-
摘要: 血管周上皮样细胞肿瘤(PEComa)是由上皮样细胞或梭形细胞形成的间充质肿瘤，它们对平滑肌和黑素细胞标志物均具有免疫反应性。PEComa包括血管平滑肌脂肪瘤、淋巴管肌瘤病、肺透明细胞“糖”瘤，以及一组罕见的、形态学和免疫表型相似的病变，起源于各种内脏和软组织部位，其特征是肌肉和黑色素生成标记物的共表达。PEComa通常最多出现在内脏(尤其是胃肠道和子宫)、腹膜后和腹盆腔部位，一个子集出现在躯体软组织和皮肤中。几乎所有PEComa都显示出对两者的免疫反应性黑色素细胞(HMB-45或Melan-A)且光滑肌肉(肌动蛋白或结蛋白)标记物。在泌尿生殖道中，PEComa可见于肾脏、膀胱、前列腺、睾丸和尿道。PEComa肿瘤多呈良性，以肾脏血管平滑肌脂肪瘤最为常见，肾脏恶性PEComa少见，现将苏州大学附属第一医院收治的1例报道如下。
-
关键词:
- 恶性血管周上皮样细胞肿瘤 /
- 肾肿瘤
Abstract: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor composed of epithelioid cells or spindle cells. PEComa is immunoreactive to both smooth muscle and melanocyte markers. PEComa includes angiomyolipomas, lymphangiomyomatosis, clear cell "sugar" tumors of the lung, and a group of rare, morphologically and immunophenotypically similar lesions that arise from a variety of visceral and soft tissue sites and are characterized by co-expression of muscle and melanogenic markers. PEComa are most commonly found in the viscera (especially the gastrointestinal tract and uterus), retroperitoneum, and abdominal and pelvic areas, with a subset occurring in the soft tissues and skin of the body. Almost all PEComa showed immunoreactive melanocytes (HMB-45 or Melan-A) and smooth muscle (actin or desmin) markers against both. In the urogenital tract, PEComa can be seen in the kidney, bladder, prostate, testis and urethra. PEComa tumors are mostly benign, and renal angiomyolipoma is the most common. Renal malignant PEComa is rare, so this case admitted to First Affiliated Hospital of Soochow University is reported as follows.-
Key words:
- malignant perivascular epithelioid cell tumor /
- renal tumor
-
-
[1] Valencia-Guerrero A, Pinto A, Anderson WJ, et al. PNL2: A useful adjunct biomarker to HMB45 in the diagnosis of uterine perivascular epithelioid cell tumor(PEComa)[J]. Int J Gynecol Pathol, 2020, 39(6): 529-536. doi: 10.1097/PGP.0000000000000653
[2] Gulavita P, Fletcher C, Hirsch MS. PNL2: an adjunctive biomarker for renal angiomyolipomas and perivascular epithelioid cell tumours[J]. Histopathology, 2018, 72(3): 441-448. doi: 10.1111/his.13369
[3] Chiang S, Vasudevaraja V, Serrano J, et al. TSC2-mutant uterine sarcomas with JAZF1-SUZ12 fusions demonstrate hybrid features of endometrial stromal sarcoma and PEComa and are responsive to mTOR inhibition[J]. Mod Pathol, 2022, 35(1): 117-127. doi: 10.1038/s41379-021-00922-7
[4] Igaz P, Toth G, Nagy P, et al. Surprising genetic and pathological findings in a patient with giant bilateral periadrenal tumours: PEComas and mutations of PTCH1 in Gorlin-Goltz syndrome[J]. J Med Genet, 2022, 59(9): 916-919. doi: 10.1136/jmedgenet-2021-108082
[5] Yang Y, Yu X, Lu B, et al. Perivascular epithelioid cell tumor(PEComa)of the kidney: an overview of its management and outcomes[J]. J Int Med Res, 2020, 48(10): 300060520961223.
[6] Wagner AJ, Ravi V, Riedel RF, et al. Nab-Sirolimus for patients with malignant perivascular epithelioid cell tumors[J]. J Clin Oncol, 2021, 39(33): 3660-3670. doi: 10.1200/JCO.21.01728
[7] Çalişkan S, Gümrükçü G, Özsoy E, et al. Renal angiomyolipoma[J]. Rev Assoc Med Bras(1992), 2019, 65(7): 977-981. doi: 10.1590/1806-9282.65.7.977
[8] Koenig KL, Sahasrabudhe KD, Sigmund AM, et al. AML with myelodysplasia-related changes: development, challenges, and treatment advances[J]. Genes(Basel), 2020, 11(8): 111.
[9] Caliò A, Brunelli M, Marletta S, et al. Epithelioid angiomyolipoma: a pathological entity discovered in Verona with the endorsement of Doctor Rosai[J]. Pathologica, 2021, 113(5): 307-315. doi: 10.32074/1591-951X-335
[10] Liapi A, Mathevet P, Herrera FG, et al. VEGFR inhibitors for uterine metastatic perivascular epithelioid tumors(PEComa)resistant to mtor inhibitors. a case report and review of literature[J]. Front Oncol, 2021, 11: 641376. doi: 10.3389/fonc.2021.641376
[11] 王东艳, 张强, 杨志刚. 肾血管周上皮样细胞瘤1例[J]. 临床泌尿外科杂志, 2020, 35(6): 497-498. https://www.cnki.com.cn/Article/CJFDTOTAL-LCMW202006019.htm
[12] 柳赫, 朱永锋, 张春霆. 肾上腺恶性血管周上皮样细胞肿瘤1例[J]. 临床泌尿外科杂志, 2020, 35(12): 1021-1022. https://www.cnki.com.cn/Article/CJFDTOTAL-LCMW202012020.htm
-
下载:
图(3)
计量
- 文章访问数: 940
- PDF下载数: 503
- 施引文献: 0