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Cureus Sep 2021Desmoplastic fibroma (DF) is a non-metastatic primary bone tumor that is extremely rare with local aggressive behavior. To the best of our knowledge, only few cases were...
Desmoplastic fibroma (DF) is a non-metastatic primary bone tumor that is extremely rare with local aggressive behavior. To the best of our knowledge, only few cases were published discussing this type of tumor and its management. This case report aimed to discuss a novel case as well as its management scheme. We present a case of a 36-year-old male with DF lesion involving the proximal tibial who underwent an extended curettage, triple type of adjuvant thereby, and internal fixation.
PubMed: 34660062
DOI: 10.7759/cureus.17857 -
Surgical Case Reports Apr 2021Desmoplastic fibroblastoma is an uncommon, benign, fibrous tumor exhibiting infiltrative growth. Most of these tumors are small, slow-growing, and develop as...
BACKGROUND
Desmoplastic fibroblastoma is an uncommon, benign, fibrous tumor exhibiting infiltrative growth. Most of these tumors are small, slow-growing, and develop as subcutaneous lesions in the extremities. Cases of desmoplastic fibroblastoma in the chest wall are quite rare, and the preoperative diagnosis of such cases remains challenging as these tumors can mimic the characteristics of desmoid-type fibromatosis, which often occurs in the chest wall. We aimed to describe a rare case of desmoplastic fibroblastoma exhibiting rapid growth in the chest wall of a patient that was successfully treated with marginal excision only by diagnostic imaging before surgery.
CASE PRESENTATION
A 79-year-old man was admitted to our hospital after experiencing right shoulder pain lasting for a few months. A 4 × 4 × 2 cm mass was incidentally detected at the right second rib two years prior. Chest computed tomography revealed a well-defined homogeneous mass with a muscle-like density along the right lateral chest wall, the size of which had increased to 12 × 10 × 4.5 cm in two years. Dynamic contrast-enhanced computed tomography revealed abundant vascularity at the periphery of the tumor. Magnetic resonance imaging revealed iso-intensity to muscle on T1-weighted images, slightly high intensity on T2-weighted images, and rim-like contrast enhancement at the periphery of the tumor, with uniform thickness on gadolinium-enhanced T1-weighted images with fat suppression. Rim-like contrast enhancement is an imaging feature that can distinguish cases of desmoplastic fibroblastoma from desmoid-type fibromatosis. We diagnosed the tumor as desmoplastic fibroblastoma by diagnostic imaging without tissue biopsy. Marginal excision with videoscopic assistance was performed through a small incision. The pathological diagnosis was desmoplastic fibroblastoma. The patient's postoperative course was uneventful, and his shoulder pain was relieved after the surgery.
CONCLUSIONS
Desmoplastic fibroblastoma in the chest wall is extremely rare, but should be considered in the differential diagnosis when desmoid-type fibromatosis is clinically suspected. Gadolinium-enhanced magnetic resonance imaging is helpful in confirming the differential diagnosis.
PubMed: 33829348
DOI: 10.1186/s40792-021-01171-1 -
ELife Jan 2017Desmoplasia, a fibrotic mass including cancer-associated fibroblasts (CAFs) and self-sustaining extracellular matrix (D-ECM), is a puzzling feature of pancreatic ductal...
Desmoplasia, a fibrotic mass including cancer-associated fibroblasts (CAFs) and self-sustaining extracellular matrix (D-ECM), is a puzzling feature of pancreatic ductal adenocarcinoma (PDACs). Conflicting studies have identified tumor-restricting and tumor-promoting roles of PDAC-associated desmoplasia, suggesting that individual CAF/D-ECM protein constituents have distinguishable tumorigenic and tumor-repressive functions. Using 3D culture of normal pancreatic versus PDAC-associated human fibroblasts, we identified a CAF/D-ECM phenotype that correlates with improved patient outcomes, and that includes CAFs enriched in plasma membrane-localized, active αβ-integrin. Mechanistically, we established that TGFβ is required for D-ECM production but dispensable for D-ECM-induced naïve fibroblast-to-CAF activation, which depends on αβ-integrin redistribution of pFAK-independent active αβ-integrin to assorted endosomes. Importantly, the development of a simultaneous multi-channel immunofluorescence approach and new algorithms for computational batch-analysis and their application to a human PDAC panel, indicated that stromal localization and levels of active SMAD2/3 and αβ-integrin distinguish patient-protective from patient-detrimental desmoplasia and foretell tumor recurrences, suggesting a useful new prognostic tool.
