Hospices Civils de Lyon, Hôpital Lyon Sud, Service d'anatomie et de cytologie pathologiques, Pierre Bénite, France.
Hospices Civils de Lyon, Hôpital Lyon Sud, Service de biochimie et de biologie moléculaire, Pierre Bénite, France.
Am J Dermatopathol. 2020 Nov;42(11):881-884. doi: 10.1097/DAD.0000000000001734.
Lipofibromatosis-like neural tumors (LPF-NT) are soft tissue tumors characterized by a lipofibromatosis-like pattern, CD34/PS100 positivity, and recurrent NTRK1 gene rearrangement. It occurs mainly in pediatric patients or young adults. We report here, the first case of LPF-NT in a middle-aged adult initially misdiagnosed as a myoepithelial tumor. LPF-NT may have a locally aggressive course but no recurrence was seen after complete surgeries, whereas metastatic diseases remain exceptional.
脂肪纤维瘤病样神经肿瘤（LPF-NT）是一种软组织肿瘤，其特征为具有脂肪纤维瘤病样形态、CD34/PS100 阳性和 NTRK1 基因重排复发。它主要发生在儿童或年轻成人中。我们在此报告首例中年成人 LPF-NT，最初误诊为肌上皮瘤。LPF-NT 可能具有局部侵袭性病程，但完全手术后未见复发，而转移疾病仍然罕见。
