journal of Neurosciences in Rural Practice
Arjen H G Cleven, Ghadah A Al Sannaa, Inge Briaire-de Bruijn, Davis R Ingram, Matt van de Rijn, Brian P Rubin, Maurits W de Vries, Kelsey L Watson, Kelia E Torres, Wei-Lien Wang, Sjoerd G van Duinen, Pancras C W Hogendoorn, Alexander J Lazar, Judith V M G Bovée
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas that can show overlapping features with benign neurofibromas as well as high-grade sarcomas. Additional diagnostic markers are needed to aid in this often challenging differential diagnosis. Recently mutations in two critical components of the polycomb repressor 2 (PRC2) complex, SUZ12 and EED, were reported to occur specifically in MPNSTs while such mutations are absent in neurofibromas, both in the setting of neurofibromatosis (NF) and sporadic cases...
June 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Bryan Lieber, ByoungJun Han, Jeffrey Allen, Girish Fatterpekar, Nitin Agarwal, Noojan Kazemi, David Zagzag
Schwannomatosis is characterized by multiple non-intradermal schwannomas with patients often presenting with a painful mass in their extremities. In this syndrome malignant transformation of schwannomas is rare in spite of their large size at presentation. Non-invasive measures of assessing the biological behavior of plexiform neurofibromas in neurofibromatosis type 1 such as positron emission tomography (PET), CT scanning and MRI are well characterized but little information has been published on the use of PET imaging in schwannomatosis...
August 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Manuel Röhrich, Christian Koelsche, Daniel Schrimpf, David Capper, Felix Sahm, Annekathrin Kratz, Jana Reuss, Volker Hovestadt, David T W Jones, Melanie Bewerunge-Hudler, Albert Becker, Joachim Weis, Christian Mawrin, Michel Mittelbronn, Arie Perry, Victor-Felix Mautner, Gunhild Mechtersheimer, Christian Hartmann, Ali Fuat Okuducu, Mirko Arp, Marcel Seiz-Rosenhagen, Daniel Hänggi, Stefanie Heim, Werner Paulus, Jens Schittenhelm, Rezvan Ahmadi, Christel Herold-Mende, Andreas Unterberg, Stefan M Pfister, Andreas von Deimling, David E Reuss
The vast majority of peripheral nerve sheath tumors derive from the Schwann cell lineage and comprise diverse histological entities ranging from benign schwannomas and neurofibromas to high-grade malignant peripheral nerve sheath tumors (MPNST), each with several variants. There is increasing evidence for methylation profiling being able to delineate biologically relevant tumor groups even within the same cellular lineage. Therefore, we used DNA methylation arrays for methylome- and chromosomal profile-based characterization of 171 peripheral nerve sheath tumors...
June 2016: Acta Neuropathologica
Hillary Elwood, Janis Taube
This article presents an overview of soft tissue tumors that have a plexiform histomorphology. The more commonly encountered entities, including plexiform fibrohistiocytic tumor, cellular neurothekeoma, dermal nerve sheath myxoma, plexiform schwannoma, and plexiform neurofibroma, are discussed in detail, and other tumors are noted. Information on clinical features, microscopic findings, ancillary studies, differential diagnosis, and prognosis is provided for each entity.
September 2011: Surgical Pathology Clinics
Subha Dhua
An 18-year-old female presented with a swelling on the lower lip, which was insidious in onset and gradually progressive. The mass was completely excised under local anaesthesia. Complete histopathologic and immunohistochemical studies were conducted. The Antoni A areas were found along with typical verocay bodies composed of palisading nuclei and surrounding spaces filled with eosinophilic filaments. No necrosis was noted and there were no atypical mitotic figures. In the Antoni B region, a closely textured matrix with areas of edema, myxomatous changes, cystic degeneration and dilated vessels were noted...
May 2015: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
Masashi Nagata, Hiroyuki Ito, Tomohiko Matsuzaki, Hideyuki Furumoto, Tetsuya Isaka, Teppei Nishii, Tomoyuki Yokose, Haruhiko Nakayama
Plexiform schwannoma is an infrequent variant of schwannoma characterized grossly and microscopically by multi-nodular growth. Although plexiform schwannoma has such growth patterns, it is a benign tumor as well as a conventional schwannoma. It rarely infiltrates adjacent organs or arises from the organ itself. In this report, we describe a case in which plexiform schwannoma involved the tracheal wall and left recurrent laryngeal nerve to a great extent. As it was expected to be difficult to achieve complete resection even if the longer tracheal resection were performed, we preserved the trachea and resected as much of the tumor as possible...
2015: Surgical Case Reports
María del Carmen Gómez-Mateo, Amparo Compañ-Quilis, Carlos Monteagudo
Glandular structures are well documented to appear in peripheral nerve sheath tumors. These epithelial elements are usually present in malignant peripheral nerve sheath tumors although a few cases of glandular benign peripheral nerve sheath tumors have also been described, most of them being schwannomas. A neurofibroma with glands is considered to be a rare type of divergent differentiation, but a neurofibroma containing gland-like or pseudoglandular structures have not, to our knowledge, been described. We report a 33-year-old patient with a well-demarcated dermal neoplasm, composed of neoplastic Schwann cells, perineurial-like cells and fibroblasts in a matrix with collagen fibers and myxoid areas...
November 2015: Journal of Cutaneous Pathology
Fumio Ide, Takashi Muramatsu, Kentaro Kikuchi, Ichiro Saito, Kaoru Kusama
Rare epithelial structures in benign nerve sheath tumors are almost always glandular in appearance. We describe a case of intraoral plexiform schwannoma with concurrent squamous epithelial hyperplasia. The lesion occurred as a pigmented nodule on the gingiva of a 35-year-old woman with no systemic involvement. Histologically, unencapsulated, plexiform fascicular proliferations of schwann cells could be traced from the submucosa to the lamina propria, finally making direct contact with heavily pigmented, elongated rete ridges of the overlying epithelium...
