关键词: aggressive fibromatosis beta-catenin ctnnb1 desmoid tumor msh6 neuromuscular choristoma oncology orthopedic oncology

来  源:   DOI:10.7759/cureus.53771   PDF(Pubmed)

Abstract:
Multifocal desmoid-type fibromatosis (DTF) is very rare and usually regional. We report three cases that initially appeared to be multifocal, but subsequent detailed imaging revealed unsuspected tracking along nerves in two cases. This neural spread is reminiscent of neuromuscular choristoma (NMC), a rare developmental lesion in which mature skeletal muscle cells, or rarely smooth muscle cells, infiltrate and enlarge peripheral nerves. NMC is frequently associated with DTF. These two cases suggest that DTF spread along nerves and appeared as distinct multifocal lesions while actually being contiguous. The third case was felt to represent true multifocal tumor development, possibly due to tumor seeding at the time of chest surgery. The relationship of DTF to NMC is discussed.
摘要:
多灶性纤维瘤病(DTF)非常罕见,通常是区域性的。我们报告了三例最初似乎是多灶性的病例,但是随后的详细成像显示,在两个病例中,没有怀疑地追踪神经。这种神经扩散让人想起神经肌肉性脉络膜瘤(NMC),一种罕见的发育损伤,其中成熟的骨骼肌细胞,或者很少有平滑肌细胞,浸润并扩大周围神经。NMC经常与DTF相关联。这两种情况表明,DTF沿神经扩散,并表现为明显的多灶性病变,但实际上是连续的。第三个病例被认为代表真正的多灶性肿瘤发展,可能是由于胸部手术时的肿瘤种植。讨论了DTF与NMC的关系。
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