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Peripheral nerve sheath tumor

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https://www.readbyqxmd.com/read/28447903/low-grade-sinonasal-sarcoma-with-neural-and-myogenic-features-a-recently-discovered-entity-with-unique-features-and-diagnostic-challenge
#1
Meriam Triki, Lobna Ayadi
Low-grade sinonasal sarcoma with neural and myogenic features is an entity recently described in the literature. Little is known about its etiopathogenesis, natural history, or optimal treatment. In fact, it has relatively unique findings: it has a distinctive cytogenetic signature, and it expresses both smooth muscle actin and S100 protein. However, its diagnosis is challenging on biopsies showing negative staining for these 2 markers. The differential diagnoses include fibrosarcoma, malignant peripheral nerve sheath tumors, and other benign and malignant lesions...
May 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28443217/malignant-peripheral-nerve-sheath-tumor-of-the-c2-nerve-root-case-report
#2
Joshua D Burks, Robert G Briggs, Chad A Glenn, John C Greenert, Cordell M Baker, Keren Bartal, Greg A Krempl, Michael E Sughrue
Here we present the case of a 36-year-old man who was found to have a symptomatic malignant neural sheath tumor growing from the C2 nerve root following a period of progressively worsening headaches. The patient was successfully treated with surgical resection resulting in resolution of cranial nerve deficits. Though uncommon, malignant peripheral nerve sheath tumor must be considered in the differential diagnosis of tumors involving the cervical nerve roots and carotid space.
April 2017: Journal of Neurological Surgery Reports
https://www.readbyqxmd.com/read/28436992/corrigendum-kank1-inhibits-cell-growth-by-inducing-apoptosis-through-regulating-cxxc5-in-human-malignant-peripheral-nerve-sheath-tumors
#3
Zhibin Cui, Yingjia Shen, Kenny H Chen, Suresh K Mittal, Jer-Yen Yang, GuangJun Zhang
No abstract text is available yet for this article.
April 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28428901/microcystic-reticular-schwannoma-of-the-frontal-lobe-an-unusual-occurrence
#4
Lauren Pearson, Erinc Akture, Julien Wonderlick, Gregory Fuller, Maryam Zenali
Schwannoma is a benign peripheral nerve sheath tumor that typically involves cranial nerves of the head and neck region. Intraparenchymal occurrence of this tumor is uncommon. Even rarer in this site is the microcystic/reticular pattern of schwannoma. This histologic variant, first described in 2008, has a predilection for visceral organs. Herein, we report the first case of microcystic/reticular schwannoma of the frontal lobe.
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28413533/persistent-l5-lumbosacral-radiculopathy-caused-by-lumbosacral-trunk-schwannoma
#5
Guive Sharifi, Amin Jahanbakhshi
Schwannomais, usually, benign tumor of nerve sheath that occurs evenly along the spinal cord. Intra-pelvic schwannoma is very rare entity that may arise from lumbosacral nerve roots or from sciatic nerve. Radicular pain of the lower limb as a presenting symptom of pelvic schwannoma is extremely rare. In the current report, the patient is presented with a right sided L5 radicular pain typical of lumbar discopathy. Interestingly, a herniated lumbar disc was noted on lumbosacral magnetic resonance imaging (MRI)...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28409885/long-term-results-of-the-transmanubrial-osteomuscular-sparing-approach-for-pediatric-tumors
#6
Aziz El Madi, Sabine Irtan, Frédérique Sauvat, Michel Zérah, Gudrun Schleiermacher, Louise Galmiche-Roland, Véronique Minard-Colin, Hervé Brisse, Sabine Sarnacki
BACKGROUND: The complete and safe resection of pediatric cervicothoracic tumors, mostly represented by neurogenic tumors, remains a surgical challenge because of the complex anatomy of this region. The transmanubrial osteomuscular-sparing approach (TOSA) is an alternative to isolated or combined cervical and thoracic approaches enabling the control of supra-aortic vessels and nerves through the thoracic inlet. METHODS: We retrospectively reviewed the tumor characteristics, completeness of resection, morbidity, and long-term outcome of patients with cervicothoracic tumors removed by TOSA between 2000 and 2012 in our institution...
April 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28403573/desmoplastic-melanoma-may-mimic-a-cutaneous-peripheral-nerve-sheath-tumor-report-of-three-challenging-cases
#7
Isidro Machado, Beatriz Llombart, Julia Cruz, Víctor Traves, Celia Requena, Eduardo Nagore, Antonina Parafioriti, Carlos Monteagudo, Antonio Llombart-Bosch
Desmoplastic melanoma (DM) and cutaneous malignant peripheral nerve sheath tumors (MPNST) reveal histological and immunohistochemical similarities, including S100 positivity and negative staining for conventional melanocytic markers. We present three cases of cutaneous S100-positive spindle cell tumors in elderly patients, in which first findings led to initial misdiagnoses as cutaneous MPNST and benign peripheral sheath nerve tumor (neurofibroma). The identification of adjacent atypical melanocytic hyperplasia in the overlying skin along with tumor cell proliferation, also in the superficial dermis, the neurotropic component and the absence of any relationship between the tumor and a major nerve, pre-existing neural benign tumor or the existence of stigmata suggestive of neurofibromatosis raised consideration of a desmoplastic melanoma...
