-
The Open Orthopaedics Journal 2013Desmoplastic fibroma (DF) is an extremely rare locally aggressive bone tumor with an incidence of 0.11% of all primary bone tumors. The typical clinical presentation is...
BACKGROUND
Desmoplastic fibroma (DF) is an extremely rare locally aggressive bone tumor with an incidence of 0.11% of all primary bone tumors. The typical clinical presentation is pain and swelling above the affected area. The most common sites of involvement are the mandible and the metaphysis of long bones. Histologically and biologically, desmoplastic fibroma mimics extra-abdominal desmoid tumor of soft tissue.
CASE PRESENTATION AND LITERATURE REVIEW
A case of a 27-year old man with DF in the ilium, including the clinical, radiological and histological findings over a 4-year period is presented here. CT scans performed in 3-year intervals prior to surgical intervention were compared with respect to tumor extension and cortical breakthrough. The patient was treated with curettage and grafting based on anatomical considerations. Follow-up CT scans over 18-months are also documented here. Additionally, a review and analysis of 271 cases including the presented case with particular emphasis on imaging patterns in MRI and CT as well as treatment modalities and outcomes are presented.
CONCLUSION
In patients with desmoplastic fibroma, CT is the preferred imaging technique for both the diagnosis of intraosseus tumor extension and assessment of cortical involvement, whereas MRI is favored for the assessment of extraosseus tumor growth and preoperative planning. While tumor resection remains the preferred treatment for DF, curettage and grafting prove to be an acceptable alternative treatment modality with close follow-up when resection is not possible. Curettage and grafting have been shown to provide good clinical results and are associated with long recurrence free intervals.
PubMed: 23459513
DOI: 10.2174/1874325001307010040 -
Case Reports in Oncology 2023Desmoplastic fibroma is a rare primary benign bone tumour that typically affects the long bones, mandible, and pelvis. It has a similar local aggressiveness to soft...
Desmoplastic fibroma is a rare primary benign bone tumour that typically affects the long bones, mandible, and pelvis. It has a similar local aggressiveness to soft tissue fibromatosis. It rarely involves the small bones of the hand. We describe an extremely rare case of desmoplastic fibroma of the proximal phalanx of the hand in a patient who presented with an aggressively enlarging but painless mass on the left ring finger. Radiological features suggested malignancy; however, an initial biopsy revealed fibrotic tissue. Trans-metacarpal amputation of the ring and little fingers and soft tissue reconstruction were performed using a local ulnar-based flap of the little finger. The final histopathological evaluation revealed desmoplastic fibroma. Aggressively growing masses in the hand should be treated according to a sarcoma management protocol, and desmoplastic fibroma should be included in the differential diagnosis.
PubMed: 37485013
DOI: 10.1159/000529895 -
Journal of Medical Case Reports Jan 2023Collagenous fibroma or desmoplastic fibroblastoma is a rare benign fibrous tissue tumor. It usually presents as a painless, slowly growing mass. Collagenous fibroma... (Review)
Review
BACKGROUND
Collagenous fibroma or desmoplastic fibroblastoma is a rare benign fibrous tissue tumor. It usually presents as a painless, slowly growing mass. Collagenous fibroma arises ordinarily inside the subcutaneous tissues or skeletal muscles. Histopathologically, the tumor consists of scattered stellate and spindle cells in a hypovascular collagenous stroma without atypia or infiltration. The oral cavity is a very uncommon site for desmoplastic fibroblastoma. Only 15 published articles in the literature reported the intraoral location. We present a case of collagenous fibroma with a bilateral distribution on the hard palate. This is the second case of bilateral collagenous fibroma after a previously reported one in literature; however, our case was larger, occupying almost the whole palate. We discuss the management of this rare tumor and how we can reach definite diagnosis.
CASE PRESENTATION
A 37-year-old Caucasian female patient had a huge bilateral firm palatal mass that caused breathing problems. There was no history of trauma and the patient had no relevant medical history Total surgical excision under general anesthesia was carried out and histopathological examination suggested a benign mesenchymal tumor. Immunohistochemistry was necessary to confirm the tumor origin and to exclude aggressive fibromatosis. A diagnosis of bilateral collagenous fibroma was reached. Six months after surgery, there was no recurring lesion and the patient's health was good.
