Watarai Akira, Amoh Yasuyuki, Aki Ryoichi, Takasu Hiroshi, Katsuoka Kensei
Kitasato University School Of Medicine, Sagamihara, Kanagawa, Japan.
Dermatol Online J. 2011 Sep 15;17(9):5.
A 46-year-old man noticed a nodule on his sole. The nodule was removed and the specimen showed a lobular proliferation of tumor cells with glandular differentiation embedded in mucinous stroma. A diagnosis of chondroid syringoma was made. Twelve years later, he noted a swelling in the right inguinal region. The mass was surgically removed. The histopathological findings of the lymph node showed the more atypical tumor cells in the mucoid stroma. Upon reexamination, the primary tumor contained malignant chondroid syringoma (MCS) cells; the tumor cells metastasized to lymph node. MCS is rare with 43 reported cases in the literature. The site of the primary tumor was the lower extremity in 35 percent, the head in 28 percent, and the upper extremity in 23 percent. The percentage of malignant cases with local recurrence, nodal metastasis, and distant metastasis was 49 percent, 42 percent, and 40 percent, respectively. In these cases, the average time period until disease recurrence was 23 months, 50 months, and 66 months for local recurrence, nodal metastasis, and distant metastasis, respectively. Of these, 23 percent of the cases succumbed. As MCS may progress very slowly and disease recurrence including metastasis occurs in a relatively high percentage of cases, long-term follow-up of MCS cases is required.
一名46岁男性发现足底有一个结节。该结节被切除，标本显示肿瘤细胞呈小叶状增生，伴有黏液样间质中的腺管分化。诊断为软骨样汗腺螺旋瘤。12年后，他注意到右腹股沟区肿胀。该肿块被手术切除。淋巴结的组织病理学检查发现黏液样间质中有更多非典型肿瘤细胞。重新检查时，原发肿瘤中含有恶性软骨样汗腺螺旋瘤（MCS）细胞；肿瘤细胞已转移至淋巴结。MCS很罕见，文献报道有43例。原发肿瘤部位下肢占35%，头部占28%，上肢占23%。局部复发、淋巴结转移和远处转移的恶性病例百分比分别为49%、42%和40%。在这些病例中，局部复发、淋巴结转移和远处转移的疾病复发平均时间分别为23个月、50个月和66个月。其中，23%的病例死亡。由于MCS可能进展非常缓慢，且包括转移在内的疾病复发在相当高比例的病例中发生，因此需要对MCS病例进行长期随访。
