Pediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature

Buffoni, Isabella; Nuri, Halkawt; Pomè, Giuseppe; Sementa, Angela Rita; Stagnaro, Nicola; Barra, Salvina; Manzitti, Carla; Garaventa, Alberto

doi:10.1097/mph.0000000000001622

Cited by 5 publications

(6 citation statements)

References 19 publications

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“…Only one patient received radiotherapy along with pre- and post-surgical chemotherapy. 14 Of 11 other cases, 5 of them had just tumor resection, 15–19 3 had tumor resection with both chemotherapy and radiotherapy, 20–22 2 of them had cardiac transplantation, 23,24 and 1 had tumor resection with chemotherapy. 25…”

Section: Resultsmentioning

confidence: 99%

“…Only one patient received radiotherapy along with pre-and post-surgical chemotherapy. 14 Of 11 other cases, 5 of them had just tumor resection, [15][16][17][18][19] 3 had tumor resection with both chemotherapy and radiotherapy, [20][21][22] 2 of them had cardiac transplantation, 23,24 and 1 had tumor resection with chemotherapy. 25 In follow-up of different durations from the beginning to a maximum of 30 months, 53% of patients were alive (n = 9); three had no evidence of disease after 24 months (NED = 24), two had NED = 12, and four of them with NED = 30,86,2 months.…”

Section: Primary Pnetmentioning

confidence: 99%

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Primary and metastatic primitive neuroectodermal tumor of the heart: A systematic review

Hajsadeghi

Iranpour

Amouei

et al. 2022

Asian Cardiovasc Thorac Ann

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Objective Primary and metastatic primitive neuroectodermal tumors of the pericardium are uncommon. Two cases are presented and discussed. Methods The cases of a 17- and 38-year-old male patients with neuroectodermal tumors of the pericardium are presented. In addition, a systematic review was performed according to the Preferred Reporting Items and checklist for Systematic reviews and Meta-Analyses (PRISMA). All selected articles’ quality assessment was done using the Joanna Briggs Institute (JBI) Critical Appraisal Checklist for Case Reports. Results The systematic review yielded 29 patients with primary or metastatic PNET. Two patients underwent cardiac transplantation. It seems that unlike considering total resection in other organs, pericardiectomy in PNET patients with pericardial origin may lead to further invasion of the lesions, and it is not recommended. The average disease-free follow-up was 10 months. The combination of neoadjuvant and adjuvant chemotherapy along with surgery and radiotherapy offered the best disease-free outcomes. Conclusion Neuroectodermal heart tumors are rare, aggressive tumors requiring chemotherapy and radiotherapy in association with tumor resection surgery to have the best disease-free duration.

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Section: Resultsmentioning

confidence: 99%

Section: Primary Pnetmentioning

confidence: 99%

Primary and metastatic primitive neuroectodermal tumor of the heart: A systematic review

Hajsadeghi

Iranpour

Amouei

et al. 2022

Asian Cardiovasc Thorac Ann

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“…1 The most common sites of EES include extremities, chest wall, paravertebral space and retroperitoneum but rare anatomic locations have been reported such as jejunum, kidneys, heart, vulva and larynx. 2,3 EES in the pelvic region is also rare and here we represent a rare case of it.…”

Section: Introductionmentioning

confidence: 82%

Extraosseous Ewing Sarcoma in a Pelvic Region: A Case Report

2022

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Ewing sarcoma is the second most common malignant bone tumour in children. It rarely originates from extraskeletal soft tissue sites such as the upper thigh, buttocks, upper arm and shoulder. Primary extraosseous Ewing sarcoma located in the pelvic region is rare. We report a 17-year-female who had gradual onset of progressive lower abdominal mass and pain. A computed tomography scan revealed well defined lobulated heterogeneously enhancing lesion noted in the pelvic region measuring approximately 12.9 x 9.8 x 9.3 cm. Incisional biopsy showed a small round blue cell tumour which was strongly positive for Cluster of Differentiation 99, vimentin, Friend Leukaemia Integration 1 with 40% Ki-67. Following treatment with chemotherapy, surgery and radiotherapy, there was complete resolution of the tumour. Although extraosseous Ewing sarcoma is rare, it can occur virtually in any soft tissue site. Therefore, clinicians need to distinguish it from soft tissue sarcoma because rapid progression, early diagnosis and timely treatment are crucial for a favourable prognosis.

