PMC7667415_01
Patient's family condition regarding NF1
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At the time of this study, he was too old to participate (Figure 1).
Preoperative photos: A - anterior view, B - norma lateralis, C - norma occipitalis, D - the diameter of the mass reached 95 cm, E - the longitudinal length of the mass reached 50 cm
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The patient's physical examination showed scoliosis, multiple cafe-au-lait spots of different sizes all over the body, a huge mass on the lateral side of the right thigh, with medium texture, general mobility and obvious skin veins on the surface of the mass, which had a size of 50 x 33 x 32 cm (Figures 2 A, B).
Magnetic resonance imaging showed space occupation in the lateral muscle group and a subcutaneous fat layer in the right thigh. A - Coronal section, B - transverse section
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Magnetic resonance imaging (MRI) of the right thigh revealed a large soft tissue mass (Figure 3), and therefore a diagnosis of neurofibromatosis was suspected.
(1) Lateral curvature of the spine;(2) right hip and thigh area-sheet radiodistribution, considering ectopic tumor uptake
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The bone scan showed a slightly increased area of large flaky radioactive uptake in the right hip and thigh, an uneven distribution of radioactivity, and no abnormalities were observed in the other bones (Figure 4).
Microscopic findings. A - Under high power microscope, the cells were round, oval, fusiform hyperplasia and obvious heteromorphism, B - immunohistochemistry showed tumor negativity for S-100 protein, C - the Ki-67 labeling index was approximately 40%
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The tumor cells were negative for CKpan, desmin, S-100, CD34, NF, EMA and SMA and the Ki-67 labeling index was approximately 40% (Figure 5).
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