Desmoplastic fibroma of bone. A report of six cases

The clinical and pathological features of six cases of desmoplastic fibroma of bone are presented. Desmoplastic fibroma is rarely seen as a primary tumour of bone; when it does occur the sites of predilection are the long bones, but other sites such as the scapula and os calcis can be involved. Radiographically the lesion tends to expand the bone from within; it is well-demarcated and lytic, often with a trabeculated soap-bubble appearance. The cellular structure and the morphological arrangement are similar to those of aggressive fibromatosis of soft tissues. Differential diagnosis from malignant spindle-cell lesions of bone is important because the treatment of choice for desmoplastic fibroma of bone is simply excision with a thin layer of healthy tissue.     

Calcifying aponeurotic fibroma: a report of three cases.

Three cases of calcifying aponeurotic fibroma in an 8-year-old boy, a 43-year-old woman and a 48-year-old man are presented. The lesions involved the substance of the palmaris longus tendon, the lateral aspect of the foot at the base of the fifth metatarsal and the fifth digit of the foot, respectively. The clinical and radiologic findings, including ultrasound and computed tomography, are described. Histologic features included chondroid tissue and calcification within areas of proliferative plump fibroblasts in the younger patient, while in the two adults the lesions were more sharply defined showing increased calcification of dense connective tissue with less pronounced cellularity and no evidence of chondroid differentiation.     

Desmoplastic fibroma of mandible in a child: case report

The aim of this report is to present a rare case of a child with a desmoplastic fibroma (DF) at the mandibular angle. This lesion commonly shows a radiolucent and radiopaque radiographic picture. Due to its aggressiveness and high recurrence rate, an early diagnosis is essential and the complete removal of the lesion is the treatment of choice. We present a case of 11-year-old boy who was referred to be treated for an growth in the left mandibular angle area, a radiolucent and radiopaque feature. After an incisional biopsy, the complete removal of the lesion was done without vascular or nerve damage of the inferior alveolar bundle and without causing mandibular discontinuity. The clinical and radiographic features suggested the diagnosis and allowed the differentiation of DF from other lesions in the maxillofacial area. However, the final diagnosis was only possible through histopathological examination.     

Desmoplastic fibroma of the hand: case report

Desmoplastic fibroma is a benign tumor of the soft tissue and rarely of the bone. It typically presents in the trunk and proximal limbs, but it is quite rare in the hands. We present a rare case of desmoplastic fibroma of the soft tissues of the hand that presented as a slow-growing, painless, well-encapsulated mass.     

Desmoplastic fibroma. A case report

A desmoplastic fibroma occurring in the distal forearm of a 14-year-old black boy is described and the historical background of the tumour together with an outline of the disease emphasizes the differential diagnosis between this condition and other fibrous lesions of soft tissue and bone. The clinical and radiological features as well as the treatment of desmoplastic fibroma are discussed. It is believed that this is the first such patient reported in Africa.     

Desmoplastic fibroma or bone desmoid tumor: two cases

Desmoplastic fibroma or desmoid bone tumor is a rare tumor described for the first time by Jaffe in 1958. It accounts for 0.1 to 0.3% of all benign bone tumors. To date, about 150 cases involving the locomotor system have been reported. Histology is required for certain diagnosis of desmoplastic fibroma. The tumor is composed of sparse fibroblasts in a rich background of collagen fibers, a histological presentation exactly the same as soft tissue desmoid fibroma. We report two new cases of desmoplastic fibroma of the locomotor system. The observations illustrate the radiological diagnosis. MRI was used to search for local extension in bone or soft tissues. Biopsy is necessary to confirm the diagnosis. The histological presentation may be difficult to recognized and distinguish from low grade fibrosarcoma. After treatment, the rate of local recurrence is high in the event of partial resection. Surgery is the optimal treatment, with tumor resection as wide as possible depending on the localization.     

