Cytodiagnosis of Extraskeletal Ewing’s Sarcoma and its Confirmation by Fluorescence in situ Hybridization

Dey, Biswajit; Singh, Ankita; Barwad, Adarsh; Dange, Prasad; Siddaraju, Neelaiah

doi:10.7860/jcdr/2016/20155.8317

Cited by 3 publications

(3 citation statements)

References 6 publications

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“…ES/ PNETs exhibit an aggressive course characterized by early metastatic disease [ 9 , 10 ]. Extraosseous ES has a poorer prognosis as compared to its osseous counterpart with a high incidence of local recurrence and distant metastasis [ 11 ]. Despite aggressive treatment, the prognosis of renal ES is poor, with an overall cure rate of 20% [ 2 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Primary Adult Renal Ewing’s Sarcoma: A Rare Entity

Shangpliang¹,

Rangad²,

Das³

et al. 2022

Cureus

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Ewing's sarcoma/primitive neuroectodermal tumors are high-grade small round blue cell tumors traditionally found in children and adolescents. These tumors primarily affect the bone and soft tissue, with extraskeletal sites rarely being affected. The clinical presentation and imaging findings are non-specific and are not characteristic. The diagnosis is essentially based on the histopathologic findings assisted by immunohistochemistry and/or cytogenetic molecular studies. Proper diagnoses and timely management of this tumor are essential owing to the aggressive nature and poor prognosis of the disease.

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Section: Discussionmentioning

confidence: 99%

Primary Adult Renal Ewing’s Sarcoma: A Rare Entity

Shangpliang¹,

Rangad²,

Das³

et al. 2022

Cureus

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“…IHC could be a helpful adjunct for further tumor characterization, and various studies have demonstrated the feasibility of performing these on cell blocks of FNA samples [13,[20][21][22][23]. Ewing sarcoma shows strong nuclear immunopositivity for FLI1 in >90% of cases, and ERG immunopositivity is demonstrated in most tumors that harbor a t(21;22) (EWSR1-ERG) translocation [12,14,24].…”

Section: Ewing Sarcomamentioning

confidence: 99%

A Review of Effusion Cytomorphology of Small Round Cell Tumors

Han¹,

VandenBussche²,

Abildtrup³

et al. 2021

Acta Cytologica

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Background: Small round cell tumors (SRCTs) are a broad category of diverse malignant tumors composed of monotonous undifferentiated cells. Involvement of serous fluids by SRCT is rare; however, the identification of exfoliated malignant cells is a crucial component of management and has significant implications for treatment and prognosis. The most common effusion tumors with SRCT morphology include Ewing sarcoma, synovial sarcoma, rhabdomyosarcoma (RMS), small-cell neuroendocrine carcinoma (SCNC), and desmoplastic SRCT, and the cytomorphologic distinction between these tumors is challenging. The purpose of this article is to describe the morphologic features of the most common SRCT in fluids and propose helpful ancillary testing. Summary: Effusion SRCTs display similar primitive and undifferentiated morphologic features although each has subtle variations. Ewing sarcoma is a mesenchymal neoplasm and harbors characteristic translocations t(11;22) (EWSR1-FLI1) or t(21;22) (EWSR1-ERG). In fluids, Ewing sarcoma shows poorly differentiated cells of variable size with round to oval nuclei, prominent nucleoli, and scant cytoplasm. In contrast, synovial sarcoma typically involves extremities and expresses a fusion transcript in t(X;18) (SS18-SSX). This soft tissue neoplasm demonstrates uniform cells with irregular nuclear contours, characteristic nuclear folding, and scant cytoplasm. RMS is a neoplasm arising from skeletal muscle, and the alveolar subtype demonstrates a translocation in t(2;13) (PAX3-FOXO1). The malignant cells show a spectrum of small round cells and pleomorphic large cells with rhabdoid morphology. RMS cells characteristically express myogenin and MyoD1, markers of skeletal muscle differentiation. Although SCNC is not a classic SRCT, the morphology is similar. SCNC demonstrates tight clusters of malignant cells with nuclear molding and salt-and-pepper chromatin. This tumor classically has neuroendocrine differentiation and is positive for synaptophysin and chromogranin on immunohistochemistry. And last, desmoplastic SRCT typically presents as an intra-abdominal mass in young men and characteristically harbors the translocation t(11;22) (p13;q12) (EWSR1-WT1). Cytomorphologically, the tumor shows small monomorphic cells occasionally arranged as rosette-like structures. Key Message: The diagnosis of SRCT can be made in effusion samples and is best achieved with a combination of morphologic features, clinical history, and ancillary testing.

