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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 79-83

Adult-onset clavicular Ewing’s sarcoma/peripheral neuroectodermal tumor: A diagnostic challenge


1 Department of Orthopaedic Oncology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India
2 Department of Medical Oncology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India
3 Department of Radiation Oncology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India
4 Department of Histopathology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India

Date of Web Publication30-Dec-2019

Correspondence Address:
Dr. Himanshu Rohela
Department of Orthopaedic Oncology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jco.jco_13_19

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  Abstract 

Primary Ewing’s sarcoma (ES) of clavicle is rare; moreover, an adult patient diagnosed with clavicular ES is extremely rare. We report a case of adult-onset clavicular ES under our tracking and review of the related literature on the management of this rare tumor. A 24-year-old adult presented with an incidentally detected clavicle mass. A biopsy from the clavicle was diagnosed as ES. Whole body positron emission technique scan was suggestive of nonmetastatic presentation. The patient received both neoadjuvant and adjuvant chemotherapy. Medial clavicle resection was performed and adjuvant XRT was started. At follow-up, the patient had good range of painless shoulder movement. In conclusion, it was observed that ES of clavicle is a very rare tumor and poses a diagnostic challenge. Clavicular tumors rarely need reconstruction after resection.

Keywords: Ewing’s sarcoma, positron emission technique, magnetic resonance imaging, immunohistochemistry


How to cite this article:
Rohela H, Limaye S, Chadha P, Adamane S, Vhatkar R. Adult-onset clavicular Ewing’s sarcoma/peripheral neuroectodermal tumor: A diagnostic challenge. J Curr Oncol 2019;2:79-83

How to cite this URL:
Rohela H, Limaye S, Chadha P, Adamane S, Vhatkar R. Adult-onset clavicular Ewing’s sarcoma/peripheral neuroectodermal tumor: A diagnostic challenge. J Curr Oncol [serial online] 2019 [cited 2020 Jul 7];2:79-83. Available from: http://www.journalofcurrentoncology.org/text.asp?2019/2/2/79/274298




  Introduction Top


Ewing’s sarcoma (ES) is the second most common bone cancer of the childhood. Although any bone can be affected, a proclivity for the femur and pelvis is known. Multiple series are previously published for primary ES with the age usually younger than 30 years. Malignant tumors of clavicle are very rare and comprise less than 0.5% of all malignant bone tumors[1] and 1.4% of all ES.[2] Histologically, ES is characterized by malignant round cell tumor and is differentiated from other tumors by its characteristic immunohistochemistry expression of membranous staining for Microneme 2, Cluster of differentiation 99 and nuclear staining for Friend leukemia integration 1. At the molecular level, ES harbors translocation between EWS gene on chromosome 11 to the FLI-1 gene in chromosome 22.[2]


  Case Report Top


We report a case of an adult healthy male of 24 years of age with a palpable mass over the right clavicle with intermittent pain since the last 6 months. Pain was of low intensity and limited to the site of swelling and was relieved with analgesic medication. No history of any constitutional symptoms was observed. Physical examination revealed a 7 × 6 cm palpable mass over the sternal end of the right clavicle. It was firm and fixed to the underlying bone, well defined with regular margins and smooth surface. No history of trauma was elicited. The patient had full shoulder range of motion with normal strength and sensation on the right upper extremity.

Plain radiograph showed expansile lytic lesion on medial end right clavicle with surrounding soft tissue swelling shadow involving medial two-third of right clavicle [Figure 1]. However, the shoulder joint was normal. Magnetic resonance imaging (MRI) documented a soft tissue mass surrounding the right clavicle as well as marrow involvement by the lesion involving medial two-third of right clavicle [Figure 2] and [Figure 3].
Figure 1: Radiograph showing lytic destruction medial end right clavicle

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Figure 2: MRI showing inflammatory or neoplastic lesion right clavicle with soft tissue component

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Figure 3: MRI showing inflammatory or neoplastic lesion right clavicle with soft tissue component

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In view of suspicion of inflammatory pathology, the relevant inflammatory markers were sent and a needle biopsy was planned. Laboratory investigation revealed erythrocyte sedimentation rate of 19 mm/h, alkaline phosphatase of 59.90 U/L, and normal C-reaction protein. The core needle biopsy was suggestive of Ewing’s sarcoma of right clavicle [Figure 4]. Staging whole body positron emission technique scan was performed, suggestive of nonmetastatic primary neoplasm of right clavicle.
Figure 4: Core needle biopsy report confirming ES with detailed Immunohistochemistry panel test

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Neoadjuvant chemotherapy vincristine, adriamycin, cyclophosphamide/ifosfamide, etoposide (IE-VAC)-based protocol was started, and MRI was performed after seven cycles to observe the response to treatment [Figure 5] and [Figure 6]. Wide resection of medial two-third of clavicle, preserving the acromioclavicular ligament attachment, was carried out [Figure 7]. Postoperative period was uneventful and stitch was removed on 10th postoperative day with good range of shoulder and neck movement [Figure 8][Figure 9][Figure 10].
Figure 5: Post-chemotherapy MRI shows significant reduction in the size of lesion

