Apocrine Carcinoma of Breast in a Male Patient: Case Report

S. Touimi, D. N’chiepo, I. Mbarki, H. Elkacemi, S. ElMajjaoui, T. Kebdani, N. Benjaafar, C. Neftah, A. Saoud, J. Boulaarab, H. Jerguigue, Y. Omor, R. Latib, H. El Agouri, B. El khannoussi
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Physical examination revealed a left axilary lymphadenopathy movable relative to superficial and deep plans with no evidence mass of breasts. MRI of the breast was performed and revealed a mass that was 38 x 10 mm in size.A biopsy of the lymphadenopathy was performed. It objectified a carcinomatous proliferation. An immunohistochemical study showed that tumor cells express Her 2, but do not express estrogenic and progesterone receptors. A tumorectomy of the left breast was performed and didn’t show any malignant lesion of the breast. The axiler dissection of 13 lymphnodes showed 11 metastatics ones with 3 breaking capsular. The diagnosis of apocrine carcinoma of the breast was made in despite of the result of the tumorectomy. The CT did not indicate metastasis. The patient was administered adjuvant chemotherapy then he received radiation therapy on left susclavicular, axila and breast with a total dose of 42Gy,15 fractions of 2.8 Gy on 21 days with no late effects. 1 year of trastuzumab was administrated. There was no recurrence or metastasis approximately 2 years after radiation therapy. Then the patient presented a susclavicular lymphnode that was comfirmed on the pet-scanner with multiple mediastinal lymphnodes. A biopsy of the susclavicular lymphnode comfirmed the progression of the disease. The patient started chemotherapy in association with pertuzumab and trastuzumab. Discussion: Apocrine carcinoma of the breast is a rare malignant tumor whose incidence varies between 0.3% and 4% of all female’s breast cancer and represents 0.5 % of all invasive breast cancers. This tumor is exceptional in men. Indeed, only a dozen cases have been described in the literature . Most neoplasms are slowly progressive, small in size, and are most frequently seen in the axilla. They can be recurrent and metastasize to the lymph node, lung, and bone. Male patients have been advanced disease at presentation compared to women which may be due to lack of public awareness of breast cancer in male. Histologically, it has glandular structures with apocrine features and decapitation secretions. There is cytoplasmic PAS positivity of the tumor cells. The presence of neoplastic glands high in the dermis and immediate subepidermis favors the primary origin of tumor cells from apocrine sweat glands. Apocrine adenocarcinomas are positive for cytokeratins, carcinoembryogenic antigen (CEA) and epithelial membrane antigen (EMA) . Usually, these tumors do not express the estrogen receptor-alpha, progesterone receptors and bcl-2. Apocrine adenocarcinoma has poor prognosis and the prognostic factors include size, histological type, lymph node involvement, and distant metastasis. The 10-year disease free survival rate in the absence of metastasis to the lymph nodes is 56%. Treatment protocols of apocrine carcinoma are similar to non apocrine carcinoma of breast. However studies involving the use of anti androgens are in progress. The treatment of choice is wide local excision with clear margins, with or without regional lymph node dissection. The role of radiation therapy also remains uncertain in the absence of clinical trials. No clear correlation between treatment modality and recurrence in apocrine carcinoma was apparent, and survival rates of apocrine carcinoma were not different from other breast carcinomas. Conclusion: In conclusion, male apocrine carcinoma is a very rare, unique and morphologically-distinctive, invasive ductal carcinoma.. Although immunohistochemical staining might show differences in males, the prognosis is not different from other breast carcinomas. 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引用次数: 2

