A unique case of adamantinoma-like Ewing sarcoma in the calcaneus, exhibiting prominent squamous differentiation and displaying EWSR1 gene rearrangement

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma. This review contains 14 figures, 5 tables and 50 references. Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22)

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CLO21-015: A Unique Case of Duodenal Extraosseous Ewing Sarcoma in an Elderly Filipina

Journal of the National Comprehensive Cancer Network ◽

10.6004/jnccn.2020.7787 ◽

2021 ◽

Vol 19 (3.5) ◽

pp. CLO21-015

Author(s):

Julie R. Heitz ◽

Mintcho E. Mintchev ◽

Joseph W. Howells

Keyword(s):

Ewing Sarcoma ◽

Unique Case ◽

Extraosseous Ewing Sarcoma

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EWSR1 gene rearrangement occurs in a subset of cutaneous myoepithelial tumors: a study of 18 cases

Modern Pathology ◽

10.1038/modpathol.2011.108 ◽

2011 ◽

Vol 24 (11) ◽

pp. 1444-1450 ◽

Cited By ~ 84

Author(s):

Uta Flucke ◽

Gabriele Palmedo ◽

Nina Blankenhorn ◽

Pieter J Slootweg ◽

Heinz Kutzner ◽

...

Keyword(s):

Gene Rearrangement ◽

Ewsr1 Gene

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Combined hepatocellular carcinoma and high grade neuroendocrine carcinoma with EWSR1 gene rearrangement

Pathology ◽

10.1016/j.pathol.2018.07.005 ◽

2018 ◽

Vol 50 (7) ◽

pp. 779-782 ◽

Cited By ~ 1

Author(s):

Khoon Leong Chuah ◽

Brendan Pang ◽

Diana Lim ◽

Chi Kuen Lee ◽

Cora Yuk-Ping Chau

Keyword(s):

Hepatocellular Carcinoma ◽

Neuroendocrine Carcinoma ◽

Gene Rearrangement ◽

High Grade ◽

Ewsr1 Gene

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Adamantinoma-like Ewing Sarcoma in a Metatarsal Bone After Chemotherapy Treated with an Osteocutaneous Fibular Transfer: A Case Report.

10.21203/rs.3.rs-96041/v1 ◽

2020 ◽

Author(s):

Yolandi Anne Marais ◽

Aaron Saini ◽

Nando Ferreira ◽

Kershinee Reddy ◽

Alexander Zühlke ◽

...

Keyword(s):

Adjuvant Chemotherapy ◽

Ewing Sarcoma ◽

Neck Region ◽

Metastatic Lesion ◽

Squamous Differentiation ◽

Diagnostic Challenge ◽

Initial Biopsy ◽

Neo Adjuvant Chemotherapy ◽

One Year ◽

Painless Mass

Abstract Background: Adamantinoma-like Ewing sarcoma is a rare variant of Ewing sarcoma known to have cytokeratin expression and squamous differentiation. It occurs more commonly in the head and neck region with only a few cases reported in long bones of the limbs. It also harbours EWSR1-FLI1 gene fusion which is required for the diagnosis of Ewing sarcoma. We present a case of Ewing sarcoma that manifested Adamantinoma-like morphology only post chemotherapy. Chemotherapy has been reported to induce neuronal maturation and rhabdoid morphology in cases of Ewing sarcoma, but no reports of treatment induced squamous differentiation with P40/P63 expression have been demonstrated to date.Case presentation: An 11 year old boy presented with a one year history of an enlarging painless mass over the left first metatarsal. Initial biopsy showed Ewing sarcoma with typical morphological features and EWSR1 rearrangement confirmed on fluorescent in-situ hybridization. The patient underwent neo-adjuvant chemotherapy and a subsequent wide local excision with an ipsilateral pedicled osteocutaneous fibula transfer. Subsequent histological examination showed frank squamous differentiation in the soft tissue component with keratin pearl formation and P40/P63 expression which was not observed in the initial biopsy and is compatible with Adamantinoma-like Ewing sarcoma.Conclusion: This case describes Adamantinoma-like Ewing sarcoma with P40/P63 expression after neo-adjuvant chemotherapy treatment. This immunophenotype was not apparent on the initial biopsy. It remains uncertain as to the reason for this change. This variant carries a poorer clinical outcome compared to the more conventional variant and could pose a diagnostic challenge(s) particularly if it occurs in an older patient or as a metastatic lesion.

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Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

Case Reports in Oncology ◽

10.1159/000449126 ◽

2016 ◽

Vol 9 (3) ◽

pp. 666-671 ◽

Cited By ~ 4

Author(s):

Safi Khuri ◽

Hayim Gilshtein ◽

Sa’d Sayidaa ◽

Bishara Bishara ◽

Yoram Kluger

Keyword(s):

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Gene Rearrangement ◽

Neoadjuvant Treatment ◽

Neuroectodermal Tumor ◽

Surgical Ward ◽

Healthy Female ◽

Lesser Curvature ◽

Upper Gastrointestinal

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation) was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery.

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