Malignant peripheral nerve sheath tumour of the oesophagus: a case report
Abstract Background Malignant 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 presentation A 30-year-old female presented with dysphagi...
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Japan Surgical Society
2020-07-01
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| Series: | Surgical Case Reports |
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| Online Access: | http://link.springer.com/article/10.1186/s40792-020-00954-2 |
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| author | Kento Tomizawa Tatsuya Miyazaki Arisa Yamaguchi Ryoya Honda Marie Hoshino Mitsuhiro Yanai Yohei Miyamae Ryo Kurosaki Hisashi Shimizu Kazuhisa Arakawa Munenori Ide |
| author_facet | Kento Tomizawa Tatsuya Miyazaki Arisa Yamaguchi Ryoya Honda Marie Hoshino Mitsuhiro Yanai Yohei Miyamae Ryo Kurosaki Hisashi Shimizu Kazuhisa Arakawa Munenori Ide |
| author_sort | Kento Tomizawa |
| collection | DOAJ |
| description | Abstract Background Malignant 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 presentation A 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. Conclusions MPNST in the oesophagus is a relatively rare disease. Diagnosis before treatment is sometimes difficult, but the prognosis is good if radical resection is possible. |
| format | Article |
| id | doaj-art-6d6c57dddbde4a76901f2d5da7770513 |
| institution | DOAJ |
| issn | 2198-7793 |
| language | English |
| publishDate | 2020-07-01 |
| publisher | Japan Surgical Society |
| record_format | Article |
| series | Surgical Case Reports |
| spelling | doaj-art-6d6c57dddbde4a76901f2d5da77705132025-08-20T02:52:25ZengJapan Surgical SocietySurgical Case Reports2198-77932020-07-01611710.1186/s40792-020-00954-2Malignant peripheral nerve sheath tumour of the oesophagus: a case reportKento Tomizawa0Tatsuya Miyazaki1Arisa Yamaguchi2Ryoya Honda3Marie Hoshino4Mitsuhiro Yanai5Yohei Miyamae6Ryo Kurosaki7Hisashi Shimizu8Kazuhisa Arakawa9Munenori Ide10Maebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentMaebashi Red Cross Hospital Surgery DepartmentAbstract Background Malignant 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 presentation A 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. Conclusions MPNST in the oesophagus is a relatively rare disease. Diagnosis before treatment is sometimes difficult, but the prognosis is good if radical resection is possible.http://link.springer.com/article/10.1186/s40792-020-00954-2FDG-PETKi67 indexsurgical treatment |
| spellingShingle | Kento Tomizawa Tatsuya Miyazaki Arisa Yamaguchi Ryoya Honda Marie Hoshino Mitsuhiro Yanai Yohei Miyamae Ryo Kurosaki Hisashi Shimizu Kazuhisa Arakawa Munenori Ide Malignant peripheral nerve sheath tumour of the oesophagus: a case report Surgical Case Reports FDG-PET Ki67 index surgical treatment |
| title | Malignant peripheral nerve sheath tumour of the oesophagus: a case report |
| title_full | Malignant peripheral nerve sheath tumour of the oesophagus: a case report |
| title_fullStr | Malignant peripheral nerve sheath tumour of the oesophagus: a case report |
| title_full_unstemmed | Malignant peripheral nerve sheath tumour of the oesophagus: a case report |
| title_short | Malignant peripheral nerve sheath tumour of the oesophagus: a case report |
| title_sort | malignant peripheral nerve sheath tumour of the oesophagus a case report |
| topic | FDG-PET Ki67 index surgical treatment |
| url | http://link.springer.com/article/10.1186/s40792-020-00954-2 |
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