Journal of the Korean Orthopaedic Association (대한정형외과학회지)

Korean Orthopaedic Association (대한정형외과학회)
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Giant Schwannoma May Mimic Soft Tissue Sarcoma

악성 연부 종양으로 오인하기 쉬운 신경 및 연부조직의 거대 신경초종

  • Kim, Yongsung (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Jeon, Dae-Geun (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Cho, Wan Hyeong (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Song, Won Seok (Department of Orthopedic Surgery, Korea Cancer Center Hospital) ;
  • Kim, Kyunghoon (Department of Orthopedic Surgery, Korea Cancer Center Hospital)
  • Received : 2020.01.02
  • Accepted : 2020.04.01
  • Published : 2020.12.30
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Abstract

Purpose: Schwannoma is a benign tumor that occurs mainly in the peripheral nerve. If the tumor is large or is in areas other than the nerves, it is likely to be mistaken for malignant soft tissue tumors. The authors reviewed 50 cases of giant schwannomas and assessed the distribution of the primary locations, clinical symptoms, radiological and pathological diagnosis, and diagnostic accuracy. Materials and Methods: Of the 214 pathologically confirmed schwannomas, 50 cases with a maximum diameter of 5 cm or more were extracted. The entire cohort was classified into three subgroups (major peripheral nerve, intramuscular, bone) according to the primary location, and the anatomical locations were specified. Results: When the entire cohort was classified according to the primary location, 14 tumors occurred in the major peripheral nerve, 31 cases in the muscle, and 5 cases in the bone. The mean size of the tumor in the entire cohort was 7.0 cm, and the intramuscular subgroup had the largest size with 8.0 cm. The radiological diagnosis revealed 33 out of 50 cases to be benign schwannoma (66.0%), 15 cases as low-grade malignancy (30.0%), and the remaining two cases (4.0%) as a suspicious tuberculosis abscess and tenosynovial giant cell tumor, respectively. On the clinical symptoms, Tinel sign was the most common in the peripheral nerve group with 78.6% (11/14), while 93.5% of the intramuscular group had palpation of the mass with a mean duration of 66.6 months. In the bone group, one out of five cases was reported as a low-grade malignancy. Two cases of postoperative complications were encountered; one was bleeding after tumor excision, which required hemostasis, and the other was peroneal nerve palsy after surgery. Conclusion: When assessing the large-sized soft tissue tumors in the muscles, the possibility of a benign schwannoma should be considered if 1) there is a long period of mass palpation and 2) non-specific findings in MRI. Preoperative pathology confirmation with a biopsy can help reduce the risk of overtreatment.

목적: 신경초종은 말초신경에 주로 발생하는 양성종양이나 크기가 크거나 신경 이외의 부위에서 발견될 경우 악성 연부조직 종양으로 오인하기 쉽다. 저자들은 크기 5 cm 이상의 신경초종 50예의 1) 원발 위치의 분포 및 임상증상여부, 2) 술 전 방사선 및 병리학적 진단 결과와 진단적 정확도에 대해 알아보았다. 대상 및 방법: 신경초종으로 확진된 214예 중 자기공명영상(magnetic resonance imaging, MRI)상 종양의 최대직경이 5 cm 이상인 종양 50예를 추출하였다. 이를 주요신경 기원, 근육 내, 골 내의 원발위치에 따라 분류하였고 구체적인 해부학적 위치를 분석하였다. 결과: 전체 코호트에서 원발위치에 따라 분류하였을 때 주요 말초신경에 생긴 종양이 14예, 근육 내 발생이 31예, 골 내 발생이 5예였다. 신경초종의 평균크기는 7 cm였으며 근육내 신경초종이 평균 8 cm로 가장 컸다. MRI를 통한 영상학적 진단에서 전체 50예 중 33예(66.0%)는 양성 신경종양으로, 15예(30.0%)는 악성종양으로 판독하였으며, 나머지 2예(4.0%)는 각각 결핵 농양, 건활막 거대세포종으로 보고되었다. 골내 신경초종 5예 중 영상학적으로 신경초종으로 진단한 예는 없었다. 임상증상에서는 주요 신경기원의 경우는 Tinel sign이 78.6%로 많았고(11/14), 주요 신경 외 기원 종양에서는 종괴 촉지 증상이 93.5%로 가장 많았으며(29/31) 증상기간도 평균 66.6개월로 가장 길었다. 병리학적 진단을 위해 생검을 시행한 총 38예 중 86.8%에서는 신경초종으로 진단하였다. 합병증은 수술 후 합병증이 총 2예였으며 종양절제 후 출혈로 재수술이 필요했던 경우와 수술 후 경한 신경마비가 발생한 경우였다. 결론: 5 cm 이상의 근육 내 종양을 진단할 때 장기간의 종괴인지 소견이 있고, MRI상 비특이적인 소견일 경우에는 양상 신경종양일 가능성도 염두에 두고 조직학적 확진 후 치료를 시행하는 것이 과잉 치료 가능성을 줄이는 데 도움이 될 것으로 생각된다.

Keywords

References

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