Journal of Orthopaedics and Traumatology

Official Journal of the Italian Society of Orthopaedics and Traumatology

Journal of Orthopaedics and Traumatology Cover Image
Open Access

Shoulder pain: a hematologist’s perspective

Journal of Orthopaedics and TraumatologyOfficial Journal of the Italian Society of Orthopaedics and Traumatology 201617:403

Received: 21 February 2016

Accepted: 17 March 2016

Published: 13 April 2016


Acute Myeloid LeukemiaChronic Myeloid LeukemiaShoulder PainSeptic ArthritisSoft Tissue Mass


Orthopedic surgeons commonly encounter cases of shoulder pain in their clinical practice, differential diagnosis of which includes, but is not limited to, septic arthritis. Usually perceived as ‘benign’, shoulder pain may be a harbinger of a serious underlying disorder including hematological malignancies. Jain et al. recently reported a case of chronic myeloid leukemia (CML) presenting as shoulder pain mimicking septic arthritis, and later diagnosed as myeloid sarcoma (MS) [1]. MS represents an extramedullary accumulation of immature cells of granulocytic series and occurs most commonly in the setting of acute myeloid leukemia (AML), where its incidence is 2–8 %, occurring after (50 %), prior to diagnosis (25 %) or concurrently (15–35 %) with AML and may rarely be a first site of AML relapse. Diagnosis requires fine needle aspiration (FNA) and immunohistochemistry (MS being positive for myeloperoxidase) [1]. Although bone and periosteum are amongst the commonest sites of MS, joint involvement is extremely rare and results from tumor invasion of the cortex and medulla resulting into a soft tissue mass [3]. Considering the shoulder as an important though rare site of MS, we reviewed all cases of MS in the English literature involving the shoulder. A brief review of all cases of shoulder MS, including their clinical/radiological findings, treatment and outcome is presented in Table 1 [16]. Amongst six cases of shoulder MS, CML was the commonest underlying etiology, and in one of them MS was an initial presentation of CML. Males in their fourth decade were most commonly affected. Pain and mass in the shoulder were the commonest presenting complaints. Examination could identify splenomegaly (two cases) and axillary lymphadenopathy (one case), and MRI could identify a soft tissue mass (five cases) with or without an associated lytic lesion. From an orthopedic view point, septic arthritis was the commonest primary diagnosis. All the cases of CML received tyrosine kinase inhibitors, and three of them also received an additional systemic chemo-radiotherapy. Prognosis of shoulder MS is guarded and long term survival is unreported. Though MS is uncommon in CML, myelodysplastic syndrome and other myeloproliferative neoplasms, our literature review identified CML as the leading diagnosis in cases of MS of the shoulder. Considering its rarity, no definite treatment guidelines are available. Although systemic chemotherapy followed by allogeneic stem cell transplantation clearly offers a survival advantage in cases of MS, lack of matched sibling/unrelated donors and financial costs are real concerns in developing countries like India, where combined chemo-radiotherapy holds promise as the best form of ‘palliation’ due to lack of its survival benefits and the poorer prognosis of such cases [1]. We conclude that, although septic arthritis is the commonest entity producing shoulder pain and swelling, presence of an associated lymphadenopathy, splenomegaly, soft tissue component with/without lytic lesion on MRI, peripheral leucocytosis with immature granulocytes/blasts, and absence of response to antibiotics should prompt an orthopedic surgeon to seek a hematology consultation maintaining a high index of suspicion for MS. Hematologists should henceforth realize the urgent need for FNA, and the importance of performing immunohistochemistry (IHC) in cases of septic arthritis with the above features being referred from the orthopedic side for timely and accurate diagnosis and treatment.
Table 1

Review of cases of shoulder myeloid sarcoma with clinical details, treatment given, and outcome

Study no.





