Primary Ewing’s Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) of the Penis

Ma, Zheng; Brimo, Fadi; Zeizafoun, Nebras

doi:10.1177/1066896912454565

Cited by 12 publications

(3 citation statements)

References 19 publications

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“…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). Due to the lack of specific clinical, radiological features and the rarity of the condition, it can easily be misdiagnosed by surgeons and radiologists.…”

Section: Discussionmentioning

confidence: 99%

“…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). However, none of those reported cases was accompanied by multiple metastatic lesions, especially osseous metastases.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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“…The most common soft tissue sites are the chest wall, lower extremities, and pelvis/hip region. They are rarely found in the retroperitoneum, upper extremities, or internal organs ( 2 ) . Patients often present with a painless mass or vague abdominal or chest pain, depending on tumor site ( 1 ) .…”

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confidence: 99%