Tatla Taran, Hungerford John, Plowman Nick, Ghufoor Khalid, Keene Malcolm
Department of Otorhinolaryngology--Head and Neck Surgery, St. Bartholomew's Hospital, West Smithfield, London, UK.
Laryngoscope. 2005 May;115(5):817-22. doi: 10.1097/01.MLG.0000157327.10597.86.
To evaluate prognostic factors and determine the role of conservative surgery and radiotherapy in managing metastatic conjunctival malignant melanoma (MM) involving preauricular/submandibular lymph nodes.
A retrospective analysis (1990-2003) of clinical and histopathologic data from 12 patients presenting with regional metastases after failed local treatment for conjunctival MM. Patients received a common, multispecialty, conservative management approach: wide local excision, topical cryotherapy or radiotherapy to conjunctival MM (orbital exenteration for more advanced local disease), lumpectomy, and adjuvant "ring" radiotherapy of regional metastases, with chemotherapy for distant metastases.
Median age at primary diagnosis was 51 (range 28-86) years with equal sex predilection. Six of the 12 patients had primary tumors of the bulbar conjunctiva; the remainder arose in the palpebral conjunctiva, the caruncle, or the fornix. Of 11 originating in primary acquired melanosis (PAM), 2 were amelanotic. Epithelioid tumor cells were noted histologically in seven of eight specimens in which cell type could be determined. Eight tumors metastasised to preauricular nodes, three to submandibular and one to both, with a median interval of 23 (range 12-108) months after primary diagnosis. After conservative surgery and "ring irradiation," 7 of 12 patients remained free of regional nodal relapse at median interval of 16 (range 3-126) months. Five patients developed regional nodal recurrence at median interval of 11 (range 6-13) months, 3 of whom were within radiotherapy portals. Eight patients developed distant metastasis at median interval of 44 (range 22-138) months. Eleven patients had tumor-related death. The mean Kaplan-Meier adjusted survival time after primary diagnosis was 76 months with death ensuing postregional metastasis within a median 18 (range 4-127) months. The sole survivor's follow-up duration was 56 months.
Locoregional metastasis after treatment for conjunctival MM is associated with a poor prognosis. Both epithelioid tumor cells and PAM are associated with disseminating disease and poorer outcome. Literature review has failed to demonstrate advantages of mutilating radical surgery over a conservative approach in this rare disease.
评估预后因素，并确定保守手术和放疗在治疗累及耳前/颌下淋巴结的转移性结膜恶性黑色素瘤（MM）中的作用。
对12例结膜MM局部治疗失败后出现区域转移的患者的临床和组织病理学数据进行回顾性分析（1990 - 2003年）。患者接受了一种常见的多专科保守治疗方法：结膜MM的广泛局部切除、局部冷冻疗法或放疗（对于更晚期的局部病变行眶内容剜除术）、肿块切除术以及区域转移灶的辅助“环形”放疗，远处转移则采用化疗。
初次诊断时的中位年龄为51岁（范围28 - 86岁），男女比例相近。12例患者中有6例原发肿瘤位于球结膜；其余起源于睑结膜、泪阜或穹窿部。在11例起源于原发性获得性黑色素沉着症（PAM）的患者中，2例为无色素性。在8个可确定细胞类型的标本中，有7个在组织学上可见上皮样肿瘤细胞。8个肿瘤转移至耳前淋巴结，3个转移至颌下淋巴结，1个同时转移至两者，初次诊断后的中位间隔时间为23个月（范围12 - 108个月）。经过保守手术和“环形照射”后，12例患者中有7例在中位间隔时间16个月（范围3 - 126个月）时区域淋巴结无复发。5例患者在中位间隔时间11个月（范围6 - 13个月）时出现区域淋巴结复发，其中3例在放疗野内。8例患者在中位间隔时间44个月（范围22 - 138个月）时出现远处转移。11例患者因肿瘤相关死亡。初次诊断后的平均Kaplan - Meier调整生存时间为76个月，区域转移后中位18个月（范围4 - 127个月）死亡。唯一幸存者的随访时间为56个月。
结膜MM治疗后的局部区域转移预后不良。上皮样肿瘤细胞和PAM均与疾病播散及较差的预后相关。文献综述未能证明在这种罕见疾病中，根治性手术相较于保守治疗方法具有优势。
