Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report and Review

Myeloid sarcoma: an uncommon presentation of myeloid neoplasms; a case series of 4 rare cases reported in a tertiary care institute

Toyaja Jadhav; Puneet Baveja; Arijit Sen

Downloads: 0
Views: 111


Myeloid sarcoma (MS) is a rare extramedullary neoplasm of myeloid cells, which can arise before, concurrently with, or following hematolymphoid malignancies. We report 04 such cases of MS, diagnosed in this institute over a period of 6 years, during various phases of their respective myeloid neoplasms/leukemias. These cases include MS occurring as a relapse of AML (Case 1), MS occurring as an initial presentation of CML (Case 2), MS occurring during ongoing chemotherapy in APML (Case 3), and MS presenting as a progression of MDS to AML (Case 4). In the absence of relevant clinical history and unemployment of appropriate immunohistochemical (IHC) studies, these cases have a high risk of being frequently misdiagnosed either as Non-Hodgkin’s Lymphoma (NHL) or small round cell tumors or undifferentiated carcinomas, which may further delay their management, making an already bad prognosis worse. This case series has been designed to throw light on the varied presentation of MS and the lineage differentiation of its neoplastic cells through the application of relevant IHC markers along with their clinical correlation.


Sarcoma, Myeloid, Leukemia, Myeloid, Acute, Leukemia, Promyelocytic, Acute, Leukemia, Myelogenous, Chronic, BCR-ABL Positive, Myelodysplastic Syndromes


1 Baveja P, Jadhav T, Pendkur G. Granulocytic sarcoma presenting as a palpable breast lump in a 13-year-old female as a relapse of AML. Indian J Pathol Oncol. 2020;7(4):669-73.

2 Lee JW, Kim YT, Min YH, et al. Granulocytic sarcoma of the uterine cervix. Int J Gynecol Cancer. 2004;14(3):553-7. PMid:15228434.

3 Di Veroli A, Micarelli A, Cefalo M, et al. Recurrence of a t(8;21)-positive acute myeloid leukemia in the form of a granulocytic sarcoma involving cranial bones: a diagnostic and therapeutic challenge. Case Rep Hematol. 2013;2013:245395. PMid:24109526.

4 Rappaport H. Tumors of the Hematopoietic System. In: Armed Forces Institute of Pathology, editor. Atlas of tumor pathology. Washington: AFIP; 1966.

5 Guermazi A, Feger C, Rousselot P, et al. Granulocytic sarcoma (chloroma): imaging findings in adults and children. AJR Am J Roentgenol. 2002;17(2):319-25. PMid:11804886.

6 Kahali B. Myeloid sarcoma: the other side of acute leukemia. In: Guenova M, Balatzenko G, editors. Hematology: latest research and clinical advances. London: IntechOpen; 2018.

7 Avni B, Koren Michowitz M. Myeloid sarcoma: current approach and therapeutic options. Ther Adv Hematol. 2011;2(5):309-16. PMid:23556098.

8 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. PMid:17170724.

9 Tsimberidou AM, Kantarjian HM, Estey E, et al. Outcome in patients with nonleukemic granulocytic sarcoma treated with chemotherapy with or without radiotherapy. Leukemia. 2003;17(6):1100-3. PMid:12764375.

10 Chan JKC, Jaffe ESKYH. WHO classification of tumors of hematopoietic and lymphoid tissues. 4th ed. rev. Lyon: IARC; 2017. p. 353-4.

11 Roth M, Medeiros L, Elenitoba-Johnson K, Kuchnio M, Jaffe E, Stetler-Stevenson M. Extramedullary myeloid cell tumors: an immunohistochemical study of 29 cases using routinely fixed and processed paraffin-embedded tissue sections. Arch Pathol Lab Med. 1995;119(9):790-8. PMid:7668936.

12 Neiman R, Barcos M, Berard C, et al. Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer. 1981;48(6):1426-37.<1426::AID-CNCR2820480626>3.0.CO;2-G. PMid:7023656.

13 Dubrovina E, Kryzhanovsky O, Maschan A, Samochatova E, Baydun L, Bartseva O. Acute myeloblastic leukemia m2 with the 8;21 translocation associated with granulocytic sarcoma. In: Büchner T, Schellong G, Ritter J, Creutzig U, Hiddemann W, Wörmann B, editors. Acute Leukemias VI. Berlin: Springer.

14 Çakan M, Koç A, Cerit K, Bozkurt S, Ergelen R, Vural I. A case of acute myeloid leukemia (FAB M2) with inversion 16 who presented with pelvic myeloid sarcoma. Case Rep Pediatr. 2014;2014(16):246169. PMid:25610688.

15 Bloomfield C, Lawrence D, Byrd J, et al. Frequency of prolonged remission duration after highdose cytarabine intensification in acute myeloid leukemia varies by cytogenetic subtype. Cancer Res. 1998;58(18):4173-9. PMid:9751631.

16 Tallman MS, Hakimian D, Shaw JM, Lissner GS, Russell EJ, Variakojis D. Granulocytic sarcoma is associated with the 8;21 translocation in acute myeloid leukemia. J Clin Oncol. 1993;11(4):690-7. PMid:8478662.

17 Liu PI, Ishimaru T, McGregor DH, Okada HSA, Steer A. Autopsy study of granulocytic sarcoma (chloroma) in patients with myelogenous leukaemia, hiroshima and Nagasaki 1949-1969. Cancer. 1973;31(4):948-55.<948::AID-CNCR2820310428>3.0.CO;2-N. PMid:4513297.

18 Javed Mohammadiasi SN, Mohammad S, Shirin A. Khosravi Abbas, Mohammadiasl J. Molecular and cellular aspects of extramedullary manifestations of acute myeloid leukemia. J Cancer Metastasis Treat. 2016;2(2):44-50.