Desmoplastic fibroma of the spine: a series of 12 cases and outcomes

Background contextDesmoplastic fibroma (DF) is a benign, yet locally aggressive, tumor of the connective tissue. Desmoplastic fibroma in the spine is extremely rare, and only a few cases have been reported. Although surgical resection of DF arising in the spine is commonly regarded as a recommended treatment, it is difficult to achieve satisfactory results.PurposeThis study reviews the clinical patterns and follow-up data of patients with DF in the spine who underwent surgical treatment. We attempted to correlate surgical treatment and outcomes over time.Study designA retrospective clinical study of the surgical managements, including subtotal resection, total spondylectomy, and en bloc resection, for DF in the spine. Desmoplastic fibroma of the spine treatment occurred from 2004 to 2009 at the Department of Bone Tumor Surgery, AA Hospital.Patient sampleTwelve consecutive cases of DF of the spine underwent surgical treatment at our center between 2004 and 2009.Outcome measuresNeurologic outcomes were evaluated using Frankel score system and recurrence and metastasis were evaluated by computed tomography or magnetic resonance imaging of the surgical segments involved. Imaging was performed 3, 6, and 12 months after surgery, every 6 months for the next 2 years, and then annually for life.MethodsOverall, two different surgery protocols were applied. One protocol involved subtotal resection followed by radiotherapy (n=4), whereas the other involved total tumor resection (n=8). Postoperative radiotherapy was administered in six cases. Clinical data and surgery efficacy were analyzed via chart review.ResultsEleven patients were disease-free during their follow-up period, whereas one patient experienced recurrence without metastasis. Radicular pain nearly disappeared, and patients suffering from spinal cord compression recovered well. Local recurrence was detected in one-fourth (25%) of the cases that underwent subtotal resection and was not detected in any of the cases involving total spondylectomy.ConclusionsLocal recurrence of DF is not uncommon after insufficient removal. Therefore, total excision, while also preserving neural function, is recommended. In our study, patients who underwent a total spondylectomy had significantly lower local recurrence rates for DF in the spine. Radiotherapy may be an acceptable alternative therapy, whereas en bloc resection has the potential to result in significant functional impairment.     

Desmoplastic fibroma of the ulna. A case report

This report of desmoplastic fibroma of bone (DFB) in the left ulna represents the 56th published case of the neoplasm. To the best of the authors' knowledge, the present case is the first instance of this type of excision of the distal ulna performed for DFB with a metallic prosthesis and successful restoration of distal radial ulnar anatomy and function.     

Desmoplastic fibroma of the axis. A case report

Desmoplastic fibroma of the bone appears in a wide variety of bones but rarely involves the spine. A 24-year-old male with desmoplastic fibroma of the axis treated by complete resection and posterior spinal fusion is reported. There was no recurrence of the tumor three years after surgery. Complete resection and reconstructive surgery for spinal instability are recommended for this benign spinal tumor.     

Desmoplastic fibroma of bone. A report of eight cases and review of the literature

Desmoplastic fibroma of bone is a rare benign tumor consisting of thin, wavy fibroblasts set in an abundant matrix of collagen fibers. At times it is difficult to distinguish desmoplastic fibroma from other fibrous lesions, especially low-grade fibrosarcomas. Fewer than eight cases have been previously reported. We have reviewed the diagnostic and therapeutic findings of eighty additional cases. Six patients had the lesions located in an extremity and two had an axial lesion. The average age of the patients was twenty-five years (range, twelve to fifty-six years) and all of the patients had more than two years of follow-up (range, two to seventeen years). The radiographic findings in all but one patient were of a purely lytic, honeycombed lesion that often widened the bone, and was metaphyseal in long bones. The tumor replaced the medullary cavity with a grayish-white, rubbery to firm tissue that was often, but not always, contained by a rim of periosteal reactive bone. Histologically, the features were: (1) prominent loose bundles of fibrous tissue composed of slim, spindle-shaped fibroblasts with wavy, elongated nuclei; (2) variable amounts of bands of collagen fibers; and (3) absence of mitoses or atypical cells. Areas of metaplastic bone were found only around sites, of pathological fractures. The biology of desmoplastic fibroma is different from that of other benign fibrous lesions in that the lesion is very destructive locally and often recurs after incomplete excision. It is also distinguished from low-grade malignant lesions (for example, fibrosarcoma) in that metastases have never been reported. In our series an intralesional excision was initially performed in six of the eight patients and a marginal resection, in two. There were four recurrences, treated by a marginal resection in two patients and repeat curettage in one. The recurrence in the fourth patient required an amputation above the knee after two additional intralesional procedures had been unsuccessful. Wide or marginal resection appears to be the treatment of choice when the lesion is located in a site that can be resected without significant loss of function. In other areas, an attempt at curettage, instillation of phenol, and bone-grafting seems to be warranted, resorting to more radical procedures only if this fails to control local disease.     

