Overlapping morphological, immunohistochemical and genetic features of superficial CD34-positive fibroblastic tumor and PRDM10-rearranged soft tissue tumor

Puls, Florian; Carter, Jodi M.; Pillay, Nischalan; McCulloch, Tom; Sumathi, Vaiyapuri P.; Rissler, Pehr; Fagman, Henrik; Hansson, Magnus; Amary, Fernanda; Tirabosco, Roberto; Magnusson, Linda; Nilsson, Jenny; Flanagan, Adrienne M.; Folpe, Andrew L.; Mertens, Fredrik

doi:10.1038/s41379-021-00991-8

Cited by 24 publications

(32 citation statements)

References 15 publications

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“…The other tumors that we assessed (fibrous histiocytoma, atypical fibrous histiocytoma, and DFSP) were all completely negative. Puls et al8 also observed moderate expression in granular cell tumor and schwannoma, and focal expression in dedifferentiated liposarcoma (DDLPS) and myxofibrosarcoma.…”

Section: Discussionmentioning

confidence: 95%

“…To our knowledge, both patients are alive and well following resection of involved lymph nodes. 8 Therefore, although SCD34FT may metastasize, this appears to be a very rare phenomenon, as yet limited to regional lymph nodes and unassociated with histologic progression to frank sarcoma.…”

Section: Discussionmentioning

confidence: 99%

“…Most recently, 2 studies have systematically evaluated the relationship of SCD34FT and PRDM10 -STT using molecular methods 6,8. In 2021, Perret et al6 used array comparative genomic hybridization, RNA sequencing, and whole exome sequencing, to perform a direct comparison of 20 tumors ultimately classified as either SCD34FT (n=10) or PRDM10 -STT (n=10), depending on PRDM10 rearrangement status.…”

Section: Discussionmentioning

confidence: 99%

“…Most recently, 2 studies have systematically evaluated the relationship of SCD34FT and PRDM10-STT using molecular methods. 6,8 In 2021, Perret et al 6 used array comparative genomic hybridization, RNA sequencing, and whole exome sequencing, to perform a direct comparison of 20 tumors ultimately classified as either SCD34FT (n = 10) or PRDM10-STT (n = 10), depending on PRDM10 rearrangement status. Very recently, Puls et al 8 applied FISH, RNA sequencing, and reverse transcription polymerase chain reaction to a large series of SCD34FT (n = 35) and tumors known to harbor PRDM10 rearrangement (n = 8).…”

Section: Discussionmentioning

confidence: 99%

“…Since the recognition of SCD34FT, <100 cases have been described in the literature, and long-term follow-up data are more limited. [1][2][3][4][5][6][7][8] While it appears to behave indolently, 1 patient in the original series developed a lymph node metastasis, and therefore some questions remain regarding the extent of its biological potential. 2 Recently, it has become apparent that SCD34FT shows considerable clinicopathologic overlap with mesenchymal tumors harboring PRDM10 gene fusions (most commonly MED12::PRDM10 and CITED2::PRDM10), which were first identified in 2015 from genetic analysis of tumors originally diagnosed as undifferentiated pleomorphic sarcoma (UPS).…”

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confidence: 99%

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Superficial CD34-Positive Fibroblastic Tumor

Anderson

Mertens

Mariño-Enríquez

et al. 2022

American Journal of Surgical Pathology

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Superficial CD34-positive fibroblastic tumor (SCD34FT) is a rare soft tissue neoplasm that shows overlapping features with PRDM10-rearranged soft tissue tumor (PRDM10-STT). This study characterizes the clinicopathologic, immunohistochemical, and molecular features of SCD34FT in a series of 59 cases. Fluorescence in situ hybridization to assess for PRDM10 rearrangement was performed in 12 tumors. Immunohistochemistry for CADM3 and WT1 was performed; CADM3 was also assessed in histologic mimics. Our cohort of 33 male and 26 female had a median age of 42 (range: 14 to 85) years. Tumors were most commonly located in the lower limb (73%), upper limb (8%), back (7%), and supraclavicular region (3%). The median tumor size was 3.0 cm (range: 1.0 to 9.0 cm). Clinical follow-up in 32 patients (median duration: 26 mo) revealed 2 local recurrences (6%). One patient developed regional lymph node metastases which were completely excised. Microscopically, SCD34FT comprised spindled and pleomorphic cells with glassy cytoplasm and occasional granular cell change. Fluorescence in situ hybridization confirmed PRDM10 rearrangement in 3/8 cases (38%). SCD34FT frequently expressed CADM3 (95%) and WT1 (75%). CADM3 was less diffusely positive in pleomorphic hyalinizing angiectatic tumor (40%), pleomorphic liposarcoma (20%), and undifferentiated pleomorphic sarcoma (10%). We corroborate that SCD34FT is indolent but may rarely metastasize to lymph nodes without adverse outcomes. CADM3 and WT1 may be useful in the distinction from histologic mimics. Since cases of SCD34FT with and without demonstrable PRDM10 rearrangement were clinicopathologically indistinguishable, our study further supports that SCD34FT and PRDM10-STT likely constitute a single entity.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Superficial CD34-Positive Fibroblastic Tumor

Anderson

Mertens

Mariño-Enríquez

et al. 2022

American Journal of Surgical Pathology

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Update on cutaneous mesenchymal tumors in the 5th edition of WHO classification of skin tumors with an emphasis on new fusion-associated neoplasms

Kalmykova,

Baranovska-Andrigo,

Michal

2024

Virchows Arch

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The section on mesenchymal tumors in the 5th edition of WHO classification of skin tumors has undergone several changes, the most important of which is the inclusion of newly identified tumor entities, which will be the main focus of this review article. These specifically include three novel cutaneous mesenchymal tumors with melanocytic differentiation, and rearrangements of the CRTC1::TRIM11, ACTIN::MITF, and MITF::CREM genes as well as EWSR1::SMAD3-rearranged fibroblastic tumors, superficial CD34-positive fibroblastic tumors, and NTRK-rearranged spindle cell neoplasms. Some of the other most important changes will be briefly mentioned as well.

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