Papageorgiou K I, Kaniorou-Larai M G
St Andrews Center of Burns and Plastic Surgery, Broomfield Hospital, Chelmsford, CM1 7ET, UK.
BMC Cancer. 2005 Aug 19;5:106. doi: 10.1186/1471-2407-5-106.
Chronic lymphocytic leukemia (CLL) is a monoclonal disorder, characterized by a progressive proliferation of functionally incompetent B lymphocytes. There is increased evidence of association between CLL and skin cancers, including the uncommon Merkel cell carcinoma (MCC).
A case report of an 84-year old male, who presented with an aggressively recurrent form of MCC on the lower lip, on the background of an 8-year history of untreated CLL. During the recurrences of MCC, coexisting regional lymphadenopathy, posed a problem in the differential diagnosis and treatment of lymph node involvement. Histopathology and immunoistochemistry showed that submandibular lymphadenopathy coexisting with the second recurrence of MCC, was due to B-cell small lymphocytic lymphoma. The subsequent and more aggressive recurrence of the skin tumor had involved the superficial and deep cervical lymph nodes. Surgical excision followed by involved field radiation therapy has been proven effective for both malignancies.
MCC has a high incidence of regional lymphadenopathy at presentation (12-45%) and even when it arises on the background of chronic leukemia, lymphadenopathy at presentation should be managed agressively with elective lymph node dissection. We overview the postulated correlation between Merkel tumor and CCL, the differential diagnosis of regional lymphadenopathy during the recurrences of the skin tumor and the strategies of treatment.
慢性淋巴细胞白血病(CLL)是一种单克隆疾病,其特征为功能不全的B淋巴细胞进行性增殖。越来越多的证据表明CLL与皮肤癌之间存在关联,包括罕见的默克尔细胞癌(MCC)。
一名84岁男性的病例报告,该患者在未经治疗的CLL病史8年的背景下,下唇出现侵袭性复发性MCC。在MCC复发期间,并存的区域淋巴结病给淋巴结受累的鉴别诊断和治疗带来了问题。组织病理学和免疫组织化学显示,与MCC第二次复发并存的下颌下淋巴结病是由B细胞小淋巴细胞淋巴瘤引起的。皮肤肿瘤随后更具侵袭性的复发累及了颈浅和颈深淋巴结。手术切除后行受累野放射治疗已被证明对这两种恶性肿瘤均有效。
MCC初诊时区域淋巴结病的发生率较高(12 - 45%),即使它发生在慢性白血病的背景下,初诊时的淋巴结病也应积极进行选择性淋巴结清扫处理。我们概述了默克尔肿瘤与CCL之间的假定相关性、皮肤肿瘤复发期间区域淋巴结病的鉴别诊断以及治疗策略。