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. 2001 Aug;54(8):631–636. doi: 10.1136/jcp.54.8.631

Malignant peripheral nerve sheath tumour arising within neurofibroma. An immunohistochemical analysis in the comparison between benign and malignant components

T Watanabe 1, Y Oda 1, S Tamiya 1, K Masuda 1, M Tsuneyoshi 1
PMCID: PMC1731495  PMID: 11477120

Abstract

Aims—To compare the expression of immunohistochemical variables between benign and malignant components of malignant peripheral nerve sheath tumour (MPNST) arising within neurofibroma.

Methods—Eight cases of MPNST arising within a neurofibroma, associated with neurofibromatosis type 1 (NF1), were studied. The areas of MPNST and neurofibroma were compared immunohistochemically with regard to the expression of proliferative activity (MIB-1), growth factors, p53, bcl-2, neural cell adhesion molecule (N-CAM), and CD34.

Results—The expression of transforming growth factor ß1 (TGF-ß1), TGF-ß receptor type II, hepatocyte growth factor α (HGF-α), c-met, p53, and N-CAM was higher in the areas of MPNST than in the neurofibromatous areas in four, five, five, eight, five, and three of the eight cases, respectively. CD34 expression was lower in the areas of MPNST than in the neurofibroma areas in three of the eight cases.

Conclusions—On the basis of these findings, TGF-ß1, HGF-α, and p53 might be involved in the malignant transformation of neurofibroma to MPNST.

Key Words: malignant peripheral nerve sheath tumour • neurofibroma • immunohistochemistry • transforming growth factor ß • hepatocyte growth factor • p53

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Figure 1 (A) Cut surface of the resected tumour in case 3. Section shows a multinodular solid and white mass. The central firm portion blends with the surrounding subcutaneous fat and muscular tissue. (B) Topographical distribution of histological features of both malignant peripheral nerve sheath tumour and neurofibroma in case 3.

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Figure 2 (A) Cut surface of the resected tumour in case 4. The section shows a well circumscribed solid and greyish yellow mass with nodular growth pattern and interlacing fascicles. (B) Topographical distribution of both sarcomatous and neurofibromatous components in case 4.

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Figure 3 (A–D) Histological picture exhibiting the features of neurofibroma and malignant peripheral nerve sheath tumour (MPNST) (case 4). (A) The MPNST area is surrounded by a neurofibromatous area. Densely cellular fascicles alternate with hypocellular zones in the MPNST area (haematoxylin and eosin stained; original magnification, x12). (B) The tumour is composed of interlacing bundles of elongated cells with wavy nuclei, without atypism in the neurofibroma (top), and with atypism in the MPNST (bottom) (haematoxylin and eosin stained; original magnification, x20). (C) The tumour is composed of wavy spindle cells arranged in fascicles and with wavy nuclei. Densely cellular fascicles alternate with hypocellular zones (haematoxylin and eosin stained; original magnification, x25). (D) The nuclei of some tumour cells are wavy or buckled. Mitotic figures are seen frequently (haematoxylin and eosin stained; original magnification, x570). (E) Histological picture exhibiting the features of neurofibroma (case 4). The tumour is made up of interlacing bundles of elongated cells with wavy nuclei, without atypism, associated with wire-like strands of collagen (haematoxylin and eosin stained; original magnification, x125).

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Figure 4 Histological picture exhibiting the features of malignant peripheral nerve sheath tumour (case 5). The tumour is composed of spindle cells arranged in fascicles reminiscent of tactoid differentiation. Nuclei are wavy and twisted (haematoxylin and eosin stained; original magnification, x200).

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Figure 5 Immunohistochemical staining patterns of the transforming growth factor ß receptor type II. The degree of staining in the neurofibroma (top) is graded as 1+, whereas that in the MPNST (bottom) is graded as 2+ (case 5) (original magnification, x200).

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Figure 6 Immunohistochemical staining patterns for p53. Staining in the neurofibroma (B) is negative, whereas that in the MPNST (A) is graded as 1+ (case 8) (original magnification, x400).

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Figure 7 Immunohistochemical staining patterns for CD34. The degree of staining in the neurofibroma (top) is graded as 2+, whereas the MPNST (bottom) is negative (case 5) (original magnification, x200).

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