Neurofibroma vs Schwannoma pathology
Variants of neurofibroma
Specific clinico-pathological subtypes based on architectural growth patterns include localized, diffuse and plexiform neurofibromas. Localized cutaneous neurofibroma is the most common and occurs sporadically in most cases. Localized neurofibromas can also affect an important nerve and, generally, give rise to a fusiform expansion of the nerve trunk (intraneural subtype). Diffuse neurofibromas are characterized by an enlargement in the form of plaque, usually in the region of the head and neck. S100 positive pseudomeissnerian corpuscles can be abundant. Most neurofibromas occur sporadically, although approximately 10% are eventually associated with neurofibromatosis type 1 (NF1).
Plexiform neurofibroma located in an important nerve trunk and the rarest form, massive soft tissue neurofibroma is almost always associated with NF1. Plexiform neurofibroma is defined by the involvement of numerous adjacent nerve fascicles or multiple components of a nerve plexus. Microscopically, plexiform neurofibromas often show a mixture of areas that resemble localized and diffuse neurofibromas. Plexiform neurofibroma has a potential for malignant degeneration and is a recognized precursor of MPNST in patients with NF1.
Some neurofibromas show unusual characteristics such as degenerative cytological atypia (neurofibroma with old change, atypical neurofibroma) and / or increased cellularity (cellular neurofibroma), often raising the differential diagnosis with MPNST. Cellular neurofibromas may show moderate cellularity and a more pronounced fascicular growth pattern, but lack the "monotone" cytological atypia, chromatin abnormalities, and mitotic activity observed in the MPNST. Neurofibromas with old change have degenerative nuclear atypia, which contains scattered cells with markedly enlarged hyperchromatic nuclei, often with "diffused" chromatin; however, they lack increased cellularity, fascicular growth or mitotic activity. You can see similar changes in the so-called "ancient schwannomas". Other less common morphological findings in neurofibroma include the presence of melanin pigment, metaplastic bone (Figure 3b) and glandular differentiation. Massive soft tissue neurofibroma, a very rare subtype, is characterized by large size, soft tissue infiltration and skeletal muscle, which often involves large anatomic regions, and histologically demonstrates the presence of a cellular component (Figure 3c) . They may contain plexiform components, but usually do not suffer from malignant degeneration.
Schwannoma
Schwannomas are benign neoplasms of cellular origin of Schwann. The general appearance is characteristic, in the form of well-circumscribed masses with degenerative changes and variable mixing of compact spindle areas and hypocellular, microcystic (Antoni B) areas rich in macrophages and collagen fibers. A well-formed collagen capsule is a consistent finding, as are the hyalinized vessels. For immunohistochemistry, schwannomas typically show a strong and diffuse expression of the S100 protein and abundant pericellular type IV collagen (Figure 4f), consistent with the presence of a continuous pericellular basal lamina. The glial fibrillary acid (GFAP) protein is expressed in a subset of schwannomas. Recent markers frequently positive in schwannomas include podoplanin, calretinin and SOX10. Very rarely, typical schwannomas may show an abnormal expression of cytokeratins. In our experience, such tumors are always strongly positive for GFAP, suggesting a cross-reactivity of cytokeratin antibodies with GFAP, rather than true protein expression. Unlike neurofibroma, neurofilament protein staining is generally limited to axons trapped in the periphery of the tumor, although some recent studies suggest that the presence of intralesional axons is actually more frequent than previously reported.
Keywords: peripheral nerve, neurofibroma, schwannoma, perineurioma, MPNST, Neurofibroma vs Schwannoma pathology
Specific clinico-pathological subtypes based on architectural growth patterns include localized, diffuse and plexiform neurofibromas. Localized cutaneous neurofibroma is the most common and occurs sporadically in most cases. Localized neurofibromas can also affect an important nerve and, generally, give rise to a fusiform expansion of the nerve trunk (intraneural subtype). Diffuse neurofibromas are characterized by an enlargement in the form of plaque, usually in the region of the head and neck. S100 positive pseudomeissnerian corpuscles can be abundant. Most neurofibromas occur sporadically, although approximately 10% are eventually associated with neurofibromatosis type 1 (NF1).
Plexiform neurofibroma located in an important nerve trunk and the rarest form, massive soft tissue neurofibroma is almost always associated with NF1. Plexiform neurofibroma is defined by the involvement of numerous adjacent nerve fascicles or multiple components of a nerve plexus. Microscopically, plexiform neurofibromas often show a mixture of areas that resemble localized and diffuse neurofibromas. Plexiform neurofibroma has a potential for malignant degeneration and is a recognized precursor of MPNST in patients with NF1.
Some neurofibromas show unusual characteristics such as degenerative cytological atypia (neurofibroma with old change, atypical neurofibroma) and / or increased cellularity (cellular neurofibroma), often raising the differential diagnosis with MPNST. Cellular neurofibromas may show moderate cellularity and a more pronounced fascicular growth pattern, but lack the "monotone" cytological atypia, chromatin abnormalities, and mitotic activity observed in the MPNST. Neurofibromas with old change have degenerative nuclear atypia, which contains scattered cells with markedly enlarged hyperchromatic nuclei, often with "diffused" chromatin; however, they lack increased cellularity, fascicular growth or mitotic activity. You can see similar changes in the so-called "ancient schwannomas". Other less common morphological findings in neurofibroma include the presence of melanin pigment, metaplastic bone (Figure 3b) and glandular differentiation. Massive soft tissue neurofibroma, a very rare subtype, is characterized by large size, soft tissue infiltration and skeletal muscle, which often involves large anatomic regions, and histologically demonstrates the presence of a cellular component (Figure 3c) . They may contain plexiform components, but usually do not suffer from malignant degeneration.
Schwannoma
Schwannomas are benign neoplasms of cellular origin of Schwann. The general appearance is characteristic, in the form of well-circumscribed masses with degenerative changes and variable mixing of compact spindle areas and hypocellular, microcystic (Antoni B) areas rich in macrophages and collagen fibers. A well-formed collagen capsule is a consistent finding, as are the hyalinized vessels. For immunohistochemistry, schwannomas typically show a strong and diffuse expression of the S100 protein and abundant pericellular type IV collagen (Figure 4f), consistent with the presence of a continuous pericellular basal lamina. The glial fibrillary acid (GFAP) protein is expressed in a subset of schwannomas. Recent markers frequently positive in schwannomas include podoplanin, calretinin and SOX10. Very rarely, typical schwannomas may show an abnormal expression of cytokeratins. In our experience, such tumors are always strongly positive for GFAP, suggesting a cross-reactivity of cytokeratin antibodies with GFAP, rather than true protein expression. Unlike neurofibroma, neurofilament protein staining is generally limited to axons trapped in the periphery of the tumor, although some recent studies suggest that the presence of intralesional axons is actually more frequent than previously reported.
Keywords: peripheral nerve, neurofibroma, schwannoma, perineurioma, MPNST, Neurofibroma vs Schwannoma pathology
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