Topics: Biomarkers, Tumor; Cancer-Associated Fibroblasts; Carcinoma, Pancreatic Ductal; Cell Membrane; Extracellular Matrix; Fibroma, Desmoplastic; Humans; Integrin alpha5beta1; Transforming Growth Factor beta
PubMed: 28139197
DOI: 10.7554/eLife.20600 -
Dental Research Journal 2020Desmoplastic fibroma (DF) is a rare benign but aggressive fibrous lesion with an unknown etiology. It has an affinity for the mandible. DF has a high rate of recurrence...
Desmoplastic fibroma (DF) is a rare benign but aggressive fibrous lesion with an unknown etiology. It has an affinity for the mandible. DF has a high rate of recurrence after surgical resection. In this study, we report the therapeutic management of a recurrence of DF in the lower jaw in a 2-year-old boy. He responded well to second surgical intervention with wide resection and immediate reconstruction with plate. The purpose of this case report is to emphasize the particularity of the case, treatment modalities, and differential diagnosis in DF.
PubMed: 32774802
DOI: No ID Found -
Medicine Dec 2018Desmoplastic fibroma of the bone (DFB) is an extremely rare benign bone tumor, which can occur in any bone at all ages. Herein, we report a case of non-specific imaging...
RATIONALE
Desmoplastic fibroma of the bone (DFB) is an extremely rare benign bone tumor, which can occur in any bone at all ages. Herein, we report a case of non-specific imaging findings.
PATIENT CONCERNS
A 25-year-old female patient was consulted in the Armed Police General Hospital (Beijing, China) due to repeated pain in the right thigh lasting over 1 year. Imaging examination revealed a space-occupying lesion in the right femur.
DIAGNOSIS
Desmoplastic fibroma of the femur INTERVENTIONS:: Wide surgical resection OUTCOMES:: At the 1-year follow-up visit, no relapse in the tumor was observed.
LESSONS
In this case report, we described and emphasized the particularity of the case examined and the possible non-specificity of the imaging results of some DFB cases.
Topics: Adult; Female; Femoral Neoplasms; Femur; Fibroma, Desmoplastic; Humans; Magnetic Resonance Imaging; Radiography; Tomography, X-Ray Computed
PubMed: 30593162
DOI: 10.1097/MD.0000000000013787 -
Head and Neck Pathology Jun 2021A patient with an odontogenic fibroma (OF) with the presence of fibroblasts of varying morphology is presented, and the main microscopic differential diagnosis is...
A patient with an odontogenic fibroma (OF) with the presence of fibroblasts of varying morphology is presented, and the main microscopic differential diagnosis is discussed. Case report: a 51-year-old man complained of a swelling in the anterior region of the mandible. The panoramic radiograph showed a radiolucent, well-defined multilocular image, with displacement of the roots of the left mandibular incisors and canine. A cone beam computed tomography examination revealed a hypodense image, with delicate bone spicules inside, divergence of the tooth roots and destruction of the buccal cortical bone. Under the differential diagnosis of a desmoplastic ameloblastoma or a squamous odontogenic tumor, an incisional biopsy was made. Microscopically, deeply collagenized tissue with scarce odontogenic epithelium islands, large fibroblasts of varying morphology and calcified material was observed. A diagnosis of OF was made. Surgical removal was carried out, and the specimen was examined. Identical aspects were found, and the immunohistochemical reaction for pan-cytokeratin was carried out to identify the odontogenic epithelial cells. The definitive diagnosis of OF was confirmed, and we emphasize the importance of the correlation of all characteristics for a definitive diagnosis.
