Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 9  |  Issue : 5  |  Page : 651-653  

Fine needle aspiration cytology of myeloid sarcoma presenting as soft tissue swelling


1 Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra  Pradesh, India
2 Department of Medical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra  Pradesh, India
3 Department of Surgical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra  Pradesh, India

Date of Web Publication13-Oct-2016

Correspondence Address:
Rashmi Patnayak
Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati - 517 507, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.192150

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  Abstract 


Myeloid sarcoma is rare. It is a tumor of immature myeloid cells. We present one such case where the patient presented with a soft tissue swelling in the thigh. The fine needle aspiration cytology (FNAC) was reported as myeloid sarcoma. Simultaneously, the patient was subjected to routine hematological examination which revealed chronic myeloid leukemia in chronic phase. The diagnosis was further confirmed by bone marrow aspiration and cytogenetic study. This case emphasizes FNAC as the easy, rapid, and cost-effective method of diagnosis. The patient was subsequently treated and is currently under follow-up. Hereby, we are presenting an uncommon case of myeloid sarcoma with brief literature review and discussion about the differential diagnoses.

Keywords: Chronic myeloid leukemia, fine needle aspiration cytology, myeloid sarcoma, soft tissue swelling


How to cite this article:
Rajitha J, Patnayak R, Reddy S, Asha T, Jena A. Fine needle aspiration cytology of myeloid sarcoma presenting as soft tissue swelling. Med J DY Patil Univ 2016;9:651-3

How to cite this URL:
Rajitha J, Patnayak R, Reddy S, Asha T, Jena A. Fine needle aspiration cytology of myeloid sarcoma presenting as soft tissue swelling. Med J DY Patil Univ [serial online] 2016 [cited 2024 Mar 29];9:651-3. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2016/9/5/651/192150




  Introduction Top


The synonyms of myeloid sarcoma are chloroma, extramedullary myeloid cell tumor, and granulocytic sarcoma. It is a rare disease. It is characterized by the occurrence of extramedullary myeloid cell masses with or without bone marrow involvement.[1] It has variable presentation. It is associated with acute myeloid leukemia (AML), myelodysplastic syndrome, and other myeloproliferative disorders, especially chronic myeloid leukemia (CML) in blast crisis.[1],[2] Fine needle aspiration cytology (FNAC) can diagnose myeloid sarcoma easily and accurately.[3],[4] We present a case of myeloid sarcoma in a patient whose initial presentation was as a soft tissue swelling in the thigh. The routine blood investigation done at the same time revealed CML in chronic phase. This was subsequently followed by bone marrow aspiration and cytogenetics, which confirmed the diagnosis.


  Case Report Top


A 44-year-old male with complaint of subcutaneous swellings for 1 month was referred to the Department of Pathology for fine needle aspiration (FNA) in the month of March 2015. The swelling had started first at the region of medial aspect of the right thigh, 15 days back as a small swelling and gradually increased in size. It was followed by another swelling over the upper abdominal wall on the left side. There was no history of trauma. He also complained of loss of weight for last 1 month.

On examination, there were two swellings over the thigh and abdominal wall measuring 3 cm × 2 cm and 2 cm × 1 cm, respectively. Swellings were firm in consistency, mobility was restricted, and there was blackish discoloration over the skin. There was no associated tenderness, ulceration, or rise of temperature.

FNA was done from both the swellings. The smears showed plenty of neutrophils admixed with immature myeloid precursor cells with abundant eosinophilic cytoplasm with large nuclei (myeloblast). There were many scattered metamyelocytes [Figure 1] and [Figure 2]. The FNA report was suggestive of myeloid sarcoma.
Figure 1: Fine needle aspiration of the soft tissue swelling showing neutrophils and immature myeloid series cells (band forms, metamyelocytes, and myelocytes) (H and E, ×400)

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Figure 2: Cyto smear showing both mature and immature myeloid series cells including occasional myeloblast (May-Grunwald Giemsa, ×400)

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On general examination, he had pallor. There was no icterus, cyanosis, clubbing, and/or lymphadenopathy. Massive splenomegaly was present. There was no hepatomegaly. At the same time, routine hematological investigations done revealed hemoglobin of 9.1 g/dl. Total leukocyte count was markedly increased (137,900 cells/cmm) with differential count of myeloblasts-8%, myelocytes-15%, promyelocytes-3%, metamyelocytes-6%, band forms and neutrophils-38%, lymphocytes-2, monocytes-1, eosinophils-5%, and basophils-20%. Platelets were slightly reduced (1.32 lakhs/cmm). The peripheral smear was reported as CML — chronic phase [Figure 3].
Figure 3: Peripheral smear with left shift in myeloid cell series and occasional nucleated red blood cell (Leishman, ×400)

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Subsequently, he underwent bone marrow aspiration and trephine biopsy. Cytogenetics was reported as BCR/ABL fusion signal in 90% cells and sample was positive for t(9:22).

The patient was referred to the Medical Oncology Department. He received tablet imatinib 600 mg/week. The splenomegaly subsided and the subcutaneous swellings disappeared 2 weeks after treatment. He was put on imatinib 600 mg once in a month. His total leukocyte count was reduced to normal range after 2 months of treatment. He is under regular follow-up and presently without any complaint.


