Orthoplastic Approach to the Treatment and Reconstruction of a Neurotrophic Receptor Tyrosine Kinase Type 3 Soft Tissue Sarcoma Arising From the Occipitalis Muscle

采用骨科整形方法治疗和重建起源于枕肌的神经营养受体酪氨酸激酶3型软组织肉瘤

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Abstract

We present a multidisciplinary approach to the treatment of a neurotrophic receptor tyrosine kinase type 3 (NTRK3) soft tissue sarcoma (STS), arising from the occipitalis muscle. NTRK3 is a mutation only recently described in STS using next-generation sequencing and is rarely implicated in STS.Currently, there is limited literature to guide care. This case demonstrates a successful treatment option utilizing a multidisciplinary team and unique reconstruction with a hair-bearing scalp. To the best of our knowledge, this is the first case report showing an NTRK3 mutation STS arising from the occipitalis muscle. The utilization of staged hair-bearing tissue expansion post-wide R0 resection to reconstruct the scalp defect is a challenging reconstruction method. We present a single clinical experience discussing a 40-year-old female with an NTRK3 mutation STS arising from the occipitalis muscle and involving the scalp. The diagnosis was made after the analysis of a punch biopsy specimen by a bone and soft tissue pathologist as a low-grade sarcoma harboring a sperm antigen with calponin homology and coiled-coil domains 1-like (SPECC1L)-NTRK3 fusion transcript. The patient underwent R0 resection by orthopedic oncology surgery and craniofacial microvascular plastic surgery. Staged reconstruction via hair-bearing tissue expansion was performed by the latter. Eighteen months after the index procedure, no recurrent disease was detected, and the hair-bearing reconstruction was fully healed with well-concealed scars. This case is a successful treatment method for a low-grade STS harboring a SPECC1L-NTRK3 fusion transcript. There is little published literature to guide care for low-grade NTRK3 mutation STS. This case highlights the importance of multidisciplinary care for STS.

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