Clinical presentation

Time of presentation with shoulder sarcoma

Additional findings

Imaging features

Systemic involvement

Underlying diagnosis

Molecular/cytogenetic abnormalities

Treatment given





Levy et al. [2]


Right posterior shoulder pain

Initial presentation at diagnosis

Firm mass at the back, axillary lymphadenopathy

Lytic lesion in inferior angle of scapula

2 % blasts in periphery (CML-CP)


BCR-ABL1 (p210) rearrangement

Dasatinib followed by allogeneic SCT

Not reported




Upadhyay et al. [3]


Right shoulder pain

Diagnosed case of CML since 2009, on hydroxyurea for last 2 years

Swelling over anterolateral aspect of right proximal arm, splenomegaly

Soft tissue mass lesion involving proximal part of

right humerus with cortical breaks in the humeral head and

neck completely encasing and infiltrating it



Not available

Systemic chemotherapy and RT

Died 6 months after diagnosis




Cozzi et al. [4]


Incidentally found to have myeloid sarcoma following a fracture after accident

Diagnosed as CML in 1989, received interferon, hydroxyurea and imatinib

Pain in left shoulder

Proximal humerus osteolytic lesion

associated with extensive substitutive tissue

Bone marrow in CP


Not available

Imatinib +dexamethasone + cytarabine followed by local RT

Died due to mycotic pulmonary infection




Alkubaidan et al. [5]


Painful swelling of left shoulder 1 year after Allo-SCT

Diagnosed as SDS in teenage years, on pancreatic enzyme supplementation, with history of MDS, and underwent Allo-SCT 1 year back

Avulsion fracture of greater tubercle

Soft tissue mass circumferentially engulfing

the proximal humerus, the rotator cuff and the long head

of biceps tendon


Shwachman-Diamond syndrome








Lincopan et al. [6]


Mass in right shoulder

Initial presentation

Mass in inner thigh, rib cage, middle-posterior mediastinum

Soft issue masses in the sub-dermal region

Not present

Isolated MS

Trisomy 11






Jain et al. [1]


Pain and swelling of left shoulder

Diagnosed as CML-CP in 2004 (on Imatinib 400 mg OD), progressed to AP in 2014 (imatinib 600 mg) and had left shoulder pain in 2015

Redness and induration of left shoulder, splenomegaly

MRI of the left shoulder showing an ill-defined

heterogeneously enhancing

lesion involving the muscles

around the shoulder and

infiltrating into clavicle

CML-CP (peripheral blood and Bone marrow)


BCR-ABL (H396R mutation in kinase domain)

High dose imatinib, hydroxyurea, low dose cytarabine and local radiotherapy



CML Chronic myeloid leukemia, CP chronic phase, AP accelerated phase, SCT stem cell transplantation, NA not available, RT radiotherapy


Compliance with ethical standards

Conflict of interest

The authors declare no conflicts of interests

Ethical statement

The authors state that the work has not been published elsewhere and is not under the consideration for publication by any other journal. The work abides by the Helsinki’s guidelines for publication, 1976.

Sources of funding


Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Authors’ Affiliations

Department of Hematology, Nehru Hospital, PGIMER, Chandigarh, India


  1. Jain A, Sahu KK, Saniya S, Rajwanshi A, Suri V, Malhotra P (2016) Shoulder myeloid sarcoma: an initial presentation of CML blast crisis. Indian J Hematol Blood Transfus. doi:10.1007/s12288-016-0656-0 Google Scholar
  2. Levy RA, Mardones MA, Burch MM, Krause JR (2014) Myeloid sarcoma as the presenting symptom of chronic myelogenous leukemia blast crisis. Proc (Bayl Univ Med Cent). 27:246–249PubMedPubMed CentralGoogle Scholar
  3. Upadhyay S, Rawat SJ, Gupta G, Saxena U (2014) Chloroma (Granulocytic sarcoma): an unusual cause of shoulder pain in chronic myeloid leukemia; a diagnostic dilemma. J Orthop Allied Sci 2:20–23View ArticleGoogle Scholar
  4. Cozzi P, Nosari A, Cantoni S, Ribera S, Pungolino E, Lizzadro G et al (2004) Traumatic left shoulder fracture masking aggressive granuloblastic sarcoma in a CML patient. Haematologica 89:EIM15PubMedGoogle Scholar
  5. Alkubaidan FO, Alghamdi FA, Heffernan EJ, Stevenson WT, Skinnider BF, Munk PL (2007) Granulocytic sarcoma (chloroma) of the shoulder in Shwachman–Diamond syndrome. Eur J Radiol Extra: 107–110Google Scholar
  6. Lincopan SA, Valencia MY, Carrasco LC, Barraza OX (2011) Myeloid sarcoma: report of one case with trisomy 11. Rev Med Chil 139:914–916View ArticleGoogle Scholar


© The Author(s) 2016