scholarly journals The Neoplastic Whorls-Soft Tissue Perineurioma

2020 ◽  
Vol 1 (2) ◽  
pp. 17-25
Author(s):  
Anubha Bajaj
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Benign Neoplasm ◽  
Hypertrophic Neuropathy ◽  
Localized Hypertrophic Neuropathy ◽  
Perineurial Cells

Perineurioma is an exceptional, benign neoplasm entirely composed of neoplastic perineurial cells and arises from the peripheral nerve. Pertaining to location, the neoplasm predominantly demonstrates configurations such as intra-neural or extra-neural, confined to the soft tissue. Perineurioma was initially scripted by Lazarus and Trombetta in 1978 and is additionally nomenclated as localized hypertrophic neuropathy1. Extra-neural soft tissue perineurioma and mucosal (intestinal) perineurioma are frequent, in contrast to intra-neural perineurioma or localized hypertrophic neuropathy subtype1, 2.

Hypertrophic Neuropathy of the Facial Nerve

2001 ◽  
Vol 110 (3) ◽  
pp. 257-262 ◽  
Author(s):  
Dominique Cazals-Hatem ◽  
Fabrice Lisovoski ◽  
Romain E. Kania ◽  
Didier Bouccara ◽  
Dominique Hénin ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Facial Nerve ◽  
Pathological Examination ◽  
Nerve Lesion ◽  
Rare Entity ◽  
Peripheral Nerve Lesion ◽  
Hypertrophic Neuropathy ◽  
Seventh Cranial Nerve ◽  
History Of ◽  
Localized Hypertrophic Neuropathy

Hypertrophic neuropathy is a peripheral nerve lesion that is histologically characterized by onion bulb formations around axons. This histologic picture, which is usually seen in generalized hypertrophic neuropathies, can occasionally be observed in single nerves as localized hypertrophic neuropathy. Cranial involvement of such localized hypertrophic neuropathy represents a very rare entity; only a few cases have been reported in the literature. We report the history of a progressive facial paralysis with a tumorous enlargement of the seventh cranial nerve that was clinically suspected of being a schwannoma. Pathological examination permitted the diagnosis of hypertrophic neuropathy.

scholarly journals Localized Hypertrophic Neuropathy as a Neoplastic Manifestation of KRAS-Mediated RASopathy

2020 ◽  
Vol 79 (6) ◽  
pp. 647-651
Author(s):  
M Adelita Vizcaino ◽  
Allan Belzberg ◽  
Shivani Ahlawat ◽  
Sarra Belakhoua ◽  
Liam Chen ◽  
...  
Keyword(s):  
Cell Proliferation ◽  
Schwann Cell ◽  
Peripheral Nerve ◽  
Mapk Pathway ◽  
Ras Signaling ◽  
Nerve Lesion ◽  
Peripheral Nerve Lesion ◽  
Hypertrophic Neuropathy ◽  
Café Au Lait Spots ◽  
Localized Hypertrophic Neuropathy

Abstract Localized hypertrophic neuropathy is a rare Schwann cell proliferation that usually affects single nerves from the extremities, and it is of unclear etiology in its pure form. RASopathies are a defined group of genetic diseases with overlapping clinical features, usually secondary to germline mutations in genes encoding either components or regulators of the RAS/MAPK pathway. Herein, we report an 11-year-old boy presenting with café au lait spots and right leg length discrepancy. A fascicular nerve biopsy of the tibial nerve demonstrated a Schwann cell proliferation with prominent onion-bulb formation, satisfying criteria for localized hypertrophic neuropathy. Molecular genetic analysis demonstrated identical KRAS mutations (c38_40dupGCG) in the peripheral nerve lesion and melanocytes from café au lait spots, but not in blood, supporting a diagnosis of a KRAS-mediated rasopathy with mosaicism. Immunohistochemical staining in the peripheral nerve lesion demonstrated strong pERK staining consistent with downstream MAPK pathway activation. This report suggests that at least a subset of localized hypertrophic neuropathies are bonafide, well-differentiated Schwann cell neoplasms developing through oncogenic RAS signaling, which provides new insights into the controversial entity historically known as localized hypertrophic neuropathy.

