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This report presents the case of a 5-year-old boy with a hard swelling on the right side of the mandible body. An important point of this case is that the primary imaging finding was fine spicules in the inferior border of the mandible on panoramic radiography without significant changes in bone density. Cone-beam computed tomography views revealed a lytic lesion on the lingual side of the right mandibular body with the destruction of the lingual cortex and periosteal reaction from the midline to the first molar area. Careful attention to this radiographic finding in the primary stage in the absence of other significant imaging findings, particularly in children, could result in the early diagnosis of desmoplastic fibroma. Therefore, a better prognosis can be expected following early surgical treatment. [ABSTRACT FROM AUTHOR]

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Competing Interests: Disclosure All authors disclosed no financial relationships. Commentary Endoscopic submucosal excavation (ESE) is an endoscopic resection technique similar to endoscopic submucosal dissection (ESD) except that no circumferential incision is performed. Instead, this technique involves making an incision in the mucosal layer above the target lesion, peeling off the mucosa, and then dissecting the lesion directly from the submucosal or muscularis layer. Limited data have shown superior R0 resection rates when compared with ESD, although with a potentially higher risk of perforation. In this case, the authors used ESE to remove a submucosal lesion incidentally found in the terminal ileum during a work-up for abdominal pain. Histologic analysis of a specimen from the resected lesion showed the lesion to be a collagenous fibroma, a rare benign fibrous soft tissue tumor most commonly arising from subcutaneous tissue or skeletal muscle. An R0 resection was achieved, without recurrence on follow-up colonoscopy. Perhaps ESE may prove beneficial in parts of the GI tract with thinner walls, such as the intestine, although more data are needed on this novel approach. Amy Tyberg, MD, FASGE, FACG, Associate Editor for Focal Points

Desmoplastic fibroma of bone is a very uncommon, benign but locally aggressive fibrogenic tumor. This report describes the case of a 45-year-old patient with a massive desmoplastic fibroma of the proximal tibia. A two-staged surgical procedure was successfully performed: wide resection and endoprosthetic reconstruction. Surgeons should be aware of the complexity of its treatment in the locally advanced and aggressive cases. A comprehensive review of the literature is also provided., Competing Interests: Conflito de Interesses Os autores não têm conflito de interesses a declarar., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit ( https://creativecommons.org/licenses/by/4.0/ ).)

Fibrocartilaginous mesenchymoma (FM) is a rare bone tumor mimicking other fibrocartilaginous lesions on imaging and histologically. Hence, it is difficult to diagnose this entity especially on small biopsies. In this article, we report a case of FM mimicking desmoplastic fibroma on biopsy. A 36-year-old male presented with pain in the left hip. Imaging showed a large expansile lytic lesion involving the acetabulum and pubis. The differential diagnosis was suggestive of giant cell tumor, aneurysmal bone cyst, intraosseous desmoplastic fibroma, and chondrosarcoma. Biopsy revealed a low-grade spindle cell lesion with no evidence of osteoid or chondroid matrix. The lack of cartilaginous nodules in the biopsy prompted a preoperative diagnosis of desmoplastic fibroma. The excised mass showed bland spindle cell proliferation, benign cartilage nodules, and epiphyseal plate-like enchondral ossification suggestive of fibrocartilaginous mesenchymoma. Negative immunostaining for SATB2, CDK4, and MDM2 ruled out low-grade central osteosarcoma. Though GNAS mutations were not performed in this case, rimming of the bony trabeculae at the periphery of the epiphyseal growth plate-like cartilaginous nodule ruled out fibrous dysplasia. The absence of cartilaginous component misleads the diagnosis preoperatively in small biopsies., (© 2022. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

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., (© 2021. The Author(s).)

