ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2021
Superficial ALK-rearranged myxoid spindle cell neoplasm: a cutaneous soft tissue tumor with distinctive morphology and immunophenotypic profile.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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28 citing papers in PubMed, 67 citations in OpenAlex.
- Rapid growth of a long-standing dermal nodule in a young Black man: Clinical correlation in an ALK-rearranged epithelioid/spindle cell tumor.JAAD case reports · 2026Article
- Meningioma-like inflammatory myofibroblastic tumor of the lung with TPM3::ALK fusion.Virchows Archiv : an international journal of pathology · 2026Article
- Protein kinase-related tumors in the pediatric population : Updated review on an emerging group with emphasis on the more rarely involved kinases.Pathologie (Heidelberg, Germany) · 2026Review
- A Giant Thoracic ALK-Rearranged Mesenchymal Neoplasm in a Child.Cancer reports (Hoboken, N.J.) · 2026Article
- Gene fusion-driven cutaneous mesenchymal neoplasms: An updated review emphasizing the emerging entities.Virchows Archiv : an international journal of pathology · 2026Review
- EMA-Positive Superficial ALK-Rearranged Myxoid Spindle Cell Neoplasm (SAMS): A Case Report of a Rare Entity and Dermoscopic Findings.Dermatology practical & conceptual · 2026Article
- Case Report: AcralFrontiers in oncology · 2026Article
- ALK rearranged malignant mesenchymal neoplasms of thorax: therapeutically targetable 'ALKomas' beyond the spectrum of non-small cell lung carcinomas and thoracic inflammatory myofibroblastic tumors.Virchows Archiv : an international journal of pathology · 2025Article
- Angiomatoid fibrous histiocytoma occurring at distal/acral extremity sites: clinicopathological and molecular study of 26 cases highlighting frequent myxoid histology and site-dependent genotypic variation.Virchows Archiv : an international journal of pathology · 2025Article
- Primary cutaneous rhabdomyosarcoma with EWSR1/FUS::TFCP2 fusion: four new cases with distinctive morphology, immunophenotypic, and genetic profile.Virchows Archiv : an international journal of pathology · 2025Article
- Article
- Case Report:Frontiers in oncology · 2025Article
- Ensartinib in a primary pulmonaryFrontiers in medicine · 2025Article
- What is new in fibroblastic/myofibroblastic tumors in children.Virchows Archiv : an international journal of pathology · 2025Review
- Case Report: CD34-Negative, S100-Positive Spindle Cell Tumor With aCase reports in pathology · 2025Article
- [Molecular testing in mesenchymal neoplasms: What, when, and how to test? : A review with a special focus on the value of next-generation immunochemistry as a substitute for molecular testing].Pathologie (Heidelberg, Germany) · 2024Review
- Update on cutaneous mesenchymal tumors in the 5th edition of WHO classification of skin tumors with an emphasis on new fusion-associated neoplasms.Virchows Archiv : an international journal of pathology · 2024Review
- Superficial Anaplastic Lymphoma Kinase-Rearranged Myxoid Spindle Cell Neoplasm in the Buttock: A Case Report.Journal of personalized medicine · 2024Article
- Novel NONO::TFE3 fusion and ALK co-expression identified in a subset of cutaneous microcystic/reticular schwannoma.Virchows Archiv : an international journal of pathology · 2023Article
- Superficial CD34 Positive Fibroblastic Tumour with Myxoid Stroma.International journal of surgical pathology · 2023Article
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Authors and funding
6 authors at 1 institution in 1 country.
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Abstract
Gene rearrangements involving the anaplastic lymphoma kinase (ALK) receptor tyrosine kinase gene have been identified in various neoplasms, including inflammatory myofibroblastic tumor and epithelioid fibrous histiocytoma. We present an ALK-rearranged cutaneous soft tissue tumor with unique morphologic and immunophenotypic features that are not shared by other entities with ALK rearrangements. The six cases involved two females and four males, aged 18-84 (mean 51) years old. Three tumors were on the back and three on the lower extremities (thigh, knee, shin); ranging from 0.5 to 5.6 (mean 2.1) cm. Four were confined to the dermis; two involved the subcutis. All six cases were characterized by the presence of spindled to ovoid cells arranged in concentric whorls and cords against a myxoid to myxohyaline stroma and relatively cellular aggregates of plump ovoid to epithelioid cells. Four cases showed distinct hyalinized blood vessels. Both cases that involved the subcutis showed peripheral lipofibromatosis-like areas. Tumor-infiltrating lymphocytes were absent to moderate. Severe cytologic atypia or conspicuous mitotic activity was not identified. Immunohistochemically, all tumors diffusely expressed ALK (D5F3) and CD34. All but one tumor was diffusely positive for S100 protein. All tumors were negative for EMA, AE1/AE3, SMA, and SOX10. Next-generation sequencing revealed ALK fusions with FLNA (3 cases), MYH10 (2 cases), and HMBOX1 (1 case) as the partner genes. In all six cases, the breakpoints involved exon 20 of ALK, which preserves the receptor tyrosine kinase domains of ALK in the fusion product. Of the four cases with limited follow-up information (2-18 months), none recurred. In conclusion, we report an ALK-rearranged cutaneous soft tissue tumor characterized by the presence of myxoid spindle cell whorls and cords, and co-expression of ALK, CD34, and frequently S100 protein, we term "superficial ALK-rearranged myxoid spindle cell neoplasm".
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