CLINICAL, X-RAY AND COMPUTER-TOMOGRAPHIC PICTURE OF DESMOPLASTIC FIBROMA OF THE LOWER JAW IN CHILDREN

The paper presents the results of a clinical observation of a 6-year-old female patient, who was admitted to the department of maxillofacial surgery of a multidisciplinary hospital under the compulsory health insurance system with a rare disease of the bone tissue of the lower jaw – desmoplastic fibroma. Desmoplastic fibroma of bone tissue (aggressive fibromatosis, desmoid fibroma) is a rare, locally aggressive, benign tumor localized in bone tissue, it is a mesenchymal fibroproliferative tumor with locally destructive growth, accounting for less than 0.03% of all human tumors. According to the literature, desmoplastic fibroma has a recurrence rate of 37% to 72%, resection of the affected bone is the preferred therapy, and, ideally, resection should be a single resection block. Wide excision is the method of choice for treating operable desmoid tumors in children. The article presents a dynamic observation within 1 year after surgical treatment. The article presents the clinical, radiological and computed tomographic picture of the disease and the results of instrumental and histological examination of a rare oncostomatological pathology. Purpose: clinical, radiological, 3D computed tomography observation of a child with desmoplastic fibroma of the lower jaw. Materials and methods: the results of a clinical examination, instrumental methods of X-ray diagnostics, stages of surgical treatment, a histological report and photographs of the patient's appearance are presented. Conclusion: Given the high regenerative capacity of bone tissue in children, it is necessary to perform surgical treatment of benign neoplasms by the type of marginal resection, that is, by the organ-preserving type, and to conduct dispensary observation of the patient during the first year.

Desmoplastic fibroma of bone: A morphological and immunohistochemical characterization

Background: Desmoplastic Fibroma (DF) of bone is a locally aggressive and infrequent benign neoplasm. Recently was described a role of vascular endothelial growth factor in the interstitial fibrotic processes. Case presentation: A 13-year-old female presented with pain, swelling and limitation of movements in right forearm. An osteolytic lesion at the distal end of the right radius was shown, with pathologic concentration of Technetium 99 and slight enhancement of soft tissue lesion employing computerized axial tomography. The surgical biopsy showed nodular formations of hyalinized collagen fibers arranged in thick bands with few well-differentiated interstitial fibroblasts / myofibroblasts, focally expressing VEGF-A. Conclusion: The intramedullary neoplastic proliferation is limited by the cortical bone, provoking compression of the intratumorally micro-vessels, favoring both, the extracellular matrix and VEGF-A synthesis. Future research should include therapeutic intervention with anti-CD117 and anti-VEGF-A drugs, with the aim of limiting tumor growth, facilitating the complete surgical excision of the neoplasm. Keywords: desmoplastic fibroma; vascular endothelial growth factor; hyalinization; neoplasm progression.

Desmoplastic Fibroma and Cemento Ossifying Fibroma of The Anterior Maxilla: A Rare Case Report

Tumors located in the maxillofacial part of the body were classified by WHO in 2017 and among these are intraosseous form of fibromatosis known as Desmoplastic and Cemento-ossifying fibromas. These tumors usually occur in the head and neck region, especially in the mandible and are relatively rare in the maxilla. Meanwhile, this study aims to discribe a rare case of the cemento ossifying fibroma that had been previously diagnosed as desmoplastic fibroma with a mass tumor in the anterior of the maxilla. A 22 years old female reported to the Cipto Mangkusumo Hospital with the main complaint of a lump in the right side of the upper jaw which appeared 2 years prior to the operation. In April 2017, the patient had a biopsy in Tarakan Hospital and the result was a desmoplastic fibroma. Due to the lump enlargement, the patient was admitted to RSCM in July 2019 and had biopsy incision with a diagnosis of cemento ossifying fibroma which was confirmed by the histopathological examination and histology report. The resection of the right part of maxilla was conducted alongside with reconstruction using the free fibular flap. Moreover, cemento ossifying fibroma and desmoplastic fibroma shared similar features, namely, clinical, histological and radiological features which are important in establishing the diagnosis and treatment of patient. Hence, extensive enucleation or resection is required due to the progressive nature of the tumor to prevent the potential for further recurrences.

