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一名患有布鲁克-施皮格勒综合征的患者,其圆柱瘤转变为基底细胞癌。

Cylindroma transforming into basal cell carcinoma in a patient with Brooke-Spiegler syndrome.

作者信息

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

机构信息

Department of Dermatology, CSK MSWiA, Warsaw, Poland.

出版信息

J Dermatol Case Rep. 2007 Dec 29;1(1):4-9. doi: 10.3315/jdcr.2007.1.1002.

Abstract

BACKGROUND

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.

MAIN OBSERVATIONS

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.

CONCLUSION

Patients with Brooke-Spiegler syndrome should be followed-up for malignant transformation of skin tumors to prevent deep penetration and possible metastases.

摘要

背景

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。

主要观察结果

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。

结论

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。

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Brooke-spiegler syndrome: a rare entity.布鲁克-施皮格勒综合征:一种罕见病症。
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本文引用的文献

1
A novel missense mutation in the CYLD gene in a Spanish family with multiple familial trichoepithelioma.
Arch Dermatol. 2007 Sep;143(9):1209-10. doi: 10.1001/archderm.143.9.1209.
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Brooke-Spiegler syndrome.布鲁克-施皮格勒综合征
Dermatol Online J. 2007 Jan 27;13(1):10.
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The tumor suppressor CYLD regulates entry into mitosis.肿瘤抑制因子CYLD调节有丝分裂的进入。
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Inhibition of tumor growth by NF-kappaB inhibitors.NF-κB抑制剂对肿瘤生长的抑制作用。
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