CONCLUSIONS
Collagenous fibroma is a benign fibrous tissue tumor of unknown cause that is treated with simple excision. The prognosis is good with no recurrence. Reaching an accurate diagnosis is mandatory to avoid aggressive treatment since collagenous fibroma may be misdiagnosed as aggressive fibromatosis in case of massive size. Clinicians and pathologists should be aware of this unusual tumor for conservative management without side effects.
Topics: Humans; Female; Adult; Fibroma, Desmoplastic; Fibromatosis, Aggressive; Palate, Hard; Fibroma; Soft Tissue Neoplasms
PubMed: 36609451
DOI: 10.1186/s13256-022-03691-2 -
Journal of Medical Case Reports Jan 2011Desmoplastic fibroma is an extremely rare primary benign bone tumor. It occurs most often in the mandible, followed by the femur and pelvis. To the best of our...
INTRODUCTION
Desmoplastic fibroma is an extremely rare primary benign bone tumor. It occurs most often in the mandible, followed by the femur and pelvis. To the best of our knowledge, fewer than 200 cases have been described in the published literature. Furthermore, this case is the first report of desmoplastic fibroma with simultaneous presentation in two different locations.
CASE PRESENTATION
We present an unusual case of desmoplastic fibroma in a 56-year-old Caucasian man, who presented to our hospital with lumbar pain. Computed tomography and magnetic resonance imaging were performed, demonstrating two lytic expansile lesions affecting both his left iliac bone and his left sacral wing. Curettage and cortical-cancellous grafting was performed, followed by postoperative computed tomography and magnetic resonance imaging.
CONCLUSION
Desmoplastic fibroma with unusual and simultaneous manifestations in two different locations has never been reported previously to the best of our knowledge. The purpose of this case report is to present the computed tomography and magnetic resonance imaging features of this rare tumor before and after the surgical treatment. Furthermore, the radiological findings with the description of the characteristics and the clinical presentation of this rare tumor, contribute to the wide spectrum of manifestations of this tumor, in order to recognize it and to have the appropriate management.
PubMed: 21261947
DOI: 10.1186/1752-1947-5-28 -
Oncology Letters Feb 2016Desmoplastic fibroma, alternatively known as aggressive fibromatosis or desmoid tumors, occurs in the form of benign locally aggressive tumors that possess a high rate...
Desmoplastic fibroma, alternatively known as aggressive fibromatosis or desmoid tumors, occurs in the form of benign locally aggressive tumors that possess a high rate of recurrence. The forearm bones are rarely involved. The current study presents a case of desmoplastic fibroma in the distal forearm of a 23-year-old man. The tumor was widely resected, and the bone defect was reconstructed using an autologous vascularized fibular graft during the resection procedure. The patient experienced recurrence three times and underwent four resections during the subsequent 3 years following the initial resection. After 10 years of follow-up, the patient's functional recovery remains positive. Despite the implication that surgical resection may be involved in the development of aggressive fibromatosis, surgical wide local excision and functional reconstruction were recommended for the treatment of the present patient.
PubMed: 26893769
DOI: 10.3892/ol.2015.4058 -
Journal of Oral Science Sep 2005Intra-osseous fibromas of the jaw are classified by origin. Intra-osseous odontogenic fibromas have odontogenic epithelia, while desmoplastic fibromas do not. However,... (Review)
Review
Intra-osseous fibromas of the jaw are classified by origin. Intra-osseous odontogenic fibromas have odontogenic epithelia, while desmoplastic fibromas do not. However, it is often difficult to determine the odontogenic origin for central fibromas. Three subjects with a diagnosis of intra-osseous fibroma were examined. Case 1 was a 35-year-old man found to have a panoramic radiograph from the right premolar to the mandibular ramus in the mandible that exhibited multilocular radiolucency. Within the radiolucency, small-radioopaque bodies were observed. Case 2 was a 13-year-old female, in whom a panoramic radiograph from the left premolar to the molar in the mandible showed multilocular radiolucency. Case 3 was a 51-year-old female who exhibited a heart-shaped radiolucency in the panoramic radiograph of the left first molar area in the mandible. We also reviewed the literature for previously reported cases of intra-osseous odontogenic and desmoplastic fibroma. In 64 cases of intra-osseous odontogenic fibroma and 68 cases of desmoplastic fibroma we extracted data on age, sex, location, and radiographic findings. Based on the analysis of the reported literature cases, re-evaluation of the patients in our study revealed that case 1 could be classified as desmoplastic fibroma, while cases 2 and 3 were intra-osseous odontogenic fibromas.
