Ronen Shira, Aguilera-Barrantes Irene, Giorgadze Tamara, Šteiner Petr, Grossmann Petr, Suster Saul
Department of Pathology, Medical College of Wisconsin, Milwaukee, WI.
Bioptic Laboratory Ltd, Molecular Pathology Laboratory, Plzen, Czech Republic.
Am J Dermatopathol. 2018 Aug;40(8):580-587. doi: 10.1097/DAD.0000000000001128.
Polymorphous sweat gland carcinoma is an uncommon low-grade malignant adnexal tumor with a marked predilection for the distal extremities. Histologically, the lesions are characterized by a cellular proliferation showing a combination of growth patterns, including trabecular, solid, tubular, cribriform, or adenoid cystic and pseudopapillary. The immunohistochemical and molecular profile of these tumors has not yet been properly addressed. We have studied 3 cases of polymorphous sweat gland carcinoma using a broad panel of immunohistochemical markers including cytokeratin AE1/AE3, CK5/6, MOC31, p40, p63, p16, chromogranin, synaptophysin, CD56, MIB-1, estrogen receptor, progesterone receptor, androgen receptor, BER-EP4, smooth muscle actin, epithelial membrane antigen, carcinoembryonic antigen, CD117, S100 protein, HBME-1, DOG1, vimentin, and mammaglobin. We also examined for the MYB-NFIB fusion by fluorescent in situ hybridization (ISH) and for human papilloma virus by ISH. Our studies show that cytokeratin AE1/AE3, CK5/6, p40, p63, p16, chromogranin, and CD56 stains were positive in all 3 cases. All 3 cases were negative for MYB-NFIB fusion by fluorescent ISH which rules out adenoid cystic carcinoma. DNA ISH studies for high-risk human papilloma virus were negative in all cases. MIB-1 proliferation index was very high (30%-70% nuclear positivity), supporting a malignant phenotype. The positivity for chromogranin and CD56 suggests partial neuroendocrine differentiation. The differential diagnosis includes metastases from internal malignancies, basal cell carcinoma, and other benign and malignant adnexal neoplasms such as adenoid cystic carcinoma, ductal eccrine carcinoma, and microcystic carcinoma. Positivity for p16 in combination with chromogranin and CD56 may be potentially good markers for differentiating this tumor from other adnexal tumors.
多形性汗腺癌是一种罕见的低级别恶性附属器肿瘤，明显好发于四肢远端。组织学上，病变的特征是细胞增殖，呈现多种生长模式的组合，包括小梁状、实性、管状、筛状、腺样囊性或假乳头状。这些肿瘤的免疫组化和分子特征尚未得到妥善研究。我们使用了广泛的免疫组化标志物研究了3例多形性汗腺癌，这些标志物包括细胞角蛋白AE1/AE3、CK5/6、MOC31、p40、p63、p16、嗜铬粒蛋白、突触素、CD56、MIB-1、雌激素受体、孕激素受体、雄激素受体、BER-EP4、平滑肌肌动蛋白、上皮膜抗原、癌胚抗原、CD117、S100蛋白、HBME-1、DOG1、波形蛋白和乳珠蛋白。我们还通过荧光原位杂交（ISH）检测了MYB-NFIB融合情况，并通过ISH检测了人乳头瘤病毒。我们的研究表明，细胞角蛋白AE1/AE3、CK5/6、p40、p63、p16、嗜铬粒蛋白和CD56染色在所有3例中均为阳性。通过荧光ISH检测，所有3例的MYB-NFIB融合均为阴性，排除了腺样囊性癌。高危人乳头瘤病毒的DNA ISH研究在所有病例中均为阴性。MIB-1增殖指数非常高（核阳性率为30%-70%），支持恶性表型。嗜铬粒蛋白和CD56阳性提示部分神经内分泌分化。鉴别诊断包括来自内部恶性肿瘤的转移、基底细胞癌以及其他良性和恶性附属器肿瘤，如腺样囊性癌、导管小汗腺癌和微囊性癌。p16与嗜铬粒蛋白和CD56联合阳性可能是将该肿瘤与其他附属器肿瘤区分开来的潜在良好标志物。
