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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 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 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 -
Cureus Jul 2023Desmoplastic fibroma (DF) is a rare, benign, yet locally aggressive bone tumor. It frequently affects the facial bones, and the mandible is the most commonly affected...
Desmoplastic fibroma (DF) is a rare, benign, yet locally aggressive bone tumor. It frequently affects the facial bones, and the mandible is the most commonly affected site. Treatment of choice is the removal of the tumor with resection of surrounding bone due to its aggressive behavior. We report a case of DF where the tumor showed resolution and almost complete bone deposition following enucleation. Although DF has a high recurrence rate, the patient remains disease-free 31 months post-surgery.
PubMed: 37602060
DOI: 10.7759/cureus.42213 -
BMC Musculoskeletal Disorders Apr 2024Desmoplastic fibroma is an extremely rare primary bone tumor. Its characteristic features include bone destruction accompanied by the formation of soft tissue masses....
BACKGROUND
Desmoplastic fibroma is an extremely rare primary bone tumor. Its characteristic features include bone destruction accompanied by the formation of soft tissue masses. This condition predominantly affects individuals under the age of 30. Since its histology is similar to desmoid-type fibromatosis, an accurate diagnosis before operation is difficult. Desmoplastic fibroma is resistant to chemotherapy, and the efficacy of radiotherapy is uncertain. Surgical excision is preferred for treatment, but it entails high recurrence. Further, skeletal reconstruction post-surgery is challenging, especially in pediatric cases.
CASE PRESENTATION
Nine years ago, a 14-year-old male patient presented with a 4-year history of progressive pain in his left wrist. Initially diagnosed as fibrous dysplasia by needle biopsy, the patient underwent tumor resection followed by free vascularized fibular proximal epiphyseal transfer for wrist reconstruction. However, a histological examination confirmed a diagnosis of desmoplastic fibroma. The patient achieved bone union and experienced a recurrence in the ipsilateral ulna 5 years later, accompanied by a wrist deformity. He underwent a second tumor resection and wrist arthrodesis in a single stage. The most recent annual follow-up was in September 2023; the patient had no recurrence and was satisfied with the surgery.
CONCLUSIONS
Desmoplastic fibroma is difficult to diagnose and treat, and reconstruction surgery after tumor resection is challenging. Close follow-up by experienced surgeons may be beneficial for prognosis.
Topics: Adolescent; Humans; Male; Bone Neoplasms; Fibroma; Fibroma, Desmoplastic; Fibula; Follow-Up Studies; Tomography, X-Ray Computed
PubMed: 38643068
DOI: 10.1186/s12891-024-07454-6 -
BMC Oral Health Feb 2024Desmoplastic fibroma (DF) is an uncommon bone tumor that originates from the mesenchymal tissue and despite being benign, exhibits aggressive behavior locally. The...
Desmoplastic fibroma (DF) is an uncommon bone tumor that originates from the mesenchymal tissue and despite being benign, exhibits aggressive behavior locally. The following report describes the case of a 7-year-old boy with a rapidly enlarging swelling on the right side of the mandible. After a thorough clinical examination, radiographic imaging, and histopathological analysis, the diagnosis of DF was confirmed. Treatment planning was formulated considering both the tumor's tendency for local recurrence and the patient's well-being. Due to the patient's young age, segmental resection was not deemed appropriate, and an aggressive curettage and enucleation of the lesion followed by the bone graft was performed instead. The patient was kept under close follow-up for the first month of post-surgery and later reviewed after 3, 6, 9, and 12 months, respectively. Good bone healing was observed on radiographs. The patient did not show any signs of recurrence based on clinical or radiographic assessments and did not exhibit any neurosensory deficits as well.
Topics: Male; Humans; Child; Fibroma, Desmoplastic; Mandible; Radiography; Bone Transplantation
PubMed: 38378640
DOI: 10.1186/s12903-024-04018-x -
Oxford Medical Case Reports Aug 2023Desmoplastic fibroma (DF) is an aggressive benign tumor that commonly affects long tubular bones. Also, the skull, mandible, pelvis and spine involvement have been...
Desmoplastic fibroma (DF) is an aggressive benign tumor that commonly affects long tubular bones. Also, the skull, mandible, pelvis and spine involvement have been reported. However, its occurrence in the scapula is extremely rare. In this case report, we present the challenging diagnosis and successful treatment of DF in a 27-year-old woman who had been experiencing worsening pain in her right shoulder for 5 years. Plain radiographs and magnetic resonance imaging revealed a lucent, trabeculated and expansile infiltrative lesion, disrupting the posterior cortex and extended to the posterior soft tissue. After ruling out malignancy through a core needle biopsy, the patient underwent wide surgical resection of the tumor, which involved a hemi-scapulectomy. And histologic diagnosis consistent with DF, no postoperative radiation was administered. Remarkably, the patient became pain-free just 2 weeks after surgery. Follow-up examinations, X-rays and computed tomography scans conducted 6 weeks, 6 months and 18 months after surgery revealed no signs of recurrence.
PubMed: 37637370
DOI: 10.1093/omcr/omad057 -
First case of collagenous fibroma in the terminal ileum treated by endoscopic submucosal excavation.Gastrointestinal Endoscopy Mar 2024
Topics: Humans; Fibroma, Desmoplastic; Ileum; Endoscopy
PubMed: 37804875
DOI: 10.1016/j.gie.2023.10.003 -
Clinical Case Reports Apr 2024Desmoplastic fibroma presents similar to other soft tissue tumors to such an extent that even a gold standard investigation can miss.
KEY CLINICAL MESSAGE
Desmoplastic fibroma presents similar to other soft tissue tumors to such an extent that even a gold standard investigation can miss.
ABSTRACT
This is to report a mass in a 47-year-old male arising from the chest wall, which was first thought to be a hemangioma but was later diagnosed as a case of desmoplastic fibroblastoma with the help of a biopsy.
PubMed: 38562578
DOI: 10.1002/ccr3.7523