Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses

Abstract

Background:Desmoplastic fibroblastoma (collagenous fibroma) is an uncommon benign soft-tissue tumor, rarely involving bone. It shares some overlapping features with other infiltrate tumors, such as desmoid-type fibromatosis, neurofibroma, and low-grade fibromyxoid sarcoma. The misdiagnosis may cause unnecessary surgical overtreatment, especially for those involving bone. In order to deepen the understanding of the diagnosis and differential diagnosis of desmoplastic fibroblastoma, we planned to analyze the clinical, radiological, and histopathological features and the outcome of desmoplastic fibroblastoma on the basis of case analysis and literature review.Methods:Sixteen cases were retrieved from the surgical pathology records from May 2011 to April 2016 in the Department of Pathology in Beijing Jishuitan Hospital. Formalin-fixed, paraffin-embedded specimens of 16 cases of desmoplastic fibroblastoma were collected. Hematoxylin and eosin stain and immunohistochemistry were used to observe the histological features of desmoplastic fibroblastoma of soft tissue and bone. The images for diagnosis obtained from the ultrasonic examination, X-ray, magnetic resonance imaging, and computed tomography were used to observe the radiological features. Related literatures were retrieved from the PubMed and CNKI databases.Results:Sixteen cases of desmoplastic fibroblastoma of soft tissue were located in the hand (n = 7), foot (n = 4), upper arm (n = 1), shoulder (n = 1), forearm (n = 2), and one case occurred in the proximal femur. Age ranged from 32 to 82 years (median age: 58 years). There were six females and ten males. Histologically, the lesions of soft tissue appeared as well-circumscribed masses with abundant collagenous matrix and low vascularity. Tumor cells were stellate- or spindle-shaped and uniformly distributed within the extracellular matrix. In five cases, the desmoplastic fibroblastoma were found to have infiltrated into the skeletal muscle tissue. In one case of desmoplastic fibroblastoma of bone, radiographs revealed osteolytically well-defined lesion. Immunohistochemistry stain showed that vimentin and smooth muscle actin were positive in all cases of desmoplastic fibroblastoma.Conclusions:Desmoplastic fibroblastoma (collagenous fibroma) has prominent clinical, histopathological, and radiological features. Before the differential diagnosis from other tumors is obtained by thorough analysis and comparison of the similar and different characteristics, the appropriate surgical management and accurate prognosis evaluation could not be delivered to the patient.

A prevertebral desmoplastic fibroblastoma presenting with sleep‐related breathing dysfunction

“…Desmoplastic fibroblastomas (DFs) are uncommon benign fibrous soft tissue tumors typically occurring between the ages of 40 and 69 and are rarely observed in the tongue, palate, and neck. 4 DFs are composed of fibroblasts/myofibroblasts with variable collagen formation and are also known as collagenous fibromas 5,6 due to their tendency to show abundant collagen formation which has historically been used as a diagnostic clue. Available literature describes a few rare cases of DFs of the hard palate and oral cavity 6,7 and one case in the hepatic parenchyma.…”

Imaging Diagnosis and Differentiation of Desmoplastic Fibroma of Bone

“…Desmoplastic broma of bone (BFB) is a rare benign tumor with local aggressiveness and in ltration and has well-differentiated myo broblasts microscopically with rich deposition of dense collagens [1][2][3] . The prevalence of DFB is 0.06%-0.11% in all primary bone tumors and 0.3% in benign bone tumors [1][2][3][4][5][6] . This tumor occurs mostly in males between the ages of 16 and 83 years (median 50), and the primary locations of this tumor are the long bones (56%) followed by mandible (26%) and pelvis (14%).…”

“…This tumor occurs mostly in males between the ages of 16 and 83 years (median 50), and the primary locations of this tumor are the long bones (56%) followed by mandible (26%) and pelvis (14%). The imaging characteristics of this tumor are well-de ned osteolytic lesions with destruction of cortical bone, marginal osteosclerosis, and pseudotrabeculation, which are not speci c and cannot be differentiated from those of other bone tumors like brous dysplasia, eosinophilic granuloma, hemangioma, and central low-grade osteosarcoma [1][2][3][4][5][6] . Because of the rarity and nonspeci c imaging presentations of this tumor, it is di cult to diagnose and differentiate it from other tumors.…”

Desmoplastic fibroma of the femur with atypical image findings

“…[2] Typically, DFB is more common in the distal femoral metaphysis when it occurs in the femur. [3] Moreover, although DFB can occur at any age, the morbidity in young people is much higher than that in the elders. Evidence has revealed that DFB is lytic, occasionally with poorly defined borders on X-ray.…”