Myeloid sarcoma of the vulva as the initial presentation of acute myeloid leukaemia

Hu, Stephen Chu‐Sung; Chen, W.‐T.; Chen, G.‐S.

doi:10.1111/bjd.14063

Cited by 6 publications

(5 citation statements)

References 11 publications

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“…In our reviewed cases, three occurred t (8;21)(q22;q22), in conformity with the high incidence of t (8;21) in AML-M2 patients with MS [68]. And, one AML-M5 patient had complex chromosomal aberrations of t (1;7)(p22;q36), t (3;21)(q22;q26) and loss of chromosome 16 [20]. Recurrent AML1/ETO fusion genes were identified in two gynecological MS patients, whereas no cytogenetic defect was discovered in five patients.…”

Section: Discussionsupporting

confidence: 65%

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

et al. 2019

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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.

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Section: Discussionsupporting

confidence: 65%

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

et al. 2019

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“…Case reported by Ersahin et al [3] also had synchronous Paget's disease and infiltrating ductal adenocarcinoma of right breast. Case reported by Hu et al [7] had extensive myeloid sarcoma involving bilateral peri-renal spaces, ureters and adnexae.…”

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confidence: 96%

“…After therapy, disease of four patients (case no. 1, 3, 4 and 5) was in remission on follow up at the time of reporting individual cases by the respective authors [2,4,6,7]. Two patients-case no.…”

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confidence: 97%

“…Two patients-case no. 1 and 6 died during follow up due to sepsis and disease relapse respectively [3,7].…”

mentioning

confidence: 99%

“…We have reported in recent past few rare sites of MS like mediastinum, pleura, spinal cord and orbit [8][9][10][11]. Much rarer is [7,8,[10][11][12]. Female genital tract is also one of the rare site of involvement in acute leukaemia.…”

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confidence: 99%

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