Abstract

Introduction: Apocrine carcinoma of breast is a rare type of malignant tumor, the incidence of which varies between 0.3 - 0.4 % of all female breast cancers.Apocrine carcinoma is exceptional in male patients and very few cases have been described in literature. This tumor shows distinct microscopic and immunohistological features. We report an exceptional observation of apocrine carcinoma of breast in a man. Patient and observation: He’s a 54 years old man who had for 2months a painless nodule at the left axillary..The patient had family history of breast and prostate cancers. Physical examination revealed a left axilary lymphadenopathy movable relative to superficial and deep plans with no evidence mass of breasts. MRI of the breast was performed and revealed a mass that was 38 x 10 mm in size.A biopsy of the lymphadenopathy was performed. It objectified a carcinomatous proliferation. An immunohistochemical study showed that tumor cells express Her 2, but do not express estrogenic and progesterone receptors. A tumorectomy of the left breast was performed and didn’t show any malignant lesion of the breast. The axiler dissection of 13 lymphnodes showed 11 metastatics ones with 3 breaking capsular. The diagnosis of apocrine carcinoma of the breast was made in despite of the result of the tumorectomy. The CT did not indicate metastasis. The patient was administered adjuvant chemotherapy then he received radiation therapy on left susclavicular, axila and breast with a total dose of 42Gy,15 fractions of 2.8 Gy on 21 days with no late effects. 1 year of trastuzumab was administrated. There was no recurrence or metastasis approximately 2 years after radiation therapy. Then the patient presented a susclavicular lymphnode that was comfirmed on the pet-scanner with multiple mediastinal lymphnodes. A biopsy of the susclavicular lymphnode comfirmed the progression of the disease. The patient started chemotherapy in association with pertuzumab and trastuzumab. Discussion: Apocrine carcinoma of the breast is a rare malignant tumor whose incidence varies between 0.3% and 4% of all female’s breast cancer and represents 0.5 % of all invasive breast cancers. This tumor is exceptional in men. Indeed, only a dozen cases have been described in the literature . Most neoplasms are slowly progressive, small in size, and are most frequently seen in the axilla. They can be recurrent and metastasize to the lymph node, lung, and bone. Male patients have been advanced disease at presentation compared to women which may be due to lack of public awareness of breast cancer in male. Histologically, it has glandular structures with apocrine features and decapitation secretions. There is cytoplasmic PAS positivity of the tumor cells. The presence of neoplastic glands high in the dermis and immediate subepidermis favors the primary origin of tumor cells from apocrine sweat glands. Apocrine adenocarcinomas are positive for cytokeratins, carcinoembryogenic antigen (CEA) and epithelial membrane antigen (EMA) . Usually, these tumors do not express the estrogen receptor-alpha, progesterone receptors and bcl-2. Apocrine adenocarcinoma has poor prognosis and the prognostic factors include size, histological type, lymph node involvement, and distant metastasis. The 10-year disease free survival rate in the absence of metastasis to the lymph nodes is 56%. Treatment protocols of apocrine carcinoma are similar to non apocrine carcinoma of breast. However studies involving the use of anti androgens are in progress. The treatment of choice is wide local excision with clear margins, with or without regional lymph node dissection. The role of radiation therapy also remains uncertain in the absence of clinical trials. No clear correlation between treatment modality and recurrence in apocrine carcinoma was apparent, and survival rates of apocrine carcinoma were not different from other breast carcinomas. Conclusion: In conclusion, male apocrine carcinoma is a very rare, unique and morphologically-distinctive, invasive ductal carcinoma.. Although immunohistochemical staining might show differences in males, the prognosis is not different from other breast carcinomas. It has different hormonal profile, androgen receptor positivity makes patient with apocrine carcinoma eligible for targeted therapy.
男性乳腺大汗腺癌1例
简介:乳腺大汗腺癌是一种罕见的恶性肿瘤,其发病率在所有女性乳腺癌的0.3 - 0.4%之间。大汗腺癌在男性患者中是罕见的,文献中很少有病例描述。该肿瘤表现出明显的显微镜和免疫组织学特征。我们报告一个特殊的观察大汗腺癌的乳房在一个男人。患者与观察:54岁男性,左腋窝无痛性结节2个月,有乳腺癌、前列腺癌家族史。体格检查显示左侧腋窝淋巴结病变,相对于浅表和深部计划可移动,没有乳房肿块的证据。乳房MRI显示肿块大小为38 x 10mm。对淋巴结病变进行活检。这是一种癌性增生。一项免疫组织化学研究表明,肿瘤细胞表达Her 2,但不表达雌激素受体和孕激素受体。左乳房肿瘤切除后未发现任何恶性病变。13个淋巴结腋部解剖显示11个淋巴结转移,3个被囊破裂。乳腺大汗腺癌的诊断是尽管肿瘤切除术的结果。CT未见转移。患者给予辅助化疗后,行左锁骨、腋窝、乳房放射治疗,总剂量42Gy,15次2.8 Gy,持续21天,无迟发效应。给予曲妥珠单抗治疗1年。放疗后约2年无复发或转移。然后患者表现为锁骨淋巴结,pet扫描证实为多发纵隔淋巴结。锁骨淋巴结活检证实了病情的进展。患者开始化疗与帕妥珠单抗和曲妥珠单抗相关。讨论:乳腺大汗腺癌是一种罕见的恶性肿瘤,其发病率在所有女性乳腺癌的0.3%至4%之间,占所有浸润性乳腺癌的0.5%。这种肿瘤在男性中很少见。事实上,文献中只描述了十几个病例。大多数肿瘤进展缓慢,体积小,最常见于腋窝。它们可以复发并转移到淋巴结、肺和骨。与女性患者相比,男性患者在发病时已处于疾病晚期,这可能是由于公众对男性乳腺癌缺乏认识。组织学上,它具有腺状结构,具有大汗腺特征和头颅分泌物。肿瘤细胞胞质PAS阳性。肿瘤腺体位于真皮和直接表皮下,有利于肿瘤细胞主要来自大汗腺。大汗腺癌细胞角蛋白、癌胚性抗原(CEA)和上皮膜抗原(EMA)呈阳性。通常,这些肿瘤不表达雌激素受体- α、孕激素受体和bcl-2。大汗腺癌预后较差,其预后因素包括大小、组织学类型、淋巴结累及及远处转移。无淋巴结转移的10年无病生存率为56%。大汗腺癌的治疗方案与乳腺非大汗腺癌相似。然而,有关使用抗雄激素的研究正在进行中。治疗的选择是广泛的局部切除,边界清晰,有或没有区域淋巴结清扫。在缺乏临床试验的情况下,放射治疗的作用仍然不确定。大汗腺癌的治疗方式与复发无明显的相关性,大汗腺癌的生存率与其他乳腺癌无明显差异。结论:男性大汗腺癌是一种非常罕见、独特、形态独特的浸润性导管癌。虽然免疫组化染色可能在男性中显示差异,但预后与其他乳腺癌没有区别。它具有不同的激素谱,雄激素受体阳性使大汗腺癌患者适合靶向治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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