Fibroma pathophysiology: Difference between revisions

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|[[File:Cardiac fibroma.jpg|300px|thumb|none|Fibroblast cells in fascicles with abundant collagen fiber,Heidari A, Sabzi F, Faraji R. Right atrial fibroma in an adult patient. Ann Card Anaesth. 2018;21(1):65–67. doi:10.4103/aca.ACA_121_17,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5791493/]]
|[[File:Cardiac fibroma.jpg|300px|thumb|none|Fibroblast cells in fascicles with abundant collagen fiber,Heidari A, Sabzi F, Faraji R. Right atrial fibroma in an adult patient. Ann Card Anaesth. 2018;21(1):65–67. doi:10.4103/aca.ACA_121_17,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5791493/]]
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|[[File:Giant cell fibroma.jpg|300px|thumb|none|Pebbly appearance in right retromolar region,Sabarinath B, Sivaramakrishnan M, Sivapathasundharam B. Giant cell fibroma: A clinicopathological study. J Oral Maxillofac Pathol. 2012;16(3):359–362. doi:10.4103/0973-029X.102485,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3519210/
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Revision as of 19:49, 29 May 2019

Fibroma Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

On gross pathology, polypoid lesion which is usually small, are characteristic findings of oral fibroma. On microscopic histopathological analysis, fibrous stroma, collagen bundles, prominent vessels, and overlying squamous mucosa with hyperkeratosis and focal ulceration are characteristic findings of oral fibroma. On gross pathology, well circumscribed, metaphyseal lesion, and fragments of white-grey rubbery tissue are characteristic findings of chondromyxoid fibroma. On microscopic histopathological analysis, spindle cells or stellate cells in a myxoid or chondroid stroma, lobules with hypocellular centers and hypercellular peripheries, giant cells in a hypercellular periphery, and scattered calcifications are characteristic findings of chondromyxoid fibroma. On gross pathology, fleshy, fibrous, yellow or tan-brown lesion with variable areas of haemorrhage are characteristic findings of non-ossifying fibroma. On microscopic histopathological analysis, spindle cells without cytologic atypia are arranged in a storiform pattern, scattered chronic inflammatory cells and benign giant cells, foam cells and hemosiderin deposition, and mitoses are characteristic findings of non-ossifying fibroma. On gross pathology, discrete mass that is well delineated from surrounding bone, tan-white, rubbery cut surface, firm to gritty and no encapsulation are characteristic findings of ossifying fibroma. On microscopic histopathological analysis, haphazardly distributed lamellated bony spicules on a background of fibrous stroma, a zonal architecture with a center of immature bone surrounded by more mature lamellar bone, and central spicules of woven bony trabeculae are lined by a layer of osteoblasts are characteristic findings of ossifying fibroma.[1][2]

Pathophysiology

Type of fibroma Location Gross pathology Microscpoic pathology Genetics and Immunohistochemistry Associated conditions
Ovarian Fibroma [3][4][5][6][7][8][9][10]
Oral Fibroma [11][12][13]
  • Buccal mucosa
  • Histologically similar to fibrous papule
  • Fibrous stroma is a key feature
  • Collagen bundles may be present
  • Multiple oral fibromas may be seen in Cowden disease.
Chondromyxoid fibroma [14][15][16][17][18][19][20][21][22]
  • Proximal tibia metaphysis Distal femoral metaphysis.
  • Well circumscribed, metaphyseal lesion, and fragments of white-grey rubbery tissue are noted
  • Spindle cells or stellate cells in a myxoid or chondroid stroma
  • Lobules with hypocellular centers and hypercellular peripheries
  • Giant cells in the hypercellular periphery
  • Scattered calcifications.
  • No true hyaline cartilage formation is seen
  • No mitotic activity is seen
  • GRM1 gene fusion or promoter swapping noted
  • It can be associated with a translocation at t(1;5)(p13;p13)
Uterine fibroma [23][24][25][26][27][28][29][30][31][32][33]
  • Round, well circumscribed (but not encapsulated), solid nodules that are white or tan, and show whorled appearance
  • When multiple fibroids are present they frequently have unrelated genetic defects. Specific mutations of the MED12 protein have been noted in 70% of fibroids.