April 12, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28394552/multiple-intracranial-schwannomas-case-report
#8
Mario Mihalj, Krešimir Dolić, Pavao Jurinović, Nikolina Ivica Miše, Marina Titlić, Irena Pintarić
Schwannomas are benign encapsulated tumors arising from the sheaths of peripheral nerves. They present as slowly enlarging solitary lumps, which may cause neurological defects. Multiple schwannomas in non-neurofi bromatosis type 2 patients are extremely rare. We report a case of a 60-year-old female patient, without any family history of neurofibromatosis or schwannomatosis, presented with trigeminal neuralgia and progressive facial nerve palsy. Magnetic resonance imaging revealed the presence of acoustic schwannoma involving facial nerve and trigeminal schwannoma of the cisternal part of the nerve involving gasserian ganglion (Meckel’s cave)...
June 2016: Acta Clinica Croatica
https://www.readbyqxmd.com/read/28392281/human-stem-cell-modeling-in-neurofibromatosis-type-1-nf1
#9
REVIEW
Michelle L Wegscheid, Corina Anastasaki, David H Gutmann
The future of precision medicine is heavily reliant on the use of human tissues to identify the key determinants that account for differences between individuals with the same disorder. This need is exemplified by the neurofibromatosis type 1 (NF1) neurogenetic condition. As such, individuals with NF1 are born with a germline mutation in the NF1 gene, but may develop numerous distinct neurological problems, ranging from autism and attention deficit to brain and peripheral nerve sheath tumors. Coupled with accurate preclinical mouse models, the availability of NF1 patient-derived induced pluripotent stem cells (iPSCs) provides new opportunities to define the critical factors that underlie NF1-associated nervous system disease pathogenesis and progression...
April 6, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28387148/malignant-triton-tumor-malignant-peripheral-nerve-sheath-tumor-with-rhabdomyoblastic-differentiation-occurring-in-a-vascularized-free-flap-reconstruction-graft
#10
Roopa Ram, Jerad Gardner, Sindhura Alapati, Kedar Jambhekar, Tarun Pandey, Corey Montgomery, Richard Nicholas
Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT) is a subtype of MPNST with a component of malignant rhabdomyoblasts in addition to malignant Schwann cells. MPNST and MTT are both aggressive malignancies that most commonly arise from large deep neurofibromas in patients with neurofibromatosis type 1 (NF-1). However, sporadic non-NF-1 cases of MTT have also been reported in the literature...
April 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28381143/malignant-peripheral-nerve-sheath-tumor-with-divergent-glandular-differentiation
#11
Yurina Miki, Khin Thway
Malignant peripheral nerve sheath tumors (MPNST) are soft tissue neoplasms with evidence of nerve sheath differentiation. They usually arise from peripheral nerves or from preexisting benign nerve sheath neoplasms, often in patients with neurofibromatosis type 1 (NF1). The histologic diagnosis of MPNST is challenging as their morphology is highly variable, and there has been a lack of routine diagnostic immunohistochemical markers and specific genetic aberrations. Although divergent differentiation is well documented in MPNST, it is most frequently toward mesenchymal elements...
March 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28371884/neurofibromatosis-type-1-disseminated-malignant-peripheral-nerve-sheath-tumor
#12
Ashok Kumar Pannu, Navneet Sharma
No abstract text is available yet for this article.
March 24, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28368924/smarcb1-ini1-loss-in-epithelioid-schwannoma-a-clinicopathologic-and-immunohistochemical-study-of-65-cases
#13
Vickie Y Jo, Christopher D M Fletcher
The epithelioid variant of schwannoma is rare, and loss of SMARCB1/INI1 expression has been observed in a subset of cases. Our aim was to further define the clinicopathologic features and to evaluate SMARCB1/INI1 deficiency in a large cohort of 65 epithelioid schwannomas diagnosed between 2002 and 2015, which consisted of 32 men and 33 women with median age at diagnosis of 45 years (range, 13 to 75 y). Most tumors arose in the extremities (upper, 20, lower, 15) and trunk (17); 9 were visceral (8 gastrointestinal)...
March 31, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28349408/cancer-of-the-peripheral-nerve-in-neurofibromatosis-type-1
#14
REVIEW
Verena Staedtke, Ren-Yuan Bai, Jaishri O'Neill Blakeley
The RASopathy neurofibromatosis 1 is an autosomal dominant hereditary cancer syndrome that represents a major risk for the development of malignancies, particularly malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are unique sarcomas that originate from the peripheral nerve and represent the only primary cancer of the peripheral nervous system. To date, surgery is the only treatment modality proven to have survival benefit for MPNSTs and even when maximal surgery is feasible, these tumors are rarely curable, despite the use of chemotherapy and radiation...
