Primary malignant peripheral nerve sheath tumour of the trachea: a case report and literature review
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(2020) 15:247
CASE REPORT
Open Access
Primary malignant peripheral nerve sheath tumour of the trachea: a case report and literature review Yan Hu1, Siying Ren2, Wei Han1, Boyou Zhang1, Lu Shu1, Yi Sun3, Fenglei Yu1 and Wenliang Liu1*
Abstract Background: Malignant peripheral nerve sheath tumours (MPNSTs) of the trachea are extremely uncommon neoplasms with unknown genetic and clinical profiles. Only individual cases have been reported in the literature to date. Case presentation: Here, we present a rare case of a 61-year-old female patient with a primary MPNST of the trachea who complained of irritating cough and progressively increasing breathlessness for 4 weeks. This patient initially underwent intraluminal resection of the mass and was misdiagnosed with clear cell sarcoma. Less than a year later, the mass relapsed, and the obstructive symptoms reappeared and gradually worsened. Debulking of the endotracheal tumour mass was performed once again, and an MPNST was definitively diagnosed. Open sleeve tracheal resection and tracheoplasty were later performed with curative intent. This patient was alive without recurrence at her six-month postoperative follow-up. We also compared the clinical outcomes of previously reported cases of MPNSTs and our case. Conclusions: This paper emphasizes that thoracic surgeons should be aware that malignant peripheral nerve sheath tumours of the trachea can be misdiagnosed in clinical practice and must be included in the differential diagnosis of tracheal neoplasms. Keywords: Malignant peripheral nerve sheath tumour, Trachea, Misdiagnosis
Background Primary tracheal cancer is one of the rare malignancies in the upper airway, accounting for only 0.1–0.4% of all newly diagnosed tumours. Currently, there is no validated staging system for diagnosis, survival prediction, or management. The prognosis of patients diagnosed with tracheal cancer is dismal. MPNST commonly occurs in the extremities. Primary MPNST of the trachea has rarely been reported in the literature. We herein present a rare case of an MPNST in the trachea initially misdiagnosed as clear cell sarcoma and present a literature review of previously reported cases of tracheal MPNSTs.
* Correspondence: [email protected] 1 Department of Thoracic Surgery, Second Xiangya Hospital of Central South University, No. 139 Renmin Road, Changsha 410011, China Full list of author information is available at the end of the article
Case presentation A 61-year-old female patient was admitted to a local hospital in August 2018 because of irritating cough and progressively increasing breathlessness over a duration of 4 weeks. Rinopharyngeal thoracic computed tomography scans showed severe luminal obstruction of the middle upper segment of the trachea caused by a mass. Rigid tracheoscopy was performed, revealing an endotracheal mass with a wide base originating in the posterior lateral wall of the trachea occluding 75 % of the lumen. The tumour was then endoscopically resected through high-frequency electric coagulation, cutting and
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