Department of Dermatology, Changhai Hospital, Second Military Medical University, Shanghai, 200433, China.
Department of Dermatology, Shanghai Skin Disease Hospital, Shanghai, 200433, China.
Diagn Pathol. 2019 Nov 7;14(1):126. doi: 10.1186/s13000-019-0892-3.
Myeloid sarcoma (MS), which represents a rare malignancy that comprises of myeloid blasts occurring at extra-medullary sites, closely correlates with the onset and relapse of acute myeloid leukemia (AML) and other hemopoietic neoplasm. Female genital system is an uncommon location of MS, with the vulvar MS being even rarer that only eight cases have been reported in English-written literature.
A 47-year-old woman presented with chronic ulceration on her vulva for one and a half month. Microscopic examination of incisional biopsy revealed dermal infiltration of myeloid precursor cells, which were positive for MPO, lysozyme, CD43, CD68, CD38 and CD117. Bone marrow flowcytometric analysis showed myeloblast count of 74%, which expressed CD13, CD33, CD117 and HLA-DR. A diagnosis of AML (M2 type) was made and vulvar MS was the earliest symptom. The patient achieved complete remission after chemotherapy with no evidence of recurrence in a 27-month follow-up. We reviewed the literature and identified 54 cases of Chinese patients with gynecological MS between 1999 and 2018, and discovered that in Chinese population, MS most frequently involved uterine cervix followed by the ovary and vulva, and ovarian MS onset much earlier than other sites. Remarkably, vulvar MS exhibited a high rate of concurrent AML and secondary myeloid leukemia within a short time of its occurrence. Despite its limited distribution, MS should be tackled aggressively with chemotherapy followed by allogeneic hematopoietic stem cell transplantation if the appropriate donor is available.
Female genital MS, especially vulvar MS, should be included in the differential diagnosis of gynecological neoplasm, which will facilitate its early diagnosis and prompt management.
骨髓肉瘤（MS）代表一种罕见的恶性肿瘤，由骨髓中原始细胞在骨髓外部位发生，与急性髓系白血病（AML）和其他造血肿瘤的发生和复发密切相关。女性生殖系统是 MS 的罕见部位，外阴 MS 更为罕见，仅在英文文献中报道了 8 例。
一名 47 岁女性因外阴慢性溃疡就诊，病史 1.5 个月。切取活检的显微镜检查显示真皮中有髓样前体细胞浸润，MPO、溶菌酶、CD43、CD68、CD38 和 CD117 阳性。骨髓流式细胞术分析显示原始细胞占 74%，表达 CD13、CD33、CD117 和 HLA-DR。诊断为 AML（M2 型），外阴 MS 是最早的症状。患者在 27 个月的随访中无复发，化疗后完全缓解。我们查阅文献，发现 1999 年至 2018 年间中国患者妇科 MS 共 54 例，发现中国人群中 MS 最常累及宫颈，其次是卵巢和外阴，且卵巢 MS 发病更早。值得注意的是，外阴 MS 在发生后短时间内并发 AML 和继发性骨髓性白血病的比例较高。尽管分布有限，但如果有合适的供体，应积极采用化疗联合异基因造血干细胞移植治疗 MS。
女性生殖 MS，特别是外阴 MS，应纳入妇科肿瘤的鉴别诊断，以便早期诊断和及时处理。
