Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor
© Vanel et al; licensee BioMed Central Ltd. 2011
Received: 13 April 2011
Accepted: 1 August 2011
Published: 1 August 2011
A 35-year-old female presented with pain and swelling of the distal left radius. A diagnosis of Ewing sarcoma was made and she underwent neoadjuvant chemotherapy and surgery. Macroscopic viable areas remained on the map of the surgical specimen; as such, she was classified as a poor responder and received high dose adjuvant chemotherapy. She remained disease-free for five years, until age 40. A vaginal polyp was then detected during a routine gynaecologic examination. It was removed and histopathology revealed metastatic Ewing sarcoma.
To our knowledge, this is the first reported case of a vaginal metastasis of Ewing sarcoma.
Ewing sarcoma (ES) is a small blue round cell tumor belonging to the Ewing Family Tumour (EFT) together with Primary Neuroectodermal Tumour (PNET) and ASKIN tumor (of the thoracic wall). Eight hundred and ninety six cases have been reported in our institute since 1982. Ewing sarcoma has a distinct predilection for males and occurs in the first two decades of life in more than 75 percent of cases [1, 2].
Metastases are frequent  and are mostly pulmonary and osseous, but can be found in various other locations.
We present here the first description of a vaginal metastasis of Ewing sarcoma.
A biopsy was performed and histological examination revealed a typical Ewing sarcoma. The diagnosis was confirmed by FISH analysis, which demonstrated the translocation t (11, 22).
Staging revealed a solitary non-specific pulmonary nodule of the inferior right lobe that did not change with time and was not considered metastatic.
The patient underwent neoadjuvant chemotherapy and resection and allograft of the distal radius. She was considered as poor responder as macroscopic areas remained on the surgical specimen, but all margins were free of disease. High dose chemotherapy was then performed.
After completion of her treatment, it was followed up as per protocol and remained disease-free.
No treatment has been undertaken.
Ewing sarcoma (ES) represents approximately ten percent of primary malignant bone tumours and one percent of soft tissue tumours. It tends to arise in the diaphysis or metaphyseal-diaphyseal portion of long bones, although any bone may be involved.
Frequently, the first symptoms are pain and swelling.
Twenty percent of ES at diagnosis have radiographic evidence of metastasis. Lungs and bone are the main metastatic locations.
Radiologically, an aggressive osteolytic lesion is commonly observed.
ES is characterized by a morphologically uniform round cell proliferation with round nuclei containing fine chromatin.
CD99 is expressed in nearly all ES and thereby is a highly sensitive immunohistochemical marker.
Several studies have confirmed a characteristic 11, 22 (q24, q12) chromosomal translocation in 85 percent of the cases ; the translocation t (21, 22) and three even rarer translocations (t (7, 22), t(2, 22) t(17, 22)) have also been found.
Unusual metastasic locations have been described, for example, the breast , myocardial muscle , paranasal sinuses , iris ), and pancreas . That explains why, even if a second primary cannot be completely excluded in our case, the probability of a metastasis is much higher. To our knowledge, this is the first case ever reported of a vaginal Ewing metastasis.
This case exemplifies the idea that every new lesion in a patient with Ewing sarcoma should be considered as a possible metastasis.
- Dahin's Bone Tumors. Lippincott. 18: 249-6,Google Scholar
- Bacci G, Ferrari S, Rosito P, Avella M, Barbieri E, Picci P, Battistini A, Brach del Prever A: Minerva Pediatr. Ewing's sarcoma of the bone. Anatomoclinical study of 424 cases. 1992, 44 (7-8): 345-59.Google Scholar
- Zoubek A, Kovar H, Gadner H: Cytogenetic and molecular genetic changes in malignant primary bone tumors] Radiologe. 1998, 38 (6): 467-71.Google Scholar
- Picci P, Böhling T, Bacci G, Ferrari S, Sangiorgi L, Mercuri M, Ruggieri P, Manfrini M, Ferraro A, Casadei R, Benassi MS, Mancini AF, Rosito P, Cazzola A, Barbieri E, Tienghi A, Brach del Prever A, Comandone A, Bacchini P, Bertoni F: Chemotherapy-induced tumor necrosis as a prognostic factor in localized Ewing's sarcoma of the extremities. J Clin Oncol. 1997, 15 (4): 1553-9.PubMedGoogle Scholar
- Ferrari S, Sundby Hall K, Luksch R, Tienghi A, Wiebe T, Fagioli F, Alvegard TA, Brach Del Prever A, Tamburini A, Alberghini M, Gandola L, Mercuri M, Capanna R, Mapelli S, Prete A, Carli M, Picci P, Barbieri E, Bacci G, Smeland S: Nonmetastatic Ewing family tumors: high-dose chemotherapy with stem cell rescue in poor responder patients. Results of the Italian Sarcoma Group/Scandinavian Sarcoma Group III protocol. Ann Oncol. 2010, ,Google Scholar
- McCluggage WG, Sumathi VP, Nucci MR, Hirsch M, Dal Cin P, Wells M, Flanagan AM, Fisher C: Ewing family of tumours involving the vulva and vagina: report of a series of four cases. J Clin Pqthol. 2007, 60 (6): 674-80. 10.1136/jcp.2006.040931.View ArticleGoogle Scholar
- Raney RB, Asmar L, Newton WA, Bagwell C, Breneman JC, Crist W, Gehan EA, Webber B, Wharam M, Wienes ES, Anderson JR, Maurer HM: Ewing's sarcoma of soft tissue in childhood: a report of the Intergroup Rhabdomyosarcoma study, 1972 to 1991. J Clin Oncol. 1997, 15 (2): 574-82.PubMedGoogle Scholar
- Astudillo L, Lacroix-triki M, Ferron G, Rolland F, Maisongrosse V, Chevreau C: Bilateral breast metastases from Ewing sarcoma of the femur. Am I clin Oncol. 2005, 28 (1): 102-3. 10.1097/01.coc.0000149741.46332.17.View ArticleGoogle Scholar
- Larbre F, Elbaz N, Verney R, Gilly J, Rousson R: Acute cardiac failure caused by myocardial metastasis of an unrecognised Ewing sarcoma. Pediatrie. 1981, 36 (2): 135-40.PubMedGoogle Scholar
- Gaba RC, Cousins JP, Basil IS, Shadid H, Valyi-Nagy T, Mafee MF: Metastatic Ewing sarcoma masquerading as olfactory neuroblastoma. Eur Arch Otorhinolaryngol. 2006, 263 (10): 960-2. Epub 2006 Jun 27, 10.1007/s00405-006-0089-8View ArticlePubMedGoogle Scholar
- Gündüz K, Shields JA, Shields CL, De Potter P, Wayner MJ: Ewing sarcoma metastatic to the iris. Am J Ophthalmol. 1997, 124 (4): 550-2.View ArticlePubMedGoogle Scholar
- Mulligan ME, Fellows DW, Mullen SE: Pancreatic metastasis from Ewing's sarcoma. Clin Imaging. 1997, 21 (1): 23-6. 10.1016/0899-7071(95)00064-XView ArticlePubMedGoogle Scholar
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