초기에 안와침범으로 나타난 원발 비부비동 신경내분비암 1예
Orbital Involvement as an Initial Presentation of Sinonasal Neuroendocrine Carcinoma
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목적 : 초기에 안와 침범으로 나타난 비부비동 신경내분비암을 보고하고자 한다. 증례요약 : 63세 여자 환자가 3주 전 시작된 우안 시력저하, 색감 이상, 윗눈꺼풀 부종으로 내원하였다. 우안 시력은 0.8이었고 우안의 상대구심동공운동장애, 외전, 상전, 내전시 안구운동장애, 안구 돌출 및 눈꺼풀 부종, 시신경유두 부종 소견 보였다. 안와 전산화단층촬영에서 우측 안와첨, 안와 및 비부비동을 침범하는 종괴가 발견되었고, 비강 내시경을 통한 조직검사상 Ki-67, NSE, p16, P-53 및 CD56 양성으로 소세포 신경내분비암으로 진단되었다. 자기공명영상촬영에서 원발 병소인 것이 확인되었고, 절제불가능 종양으로 진단되었다. 진단 기간 중 우안 시력 안전수지로 떨어졌으나, 동시 항암화학방사선치료를 시작하며 고용량 스테로이드 치료를 병행하여 우안 시력 및 안구돌출 호전 추세를 보였다. 6개월 뒤 안와 전산화단층촬영상 잔여 병변 없었으며, 최대교정시력 우안 0.3이나, 색각 및 안구운동은 정상으로 회복되었다. 결론 : 안와 침범에 대해서는 보고된 증례수가 매우 적으나, 빠르게 진행하는 압박시신경병증에 있어 드물지만 신경내분비암의 가능성을 고려해야 할 것으로 생각된다.
Abstract
Purpose: To present a case of sinonasal neuroendocrine cancer initially manifesting with orbital involvement. Case summary: A 63-year-old female patient visited a neuro-ophthalmologic clinic due to a 3-week history of decreased visual acuity (VA), color vision abnormalities, and swelling of the upper eyelid in right eye. Best-corrected VA (BCVA) in the right eye was 0.8. Clinical findings included a relative afferent pupillary defect, restricted eye movement in lateral, superior, and medial gaze, and exophthalmos in the right eye. Wide-field fundus photography and optical coherence tomography indicated swelling of right optic disc. Magnetic resonance imaging was performed revealing a mass invading nasal cavity, sinus, and right orbital apex, and compressing the medial rectus, inferior rectus, superior oblique, and optic nerve. An otolaryngological nasal biopsy was conducted and immunohistochemical staining showed positive results for Ki-67, NSE, p16, P-53, and CD56, leading to a diagnosis of small cell neuroendocrine carcinoma with an irreducible tumor stage. Prior to initiation of treatment (concurrent chemoradiotherapy, CCRT), there was rapid worsening of VA and eye movement in the right eye. CCRT commenced alongside high-dose steroid treatment. One month following treatment, the BCVA of the right eye improved and protrusion of the right eye resolved. Six months after starting CCRT, a contrast-enhanced orbital computed tomography scan showed no residual lesion. The BCVA of the right eye stabilized at 0.3 with complete recovery of color vision and eye movement. Conclusions: Neuroendocrine cancer should be considered as a possible diagnosis in cases of rapidly progressing compressive optic neuropathy.
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