NKX3.1 a useful marker for mesenchymal chondrosarcoma: An immunohistochemical study

Syed, Mubbasher; Mushtaq, Sajid; Loya, Asif; Hassan, Usman

doi:10.1016/j.anndiagpath.2020.151660

Cited by 18 publications

(13 citation statements)

References 14 publications

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“…The first shows a bimorphic histological pattern consisting of undifferentiated, small, round, uniform cells and well-differentiated hyaline cartilage areas [ 27 , 29 ]. Mesenchymal CHS stains positive for S100 and SRY-box transcription factor 9 (SOX9) ( Figure 2 ), like conventional subtype, but it is also CD99 and NK2 homeobox 2 (NKX2.2) positive [ 30 ]. Furthermore, the co-expression of epithelial membrane antigen (EMA), mucin 1 (MUC1), desmin, myogenin, myoblast determination protein 1 (MyoD1) can be found.…”

Section: Morphology and Differentiation Of Chondrosarcomamentioning

confidence: 99%

Chondrosarcoma-from Molecular Pathology to Novel Therapies

Zając

Kopeć

Szostakowski

et al. 2021

Cancers

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Chondrosarcoma (CHS) is the second most common primary malignant bone sarcoma. Overall survival and prognosis of this tumor are various and often extreme, depending on histological grade and tumor subtype. CHS treatment is difficult, and surgery remains still the gold standard due to the resistance of this tumor to other therapeutic options. Considering the role of differentiation of CHS subtypes and the need to develop new treatment strategies, in this review, we introduced a multidisciplinary characterization of CHS from its pathology to therapies. We described the morphology of each subtype with the role of immunohistochemical markers in diagnostics of CHS. We also summarized the most frequently mutated genes and genome regions with altered pathways involved in the pathology of this tumor. Subsequently, we discussed imaging methods and the role of currently used therapies, including surgery and the limitations of chemo and radiotherapy. Finally, in this review, we presented novel targeted therapies, including those at ongoing clinical trials, which can be a potential future target in designing new therapeutics for patients with CHS.

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Section: Morphology and Differentiation Of Chondrosarcomamentioning

confidence: 99%

Chondrosarcoma-from Molecular Pathology to Novel Therapies

Zając

Kopeć

Szostakowski

et al. 2021

Cancers

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“…Histologically, the tumor is characterized by poorly differentiated small round cells with an abrupt transition to hyaline cartilage [ 3 ]. The immunohistochemical stains are usually positive for NKX2.2, CD99, S100, and SOX9 [ 15 ]. Recently, SOX-9 has been reported as a marker that stains both the undifferentiated and the cartilaginous components in ESMC [ 16 , 17 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case Report of Mesenchymal Chondrosarcoma with Pancreatic Metastasis

Chen

Chou

2022

Medicina

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Background: Mesenchymal chondrosarcoma is a rare but aggressive subtype of sarcoma. The majority of involvement locates in the axial skeleton. Treatment modalities include radical surgery, local radiotherapy, and systemic chemotherapy. However, the long-term survival outcome remains poor. Case presentation: We present the case of a 33-year-old male with a palpable chest wall mass for one year, diagnosed with mesenchymal chondrosarcoma with surgical removal. Later, he had an unusual pancreatic tail tumor as the first presentation of disease metastasis which was proven by surgical resection one year later. Conclusion: Although mesenchymal chondrosarcoma locates mainly in the axial skeletal system, extra-skeletal soft tissue or organ involvement might be seen occasionally. Active surveillance with multidisciplinary team management could significantly prolong survival outcomes.

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“…The immunophenotype of mesenchymal chondrosar-Acta Cytologica 2022;66:279-294 DOI: 10.1159/000524260 coma includes S100 protein positivity in the cartilaginous component and CD99 and SOX9 positivity in the small cell population. NKX3.1 is a useful immunohistochemical marker in differentiating mesenchymal chondrosarcoma from its histological mimics [98]. Negative staining for FLI-1 and CD45 help in distinguishing mesenchymal chondrosarcoma from ES and non-Hodgkin lymphoma.…”

Section: Discussionmentioning

confidence: 99%

The Small Round Cell Sarcomas Complexities and Desmoplastic Presentation

Domanski¹

2022

Acta Cytologica

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Background: Small round cell sarcomas (SRCSs) account for most solid malignancies in the pediatric age group and are a part of group of malignant tumors characterized by heterogenous clinical presentation and overlapping microscopic features of small, round, primitive cells. In addition to the recently established certain genetically defined subset of undifferentiated round cell sarcomas of soft tissue and bone, this group of sarcomas include desmoplastic small round cell tumor, poorly differentiated synovial sarcoma, alveolar rhabdomyosarcoma, mesenchymal chondrosarcoma, and small cell osteosarcoma. Although, those entities share clinical and cytomorphologic features and cannot be unequivocally classified based on clinical presentation and morphology alone. Most of SRCSs characterizes of particular patterns of protein expression or genetic changes and ancillary tests remain necessary to confirm or rule out a specific diagnosis. Subtle but occasionally distinctive cytologic features narrows the number of differential diagnoses and helps to select appropriate ancillary tests necessary for the final diagnosis. Thus, when adequate fine needle aspiration (FNA) biopsy specimen is combined with ancillary tests, a specific histologic diagnosis can be made in almost all cases. However, due to complex cytologic features of SRCS as well as various quality and diversity of FNA smears, there are cases in that cytologic features which do not entirely match the known diagnostic criteria. Summary: The aim of this review was to summarize cytomorphologic criteria and to present rare and divergent cytological features of SRCSs. Careful assessment of clinical presentation, cytological features, immunohistochemical patterns, and molecular alternations is necessary for an accurate diagnosis. Knowing of rare and divergent microscopic findings that does not fit with the known cytological criteria will help to avoid misdiagnosis. Key Messages: The role of FNA biopsies diagnosing soft tissue and bone tumors has been increasing because of the ability of ancillary tests to assist in the diagnosis of specific tumors. SRCSs may be diagnosed accurately in cytology specimens. Access to clinical and radiographic presentation, utility of ancillary tests, understanding complexity of cytological features, and awareness of the rare cytologic findings that differ from that of the established diagnostic criteria are essential to make correct diagnosis.

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NKX3.1 a useful marker for mesenchymal chondrosarcoma: An immunohistochemical study

Cited by 18 publications

References 14 publications

Chondrosarcoma-from Molecular Pathology to Novel Therapies

Chondrosarcoma-from Molecular Pathology to Novel Therapies

A Rare Case Report of Mesenchymal Chondrosarcoma with Pancreatic Metastasis

The Small Round Cell Sarcomas Complexities and Desmoplastic Presentation

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