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Fibrous histiocytoma (FH) or dermatofibroma is a common cutaneous lesion mostly seen in adults and rare in the first two years of life. Two hundred sixty-seven patients younger than 18 years with a diagnosis of FH or dermatomyofibroma, a lesion with morphologic overlap with FH, were identified from the files of a single institution, with only 13 (4.8%) occurring in patients younger than 5 years. Ten patients had either underlying neurologic, autoimmune, or metabolic disorders or a family history of autoimmune conditions. Histologic review of hematoxylin and eosin staining and immunostaining on 75 FHs and dermatomyofibroma in 70 patients showed the following results: 33 classic FHs, 8 classic FHs characterized by a peculiar retiform morphology with thin fascicles of elongated cells forming a network reminiscent of the eruptive variant of FH, 19 deep/cellular variants, 5 aneurysmal variants, 3 lipidized variants (including two lesions in a patient affected by mucopolysaccharidosis IV), 3 dermatomyofibromas, and 4 isolated cases of hemosiderotic, granular cell atypical, and epithelioid FH. Immunostaining for factor XIIIa highlighted a dense network of dendritic cells in FH, which was significantly reduced in the FH with retiform morphology. Smooth muscle actin staining was positive in a high percentage of FHs (85.3%). The current series demonstrates that FH in children may show unique clinical and morphologic features. The retiform pattern with decreased dendritic cells found in congenital lesions and in two older patients with lesions in two locations might have a different pathogenesis, probably related to an altered immune response in very young patients., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Atypical fibroxanthoma (AFX) and undifferentiated pleomorphic sarcoma (UPS) are uncommon, spindle cell cutaneous malignancies. Solid organ transplant recipients (SOTRs) are immunosuppressed and therefore have a higher incidence of cutaneous malignancies., Objective: We describe the clinical spectrum of AFX and a more-aggressive, deeper variant, UPS, in SOTRs., Materials and Methods: A retrospective chart review of AFX and UPS in SOTRs was implemented. Cases from Vanderbilt University, Emory University, Mayo Clinic-Jacksonville, and University of Rochester were included. A literature search included previously published cases., Results: The average age of SOTRs at time of tumor presentation was younger than typically seen in immunocompetent patients for AFX. Rates of local recurrences and metastases were higher in the SOTRs than is noted in the immunocompetent literature. Rates of recurrence were higher in those treated with excision than in those treated with Mohs micrographic surgery (MMS)., Conclusion: AFX and UPS may have a greater risk for recurrence, metastases, and mortality in SOTRs, in whom early treatment with MMS may demonstrate certain advantages in terms of minimizing risk of recurrence and metastasis. UPS and recurrent tumors should be staged appropriately and may respond to adjuvant radiation therapy and reduction of immunosuppression. Immunohistochemical evaluation is recommended to exclude other spindle cell tumors., (© 2011 by the American Society for Dermatologic Surgery, Inc. Published by Wiley Periodicals, Inc.)

Background: Abnormalities in the expression of human leucocyte antigens (HLA) by tumour cells impair cellular immune responses promoting immune evasion and tumour survival. To date, studies analysing HLA class I and class II expression levels in malignant fibrous histiocytomas, fibrosarcomas and dermatofibrosarcoma protuberans are limited., Aims: Therefore, we investigated the in vivo expression profile of HLA class I and class II antigens in 99 malignant fibrous histiocytomas, 20 fibrosarcomas and 34 dermatofibrosarcoma protuberans from different anatomical sites., Material and Methods: Immunohistochemistry using monoclonal antibodies to HLA class I and class II antigens was used to define the expression levels of these antigens on respective tumour samples., Results: Frequent loss or downregulation of HLA class I and class II expression in malignant fibrous tumours was observed for the different types of tumours examined., Discussion: The data presented suggest for the first time a high frequency of HLA class I and class II abnormalities in malignant fibrous histiocytomas, fibrosarcomas and dermatofibrosarcoma protuberans in vivo., Conclusion: This information might be useful in the practical and clinical design of tumour vaccination strategies., (© 2010 The Authors. Journal of the European Academy of Dermatology and Venereology © 2010 European Academy of Dermatology and Venereology.)

We report a case of multiple dermatofibromas in a patient with systemic lupus erythematosus (SLE) and Sjögren's syndrome. He was treated with prednisolone, diaphenylsulfone, and cyclosporine for SLE. He noticed two brown nodules on his right lower leg 3 years after the first consultation. Subsequently, six nodules developed within 6 months, and 2 more nodules after 10 months. Histopathological examination of a nodule on his left hand showed fibrotic proliferation with a storiform pattern in the whole dermis, but neither necrosis nor mitosis was observed. From these findings, a diagnosis of multiple dermatofibromas was made. As these tumors appeared during the remission stage of SLE, they might have been under immunosuppressive conditions caused by immunosuppressing agents rather than collagen disease itself.