Cutaneous and breast Langerhans cell sarcoma
Cutaneous and breast Langerhans cell sarcoma
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皮肤和乳房朗格汉斯细胞肉瘤
DOI：
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发表时间：
2016
影响因子：
9.2
通讯作者：
P. Valerón
中科院分区：
文献类型：
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作者：
E. Castro;J. Bastida;J. C. Rivero;R. Camacho;M. Limeres;S. Jiménez;P. Valerón
编辑朗格汉斯细胞肉瘤（LCS）是一种罕见的疾病。我们报告一例LCS皮肤和乳腺受累和积极的临床过程。据我们所知，没有病例LCS乳腺受累的报告。一位81岁的女性来我们这里咨询关于一个无症状的乳晕周围病变，在过去的2个月里一直存在于左乳房，没有体重减轻或全身症状的证据。个人的经历是显着的逐渐扩大的皮肤结节下背部，已被一个私人皮肤科医生摘除7个月前，并被告知为“皮肤淋巴瘤”的病理报告。体格检查发现左侧乳晕周围有一个肿胀的浸润性斑块，背部有一个疤痕，显示以前做过手术（图1a）。未触及腺病或肥大。左乳房病变的皮肤活检显示非典型多形性细胞弥漫性真皮浸润，伴有大量有丝分裂（图2a，B）。肿瘤细胞表达CD 1a、CD 4、S-100蛋白和Langerin，表明Langerhans细胞（LC）表型（图2c）。超微结构研究证实了这些发现，显示了浸润细胞中Birbeck颗粒特征性的细胞质结构（图2d）。根据组织学和超微结构所见，诊断LCS。此外，我们有机会审查了先前切除的背部皮肤病变的活检，这些病变具有相似的病理学发现。乳房X线摄影及超音波摄影显示弥漫性骨小梁增厚及腋窝腺病。身体CT扫描显示乳腺受累伴不对称和体积增大（图2 e）。左乳腺和腺病的细针穿刺活检显示与LCS浸润相符的特征。外周血涂片未发现循环恶性细胞的存在。未考虑骨髓穿刺检查。根据皮肤和乳腺LCS伴淋巴结转移的诊断，患者接受了化疗（长春碱、地塞米松和环磷酰胺）和辅助放疗，但疾病进展伴广泛的皮肤、乳腺和淋巴结受累，直至患者在诊断后9个月死亡（图1b）。朗格汉斯细胞肉瘤是一种肿瘤性疾病，其特征是具有明显恶性细胞学特征的LC增殖。LCS诊断的建议标准是：（i）增殖的典型Birbeck颗粒细胞，和（ii）恶性细胞学特征，如核分裂像和多核分裂像。朗格汉斯细胞肉瘤被认为是一种恶性侵袭性肿瘤，预后差。迄今已报告了大约50起案件。皮肤和淋巴结是最常见的受累部位，包括一些皮肤乳腺受累的病例，但没有真正的乳腺浸润。与此相反，我们的病例显示真正的乳腺内浸润证实细针穿刺活检。我们认为这是第一例在诊断时有乳房受累的LCS报告。在这种情况下，乳腺受累可能代表了转移事件从以前删除的皮肤病变。LCS（a）（B）
Editor Langerhans cell sarcoma (LCS) is a rare disease. We report a case of LCS with cutaneous and breast involvement and an aggressive clinical course. To the best of our knowledge, no cases of LCS with breast involvement have been reported. An 81-year-old female consulted us about an asymptomatic periareolar lesion that had been present in the left breast for the last 2 months, with no evidence of weight loss or systemic symptoms. Personal antecedents were remarkable for a gradually enlarged cutaneous nodule on lower back, that had been extirpated by a private dermatologist 7 months before and was informed as “cutaneous lymphoma” in the pathological report. Physical examination revealed an erythematous, infiltrated plaque in left periareolar area, and a scar revealing the surgery that had been previously practiced in the back (Fig. 1a). No adenopathies or megalies were palpated. A skin biopsy of the lesion on the left breast demonstrated a diffuse dermal infiltration of atypical pleomorphic cells with numerous mitoses (Fig. 2a,b). The tumour cells expressed CD1a, CD4, S-100 protein and Langerin, suggesting a Langerhans cells (LC) phenotype (Fig. 2c). The ultrastructural study confirmed these findings, showing the cytoplasmic structures that are characteristic of the Birbeck granules in the infiltrate cells (Fig. 2d). Based on the histological and ultrastructural findings a diagnosis of LCS was made. In addition, we had the opportunity to review the biopsy of the previously excised skin lesion on the back that shared similar pathological findings. Mammography and echography showed diffuse trabecular thickening and axilar adenopathies. A body CT scan disclosed mammary involvement with asymmetry and volumetric augmentation (Fig. 2e). Fine needle aspiration biopsy of left mammary gland and adenopathies showed features compatible with infiltration by LCS. Peripheral blood smear did not reveal the presence of circulant malignant cells. Bone marrow aspirate examination was not considered. According to a diagnosis of cutaneous and mammary LCS with nodal metastasis the patient was treated with chemotherapy (Vinblastine, Dexamethasone and Cyclophosphamide) and adjuvant radiotherapy, but the disease progressed with extensive cutaneous, breast and nodal involvement until the death of the patient 9 months after the diagnosis (Fig. 1b). Langerhans cell sarcoma is a neoplastic disease characterized by a proliferation of LC with markedly malignant cytological features. Suggested criteria for the diagnosis of LCS are: (i) proliferating typical Birbeck granule-containing cells, and (ii) malignant cytological features like atypia and multiple mitotic figures. Langerhans cell sarcoma is considered a malignant aggressive neoplasm with poor prognosis. About 50 cases have been reported to date. Skin and lymph nodes are the most frequent sites of involvement, including some cases of cutaneous involvement of skin breast, but with no true infiltration of the breast. In contrast, our case showed true intramammary infiltration confirmed by fine needle aspiration biopsy. We think that this is the first case of LCS reported with breast involvement at the time of diagnosis. The breast involvement in this case could represent a metastatic event from the previously removed cutaneous lesion. LCS (a) (b)
CD56/NCAM 阳性朗格汉斯细胞肉瘤：4 例的病理病理学研究。
DOI：
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发表时间：
2005
期刊：
Int J Hematol 81
影响因子：
--
作者：
Saeki K;Yasugi E;Okuma E;Breit SN;Nakamura M;Toda T;Kaburagi Y;Yuo A;Takakazu Kawase
通讯作者：
Takakazu Kawase