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.

The Spread of Soft-Tissue Sarcomata of the Extremities Along Peripheral-Nerve Trunks

1957 ◽  
Vol 39 (3) ◽  
pp. 534-540 ◽  
Author(s):  
JOHN R. BARBER ◽  
MARK B. COVENTRY ◽  
JOHN R. MCDONALD
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve

scholarly journals Sensorimotor Perineuritis – An Autoimmune Disease?

1985 ◽  
Vol 12 (2) ◽  
pp. 129-133 ◽  
Author(s):  
Christopher N. Bourque ◽  
Brian A. Anderson ◽  
C. Martin del Campo ◽  
Anders A. F. Sima
Keyword(s):  
Peripheral Nerve ◽  
Mononuclear Cells ◽  
Sensory Neuropathy ◽  
Nerve Biopsy ◽  
Sural Nerve Biopsy ◽  
Gradual Improvement ◽  
Immune Mediated ◽  
Nerve Dysfunction ◽  
Inflammatory Infiltrates ◽  
Perineurial Cells

ABSTRACT:The literature contains a single description of sensory perineuritis (Asbury et al 1972). These patients demonstrated a painful, distal, sensory neuropathy, and examination of peripheral nerve biopsies revealed focal thickening and inflammatory infiltrates of the perineurium. We report a patient with sensorimotor peripheral nerve dysfunction, accompanied by progressive slowing of nerve conduction velocity. Examination of a sural nerve biopsy demonstrated focal thickening of the perineurium, inflammatory infiltrates, and necrosis of perineurial cells. Immunohistology revealed a patchy precipitation of IgG and IgM on perineurial cells. Ultrastructurally, mononuclear cells were found adjacent to perineurial cells undergoing necrosis. The patient showed gradual improvement partially coinciding with a course of steroid therapy. We suggest that this neuropathy is caused by damage to the perineurial barrier possibly by an immune-mediated destruction of perineurial cells and subsequent compression of the endoneurial content by perineurial scarring.

Neurogenic Tumors of Soft Tissue

2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 62-107 ◽  
Author(s):  
Justin M.M. Cates ◽  
Cheryl M. Coffin
Keyword(s):  
Differential Diagnosis ◽  
Soft Tissue ◽  
Peripheral Nerve ◽  
Neural Crest ◽  
Children And Adolescents ◽  
Soft Tissue Tumors ◽  
Cellular Composition ◽  
Clinicopathologic Features ◽  
Genetic Syndromes ◽  
Neurogenic Tumors

Neurogenic tumors are an uncommon yet important category of soft tissue tumors in children and adolescents because of their frequent association with various genetic syndromes. The heterogeneous cellular composition of the peripheral nerve and the wide metaplastic capacity of the neural crest and its derivatives generate a variety of neoplasms with neurogenic differentiation. This article reviews the clinicopathologic features and differential diagnosis of neurogenic tumors in the first two decades of life, and highlights use of selected ancillary methods for diagnosis.

scholarly journals Spindle Cell Hemangioendothelioma of the Temporal Muscle Resected with Zygomatic Osteotomy: A Case Report of an Unusual Intramuscular Lesion Mimicking Sarcoma

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Tomohiro Minagawa ◽  
Takeshi Yamao ◽  
Ryuta Shioya
Keyword(s):  
Case Report ◽  
Surgical Treatment ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Spindle Cell ◽  
Neck Region ◽  
Benign Neoplasm ◽  
Low Grade ◽  
Temporal Muscle ◽  
Head And Neck Region

Spindle cell hemangioendothelioma (SCH) was originally described by Weiss and Enzinger (1986) as a low-grade angiosarcoma resembling both cavernous hemangioma and Kaposi's sarcoma. Recent studies suggest that SCH is a benign neoplasm or reactive lesion accompanying a congenital or acquired vascular malformation. Most SCHs present as one or more nodules affecting the dermis or subcutis of the distal extremities. Few reports describe SCH of the head and neck region; even fewer note intramuscular SCH. Here, we describe a case of SCH involving the temporal muscle mimicking soft tissue sarcoma, who had a successful surgical treatment with a coronal approach and zygomatic osteotomy.

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