Perineurioma: A distinctive and underrecognized peripheral nerve sheath neoplasm
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.
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.
English-language literature published between 1966 and 2005 was reviewed.
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.
Available from: PubMed Central
- "Benign fibrous histiocytoma or low-grade malignant fibrous histiocytoma cells stain negative for S-100, CD34, and EMA, and stain positive for CD68. Overall, immunohistochemical studies will help distinguish these three entities [1,4]. Smooth muscle actin has been reported to be positive in 50% of perineurioma cases; this indicates that it is also an important diagnostic marker. "
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ABSTRACT: Perineurioma is a rare benign tumor of the peripheral nervous system distinct from schwannomas and neurofibromas. It may be intraneural or extraneural (in the soft tissue). Extraneural soft tissue perineuriomas are uncommon; rare cases have been reported in the oral cavity. We present a case of soft tissue perineurioma in the tip of the tongue. The tumor was characterized by slender spindle cells, arranged in short fascicles or whorls, and focal areas showing a distinct storiform pattern. Tumor cells showed the immunohistochemical profile of perineurial cells, including epithelial membrane antigen. Smooth muscle actin, S-100, and CD34 were not expressed by the tumor cells. The tumor was surgically excised and in 2 years there has been no recurrence. Knowledge of the tumor in the oral cavity is important to reach a correct diagnosis and to avoid unnecessary aggressive local excision.
Available from: Yoshiaki Katada
- "Soft-tissue perineuriomas are rare benign peripheral nerve sheath tumors with perineurial cell differentiation [1-4]. They were first described by Lazarus and Trombetta in 1978 . "
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ABSTRACT: Soft-tissue perineuriomas are rare benign peripheral nerve sheath tumors in the subcutis of the extremities and the trunks of young patients. To our knowledge, this the first presentation of the computed tomography and magnetic resonance imaging of a soft-tissue perineurioma in the retroperitoneum with pathologic correlation.
A 63-year-old Japanese man was referred for assessment of high blood pressure. Abdominal computed tomography and magnetic resonance imaging showed a well-defined, gradually enhancing tumor without focal degeneration or hemorrhage adjacent to the pancreatic body. Tumor excision with distal pancreatectomy and splenectomy was performed, as a malignant tumor of pancreatic origin could not be ruled out. No recurrence has been noted in the 16 months since the operation. Pathologic examination of the tumor revealed a soft-tissue perineurioma of the retroperitoneum.
Although the definitive diagnosis of soft-tissue perineurioma requires biopsy and immunohistochemical reactivity evaluation, the computed tomography and magnetic resonance imaging findings described in this report suggest inclusion of this rare tumor in the differential diagnosis when such findings occur in the retroperitoneum.
Available from: Erich Peter Bosch
- "Intraneural (IN) perineurioma is a benign neoplasm composed exclusively of whorls of perineurial cells surrounding nerve fibres and restricted to the boundaries of a peripheral nerve (Macarenco et al., 2007). Extraneural (EN) perineurioma is also composed of perineurial cells but is found mainly in soft tissues and skin (Macarenco et al., 2007). The IN form was first described in 1964 as interstitial hypertrophic neuritis (Imaginariojda et al., 1964) and was referred to by several other names including IN neurofibroma (Lallemand and Weller, 1973), localized hypertrophic neuropathy (Hawkes et al., 1974; Snyder et al., 1977; Peckham et al., 1982; Boker et al., 1984; Iyer et al., 1988; Stanton et al., 1988; Johnson and Kline, 1989; Phillips et al., 1991; Gullotta, 1992; Suarez et al., 1994; Simmons et al., 1999) and hypertrophic neurofibrosis (Simpson and Fowler, 1966; de los Reyes et al., 1981). "
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ABSTRACT: The natural history of intraneural perineurioma has been inadequately studied. The aim of this study was to characterize the clinical presentation, electrophysiologic and imaging features and outcome of intraneural perineurioma. We ask if intraneural perineurioma is a pure motor syndrome that remains confined to one nerve and should be treated by surgical resection. We examined the nerve biopsies of cases labelled perineurioma and selected those with diagnostic features. Thirty-two patients were identified; 16 children and 16 adults; 16 males and 16 females. Median age of onset of neurological symptoms was 14 years (range 0.5-55 years) and median age at evaluation was 17 years (range 2-56 years). All patients had motor deficits; however, mild sensory symptoms or signs were experienced by 27 patients; 'prickling' or 'asleep numbness' in 20, mild pain in 13 and sensory loss in 23. The sciatic nerve or its branches was most commonly affected in 15, followed by brachial plexus, radial nerve and ulnar nerve (four each). Magnetic resonance imaging demonstrated nerve enlargement (29/32), T1 isointensity (27/32), T2 hyperintensity (25/32) and contrast enhancement (20/20). Diagnoses were made based on targeted biopsy of the focal nerve enlargement identified by imaging. Neurological impairment was of a moderate severity (median Neuropathy Impairment Score was 12 points, range 2-49 points). All patients had focal involvement with 27 involving one nerve and five involving a plexus (one bilateral). Long-term follow-up was possible by telephone interview for 23 patients (median 36 months, range 2-177 months). Twelve patients also had follow-up neurologic evaluation (median 45 months, range 10-247 months). The median Neuropathy Impairment Score had changed from 12.6 to 15.4 points (P = 0.19). In all cases, the distribution of neurologic findings remained unchanged. Median Dyck Disability Score was 3 (range 2-5) indicating a mild impairment without interfering with activities of daily living. Ten patients judged their symptoms unchanged, nine slightly worse and four slightly better. We conclude intraneural perineurioma is a benign hypertrophic (non onion bulb) peripheral nerve tumour that presents insidiously in young people and is motor predominant with mild sensory involvement. It is most often a mononeuropathy, but a plexopathy can occur. Diagnosis of this condition requires clinical suspicion, imaging, targeted fascicular biopsy of the lesion and expertise of nerve pathologists. As these tumours are static or slowly progressive, remain confined to their original distribution and have low morbidity, they probably should not be resected routinely. Because intensive evaluation is needed for diagnosis, intraneural perineurioma is probably under-recognized.
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