Perineurioma: A Distinctive and Underrecognized Peripheral Nerve Sheath Neoplasm

2007 ◽  
Vol 131 (4) ◽  
pp. 625-636
Author(s):  
Ricardo S. Macarenco ◽  
Fred Ellinger ◽  
Andre M. Oliveira
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Molecular Genetic ◽  
S100 Protein ◽  
Correct Identification ◽  
Histologic Diagnosis ◽  
Genetic Studies ◽  
Nerve Sheath ◽  
Immunohistochemical Studies ◽  
Perineurial Cells

Abstract Context.—Perineuriomas are benign peripheral nerve sheath neoplasms composed of perineurial cells with characteristic immunohistochemical and ultrastructural features. They have been traditionally classified into two main types according to their location—intraneural and extraneural—and overlap histologically with many other tumors, which may be diagnostically challenging to general surgical pathologists. Objective.—To review the clinical, morphologic, immunohistochemical, ultrastructural, cytogenetic, and molecular genetic aspects of perineurioma, as well as to discuss its clinicopathologic variants and differential diagnosis. Data Sources.—English-language literature published between 1966 and 2005 was reviewed. Conclusions.—The correct identification of perineuriomas is important to avoid unnecessary overtreatment. The histologic diagnosis should be confirmed through immunohistochemical studies (including epithelial membrane antigen, S100 protein, and more recently described antibodies such as claudin-1 and GLUT1) or electron microscopy. Cytogenetic and molecular genetic studies are still of limited value for the diagnosis of perineuriomas but may play a fundamental role in excluding important differential diagnoses and also in helping elucidate the biology of these poorly known neoplasms.

Malignant Peripheral Nerve Sheath Tumor of the Parotid Gland

2003 ◽  
Vol 112 (7) ◽  
pp. 637-643 ◽  
Author(s):  
Shun-Ichi Imamura ◽  
Haruko Suzuki ◽  
Shin-Ichi Usami ◽  
Eiki Koda ◽  
Akihiko Yoshizawa
Keyword(s):  
Parotid Gland ◽  
Peripheral Nerve ◽  
Head And Neck ◽  
English Language ◽  
Immunohistochemical Analysis ◽  
Radical Resection ◽  
Nerve Sheath Tumor ◽  
Histologic Diagnosis ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath

Malignant peripheral nerve sheath tumor (MPNST) has been defined as any malignant tumor arising from or differentiating toward cells of the peripheral nerve sheath. We treated a case of MPNST arising from the right parotid gland that showed a highly aggressive course. We reviewed the English-language literature published since 1990 and found 142 cases of head and neck MPNST reported within the past 13 years. The results of the review suggested that MPNSTs may arise from any organs of the head and neck. Immunohistochemical analysis of various neural markers plays a significant role in the evaluation of the histologic diagnosis. Curative treatment based on radical resection of MPNSTs of head and neck origin is more difficult than treatment of MPNSTs of other origins.

scholarly journals Erratum: Malignant Peripheral Nerve Sheath Tumors with t(X;18). A Pathologic and Molecular Genetic Study

2000 ◽  
Vol 13 (12) ◽  
pp. 1336-1346 ◽  
Author(s):  
Maureen J O'Sullivan ◽  
Michael Kyriakos ◽  
Xiaopei Zhu ◽  
Mark R Wick ◽  
Paul E Swanson ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Genetic Study ◽  
Molecular Genetic ◽  
Molecular Genetic Study ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Tyrosinase Expression in Malignant Melanoma, Desmoplastic Melanoma, and Peripheral Nerve Tumors

2002 ◽  
Vol 126 (7) ◽  
pp. 816-822 ◽  
Author(s):  
Jenny L. Boyle ◽  
Helen M. Haupt ◽  
Jere B. Stern ◽  
Hinke A. B. Multhaupt
Keyword(s):  
Peripheral Nerve ◽  
S100 Protein ◽  
Dermatofibrosarcoma Protuberans ◽  
Antigen Retrieval ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Desmoplastic Melanoma ◽  
Nerve Sheath ◽  
Tyrosinase Expression ◽  
Hmb 45