  • If a mother had fibroids, risk in the daughter is about three times higher than average. Researchers have found that only a few specific genes or cytogenetic deviations are associated with uterine leiomyomas. An association with fatty acid synthase has been reported.
  • CD10 positive
  • SMA positive
  • Desmin positive
  • H-caldesmon positive
  • p16 negative
  • Ki-67 negative
Ossifying Fibroma [34][35][36][37][38][39][40]
  • Tibia: predilection for the cortex of the tibial diaphysis.
  • Femur
  • Discrete mass that is well delineated from surrounding bone, tan-white, rubbery cut surface, firm to gritty and no encapsulation
  • They comprise of haphazardly distributed lamellated bony spicules on a background of fibrous stroma
  • The lesion has a zonal architecture with a center of immature bone surrounded by more mature lamellar bone.
  • The central spicules of woven bony trabeculae are lined by a layer of osteoblasts. The background is a loose and storiform fibrous tissue.
  • Clonal chromosomal abnormalities such as trisomies of chromosomes 7, 8, 12 ,21, and/or 22
Non-ossifying Fibroma [41][42][43]
  • Metaphysis of distal femur or proximal tibia (80%)
  • Cortical
  • Fleshy, fibrous, yellow or tan-brown lesion with variable areas of haemorrhage are noted
  • Spindle cells without cytologic atypia are arranged in a storiform pattern.
  • Scattered chronic inflammatory cells and benign giant cells.
  • Foam cells and hemosiderin deposition are present.
  • Mitoses are seen but cytologic atypia is absent.
  • Clonal rearrangements in chromosomes 1, 3, 4, 11, and 14 are noted in NOFs (non-ossifying fibroma) of bone
Desmoplastic Fibroma [44][45][46][47][48][49][50]
  • Mandible (22%) and the metaphysis of long bones (56%).
  • Pelvis
  • Lamellar bone
  • Fibrotic marrow space has following features:
    • Collagen
    • Low cellularity
    • Spindle cells without significant atypia
  • On histopathology, desmoplastic fibromas are identical to soft tissue desmoid tumors, with abundant collagenous stroma and little cellularity or pleomorphism.
  • The main cell types that are seen include the following:
  • Strong positive for the adhesion protein β-Catenin
  • Weak positive labelling for S-100
  • No positive stain for the proliferation marker Ki67
  • No associated conditions
Pleural Fibroma [51][52][53][54][55][56][57][58][59][60][61][62]
  • 80% of pleural fibromas originate in the visceral pleura, while 20% arise from parietal pleura.
  • Composed of irregularly arranged fascicles comprising of spindle cells with collagen separation.
  • They originate from submesothelial mesenchymal cells.
  • Myxoid or cystic degeneration can occur.
  • Recurrent somatic fusions of the two genes, NGFI-A–binding protein 2 (NAB2) and STAT6, located at chromosomal region 12q13, have been identified in pleural fibromas.
  • Some pleural fibromas are associated with the paraneoplastic Doege–Potter syndrome, which is caused by tumor production of IGF-2.
  • Hypoglycaemia: 2-4% 6, thought to be due to production of IGF-2
  • Hypertrophic pulmonary osteoarthropathy (HPOA): ~20% 6: thought to be due to abnormal production of hyaluronic acid
Cardiac Fibroma [63][64][65][66][67][68][69][70][71][72]
  • Ventricular septum and left ventricular free wall.
