Keywords
therapeutic approach
chronic pain syndrome
Categories
How to Cite
Abstract
Ewing's sarcoma is the second most common bone tumor in childhood and adolescence. Its incidence varies significantly among races, but is relatively rare among black children and the Chinese population. Patients with Ewing sarcoma exhibit local symptoms characterized by tumor mass formation, pain, swelling, venous dilatation, and hyperemia. Pathological fractures due to bone metastases with progressive neurological deficit to paralysis may occur. In cases where Ewing's sarcoma originates in the chest wall, pleural infiltration combined with carcinomatous pleuritis is often presented .
We present the case of a 40-year-old patient initially diagnosed with a Baker's cyst in the left popliteal fossa. After consultation with an orthopedic surgeon, the patient was referred for surgical removal of the formation. An extirpation was performed, but the histological result did not confirm the diagnosis and characterized the finding as "cystic lobular capillary hemangioma ". A re-excision was performed in November 2018 due to recurrence of the formation. Immunohistochemical tests performed subsequently found that immunophenotypically and morphologically the finding correlated most highly with Ewing's extraskeletal sarcoma. Two months after excision, PT/CT was performed, which showed no recurrence and no secondary dissemination. One year later, due to arthralgias in the right shoulder joint, the patient underwent MRI and CT. The result of imaging studies revealed tumor recurrence in the diaphysis of the right humerus with secondary intrapulmonary dissemination. The patient was started on chemotherapy with good clinical response.
References
Balamuth NJ, Womer RB. Ewing’s sarcoma. Lancet Oncol. 2010; 11: 184-192.
Ewing J. Classics in oncology. Diffuse endothelioma of bone. CA Cancer J Clin. 1972; 22(2): 95-98. doi: 10.3322/canjclin.22.2.95.
Lawlor ER, Sorensen PH. Twenty Years on: What Do We Really Know about Ewing Sarcoma and What Is the Path Forward? Crit Rev Oncog. 2015; 20: 155-171.
Rochefort P, Italiano A, Laurence V et al. A Retrospective Multicentric Study of Ewing Sarcoma Family of Tumors in Patients Older Than 50: Management and Outcome. Sci Rep. 2017; 7: 17917. doi: 10.1038/s41598-017-17733-z.
Grünewald TGP, Cidre-Aranaz F, Surdez D et al. Ewing sarcoma. Nat Rev Dis Primers. 2018; 4: 5. doi: 10.1038/s41572-018-0003-x.
Koelsche C, Hartmann W, Schrimpf D et al. Array-based DNA-methylation profiling in sarcomas with small blue round cell histology provides valuable diagnostic information. Mod Pathol. 2018; 31: 1246-1256. doi: 10.1038/s41379-018-0045-3.
Turc-Carel C, Philip I, Berger MP et al. Chromosome study of Ewing‘s sarcoma (ES) cell lines. Consistency of a reciprocal translocation t(11;22)(q24;q12). Cancer Genet Cytogenet. 1984; 12(1): 1-19. doi: 10.1016/0165-4608(84)90002-5.
Turc-Carel C, Aurias A, Mugneret F et al. Chromosomes in Ewing‘s sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12). Cancer Genet Cytogenet. 1988; 32(2): 229-38. doi: 10.1016/0165-4608(88)90285-3.
Bernstein M, Kovar H, Paulussen M et al. Ewing‘s sarcoma family of tumors: current management. Oncologist. 2006; 11(5): 503-519.
Biswas B, Shukla NK, Deo SVS et al. Evaluation of Outcome and Prognostic Factors in Extraosseous Ewing Sarcoma. Pediatr Blood Cancer. 2014; 61: 1925-1931.
Sordillo PP, Hajdu SI, Magill GB, Golbey RB. Extraosseous osteogenic sarcoma. Cancer. 1983; 51(4): 727-34.
Alpert LI, Abaci IF, Werthamer S. Radiation-induced extraskeletal osteosarcoma. Cancer. 1973; 31(6): 1359-63.
Paoletti H, Colineau X, Acalet L, et al. Ewing’s sarcoma of the soft tissues: apropos of 3 cases and review of the literature. J Radiol. 1999; 80: 477-482.
Nystrom LM, Reimer NB, Reith JD et al. The Treatment and Outcomes of Extraskeletal Osteosarcoma: Institutional Experience and Review of the Literature. Iowa Orthop J. 2016; 36: 98-103.
Maheshwari V, Siddiqui F, Adreena K et al. Extraskeletal Ewing’s sarcoma – a case report. Internet J Orthop Surg. 2009; 14: 1.
Galyfos G, Karantzikos GA, Kavouras N et al. Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management. Indian J Surg. 2016; 78(1): 49-53. doi: 10.1007/s12262-015-1399-0.
Murphey MD, Senchak LT, Mambalam PK et al. From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 2013; 33(3): 803-31.
Robbin MR, Murphey MD, Jelinek JS, Temple HT. Imaging of soft tissue Ewing sarcoma and primitive neuroectodermal tumor [abstr]. Radiology. 1998; 209(P): 311.
O’Keeffe F, Lorigan JG, Wallace S. Radiological features of extraskeletal Ewing sarcoma. Br J Radiol. 1990; 63: 456-460. doi: 10.1259/0007-1285-63-750-456.
Desai SS, Jambhekar NA. Pathology of Ewing‘s sarcoma/PNET: Current opinion and emerging concepts. Indian J Orthop. 2010; 44(4): 363-368. doi: 10.4103/0019-5413.69304.
Khoury JD. Ewing sarcoma family of tumors. Adv Anat Pathol. 2005; 12: 212-20.
Folpe AL, Hill CE, Parham DM, O’Shea PA, Weiss SW. Immunohistochemical detection of FLI-1 protein expression: A study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing’s sarcoma. Am J Surg Pathol. 2000; 24: 1657-62.
Nilsson G, Wang M, Wejde J et al. Detection of EWS/FLI-1 by immunostaining. Sarcoma. 1999; 3: 25-32.
Pritsch T, Bickels J, Winberg T, Malawer MM. Popliteal sarcomas: presentation, prognosis, and limb salvage. Clin Orthop Relat Res. 2007; 455: 225-33. doi: 10.1097/01.blo.0000238812.95035.52.
Wilson PD, Eyre-Brook AL, Francis JD. A clinical and anatomical study of the semimembranosus bursa in relation to popliteal cyst. J Bone Joint Surg Am. 1938; 20: 963-984.
Cripe TP. Ewing sarcoma: an eponym window to history. Sarcoma. 2011; 2011: 457532. doi: 10.1155/2011/457532.
Paulussen M, Ahrens S, Burdach S et al. Primary metastatic (stage IV) Ewing tumor: survival analysis of 171 patients from the EICESS studies. Ann Oncol. 1998; 9(3): 275-281. doi: 10.1023/A:1008208511815.
Bolling T, Schuck A, Paulussen M et al. Whole lung irradiation in patients with exclusively pulmonary metastases of Ewing tumors. Toxicity analysis and treatment results of the EICESS-92 trial. Strahlenther Onkol. 2008; 184(4): 193-197. doi: 10.1007/s00066-008-1810-x.
This work is licensed under a Creative Commons Attribution 4.0 International License.