Abstract Context.—Pathologists may encounter problems in the differential diagnosis of malignant melanoma, spindle and epithelioid neoplasms of peripheral nerves, and fibrohistiocytic tumors. Tyrosinase has been demonstrated to be a sensitive marker for melanoma. Objective.—To determine the specificity of tyrosinase expression in the differential diagnosis of melanoma, desmoplastic melanoma, and peripheral nerve sheath tumors. Design.—Immunoreactivity for tyrosinase, HMB-45 (anti-gp100 protein), S100 protein, CD34, and vimentin was studied in 70 tumors, including 15 melanomas (5 desmoplastic, 4 amelanotic, 6 melanotic), 13 malignant peripheral nerve sheath tumors; 10 schwannomas (1 pigmented), 12 neurofibromas (4 pigmented), and 20 fibrohistiocytic tumors (10 dermatofibrosarcoma protuberans and 10 dermatofibromas). Microwave-based antigen retrieval was performed in 10mM citrate buffer, pH 6.0, for 20 minutes at 121°C. Results.—All melanomas demonstrated positive immunostaining for tyrosinase, HMB-45, and S100 protein. Immunoreactivity for HMB-45 was generally stronger than that for tyrosinase in amelanotic lesions and significantly stronger in 1 of the desmoplastic lesions. The 4 pigmented neurofibromas were focally positive for tyrosinase, but did not stain for HMB-45. The pigmented schwannoma was focally positive for both tyrosinase and HMB-45. The malignant peripheral nerve sheath tumors, dermatofibrosarcoma protuberans, and dermatofibromas were nonreactive for tyrosinase and HMB-45. Conclusions.—Our results support the sensitivity of tyrosinase expression and demonstrate the relative specificity of tyrosinase as a marker for melanocytic lesions, including desmoplastic melanoma, although pigmented peripheral nerve tumors may demonstrate focal positive staining. Immunoreactivity for tyrosinase and HMB-45 may have been enhanced by the microwave-based antigen-retrieval technique used in this study.

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

2014 ◽  
Vol 21 (3) ◽  
pp. 367-371 ◽  
Author(s):  
Yaxiong Li ◽  
Fengshi Fan ◽  
Jianguo Xu ◽  
Jie An ◽  
Weining Zhang
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Standard Of Care ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
History Of ◽  
Brain Ct Scan ◽  
The Right

Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

scholarly journals The Ultrastructure of Bovine Peripheral Nerve Sheath Tumours

1978 ◽  
Vol 15 (3) ◽  
pp. 292-300 ◽  
Author(s):  
P. Canfield
Keyword(s):  
Peripheral Nerve ◽  
Schwann Cells ◽  
Extracellular Material ◽  
Cell Nuclei ◽  
Principal Cells ◽  
Nerve Sheath ◽  
Cytoplasmic Processes ◽  
Antoni Type ◽  
Perineurial Cells ◽  
Electron Lucent

Bovine peripheral nerve sheath tumours from 30 cattle were similar ultrastructurally to human schwannomas and neurofibromas. Bovine neurofibromatous tissue had large amounts of extracellular material, primarily collagen and electron lucent granular material. The principal cells had basal laminae and a disorganized proliferation of the plasmalemma. Axons were consistently seen and were surrounded by the plasmalemma of principal cells. The principal cells seemed to be Schwann cells or variants of them. Bovine schwannomas had areas similar to Antoni type A tissue with sparse extracellular material, few, if any, axons, and an apparent organized layering of cytoplasmic processes clad in a basal lamina. Cell nuclei often formed palisades. The principal cells in bovine schwannomas might be derived either from Schwann cells or perineurial cells. Bovine schwannomas appeared together with bovine neurofibromatous tissue in affected nerves.

Cerebral Metastasis from an Epithelioid Malignant Schwannoma: Case Report

Neurosurgery ◽  
1991 ◽  
Vol 29 (6) ◽  
pp. 906-909 ◽  
Author(s):  
Vincenzo D'Angelo ◽  
Gianpiero Casadei ◽  
Luigi Bizzozero
Keyword(s):  
Case Report ◽  
Brain Metastasis ◽  
Peripheral Nerve ◽  
Spindle Cell ◽  
S100 Protein ◽  
Neuron Specific Enolase ◽  
Cerebral Metastasis ◽  
Malignant Schwannoma ◽  
Nerve Sheath ◽  
The Right

Abstract The authors present a case of brain metastasis from an epithelioid malignant schwannoma. The patient previously had undergone a surgical resection of the primary tumor in the right forearm. The neoplasm was composed of nests of cells with an entirely epithelioid appearance without spindle cell areas. Immunohistochemically, the tumor cells stained positive for S100 protein and negative for cytokeratin, neuron-specific enolase, and anti-melanoma antiserum. To our knowledge, this is the first reported case of cerebral metastasis from an epithelioid malignant tumor of the peripheral nerve sheath.

scholarly journals Malignant Peripheral Nerve Sheath Tumors with t(X;18). A Pathologic and Molecular Genetic Study

2000 ◽  
Vol 13 (11) ◽  
pp. 1253-1263 ◽  
Author(s):  
Maureen J O'Sullivan ◽  
Michael Kyriakos ◽  
Xiaopei Zhu ◽  
Mark R Wick ◽  
Paul E Swanson ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Genetic Study ◽  
Molecular Genetic ◽  
Molecular Genetic Study ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

INNV-04. A MULTI-INSTITUTIONAL CLINICAL AND MRI REPOSITORY OF NEUROFIBROMATOSIS TYPE 1-ASSOCIATED PERIPHERAL NERVE SHEATH TUMORS