19 Chen Z, Wang W, Cortes JE, et al. Differential clinical and prognostic impact of myeloid sarcoma vs medullary myeloid blast phase of chronic myelogenous leukemia in the era of tyrosine kinase inhibitor therapy. Blood Cancer J. 2016;6(5):e418. PMid:27152845.

20 Bangerter M, Hildebrand A, Waidmann O, Griesshammer M. Diagnosis of granulocytic sarcoma by fine-needle aspiration cytology. Acta Haematol. 2000;103(2):102-8. PMid:10838454.

21 Paydas S, Zorludemir S, Ergin M. Granulocytic sarcoma: 32 cases and review of the literature. Leuk Lymphoma. 2006;47(12):2527-41. PMid:17169797.

22 Torres F, Ivanova-Dragoeva A, Pereira M, et al. An e6a2 BCR-ABL fusion transcript in a CML patient having an iliac chloroma at initial presentation. Leuk Lymphoma. 2007;48(5):1034-7. PMid:17487751.

23 Nagarajarao HS, Akhtar I, Heard K, Baliga M. Unusual presentation of chronic myelogenous leukemia as multiple skin chloromas: report of a case with clinical and cytologic correlation. Acta Cytol. 2009;53(2):235-8. PMid:19365984.

24 Chen X, Rutledge JC, Wu D, Fang M, Opheim KE, Xu M. Chronic myelogenous leukemia presenting in blast phase with nodal, bilineal myeloid sarcoma and T-lymphoblastic lymphoma in a child. Pediatr Dev Pathol. 2013;16(2):91-6. PMid:23171293.

25 Kumar V, Jain N, Chaudhary SC, Mishra S. Multiple skin chloromas: a rare presentation of chronic myelogenous leukaemia in chronic stable phase. BMJ Case Rep. 2013;2013:bcr2013008626. PMid:23592814.

26 Levy RA, Mardones MA, Burch MM, Krause JR. Myeloid sarcoma as the presenting symptom of chronic myelogenous leukemia blast crisis. Baylor Univ Med Cent Proc. 2014;27(3):246-9. PMid:24982578.

27 Ai DI, Liu W, Lu G, Patel KP, Chen ZI. Extramedullary blast crisis as initial presentation in chronic myeloid leukemia with the e1a2 BCR-ABL1 transcript: a case report. Mol Clin Oncol. 2015;3(6):1319-22. PMid:26807241.

28 Dhar J, Gupta N. Lymph node myeloid sarcoma as a first presentation of CML blast crisis: a rare case report with review of literature. J Clin Diagn Res. 2018;12(5):OD05.

29 Vassallo J, Altemani AM, Cardinalli IA, et al. Granulocytic sarcoma of the larynx preceding chronic myeloid leukemia. Pathol Res Pract. 1993;189(9):1084-6. PMid:8302728.

30 Marlton P, Keating M, Kantarjian H, et al. Cytogenetic and clinical correlates in AML patients with abnormalities of chromosome 16. Leukemia. 1995;9(6):965-71. PMid:7596186.

31 Showalter JA, Tandon N, Zhao B, Tang G, Nguyen ND, Medeiros LJ. Myeloid sarcoma in a patient with myelodysplastic syndrome associated with del(5q-): case report and literature review. Ann Clin Lab Sci. 2017;47(4):466-73. PMid:28801374.

32 Pathak B, Bruchim I, Brisson ML, Hammouda W, Bloom C, Gotlieb WH. Granulocytic sarcoma presenting as tumors of the cervix. Gynecol Oncol. 2005;98(3):493-7. PMid:16019058.

33 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. PMid:16707383.

34 Ravandi-Kashani F, Cortes J, Giles FJ. Myelodysplasia presenting as granulocytic sarcoma of mediastinum causing superior vena cava syndrome. Leuk Lymphoma. 2000;36(5-6):631-7. PMid:10784409.

35 Geisse M, Mall G, Fritze D, Gartenschläger M. Granulocytic sarcoma of the tonsils associated with myelodysplastic syndrome. Dtsch Med Wochenschr. 2002;127(50):2673-6. PMid:12481238.

36 Cheng CC, Ueng SH, Li HY, et al. Post-tonsillectomy pulmonary complication in a patient with tonsillar myeloid sarcoma. Int J Hematol. 2011;93(2):220-3. PMid:21234822.

37 Cornfield DB. Erythroblastic sarcoma, an extremely rare variant of myeloid sarcoma. Hum Pathol. 2012;43(11):2080-3. PMid:22795354.

38 Grantham J, Howell D, Bacaj P, Coad JE, Vos JA. Myeloid sarcoma of the bladder in the setting of refractory anemia with excess blasts-2 (RAEB-2). W V Med J. 2015;111(6):34-6. PMid:26665895.

39 Koppisetty S, Edelman BL, Rajpurkar A. Myeloid sarcoma of the periprostatic tissue and prostate: case report and review of literature. Urol Ann. 2016;8(3):348-54. PMid:27453659.

40 Lan T-Y, Lin D-T, Tien H-F, Yang R-S, Chen C-Y, Wu K. Prognostic factors of treatment outcomes in patients with granulocytic sarcoma. Acta Haematol. 2009;122(4):238-46. PMid:19887783.

41 Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary acute myeloid leukemia. Blood. 2011;118(14):3785-93. PMid:21795742.

42 Goyal G, Bartley AC, Patnaik MM, Litzow MR, Al-Kali A, Go RS. 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. PMid:28841208.

Submitted date:

Accepted date:

Publication date:

618512aca9539565e770b693 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections