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Arthroplasty Today Aug 2022Desmoplastic fibroma is an extremely rare primary bone tumor that can mimic the presentation of other bone lesions. We describe the case of a middle-aged male with a...
Desmoplastic fibroma is an extremely rare primary bone tumor that can mimic the presentation of other bone lesions. We describe the case of a middle-aged male with a mass on the left distal femur initially diagnosed as fibrous dysplasia that underwent a wide margin excision followed by a distal femoral replacement to restore anatomy and functionality. Histologic examination of the complete surgical specimen was consistent with a desmoplastic fibroma. This case is the first report of a successful application of endoprosthetic reconstruction after desmoplastic fibroma resection.
PubMed: 35647245
DOI: 10.1016/j.artd.2022.04.005 -
Clinical Nuclear Medicine Oct 2022Desmoplastic fibroma is a very rare benign bone tumor with local aggressiveness. We describe imaging findings in 2 cases with desmoplastic fibroma of the bone. One case...
Desmoplastic fibroma is a very rare benign bone tumor with local aggressiveness. We describe imaging findings in 2 cases with desmoplastic fibroma of the bone. One case had a desmoplastic fibroma in the right distal femur, showing bone destruction with sclerotic margins and inhomogeneous FDG uptake. The other case had a desmoplastic fibroma in the left ninth rib, showing progressive peripheral enhancement and increased 99m Tc-MDP uptake.
Topics: Bone Neoplasms; Fibroma, Desmoplastic; Fluorodeoxyglucose F18; Humans; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography; Technetium Tc 99m Medronate
PubMed: 35426851
DOI: 10.1097/RLU.0000000000004186 -
Current Medical Imaging 2022The objective of this study is to analyze the clinical and imaging features of desmoplastic fibroma of bone (DFB) for correct diagnosis.
OBJECTIVE
The objective of this study is to analyze the clinical and imaging features of desmoplastic fibroma of bone (DFB) for correct diagnosis.
MATERIALS AND METHODS
Twenty patients with DFB confirmed by pathology were enrolled, and the imaging presentations were analyzed. Among 20 patients, plain X-ray was performed in all patients, computed tomography (CT) was performed in 12, and magnetic resonance imaging (MRI) was conducted on eight. The clinical and imaging presentations were analyzed and classified to assist in correct diagnosis.
RESULTS
Twenty patients with DFB were retrieved, including eleven males and nine females with an age range of 2-52 years (median 27). The DFB involved the femur in six patients, ilium in five, tibia in four, humerus in two, lumbar vertebra in one, radius in one, and calcaneus in the remaining one. DFB was common in the metaphysis of long bones and could involve the diaphysis and epiphysis. The imaging presentations were divided into four types: the cystic expansile destruction in ten patients, osteolytic destruction in five, mixed destruction in four, and paraosseous destruction in one. CT value was 30 -60 Hu in the lesion area (6 cases CT value45Hu). In eight patients with MRI scanning, the lesion in five patients presented with unevenly equal or low signal on T1WI and unevenly equal or high signal on T2WI, with irregular stripes or patches of low signal on both T1WI and T2WI. In the rest three patients, the lesion was evenly equal or low signal on T1WI and evenly high signal on T2WI. MRI more clearly showed a mass in the adjacent soft tissue and the range of edema in the DFB lesion.
CONCLUSION
DFB is a rare tumor with strong local aggressiveness, cystic bone destruction, formation of tumor bone trabeculae, soft tissue masses on imaging presentations, low signals on T1WI and T2WI in the lesion, but no periosteal reaction or calcification, which are helpful for diagnosis of the disease and differentiation from other ones.
Topics: Adolescent; Adult; Bone Neoplasms; Child; Child, Preschool; Female; Fibroma, Desmoplastic; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Tibia; Tomography, X-Ray Computed; Young Adult
PubMed: 35410618
DOI: 10.2174/1573405618666220411125408 -
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 -
World Journal of Clinical Cases Oct 2021Granular cell tumor (GCT) is a neurogenic tumor mainly occurring in the head and neck. GCT in the genitourinary system is extremely rare and only sporadic cases of...
BACKGROUND
Granular cell tumor (GCT) is a neurogenic tumor mainly occurring in the head and neck. GCT in the genitourinary system is extremely rare and only sporadic cases of urinary bladder GCT have been reported. Most urinary bladder GCT cases are benign and only two malignant cases have been reported. Due to its rarity, no consensus criteria for the treatment of urinary bladder GCT are available at present.
CASE SUMMARY
A 62-year-old Chinese woman was found to have a urinary bladder tumor without any clinical manifestations on physical examination. Cystoscopy revealed a semispherical shaped lesion measuring approximately 4.0 cm in diameter at the junction of the left wall and roof of the bladder, which was covered with normal bladder mucosa. Computed tomography scan demonstrated a high-density lesion on the left wall of the bladder, measuring approximately 2.9 cm × 2.4 cm with clear boundaries. Contrast-enhanced pelvic magnetic resonance imaging revealed a space-occupying lesion on the left wall of the bladder (non-mucosal origin/ external pressure), which was preliminarily suspected to be a desmoplastic fibroma or leiomyoma. In the context of the above findings, a pre-operative diagnosis of bladder leiomyoma was made. The patient consequently underwent a laparoscopic partial cystectomy. The resected bladder mass looked yellowish and well-demarcated, measuring 4.0 cm × 3.5 cm and infiltrated the muscular layer. The diagnosis of urinary bladder GCT was finally made by postoperative pathology, with positive immunohistochemical S-100 staining and negative pancytokeratin. The patient has been followed for 6 mo so far, with no tumor recurrence detected.
CONCLUSION
This case highlights the biological feature and differential diagnosis of urinary bladder GCT at the pathological and molecular levels. Transurethral resection of the bladder tumor and partial cystectomy are recommended in most urinary bladder GCT cases, while radical cystectomy is recommended in malignant cases.
PubMed: 34754853
DOI: 10.12998/wjcc.v9.i28.8453 -
Surgical Pathology Clinics Dec 2021Fibrous and fibro-osseous tumors are some of the most common benign lesions involving bones. Although many of the histomorphologic features of these tumors overlap... (Review)
Review
Fibrous and fibro-osseous tumors are some of the most common benign lesions involving bones. Although many of the histomorphologic features of these tumors overlap significantly, an interdisciplinary approach helps to consolidate the classification of these tumors. Herein, the clinical, radiologic, and pathologic features of lesions within these categories are described.
Topics: Bone Neoplasms; Fibroma, Ossifying; Fibrous Dysplasia of Bone; Humans
PubMed: 34742489
DOI: 10.1016/j.path.2021.06.011 -
Journal of Medical Case Reports Oct 2021Desmoplastic fibroblastoma (also known as collagenous fibroma) is a benign, slowly growing soft-tissue tumor. Most desmoplastic fibroblastomas develop in the limbs,...
BACKGROUND
Desmoplastic fibroblastoma (also known as collagenous fibroma) is a benign, slowly growing soft-tissue tumor. Most desmoplastic fibroblastomas develop in the limbs, neck, or trunk. A mediastinal origin is quite rare.
CASE PRESENTATION
A 32-year-old Asian female was referred to us for the diagnosis and treatment of an anterior mediastinal tumor. The tumor was 80 mm in the largest diameter and was located on the pericardium. No invasion was evident. She underwent resection of the tumor via video-assisted thoracoscopic resection. The tumor was totally encapsulated, and its pedicle was on the pericardium. The resected specimen was very rigid, making it difficult to remove from the intercostal space. Histologically, the tumor was composed of a paucicellular dense collagenous tissue. Mitosis was rarely observed, and cellular atypia was not evident, suggesting that the tumor was benign. We diagnosed the tumor as a desmoplastic fibroblastoma by morphology and immunohistochemistry.
CONCLUSIONS
Desmoplastic fibroblastoma of the mediastinum is an extremely rare disease. Preoperative diagnosis is difficult. Early surgical resection is suitable for diagnosis and treatment planning.
Topics: Adult; Female; Fibroma, Desmoplastic; Humans; Immunohistochemistry; Mediastinum; Soft Tissue Neoplasms; Thoracic Wall
PubMed: 34663426
DOI: 10.1186/s13256-021-03014-x -
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 -
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 Pathology &... 2021Desmoplastic fibroma (DF) is a rare myofibroblastic primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue....
Desmoplastic fibroma (DF) is a rare myofibroblastic primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. The surgical management of this tumor has been a matter of controversy and its recurrence has been a matter of clinical relevance. In this case report, we describe an unusual case of DF in a 15-year-old male patient who presented with a slow-growing mass in the right posterior mandibular region of 2 years duration. The presence of areas mimicking Giant cell angiofibroma (GCA) is the highlight of the case. The possibility of misdiagnoses is more in early lesions since the available literature shows that immunohistochemistry (IHC) is not of much benefit while differentiating DFs from other spindle cell lesions.
Topics: Adolescent; Angiofibroma; Diagnosis, Differential; Fibroma; Fibroma, Desmoplastic; Humans; Immunohistochemistry; Male; Mandible; Neoplasm Recurrence, Local; Radiography, Panoramic; Tomography, X-Ray Computed
PubMed: 34341270
DOI: 10.4103/IJPM.IJPM_698_20