Desmoplastic fibroma in a child: a 9-year follow-up case report.

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.

Unraveling the challenges in the diagnosis and management of desmoplastic fibroma of the mandible-a case report.

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.

Bilateral collagenous fibroma of the hard palate: a case report and review of the literature.

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.

Desmoplastic Fibroblastoma: A Case of a Rapidly Growing Mass in the Foot.

Desmoplastic fibroblastomas are benign and uncommon soft-tissue tumors. They are typically slow-growing, painless masses found in adult men. Rapidly growing masses have been previously reported, but are more rare. A 56-year-old man presented with a rapidly growing mass in his left foot, which was diagnosed as a desmoplastic fibroblastoma after pathologic evaluation. Although many case reports have been published in the dermatology literature, it is important to be aware of this benign neoplasm to avoid confusion with other rapidly growing malignant soft-tissue masses reported in the podiatry literature.

Unusual Appearance of Nasal Collagenous Fibroma (Desmoplastic Fibroblastoma) on Imaging.

Collagenous fibroma (CF) is a benign soft tissue tumour with good prognosis. Preoperative imaging diagnosis is essential to avoid unnecessarily extended resection, which may result in irreversible functional damage. However, few studies published to date have reported on the imaging features, associated with this condition. We, therefore, present a patient with histologically proven CF on the dorsum of nose, associated with an unusual appearance on magnetic resonance imaging. Low signal intensity on T2-weighted images and rim enhancement on gadolinium-DTPA (Gd-DTPA) enhanced images are reported in some previously reported cases. However, the unique point about this case is the markedly enhanced nodule revealed in the middle part of the lesion five hours after the administration of contrast media. To our knowledge, this is the first description of late gadolinium enhancement features associated with the condition. Key words: Benign, Soft tissue, Collagenous fibroma, Magnetic resonance imaging.

Desmoplastic Fibroma of Radius Managed with Non-Vascularised Fibular Autograft - A Case Report.

Desmoplastic fibromas of bone are extremely rare, slow growing, locally invasive, benign primary bone tumours, bearing close resemblance to the extra-abdominal desmoid tumours of soft tissue. They typically occur in patients around 30 years of age, and most commonly affect the mandible, pelvis and meta-diaphyseal region of long bones. En bloc or wide resection has typically been the treatment of choice to avoid recurrence, however, recent reports support curettage with bone grafting and adjuvant therapy to minimise functional loss. We report a 9-year-old child with a desmoplastic fibroma of right radius. This is an unusual age group for this bone tumor. The tumor was managed with en bloc resection and reconstruction with a non-vascularised fibula autograft. The patient had good functional outcome and no recurrence at 1-year follow-up. Level of Evidence: Level V (Therapeutic).

Desmoplastic Fibroblastoma (Collagenous Fibroma) of the Foot: A Case Report of a Rare Benign Tumor in an Atypical Patient.

Desmoplastic fibroblastoma (collagenous fibroma) is a rare benign soft-tissue tumor. Often found in the subcutaneous and muscle tissue, it is slowly enlarging and generally not painful or invasive. The literature often describes the tumor to be found in the upper extremities, neck, and back. Full excision of the tumor is the treatment of choice, and the prognosis is generally favorable; there are no documented cases of recurrence after full excision. We present an atypical case of desmoplastic fibroblastoma found on the dorsum of the foot with a larger tumor present in a substantially younger patient than is typical.

Primary desmoplastic fibroblastoma of diaphragm.

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.

Does a Vertebra Fracture Cause a Tumor? A Dorsolumbar Paraspinal Desmoid Tumor: Case Report

Extraabdominal desmoid tumors are uncommon soft-tissue tumors. The etiology of the tumor is still unclear. Injury is one of the etiological factors of soft-tissue tumors. A 41-year-old female patient who had a traumatic vertebral body fracture on the thoracic spine was treated conservatively. Two and a half years later, she presented a painful, palpable swelling on the thoracolumbar region. In the present report, was discuss the patient, who underwent a surgery to remove the desmoid tumor (aggressive fibromatosis), within the context of the current literature. The literature on desmoid tumor caused by a trauma is rare. This is the first case that demonstrates an extraabdominal desmoid tumor following a spinal fracture. The swelling on the region of the trauma must be examined carefully and desmoid tumor must be kept in mind as a possible diagnosis.

Desmoplastic Fibroma in the Distal Humerus of a 14-Year-Old Boy: A Case Report.

CASE: We present the case of a 14-year-old male who presented with a pathologic elbow fracture. Imaging demonstrated a lytic distal humerus metaphyseal lesion with a soap bubble appearance. After fracture healing, frozen sections obtained at open biopsy suggested fibrous tissue; thus, curettage and bone grafting was performed. Pathology confirmed desmoplastic fibroma. The patient experienced a recurrence 18 months later and had repeat curettage and allograft strutting. He had no evidence of re-recurrence 2 years after the second surgery. CONCLUSIONS: Desmoplastic fibroma may mimic the presentation of many bone lesions. Recurrence is common despite curettage and bone grafting. This patient performed well without wide resection.