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“…As a subtype of Ewing sarcoma, EES is far less frequent than skeletal Ewing sarcoma (SES). EES may develop in soft tissues at virtually any anatomical location, but the main sites of involvement are the paravertebral region, the trunk, and the extremities ( 4 , 6 , 7 , 10 – 13 ). However, primitive localization in the penis is extremely rare; only a few relevant cases have been reported in the previous literature ( 8 , 9 , 14 , 15 ).…”

Section: Discussionmentioning

confidence: 99%

“…Classically, the lesions most commonly involve flat bones of the axial skeleton and the diaphysis of long bones, while 10-20% of cases originate from soft tissue sites (2). Extra-skeletal Ewing sarcoma (EES) is frequently located in the extremities, chest wall, paravertebral space, and retroperitoneum, but some rare anatomic locations have also been reported, such as the jejunum, heart, larynx, vulva, kidneys (3)(4)(5)(6)(7). To our knowledge, the occurrence of EES in the penis is extremely rare, and there are only eight relevant case reports that have been discussed (8,9).…”

Section: Introductionmentioning

confidence: 99%

Case Report: Primary Ewing Sarcoma of the Penis With Multiple Metastases

et al. 2021

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Background: Ewing sarcoma is the second most common malignant bone tumor in children, but it rarely originates from extra-skeletal sites. The commonly involved sites of soft tissue include paravertebral spaces, lower extremities, the pelvis, head, and neck, while primary extra-skeletal Ewing sarcoma (EES) located in the genitals is extremely rare.Case Presentation: We report a young patient who presented to our hospital with a painful erection of the penis and limited motion of the left hip. Magnetic resonance imaging showed a hyperintense mass with invasion of adjacent tissue in the penis and a heterogeneously high signal lesion in the left proximal femur. 18F-fluorodeoxyglucose positron-emission tomography detected widespread metastatic lesions in the bilateral lung and multiple skeletons. An incisional biopsy of the penis was performed; the histopathological findings and EWS gene translocation identified by molecular analysis confirmed the diagnosis of Ewing sarcoma. Subsequently, the punch-biopsy specimen from the left femur showed undifferentiated small round cells, a finding consistent with the microscopic presence of Ewing sarcoma metastasis. However, after the first course of multiagent chemotherapy, the penile mass did not obtain stabilization but instead grew progressively with surface ulceration and multidrug resistant bacteria infection. Despite receiving antibiotics and maximal supportive therapy, the patient died from sepsis and lung metastasis complications in the intensive care unit 2 months later.Conclusion: This case indicates that although EES as a subtype of Ewing sarcoma is rare, it can occur virtually in any soft tissue site, even in the genitals. Therefore, clinicians need to distinguish this entity from other soft tissue sarcomas with rapid progression since early diagnosis and timely treatment of EES are pivotal for a favorable prognosis.

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Pediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature

Abstract: Extraosseous Ewing sarcoma of primary cardiac origin is an extremely rare variety among pediatric cardiac neoplasms. We report a case of extraosseous Ewing sarcoma of primary cardiac origin in a 9-year-old girl, treated with debulking surgery, adjuvant chemotherapy, and radiotherapy.

Cited by 5 publications

References 19 publications

Primary and metastatic primitive neuroectodermal tumor of the heart: A systematic review

Primary and metastatic primitive neuroectodermal tumor of the heart: A systematic review

Extraosseous Ewing Sarcoma in a Pelvic Region: A Case Report

Case Report: Primary Ewing Sarcoma of the Penis With Multiple Metastases

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