  • Well defined, solitary, intramyocardial lesions with smooth margins and usually large, with a mean diameter of ~5 cm
  • Usually have no foci of cystic change, hemorrhage, or necrosis. Calcification is common
Renal Medullary Fibroma [73][74][75]
  • Small, and white well circumscribed nodule in medulla typically less than 3mm are characteristic findings of renal medullary fibroma
  • Small polygonal/stellate cells
  • Abundant loose/myxoid stroma
  • Entrapped renal tubules may be present
  • No associated conditions
Cemento-ossifying Fibroma [76][77][78][79][80][81][82][83][84]
  • Maxillary gingiva is involved more often than the mandibular gingiva
  • Solid, sessile or pedunculated mass, which is often ulcerated, and generally has a diameter of less than 2 cm are characteristic findings of cemento-ossifying fibroma.
  • Cemento -ossifying tumors are composed of fibrous tissue, calcified tissue resembling bone and/or cementum.
  • The bone-like component is predominant reminiscent of woven bone and is found in more 'mature' lesions.
  • In some instances, this entity has been divided into cementifying fibroma and ossifying fibroma depending on the relative amounts of the tumor's constituent tissues. *However, in majority of cases both features are present, warranting the generic term cemento-ossifying fibromas.
  • Surface ulceration is common
  • No associated conditions
Desmoplastic Fibroblastoma [85][86][87][88][89][90]
  • Shoulder region
  • Spindle cells or stellate cells without nuclear atypia
  • Acellular stroma with abundant collagen
  • Myxoid areas may be present
  • Mitoses is rare
  • FOSL1 gene is involved in the pathogenesis of desmoplastic fibroblastoma, llq 12 breakpoint described as being characteristic.
  • Beta-catenin -ve
    • Positive in desmoid-type fibromatosis
  • Desmin negative
  • S-100 negative
  • CD34 negative
  • MSA positive
  • Alpha-SMA positive
  • No associated conditions
Elastofibroma [91][92][93][94][95][96][97]
  • Subscapular region.
  • I'll defined, nonencapsulated, rubbery, firm, white lesion with interspersed fat and with moderate demarcation to surrounding tissue are characteristic findings of elastofibroma.
  • The tumors can be quite large (up to 20 cm), although most are around 5 cm.
  • Thick bundles of collagen and elastin fibres are characteristic findings of elastofibroma.
  • Admixture of heavy dense bands of collagenous tissue dissected by fat and abnormal elastic fibers.
  • The elastic fibers are coarse, thick, and darkly eosinophilic, often fragmented into globules, creating a "string of pearls" or "pipe cleaner" appearance. Because of degeneration, the elastic fibers will appear as globules with a serrated or "prickled" edge.
  • There are alterations of short arm of chromosome 1
  • Multifocality may suggest systemic enzymatic defect, resulting in abnormal elastogenesis
  • The elastic fibers will be visible on a Weigert or von Gieson elastic stains.
  • No associated conditions
Sclerotic Fibroma [98][99][100][101][102][103][104][105][106]
  • Skin, may be solitary or multifocal
  • Well-delineated but unencapsulated mass of densely collagenized, hypocellular fibrous tissue with a storiform pattern, and prominent clefts between collagen bundles are characteristic findings of elastofibroma.
Giant cell fibroma [107][108][109][110][111][112][113][114]
  • Mandibular gingiva, followed by the maxillary gingiva, the tongue, and the palate.
  • Sessile or pedunculated nodule that is smaller than 1cm in diameter are characteristic findings of giant cell fibroma. Often, it has a bosselated or somewhat papillary surface.
  • An unencapsulated mass of fibrous connective tissue that contains numerous characteristic large, plump, stellate and spindle-shaped fibroblasts, some of which are multinucleated.
  • The surface epithelium is often atrophic and corrugated.
Peripheral Odontogenic Fibroma [115][116][116]
  • Mandible
Firm, slowly growing, sessile, and nodular growth of the gingiva are characteristic findings of peripheral odontogenic fibroma.
  • An unencapsulated mass of interwoven cellular fibrous connective tissue that contains scattered nests or strands of odontogenic epithelium
  • It consists of cellular fibrous connective tissue parenchyma with non neoplastic islands, strands of columnar or cuboidal odontogenic epithelium.
  • Myxoid foci, osteoid, cementoid, or dystrophic calcifications are sometimes seen.
