Gynecological Myeloid Sarcoma: Literature Review and a Case Report

Aleksina Alekseevna Shatilova, L.L. Girshova, D.V. Zaitsev, I.G. Budaeva, Yu.V. Mirolyubova, D.V. Ryzhkova, R.V. Grozov, K.V. Bogdanov, T.S. Nikulina, D.V. Motorin, D.B. Zammoeva, S.V. Efremova, V.V. Ivanov, A.V. Petukhov, Yu.A. Alekseeva, A.Yu. Zaritskey,

DOI:

https://doi.org/10.21320/2500-2139-2021-14-1-31-44

Myeloid sarcoma, also known as chloroma or granulocytic sarcoma, is a rare disease characterized by the proliferation of immature myeloid cells in extramedullary lesions. Chloroma is more commonly observed in patients with acute myeloid leukemias, other myeloproliferative neoplasms, or myelodysplastic syndrome. However, it can also manifest itself as solitary tumor. Sarcoma can develop in different organs and tissues, but most frequently it appears in lymph nodes, soft tissues, and bones. Myeloid sarcoma with primary gynecological lesion is very rarely mentioned. In literature cases of cervical lesions are described. The present article summarizes the literature data concerning different aspects of myeloid sarcoma diagnosis and treatment. The issue under discussion is the role of chemotherapy, radiotherapy, surgery, and bone marrow transplantation in the treatment of this malignant tumor. It appears that whatever the primary tumor localization, the best treatment options are chemotherapy and allogeneic bone marrow transplantation (allo-BMT). A promising trend is the use of novel targeted drugs improving outcomes of treatment. The article provides a case report of a female patient with cervical myeloid sarcoma and concomitant bone marrow involvement, as well as the description of clinical course, diagnosis, and treatment. The patient received chemotherapy with subsequent allo-BMT. The pre-transplant therapy enabled allo-BMT with the deepest response possible. The patient achieved PET- and MRD-negative complete remission of cervical myeloid sarcoma and bone marrow.