U xơ thể xơ cứng tại xương sườn: Báo cáo ca lâm sàng và y văn

U xơ thể xơ cứng (Desmoplastic fibroma) tại xương sườn rất hiếm gặp. Các dấu hiệu chẩn đoán hình ảnh của u thường không đặc hiệu có thể tương tự các u xương khác. Mặc dù vậy, do tính chất xâm lấn và tỷ lệ tái phát cao sau phẫu thuật nên sự hiểu biết về các đặc điểm hình ảnh và mô bệnh học của loại u này là cần thiết trong chẩn đoán và điều trị. Chúng tôi thu thập thông tin và báo cáo một trường hợp bệnh nhân nữ 30 tuổi có tổn thương tiêu xương sườn 9 bên phải trên cắt lớp vi tính, được phẫu thuật cắt hoàn toàn khối u với kết quả mô bệnh học u xơ thể xơ cứng, đồng thời tham khảo y văn các ca bệnh đã được báo cáo.

Desmoplastic fibroma of the left 6th rib in a 35-year-old man: a case report and literature review

Background: Desmoplastic fibroma (DF) is a rare, benign tumor. The most common sites are the long tubular bones and mandible. Although generally considered a benign tumor, it can invade surrounding tissues and has a high rate of local recurrence after incomplete surgical excision. However, there is currently no stantard treatment. Here we present a novel case of DF in the left 6th rib in a 35-year-old man.Case presentation: The man presented to our center with left chest pain and swelling. Enhanced computed tomography (CT) showed a 4.5×2.0 cm mass on the left 6th rib with pathological fracture.Wide resection was performed. Open biopsy revealed that the tumor destroyed medullary cavity and partially broke through the bone cortex. Pathologically, the tumor was composed of spindle-shaped cells arranging in a woven pattern on a backgroung of abundant collagenous fiber. No β-catenin were detected. Based on the pathological and radiological findings, a final diagnosis of DF was made. No postoperative adjuvant treatments were administerded. Fortunately, there was no evidence of recurrence 22 months after surgery. Conclusions: DF originated from rib is a kind of extremly rare benign tumor but locally aggressive and show unique biologically features. Wide resection or total resection can effectively reduce the risk of local recurrence when compared with curettage. Rarity of the tumor favors documentation in literature.

Desmoplastic Fibroma of the Left 6th Rib in a 35-year-old Man: a Case Report and Literature Review

Background: Desmoplastic fibroma (DF) is a rare, benign tumor. The most common sites are the long tubular bones and mandible. Although generally considered a benign tumor, it can invade surrounding tissues and has a high rate of local recurrence after incomplete surgical excision. However, there is currently no stantard treatment. Here we present a novel case of DF in the left 6th rib in a 35-year-old man.Case presentation: The man presented to our center with left chest pain and swelling. Enhanced computed tomography (CT) showed a 4.5×2.0 cm mass on the left 6th rib with pathological fracture. Wide resection was performed. Open biopsy revealed that the tumor destroyed medullary cavity and partially broke through the bone cortex. Pathologically, the tumor was composed of spindle-shaped cells arranging in a woven pattern on a backgroung of abundant collagenous fiber. No β-catenin were detected. Based on the pathological and radiological findings, a final diagnosis of DF was made. No postoperative adjuvant treatments were administerded. Fortunately, there was no evidence of recurrence 22 months after surgery. Conclusions: DF originated from rib is a kind of extremly rare benign tumor but locally aggressive and show unique biologically features. Wide resection or total resection can effectively reduce the risk of local recurrence when compared with curettage. Rarity of the tumor favors documentation in literature.