Vulvar myeloid sarcoma as the presenting symptom of acute myeloid leukemia: a case report and literature review of Chinese patients, 1999–2018

Abstract

BackgroundMyeloid 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.Case presentationA 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.ConclusionsFemale genital MS, especially vulvar MS, should be included in the differential diagnosis of gynecological neoplasm, which will facilitate its early diagnosis and prompt management.

Granulocytic sarcoma of cervix

“…The female reproductive system is less involved, and it was reported that the cervix was the most involved organ, estimated at 40.0% (22/54) in Chinese patients, followed by the ovaries (23.6%,13/55). [ 5 ] In our case, the patient was diagnosed with cervical GS with mild abnormalities in her bone marrow test, which was not sufficient to diagnose AML. However, the patient relapsed rapidly and progressed to GS with AML during the follow-up.…”

“…[ 6 , 11 – 13 ] Chemotherapy plus surgery (34.5%) was reported to be the most common option in Chinese patients with gynecological GS. [ 5 ] In our case, the patient underwent hysterectomy, bilateral adnexectomy, and AML-type chemotherapy. Regrettably, she relapsed 3 months after surgery and chemotherapy and developed to AML.…”

Incidence of Hematopoietic Sarcoma in Iranian Population

“…21 In another study, a case of a 47-year-old woman with MS of the vulva was reported who presented with symptoms of AML. 22 Also, Wang et al described a case of MS of the small bowel, kidney, and mesenteric lymph nodes in a 26-year-old male patient who suffered from intermittent upper abdominal pain. 23 In spite of a multitude of case-report studies, still little is known about the epidemiologic features of the disease.…”

The histopathologic examination of a second muscle biopsy specimen at a later date may sometimes be the best approach to make a differential diagnosis in neuromuscular disorders

“…Twenty-five cases have been reported as case reports . Two relevant series comprising 19 cases were published (34)(35). The patient ages in the case reports ranged from 3 months to 58 years.…”