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Biomedicines Feb 2022Giant cell tumor of bone (GCTB) and desmoplastic fibroma (DF) are bone sarcomas with intermediate malignant behavior and unpredictable prognosis. These locally...
A Rationale for the Activity of Bone Target Therapy and Tyrosine Kinase Inhibitor Combination in Giant Cell Tumor of Bone and Desmoplastic Fibroma: Translational Evidences.
Giant cell tumor of bone (GCTB) and desmoplastic fibroma (DF) are bone sarcomas with intermediate malignant behavior and unpredictable prognosis. These locally aggressive neoplasms exhibit a predilection for the long bone or mandible of young adults, causing a severe bone resorption. In particular, the tumor stromal cells of these lesions are responsible for the recruiting of multinucleated giant cells which ultimately lead to bone disruption. In this regard, the underlying pathological mechanism of osteoclastogenesis processes in GCTB and DF is still poorly understood. Although current therapeutic strategy involves surgery, radiotherapy and chemotherapy, the benefit of the latter is still debated. Thus, in order to shed light on these poorly investigated diseases, we focused on the molecular biology of GCTB and DF. The expression of bone-vicious-cycle- and neoangiogenesis-related genes was investigated. Moreover, combining patient-derived primary cultures with 2D and 3D culture platforms, we investigated the role of denosumab and levantinib in these diseases. The results showed the upregulation of , and and the downregulation of and genes, underlining their involvement and promising role in these neoplasms. Furthermore, in vitro analyses provided evidence for suggesting the combination of denosumab and lenvatinib as a promising therapeutic strategy in GCTB and DF compared to monoregimen chemotherapy. Furthermore, in vivo zebrafish analyses corroborated the obtained data. Finally, the clinical observation of retrospectively enrolled patients confirmed the usefulness of the reported results. In conclusion, here we report for the first time a molecular and pharmacological investigation of GCTB and DF combining the use of translational and clinical data. Taken together, these results represent a starting point for further analyses aimed at improving GCTB and DF management.
PubMed: 35203581
DOI: 10.3390/biomedicines10020372 -
Indian Journal of Otolaryngology and... Dec 2022Desmoplastic fibroma is a benign, rare, but locally aggressive lesion. The intraosseous type rarely presents in the jaws. Desmoplastic fibroma represents the...
Desmoplastic fibroma is a benign, rare, but locally aggressive lesion. The intraosseous type rarely presents in the jaws. Desmoplastic fibroma represents the intraosseous counterpart of the soft tissue fibromatoses or desmoid tumor, affects predominantly young people with an affinity for the mandible. The aim of this article is to describe a rare case of spontaneous bone regeneration after resection of intraosseous type of Desmoplastic fibroma. We report a case of intraosseous Desmoplastic fibroma involving right body and ramus of the mandible of a 17 years old, male patient, that underwent surgical procedure by Risdon access to remove all lesion with 1 cm free margins and reconstructed with 2.4 mm plate. After 3 years follow up, spontaneous bone regeneration was found with no signs of recurrence of the lesion. In conclusion, cases of spontaneous bone regeneration after mandibular resection is rare and there are few studies and case report in the literature.
PubMed: 36742857
DOI: 10.1007/s12070-021-02608-7 -
International Journal of Surgery Case... 2013The desmoplastic fibroma is a rare locally invasive bone tumour. Surgical resection with minimal margins is recommended.
INTRODUCTION
The desmoplastic fibroma is a rare locally invasive bone tumour. Surgical resection with minimal margins is recommended.
PRESENTATION OF CASE
A 15 year-old boy was referred with chronic left thigh pain. MRI revealed a bone lesion within the cavity of the inner table of the left iliac wing without invasion of the underlying bone marrow. A surgical biopsy revealed a desmoplastic bone fibroma. A partial resection of the inner table of the iliac wing sparing the outer table was performed. At the latest follow-up the initially spared iliac wing had needed further resection. The reason proposed for this is devascularisation by substantial periosteal stripping causing partial resorption initially, then necrosis and ultimately ulceration through the skin necessitating further surgical resection.
DISCUSSION
The technique of resection of a pelvic desmoplastic fibroma sparing the outer table of the iliac wing has not previously been reported. The objective of a limited resection was to minimize the risk of a postsurgical limp caused by weakness of the gluteus medius muscle. However we report that this technique did not work in this case. A wider resection of the iliac wing as it is recommended for a malignant tumour would have yielded a similar final outcome.
CONCLUSION
A partial resection of the iliac wing seemed an appealing technique for a benign tumour of the inner table of the iliac wing. However, considering the complications encountered, the authors advise a simple "en bloc" resection of the iliac wing for this type of tumour in this location.
