DermKnowledgeBASE: Giant Cell Fibroblastoma

Giant Cell Fibroblastoma

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The text is the summary of recent articles on Giant Cell Fibroblastoma at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.

Giant cell fibroblastoma was excluded by a negative COL1A1/ PDGFB fusion by reverse transcriptase-polymerase chain reaction [1]. All displayed triphasic Morphology, but varied widely in the relative percentages of fat, fibroblastic fascicles, and primitive mesenchyme [2]. Eight cases of malignancies have been reported in young patients with Kabuki syndrome although a causative association to the syndrome has not been established [3]. However, local Recurrence after apparently adequate Surgical Excision is well recognized [4]. Histologically, spindle cell lipoma/pleomorphic lipoma consists of varying quantity of mature fat, bland spindle Cells and ropey collagen [5].

Herein, we report a peculiar case of Pleomorphic lipoma occurring in Axilla with entirely devoid of mature fat in a 71-year-old male [6]. Amongst the 16 cases studied, 12 occurred de novo and 4 represented local Recurrence [7]. Histologically, they were poorly circumscribed and located in the Dermis, with focal Infiltration into the underlying Subcutaneous tissue [8]. It has high incidence of local Recurrence but Metastasis has never been reported [9]. giant cell fibroblastoma merits attentive interpretation as they have been misdiagnosed as Sarcoma in the past leading to erroneous remedies [10].

Accordingly, it has been suggested that their detection, especially in an otherwise typical neurofibroma, could be a morphological clue to diagnosis of NF1 [11]. The aim of the present study was the identification of MNFGCs in a large series (94 cases) of sporadic and NF1-associated neurofibromas, to assess if their presence may indeed be a morphological marker of NF1 [12]. All displayed Pleomorphic and multinucleated floret cells interspersed among bland spindle Cells and ropey collagen [13]. This same fusion gene is also seen in the supernumerary ring chromosome of dermatofibrosarcoma protuberans (DFSP) [14]. Immunohistochemistry was performed using the ABC method, and three cases were studied by polymerase chain reaction technique [15].

Polymerase chain reaction technique showed in one case the characteristic COL1A1-PDGFB fusion gene [16]. It often presents a diagnostic challenge and is important to recognize to prevent both undertreatment and overtreatment [17]. Clinical follow-up in 8 cases, ranging from 3-21 years, (median follow-up 6 years), revealed local Recurrence in 2 cases and no evidence of Metastasis [18]. They proposed a relationship of this childhood tumor to dermatofibrosarcoma protuberans (DFSP) [19]. Thirty-nine GCF cases with evaluable epidermis were observed to be protuberant, one with superficial ulceration [20].

Most cases were Dermal and subcutaneous, 3 purely Dermal, and 5 involved superficial skeletal Muscle [21]. Almost all cases demonstrated a honeycomb pattern, and several, a parallel pattern of infiltration [22]. The COL1A1-PDGFB fusion gene is detectable either by multiplex RT-PCR with a combination of forward primers designed from a variety of COL1A1 exons and one reverse primer for PDGFB exon 2, or by in situ fluorescence hybridization (FISH) on interphase nuclei from frozen or fixed paraffin-embbeded sections [23]. It seems to be the Juvenile form of dermatofibrosarcoma protuberans, which is seen less frequently in Children [24].

References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 ,

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I have varied research interests ranging from eHealth, Health Information Exchange, Clinical Trials and Research, Contact Dermatitis, Bioinformatics, and Cosmetic Dermatology. I have work experience in Canada as an eHealth analyst, and in Dubai and India as a Specialist Dermatologist.


Bell Raj Eapen
Hamilton, ON