Desmoplastic fibroma of the jaw: a case report and review of literature.

Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. It has a propensity for locally aggressive behavior and local recurrence. In the present report, we define the clinicopathological and radiographic features of a desmoplastic fibroma of the mandible in an 8-year-old white boy who initially presented with a 2-month history of a rapidly expanding, painless mass along the right inferior border of his mandible. A critical and comprehensive review of the English language literature is also provided.     

Desmoplastic fibroma of bone. Case report and literature review

A case is described of the rare, benign intraosseous desmoplastic fibroma, occurring in the humerus of a 20-year-old woman. A total of 121 cases is briefly reviewed and a table of 78 references dealing with desmoplastic fibroma is given. Treatment with indomethacin and ascorbic acid is proposed for cases of desmoplastic fibroma of bone when mutilating operations are the alternative or in inoperable patients.     

Desmoplastic fibroma of the first metatarsal. A case report

Desmoplastic fibroma is a benign but frequently aggressive tumor of bone. A 27-year-old man was treated for this lesion in the first metatarsal. The lesion recurred, extending into the medial cuneiform necessitating ray resection. The similar clinical and histologic findings of this tumor and other tumors, both benign and malignant, makes familiarity with the character of desmoplastic fibroma essential for proper treatment.     

Desmoplastic fibroma of the mandible

A desmoplastic fibroma of the mandible in an 11-year-old girl provided a difficult diagnosis because of its similarity, both clinically and histologically, to a low-grade fibrosarcoma following its rapid recurrence after primary local curettage. Immediate one-stage microvascular musculocutaneous reconstruction served to allow complete excision of the lesion and minimize the degree of psychological trauma. The differential diagnosis and the significance of cartilagenous elements present in the lesion are discussed.     

Desmoplastic fibroma of the ilium

INTRODUCTIONThe desmoplastic fibroma is a rare locally invasive bone tumour. Surgical resection with minimal margins is recommended.PRESENTATION OF CASEA 15 year-old boy was referred with chronic left thigh pain. MRI revealed a bone lesion within the cavity of the inner table of the left iliac wing without invasion of the underlying bone marrow. A surgical biopsy revealed a desmoplastic bone fibroma. A partial resection of the inner table of the iliac wing sparing the outer table was performed. At the latest follow-up the initially spared iliac wing had needed further resection. The reason proposed for this is devascularisation by substantial periosteal stripping causing partial resorption initially, then necrosis and ultimately ulceration through the skin necessitating further surgical resection.DISCUSSIONThe technique of resection of a pelvic desmoplastic fibroma sparing the outer table of the iliac wing has not previously been reported. The objective of a limited resection was to minimize the risk of a postsurgical limp caused by weakness of the gluteus medius muscle. However we report that this technique did not work in this case. A wider resection of the iliac wing as it is recommended for a malignant tumour would have yielded a similar final outcome.CONCLUSIONA partial resection of the iliac wing seemed an appealing technique for a benign tumour of the inner table of the iliac wing. However, considering the complications encountered, the authors advise a simple “en bloc” resection of the iliac wing for this type of tumour in this location.