  Discussion Top


Myeloid sarcoma was first described in the year 1812.[5] It is also termed as chloroma because of its green appearance on gross morphology.[6] This green appearance is due to the content of myeloperoxidase enzymes in the myeloblasts.[6] Almost always, the diagnosis of myeloid sarcoma is preceded by or is associated with either known preexisting or concomitant diagnosis of acute leukemia, myelodysplastic syndrome, or chronic myeloproliferative disorder.[7] Most commonly myeloid sarcoma is associated with AML.[1] Association of myeloid sarcoma with CML in chronic phase is relatively rare.

In our case, the diagnosis of soft tissue myeloid sarcoma and CML was concomitant. CML in chronic phase with concurrent myeloid sarcoma is quite unusual.[8]

The age of presentation of myeloid sarcoma is highly variable. Most common extramedullary sites involved by myeloid sarcoma include skin, bone, and lymphnodes.[9] Soft tissue involvement is also commonly seen.[4]

FNA is a cost-effective method and can provide rapid diagnosis particularly in accessible lesions. Our patient's FNA of the soft tissue swelling over thigh revealed myeloid sarcoma, and the peripheral blood examination done as part of the initial work-up revealed CML in chronic phase. This presentation of CML in chronic phase with soft tissue swelling is quite rare. There are few similar case reports available in literature emphasizing the role of FNAC.[3],[4]

Isolated myeloid sarcoma diagnosis is a challenge both clinically and microscopically. Available literature shows that myeloid sarcomas, particularly those with blast predominance, are most commonly erroneously diagnosed as non-Hodgkin lymphoma.[1],[7],[10],[11]

In FNA smears, the differential diagnosis of myeloid sarcoma includes non-Hodgkin lymphoma, poorly differentiated carcinoma, extramedullary hematopoiesis, and infectious processes. Non-Hodgkin lymphoma and poorly differentiated carcinoma may be considered in the differential diagnosis of myeloid sarcoma showing predominantly blasts. For myeloid sarcoma showing more mature forms, extramedullary hematopoiesis and infection may be considered. In extramedullary hematopoiesis, in addition to the mature and immature form of myeloid series cells, erythroid series and megakaryocytic series cells also can be seen, and in cases of infection apart from relevant history, there will be more number of neutrophils and some may show the presence of toxic granules.[4],[11]

The further classification of myeloid sarcoma is based on the predominant cell type and according to cell maturation. Thus, it can be subclassified as granulocytic, monoblastic, or myelomonocytic type or into immature, mature, and blastic types.[8] Depending on the cytomorphology, our case can be subtyped as granulocytic according to cell type and immature type according to cell maturation. We did not receive any soft tissue biopsy of the swelling as it subsided after initiation of treatment.

To diagnose myeloid sarcomas accurately and to sub-classify them, correlation of morphology, immunophenotype, cytogenetics, and molecular diagnostics is required.[1] In our case, the typical cytological appearance of the FNA smear, peripheral smear, and bone marrow examination helped us to conclude this case as myeloid sarcoma. Later on, the cytogenetics confirmed it as a case of CML.

We present this unusual case of myeloid sarcoma diagnosed by FNAC emphasizing the importance of FNA. Our patient received chemotherapy and is currently doing well.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hagen PA, Singh C, Hart M, Blaes AH. Differential diagnosis of isolated myeloid sarcoma: A case report and review of the literature. Hematol Rep 2015;7:5709.  Back to cited text no. 1
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2.
Tuset E, Ribera JM, Vaquero M, Jiménez C, Millá F, Juncá J, et al. Granulocytic sarcoma: A study of 5 cases. Med Clin (Barc) 1995;104:377-80.  Back to cited text no. 2
    
3.
Bothale KA, Wilkinson A, Mahore SD, Patrikar AD, Bothale A. Extramedullary granulocytic sarcoma as an initial presenting feature of chronic myeloid leukemia. J Case Rep Pract 2013;3:64-6.  Back to cited text no. 3
    
4.
Suh YK, Shin HJ. Fine-needle aspiration biopsy of granulocytic sarcoma: A clinicopathologic study of 27 cases. Cancer 2000;90:364-72.  Back to cited text no. 4
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5.
Burns A. Observations of Surgical Anatomy, Head and Neck. Edinburgh: Thomas Royce and Co.; 1811. p. 364-6.  Back to cited text no. 5
    
6.
King A. A case of chloroma. Mon J Med 1853;17:97.  Back to cited text no. 6
    
7.
Byrd JC, Edenfield WJ, Shields DJ, Dawson NA. Extramedullary myeloid cell tumors in acute nonlymphocytic leukemia: A clinical review. J Clin Oncol 1995;13:1800-16.  Back to cited text no. 7
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8.
Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: Clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia 2007;21:340-50.  Back to cited text no. 8
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9.
Avni B, Koren-Michowitz M. Myeloid sarcoma: Current approach and therapeutic options. Ther Adv Hematol 2011;2:309-16.  Back to cited text no. 9
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10.
Menasce LP, Banerjee SS, Beckett E, Harris M. Extra-medullary myeloid tumour (granulocytic sarcoma) is often misdiagnosed: A study of 26 cases. Histopathology 1999;34:391-8.  Back to cited text no. 10
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11.
Yilmaz AF, Saydam G, Sahin F, Baran Y. Granulocytic sarcoma: A systematic review. Am J Blood Res 2013;3:265-70.  Back to cited text no. 11
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    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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