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Simões AM, Sequeira-Mendes J, Santos AI. Beyond the Bones: A Case Report of Extraosseous Ewing Sarcoma. Cureus 2023; 15:e47961. [PMID: 38034204 PMCID: PMC10685669 DOI: 10.7759/cureus.47961] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/30/2023] [Indexed: 12/02/2023] Open
Abstract
Ewing sarcoma (ES) is primarily recognized as a primary bone tumor; however, its extraosseous variant is exceptionally rare and presents unique clinical challenges. In this article, we report the case of a 22-year-old male who initially presented with abdominal swelling. Diagnostic tests included abdominal imaging and a CT scan, revealing a solid liver mass. A thorough evaluation confirmed it to be an extraosseous ES, supported by liver biopsy and immunohistochemistry demonstrating positive expression for AE1/AE3 and CD-99, along with genetic analysis revealing a rearrangement of the EWSR1 gene (translocation 22q12). The patient's treatment involved a multimodal approach, including perioperative chemotherapy, surgery, and postoperative chemotherapy, following which the patient remained in complete remission after 24 months. This case emphasizes the importance of considering rare malignancies such as ES in differential diagnoses for young patients with liver masses. It also accentuates the pivotal role of family physicians in early detection and holistic patient care, underscoring the need for comprehensive investigations when encountering persistent symptoms.
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Affiliation(s)
- Ana Margarida Simões
- Family Medicine, Unidade de Saúde Familiar A Ribeirinha, Unidade Local de Saúde Guarda, Guarda, PRT
| | - Joana Sequeira-Mendes
- Family Medicine, Unidade de Saúde Familiar A Ribeirinha, Unidade Local de Saúde Guarda, Guarda, PRT
| | - Ana Isabel Santos
- Family Medicine, Unidade de Saúde Familiar A Ribeirinha, Unidade Local de Saúde Guarda, Guarda, PRT
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Wu SY, Hsu CK, Yue CT, Tsai YC. Large retroperitoneal extraskeletal Ewing's sarcoma with renal pedicle invasion: a case report. BMC Urol 2023; 23:95. [PMID: 37194057 DOI: 10.1186/s12894-023-01272-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2023] [Accepted: 05/11/2023] [Indexed: 05/18/2023] Open
Abstract
BACKGROUND Extraskeletal Ewing's sarcoma (EES) is a rare malignant tumor primarily found in children and young adults. Localized disease can present with nonspecific symptoms such as local mass, regional pain, and increased skin temperature. More severe cases may present with systemic symptoms such as malaise, weakness, fever, anemia, and weight loss. Among these lesions, retroperitoneal sarcomas are relatively uncommon and difficult to diagnose. Since they are usually asymptomatic until large enough to compress or invade the surrounding tissues, most are already advanced at first detection. Traditionally, the treatment of choice is complete surgical resection, sometimes combined with postoperative radiotherapy and chemotherapy. We report a case of EES with left renal artery invasion in the left retroperitoneal cavity successfully treated with transarterial embolization and surgery. CASE PRESENTATION A 57-year-old woman with a negative family history of cancer presented at our Urology Department with a large left retroperitoneal tumor found by magnetic resonance imaging during the health exam. Physical examination showed a soft abdomen and no palpable mass or tenderness. Imaging studies showed that the tumor covered the entire left renal pedicle, but the left kidney, left adrenal gland, and pancreas appeared tumor free. Since the tumor tightly covered the entire renal pedicle, tumor excision with radical nephrectomy was advised. The patient underwent transarterial embolization of the left renal artery with 10 mg of Gelfoam pieces daily before surgical excision. Tumor excision and left radical nephrectomy were uneventful the day after embolization. Post-operatively, the patient recovered well and was discharged on day 10. The final histopathological analysis showed a round blue cell tumor consistent with an Ewing sarcoma, and the surgical margins were tumor free. CONCLUSIONS Retroperitoneal malignancies are rare but usually severe conditions. Our case report showed that retroperitoneal EES with renal artery invasion could be treated safely with transarterial embolization and surgery.
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Affiliation(s)
- Shu-Yu Wu
- Department of Urology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, No. 289, Jianguo Rd., Xindian Dist, New Taipei City, 231, Taiwan
- Department of Urology, School of medicine, Tzu Chi University, Hualien, Taiwan
| | - Chun-Kai Hsu
- Department of Urology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, No. 289, Jianguo Rd., Xindian Dist, New Taipei City, 231, Taiwan
- Department of Urology, School of medicine, Tzu Chi University, Hualien, Taiwan
| | - Chung-Tai Yue
- Department of Pathology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan
| | - Yao-Chou Tsai
- Department of Urology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, No. 289, Jianguo Rd., Xindian Dist, New Taipei City, 231, Taiwan.