August 13, 2015: Journal of Cutaneous Pathology
Reinhard E Friedrich, Urs Naber, Markus Glatzel, Christian Hagel
BACKGROUND/AIM: Peripheral nerve sheath (PNS) tumors constitute a heterogeneous group of solid tumors. Neurofibroma and schwannoma are the most frequently diagnosed entities. Both tumor types occur sporadically and are associated with syndromes. Current strategies to fight PNS progression by means of pharmaceuticals aim to specifically interfere with vascular growth factors identified in PNS. Furthermore, malignant transformation of PNS tumors is known to be associated with a change in vascularization...
September 2015: Anticancer Research
Jun Nishio, Shun Mori, Kazuki Nabeshima, Masatoshi Naito
BACKGROUND: It is often challenging to completely resect multinodular/plexiform schwannomas involving important deep nerves using minimally invasive surgically techniques. CASE DESCRIPTION: A 32-year-old woman presented with a 5-year history of a slowly growing, painful mass in the medial aspect of the right ankle. Magnetic resonance imaging (MRI) demonstrated multiple nodular lesions with iso-signal intensity relative to skeletal muscle on T1-weighted sequences and heterogeneous high signal intensity on T2-weighted sequences...
2015: SpringerPlus
Asmita Parihar, Sarika Verma, Tarun Suri, Anil Agarwal, Kalpana Bansal, Ruchika Gupta
Plexiform schwannoma is an unusual peripheral nerve sheath tumor. It can mimic plexiform neurofibroma. A five-year-old girl presented with painful swelling in left lumbar region. Radiologic investigations showed a multinodular tumor in the subcutaneous plane of lumbosacral region. A complete excision and histopathologic examination revealed a plexiform tumor composed of hypocellular and hypercellular areas with verocay bodies. The tumor cells showed strong positivity for S-100 protein, rendering a final diagnosis of plexiform schwannoma...
May 2015: APSP Journal of Case Reports
Qunfeng Yao
Summary A 36 years old patient with hoarseness for 2 years and got worsen for one month, electronic laryngoscopy showed a red smooth-faced wide based neoplasm on the posterior 2/3 of the right side of the vocal cords. The neoplasm was excised under suspension laryngoscope . The pathologic results showed:Cells were weave patterned, infiltrative growth, mitotic figure was rare. Immunohistochemical results showed CD34 (-), SMA (-), DM (-), S - 100 (+). The pathological diagnosis was plexiform schwannoma.
December 2014: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Luca Raimondo, Massimiliano Garzaro, Jasenka Mazibrada, Giancarlo Pecorari, Carlo Giordano
We report a case of plexiform schwannoma of the posterior pharyngeal wall that occurred in a 37-year-old man who had been previously diagnosed with neurofibromatosis type 2 (NF2). Plexiform schwannoma has been rarely reported in association with NF2. Moreover, as far as we know, only 6 cases of posterior pharyngeal wall schwannoma have been previously reported in the literature, none of which was associated with NF2 and none of which was histopathologically differentiated in schwannoma or plexiform schwannoma...
March 2015: Ear, Nose, & Throat Journal
Xiao-na Li, Jian-ling Cui, Seemon Petrus Christopasak, Abhinav Kumar, Zhi-gang Peng
BACKGROUND: Plexiform schwannoma (PS) is a rare, peripheral nerve sheath tumor arranged in a plexiform pattern. CASE PRESENTATION: We report an unusual case of a 19-year-old woman, who complained of pain in the plantar aspect of the left foot. Magnetic resonance image (MRI) demonstrates three solitary nodules of varying sizes in the deep soft tissue of the plantar aspect of the foot that are homogeneously isointense to skeletal muscle on T1-weighted images and hyperintense on T2-weighted fat-suppressed images, especially the rim of the lesion...
2014: BMC Musculoskeletal Disorders
Kanji Mori, Shinji Imai, Kazuya Nishizawa, Akira Nakamura, Mitsuaki Ishida, Yoshitaka Matsusue
No abstract text is available yet for this article.
January 1, 2015: Spine Journal: Official Journal of the North American Spine Society
Erkam Komurcu, Burak Kaymaz, Gurhan Adam, Ferhat Gokmen, Asli Murath
Schwannomas are the most common benign neoplasms of the peripheral nerves in the upper extremity originating from the schwann cells of nerve sheath. They account for 5% of all tumours in upper extremity. Schwannomas are usually solitary, encapsulated and homogeneous masses and present with slowly growing masses, sometimes associated with pain and paresthesia. Pre-operative evaluation is based on ultrasonography and magnetic resonance imaging, but final diagnosis requires histopathology. It is important to distinguish plexiform schwannoma from plexiform neurofibroma because of the possibility of malignant transformation in plexiform neurofibroma...
March 2014: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Samirah A Mohammed, Martin M Pressman, Brian Schmidt, Nina Babu
Plexiform schwannoma is a soft tissue tumor that rarely presents in the lower extremity. We have reported on 2 cases of plexiform schwannomas that occurred in the foot and ankle. Owing to the relative obscurity of this condition in the lower extremity, we have also provided an overview of plexiform schwannomas and discussed the differences between this and other suspicious nodular soft tissue masses. The first patient was a 38-year-old female, who had a soft tissue mass located on the lateral aspect of her right foot that became symptomatic...
March 2014: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
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