PubMed: 23973899
DOI: 10.1016/j.ijscr.2013.06.016 -
Head and Neck Pathology Jun 2015The desmoplastic fibroma (DF) is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Although classified as benign, it... (Comparative Study)
Comparative Study Review
The desmoplastic fibroma (DF) is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. We present three cases of DF in the mandible: the first in a 13 year old female involving the mandibular body in the region of teeth #s 27-#28, the second in a 57 year old female with a lesion apical to tooth #30, and the third in a 20-year-old female involving the left posterior mandible. Clinical, histologic, immunohistochemical (IHC) and radiographic features of this rare neoplasm are discussed. The challenges encountered in establishing an accurate diagnosis due to significant microscopic overlap with other spindle cell lesions are also detailed. Additionally, the findings of IHC stains including vimentin, smooth muscle actin, S-100 protein, β-catenin, HHF-35 and proliferation marker, Ki-67 on 3 cases are reported. The potential for misdiagnosis is high, especially in early lesions, since immunohistochemistry has been reported in literature to be inconsistent when differentiating DFs from other spindle cell lesions. A comparative review of DF and similar entities in the jaws with current considerations in treatment and prognosis is presented.
Topics: Actins; Adolescent; Bone Neoplasms; Female; Fibroma, Desmoplastic; Humans; Mandibular Neoplasms; Middle Aged; S100 Proteins; Vimentin; Young Adult; beta Catenin
PubMed: 25113037
DOI: 10.1007/s12105-014-0561-5 -
Thoracic Cancer Nov 2021Desmoplastic fibroblastoma is an extremely rare benign soft tissue tumor and desmoplastic fibroblastoma originating from the diaphragm has not been documented...
Desmoplastic fibroblastoma is an extremely rare benign soft tissue tumor and desmoplastic fibroblastoma originating from the diaphragm has not been documented previously. In our case, we report the first primary diaphragm desmoplastic fibroblastoma.
Topics: Diagnosis, Differential; Diaphragm; Female; Fibroma, Desmoplastic; Humans; Middle Aged; Soft Tissue Neoplasms; Tomography, X-Ray Computed
PubMed: 34581000
DOI: 10.1111/1759-7714.14168 -
Indian Journal of Orthopaedics Mar 2013Desmoplastic fibroma is a rare, well differentiated, locally aggressive fibrous tumor usually arising from soft tissues, and rarely from bones. Involvement of forearm...
Desmoplastic fibroma is a rare, well differentiated, locally aggressive fibrous tumor usually arising from soft tissues, and rarely from bones. Involvement of forearm bones is extremely unusual. We present a large desmoplastic fibroma of right ulna in a 15-year-old male. The tumor was excised with a wide margin, and the bony defect was reconstructed with nonvascular autologous fibular graft. Reconstruction of the olecranon process was attempted using the fibular head and the remaining olecranon. At 2-years followup, there was no recurrence, flexion extension arc of the elbow joint was 40°-130° and there was no restriction of activities of daily living.
PubMed: 23682186
DOI: 10.4103/0019-5413.108928 -
Journal of Ultrasound Mar 2014Collagenous fibroma (desmoplastic fibroblastoma) is a recently described rare benign tumor. Since this tumor was characterized by Evans in 1995, fewer than 100 cases...
Collagenous fibroma (desmoplastic fibroblastoma) is a recently described rare benign tumor. Since this tumor was characterized by Evans in 1995, fewer than 100 cases have been reported in English literature with the largest series published by Miettinen and Fetsch. Preoperative radiological diagnosis is important to avoid over-treatment and unnecessary extensive procedures, but is difficult because diagnostic imaging findings for collagenous fibroma have not been established. Here we report the US and MRI appearance of a lesion localized deep under the deltoid muscle in a 34-year-old woman with a palpable painless mass. Histological findings were consistent with a collagenous fibroma. As far as we know, this is the first description of US appearance of a collagenous fibroma in English literature.
PubMed: 24616744
DOI: 10.1007/s40477-013-0051-8 -
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 Stomatology, Oral and... Feb 2024Desmoplastic fibroma (DF) is a rare benign bone tumor adopting an aggressive behavior, representing a challenge for clinical and radiographic diagnosis. This case report...
Desmoplastic fibroma (DF) is a rare benign bone tumor adopting an aggressive behavior, representing a challenge for clinical and radiographic diagnosis. This case report focused on a 31-year-old man with a large mandibular lesion with severe displacements of the mandibular teeth. Only a combination of paraclinical findings allows a definitive diagnosis to be made. Cervicofacial MRI revealed a low T1 signal intensity with peripheral enhancement after Gadolinium, and T2 hyperintense signal, while PET scan showed a moderate metabolism. Bone biopsy with immunohistochemical analysis allowed for definitive diagnosis of DF after eliminating the main differential diagnosis (fibrous dysplasia, fibrosarcoma, desmoid tumor, and osteosarcoma). The patient was successfully treated by large mandibular resection and reconstruction with a free-fibular bone flap".
PubMed: 38431083
DOI: 10.1016/j.jormas.2024.101805 -
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