Abstract
BackgroundMalignant 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 presentationHere, 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.ConclusionsThis 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.
Highlights
BackgroundPrimary tracheal cancer is one of the rare malignancies in the upper airway, accounting for only 0.1–0.4% of all newly diagnosed tumours
Malignant peripheral nerve sheath tumours (MPNSTs) of the trachea are extremely uncommon neoplasms with unknown genetic and clinical profiles
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
Summary
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. Tumour debulking was performed through high-frequency electric coagulation and cutting to alleviate airway obstruction. This patient gained obvious relief from the obstructive symptoms postoperatively. The tumour cells were positive for S100 (Fig. 1), SOX-10, and vimentin but negative for pancytokeratin, SMA, desmin, transcription factor E3 (TFE3), and master myogenic regulatory factor (MyoD1) by immunohistochemistry. Owing to the epithelioid morphology and diffuse S100 immunoreactivity, an unusual variant of an epithelioid MPNST was determined The tumour of this patient was further classified as a sporadic MPNST since no related signs of neurofibromatosis type 1 (NF1) were noted. The airway was reconstructed with a posterior continuous suture and an anterior row of interrupted sutures, and the anastomosis was covered with autologous thymus tissue This patient experienced an uneventful postoperative course. The patient is scheduled to receive regular follow-up evaluations every 6 months using computed tomography and bronchoscopy to monitor for recurrence
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