  • Aleksina Alekseevna Shatilova VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • L.L. Girshova VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • D.V. Zaitsev VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • I.G. Budaeva VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • Yu.V. Mirolyubova VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • D.V. Ryzhkova VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • R.V. Grozov VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • K.V. Bogdanov VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • T.S. Nikulina VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • D.V. Motorin VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • D.B. Zammoeva VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • S.V. Efremova VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • V.V. Ivanov VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • A.V. Petukhov VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341; Institute of Cytology, 4 Tikhoretskii pr-t, Saint Petersburg, Russian Federation, 194064 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341; ФГБУН «Институт цитологии РАН», Тихорецкий пр-т, д. 4, Санкт-Петербург, Российская Федерация, 194064
  • Yu.A. Alekseeva VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  • A.Yu. Zaritskey VA Almazov National Medical Research Center, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341 ; ФГБУ «НМИЦ им. В.А. Алмазова» Минздрава России, ул. Аккуратова, д. 2, Санкт-Петербург, Российская Федерация, 197341
  1. Злокачественные новообразования в России в 2018 году (заболеваемость и смертность). Под ред. А.Д. Каприна, В.В. Старинского, Г.В. Петровой. М.: МНИОИ им. П.А. Герцена, 2019. С. 10, 34, 125.
  2. [Kaprin AD, Starinskii VV, Petrova GV, eds. Zlokachestvennye novoobrazovaniya v Rossii v 2018 godu (zabolevaemost i smertnost). (Malignant neoplasms in Russia in 2018: incidence and mortality.) Moscow: MNIOI im. P.A. Gertsena Publ.; 2019. pp. 10, 34, 125. (In Russ)]
  3. Michelle SР, Karen JK, Ursula AM. Gynecologic Tumors and Malignancies. In: Atlas of Diagnostic Oncology. 4th edition. Philadelphia: Mosby Elsevier; рр. 278–324.
  4. Онкогинекология: национальное руководство. Под ред. А.А. Каприна. М.: ГЭОТАР-Медиа, 2019. С. 27–31, 114–115, 161.
  5. [Kaprin AD, ed. Onkoginekologiya: natsionalnoe rukovodstvo. (Oncogynecology: national guide.) Moscow: GEOTAR-Media Publ.; 2019. pp. 27–31, 114–115, 161. (In Russ)]
  6. Kurman RJ, Carcangiu ML, Herrington CS, et al. WHO Classification of Tumours of Female Reproductive Organs. Lyon: IARC Press; 2014.
  7. Almond L, Charalampakis M, Ford S, et al. Myeloid Sarcoma: Presentation, Diagnosis, and Treatment. Clin Lymphoma Myel Leuk. 2017;17(5):263–7. doi: 10.1016/j.clml.2017.02.027. DOI: https://doi.org/10.1016/j.clml.2017.02.027
  8. Swerdlow SH. WHO Classification Of Tumours Of Haematopoietic And Lymphoid Tissues. Lyon: IARC Press; 2017. рр. 167–8.
  9. Kawamoto K, Miyoshi H, Yoshida N, et al. Clinicopathological, Cytogenetic, and Prognostic Analysis of 131 Myeloid Sarcoma Patients. Am J Surg Pathol. 2016;40(11):1473–83. doi: 10.1097/PAS.0000000000000727. DOI: https://doi.org/10.1097/PAS.0000000000000727
  10. Campidelli C, Agostinelli C, Stitson R, et al. Myeloid sarcoma: extramedullary manifestation of myeloid disorders. Am J Clin Pathol. 2009;132(3):426–37. doi: 10.1309/AJCP1ZA7HYZKAZHS. DOI: https://doi.org/10.1309/AJCP1ZA7HYZKAZHS
  11. Byrd JC, Edenfield WJ, Shields DJ, et al. Extramedullary myeloid cell tumors in acute nonlymphocytic leukemia: a clinical review. J Clin Oncol. 1995;13(7):1800–16. doi: 10.1200/JCO.1995.13.7.1800. DOI: https://doi.org/10.1200/JCO.1995.13.7.1800
  12. Goyal G, Bartley AC, Patnaik MM, et al. Clinical features and outcomes of extramedullary myeloid sarcoma in the United States: analysis using a national data set. Blood Cancer J. 2017;7(8):e592. doi: 10.1038/bcj.2017.79. DOI: https://doi.org/10.1038/bcj.2017.79
  13. Bakst RL, Tallman MS, Douer D, et al. How I treat extramedullary acute myeloid leukemia. Blood. 2011;118(14):3785–93. doi: 10.1182/blood-2011-04-347229. DOI: https://doi.org/10.1182/blood-2011-04-347229
  14. Shahin O, Ravandi F. Myeloid sarcoma. Curr Opin Hematol. 2020;27(2):88–94. doi: 10.1097/moh.0000000000000571. DOI: https://doi.org/10.1097/MOH.0000000000000571
  15. Avni B, Koren-Michowitz M. Myeloid sarcoma: current approach and therapeutic options. Ther Adv Hematol. 2011;2(5):309–16. doi: 10.1177/2040620711410774. DOI: https://doi.org/10.1177/2040620711410774