  • Generally, the surface is not ulcerated.
Type of fibroma Gross image Microscopic image
Ovarian fibroma
Ed Uthman, MD [CC BY-SA 2.0 (https://creativecommons.org/licenses/by-sa/2.0)],https://upload.wikimedia.org/wikipedia/commons/0/04/Fibroma_of_the_Ovary.jpg,https://commons.wikimedia.org/wiki/File:Fibroma_of_the_Ovary.jpg
Histopathology of ovarian fibroma showing benignspindle cellproliferation,Boujoual M, Hakimi I, Kouach J, Oukabli M, Moussaoui DR, Dehayni M. Large twisted ovarian fibroma in menopausal women: a case report. Pan Afr Med J. 2015;20:322. Published 2015 Apr 6. doi:10.11604/pamj.2015.20.322.5998,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4491469/
Oral fibroma
Fibroma of oral mucosa,Klaus D. Peter, Gummersbach, Germany [CC BY 3.0 de (https://creativecommons.org/licenses/by/3.0/de/deed.en)],https://upload.wikimedia.org/wikipedia/commons/c/c5/Fibroma.jpg,https://commons.wikimedia.org/wiki/File:Fibroma.jpg
Librepath [CC BY-SA 3.0 (https://creativecommons.org/licenses/by-sa/3.0)],https://upload.wikimedia.org/wikipedia/commons/d/db/Oral_fibroma_--_low_mag.jpg,https://commons.wikimedia.org/wiki/File:Oral_fibroma_--_low_mag.jpg
Chondromyxoid fibroma
arahkayb [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)],https://upload.wikimedia.org/wikipedia/commons/a/a6/Bone_ChondromyxoidFibroma_Calcium_MP_PA.JPG,https://commons.wikimedia.org/wiki/File:Bone_ChondromyxoidFibroma_Calcium_MP_PA.JPG
Uterine fibroma
Hic et nunc [CC BY-SA 3.0 (https://creativecommons.org/licenses/by-sa/3.0)],https://upload.wikimedia.org/wikipedia/commons/f/f7/Uterine_fibroids.jpg,https://commons.wikimedia.org/wiki/File:Uterine_fibroids.jpg
Calicut Medical College [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0)],https://upload.wikimedia.org/wikipedia/commons/e/ea/Leiomyoma_Uterus_40x.jpg,https://commons.wikimedia.org/wiki/File:Leiomyoma_Uterus_40x.jpg
Ossifying Fibroma
Histopathologicaly, this lesion is characterized by spindle cell proliferation packed with globular cementum droplets and round/ovoid fragments of woven bone. These spindle cells appear benign and associated with psammomatoid cementum droplets and some woven bone and consistent with aggressive ossifying fibroma, psammomatous type IV,Alghonaim Y, ALRashed ALHumaid S, Arafat A. Aggressive ossifying fibroma of right ethmoidal sinus: A case report. Int J Surg Case Rep. ;53:513–516. doi:10.1016/j.ijscr.2017.12.026,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6290393/
Cardiac fibroma
Jha NK, Kiraly L, Tamas C, et al. Large cardiac fibroma and teratoma in children- case reports. J Cardiothorac Surg. 2015;10:38. Published 2015 Mar 22. doi:10.1186/s13019-015-0242-9,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4373309/
Fibroblast cells in fascicles with abundant collagen fiber,Heidari A, Sabzi F, Faraji R. Right atrial fibroma in an adult patient. Ann Card Anaesth. 2018;21(1):65–67. doi:10.4103/aca.ACA_121_17,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5791493/
Giant cell fibroma [[File:Giant cell fibroma.jpg|300px|thumb|none|Pebbly appearance in right retromolar region,Sabarinath B, Sivaramakrishnan M, Sivapathasundharam B. Giant cell fibroma: A clinicopathological study. J Oral Maxillofac Pathol. 2012;16(3):359–362. doi:10.4103/0973-029X.102485,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3519210/

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