Primary clear cell sarcoma in the lung apex with spinal metastasis: a fatal outcome

Sass, Remy Grace; Tandra, Pavan K.; Shonka, Nicole; Manzer, Melissa

doi:10.1136/bcr-2019-231556

Cited by 2 publications

(4 citation statements)

References 15 publications

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“…The three previously described cases of the primary lung CCSST presented differently. The first case was a 55-year-old, and the second was a 50-year-old; both were asymptomatic ( 3 ), while the 3 rd was a 28-year-old who presented with Pancoast syndrome, spinal cord compression, and paraplegia ( 4 ). The lesions’ sizes were 4, 2.5, and 8*6 cm locally advanced tumor, respectively ( 3 , 4 ).…”

Section: Discussionmentioning

confidence: 99%

“…The 3 rd case was treated by T8-T10 laminectomy followed by radiotherapy because it presented with a locally advanced tumor associated with local invasion, hilar, mediastinal, supraclavicular, and cervical lymphadenopathy, as well as spine metastasis. The outcome in the latter case was fatal immediately after discontinuation of therapy ( 4 ).…”

Section: Discussionmentioning

confidence: 99%

“…Other sites, including the head and neck, kidneys, gastrointestinal tract, chest wall, mediastinum, trunk, and penis, have been described ( 2 ). To our best knowledge, three cases were reported in the lung ( 3 , 4 ). Herein we describe a case of CCSST, which presented as a central left-sided lung mass.…”

Section: Introductionmentioning

confidence: 98%

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Primary Clear Cell Sarcoma of the Lung: a Case Report

et al. 2021

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Background: Clear Cell Sarcoma of Soft Tissue (CCSST), or melanoma of the soft part, is a rare, aggressive tumor that originates in the aponeurosis and fasciae of the distal parts of the extremities. Reports from other sites of the body are rare. Objective: We are reporting an extremely rare tumor that presented as a central left-sided lung mass and found to be clear cell sarcoma of soft tissue. Methods: We report a 24-year-old male patient presented with recurrent attacks of left-sided chest pain associated with cough and dyspnea. Results: Imaging showed a central left-sided 8*5.5*5 cm lung mass. The age of the patient and the radiological characteristics of the lesion were suggestive of a benign pathology. After histopathological assessment of the lesion, suspicion of the malignant process was raised, mainly melanoma of soft part and PEComa. The patient underwent left-sided pneumonectomy. The postoperative histological examination, immunohistochemical findings including positive staining for S-100, HMB-45, and Melan-A, and positive FISH study for EWSR1 gene rearrangements supported the diagnosis of CCSST originating primarily in the major fissure of left the lung. Conclusion: The rarity of CCSST in general and tumors originating in the lung primarily raise the challenges in hypothesizing a differential diagnosis, choosing proper investigations and treatment methods. The histological examination, immunohistochemical, and cytogenetics of the tumor are mandatory to reach the final diagnosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Primary Clear Cell Sarcoma of the Lung: a Case Report

et al. 2021

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“…This rare tumor type can also arise in some unusual sites, including the head and neck region, 1,2 pleura, 3,4 trunk, 5 retroperitoneum, 6 penis, 7,8 bone, 9,10 and visceral organs. 11 In 2003, Zambrano et al 12 reported a small series of 6 gastrointestinal (GI) tumors sharing morphological features in common with CCS-ST but showing essential differences, particularly the absence of expression of melanocytic markers or ultrastructural evidence of melanocytic differentiation. This distinctive entity was proposed as “osteoclast-rich tumor of the GI tract with features resembling CCS of soft parts,” and later as CCS-like tumor/malignant gastrointestinal neuroectodermal tumor (CCS-LT/MGNET).…”

Section: Introductionmentioning

confidence: 99%

Primary Clear Cell Sarcoma of the Ileum: A Case Report With Next-Generation Sequencing Analysis

Zhu

Zhang

et al. 2021

Int J Surg Pathol

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As the concept of clear cell sarcoma–like tumor or malignant gastrointestinal neuroectodermal tumor (CCS-LT/MGNET) has been widely accepted, primary CCS of the gastrointestinal tract (CCS-GI) is becoming a rare entity. In this article, we describe a case of primary CCS-GI that occurred in the ileum of a 65-year-old male to further illustrate its rare occurrence. Similar to CCS of soft tissue (CCS-ST), the tumor was composed of spindled to epithelioid cells displaying fascicular, nested, or pseudopapillary arrangement. The tumor cells had large round to ovoid nuclei with vesicular chromatin and prominent nucleoli, containing eosinophilic to pale cytoplasm. In contrast to CCS-LT/MGNET, immunohistochemical study also showed variable positivity of HMB45, melan A, and MiTF besides the strong and diffuse staining of S100 protein and SOX10. Fluorescence in situ hybridization (FISH) using fusion probes identified EWSR1 and ATF1 genes rearrangement. Next-generation sequencing (NGS) analysis further revealed EWSR1 exons9/8- ATF1 exon4 and ATF1 exon3- EWSR1 exon11 fusion genes. CCS-GI and CCS-LT/MGNET possibly represent 2 related entities of the same spectrum, which differentiate along 2 different pathways.

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Primary clear cell sarcoma in the lung apex with spinal metastasis: a fatal outcome

Cited by 2 publications

References 15 publications

Primary Clear Cell Sarcoma of the Lung: a Case Report

Primary Clear Cell Sarcoma of the Lung: a Case Report

Primary Clear Cell Sarcoma of the Ileum: A Case Report With Next-Generation Sequencing Analysis

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