Fibroblastic and myofibroblastic tumors
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| Fibroblastic/myofibroblastic tumors | |
|---|---|
| Specialty | Pathology, Dermatology, General surgery, Oncology, Surgical oncology |
| Types | Benign, locally invasive, rarely metastasizing, malignant |
Fibroblastic/myofibroblastic tumors (FMTs) develop from the mesenchymal stem cells which differente into fibroblasts (the most common cell type in connective tissue) and/or the myocytes/myoblasts that differentiate into muscle cells. FMTs are a heterogeneous group of soft tissue neoplasms (i.e. abnormal and excessive tissue growths). The World Health Organization (2020) defined tumors as being FMTs based on their morphology and, more importantly, newly discovered abnormalities in the expression levels of key gene products made by these tumors' neoplastic cells.[1] Histopathologically, FMTs consist of neoplastic connective tissue cells which have differented into cells that have microscopic appearances resembling fibroblasts and/or myofibroblasts. The fibroblastic cells are characterized as spindle-shaped cells with inconspicuous nucleoli that express vimentin, an intracellular protein typically found in mesenchymal cells, and CD34, a cell surface membrane glycoprotein. Myofibroblastic cells are plumper with more abundant cytoplasm and more prominent nucleoli; they express smooth muscle marker proteins such as smooth muscle actins, desmin, and caldesmon.[2] The World Health organization further classified FMTs into four tumor forms based on their varying levels of aggressiveness: benign, intermediate (locally-aggressive), intermediate (rarely metastasizing), and malignant.[1]
Benign FMTs
- Nodular fasciitis
- Proliferative fasciitis and proliferative myositis, originally considered separate entities but now considered to differ only in the tissues involved.[3]
- Myositis ossificans and fibro-osseous pseudotumor of digits, previously considered separate but similar tumors have been reclassified as belong to the same neoplastic bone-forming Fibroblastic/myofibroblastic tumors.[4]
- Ischaemic fasciitis, previously termed decubital fibroplasia and decubital ischemic fasciitis for tumors in the deep subcutaneous tissue at pressure points or bone prominences have more recently been found in various tissue sites.[5]
- Elastofibroma, also termed elastofibroma dorsi, originally considered but now considered to belong to the same neoplastic spectrum with bone-forming capacity.[4]
- Fibrous hamartoma of infancy
- Fibromatosis colli, also termed sternomastoid tumor of infancy
- Juvenile hyaline fibromatosis, also termed fibromatosis hyalinica multiplex juvenilis and the Murray–Puretic–Drescher syndrome
- Infantile digital fibromatosis, also termed inclusion body fibromatosis
- Fibroma of tendon sheath
- Desmoplastic fibroblastoma, also termed collagenous fibroma.[6]
- Myofibroblastoma
- Mammary-type myofibroblastoma
- Calcifying aponeurotic fibroma, also termed aponeurotic fibroma
- EWSR1-SMAD3-positive fibroblastic tumour, classified as an emerging entity by the World Health Organization, 2020.[7]
- Angiomyofibroblastoma
- Cellular angiofibroma, a benign, usually small, slow-growing tumor arising in the groin, scrotal or vulva regions.[8]
- Angiofibroma NOS, i.e. angiofibroma of soft tissue, develops in the extremities, particularly around or in the large joints.[9]
- Nuchal fibroma
- Superficial acral fibromyxoma, also termed acral fibromyxoma.[8]
- Gardner fibroma: a benign proliferation of thick, irregularly arranged collagen bundles with interspersed fibroblasts; >80% of cases are found in association with Gardner's syndrome, a hereditary disorder.[10]
Intermediate (locally aggressive) FMTs
- Palmer/plantar-type fibromatosis, also known as Plantar fibroma and Ledderhose disease.[11]
- Desmoid-type fibromatosis, also termed Desmoid tumor and aggressive fibromatosis[12]
- Lipofibromatosis
- Giant cell fibroblastoma
- Dermatofibrosarcoma protuberans
- Fibrous hamartoma of infancy
Intermediate (rarely metastasizing) FMTs
- Dermatofibrosarcoma protuberans fibrosarcomatous, a variant of the Dermatofibrosarcoma protuberans tumors
- Solitary fibrous tumour, also fibrous termed tumor of the pleura
- Inflammatory myofibroblastic tumour
- Low-grade myofibroblastic sarcoma
- Superficial CD34-positive fibroblastic tumour[13]
- Myxoinflammatory fibroblastic sarcoma[14], also termed acral myxoinflammatory fibroblastic sarcoma because it was initially thought to be limited to acral (i.e. leg and arm) areas.[15]
- Infantile fibrosarcoma, also termed congenital infantile fibrosarcoma and fibrosarcoma, infantile type.[16]
Malignant FMTs
- Solitary fibrous tumor, malignant type, a malignant form of the solitary fibrous tumors
- Fibrosarcoma NOS, i.e. fibrosarcoma, not otherwise specified or, alternatively, adult fibrosarcoma to distinguish it from rarely metastasizing infantile fibrosarcoma. [16]
- Myxofibrosarcoma, once classified as a histiocyte-derived histiocytoma is now reclassified as a Fibroblastic/myofibroblastic tumor.[17]
- Low-grade fibromyxoid sarcoma
- Sclerosing epithelioid fibrosarcoma[16][18]
References
| This article, Fibroblastic and myofibroblastic tumors, has recently been created via the Articles for creation process. Please check to see if the reviewer has accidentally left this template after accepting the draft and take appropriate action as necessary.
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- ^ a b Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC 8167394. PMID 33179614.
- ^ Slack JC, Bründler MA, Nohr E, McIntyre JB, Kurek KC (May 2021). "Molecular Alterations in Pediatric Fibroblastic/Myofibroblastic Tumors: An Appraisal of a Next Generation Sequencing Assay in a Retrospective Single Centre Study". Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society: 10935266211015558. doi:10.1177/10935266211015558. PMID 33970051.
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