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Fibrous tumors
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Provided the fibrokeratoma is sectioned longitudinally, no difficulties should arise in making the correct diagnosis. However, in transverse sections, this may be delicate. A solitary fibrous tumor of the skin mimicking an accessory nail of the little toe was recently described; it was diffusely positive for vimentin and CD34 and exhibited patchy postivity for bcl-2.60 A soft fibroma on the proximal nail fold was descibed once.61 Onychomatricoma has a peculiar cell-rich stroma.62 When bone formation is seen, the pseudomalignant osseous soft tissue tumor has to be ruled out.63 Recurring digital fibrous tumors of childhood (Reye's tumors) do not arise directly from the nail unit but in its immediate vicinity and may impair nail growth; their hallmark are small intracytoplasmic nonmembrane bound eosinophilic granules in approximately 2% of the fibroblasts that stain best with phosphotungstic acid–hematoxylin. Giant cell fibroblastoma stands out by its unique fibroblasts with their wreath or floret like nuclei.64 Dermatofibrosarcoma protuberans and fibrosarcoma are exceedingly rare in the nail region; the former can be ruled out by its immunohistochemical characteristics.
Tumors of Fibrous Tissue
Published in Omar P. Sangueza, Sara Moradi Tuchayi, Parisa Mansoori, Saleha A. Aldawsari, Amir Al-Dabagh, Amany A. Fathaddin, Steven R. Feldman, Dermatopathology Primer of Cutaneous Tumors, 2015
Other features: Mitotic figures are low-to-moderate (less than 5/10 HPF)Tumor cells tightly encase skin appendages without destroying themSome tumors have a prominent myxoid matrixIn some tumors, multinucleated cells are seen (giant cell fibroblastoma)
The landscape of tyrosine kinase inhibitors in sarcomas: looking beyond pazopanib
Published in Expert Review of Anticancer Therapy, 2019
Christopher P Wilding, Mark L Elms, Ian Judson, Aik-Choon Tan, Robin L Jones, Paul H Huang
Imatinib (Glivec®/CGP057148B/ST-1571) was the first TKI approved for the treatment of advanced and metastatic GIST in 2002 and has been evaluated in non-GIST STS [5]. Imatinib has shown promising preclinical activity in models of malignant peripheral nerve sheath tumor (MPNST), malignant rhabdoid tumor (MRT), leiomyosarcoma (LMS), and DFSP. In MPNST cell lines, imatinib suppressed ligand-induced PDGFRβ phosphorylation and associated cellular proliferation/invasion, with a consistent phenotype also seen in vivo [60,61]. Imatinib has also shown antitumor effect in preclinical models of DFSP and giant-cell fibroblastoma, which are rare, recurrent, and infiltrative tumors of the dermis classically characterized by a COL1A1/PDGFB translocation [62,63]. Imatinib reduced DFSP and giant-cell fibroblastoma cellular proliferation and PDGFRβ autophosphorylation in a dose-dependent manner, with concomitant induction of apoptosis, in both in vitro and in vivo models [62,63]. Finally, imatinib has been shown to reduce in vitro proliferation of MRT cells, an aggressive pediatric malignancy characterized by loss of the tumor suppressor SMARCB1, which display constitutive ABL1 expression, as well as the SK-UT-1B LMS cell line model [64–66].
Recurrent Giant Cell Fibroblastoma in an Infant: A Diagnostic Challenge
Published in Fetal and Pediatric Pathology, 2022
Priyanka Maity, Uttara Chatterjee, Mou Das, Sabita Patra
Giant cell fibroblastoma (GCF) is a pediatric soft tissue neoplasm, first described by Enzinger in 1982 [1]. It has a wide spectrum of morphological patterns which led to misdiagnosis of sarcoma in 40% of cases [2]. Local recurrence is as high as 47%. [2] GCF is a fibroblastic tumor of intermediate grade. Recently it has been shown that GCF shares commonality with dermatofibrosarcoma protuberans (DFSP) with regard to histopathology, immunohistochemistry and molecular features [3].
Infantile Myofibroma: A Series of 2 Cases with Special Reference to Cytological Features
Published in Fetal and Pediatric Pathology, 2022
Soumya Dey, Farjana Khatun, Raktim Ray, Shibsankar Barman, Uttara Chatterjee
The common cytological differentials of fibroblastic-myofibroblastic tumors in infants are fibrous hamartoma of infancy, nodular fasciitis, infantile digital fibromatosis, desmoid fibromatosis, lipofibromatosis, inflammatory myofibroblastic tumor, low-grade fibromyxosarcoma and infantile fibrosarcoma. The cytological features of these tumors are compared in Table 1. In our cases, the closest cytological differential was nodular fasciitis (NF). Salient cytological features of NF are the presence of a myxoid matrix, ganglion-like cells and occasional multinucleated giant cells. In contrast to FNA from IM, the smears of NF are usually highly cellular and feasible for cell block preparation to look for MYH9-USP6 (myosin heavy chain 9-ubiquitin specific peptidase 6) gene fusion [8]. Our cases had focal myxoid areas but consistently lacked these specific cytological features of NF. Epidemiologically, NF is uncommon in infants and shows predilection of head-neck region. Low-grade fibromyxosarcoma can show similar morphology on cytology, however it is most often seen in the trunk and deep extremities of young- to middle-aged adults. Infantile fibrosarcoma tends to be far more cellular and shows many mitoses which is quite conspicuous. Fibrous hamartoma of infancy shows variable admixture of mature adipocytes along with fibroblastic and primitive elements [9]. Inflammatory myofibroblastic tumor also shows low cellularity like IM along with small clusters of bland spindle cells and variably myxoid stoma. However, unlike IM, plasma cells and lymphocytes are a conspicuous component. Although the age group of this tumor is similar to IM, mesentery, omentum and the lungs are the common sites. In this lesion, the cell yield is poor due to its fibrous nature. Being myofibroblastic in nature, it shares common SMA positivity with IM. Immunopositivity for ALK is useful for confirmation in the majority of the cases [10]. FNAC smears of lipofibromatosis, an uncommon soft tissue neoplasm that often recurs locally and has infiltrative margins, shows admixture of mature adipose tissue, benign fibrous component and skeletal muscle fibers [11]. Another possible differential is giant cell fibroblastoma that also shows bland spindle cells lying singly or in clusters with bland nuclei. The background often contains fragments of metachromatic stroma. Giant cell fibroblastoma has floret-like giant cells with polarized aggregates of nuclei that are not seen in IM [12, 13]. Cell block from giant cell fibroblastoma shows diffuse and intense CD 34 positivity [14].