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Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor

Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor 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. Keywords: Ewing sarcoma, vaginal metastasis Introduction confirmed by FISH analysis, which demonstrated the Ewing sarcoma (ES) is a small blue round cell tumor translocation t (11, 22). belonging to the Ewing Family Tumour (EFT) together Staging revealed a solitary non-specific pulmonary with Primary Neuroectodermal Tumour (PNET) and nodule of the inferior right lobe that did not change ASKIN tumor (of the thoracic wall). Eight hundred and with time and was not considered metastatic. ninety six cases have been reported in our institute The patient underwent neoadjuvant chemotherapy and since 1982. Ewing sarcoma has a distinct predilection resection and allograft of the distal radius. She was con- for males and occurs in the first two decades of life in sidered as poor responder as macroscopic areas more than 75 percent of cases [1,2]. remained on the surgical specimen, but all margins were Metastases are frequent [3] and are mostly pulmonary free of disease. High dose chemotherapy was then and osseous, but can be found in various other performed. locations. After completion of her treatment, it was followed up We present here the first description of a vaginal as per protocol and remained disease-free. metastasis of Ewing sarcoma. Five years later, during a routine gynaecologic exam, a vaginal polyp was found and removed. Histology Case Report revealed a ES metastasis (Figure 4), as confirmed by the A 35 year-old woman with no significant medical his- characteristic translocation t(11, 22).(Figure 5). The rest tory presented with pain and swelling of the left wrist of the evaluation (CT and bone scintigraphy) was over the past year. A radiograph and computed tomo- normal. graphy scan revealed a lytic lesion of the distal left No treatment has been undertaken. radius (Figure 1), with soft tissue extension on MR examination (Figure 2, 3). Discussion A biopsy was performed and histological examination Ewing sarcoma (ES) represents approximately ten per- revealed a typical Ewing sarcoma. The diagnosis was cent[1] 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 * Correspondence: daniel.vanel@ior.it 1 bones, although any bone may be involved. Departement of Pathology, the Rizzoli Institute, Via del Barbiano 1/10, 40106, Bologna, Italy Frequently, the first symptoms are pain and swelling. Full list of author information is available at the end of the article © 2011 Vanel et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Vanel et al. Clinical Sarcoma Research 2011, 1:9 Page 2 of 3 http://www.clinicalsarcomaresearch.com/content/1/1/9 Figure 1 Initial evaluation. CT: lytic lesion with partial cortical destruction. 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 com- monly observed. ES is characterized by a morphologically uniform Figure 3 Sagital T1W MR image, after contrast medium round cell proliferation with round nuclei containing injection. Medullary and soft tissue extensions are well evaluated. fine chromatin. CD99 is expressed in nearly all ES and thereby is a Several studies have confirmed a characteristic 11, 22 highly sensitive immunohistochemical marker. (q24, q12) chromosomal translocation in 85 percent of the cases [3]; the translocation t (21, 22) and three even rarer translocations (t (7, 22), t(2, 22) t(17, 22)) have also been found. Necrosis has a strong prognostic value [4]. High dose chemotherapy is used in poor responders [5]. Figure 2 Axial T1W MR image after contrast medium injection: Figure 4 The metastasis is made of homogeneous small round the soft tissue extension is well studied. cells. Vanel et al. Clinical Sarcoma Research 2011, 1:9 Page 3 of 3 http://www.clinicalsarcomaresearch.com/content/1/1/9 2. 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. 3. Zoubek A, Kovar H, Gadner H: Cytogenetic and molecular genetic changes in malignant primary bone tumors] Radiologe. 1998, 38(6):467-71. 4. 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. 5. 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. 6. McCluggage WG, Sumathi VP, Nucci MR, Hirsch M, Dal Cin P, Wells M, Figure 5 Interphase FISH with the EWSR1 (22q12) break-apart Flanagan AM, Fisher C: Ewing family of tumours involving the vulva and probe. Within a single nucleus fused red/green signals mark one vagina: report of a series of four cases. J Clin Pqthol 2007, 60(6):674-80. intact 22q12 region, whereas split red/green signals indicate the 7. Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, presence of an EWSR1 gene rearrangement. 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. 8. Astudillo L, Lacroix-triki M, Ferron G, Rolland F, Maisongrosse V, Chevreau C: We found 17 cases of primary ES involving the vagina Bilateral breast metastases from Ewing sarcoma of the femur. Am I clin and/or vulva in the literature[6]. A few cases of primary Oncol 2005, 28(1):102-3. 9. Larbre F, Elbaz N, Verney R, Gilly J, Rousson R: Acute cardiac failure caused neuro ectodermal tumors (PNET) in the pelvis have also by myocardial metastasis of an unrecognised Ewing sarcoma. Pediatrie been reported [7]. 1981, 36(2):135-40. Unusual metastasic locations have been described, for 10. Gaba RC, Cousins JP, Basil IS, Shadid H, Valyi-Nagy T, Mafee MF: Metastatic Ewing sarcoma masquerading as olfactory neuroblastoma. Eur Arch example, the breast [8], myocardial muscle [9], paranasal Otorhinolaryngol 2006, 263(10):960-2, Epub 2006 Jun 27. sinuses [10], iris [11]), and pancreas [12]. That explains 11. Gündüz K, Shields JA, Shields CL, De Potter P, Wayner MJ: Ewing sarcoma why, even if a second primary cannot be completely metastatic to the iris. Am J Ophthalmol 1997, 124(4):550-2. 12. Mulligan ME, Fellows DW, Mullen SE: Pancreatic metastasis from Ewing’s excluded in our case, the probability of a metastasis is sarcoma. Clin Imaging 1997, 21(1):23-6. much higher. To our knowledge, this is the first case ever reported of a vaginal Ewing metastasis. doi:10.1186/2045-3329-1-9 Cite this article as: Vanel et al.: Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor. Clinical Sarcoma Research Conclusion 2011 1:9. This case exemplifies the idea that every new lesion in a patient with Ewing sarcoma should be considered as a possible metastasis. Author details Departement of Pathology, the Rizzoli Institute, Via del Barbiano 1/10, 40106, Bologna, Italy. Departement of Nuclear Medicine, San Orsola Hospital, Via Giuseppe Massarenti, 940138 Bologna, Italy. Authors’ contributions NV wrote the article, VV checked the case, JK corrected the writing (English and scientific content) MG checked and selected the histology, SC checked and selected the FISH, CT checked the scientific content, DV proposed the Submit your next manuscript to BioMed Central subject and directed the article. All authors read and approved the final and take full advantage of: manuscript • Convenient online submission Competing interests The authors declare that they have no competing interests. • Thorough peer review • No space constraints or color figure charges Received: 13 April 2011 Accepted: 1 August 2011 Published: 1 August 2011 • Immediate publication on acceptance • Inclusion in PubMed, CAS, Scopus and Google Scholar References • Research which is freely available for redistribution 1. Dahin’s Bone Tumors. Lippincott ,618:249. Submit your manuscript at www.biomedcentral.com/submit http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Clinical Sarcoma Research Springer Journals

Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor

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Springer Journals
Copyright
Copyright © 2011 by Vanel et al; licensee BioMed Central Ltd.
Subject
Biomedicine; Cancer Research; Oncology; Surgical Oncology
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2045-3329
DOI
10.1186/2045-3329-1-9
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22612902
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Abstract

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. Keywords: Ewing sarcoma, vaginal metastasis Introduction confirmed by FISH analysis, which demonstrated the Ewing sarcoma (ES) is a small blue round cell tumor translocation t (11, 22). belonging to the Ewing Family Tumour (EFT) together Staging revealed a solitary non-specific pulmonary with Primary Neuroectodermal Tumour (PNET) and nodule of the inferior right lobe that did not change ASKIN tumor (of the thoracic wall). Eight hundred and with time and was not considered metastatic. ninety six cases have been reported in our institute The patient underwent neoadjuvant chemotherapy and since 1982. Ewing sarcoma has a distinct predilection resection and allograft of the distal radius. She was con- for males and occurs in the first two decades of life in sidered as poor responder as macroscopic areas more than 75 percent of cases [1,2]. remained on the surgical specimen, but all margins were Metastases are frequent [3] and are mostly pulmonary free of disease. High dose chemotherapy was then and osseous, but can be found in various other performed. locations. After completion of her treatment, it was followed up We present here the first description of a vaginal as per protocol and remained disease-free. metastasis of Ewing sarcoma. Five years later, during a routine gynaecologic exam, a vaginal polyp was found and removed. Histology Case Report revealed a ES metastasis (Figure 4), as confirmed by the A 35 year-old woman with no significant medical his- characteristic translocation t(11, 22).(Figure 5). The rest tory presented with pain and swelling of the left wrist of the evaluation (CT and bone scintigraphy) was over the past year. A radiograph and computed tomo- normal. graphy scan revealed a lytic lesion of the distal left No treatment has been undertaken. radius (Figure 1), with soft tissue extension on MR examination (Figure 2, 3). Discussion A biopsy was performed and histological examination Ewing sarcoma (ES) represents approximately ten per- revealed a typical Ewing sarcoma. The diagnosis was cent[1] 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 * Correspondence: daniel.vanel@ior.it 1 bones, although any bone may be involved. Departement of Pathology, the Rizzoli Institute, Via del Barbiano 1/10, 40106, Bologna, Italy Frequently, the first symptoms are pain and swelling. Full list of author information is available at the end of the article © 2011 Vanel et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Vanel et al. Clinical Sarcoma Research 2011, 1:9 Page 2 of 3 http://www.clinicalsarcomaresearch.com/content/1/1/9 Figure 1 Initial evaluation. CT: lytic lesion with partial cortical destruction. 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 com- monly observed. ES is characterized by a morphologically uniform Figure 3 Sagital T1W MR image, after contrast medium round cell proliferation with round nuclei containing injection. Medullary and soft tissue extensions are well evaluated. fine chromatin. CD99 is expressed in nearly all ES and thereby is a Several studies have confirmed a characteristic 11, 22 highly sensitive immunohistochemical marker. (q24, q12) chromosomal translocation in 85 percent of the cases [3]; the translocation t (21, 22) and three even rarer translocations (t (7, 22), t(2, 22) t(17, 22)) have also been found. Necrosis has a strong prognostic value [4]. High dose chemotherapy is used in poor responders [5]. Figure 2 Axial T1W MR image after contrast medium injection: Figure 4 The metastasis is made of homogeneous small round the soft tissue extension is well studied. cells. Vanel et al. Clinical Sarcoma Research 2011, 1:9 Page 3 of 3 http://www.clinicalsarcomaresearch.com/content/1/1/9 2. 