Soft tissue
Fibroblastic / myofibroblastic
Giant cell fibroblastoma
Author: Komal Arora, M.D.
Last author update: 1 July 2012
Last staff update: 23 November 2020
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Giant cell fibroblastoma [title]
Table of Contents
Definition / general | Epidemiology | Case reports | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Arora K. Giant cell fibroblastoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuegiantcellfibro.html. Accessed May 29th, 2023.
Definition / general
- Rare childhood fibroblastic tumor of intermediate malignancy with floret-like giant cells and ectatic pseudovascular spaces lined by stromal cells and giant cells
- Part of WHO classification for skin tumors, not soft tissue tumors
- Appears to evolve into DFSP by genomic gains of COL1A1-PDGFB (Genes Chromosomes Cancer 2008;47:260)
- Considered the juvenile form of DFSP, as both have the same translocation (Am J Surg Pathol 2003;27:27, Arch Pathol Lab Med 1996;120:1052, Ann Diagn Pathol 2007;11:81)
Epidemiology
- Usually children less than 10 years old, 2/3 male
- Painless nodule of subcutis, usually in trunk, extremities, head and neck
- 50% recur but recurrences are controllable, no metastases
Case reports
- 3 year old boy with giant cell fibroblastoma (Pathol Oncol Res 2003;9:249)
- 28 year old woman with giant cell fibroblastoma of the vulva at the site of a previous fibroepithelial stromal polyp (J Low Genit Tract Dis 2007;11:112)
Clinical images
Contributed by Mark R. Wick, M.D.
Mammogram
Gross description
- Poorly circumscribed, gray to yellow mucoid mass that is difficult to completely excise, usually in subcutis
Microscopic (histologic) description
- Dermis and subcutis contains hyperchromatic spindle or stellate shaped cells in a collagenous or myxoid matrix with scattered hyperchromatic, multinucleated, floret-like giant cells with prominent nucleoli, similar to those in pleomorphic lipomas
- Ectatic pseudovascular spaces are lined by a discontinuous row of floret-like cells and tumor cells
- Honeycomb or parallel pattern of infiltration
- Also hyalinized area, perivascular lymphocytes in onionskin pattern, intralesional hemorrhage
- Often foci of DFSP
- No histiocyte-like cells, no mitotic figures
Microscopic (histologic) images
AFIP images
Ectatic pseudovascular spaces are lined by giant cells
Stroma has giant cells and myxoid stroma
Stroma is fibrotic
with giant cells
but sparse
angiectoid spaces
Multinucleated
stromal giant cells
have vesicular nuclei
and prominent nucleoli
Contributed by Mark R. Wick, M.D.
Breast skin
Images hosted on other servers:
Trunk tumor
Cytology description
- Moderately cellular smears with mononuclear cells, usually single but occasionally in clusters
- Most cells have no/scanty cytoplasm, bland nuclei with small nucleoli
- Nuclear membranes often have notches, creases or folds
- Rare multinucleated giant cells with bland oval nuclei
- No necrosis, no mitotic figures (Diagn Cytopathol 2002;26:398, Arch Pathol Lab Med 2001;125:1091)
Positive stains
- Vimentin, CD34, CD99 (40%, J Cutan Pathol 2008;35:647)
- Variable actin
Electron microscopy description
- Myofibroblasts or fibroblasts
Molecular / cytogenetics description
- t(17,22)(q22;q13) - creates fusion of collagen type 1 alpha 1 gene and platelet derived growth factor B chain gene
- Also supernumerary ring chromosomes derived from t(17;22)
Differential diagnosis
- Angiosarcoma: older adults in head and neck, vascular tumor, cells have enlarged atypical nuclei, mitotic figures present
- Hemangioma: vascular tumor, no giant cells, CD31+
- Neurofibroma with ancient change: no ectatic vascular spaces, S100+
- Pleomorphic liposarcoma: pleomorphic cells are similar, but remaining lesion has lipoblasts and lacks collagenous matrix
Additional references
