Dedifferentiated chondrosarcoma of bone with prominent rhabdoid component

Stemm, Matthew; Beck, Chad; Mannem, Rajeev; Neilson, John C.; Klein, Michael J.

doi:10.1016/j.anndiagpath.2016.11.004

Cited by 4 publications

(8 citation statements)

References 15 publications

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“…Histopathologically, DDCS is characterized by a high‐grade, non‐chondroid sarcoma adjacent to a low‐ or intermediate‐grade chondrosarcoma 2,3 . The term “de‐differentiation” may be a misnomer, as the high‐grade component is typically identified as osteosarcoma, undifferentiated pleomorphic sarcoma (UPS), anaplastic spindle cell sarcoma, or fibrosarcoma, suggesting a process of transformation rather than de‐differentiation 4,5 . Less common patterns of “de‐differentiation” include leiomyosarcoma, giant cell tumors, and rhabdomyosarcoma 2‐6 .…”

Section: Discussionmentioning

confidence: 99%

“…The term “de‐differentiation” may be a misnomer, as the high‐grade component is typically identified as osteosarcoma, undifferentiated pleomorphic sarcoma (UPS), anaplastic spindle cell sarcoma, or fibrosarcoma, suggesting a process of transformation rather than de‐differentiation 4,5 . Less common patterns of “de‐differentiation” include leiomyosarcoma, giant cell tumors, and rhabdomyosarcoma 2‐6 . DDCS therefore may be mistaken for a number of tumors with spindled and/or epithelioid morphology, particularly if the diagnostic adjacent chondrosarcoma is not visualized in the sampled specimen.…”

Section: Discussionmentioning

confidence: 99%

“…The prognosis of DDCS is poor, with a 5‐year survival of approximately 10%. Pathologic fracture, older age, and metastasis further indicate an exceptionally poor prognosis 5,6 …”

Section: Discussionmentioning

confidence: 99%

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GATA‐3 expression in a de‐differentiated chondrosarcoma with cutaneous iatrogenic implantation: a diagnostic pitfall with important clinical implications

et al. 2021

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De‐differentiated chondrosarcoma (DDCS) is an extremely aggressive tumor of the bone characterized by a high‐grade, non‐chondroid sarcoma adjacent to a low‐ or intermediate‐grade chondrosarcoma. Adequate tumor sampling demonstrating the biphasic features is necessary to make an accurate diagnosis. The diagnosis may be challenging as histopathology may mimic other neoplasms. We present a case of a 76‐year‐old woman with a history of breast cancer who presented with a pathologic non‐displaced fracture. A bone biopsy demonstrated a high‐grade neoplasm composed of pleomorphic spindled and epithelioid cells with focal expression of AE1/3 and GATA3, most likely consistent with metastatic breast carcinoma. After a difficult clinical course, the tumor was resected demonstrating a similar morphology to her prior biopsy, as well as an area of a low‐grade cartilaginous neoplasm consistent with chondrosarcoma. The biphasic tumor alongside a low‐grade chondrosarcoma allowed for a diagnosis of DDCS. Several days after her procedure, the patient developed violaceous nodules overlying and surrounding the surgical site. Skin biopsy demonstrated a malignant epithelioid neoplasm with identical histomorphologic features identical to her prior bone resection. Given the location of the skin lesions directly within the surgical site right after resection, the clinical‐pathological picture was that of sarcomatosis cutis by iatrogenic cutaneous implantation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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GATA‐3 expression in a de‐differentiated chondrosarcoma with cutaneous iatrogenic implantation: a diagnostic pitfall with important clinical implications

et al. 2021

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“…At present, the main treatment method for CHS is surgical resection. Thus, it is of great importance to elucidate the underlying mechanism of CHS to develop a novel therapeutic method for patients with CHS (1,19). Due to their extensive effects on cell proliferation, differentiation, apoptosis, and drug resistance, miRNAs have been revealed to be widely involved in the progression of a number of tumors (20,21).…”

Section: Discussionmentioning

confidence: 99%

“…Chondrosarcoma (CHS) is a malignant tumor with pure hyaline cartilage differentiation (1). Among primary bone malignancies, CHS is ranked second most common following osteosarcoma (2).…”

Section: Introductionmentioning

confidence: 99%

MicroRNA‑525 enhances chondrosarcoma malignancy by targeting F‑spondin 1

et al. 2018

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Increasing evidence has suggested that microRNAs (miRNAs; miRs) are extensively involved in the progression of chondrosarcoma (CHS). However, few studies have investigated the functional role of miR-525 in CHS tissues and cells. In the present study, it was discovered that miR-525 levels were decreased in CHS tissues and cells. Dual luciferase assays indicated that F-spondin 1 (SPON1) is a target gene of microRNA (miR)-525. In addition, miR-525 overexpression suppressed SW1353 cell migration and invasion and enhanced SW1353 cell apoptosis. Increased SPON1 expression levels were identified in CHS tissues and cell lines. Furthermore, miR-525 overexpression significantly suppressed the activation of focal adhesion kinase (FAK)/Src/phosphatidylinositol-4,5-bisphosphate 3-kinase (PI3K)/protein kinase B (Akt) signaling in CHS cells; this suppression led to SPON1 silencing. In comparison, the SPON1 knockdown-mediated inactivation of FAK/Src/PI3K/Akt signaling was inhibited by inhibiting miR-525. In summary, the present study revealed that decreased miR-525 levels could enhance CHS malignancy as decreased miR-525 binding to the 3′ untranslated region of SPON1 activates FAK/Src/PI3K/Akt signaling.

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