Department of Colorectal Surgery, Royal Free Hospital, London, UK.
Department of Cancer Surgery, Peter MacCallum Cancer Centre, Melbourne, Australia.
Colorectal Dis. 2016 Sep;18(9):877-82. doi: 10.1111/codi.13209.
Anal melanoma is a rare malignancy with a poor prognosis.
All patients with a diagnosis of anal melanoma treated at a single institution between 2000 and 2012 were identified and their treatment and outcome were evaluated.
Sixteen patients had a median survival of 2.9 years. Fourteen had Stage I or II disease with a median survival of 4.0 years and progression-free survival of 1.5 years. When used for disease staging, whole body positron emission tomography/CT identified an additional three sites of metastasis in five patients compared with CT of the chest, abdomen and pelvis. Surgery involved wide local excision or abdominoperineal excision with respective local recurrence rates of 50% and 66%. Eleven patients underwent testing for c-Kit mutations, of whom five were positive. Four of these were treated with the tyrosine kinase inhibitor imatinib, and showed rapid response of metastases outside the central nervous system.
The outcome of this malignancy remains poor. PET is the modality of choice for disease staging. Testing tumours for c-Kit mutations may allow selected patients to participate in trials of tyrosine kinase inhibitors.
肛门黑色素瘤是一种罕见的恶性肿瘤，预后不良。
在一家医疗机构中，我们对 2000 年至 2012 年间所有被诊断为肛门黑色素瘤的患者进行了治疗，并评估了他们的治疗和预后。
16 名患者的中位生存期为 2.9 年。14 名患者为 I 期或 II 期疾病，中位生存期为 4.0 年，无进展生存期为 1.5 年。全身正电子发射断层扫描/计算机断层扫描（PET/CT）与胸部、腹部和骨盆 CT 相比，在 5 名患者中额外发现了三个转移部位。手术涉及广泛的局部切除或腹会阴切除，局部复发率分别为 50%和 66%。11 名患者接受了 c-Kit 突变检测，其中 5 名阳性。其中 4 名接受了酪氨酸激酶抑制剂伊马替尼治疗，其中枢神经系统以外的转移灶迅速得到缓解。
这种恶性肿瘤的预后仍然很差。PET 是疾病分期的首选方法。对肿瘤进行 c-Kit 突变检测可能使部分患者有机会参加酪氨酸激酶抑制剂的临床试验。
