Extraskeletal Ewing Sarcoma Disguised as a Vascular Malformation.

Extraskeletal Ewing sarcoma (EES) is a rare entity, accounting for only 3% of lesions encountered in upper extremity. We present two paediatric patients, who were initially diagnosed with a vascular malformation based on clinical assessment and imaging. Final histopathology revealed Ewing sarcoma of soft tissue origin, confirmed by immunohistochemical analysis. Hand surgeons, who are routinely approached for a myriad of hand pathologies, should be wary and consider EES as a differential when treating such lesions. A multidisciplinary approach with an appropriate treatment algorithm can help in a speedy diagnosis, improving the long-term prognosis of the disease. Level of Evidence: Level V (Therapeutic).

Ewing Sarcoma of the Larynx with Thyroid Cartilage Destruction: A Case Report.

Ewing sarcoma of the larynx is extremely rare, only a few number of cases have been reported. In this report, we describe a case of extraskeletal Ewing sarcoma of the larynx with thyroid cartilage destruction. Laryngoscope, 2024.

Rare Presentation of Primary Pleural Ewing Sarcoma With a Mass Extending Into the Right Ventricle: A Case Report.

Primary pleural Ewing sarcoma is a rare type of Ewing sarcoma with only a few case reports identified in the literature. The condition is challenging to diagnose with deceiving symptoms and wide differential diagnosis. Diagnosis is confirmed with a combination of radiological and pathological assessment. Treatment is similar to other types of Ewing sarcoma with chemotherapy and surgery being the mainstay of treatment. We identify an unusual presentation of pleural Ewing sarcoma in a 31-year-old male with a mass extending into the right ventricular outlet causing rapid deterioration of the patient.

Extraskeletal Ewing's sarcoma presenting as a mediastinal mass with vena cava superior syndrome: A rare case report.

INTRODUCTION: Extraskeletal Ewing's Sarcoma is a rare entity of sarcoma that develops rapidly within soft tissue in any anatomic region, and the symptoms depend on its location. CASE PRESENTATION: The X-ray examination of a 28-year-old man with shortness of breath, cough, weight loss, and chest pain showed malignant round cell, in which confirmed by immunohistochemical examination. The examination indicated positive Vimentin findings in the cytoplasm and positive FLI-1 in the nuclei of the tumour cells. The diagnosis was consistent with extraskeletal Ewing's sarcoma. The patient submitted to a chest conference and received radiotherapy related to SVCS before debulking surgery. DISCUSSION: The diagnostic challenges associated with Ewing's sarcoma may arise due to its diverse histological spectrum. Further examination is required in order to distinguish Ewing's sarcoma from other tumours, as its radiological specificity is limited. A multimodal approach for treatment and therapy is necessary to highlight the specific requirements of the patient's condition. CONCLUSION: Imaging modalities including X-rays and thoracic CT scans, supported by histopathological examination and immunohistochemistry, are essential for accurately diagnosing Extraskeletal Ewing's sarcoma. A multimodal approach may be considered as the best treatment for the patient with mediastinal Ewing's sarcoma.

Unveiling the Unlikely: Extraskeletal Ewing Sarcoma Masquerading as Gastrointestinal Bleeding.

While Ewing sarcoma is traditionally a malignant tumor of bone, it may uncommonly present extra-skeletally, leading to an array of puzzling presentations depending on the tissue involved. Here, we describe the case of a 66-year-old man who presented to the primary care office for evaluation of intermittent melena. He ultimately underwent capsule endoscopy and developed a secondary small bowel obstruction, unveiling his neoplasm. The tumor was then resected and managed with surveillance only, and the patient remains without evidence of disease after four years of follow-up.

Extraskeletal Ewing's sarcoma of supraglottis: A rare case report.

Ewing's Sarcoma family of tumors is a group of small round tumor cells. Ewing's sarcoma majority occurs in bone, accounts about 10 % of primary bone tumors. Extraskeletal Ewing's sarcoma (ESS) is unusual and commonly seen in trunk, paravertebral, and chest wall region. It is rarely seen in head and neck region, accounting to 2-3 %. In head and neck region, ESS is seen in nasal or oral cavities, sinuses. EES originating in the larynx is very rare. Here, we report a 22 years old female having the complaints of change in voice and noisy breathing who was diagnosed as a case of EES of supraglottis. As the disease progressed during the time of diagnosis, she had to undergo emergency tracheostomy. The disease was inoperable so she received neoadjuvant chemotherapy followed by radiation followed by adjuvant chemotherapy. At present she is symptomatically better. The aim of this report is to put forward the rare site of Ewing's Sarcoma and highlighting the early diagnosis in suspected case with IHC, providing effective multimodality treatment.