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“…ALES exhibits distinct is this meant to be epithelial? morphology and nesting pattern and shows translocation involving EWSR and FLI1, like conventional Ewing sarcoma. ALES has a relatively higher incidence in the head and neck region of about 5% with unique morphology recapitulating epithelial differentiation with the formation of squamous pearls, peripheral palisading and a diffuse cytokeratin as well as p40 positivity .…”

Section: Introductionmentioning

confidence: 97%

Cytomorphological features of adamantinoma‐like Ewing's sarcoma in parotid masquerading as sialoblastoma in a paediatric patient

et al. 2020

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The incidence of mesenchymal tumours in the salivary gland range from 0.3% to 1%. 1 Adamantinoma-like Ewing sarcoma (ALES) is an unusual variant of Ewing sarcoma with more incidences in the head and neck of young patients, but scarce in the salivary gland. 2 The closest differential is sialoblastoma, also known synonymously as congenital basal cell adenoma/embryoma, another rare tumour of the salivary gland that almost exclusively arises from the parotid gland 3 in infants. ALES exhibits distinct is this meant to be epithelial? morphology and nesting pattern and shows translocation involving EWSR and FLI1, 4,5 like conventional Ewing sarcoma. ALES has a relatively higher incidence in the head and neck region of about 5% with unique morphology recapitulating epithelial differentiation with the formation of squamous pearls, peripheral palisading and a diffuse cytokeratin as well as p40 positivity. 2,4 This tumour encompasses cells with basaloid morphology reminiscent of salivary anlage structures 6 and has an array of morphological patterns with characteristic immunohistochemistry. There are rare reports available on cytomorphological features of ALES. 7 We report an unusual case of ALES of the parotid gland in a 12-year-old, masquerading as sialoblastoma. | C A S E REP ORT | Clinical featuresA 12-year-old girl presented to the clinic with complaints of preauricular swelling for 2 months. Clinical examination revealed a 4 × 3 cm firm, non-tender swelling with well-defined borders, and restricted mobility. Haematological and biochemical investigations were mostly within normal limits. Fine needle aspiration (FNA) was performed with a radiological possibility of pleomorphic adenoma. | MicroscopyAspirate smears were highly cellular with nests and sheets of tumour cells demonstrating peritheliomatous pattern at places. These cells showed epithelioid morphology with moderate atypia, round to ovoid nuclei, finely granular chromatin, and occasional prominent nucleoli. Vague rosetting was also appreciated focally ( Figure 1A-E). As per the Milan System, the morphology fitted in category VI, malignant tumour with epithelioid morphology. Certain unique morphological features, such as a nesting of small round blue cells with epithelioid morphology posed a diagnostic challenge in categorising the lesion. Therefore, on FNA cytology, two possible differentials were suggested; sialoblastoma and Ewing sarcoma/primitive neuroectodermal tumour. Subsequently, superficial parotidectomy with right cervical lymph node excision was done. The specimen was well encapsulated; no macroscopic extracapsular extension was visualised. Cut section revealed a whitish, relatively circumscribed tumour that was replacing the entire salivary gland with patchy areas of congestion reaching up to the capsule ( Figure 1F). Macroscopically, the tumour had replaced the whole of the salivary gland with no visible healthy salivary gland tissue. Microscopic sections, however, displayed rim of normal salivary gland tissue at the periphery, with an ill circumscr...

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Cytodiagnosis of Extraskeletal Ewing’s Sarcoma and its Confirmation by Fluorescence in situ Hybridization

Cited by 3 publications

References 6 publications

Primary Adult Renal Ewing’s Sarcoma: A Rare Entity

Primary Adult Renal Ewing’s Sarcoma: A Rare Entity

A Review of Effusion Cytomorphology of Small Round Cell Tumors

Cytomorphological features of adamantinoma‐like Ewing's sarcoma in parotid masquerading as sialoblastoma in a paediatric patient

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