- Department of Urology, School of medicine, Tzu Chi University, Hualien, Taiwan.
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Hedfi M, Ben Ismail I, Zenaidi H, Bouslama S, Zoghlami A. Primary Ewing sarcoma of the liver: Diagnosis, management, and prognosis: A case report and literature review. Clin Case Rep 2022; 10:e6508. [PMID: 36381031 PMCID: PMC9647335 DOI: 10.1002/ccr3.6508] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2022] [Revised: 09/04/2022] [Accepted: 10/08/2022] [Indexed: 11/11/2022] Open
Abstract
Ewing's sarcoma tumors (ES) are a rare entity exceptionally localized on the liver. We report a case of an ES of the liver in a 26-year-old man who presented with abdominal pain. The diagnosis was confirmed with a histopathological examination of the left hepatectomy specimen and adjuvant chemotherapy was received.
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Affiliation(s)
- Mohamed Hedfi
- Department of General Surgery, Zaghouan Regional HospitalUniversity of Tunis El ManarTunisTunisia
| | - Imen Ben Ismail
- Department of General Surgery, Trauma and Burns CenterUniversity of Tunis El ManarTunisTunisia
| | - Hakim Zenaidi
- Department of General Surgery, Zaghouan Regional HospitalUniversity of Tunis El ManarTunisTunisia
| | - Sirine Bouslama
- Department of Pathology, Zaghouan Regional HospitalUniversity of Tunis El ManarTunisTunisia
| | - Ayoub Zoghlami
- Department of General Surgery, Trauma and Burns CenterUniversity of Tunis El ManarTunisTunisia
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Shrateh ON, Jobran AW, Owienah H, Sweileh T, Abulihya M, Natsheh MA, Abu-Dayyah N. Primary Extraskeletal Ewing sarcoma of the foot with extensive skeletal and pulmonary metastasis: A rare case report. Ann Med Surg (Lond) 2022; 82:104752. [PMID: 36268294 PMCID: PMC9577863 DOI: 10.1016/j.amsu.2022.104752] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/27/2022] [Revised: 09/12/2022] [Accepted: 09/18/2022] [Indexed: 11/16/2022] Open
Affiliation(s)
- Oadi N. Shrateh
- Al-Quds University-School of Medicine, Abu-Dis, East Jerusalem, Palestine
- Corresponding author. Ramallah, Palestine.
| | - Afnan W.M. Jobran
- Al-Quds University-School of Medicine, Abu-Dis, East Jerusalem, Palestine
| | - Haneen Owienah
- Radiology Department, Al-Istishari Arab Hospital, Ramallah, West Bank, Palestine
| | - Thaer Sweileh
- Radiology Department, Al-Istishari Arab Hospital, Ramallah, West Bank, Palestine
| | - Mohand Abulihya
- Pathology Department, Al-Istishari Arab Hospital, Ramallah, West Bank, Palestine
| | - Motaz A. Natsheh
- Pathology Department, Al-Istishari Arab Hospital, Ramallah, West Bank, Palestine
| | - Nazeeh Abu-Dayyah
- Orthopedic Surgery Department, Al-Istishari Arab Hospital, Ramallah, West Bank, Palestine
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Lu T, Yang W, Liu X, Yang X, Yang C, Di W. Imaging Findings of Hepatic Ewing's Sarcoma on Computed Tomography and Gadobenate Dimeglumine-enhanced Magnetic Resonance Imaging: A Case Report and Literature Review. J Clin Transl Hepatol 2022; 10:564-569. [PMID: 35836756 PMCID: PMC9240243 DOI: 10.14218/jcth.2021.00129] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/02/2021] [Revised: 07/02/2021] [Accepted: 07/22/2021] [Indexed: 12/04/2022] Open
Abstract
Ewing's sarcoma (ES) is a tumor that often occurs in the long bones and rarely arises from visceral organs primarily. Here, we report a case of primary hepatic ES, discuss its computed tomography (CT) and gadobenate dimeglumine-enhanced magnetic resonance (MRI) features. This is the first Chinese and fifth primary hepatic ES case reported, based on a literature review. Imaging examinations showed that the tumor was solid, with necrosis and hemorrhage. Contrast-enhanced images showed that the tumor was hypervascular and especially had heterogeneous signal intensity on hepatobiliary phase MRI images. Intratumoral vessels and vascular invasion were also present.
