Wong T Y, Suster S
Dermatopathology Division, Massachusetts General Hospital, Harvard Medical School, Boston.
Am J Dermatopathol. 1994 Oct;16(5):463-73. doi: 10.1097/00000372-199410000-00001.
We describe 13 cases of tricholemmal carcinoma, a rarely recognized cutaneous adnexal neoplasm. The patients were nine men and four women. In general, the tumors presented as slow-growing epidermal papules, indurated plaques, or nodules showing predilection for sun-exposed, hair-bearing skin. The lesions were most frequently misdiagnosed clinically as basal cell carcinoma. Histologically, they showed a variegation of growth patterns including solid, lobular, and trabecular; they were characterized by a proliferation of epithelial cells with features of outer root sheath differentiation, including abundant glycogen-rich, clear cytoplasm, foci of pilar-type keratinization, and peripheral palisading of cells with subnuclear vacuolization. Because of their variable growth pattern, overt cytologic atypia, abundant clear cytoplasm, occasional pagetoid intraepidermal spread, and brisk mitotic activity, these tumors may pose difficulties for diagnosis and be confused with other malignant skin tumors with clear cell changes. Despite the seemingly malignant cytological appearance of these lesions, clinical follow-up in 10 cases showed no recurrence or metastasis over a period of 2-8 years. Thus, conservative surgical excision with clear margins appears to be the treatment of choice for these neoplasms.
我们描述了13例毛鞘癌，这是一种鲜为人知的皮肤附属器肿瘤。患者中男性9例，女性4例。总体而言，肿瘤表现为生长缓慢的表皮丘疹、硬结性斑块或结节，好发于暴露于阳光、有毛发的皮肤。这些病变在临床上最常被误诊为基底细胞癌。组织学上，它们表现出多种生长模式，包括实性、小叶状和小梁状；其特征是上皮细胞增殖，具有外根鞘分化的特征，包括丰富的富含糖原的透明细胞质、毛干型角化灶以及细胞核下空泡化的细胞周围栅栏状排列。由于其生长模式多样、明显的细胞异型性、丰富的透明细胞质、偶尔的派杰样表皮内播散以及活跃的有丝分裂活性，这些肿瘤可能在诊断上存在困难，并可能与其他具有透明细胞改变的恶性皮肤肿瘤相混淆。尽管这些病变在细胞学上看似恶性，但10例患者的临床随访显示，在2至8年的时间里无复发或转移。因此，切缘清晰的保守性手术切除似乎是这些肿瘤的首选治疗方法。