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi105-vi106
Author(s):  
Ina Ly ◽  
Angela Hirbe ◽  
Justin Jordan ◽  
Olivia Michaels ◽  
Daniel Kwon ◽  
...  
Keyword(s):  
Risk Factors ◽  
Peripheral Nerve ◽  
Clinical Data ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Histologic Diagnosis ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Abstract BACKGROUND Individuals with neurofibromatosis type 1 (NF1) frequently have peripheral nerve sheath tumors (PNST), including plexiform neurofibromas (PNF), atypical neurofibromas (ANF), and malignant peripheral nerve sheath tumors (MPNST). These tumors reflect a histologic spectrum from benign to malignant. Various clinical and MRI-based features are proposed as risk factors for MPNST development based on small single-institution studies. A major barrier to study these risk factors is collation and annotation of multi-center serial MRIs. To address this, we created a standardized database of clinical data and longitudinal MRIs from NF1-associated PNST from nine international NF1 referral centers. METHODS Clinical data from NF1 patients are collected in Research Electronic Data Capture databases housed at Massachusetts General Hospital and Washington University, including demographic information, genotype, disease course, treatment history, and survival. ANF and MPNST require histologic confirmation whereas a diagnosis of PNF can also be made based on clinical/radiographic stability. Longitudinal MRIs predating the histologic diagnosis are uploaded to a HIPAA-compliant cloud-based system. RESULTS Data from 200 patients (87 females, 113 males) with 217 tumors (75 PNF, 40 ANF, 102 MPNST) have been collected. 280 regional and 108 whole-body MRIs have been identified. Median age at the time of histologic diagnosis is 30 years (range 5-64). All tumors are histologically confirmed except for 6 PNF which remained stable over time. Median follow-up time is 32 months. Of 147 patients with available survival data, 32 (21.7%) have died from MPNST progression; estimated median overall survival is 20 months. CONCLUSIONS In this ongoing work, we have assembled one of the largest systematically annotated clinical and MRI repositories of NF1-associated PNST from pediatric and adult NF1 patients. The data will be accessible to outside researchers which will promote interdisciplinary and multi-center collaborations. Active efforts include the identification of radiomic MRI features to differentiate between PNF and MPNST.

Expression of Protein Gene Product 9.5 in Epithelioid and Conventional Malignant Peripheral Nerve Sheath Tumors

2001 ◽  
Vol 125 (10) ◽  
pp. 1321-1325
Author(s):  
Mai P. Hoang ◽  
Prasanna Sinkre ◽  
Jorge Albores-Saavedra
Keyword(s):  
Peripheral Nerve ◽  
Protein Gene ◽  
S100 Protein ◽  
Dermatofibrosarcoma Protuberans ◽  
Protein Gene Product 9.5 ◽  
Nerve Sheath Tumors ◽  
Protein Gene Product ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Synovial Sarcomas

Abstract Context.—Due to the frequent lack of S100 protein expression in malignant peripheral nerve sheath tumors (MPNSTs), especially the epithelioid variant, these tumors are difficult to diagnose without the aid of electron microscopy or a clinical history of neurofibromatosis. Methods.—Protein gene product 9.5 (PGP9.5), a broad neural marker, is expressed in nerve fibers and neurons of both the peripheral and central nervous systems. We compared its expression to that of S100 protein in 16 cases of MPNST. As controls, 6 monophasic synovial sarcomas, 9 leiomyosarcomas, and 5 dermatofibrosarcoma protuberans were included. Results.—Expression of PGP9.5 was seen in 15 MPNSTs, with 3 to 4+ positivity in the majority of the cases. Ten cases, 2 epithelioid and 8 conventional MPNSTs, were reactive with PGP9.5, but were negative for S100 protein. Five cases were immunoreactive for both S100 protein and PGP9.5. One case was negative for PGP9.5 but demonstrated focal S100 protein positivity. Expression of PGP9.5 was seen in 4 of 6 synovial sarcomas, 3 of 9 leiomyosarcomas, and none of 5 dermatofibrosarcoma protuberans. Conclusion.—Although PGP9.5 is not a specific marker for MPNST, it is a more sensitive marker than S100 protein (94% vs 38%). When there is a lack of S100 protein expression and a broad panel of immunostains, such as cytokeratin, epithelial membrane antigen, and smooth muscle actin, yields only focal or equivocal staining, PGP9.5 is a useful diagnostic adjunct in confirming the neural origin of a spindle cell sarcoma.

Malignant Peripheral Nerve Sheath Tumor With Divergent Glandular Differentiation

2017 ◽  
Vol 25 (4) ◽  
pp. 310-313 ◽  
Author(s):  
Yurina Miki ◽  
Khin Thway
Keyword(s):  
Differential Diagnosis ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Histologic Diagnosis ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Immunohistochemical Markers ◽  
Nerve Sheath ◽  
Divergent Differentiation

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. Differentiation toward epithelial elements is very rare, and we illustrate a case of MPNST with glandular differentiation, comprising prominent well-formed glands, with a brief discussion of biphasic (spindle and glandular) neoplasms in the differential diagnosis. An index of suspicion for MPNST is necessary, due to the differing management from tumors in its differential diagnosis, and because of the potential for therapies toward molecular targets in future.

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