Topics: Adolescent; Adult; Bicuspid; Cell Nucleus; Collagen; Diagnosis, Differential; Female; Fibroblasts; Fibroma, Desmoplastic; Humans; Male; Mandibular Neoplasms; Middle Aged; Molar; Odontogenic Tumors; Radiography, Panoramic; Tomography, X-Ray Computed
PubMed: 16313094
DOI: 10.2334/josnusd.47.149 -
Journal of Surgical Case Reports Nov 2011Desmoplastic fibromas are rare, benign bone tumours, which often behave in a locally aggressive manner. In the head and neck region they are most commonly seen in the...
Desmoplastic fibromas are rare, benign bone tumours, which often behave in a locally aggressive manner. In the head and neck region they are most commonly seen in the mandible and have been treated in various ways. We present an unusual presentation of the lesion, which was subsequently treated by the less commonly used technique of enucleation, with good results.
PubMed: 24972395
DOI: 10.1093/jscr/2011.11.5 -
Journal of Oral and Maxillofacial... 2023Spindle cell lesions comprise a vast plethora of benign and malignant lesions with similar clinical and radiographic features. Their overlapping histopathologic features...
CONTEXT
Spindle cell lesions comprise a vast plethora of benign and malignant lesions with similar clinical and radiographic features. Their overlapping histopathologic features ensure a diagnostic dilemma.
AIM
The current multicentric study aims to delineate fibroblastic and myofibroblastic oral spindle cell lesions based on cytomorphology and comprehensive immunohistochemical analysis.
SETTINGS AND DESIGN
The experimental study was conducted at MS Ramaiah University of Applied Sciences, Bangalore, and All India Institute of Applied Sciences, Delhi.
METHODS AND MATERIAL
A comprehensive histological scoring criteria and panel of immunohistochemical makers (STAT6, CD31, CD34, S100, SMA, vimentin, pan-CK, HHF-35, Ki67, ALK, desmin, HMB-45, SATB2, ERG, EMA and CD99) were employed concurrently for the first time for fibroblastic and myofibroblastic oral spindle cell lesions. The data obtained was tabulated and studied.
STATISTICAL ANALYSIS USED
Results: Using cytological scoring criteria and panel of immunohistochemical makers, the cases analysed and characterized were desmoplastic fibroma, fibrosarcoma, leiomyosarcoma, nodular fasciitis, neurofibroma and epithelioid inflammatory myofibroblastic sarcoma (EIMS).
CONCLUSIONS
The diagnostic strategies need to be upgraded for the diagnosis of spindle cell lesions. Emphasis must be placed on cytomorphology, an immunohistochemistry (IHC) panel of markers is imperative for the accurate diagnosis of fibroblastic and myofibroblastic oral spindle cell lesions.
PubMed: 38304501
DOI: 10.4103/jomfp.jomfp_282_23 -
Journal of Oral and Maxillofacial... 2015Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm, that is, recognized as the intra-osseous counterpart of soft tissue fibromatosis in both gnathic and...
Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm, that is, recognized as the intra-osseous counterpart of soft tissue fibromatosis in both gnathic and extra-gnathic sites. It has a propensity for locally aggressive behavior and local recurrence. An occurrence of intra-osseous lesion other than that of odontogenic origin is rare in the jaws. In this case report, we define the clinico-pathological and radiographic features of DF of the mandible in a 35-year-old female, who presented to the Outpatient Department with a 3-year history of a slowly expanding painless mass in the left mandibular posterior region. Thus, we present a classic case of DF exhibiting characteristic features along with a review of the literature.
PubMed: 26604517
DOI: 10.4103/0973-029X.164564 -
Medical Ultrasonography Sep 2015Desmoplastic fibroma (DF) is a very rare bone tumor, classified as a benign lesion. It might be locally a very aggressive tumor with a high rate of recurrence after... (Review)
Review
Desmoplastic fibroma (DF) is a very rare bone tumor, classified as a benign lesion. It might be locally a very aggressive tumor with a high rate of recurrence after surgical resection. We present a case of a 3-year and 6 month-old patient, with mandibular desmoplastic fibroma. The clinical, imaging, histological findings and surgical treatment are discussed and, also, a brief review of the literature of DF is presented. The purpose of this case report is to emphasize the particularity of the case, ultrasound findings and the main differential diagnostic of this rare bone tumor.
Topics: Child, Preschool; Fibroma, Desmoplastic; Humans; Male; Mandibular Neoplasms; Ultrasonography
PubMed: 26343094
DOI: 10.11152/mu.2013.2066.173.mmg