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Revue de Stomatologie Et de Chirurgie... Dec 2009Desmoplastic fibroma is a rare benign intraosseous neoplasms. They can affect the jaw. Posterior mandibular bone involvement is the most frequent localization. They are... (Review)
Review
Desmoplastic fibroma is a rare benign intraosseous neoplasms. They can affect the jaw. Posterior mandibular bone involvement is the most frequent localization. They are locally aggressive and recurrence is frequent. Radioclinical signs are not specific and the histological diagnosis may be difficult. Extended surgical removal is the recommended treatment.
Topics: Curettage; Diagnosis, Differential; Fibroma, Desmoplastic; Humans; Jaw Neoplasms; Neoplasm Recurrence, Local
PubMed: 19828163
DOI: 10.1016/j.stomax.2009.07.007 -
In Vivo (Athens, Greece) 2021Desmoplastic fibroblastoma (also known as collagenous fibroma) is an uncommon benign fibroblastic/myofibroblastic neoplasm that primarily arises in the subcutaneous... (Review)
Review
Desmoplastic fibroblastoma (also known as collagenous fibroma) is an uncommon benign fibroblastic/myofibroblastic neoplasm that primarily arises in the subcutaneous tissue of upper extremity. Magnetic resonance imaging reveals a well-defined mass in intimate association with dense connective tissue and prominent low signal intensity on all pulse sequences. Peripheral and septal enhancement is usually seen after intravenous contrast. Histologically, the lesion is paucicellular and consists of spindle to stellate-shaped cells embedded in a collagenous or myxocollagenous stroma with low vascularity. Diffuse and strong nuclear immunoreactivity for FOS-like antigen 1 seems to be characteristic of desmoplastic fibroblastoma. Cytogenetic studies have demonstrated the presence of 11q12 rearrangements and an identical t(2;11)(q31;q12) translocation. This review provides an updated overview of the clinical, radiological, histological, cytogenetic and molecular genetic features of desmoplastic fibroblastoma and discusses the relationship to fibroma of tendon sheath.
Topics: Fibroma; Fibroma, Desmoplastic; Humans; Magnetic Resonance Imaging; Soft Tissue Neoplasms; Translocation, Genetic
PubMed: 33402451
DOI: 10.21873/invivo.12233 -
Oral Surgery, Oral Medicine, Oral... Aug 2019Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder that affects the skin, brain, kidneys, and other organ systems. It may exhibit a wide spectrum... (Review)
Review
Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder that affects the skin, brain, kidneys, and other organ systems. It may exhibit a wide spectrum of clinical manifestations. Desmoplastic fibroma (DF) of the jaw is a rare benign myofibroblastic neoplasm. Less than 10 cases of DF associated with TSC have been published previously. We report a new case of a maxillary DF in a 12-year-old girl with TSC. The presentation, diagnostic process, and management of this case are discussed, and the literature is reviewed for the additional cases of DF associated with TSC; 7 previously reported cases are summarized. Small sample size limits conclusions, but there may be differences in the presentations of DF of the jaws in patients with TSC vs those in the general population. DF of the jaws may be a manifestation of TSC, and the authors propose surveillance panoramic radiographs every 2 to 3 years in patients with TSC.
Topics: Child; Female; Fibroma, Desmoplastic; Humans; Tuberous Sclerosis
PubMed: 31078508
DOI: 10.1016/j.oooo.2019.03.008 -
Head & Neck 1989Desmoplastic fibroma of the jaws is a rare and locally aggressive tumor that has a high rate of recurrence. The cellularity, extent of the tumor, and completeness of the... (Review)
Review
Desmoplastic fibroma of the jaws is a rare and locally aggressive tumor that has a high rate of recurrence. The cellularity, extent of the tumor, and completeness of the local excision may be factors in its tendency to recur. We reviewed the literature in an attempt to determine if there was any correlation between the histologic features of a desmoplastic fibroma, or the surgical procedure utilized, and the tumor's recurrence. From the information gathered, it was found that a more cellular desmoplastic fibroma or inadequate surgical procedure may be factors that contribute to the recurrence of the tumor. A case report of desmoplastic fibroma of the mandible in a 9-year-old child is presented. The tumor, which displayed areas of moderate cellularity, was resected via a partial hemimandibulectomy. There has been no recurrence to date (46 months).
Topics: Adolescent; Adult; Child; Child, Preschool; Fibroma; Humans; Jaw Neoplasms; Male; Mandibular Neoplasms; Middle Aged; Radiography
PubMed: 2490064
DOI: 10.1002/hed.2880110112 -
Journal of Bone Oncology Feb 2021Desmoplastic fibroma (DF) is an intraosseous counterpart of desmoid-type soft tissue fibromatosis. It is most frequently seen in the jawbones. The clinical and...
Desmoplastic fibroma (DF) is an intraosseous counterpart of desmoid-type soft tissue fibromatosis. It is most frequently seen in the jawbones. The clinical and radiological features of the present cases were nonspecific. The accumulation of beta-catenin in the nuclei of neoplastic cells which is a diagnostic feature of desmoid-type soft tissue fibromatosis could not be detectED in the present DF series. The aim of this study is to report a series of 22 cases of DF involving either mandible or maxilla. A retrospective evaluation of desmoplastic fibroma and beta-catenin, smooth muscle actin, nestin, cyclin D1 immunostaining's patterns. Most of the DF cases expressed only cytoplasmic beta-catenin immunostainings. We suggest that nuclear beta-catenin staining may not be used as a corroborating the diagnosis of DF. Immunohistochemical staining difference of jaw bone desmoplastic fibromas from other soft tissue and bone lesions may be related to the origination of jaw bone from The neural crest. Strong nestin and cyclin D1 positivity in our series supported this. A combined clinical, radiological, and histopathological analysis of the DF cases is essential in the diagnosis and management.
