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Review
. 2010 Jan 9:5:2.
doi: 10.1186/1746-1596-5-2.

Malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report

Masanori Kitamura  1 , Naoki Wada, Shigenori Nagata, Norishige Iizuka, Yu-Fen Jin, Miki Tomoeda, Michiko Yuki, Norifumi Naka, Nobuhito Araki, Chikao Yutani, Yasuhiko Tomita
Affiliations
Review

Malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report

Masanori Kitamura et al. Diagn Pathol. .

Abstract

A rare case is presented of a 61-year-old man with a malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart. The primary tumor originated in the right thigh in 1982. Since then, the patient has had repeated local recurrences in spite of repeated surgical treatment and adjuvant chemotherapy. He has developed previous metastases of the lung and heart. The patient died of cardiac involvement.

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Figures

Figure 1
Figure 1
Clinical manifestations of NF1: multiple cutaneous neurofibromas and café-au-lait spots.
Figure 2
Figure 2
Hematoxylin and eosin and S-100 staining of the tumors of the thigh treated surgically in 1990 (A, B), recurrent tumor operated in 1995 (C, D) and metastatic tumor in heart (E, F) and lung (G, H) at autopsy in 2007. A, C, E, G: Hematoxylin and eosin staining; B, D, F, H: S-100 immunohistochemistry. Histological factors such as cellularity, degree of nuclear atypia, mitotic counts, and S-100 immunoreactivity were similar among the specimens.
Figure 3
Figure 3
Magnetic resonance imaging showed a large tumor in the inferior-posterior wall of the heart. (A) Frontal cross section. Size: 7.5 cm. (B) Horizontal cross section. Size: 9 cm. (C) Sagittal cross section. Size: 6.5 cm.
Figure 4
Figure 4
Autopsy specimen showing a tumor in the heart.
See this image and copyright information in PMC

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