Extraskeletal Ewing Sarcoma: A Case Report.

Ewing sarcoma is one of the most common primary bone tumors arising from neuroectodermal cells mainly presenting in the younger population. Instances of this highly malignant tumor manifesting outside of the bone and outside of the typical age range create an unfamiliar clinical scenario. In this report, we present a rare extraskeletal Ewing sarcoma in a 42-year-old woman with a subcutaneous soft tissue mass in the posterior chest displaying a positive EWSR1 gene rearrangement via fluorescence in situ hybridization. The patient is currently on a chemotherapy regimen showing favorable response to the tumor size despite additional complications. This overall presentation of Ewing sarcoma allows further understanding of the malignancy and fosters better care for future cases.

Case Report: Primary Ewing Sarcoma of the Penis.

Background: Ewing sarcoma is a malignancy that commonly affects the skeletal system and primary extraskeletal involvement is rare. Extraskeletal Ewing sarcoma (EES) arises in soft tissue anywhere in the body. These are very rarely seen aggressive tumours. There have been only 7 reported cases of EES of penis. Case Presentation: We report a 22-year-young patient who presented to our hospital with a ulcero-proliferative growth in the shaft of penis. There were no other complaints indicating any metastasis. Incisional biopsy was suggestive of invasive malignancy. He was scheduled for a partial penectomy. Final HPE and IHC were suggestive of EES. Conclusion: EES as a subtype of Ewing sarcoma is rare and it can occur in any soft tissue site. Hence, clinicians need to differentiate this entity from other soft tissue sarcomas. Early diagnosis and timely treatment of EES are pivotal for a favourable prognosis due to its aggressive nature. Supplementary Information: The online version contains supplementary material available at 10.1007/s13193-023-01793-x.

Surgical management of primary gastric Ewing-like sarcoma at the lesser curvature.

We report the case of a 65-year-old female who presented with a 6-month history of epigastric pain and dyspepsia. Computed tomography of the abdomen and pelvis showed an enhancing nodular lesion and an indeterminate 4 mm lymph node on the lesser curvature of the stomach raising concerns for gastric malignancy. Upper gastrointestinal endoscopy revealed a 10 cm malignant appearing lesion along the gastric lesser curvature. Histopathology demonstrated spindled and small round blue cell tumor with immunohistochemistry staining consistent with Ewing-like sarcoma. After multidisciplinary team discussion the patient was arranged for neoadjuvant chemotherapy with early re-imaging, followed by consideration of gastrectomy. This case highlights the unusual diagnosis of primary gastric Ewing-like sarcoma and the management of this rare condition.

Primary mediastinum Ewing's sarcoma with pleural effusion: A case report and literature review.

Ewing's sarcoma (ES) is an aggressive cancer in young adults. Primary ES occurring in the chest with pleural effusion is even rarer. We report the case of a 15-year-old girl who presented with intermittent chest pain occurring for more than 2 months and cough and wheezing for 10 days. Radiological imaging showed a large soft tissue mass with multiple small vessel shadows near the left mediastinum and bloody pleural effusion in the left thorax. ES was diagnosed by positive immunostaining for CD99, FLI-1, and NKX2 combined with fluorescence in situ hybridization detection of the EWSR1 gene arrangement. With chemotherapy, lung computed tomography revealed that the tumor had become much smaller, and the fluid was absorbed. We report a case of extraskeletal Ewing's sarcoma (EES) in the mediastinum with pleural effusion, which is unusual and challenging. EES is a highly malignant tumor with a poor prognosis. Early diagnosis and treatment can improve the survival rate of patients.

Large retroperitoneal extraskeletal Ewing's sarcoma with renal pedicle invasion: a case report.

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.