PubMed: 33204607
DOI: 10.1016/j.jbo.2020.100333 -
European Journal of Orthopaedic Surgery... Dec 2015Desmoplastic fibroma is a rare benign fibrogenic, locally aggressive, primary bone tumor. It is the intraosseous counterpart of soft tissue aggressive fibromatosis. The... (Review)
Review
Desmoplastic fibroma is a rare benign fibrogenic, locally aggressive, primary bone tumor. It is the intraosseous counterpart of soft tissue aggressive fibromatosis. The lesion may very rarely appear as a superficial bone lesion arising from the periosteum; in such cases, a soft tissue mass with changes in the adjacent bone is evident. Periosteal lesions are very rare in the literature; diagnosis is usually based on the radiographic findings, and histological proof of the tumor origin is missing. A periosteal desmoplastic fibroma of the distal tibial metaphysis in a 3-year-old boy is presented. Radiographic investigation included plain radiographs and computed tomography imaging. Both demonstrated a soft tissue lesion involving the superficial bone tissues with non-aggressive looking borders and a pressure effect with a sclerotic rim in the bone. The lesion was excised, and the surgical as well as the histological findings indicated the diagnosis of a desmoplastic fibroma of bone arising from the periosteum. No recurrence was detected 5 years after surgery.
Topics: Bone Neoplasms; Child, Preschool; Fibroma, Desmoplastic; Humans; Male; Musculoskeletal Pain; Radiography; Tibia; Treatment Outcome
PubMed: 26265404
DOI: 10.1007/s00590-015-1680-8 -
Neurological Sciences and Neurosurgery 2020Desmoplastic fibromas are rare benign bone tumors occurring primarily in long bones and mandible. In this case report, we present a desmoplastic fibroma originating from...
Desmoplastic fibromas are rare benign bone tumors occurring primarily in long bones and mandible. In this case report, we present a desmoplastic fibroma originating from the left frontal bone. This is an exceptionally rare presentation of this pathology and the associated imaging and pathologic slides are highly educational. We discuss the relevance to the literature and how to manage these patients clinically.
PubMed: 33103158
DOI: 10.47275/2692-093x-108 -
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 -
The Journal of the Michigan Dental... Mar 2014Several odontogenic and non-odontogenic tumors and related lesions affecting the jaws share clinical presentation and imaging appearance. However, all cases have to be... (Review)
Review
Several odontogenic and non-odontogenic tumors and related lesions affecting the jaws share clinical presentation and imaging appearance. However, all cases have to be approached and treated on an individual basis, based on a reliable anatomic pathology diagnosis. The present paper describes a case of a desmoplastic fibroma, which was misdiagnosed as an odontogenic keratocyst, and spotlights the potential benefit and conclusive role of a second opinion in oral and maxillofacial pathology diagnosis. A brief review of the literature is also provided.
Topics: Aged; Biopsy; Collagen; Cone-Beam Computed Tomography; Diagnostic Errors; Female; Fibroblasts; Fibroma, Desmoplastic; Follow-Up Studies; Humans; Mandibular Diseases; Mandibular Neoplasms; Odontogenic Cysts; Radiography, Panoramic
PubMed: 24961082
DOI: No ID Found -
Child's Nervous System : ChNS :... Dec 2013Desmoplastic fibromas are primary bone tumors that seldom occur in the cranial bones. Furthermore, reports of desmoplastic fibromas of the skull in children are... (Review)
Review
PURPOSE
Desmoplastic fibromas are primary bone tumors that seldom occur in the cranial bones. Furthermore, reports of desmoplastic fibromas of the skull in children are exceedingly rare. Although desmoplastic fibromas are histologically benign, they are locally aggressive and have a propensity to reoccur. Their radiographic appearance may mimic other more common central nervous system and bone neoplasms. There are only 19 reported cases of desmoplastic fibroma of the cranium in the literature, and only seven occurred in the pediatric age group. We present a case report of an 11-year-old female patient with a desmoplastic fibroma of the parieto-occipital region and review the literature.
CASE REPORT
An 11-year-old female presented to the craniofacial clinic complaining of intermittent pain and a soft mass in the occipital region. There was a distant history of trauma to the region that did not require medical intervention. Computed tomography imaging revealed a lytic bone lesion overlying the sagittal sinus in the parieto-occipital region. Surgical resection with wide margins and immediate autologous reconstruction was performed. Pathological analysis revealed a desmoplastic fibroma. At 4 months of follow-up, no recurrence has been noted.
CONCLUSION
Desmoplastic fibroma of the cranium is rare. Complete surgical resection with careful follow-up is the treatment of choice.
Topics: Child; Female; Fibroma, Desmoplastic; Humans; Skull Neoplasms
PubMed: 23812628
DOI: 10.1007/s00381-013-2210-9