소세포신경내분비암종의 안와 전이로 진단된 전체외안근마비
Metastatic Small Cell Neuroendocrine Carcinoma to the Orbit Manifesting as Total Ophthalmoplegia
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목적: 좌측 안와부에 전이된 요관 원발성으로 고려되는 전이성 소세포신경내분비암종의 1예를 문헌 고찰과 함께 보고하고자 한다. 증례요약: 79세 남자 환자가 내원 10일 전부터 발생한 좌측 안와부 통증과 안검하수 및 안구운동제한 주소로 본원에 내원하였다. 당뇨 및 전립선샘암종으로 치료를 받은 과거력이 있었고, 최대교정시력은 우안 0.8, 좌안 0.5로 좌안의 안구돌출과 안구운동제한 및 안검하수가 관찰되었다. 안와 단층촬영검사 및 자기공명영상검사에서 좌측 안와부 상외측으로 불규칙한 종괴 모양의 조영증강이 관찰되어 감염성 및 염증성 안와질환이 의심되었다. 항생제 치료 이후 고용량 전신 스테로이드 투여를 시행하였으나 호전이 없어, 재시행한 안와 단층촬영검사상 초진 시와 변화 없는 소견이었다. 이에 안와종양 감별을 위해 시행한 조직검사에서 소세포신경내분비암종으로 진단되었고, 이후 양전자방출단층촬영을 통해 다발성의 전신전이가 관찰되는 전이성 안와 소세포신경내분비암종으로 진단되었다. 결론: 안와부 통증, 안구운동제한, 안검하수와 같은 임상양상을 보이는 환자에서 영상학적 검사를 하여 종괴 소견이 관찰되면 진단 및 치료 방향 결정을 위해 조직검사를 반드시 고려해야 한다.
Abstract
Purpose: To report a case of primary ureteral small cell neuroendocrine carcinoma metastasis in the left orbital wall, with a review of the literature. Case summary: A 79-year-old male visited our clinic with a 10 day history of ocular pain, ptosis, and ophthalmoplegia in the left eye. He had been diagnosed with diabetes mellitus and was being treated for prostate adenocarcinoma. The corrected visual acuity was 0.8 in the right eye and 0.5 in the left eye. An extraocular movement test showed total ophthalmoplegia, mild exophthalmos, and ptosis in the left eye. Orbital computed tomography (CT) and magnetic resonance imaging showed an irregular mass-like enhancement in the superolateral orbital wall of the left eye, suggesting infectious and inflammatory orbital disease. After antibiotic treatment, high dose systemic steroids were administered. However, there was no improvement and the orbital CT was again performed, with no changes. A surgical biopsy for differentiating orbital tumors was performed and diagnosed as a metastatic orbital small cell neuroendocrine carcinoma. Using positron emission tomography, he was later diagnosed with a metastatic orbital small cell neuroendocrine carcinoma with multiple metastases. Conclusions: When the patient has a mass-like lesion on imaging with ocular pain, ophthalmoplegia, or ptosis, a surgical biopsy should be performed to make a diagnosis and determine the optimal management.
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