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伴有广泛胸壁受累的肉芽肿性蕈样霉菌病。

Granulomatous mycosis fungoides with extensive chest wall involvement.

作者信息

Von Nida Jamie, Randell Peter, Heenan Peter

机构信息

Department of Dermatology, Sir Charles Gairdner Hospital, Perth and Cutaneous Pathology, Nedlands, Western Australia, Australia.

出版信息

Australas J Dermatol. 2004 Feb;45(1):42-6. doi: 10.1111/j.1440-0960.2004.00029.x.

DOI:10.1111/j.1440-0960.2004.00029.x
PMID:14961908
Abstract

A 40-year-old woman presented with a 5-year history of a mass overlying her right pectoralis major muscle. Histopathology of the lesion revealed a florid granulomatous infiltrate including an atypical lymphocytic component with marked epidermotropism consistent with granulomatous mycosis fungoides. Staging investigations demonstrated the tumour to be localized to the right chest. Consequently, the patient was treated with radiotherapy (50 Gy) to the lesion with good clinical effect. However, she soon developed a clinically palpable lesion on the left chest outside the radiotherapy field. Positron emission tomography scanning demonstrated an extensive left-sided chest wall tumour and also residual tumour on the right. This left-sided lesion failed to respond to systemic chemotherapy. Further radiotherapy (50 Gy) has recently been administered to the left chest lesion; the response is being monitored. While granulomatous inflammation has been previously described in cutaneous T-cell lymphomas, it is rare and is often associated with a delay in the diagnosis and difficulty with clinical staging. The clinical presentation can be extremely variable and consequently, diagnosis rests with histological features, immunohistochemical studies and gene rearrangement analysis.

摘要

一名40岁女性,右胸大肌上方肿物已有5年病史。病变的组织病理学检查显示为大量肉芽肿浸润,包括具有明显亲表皮性的非典型淋巴细胞成分,符合肉芽肿性蕈样霉菌病。分期检查表明肿瘤局限于右胸。因此,患者接受了针对该病变的放射治疗(50 Gy),临床效果良好。然而,她很快在放疗野之外的左胸出现了临床上可触及的病变。正电子发射断层扫描显示左侧胸壁有广泛肿瘤,右侧也有残留肿瘤。左侧病变对全身化疗无反应。最近对左侧胸部病变进行了进一步放射治疗(50 Gy);正在监测其反应。虽然皮肤T细胞淋巴瘤中先前已描述过肉芽肿性炎症,但很罕见,且常伴有诊断延迟和临床分期困难。临床表现可能极其多样,因此,诊断取决于组织学特征、免疫组化研究和基因重排分析。

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引用本文的文献

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Co-existence of various clinical and histopathological features of mycosis fungoides in a young female.一名年轻女性蕈样肉芽肿多种临床和组织病理学特征并存。
Indian J Dermatol. 2015 Mar-Apr;60(2):214. doi: 10.4103/0019-5154.152588.
2
Positron emission tomography for staging and response assessment of mycosis fungoides in a child.正电子发射断层扫描在儿童蕈样肉芽肿分期及疗效评估中的应用
Indian J Dermatol. 2013 Mar;58(2):147-8. doi: 10.4103/0019-5154.108062.