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Affiliation(s)
- Tao Lu
- Department of Radiology, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan, China
| | - Wenhao Yang
- Organ Transplantation Center, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan, China
- Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, Sichuan, China
| | - Xingchao Liu
- Organ Transplantation Center, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan, China
- Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, Sichuan, China
| | - Xudan Yang
- Department of Pathology, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan, China
| | - Chong Yang
- Organ Transplantation Center, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan, China
- Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, Sichuan, China
- Correspondence to: Wenjia Di and Chong Yang, Organ Transplantation Center, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan 611731, China. ORCID: https://orcid.org/0000-0002-0060-706X (CY). Tel: +86-28-8739-3707, Fax: +86-28-8778-5585, E-mail: (WD), (CY)
| | - Wenjia Di
- Organ Transplantation Center, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan, China
- Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, Chengdu, Sichuan, China
- Correspondence to: Wenjia Di and Chong Yang, Organ Transplantation Center, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, Sichuan 611731, China. ORCID: https://orcid.org/0000-0002-0060-706X (CY). Tel: +86-28-8739-3707, Fax: +86-28-8778-5585, E-mail: (WD), (CY)
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Nedham FN, Nagaraj V, Darwish A, Al-Abbasi TA. Retroperitoneal blue cell round tumor (Ewing sarcoma in a 35 years old male)- case report. Int J Surg Case Rep 2022; 94:107045. [PMID: 35658275 PMCID: PMC9065731 DOI: 10.1016/j.ijscr.2022.107045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2022] [Revised: 03/26/2022] [Accepted: 04/05/2022] [Indexed: 11/17/2022] Open
Abstract
Introduction Ewing sarcoma is a highly aggressive tumor. It's common presentation is primary bone tumor, but very rarely as soft tissue sarcoma both of which are under the spectrum of Ewing sarcoma family of tumors (EFT). Case presentation A 35 years old male who presented with advanced locally spreading tumor. He was diagnosed after presenting with epigastric pain and melena, CT abdomen revealed the retroperitoneal mass. CT guided core biopsy taken and was diagnosed as Ewing sarcoma. The patient presented with mass already grown so he was not a surgical candidate. Patient was managed symptomatically till he was sent for palliative care in a Sarcoma centre in Jordon and passed away on October 2021. Discussion Ewing Sarcoma family of tumors are group of small round blue cell tumors that are histogenetically related. The better known is Ewing ‘s sarcoma (EWS). It is a malignant small blue round cell tumor with variable degree of neuroectodermal differentiation. Retroperitoneal tumor are mostly malignant and accounts for one third of soft tissue sarcoma. They usually present as large masses at the time of the diagnosis. So they do not produce symptoms until they grow large enough to compress or invade contagious structures. Conclusion ES-EWS is an aggressive tumor with high incidence of local recurrence and distant metastasis that's why is was given its poor prognosis characteristic. Multimodality treatment including surgical resection, chemotherapy and High dose radiotherapy will help in better survival rate.
Extraskeletal Ewing sarcoma is an aggreesive tumor with high incidence of local recurrence and distant metastasis. They usully present as large masses at time of the disgnosis so they do not produce symptoms unitil they grow large to compress on adjacent organs Multimodality treatment is recommended in terms of surgical resection, chemotherapy and high dose radiotherapy. Early diagnosis of such rare tumor will help targeting it at early stage [1], [2], [3], [4]
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Affiliation(s)
| | - Veena Nagaraj
- Department of Pathology, BDF Royal Medical Services, Bahrain
| | - Abdulla Darwish
- Consultant Pathology, Department of Pathology, BDF Royal Medical Services, Bahrain.
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Shadhu K, Ramlagun-Mungur D, Ping XC. Ewing sarcoma of the jejunum: A case report and literature review. World J Gastrointest Surg 2021; 13:507-515. [PMID: 34122739 PMCID: PMC8167841 DOI: 10.4240/wjgs.v13.i5.507] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/04/2020] [Revised: 04/22/2021] [Accepted: 04/29/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Ewing sarcomas (ESs) are highly aggressive malignancy and are predominant in the long bones of extremities of children and young adults with a slight male predilection and rarely presents at extra skeletal locations.