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. 3. Zoubek A, Kovar H, Gadner H: Cytogenetic and molecular genetic changes in malignant primary bone tumors] Radiologe. 1998, 38(6):467-71. 4. 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. 5. 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. 6. McCluggage WG, Sumathi VP, Nucci MR, Hirsch M, Dal Cin P, Wells M, Figure 5 Interphase FISH with the EWSR1 (22q12) break-apart Flanagan AM, Fisher C: Ewing family of tumours involving the vulva and probe. Within a single nucleus fused red/green signals mark one vagina: report of a series of four cases. J Clin Pqthol 2007, 60(6):674-80. intact 22q12 region, whereas split red/green signals indicate the 7. Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, presence of an EWSR1 gene rearrangement. 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. 8. Astudillo L, Lacroix-triki M, Ferron G, Rolland F, Maisongrosse V, Chevreau C: We found 17 cases of primary ES involving the vagina Bilateral breast metastases from Ewing sarcoma of the femur. Am I clin and/or vulva in the literature[6]. A few cases of primary Oncol 2005, 28(1):102-3. 9. Larbre F, Elbaz N, Verney R, Gilly J, Rousson R: Acute cardiac failure caused neuro ectodermal tumors (PNET) in the pelvis have also by myocardial metastasis of an unrecognised Ewing sarcoma. Pediatrie been reported [7]. 1981, 36(2):135-40. Unusual metastasic locations have been described, for 10. Gaba RC, Cousins JP, Basil IS, Shadid H, Valyi-Nagy T, Mafee MF: Metastatic Ewing sarcoma masquerading as olfactory neuroblastoma. Eur Arch example, the breast [8], myocardial muscle [9], paranasal Otorhinolaryngol 2006, 263(10):960-2, Epub 2006 Jun 27. sinuses [10], iris [11]), and pancreas [12]. That explains 11. Gündüz K, Shields JA, Shields CL, De Potter P, Wayner MJ: Ewing sarcoma why, even if a second primary cannot be completely metastatic to the iris. Am J Ophthalmol 1997, 124(4):550-2. 12. Mulligan ME, Fellows DW, Mullen SE: Pancreatic metastasis from Ewing’s excluded in our case, the probability of a metastasis is sarcoma. Clin Imaging 1997, 21(1):23-6. much higher. To our knowledge, this is the first case ever reported of a vaginal Ewing metastasis. doi:10.1186/2045-3329-1-9 Cite this article as: Vanel et al.: Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor. Clinical Sarcoma Research Conclusion 2011 1:9. This case exemplifies the idea that every new lesion in a patient with Ewing sarcoma should be considered as a possible metastasis. Author details Departement of Pathology, the Rizzoli Institute, Via del Barbiano 1/10, 40106, Bologna, Italy. Departement of Nuclear Medicine, San Orsola Hospital, Via Giuseppe Massarenti, 940138 Bologna, Italy. Authors’ contributions NV wrote the article, VV checked the case, JK corrected the writing (English and scientific content) MG checked and selected the histology, SC checked and selected the FISH, CT checked the scientific content, DV proposed the Submit your next manuscript to BioMed Central subject and directed the article. All authors read and approved the final and take full advantage of: manuscript • Convenient online submission Competing interests The authors declare that they have no competing interests. • Thorough peer review • No space constraints or color figure charges Received: 13 April 2011 Accepted: 1 August 2011 Published: 1 August 2011 • Immediate publication on acceptance • Inclusion in PubMed, CAS, Scopus and Google Scholar References • Research which is freely available for redistribution 1. Dahin’s Bone Tumors. Lippincott ,618:249. Submit your manuscript at www.biomedcentral.com/submit

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Clinical Sarcoma Research – Springer Journals

Published: Aug 1, 2011

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