Topics: Fibroblasts; Fibroma; Humans; Male; Mandible; Middle Aged; Odontogenic Tumors
PubMed: 32865727
DOI: 10.1007/s12105-020-01207-2 -
Journal of Maxillofacial and Oral... Mar 2015Desmoplastic fibroma (DF) is a rare, well-differentiated fibrous tumor, which is slow in growth but has an aggressive potential. In its clinical and radiographic...
Desmoplastic fibroma (DF) is a rare, well-differentiated fibrous tumor, which is slow in growth but has an aggressive potential. In its clinical and radiographic presentation it mimics many unusual pathologies of the jaws. This case report presents a DF in the mandible, which in its clinical presentation and radiographic appearance mimicked a dentigerous cyst highlighting the importance of detailed histopathological examination in the diagnosis of jaw pathologies.
PubMed: 25838689
DOI: 10.1007/s12663-012-0386-2 -
Journal of Surgical Case Reports Jan 2016Desmoplastic fibromas are rare, benign, locally aggressive bone tumors, which arise primarily in patients younger than 30 years old. Historically, even with greater...
Desmoplastic fibromas are rare, benign, locally aggressive bone tumors, which arise primarily in patients younger than 30 years old. Historically, even with greater functional loss, en bloc or wide resection of the tumors to prevent local recurrence has been the method of choice in treatment. This article discusses the presentation of a 10-year-old male who presented with a mass in the distal forearm, after reporting difficulty in pronation and supination. The patient was ultimately treated with wide resection and allo-arthrodesis with allograft. Post-operatively, the patient has exhibited excellent recovery, including normal range of motion at the shoulder and elbow, and ability to perform all activities of daily livings despite reduced grip strength compared with the contralateral side.
PubMed: 26767767
DOI: 10.1093/jscr/rjv171 -
BMJ Case Reports Oct 2018Desmoplastic fibroblastoma (DF) is a rare benign soft tissue with spindle-to-stellate-shaped fibroblasts and myofibroblasts embedded in a prominent collagenous...
Desmoplastic fibroblastoma (DF) is a rare benign soft tissue with spindle-to-stellate-shaped fibroblasts and myofibroblasts embedded in a prominent collagenous background. DF, mainly affecting subcutaneous and muscle tissue, very rarely occurs in the oral cavity. Hitherto, only one case of DF on the tongue has been reported. Here, we report another case. A 66-year-old woman was referred to our hospital with a mass formation in the tongue. On examination, a well-circumscribed, elastic, soft tumour with normal surface mucosa, measuring 13×12 mm, was observed on the left dorsal surface of her tongue. MRI and ultrasonography confirmed the mass, and a benign tongue tumour was suspected. The tumour was surgically resected under general anaesthesia 1 month later, leading to the histopathological diagnosis of DF. She experienced an uneventful clinical recovery after surgery, with no recurrence noted for more than 1 year postoperatively.
Topics: Aged; Female; Fibroma, Desmoplastic; Humans; Magnetic Resonance Imaging; Tongue Neoplasms
PubMed: 30368477
DOI: 10.1136/bcr-2018-226962 -
Clinical Sarcoma Research 2018We studied two cases of rare fibrous bone tumors, namely desmoplastic fibroma (DF) and low-grade central osteosarcoma (LGCOS) resembling desmoplastic fibroma (DF-like...
BACKGROUND
We studied two cases of rare fibrous bone tumors, namely desmoplastic fibroma (DF) and low-grade central osteosarcoma (LGCOS) resembling desmoplastic fibroma (DF-like LGCOS). As the clinical presentation, imaging features and histopathology of DF and DF-like LGOS show much overlap, the objective of this study was to investigate the value of cytogenetic analysis, molecular pathology and immunohistochemistry in discrimination of these two mimickers.
CASE PRESENTATION
A mutation in (S45F) and nuclear beta-catenin immunostaining were observed in DF. DF-LGCOS had amplification of and showed strong nuclear expression of CDK4 by IHC. Moreover, the karyotype of DF-LGCOS showed an interstitial heterozygous deletion of the long arm of chromosome 13 (q12q32), associated with loss of the tumor suppressor gene.
CONCLUSIONS
Karyotyping and molecular genetic analysis may contribute to a conclusive diagnosis.
PubMed: 30159138
DOI: 10.1186/s13569-018-0104-z