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Fibrous tumors
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Juvenile hyaline fibromatosis is a rare autosomal recessive disease of the connective tissue caused by mutations in the gene encoding the anthrax toxin receptor 2 protein, also called capillary morphogenesis gene 2 located on chromosome 4p21.99,100 The onset is usually between the age of 3 months to 5 years, but may also be later. Hyaline material is deposited in skin, mucous membranes, and other organs causing gingival hypertrophy, flexion contractures of the joints, and skin lesions. The latter are multiple cutaneous papules mainly on the face and neck, nodules, large plaques with a transparent appearance, and gelatinous consistency on ears, around the nose, and on fingers, as well as large firm subcutaneous masses on scalp, trunk, and limbs.101 Distal onycholysis with nail deformation was described.102 Periungual lesions have repeatedly been observed,103 and were clinically similar to recurrent infantile digital fibromatosis.104 Infantile systemic hyalinosis is an allelic syndrome. It bears some similarity to myofibromatosis; however, the distribution of tumors in the skin, associated clinical features, and histopathology allow it to be differentiated.
Assessment of various intralesional injections in keloid: comparative analysis
Published in Journal of Dermatological Treatment, 2022
Waleed Albalat, Sara Nabil, Fathia Khattab
It has been documented for its efficacy and safety in treating other fibroid conditions, counting infantile digital fibromatosis, knuckle pad, rheumatoid nodules, and adverse foreign body reactions and sarcoid granuloma complication following injection of soft tissue filler when employed as mono-therapy or combined with additional medicinal products (e.g. TAC). Some statistics propose that 5-FU is operative for treating hypertrophic scar and that it is very useful for minor keloid.
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].