March 27, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28341644/nerve-ultrasound-a-useful-screening-tool-for-peripheral-nerve-sheath-tumors-in-nf1
#15
Johan A Telleman, Menno D Stellingwerff, Geert J Brekelmans, Leo H Visser
OBJECTIVE: To determine ultrasonographic peripheral nerve involvement in patients with asymptomatic neurofibromatosis type 1 (NF1). METHODS: Thirteen asymptomatic and 4 minimally symptomatic patients with NF1 were included in this cross-sectional pilot study to detect asymptomatic abnormalities of the brachial plexus and upper and lower extremity nerves. Patients underwent clinical examination, nerve conduction studies (NCS), and high-resolution ultrasonography (HRUS)...
March 24, 2017: Neurology
https://www.readbyqxmd.com/read/28340171/immunohistochemical-approach-to-the-differential-diagnosis-of-meningiomas-and-their-mimics
#16
Camille Boulagnon-Rombi, Clémence Fleury, Caroline Fichel, Sophie Lefour, Aude Marchal Bressenot, Guillaume Gauchotte
The differential diagnosis between meningioma and others tumors can be challenging. This study aimed to evaluate different immunohistochemical markers for the differential diagnosis between meningioma and their morphological mimics. Immunohistochemistry was performed on tissue microarray with antiepithelial membrane antigen (EMA), progesterone receptor, somatostatin receptor 2A (SSTR2A), CD34, STAT6, S100, SOX10, HMB45, MelanA, GFAP, inhibin, and BCL2 antibodies. One hundred and twenty-seven meningiomas, 26 solitary fibrous tumor/hemangiopericytomas (SFT/HPC), 39 schwannomas, 17 hemangioblastomas, 21 melanomas, 9 gliosarcomas, 5 neurofibromas, 9 peripheral primitive neuroectodermal tumors, 7 synovial sarcomas, and 5 malignant peripheral nerve sheath tumors were included in the microarray...
April 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28335699/approaches-to-paraspinal-tumours-a-technical-note
#17
Arjun Dhar, Sumeet Pawar, Apurva Prasad, P S Ramani
Neurogenic tumours of the paraspinal space can occur in all age groups. It is common in adult population and relatively rare in elderly group. Usually they are benign, but in children, arising from the autonomic system, tends to be malignant in nature. Usually in adults, they arise from peripheral nerve sheath and are labelled as schwannomas. For a given tumour, determination of a correct surgical approach is mandatory to achieve a successful surgical outcome. Several factors like tumour size, histology, involvement of the bony spinal canal, etc...
April 2017: Neurological Research
https://www.readbyqxmd.com/read/28320420/malignant-gastrointestinal-neuroectodermal-tumor-a-case-report-and-review-of-the-literature
#18
REVIEW
Mohammed J Alyousef, Jumana A Alratroot, Tarek ElSharkawy, Mohamed A Shawarby, Mohammad A Al Hamad, Tarek M Hashem, Ahmed Alsayyah
BACKGROUND: Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare entity that was first described by Zambrano et al. in 2003 as "Clear cell sarcoma-like tumor of the gastrointestinal tract". It shares some of the histological features of clear cell sarcoma (CCS) but lacks the immunohistochemical reactivity for melanocytic markers. We report a case of GNET that was initially misdiagnosed as gastrointestinal stromal tumor (GIST). Recognizing this entity is important to avoid misdiagnosis...
March 20, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28286620/a-case-of-schwannoma-of-the-common-peroneal-nerve-in-the-knee
#19
Tayyar Taylan Öz, Birol Aktaş, Korhan Özkan, Burak Özturan, Bulent Kilic, Murat Demiroğlu
Schwannomas are benign neurogenic tumors of peripheral nerves that originate from neural sheath. The aim of this case report is to bring to mind the schwannoma in the patient with knee pain. A 39-year-old woman presented with a complaint of knee pain of three months history. After surgical intervention, the patient's complaints completely disappeared in the post-operative period. This should come to mind in the differential diagnosis of knee pain.
February 20, 2017: Orthopedic Reviews
https://www.readbyqxmd.com/read/28258916/malignant-peripheral-nerve-sheath-tumor-of-the-tongue-with-an-unusual-pattern-of-recurrence
#20
Soumyajit Roy, Ajeet Kumar Gandhi, Bharti Devnani, Lavleen Singh, Bidhu Kalyan Mohanti
Malignant peripheral nerve sheath tumor (MPNST) of oral cavity is an extremely uncommon malignancy. Less than 15 cases have been reported since 1973 though none of them describes a distant metastasis. We present a rare case of MPNST of the tongue who presented with features of hypoglossal nerve palsy. Incisional biopsy showed a malignant spindle cell tumor in the sub-epithelial connective tissue. The tumor cells were immune-positive for S-100. He underwent surgery followed by adjuvant chemo-radiation. Later the disease recurred in the form of isolated pelvic bone metastasis...
February 28, 2017: Journal of the Egyptian National Cancer Institute
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