Multi-Disciplinary Management of Hidradenocarcinoma

Mariam Hina, Asra Hasan, Maria Tariq, Muhammad Muaz Abbasi, Muneeb Uddin Karim
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Abstract

Hidradenocarcinoma makes up 6% of eccrine malignancies, and is an uncommon eccrine sweat gland tumour. The etiology is due to genetic alterations and transformation from an existing lesion or hidradenoma. It typically affects the face and scalp, although it can also cause nodal involvement and show up on the trunk, abdomen, or groin. Due to its similarities to other skin tumours, it is challenging to diagnose histopathologically. Therefore, immunohistochemical markers, such as p53, Ki-67, keratin AE1/AE3, and cytokeratin 5/6, are crucial for the diagnoses. There are no prospective randomized trials available to direct treatment of hidradenocarcinoma. A key component of therapy is early diagnoses and case discussion in multidisciplinary tumor board. Wide local excision with clear margins is the mainstay of treatment. Adjuvant treatment like chemo radiation therapy is incorporated if nodal disease or positive margins are present. Recurrences are treated by re-excision followed by adjuvant radiation therapy. Adjuvant hormonal therapy depends on the receptor status of the disease. These tumors have a 50% recurrence rate and up to 60% of the time they metastasize to the brain, bone, lung, liver, mediastinum, or peritoneum in a two-year period. The overall five year survival rate is around 30%. Keeping in view the aggressive course of this disease, research based clinically evident new treatment strategies are the need of time to optimize overall survival and local disease control. Keywords: Sweat gland tumors, Adnexal tumors, Hidradenocarcinoma, Adjuvant therapy, Multidisciplinary team, Radiation therapy.
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汗腺癌的多学科管理
汗腺癌占内分泌恶性肿瘤的6%,是一种罕见的内分泌汗腺肿瘤。病因是由于现有病变或汗腺瘤的遗传改变和转化。它通常影响面部和头皮,尽管它也可能导致淋巴结受累,出现在躯干、腹部或腹股沟。由于其与其他皮肤肿瘤的相似性,其组织病理学诊断具有挑战性。因此,免疫组织化学标志物,如p53、Ki-67、角蛋白AE1/AE3和细胞角蛋白5/6对诊断至关重要。目前尚无直接治疗腺癌的前瞻性随机试验。多学科肿瘤委员会的早期诊断和病例讨论是治疗的关键组成部分。广泛的局部切除和清晰的边缘是治疗的主要方法。如果存在淋巴结疾病或阳性边缘,则合并化疗等辅助治疗。复发的治疗是再切除,然后辅助放射治疗。辅助激素治疗取决于疾病的受体状态。这些肿瘤的复发率为50%,在两年的时间内,有高达60%的时间转移到脑、骨、肺、肝、纵隔或腹膜。总体5年生存率约为30%。考虑到本病病程的侵袭性,基于临床的新治疗策略的研究需要时间来优化总生存期和局部疾病控制。关键词:汗腺肿瘤,附件肿瘤,汗腺癌,辅助治疗,多学科团队,放射治疗
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