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Figure 6: Post-chemotherapy MRI shows significant reduction in the size of lesion

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Figure 7: Postoperative radiograph

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Figure 8: Good range of shoulder function

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Figure 9: Neck movements post resection of medial end clavicle

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Figure 10: Neck movements post resection of medial end clavicle

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The resected specimen [Figure 11] revealed a complete response to the chemotherapy without viable tumor. The marrow revealed hyalinization and fibrosis replacing the tumor entirely [Figure 12]. Adjuvant radiotherapy in a dose of 60 Gy in 30 fractions, 2 Gy/fraction, was started. Presently, the patient is under ongoing adjuvant chemotherapy protocol.
Figure 11: Resected specimen

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Figure 12: Histopathology examination showing hyalinization and fibrosis with no residual viable tumor

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  Discussion Top


ES, described by James Ewing, is a primary malignant tumor. Immunocytochemical, cytogenetic, and molecular genetic investigations have suggested a neural crest origin for these malignant tumors.[3] ES is a common bone tumor found in age-group younger than 30 years, second only to osteosarcoma. Clavicular ES is a diagnostic challenge, and only a few reports are available in the current literature. ES usually occurs in patients younger than 30 years with minimal male preponderance (male:female = 3:2).[4],[5] The most frequent sites of involvement being the diaphyseal region of long bones. In the thoracic cage, ES predominates in the ribs, with rare involvement of the scapula, clavicle, and sternum.

Primary nonmetastatic extremity ES treatment strategies included neoadjuvant chemotherapy followed by surgery or surgery plus radiotherapy followed by completion of adjuvant chemotherapy. Overall survival of 70%–75% can be achieved in nonmetastatic cases following a complete treatment. In excision for clavicular tumors, reconstruction procedures are rarely required.[6] On the contrary, metastatic ES has dismal survival patterns with overall survival ranging from 15% to 20%.[7] In our case, we did not use any reconstructive procedure as we were able to preserve the acromioclavicular and costoclavicular ligament attachment to lateral end of clavicle, which we believe resulted in good functional range of shoulder movements in our patient.

The patient is unusual for several reasons. Adult-onset primary clavicular ES is rare. There are no such instances in the series by Maygarden et al.[8] Other published series document less than 5% of lesions in the clavicle.[9] ES is rare among the African and the Asian population. Thus, our patient is the most unusual in three ways: race, age, and affected bone.

Patients with clavicular ES have late presentation owing to low suspicion. Many receive therapy for unrelated conditions such as tuberculosis and other inflammatory conditions. The treating clinician need to be aware of this rare occurrence. Our case is instructive in alerting physicians that clavicular ES is also a rare possibility even if the adult does not fit the typical demographics for ES.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. patient gave his consent for his images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Waller DA, Newman RJ. Primary bone tumours of the thoracic skeleton: An audit of the Leeds Regional Bone Tumour Registry. Thorax 1990;45:850-5.  Back to cited text no. 1
    
2.
Granowetter L, West DC. The Ewing’s sarcoma family of tumors: Ewing’s sarcoma and peripheral primitive neuroectodermal tumor of bone and soft tissue. Cancer Treat Res 1997;92:253-308.  Back to cited text no. 2
    
3.
Levine SM, Lambiase RE, Petchprapa CN. Cortical lesions of the tibia: Characteristic appearances at conventional radiography. Radiographics 2003;23:157-77.  Back to cited text no. 3
    
4.
Priemel MH, Stiel N, Zustin J, Luebke AM, Schlickewei C, Spiro AS. Bone tumours of the clavicle: Histopathological, anatomical and epidemiological analysis of 113 cases. J Bone Oncol 2019;16:100229.  Back to cited text no. 4
    
5.
Tateishi U, Gladish GW, Kusumoto M, Hasegawa T, Yokoyama R, Tsuchiya R, et al. Chest wall tumors: Radiologic findings and pathologic correlation: part 2. Malignant tumors. Radiographics 2003;23:1491-508.  Back to cited text no. 5
    
6.
Smith J, Yuppa F, Watson RC. Primary tumors and tumor-like lesions of the clavicle. Skeletal Radiol 1988;17:235-46.  Back to cited text no. 6
    
7.
Burgert EO Jr, Nesbit ME, Garnsey LA, Gehan EA, Herrmann J, Vietti TJ. Multimodal therapy for the management of nonpelvic , localized Ewing’s sarcoma of bone: Intergroup study IESS - II. J Clin Oncol1990;8:1514-24.  Back to cited text no. 7
    
8.
Maygarden SJ, Askin FB, Siegal GP, Gilula LA, Schoppe J, Foulkes M, et al. Ewing sarcoma of bone in infants and toddlers. A clinicopathologic report from the intergroup Ewing’s study. Cancer 1993;71:2109-18.  Back to cited text no. 8
    
9.
Wilkins RM, Pritchard DJ, Burgert EO Jr, Unni KK. Ewing’s sarcoma of bone. Experience with 140 patients. Cancer 1986;58:2551-5.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]



 

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