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| CASE REPORT |
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| Year : 2022 | Volume
: 2
| Issue : 1 | Page : 38-40 |
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Unusual sites of metastases at initial presentation in Ewing-like sarcoma of extremity in an elderly male
PG Nandakumar, Kamaxi Trivedi, Amitkumar Choudhari, Amitkumar Janu
Department of Radiodiagnosis, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, India
| Date of Submission | 10-Feb-2022 |
| Date of Decision | 05-Mar-2022 |
| Date of Acceptance | 07-Mar-2022 |
| Date of Web Publication | 15-Jun-2022 |
Correspondence Address:
Dr. Amitkumar Janu
Department of Radiodiagnosis, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai - 400 012, Maharashtra
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/aort.aort_1_22
Ewing and Ewing-like sarcoma are high-grade sarcomas with the histological presence of small round blue cells with a higher propensity for hematogenous metastases. The most common pattern is appendicular skeleton involvement with or without metastases. In this case report, we present a 55-year-old gentleman with Ewing-like sarcoma of the left femur with metastases to rare sites.
Keywords: Ewings, sarcoma, Metastases
How to cite this article:
Nandakumar P G, Trivedi K, Choudhari A, Janu A. Unusual sites of metastases at initial presentation in Ewing-like sarcoma of extremity in an elderly male. Ann Oncol Res Ther 2022;2:38-40 |
How to cite this URL:
Nandakumar P G, Trivedi K, Choudhari A, Janu A. Unusual sites of metastases at initial presentation in Ewing-like sarcoma of extremity in an elderly male. Ann Oncol Res Ther [serial online] 2022 [cited 2022 Jun 25];2:38-40. Available from: http://www.aort.com/text.asp?2022/2/1/38/347551 |
| Introduction | |  |
Ewing and Ewing-like sarcomas are a group of high-grade sarcomas with similar histomorphological appearance characterized by small round blue cells, with the main difference being the presence of Ewing sarcoma breakpoint region 1 (EWSR1)-educational testing service (ETS) (EWSR1: and ETS: ETS Proto-Oncogene 1, Transcription Factor) translocation in Ewing sarcoma, which is absent in other Ewing-like sarcomas.[1] The organ system and pattern of involvement are summarized in [Table 1].[2],[3] | Table 1: Ewing sarcoma-organ system affected and their involvement pattern
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| Case Report | | |
We present a 55-year-old gentleman referred to our hospital with left knee swelling for 6 months. A preliminary diagnosis of the primary bone tumor was made, and further workup with magnetic resonance imaging (MRI) of left knee region for locoregional evaluation, whole-body (18 F fluorodeoxyglucose [FDG]-positron emission tomography [PET] computed tomography [CT]) for metastatic workup, and biopsy for tissue diagnosis. Histopathological diagnosis was undifferentiated Ewing-like sarcoma; EWSR-1 ETS fusion: Negative on FISH (Fluorescent in situ hybridization). FDG-PET revealed a low-grade uptake standardized uptake value (SUV) max 4 in the distal femur mass with associated cortical destruction and extraosseous soft tissue component [Figure 1]. The CT component of PET-CT reveals enhancing pancreatic head mass with very low-grade FDG uptake (SUV max-2.3) was seen along with intraabdominal nodes, peritoneal deposits, hepatic lesion, right atrial and transverse pericardial recess lesions, and intramuscular deposits within the left thigh and gluteal muscles [Figure 1], [Figure 2], [Figure 3]. These were non-FDG avid in the PET-CT. Ultrasonography-guided (fine needle aspiration cytology) of the pancreatic head mass revealed a high-grade sarcomatous tumor with spindle cells. The multidisciplinary tumor board meeting made a diagnosis of Ewing-like sarcoma with pancreatic, soft tissue, peritoneal, cardiac, and pericardial metastases. The patient was deemed unresectable due to widespread metastases and offered the best supportive care. | Figure 1: Axial computed tomography, fluorodeoxyglucose positron emission tomography-computed tomography images show (a and b) minimally enhancing soft tissue mass involving distal femur with low-grade fluorodeoxyglucose avidity, (c and d) similar morphology peritoneal deposits seen beneath anterior abdominal wall and in the presacral region without fluorodeoxyglucose uptake
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 | Figure 2: Axial computed tomography images show (a) minimally enhancing soft tissue lesion in the transverse pericardial recess, (b) similar morphology lesion within right atrium, (c) hypoenhancing mass replacing the pancreatic head, (d) intramuscular soft tissue deposits within the muscles of left thigh
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 | Figure 3: Axial fused fluorodeoxyglucose positron emission tomography-computed tomography images show nonfluorodeoxyglucose-avid lesions at the following sites. (a) Transverse pericardial recess, (b) right atrium, (c) pancreatic head, (d) intramuscular soft tissue deposits within the muscles of left thigh
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| Discussion | | |
Metastases are relatively common and are usually hematogenous. The most frequently involved sites in incidence are lymph node, bone, lung, pleural, peritoneum, solid viscera, and brain.[4] Prior case reports by Petrovic et al., Janssen et al., and Coccia et al. had described extremity Ewing sarcoma with intracardiac metastasis [Table 2]. The patients in Petrovic and Coccia et al. had right ventricular metastasis while in Janssen et al., it was in left ventricle.[5],[6],[7] Wang et al. reported a case of primary pericardial PNET tumor.[8]
There are many atypical patterns observed with this case, such as older age of presentation, uncommon sites of metastases, and non-FDG avidity in these lesions. In concordance with the saying “The eyes cannot see what the mind does not know,” radiologists should always have a vigilant eye while evaluating any malignant tumors keeping in mind the pattern of the primary and metastatic disease, as the imaging findings have close implications on management and treatment protocols.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his image and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Renzi S, Anderson ND, Light N, Gupta A. Ewing-like sarcoma: An emerging family of round cell sarcomas. J Cell Physiol 2019;234:7999-8007.  |
| 2. | Javery O, Krajewski K, O'Regan K, Kis B, Giardino A, Jagannathan J, et al. A to Z of extraskeletal ewing sarcoma family of tumors in adults: Imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol 2011;197:W1015-22. |
| 3. | Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ. From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation. Radiographics 2013;33:803-31. |
| 4. | Huh J, Kim KW, Park SJ, Kim HJ, Lee JS, Ha HK, et al. Imaging features of primary tumors and metastatic patterns of the extraskeletal ewing sarcoma family of tumors in adults: A 17-year experience at a single institution. Korean J Radiol 2015;16:783-90. |
| 5. | Petrovic M, Zhao B, Thangam M, Loyalka P, Buja LM, Kar B, et al. Ewing sarcoma in the right ventricle. Tex Heart Inst J 2016;43:458-60. |
| 6. | Janssen DP, Van de Kaa CA, Noyez L, Van Haelst UJ, Lacquet LK. A solitary metastasis in the heart from Ewing's sarcoma. Eur J Cardiothorac Surg 1994;8:51-3. |
| 7. | Coccia P, Ruggiero A, Rufini V, Maurizi P, Attinà G, Marano R, et al. Cardiac metastases of Ewing sarcoma detected by 18F-FDG PET/CT. J Pediatr Hematol Oncol 2012;34:236-8. |
| 8. | Wang J, Li J, Zhang X, Zhang X, Xiao Y. Primitive neuroectodermal tumor of the pericardium: A case report and literature review. BMC Cardiovasc Disord 2021;21:305. |
[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]
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