Extraskeletal Ewing's sarcoma of the head and neck region in pediatric patients: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Extraskeletal Ewing's sarcoma (EES) is a collection of malignant cells that appear small and round and occur mostly in pediatrics and adolescence. Head and neck EES tumors are considered rare and require multidisciplinary care to achieve ideal results in management. CASE PRESENTATION: A 14-year-old boy who complained of a mass protruding from the back of his neck which gradually increased in size in the last few months prior to the diagnosis. He was referred to a pediatric otolaryngology clinic with a one-year history of chronic painless nape swelling. Ultrasound prior to the referral was done and the findings revealed a well-defined rounded hypoechoic lesion with internal vascularity. MRI was done and the impression was a large subcutaneous, well defined enhancing soft tissue lesion which raised the suspicion of sarcoma. The multidisciplinary team decision was to go for complete resection with a free margin followed by chemoradiation postoperatively. No evidence of recurrence was detected throughout the follow-up. CLINICAL DISCUSSION: The literature review included ages of the pediatric group from 4 months up to 18-year-old. Clinical features are highly dependable on the size and site of the lesion. Complete resection of the tumor plays an important role in the local control and prognosis. CONCLUSION: We present a rare case of extraskeletal Ewing's sarcoma of the nape. Computed tomography and magnetic resonance imaging are frequently used as imaging modalities in evaluating and diagnosing EES. Management commonly includes surgery with adjuvant chemotherapy to decrease recurrence and prolong the survival rate.

Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child.

Primary extraskeletal Ewing sarcoma (EES) is a rare small round cell malignancy that accounts for less than 1% of all sarcomas. It is found most commonly in the trunk and lower limbs and very rarely in the pleura and can be easily misdiagnosed in clinical practice. This study presents the case of an 11-year-old boy who presented to our hospital with no apparent cause of left shoulder pain for 6 months. On physical examination, tenderness was noted in the left chest wall and shoulder joint, which had a limited range of motion. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest revealed an irregular soft tissue mass in the upper left thorax, with a wide base attached to the adjacent pleura and bone destruction of the adjacent left first rib. The patient's bone scan showed a dense focus of increased radiotracer accumulation in the left first rib. A subsequent CT-guided aspiration biopsy of the left pleural mass with histomorphology and immunohistochemical phenotyping led to a diagnosis of extraskeletal Ewing sarcoma. To inhibit tumor growth, alternating systemic chemotherapy cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) and isocyclophosphamide and etoposide (IE) were administered at 3-week intervals. After completing three VDC and two IE cycles, the child's condition was well and the pain in the left shoulder joint was relieved. However, a repeat MRI of the chest showed that the mass did not shrink.

A Case of Extraskeletal Ewing Sarcoma Treated With Wide Local Excision With Latissimus Dorsi Flap and Systemic Therapy.

Extraskeletal Ewing sarcoma (EES) is a rare tumor of the soft tissue that looks the same as skeletal Ewing sarcoma (ES). A male in his 50s was diagnosed with extraskeletal Ewing sarcoma (EES) of the right shoulder, which had infiltrated the muscles around the shoulder joints. Although uncommon, all members of the ES family of tumors, including EES, were treated following the same general protocol for sarcoma tumors. Due to the significant tumor size in this patient and local invasion, wide local excision and a latissimus dorsi flap were required. This case highlighted the management of EES, including the surgical removal of the mass on the right shoulder, followed by chemotherapy, which led to a successful outcome.

Extraskeletal Ewing sarcoma of the sciatic nerve.

Extraskeletal Ewing sarcoma (EES) is a rare tumor diagnosed in children or young adults and is even more unusual in individuals over 30 years of age. Due to its rare occurrence and low index of suspicion, this tumor can pose diagnostic and therapeutic challenges. We present a case of a 60-year-old male with EES of the sciatic nerve, an unexpected entity given the patient's age, tumor type, and tumor location. This can mimic a nerve sheath tumor on imaging.

Primary renal ewing sarcoma in a 15-year-old female: Case report

@#Extraskeletal Ewing sarcoma (EES) is a rare tumor that primarily affects children and lacks specific clinical signs. Diagnosis is confirmed through imaging techniques, histology, and molecular diagnostics. Treatment typically involves surgical intervention and chemotherapy. We present the case of a 15-year-old female with a history of abnormal mass enlargement in the right flank area. An initial diagnosis of Wilms tumor was made, and the patient underwent a right open radical nephrectomy. However, the tumor recurred eight months after nephrectomy, necessitating a metastasectomy. Chemotherapy was started to immediately target the tumor recurrence. Next-generation sequencing done on the open radical nephrectomy and metastasectomy samples revealed the presence of the EWSR1-FLI1 fusion gene in both specimens, confirming the final diagnosis to be primary renal Ewing sarcoma. Despite undergoing a right open radical nephrectomy for the primary tumor site, a metastasectomy during tumor recurrence, and chemotherapy, the patient’s prognosis remained poor.

