Sicinska Justyna, Rakowska Adriana, Czuwara-Ladykowska Joanna, Mroz Andrzej, Lipinski Marcin, Nasierowska-Guttmejer Anna, Sikorska Jolanta, Sklinda Katarzyna, Slowinska Monika, Kowalska-Oledzka Elzbieta, Walecka Irena, Walecki Jerzy, Rudnicka Lidia
Department of Dermatology, CSK MSWiA, Warsaw, Poland.
J Dermatol Case Rep. 2007 Dec 29;1(1):4-9. doi: 10.3315/jdcr.2007.1.1002.
Brooke-Spiegler syndrome is a rare condition with a predisposition to develop cutaneous adnexal neoplasms, especially cylindromas, trichoepitheliomas and spiradenomas. Malignant transformation of cylindromas is rare. In such cases usually cylindrocarcinomas develop within these lesions. We present an unusual case of basal cell carcinoma developing within a preexisting cylindroma.
58-year-old woman with a 30-year history of multiple dermal cylindromas extensively involving her scalp was referred for dermatological treatment. The patient reported that one of the long-lasting lesions, 5.5 cm in size, ulcerated within the foregoing few weeks. Histopathology confirmed cylindromas and basal cell carcinoma within the ulcerating tumor. Surgical excision of largest cylindroma tumors led to cosmetic and functional improvement. Magnetic resonance and computed tomography showed tumor infiltration into the skull lamina externa. Metastases were excluded by chest radiography and abdominal ultrasound examination.
Patients with Brooke-Spiegler syndrome should be followed-up for malignant transformation of skin tumors to prevent deep penetration and possible metastases.
布鲁克 - 施皮格勒综合征是一种罕见疾病,易患皮肤附属器肿瘤,尤其是圆柱瘤、毛发上皮瘤和汗腺螺旋腺瘤。圆柱瘤恶变罕见。在这种情况下,通常在这些病变内发展为圆柱癌。我们报告了一例在既往存在的圆柱瘤内发生基底细胞癌的罕见病例。
一名58岁女性,有30年多发性皮肤圆柱瘤病史,广泛累及头皮,因皮肤病治疗前来就诊。患者报告称,其中一个持续存在的病变,大小为5.5厘米,在之前几周内发生了溃疡。组织病理学证实溃疡肿瘤内有圆柱瘤和基底细胞癌。手术切除最大的圆柱瘤肿瘤后,外观和功能均有改善。磁共振成像和计算机断层扫描显示肿瘤浸润至颅骨外板。胸部X线和腹部超声检查排除了转移。
布鲁克 - 施皮格勒综合征患者应随访观察皮肤肿瘤恶变情况,以防止肿瘤深部浸润和可能的转移。