Abstract
BackgroundMalignant peripheral nerve sheath tumour (MPNST) is a very rare disease, and its pathogenesis is unknown. There are few reports of MPNST of the oesophagus. We report a case of an MPNST that was diagnosed and resected.Case presentationA 30-year-old female presented with dysphagia. She had been aware of the dysphagia approximately 6 months before presentation. The chest X-ray showed shadows in the right mediastinum. Barium fluoroscopy revealed a semicircular raised lesion in the lower oesophagus. Upper gastrointestinal endoscopy revealed a type 1 oesophageal tumour centred on the posterior wall 26–35 cm from the incisors. The surface was ulcerated, and the tumour was exposed. The affected area showed no iodine uptake. The EUS showed an isoechoic mass. The CT scan showed a mass of 71 × 61 × 55 mm in the beginning of the lower oesophagus with low density mass and swelling of the right recurrent nerve lymph node to 12 mm. On FDG-PET, the tumour showed an SUVmax of 11.05, and no abnormal accumulation was found in lymph nodes or other organs. The MRI showed a hyperintense mass on the T2WI, which had prolonged contrast enhancement, and no findings of invasion into surrounding tissue were found. The patient underwent right thoracotomy and open thoracic oesophagectomy. The affected lymph node was tumour negative by rapid pathological diagnosis during the operation. Histologically, spindle cells with different-sized nuclei were mixed throughout the tissue. Some regions showed nuclear polymorphism or a storiform pattern, and locally, there were approximately 7 mitoses/10 HPFs. The margin was relatively clear, but spindle-shaped tumour cells infiltrated the surrounding interstitium and basal myoepithelium, and the patient was diagnosed with MPNST. In this case, the postoperative course was good, and 16 months after the operation, the patient is currently under observation at the outpatient stage without recurrence.ConclusionsMPNST in the oesophagus is a relatively rare disease. Diagnosis before treatment is sometimes difficult, but the prognosis is good if radical resection is possible.
Highlights
Malignant peripheral nerve sheath tumour (MPNST) is a very rare disease, and its pathogenesis is unknown
Diagnosis before treatment is sometimes difficult, but the prognosis is good if radical resection is possible
Its exact cellular origins remain unclear, most MPNSTs arise in association with a peripheral nerve and are hypothesized to be of neural crest origin [3, 4]
Summary
Malignant peripheral nerve sheath tumour (MPNST) is the sixth most common type of soft-tissue sarcoma, accounting for approximately 5 to 10% of cases [1,2,3]. The chest X-ray showed shadows in the right mediastinum (Fig. 1a). The CT scan showed a mass of 71 × 61 × 55 mm in the beginning of the lower oesophagus with low density mass (Fig. 2a) and swelling of the right recurrent nerve lymph node to 12 mm (Fig. 2b). The margin was relatively clear, but spindle-shaped tumour cells infiltrated the surrounding interstitium and basal myoepithelium, and the patient was diagnosed with MPNST (Fig. 5a, b). Immunostaining showed S-100 (+) (Fig. 5d), CD34 (−), and HMB-45 (−) staining These results led to a diagnosis of MPNST. The postoperative course was good, and 16 months after the operation, the patient is currently under observation at the outpatient stage without recurrence
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