  16. Claerhout H, Van Aelst S, Melis C, et al. Clinicopathological characteristics of de novo and secondary myeloid sarcoma: A monocentric retrospective study. Eur J Haematol. 2018;100(6):603–12. doi: 10.1111/ejh.13056. DOI: https://doi.org/10.1111/ejh.13056
  17. Pathak B, Bruchim I, Brisson ML, et al. Granulocytic sarcoma presenting as tumors of the cervix. Gynecol Oncol. 2005;98(3):493–7. doi: 10.1016/j.ygyno.2005.04.028. DOI: https://doi.org/10.1016/j.ygyno.2005.04.028
  18. Gui W, Li J, Zhang Z, et al. Primary hematological malignancy of the uterine cervix: A case report. Oncol Lett. 2019;18(3):3337–41. doi: 10.3892/ol.2019.10652. DOI: https://doi.org/10.3892/ol.2019.10652
  19. Pileri SA, Ascani S, Cox MC, et al. Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia. 2007;21(2):340–50. doi: 10.1038/sj.leu.2404491. DOI: https://doi.org/10.1038/sj.leu.2404491
  20. Sharma V, Dora T, Patel M, et al. Case Report of Diffuse Large B Cell Lymphoma of Uterine Cervix Treated at a Semiurban Cancer Centre in North India. Case Rep Hematol. 2016;2016:1–4. doi: 10.1155/2016/3042531. DOI: https://doi.org/10.1155/2016/3042531
  21. Lee J, Kim Y, Min Y, et al. Granulocytic sarcoma of the uterine cervix. Int J Gynecol Cancer. 2004;14(3):553–7. doi: 10.1111/j.1048-891x.2004.014321.x. DOI: https://doi.org/10.1111/j.1048-891x.2004.014321.x
  22. Yu Y, Qin X, Yan S, et al. Non-leukemic myeloid sarcoma involving the vulva, vagina, and cervix: a case report and literature review. Onco Targets Ther. 2015;8:3707–13. doi: 10.2147/OTT.S92815. DOI: https://doi.org/10.2147/OTT.S92815
  23. Kaur V, Swami A, Alapat D, et al. Clinical characteristics, molecular profile and outcomes of myeloid sarcoma: a single institution experience over 13 years. Hematology. 2018;23(1):17–24. doi: 10.1080/10245332.2017.1333275. DOI: https://doi.org/10.1080/10245332.2017.1333275
  24. Kashofer K, Gornicec M, Lind K, et al. Detection of prognostically relevant mutations and translocations in myeloid sarcoma by next generation sequencing. Leuk Lymphoma. 2018;59(2):501–4. doi: 10.1080/10428194.2017.1339879. DOI: https://doi.org/10.1080/10428194.2017.1339879
  25. Dohner H, Estey EH, Amadori S, et al. Diagnosis and management of acute myeloid leukemia in adults: recommendations from an international expert panel, on behalf of the European LeukemiaNet. Blood. 2010;115(3):453–74. doi: 10.1182/blood-2009-07-235358. DOI: https://doi.org/10.1182/blood-2009-07-235358
  26. Савченко В.Г., Паровичникова Е.Н., Афанасьев Б.В. и др. Клинические рекомендации российских экспертов по лечению больных острыми миелоидными лейкозами в возрасте моложе 60 лет. Терапевтический архив. 2014;86(7):4–13.
  27. [Savchenko VG, Parovichnikova EN, Afanasyev BV, et al. Russian experts’ clinical guidelines for acute myeloid leukemia treatment in patients less than 60 years of age. Terapevticheskii arkhiv. 2014;86(7):4–13. (In Russ)]
  28. Гиршова Л.Л., БудаеваИ.Г., Овсянникова Е.Г. и др. Прогностическое значение и корреляция динамики гиперэкспрессии гена WT1 и мутации гена NPM1 у пациентов с острым миелобластным лейкозом. Клиническая онкогематология. 2017;10(4):485–93. doi: 10.21320/2500-2139-2017-10-4-485-493.
  29. [Girshova LL, Budaeva IG, Ovsyannikova EG, et al. Prognostic Value and Correlation Between WT1 Overexpression and NPM1 Mutation in Patients with Acute Myeloblastic Leukemia. Clinical oncohematology. 2017;10(4):485–93. doi: 10.21320/2500-2139-2017-10-4-485-493. (In Russ)] DOI: https://doi.org/10.21320/2500-2139-2017-10-4-485-493
  30. Adams HJ, Kwee TC. Prognostic value of pretransplant FDG-PET in refractory/relapsed Hodgkin lymphoma treated with autologous stem cell transplantation: systematic review and meta-analysis. Ann Hematol. 2016;95(5):695–706. doi: 10.1007/s00277-016-2619-9. DOI: https://doi.org/10.1007/s00277-016-2619-9
  31. Aschoff P, Hantschel M, Oksuz M, et al. Integrated FDG-PET/CT for detection, therapy monitoring and follow-up of granulocytic sarcoma. Initial results. Nuklearmedizin. 2009;48(5):185–91. doi: 10.3413/nukmed-0236. DOI: https://doi.org/10.3413/nukmed-0236
  32. NCCN Clinical Practice Guidelines in Oncology. Acute Myeloid Leukemia. Version 3.2020. Available from: https://www.nccn.org/professionals/physician_gls/pdf/aml_blocks.pdf. (accessed 12.11.2020).
  33. Kahn RM, Gordhandas S, Chapman-Davis E, et al. Acute Myeloid Leukemia Presenting as Myeloid Sarcoma with a Predisposition to the Gynecologic Tract. Case Rep Oncol Med. 2019;2019:1–5. doi: 10.1155/2019/4189275. DOI: https://doi.org/10.1155/2019/4189275