Fibroma desmoplástico / Desmoplastic fibroma

El Fibroma Desmoplástico es una patología poco frecuente y de localización mas conocida en maxilar inferior y pocos casos en tibia, fémur y otros huesos. En rótula no se encontraron casos en la abundante bibliografía consultada. Se presenta un caso en un paciente de 15 años que después de un año de tratamiento médico y fisiokinésico se hace RMN y TAC encontrando una imágen osteolítica en rótula izquierda, por lo que después de interconsulta con Ortopedista y Traumatólogo Infantil, se resuelve tratamiento quirúrgico. El objetivo de esta presentación es llamar la atención sobre dolor que se interpreta como tendinitis cuadricipital, con Rx. neg. y dolor en borde proximal de rótula y borde externo de la misma, pero los estudios mencionados nos orientan a una patología muy poco frecuente. Además se destaca que no se encontró en la bibliografía antecedente de otro caso similar en rótula. Se hace resección con margen de seguridad de la zona afectada incluyendo hueso y cartílago articular. La evolución fue satisfactoria, controlado a los 3 años no hay dolor ni limitación funcional. Tipo de estudio: Reporte de caso. Nivel de evidencia: IV

The Desmoplastic Fibroma is an unfrequent pathology and the most common location is in the lower jaw (there are a few cases in the tibia, femur and other bones). There wasn't found a case in the kneecap, despite the extensive bibliography consulted. It is presented a case of a 15 years old patient who after a year of medical and physio-kinesic treatment, underwent MRI and CT scans, finding an osteolytic image in the left patella. After consulting with an Orthopedist and a Child Traumatologist, it was resolved a surgical treatment. The purpose of this presentation is to draw attention to a pain in the proximal and outer edges of the patella interpreted as quadriceps tendonitis, without seeing anything in a radiography. It was made a resection, with a safety margin of the affected area, including bone and articular cartilage. There was a satisfying evolution, checked after 3 years, without pain or functional limitation. Type of Study: case report. Level of evidence: IV

Uncommon presentation of desmoplastic fibroblastoma on the tongue of a female patient.

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.

Tumor desmoide gigante de pared torácica con crecimiento intratorácico / Giant Desmoid Tumor Of The Chest Wall With Intrathoracic Growth

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Desmoplastic Fibroma of the Mandible in a Pediatric Patient: A Case Report of Resection and Reconstruction With A Six-Year Follow-Up.

Desmoplastic fibroma (DF) of bone is considered a benign but locally aggressive tumor of fibroblastic origin. DF is rare, representing less than 1% of all bone tumors. Approximately 84% of patients are younger than 30 years. DF has a slow but aggressive growth potential and can recur locally when it has not been completely excised. Complete resection is the treatment of choice to decrease recurrence and morbidity. Mandibular reconstruction is mandatory in pediatric patients to ensure correct craniofacial development. The present report describes the case of a pediatric patient with mandibular DF in whom complete resection and immediate reconstruction with a fibula flap proved a satisfactory treatment option, with low morbidity and excellent esthetic and functional results at 6-year follow-up.

Calcified desmoplastic fibroblastoma of the foot: imaging findings.

We report a case of desmoplastic fibroblastoma (DF) of the foot in a 65-year-old woman. The tumor presented as a slow-growing, painless mass located in the first intermetatarsal space of the right foot. Ultrasound showed a well-circumscribed hypoechoic lesion containing hyperechoic calcifications confirmed on standard radiographs. At magnetic resonance imaging (MRI), the mass appeared isointense to the muscles on T1-weighted (T1W) images, hyperintense on proton-density-weighted fat-saturated images, and presented scattered internal hypointense foci. Post-contrast T1W spectral presaturation with inversion recovery (SPIR) images showed heterogeneous, mostly peripheral, contrast enhancement. DF must be considered in the differential diagnosis of soft-tissue calcified tumors of the foot.

Remodeling of Costochondral Graft After Mandibular Reconstruction.

Mandibular tumors are rare in children. Most mandibular tumors in children are benign and locally aggressive, and some might require resection to prevent recurrence. Mandibular reconstruction after tumor resection in children has been less documented in published studies than mandibular reconstruction in adults. In children, age, the growth potential of the mandible, and the donor site are important factors that must be considered. The costochondral graft is an option for reconstruction of the resected portion of the mandible in children, especially when the resection involves the condyle, owing to its biologic and anatomic similarity to the temporomandibular joint and its regenerative and growth potential. The objective of the present case report is to present the unusual remodeling of a nonvascularized costochondral graft after mandibular resection in a pediatric patient.