J Otolaryngol Ophthalmol Shandong Univ ›› 2018, Vol. 32 ›› Issue (4): 58-61.doi: 10.6040/j.issn.1673-3770.0.2017.068

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Clinical analysis of low-grade myofibroblastic sarcoma of the head and neck

BAI Yong1, YIN Yingchun2, HAN Xuefeng1, YIN Zhaofu1, LI Xuezhong3   

  1. Department of Pathology, Zibo Central Hospital, Zibo 255000, Shandong, China;3. Department of Otorhinolaryngology, Qilu Hospital of Shandong University, NHC Key Laboratory of Otorhinolaryngology(Shandong University), Jinan 250012, Shandong, China
  • Received:2017-02-16 Online:2018-07-20 Published:2018-07-20

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Abstract: Objective To investigate the clinical characteristics, imaging features, diagnosis, and treatment of low-grade myofibroblastic sarcoma(LGMS)of the head and neck. Methods A retrospective study of 2 patients with LGMS of the head and neck was performed, and previous cases of LGMS affecting the head and neck reported in the literature were reviewed. The clinic-pathological characteristics, imaging features, diagnosis and treatment were recorded. Results The symptoms of LGMS depend on the location of the tumor. Hoarseness is the first symptom of LGMS affecting vocal cord, along with an increase in dyspnea. LGMS of the neck manifests as a painless mass. It has been observed that LGMS of neck manifests as a painless mass with smooth surgace, capsule, and wide base, while its interface is unclear. Computed tomography demonstrated moderate or inhomogeneous density of tumor necrosis. Moreover, the tumor is characterized by calcification, or even bone destruction, and slight uniform ring enhancement. T1W2 weighted MRI showed the tumor with low signal or limited numbers, while in T2W1 weighted phase, homogeneous or inhomogeneous high signal was observed. The tumor consisted of spindle cells and small polygonal cells arranged in bundles, loose, and braided arrangement. The neoplasm showed diffuse infiltrative growth, and could invade the mucosa squamous epithelium. Tumor cells had unclear, fusiform shape, and the nucleus was slender, enlarged, irregular, and hyperchromatic. On immunohistochemistry analysis, the tumor cells were positive for vimentin, SMA, desmin, and actin, but negative for S-100. Treatment methods mainly focus on extensive surgical resection, and in the 2 cases that were followed up for 12-23 months, no recurrence and metastasis were observed. Conclusion LGMS of the head and neck is a very rare tumor, which lacks easily recognizable clinical and imaging manifestations. Pathological diagnosis must be combined with immunohistochemical examination, while extensive resection is the most effective method of treatment. Specifically, a thoroughgoing resection and negative margin are the keys to success of surgery. LGMS of the head and neck is not sensitive to chemotherapy and radiotherapy. Disease prognosis is good, while recurrence depends on complete resection.

Key words: Neoplasm, Pathology, Head and neck, Low-grade myofibroblastic sarcoma, Iconography

CLC Number: 

  • R767.04
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