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一例罕见的以严重肌炎为副肿瘤综合征的乳腺癌病例。

A rare case of severe myositis as paraneoplastic syndrome on breast cancer.

作者信息

Dias Leonardo Pires Novais, Faria Ana Luiza Antunes, Scandiuzzi Maissa Marçola, Inhaia Claudia Luci dos Santos, Shida Jorge Yoshinori, Gebrim Luiz Henrique

机构信息

Department of Senology, Pérola Byington Hospital, Avenida Brigadeiro Luís Antônio 683, Bela Vista, , CEP, 01317-000,, São Paulo - SP, Brazil.

出版信息

World J Surg Oncol. 2015 Apr 1;13:134. doi: 10.1186/s12957-015-0534-5.

DOI:10.1186/s12957-015-0534-5
PMID:25890160
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4397703/
Abstract

BACKGROUND

Dermatomyositis and polymyositis are both types of idiopathic inflammatory myositis characterized by inflammation and weakness of proximal skeletal muscles and skin rash.

CASE

A 49-year-old Caucasian woman recently diagnosed with breast cancer classified as T1N2M0, stage IIIA, presenting skin rash associated with heliotrope and Gottron's papules. In addition, there was a progression to a severe reduction in proximal muscle strength with severe dysphagia. The initial treatment was conducted, and the patient recovered from all symptoms and followed adjuvant cancer management.

TREATMENT

At first, high dose of corticosteroid was administered as pulse therapy, and a radical mastectomy was indicated due to the severe symptoms of the paraneoplastic syndrome. Then chemotherapy and radiotherapy were applied, and oral corticoid associated with immunosupressive drug was administered for dermatomyositis control.

DISCUSSION

The association between myositis and an increased risk of cancer has been demonstrated over the years. This patient has a high probability of dermatomyositis diagnosis. The initial treatment with high dose of glucocorticoids may result in an improvement of muscle lesions. Second-line treatment with azathioprine, methotrexate, or cyclophosphamide may be required for aggressive disease. Removal of the cancer induces improvement of paraneoplastic syndrome.

CONCLUSION

Dermatomyositis can be a clinical manifestation of a paraneoplastic syndrome in patients with breast cancer. It is a rare diagnosis, and there is little evidence to guide treatment until now. It is possible to control the evolution of dermatomyositis with high doses of glucocorticoids in almost all cases; however, in severe cases of paraneoplastic syndrome, cancer treatment should start immediately.

摘要

背景

皮肌炎和多肌炎均为特发性炎性肌病,其特征为近端骨骼肌炎症和无力以及皮疹。

病例

一名49岁的白种女性,最近被诊断为T1N2M0 IIIA期乳腺癌,出现与向阳疹和Gottron丘疹相关的皮疹。此外,近端肌肉力量严重下降并伴有严重吞咽困难。进行了初始治疗,患者所有症状均恢复,并接受了辅助癌症治疗。

治疗

起初,给予大剂量糖皮质激素进行冲击治疗,由于副肿瘤综合征症状严重,行根治性乳房切除术。然后进行化疗和放疗,并给予口服皮质类固醇联合免疫抑制药物以控制皮肌炎。

讨论

多年来已证实肌炎与癌症风险增加之间存在关联。该患者皮肌炎诊断可能性高。大剂量糖皮质激素初始治疗可能会改善肌肉病变。对于侵袭性疾病可能需要硫唑嘌呤、甲氨蝶呤或环磷酰胺进行二线治疗。切除癌症可使副肿瘤综合征得到改善。

结论

皮肌炎可能是乳腺癌患者副肿瘤综合征的一种临床表现。这是一种罕见的诊断,迄今为止几乎没有证据指导治疗。几乎在所有病例中,大剂量糖皮质激素都有可能控制皮肌炎的进展;然而,在严重的副肿瘤综合征病例中,应立即开始癌症治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/8db85910ec17/12957_2015_534_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/3dc4458a9df7/12957_2015_534_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/73deb075f028/12957_2015_534_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/f03b4671f5c5/12957_2015_534_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/2078c074196e/12957_2015_534_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/d2cb534e3a68/12957_2015_534_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/8db85910ec17/12957_2015_534_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/3dc4458a9df7/12957_2015_534_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/73deb075f028/12957_2015_534_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/f03b4671f5c5/12957_2015_534_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/2078c074196e/12957_2015_534_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/d2cb534e3a68/12957_2015_534_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/102e/4397703/8db85910ec17/12957_2015_534_Fig6_HTML.jpg

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