Primary Ewing's Sarcoma of the Penis: First Reported Case in the United Kingdom.

Penile cancer is generally rare, and Squamous cell cancer of the penis is the most common histological type. Sarcoma of the penis has a low incidence, but they tend to grow faster than other penile cancers. One of the rarest types of penile sarcomas is Extra-Skeletal Ewing's Sarcoma (EES). The management of such cases can be challenging, and treatment guidelines do not exist for these rare cases. We present a rare case of EES that has developed in the penis of a young patient in the United Kingdom.

Primary Extraskeletal Ewing sarcoma of the foot with extensive skeletal and pulmonary metastasis: A rare case report.

Introduction: First -degree cutaneous extraskeletal Ewing's sarcomas (ESs) are incredibly uncommon skin-specific tumors that often present as a single, tiny lesion that is restricted to the mid-to-deep dermis or involves the subcutis. ESs can be clinically and pathologically misdiagnosed because of their rarity and physical resemblance to other cutaneous cancers. Case presentation: A 47-year-old nonsmoking woman was admitted after being transferred from a nearby hospital to check her right foot pain that had been present for three months and was significantly numbing the same side. Only a few lone cases or brief series are reported in the current literature. The typical description of ESs is that they are tiny masses with positive clinical behavior. Discussion: Despite being a rather common location, only infrequent and minor ESs of the foot are present. After the recommended operation and subsequent histology analysis, we identified this uncommon sort of tumors. Conclusion: Although it's rare, it's very important to consider this tumor in the differential diagnosis of foot pain with/without visible and/or palpable cutaneous lesion.

Larynx cancer diagnosed as Ewing Sarcoma in a 41-year-old man: A case report.

Introduction: Sarcoma as a cause of laryngeal cancer is rare and is even rarer to have an Ewing sarcoma out of the bone presents as laryngeal cancer. In this report, we present this extremely rare case. Case presentation: A 41-year-old man was seen at the ENT clinic complaining of chronic hoarseness and a lump in his neck. Flexible laryngoscopy showed a large mass occupying the left side of the larynx and then a computerized tomography scan proved it. For further evaluation, the laryngoscopy was performed and the frozen section revealed a malignancy. Consequently, the surgical decision was taken and a total Laryngectomy and thyroidectomy were carried out. A final diagnosis of Ewing sarcoma was established using histological examination and immunohistochemical staining. The patient was referred for adjuvant chemo-radiotherapy as recommended by the oncology service. Clinical discussion: laryngeal cancer is rarely diagnosed as Ewing sarcoma. The defined diagnosis should be made based on histological study and immunohistochemical staining besides the clinical presentation and other examinations. Our patient was a candidate for surgical treatment and negative surgical margins were achieved. He was referred for adjuvant chemo-radiotherapy as some studies demonstrated the efficacy of multimodal therapy in treating Ewing sarcoma. Conclusion: Because of the lack of similar studies and documented data in the medical literature about this rare case, Ewing sarcoma should be included in the differential diagnosis in laryngeal cancer cases.

Primary extraskeletal Ewing's sarcoma presenting as an axillary mass: A case report and review of the literature.

Extraskeletal Ewing's sarcoma (EES) is a rare soft tissue tumor predominantly observed in adolescents and young adults, and is characterized by aggressive behavior. So far, only two cases of primary axillary soft tissue EES have been reported in the literature. One of them was a 29-year-old female patient who presented with a lump in her left axilla. Upon examination, an irregular, painless mass, measuring 5 cm × 5 cm × 3 cm, was noted in the left axilla. A histopathological examination of the mass revealed small, round, blue cells with scant cytoplasm, round nuclei, numerous mitosis, and necrosis. An immunohistochemistry (IHC) examination was positive for CD99 and negative for ER, PR, Her2neu, CK7, CK5/6, CD56, CD45, CK-pan, CKHMW, P63, desmin, S100, TdT, vimentin, myogenin, synaptophysin, and chromogranin A. The patient was diagnosed with primary axillary soft tissue EES and was started on neoadjuvant chemotherapy. Twelve months later, she is clinically free from the disease.