Fibrous dysplasia may show locally aggressive behaviour reflecting secondary intralesional changes, extension to soft tissue, or malignant transformation. We report the case of a patient with polyostotic fibrous dysplasia who had a giant mandibular lesion consisting of histologically typical, genotypically-confirmed, fibrous dysplasia merged with a fibrotic and hypocellular desmoplastic fibroma-like tissue in which the same Gsα-R201H mutation was detected. The occurrence of the same mutation in both the fibrous dysplasia and areas of desmoplastic fibroma suggests that the fibroma-like tissue reflects an unusual secondary tissue change within an otherwise typical fibrous dysplasia. To the best of our knowledge, only four cases of fibrous dysplasia with desmoplastic fibroma-like tissue changes have been reported., (Copyright © 2019 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder that affects the skin, brain, kidneys, and other organ systems. It may exhibit a wide spectrum of clinical manifestations. Desmoplastic fibroma (DF) of the jaw is a rare benign myofibroblastic neoplasm. Less than 10 cases of DF associated with TSC have been published previously. We report a new case of a maxillary DF in a 12-year-old girl with TSC. The presentation, diagnostic process, and management of this case are discussed, and the literature is reviewed for the additional cases of DF associated with TSC; 7 previously reported cases are summarized. Small sample size limits conclusions, but there may be differences in the presentations of DF of the jaws in patients with TSC vs those in the general population. DF of the jaws may be a manifestation of TSC, and the authors propose surveillance panoramic radiographs every 2 to 3 years in patients with TSC., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Case: Desmoplastic fibromas are tumors of fibrous tissue that rarely are diagnosed. We present the case of a 27-year-old man who presented with pain of the forearm that was initially diagnosed as a muscle strain. A computed tomography-guided core biopsy revealed a desmoplastic fibroma. Consequently, the patient was treated with a resection of the proximal aspect of the radius followed by reconstruction with use of a vascularized fibular autograft., Conclusion: At the 2-year follow-up, radiographs showed integration of the autograft; additionally, good results were noted with the Patient-Reported Outcomes Measurement Information System (PROMIS) Upper Extremity and Physical Function Short Form T-score and the QuickDASH (an abbreviated version of the Disabilities of the Arm, Shoulder and Hand Score [DASH]) questionnaire, along with good range of motion.

Objective: To investigate the clinical features, immunohistochemical and differential diagnosis of desmoplastic fibroblastoma. Methods: The clinical data and pathology features of 7 cases of desmoplastic fibroblastoma were collected and immunohistochemical study were carried out in all cases with a review of the literatures. Results: There were 2 males and 5 females, with age ranging from 31 to 71 years (average and mean age were 59 and 61 years, respectively). The tumors were located in extremities and abdomen (left toe and right toe, right foot back, left leg and right thigh, right forearm and left hepatic lobe). Clinically, the tumors presented as slow growing painless masses of long standing duration. Grossly, the tumors were well-circumscribed with firm, white to gray cut-off surface. Tumor size ranged from 1.2 to 4.0 cm in maximum diameter (average 3.0 cm). Microscopically, 2 cases were located in dermis, 4 cases were located in subcutaneous and 1 case was located in liver parenchyma. It was composed of spindle-shaped or stellate cells with a fibroblastic or myofibroblastic appearance, and sparsely scattered in densely fibrous or fibromyxoid background. There was small vascular component in tumor background. At high magnification, the tumor cells were medium size with abundant cytoplasm, and the nucleus were small and always with small nucleoli. In some cases, the tumor cells were slightly larger with enlarged nuclei, but without cellular atypical and mitosis. Immunohistochemical study showed that the tumor cells were strongly positive for vimentin, desmin, S-100 protein and CD34, but CKpan was negative. α-SMA showed focal positive in one case. Ki-67 index ranged from 1% to 2%. Four cases were followed-up (ranged from 11 to 21 months, average 16.5 months) and the patients had no recurrence after surgery. Conclusions: Desmoplastic firoblastoma is a rare soft benign tumor. The differential diagnosis includes other benign or low-grade fibroblastic/myofibroblastic lesions.

Skeletal desmoplastic fibroma is an intraosseous neoplasm that is recognized as a very scare benign tumor. It has a propensity for locally aggressive behavior and local recurrence. The aim of this article is to report a case of skeletal desmoplastic fibroma in right mandible of a 4-year-old boy. The patient was found to have a large skeletal desmoplastic fibroma in right mandible, which was resected by surgical intervention. The defect was successfully restored with a titanium plate. In the report, the etiopathogenisis, pathological, radiographic features, clinical diagnosis, therapy and prognosis of skeletal desmoplastic fibroma were diccussed.