成年孕婦下顎髁突之單病灶蘭格罕氏組織細胞增生症－病例報告

蘭格罕氏組織細胞增生症是一種罕見疾病，其特徵在於蘭格罕氏細胞異常的增殖，導致局部或全身症狀，臨床症狀取決於發生部位以及侵犯程度。常侵犯的骨骼為顱骨、骨盆、脊椎、顎骨等，病因目前未明。本病例為一位三十六歲懷孕女性，因右側顳顎關節處疼痛與張口受限，保守治療後改善有限，轉至本科門診就診。影像檢查顯示右側髁狀突骨缺損，切片診斷為蘭格罕氏組織細胞增生症。經全身檢查後確定為單病灶，因此採取手術切除，術後未接受化學治療。術後持續追蹤至今三年無復發及轉移。本病例因其原發部位為髁狀突，加以病患年紀較長、又遇妊娠，相關病徵亦與顳顎障礙症類似而不易區分，特此提出，藉以提高臨床醫師之警覺性，並回顧文獻。

關鍵字

蘭格罕氏組織細胞增生症；單病灶；髁突；孕婦；成人

並列摘要

Langerhans cell histiocytosis (LCH) is a rare disease, characterized by abnormal proliferation and infiltration of marrow-derived Langerhans cell. The LCH can be clinically divided into different groups based on location and degree of organ involvement. The most involved bone areas were skull, pelvis, spine, jaws with unknown etiology. This 36-year-old pregnancy female complained of pain over right temporomandibular joint and trismus for one month. After conservative treatment, the symptom was still presented. The image examination showed bony destruction over right condyle area. Incisional biopsy was performed, and the histopathologic report showed LCH. Tumor staging was done, and the final diagnosis was solitary Langerhans cell histiocytosis over right mandibular condyle. Wide excision via curette technique was done under general anesthesia, without adjuvant chemotherapy. There was no evidence of recurrence or metastasis after 3 years follow up. Considering the fact that this is a rare condition, solitary lesion involving mandibular condyle in an adult pregnancy woman that initially showed clinical symptoms mimicking the common temporomandibular disorders, which increased the difficulty on differential diagnosis. Herein we report this case to raise clinician's alertness and also review the literature.

並列關鍵字

Langerhans cell histiocytosis ； Solitary ； Condyle ； Pregnancy ； Adult

主題瀏覽