CASE SUMMARY A 55-year-old woman came to our hospital after finding elevated tumor biomarkers during her physical examination. Her enhanced computed tomography scan showed a jejunal mass. The patient underwent laparoscopic enterectomy. The mass was later diagnosed as ES, evidenced by fluorescence in situ hybridization whereby the GLP ES breakpoint region 1 probe was used, showing that more than 10% of the cells showed a red-green-yellow signal proving the breakpoint rearrangement of the ES breakpoint region 1 gene in chromosome 22.
CONCLUSION We describe a case of localized ES at the jejunum in China based on the literature.
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Affiliation(s)
- Kamleshsingh Shadhu
- Department of General Surgery, Gastrointestinal Surgery, The First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, Jiangsu Province, China
- Pre-registration House Officer, Medical Council of Mauritius, Floreal 0000, Plaine Whilhems, Mauritius
| | - Dadhija Ramlagun-Mungur
- Department of General Surgery, Gastrointestinal Surgery, The First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, Jiangsu Province, China
- Pre-registration House Officer, Medical Council of Mauritius, Floreal 0000, Plaine Whilhems, Mauritius
| | - Xiao-Chun Ping
- Department of General Surgery, Gastrointestinal Surgery, The First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, Jiangsu Province, China
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Cantu C, Bressler E, Dermawan J, Paral K. Extraskeletal Ewing Sarcoma of the Jejunum: A Case Report. Perm J 2019; 23:18-255. [PMID: 31314729 DOI: 10.7812/tpp/18-255] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
INTRODUCTION Ewing sarcoma most commonly arises in bones but rarely presents in extraskeletal locations. We report one such case arising from the jejunum. CASE PRESENTATION A 67-year-old woman presented with acute-onset, right lower quadrant pain. Computed tomography results showed a large mass within the midjejunum with pneumoperitoneum. Surgical excision was performed, and an extraskeletal Ewing sarcoma of the jejunum was suspected histologically. The diagnosis was confirmed with fluorescence in situ hybridization studies. CONCLUSION This case emphasizes the importance of recognizing this rare presentation in the small intestine to broaden the differential diagnosis of adult intraabdominal tumors.
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Affiliation(s)
- Colby Cantu
- Department of Pathology, Duke University Health System, Durham, NC
| | | | | | - Kristen Paral
- Department of Pathology, Duke University Health System, Durham, NC
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Bong C, Thomson I, Lampe G. Case report and literature review of Ewing's sarcoma in the gastrointestinal tract. SURGICAL PRACTICE 2018; 22:84-92. [DOI: 10.1111/1744-1633.12292] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Affiliation(s)
- Christopher Bong
- Department of Upper Gastrointestinal Surgery and Soft Tissue; Princess Alexandra Hospital; Woolloongabba Queensland Australia
| | - Iain Thomson
- Department of Upper Gastrointestinal Surgery and Soft Tissue; Princess Alexandra Hospital; Woolloongabba Queensland Australia
| | - Guy Lampe
- Department of Anatomical Pathology; Princess Alexandra Hospital; Woolloongabba Queensland Australia
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Javalgi AP, Karigoudar MH, Palur K. Blue Cell Tumour at Unusual Site: Retropritoneal Ewings Sarcoma. J Clin Diagn Res 2016; 10:ED19-20. [PMID: 27190820 DOI: 10.7860/jcdr/2016/18302.7618] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2015] [Accepted: 02/08/2016] [Indexed: 11/24/2022]
Abstract
Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations. Haematological profile, renal function test and liver function test were in normal limits. USG abdomen was normal, MRI showed a mass in pelvis retroperitoneum measuring 10x10cms, bilateral ovaries and tubes were normal. Because of retroperitoneal nature of tumor and suspicion of uterine sarcoma, laparotomy was performed. The large retroperitoneal mass adherent to posterior of uterus was excised and send for histopathological diagnosis. On gross and microscopy examination the diagnosis of blue cell tumor with PAS positivity, possibility of extraskeletal Ewing's sarcoma/primitive neuro-ectodermal tumor was made which was further confirmed by immunohistochemistry, positive for S100, Vementin and CD99 and negative for desmin and CK. Confirmed diagnosis help in accurate management and improves survival rate.
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Affiliation(s)
- Anita P Javalgi
- Assistant Professor, Department of Pathology, Shri B M Patil Medical College , Sholapur Road Bijapur, Karnataka, India
| | - Mahesh H Karigoudar
- Professor, Department of Pathology, Shri B M Patil Medical College , Sholapur Road Bijapur, Karnataka, India
| | - Katyayani Palur
- Post Graduate, Department of Pathology, Shri B M Patil Medical College , Sholapur Road Bijapur, Karnataka, India
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