  34. Modi G, Madabhavi I, Panchal H, et al. Primary vaginal myeloid sarcoma: a rare case report and review of the literature. Case Rep Obstet Gynecol. 2015;2015:1–4. doi: 10.1155/2015/957490. DOI: https://doi.org/10.1155/2015/957490
  35. Hernandez J-A, Navarro J-T, Rozman M, et al. Primary myeloid sarcoma of the gynecologic tract: a report of two cases progressing to acute myeloid leukemia. Leuk Lymphoma. 2002;43(11):2151–3. doi: 10.1080/1042819021000016096. DOI: https://doi.org/10.1080/1042819021000016096
  36. Ucar M, Guryildirim M. Granulocytic Sarcoma of the Uterus: A Rare Presentation of Extramedullary Relapse of AML and Importance of MRI. Case Rep Radiol. 2014;2014:1–4. doi: 10.1155/2014/501342. DOI: https://doi.org/10.1155/2014/501342
  37. Garcia MG, Deavers MT, Knoblock RJ, et al. Myeloid sarcoma involving the gynecologic tract: a report of 11 cases and review of the literature. Am J Clin Pathol. 2006;125(5):783–90. doi: 10.1309/H9MM-21FP-T7YB-L3PW. DOI: https://doi.org/10.1309/H9MM21FPT7YBL3PW
  38. Kim SCН, Natarajan-Ame S, Lioure B, et al. Successful treatment of a granulocytic sarcoma of the uterine cervix in complete remission at six-year follow-up. J Oncol. 2010;2010:1–3. doi: 10.1155/2010/812424. DOI: https://doi.org/10.1155/2010/812424
  39. Gill H, Loong F, Mak V, et al. Myeloid sarcoma of the uterine cervix presenting as missed abortion. Arch Gynecol Obstet. 2012;286(5):1339–41. doi: 10.1007/s00404-012-2454-8. DOI: https://doi.org/10.1007/s00404-012-2454-8
  40. Weingertner AS, Wilt M, Atallah I, et al. Myeloid Sarcoma of the Uterine Cervix as Presentation of Acute Myeloid Leukaemia after Treatment with Low-Dose Radioiodine for Thyroid Cancer: A Case Report and Review of the Literature. Case Rep Oncol. 2009;2(1):1–6. doi: 10.1159/000191215. DOI: https://doi.org/10.1159/000191215
  41. Bao H, Gao J, Chen YH, et al. Rare myeloid sarcoma with KMT2A (MLL)-ELL fusion presenting as a vaginal wall mass. Diagn Pathol. 2019;14(1):26. doi: 10.1186/s13000-019-0804-6. DOI: https://doi.org/10.1186/s13000-019-0804-6
  42. Otoukesh S, Zhang J, Nakamura R, et al. The efficacy of venetoclax and hypomethylating agents in acute myeloid leukemia with extramedullary involvement. Leuk Lymphoma. 2020;61(8):2020–3. doi: 10.1080/10428194.2020.1742908. DOI: https://doi.org/10.1080/10428194.2020.1742908
  43. Kanate AS, Vos J, Chargualaf MJ. Venetoclax for Refractory Myeloid Sarcoma. J Oncol Pract. 2019;15(7):413–5. doi: 10.1200/JOP.18.00753. DOI: https://doi.org/10.1200/JOP.18.00753
  44. Girshova L, Romanova E, Kholopova I, et al. Isolated Myeloid Sarcoma Involving the Breast. Blood. 2012;120(21):4345. doi: 10.1182/blood.v120.21.4345.4345. DOI: https://doi.org/10.1182/blood.V120.21.4345.4345
  45. Chevallier P, Labopin M, Cornelissen J, et al. Allogeneic hematopoietic stem cell transplantation for isolated and leukemic myeloid sarcoma in adults: a report from the Acute Leukemia Working Party of the European group for Blood and Marrow Transplantation. Haematologica. 2011;96(9):1391–4. doi: 10.3324/haematol.2011.041418. DOI: https://doi.org/10.3324/haematol.2011.041418
  46. Lachowiez C, DiNardo CD, Konopleva M. Venetoclax in acute myeloid leukemia – current and future directions. Leuk Lymphoma. 2020;61(6):1313–22. doi: 10.1080/10428194.2020.1719098. DOI: https://doi.org/10.1080/10428194.2020.1719098
  47. Neiman RS, Barcos M, Berard C, et al. Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer. 1981;48(6):1426–37. doi: 10.1002/1097-0142(19810915)48:6<1426::aid-cncr2820480626>3.0.co;2-g. DOI: https://doi.org/10.1002/1097-0142(19810915)48:6<1426::AID-CNCR2820480626>3.0.CO;2-G
  48. Meyer HJ, Ponisch W, Schmidt SA, et al. Clinical and imaging features of myeloid sarcoma: a German multicenter study. BMC Cancer. 2019;19(1):1150. doi: 10.1186/s12885-019-6357-y. DOI: https://doi.org/10.1186/s12885-019-6357-y
  49. Wang HQ, Li J. Clinicopathological features of myeloid sarcoma: Report of 39 cases and literature review. Pathol Res Pract. 2016;212(9):817–24. doi: 10.1016/j.prp.2016.06.014. DOI: https://doi.org/10.1016/j.prp.2016.06.014

Keywords:

cervical myeloid sarcoma, gynecologic tract, acute myeloid leukemias

Downloads

Download data is not yet available.

Published

01.01.2021

Issue

MYELOID TUMORS

How to Cite

Shatilova A.A., Girshova L.L., Zaitsev D.V., et al. Gynecological Myeloid Sarcoma: Literature Review and a Case Report. Clinical Oncohematology. Basic Research and Clinical Practice. 2021;14(1):31–44. doi:10.21320/2500-2139-2021-14-1-31-44.

Most read articles by the same author(s)

1 2 3 4 > >>