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Desmoplastic fibroma is a rare, benign, locally aggressive primary bone tumor. It is principally found in the mandible, pelvis, and metaphysis or meta-diaphysis of long bones and has rarely been reported in the calcaneus. The lesion is accompanied by high rates of recurrence in the literature. Treatment options include intralesional techniques via curettage ranging up to extralesional techniques via wide resection. Given the rarity of this tumor, there is a paucity of literature discussing presentation and management, especially in the lower extremity. The following case report details a 22-year-old female with an extensive desmoplastic fibroma of the calcaneus. Based on anatomic and functional considerations, this case was managed via extensive curettage with subsequent backfill with autologous and allogeneic bone graft. The post operative period was uneventful, and the patient received complete symptom relief without recurrence appreciated at 16 month follow-up.
First described by Jaffe in 1958 as an uncommon benign tumor composed of small fibroblasts, it was originally likened to the soft tissue desmoid tumor of the abdominal wall due to histologic and biologic similarities.
The large majority of this literature consists principally of case studies and series. Documentation regarding DF presentation in the distal lower extremity is particularly sparse. More specifically, to our knowledge, there have been less than 10 reported cases of DF in the calcaneus (Table 1). Owing to the extreme rarity of DF, specifically in the calcaneus, there is a paucity of literature detailing the clinical presentation, imaging findings, and treatment options for proper identification and management of a desmoplastic fibroma. As such, the purpose of the following report is to detail a rare case of desmoplastic fibroma of the calcaneus managed with operative intervention represented as a curopsy, or biopsy with intention to cure, intralesional technique.
A 22-year-old female initially presented to our outpatient clinic for evaluation of chronic right heel pain in September 2020. Pain had been present for approximately 5 years, and symptoms developed following the initial traumatic incident of striking the right foot against the support of a trampoline. Two months following the initial trauma, the patient sought care of an orthopedic specialist and was immobilized in an ankle brace. The patient subsequently visited a community podiatrist 2 years after the incident with continued pain but was unable to follow up due to work commitments. On an initial clinical encounter in our office, the patient endorsed intermittent pain described as a local soreness and sharp sensation. Pain was present at the lateral aspect of the hind foot, worse during ambulation. Initial physical examination produced tenderness with compression of the body of the calcaneus. There was also some low-grade edema localized to the lateral aspect of the hindfoot and notable palpable osseous prominence at the lateral aspect of the calcaneus. Neurovascular status was intact and the patient was otherwise in an excellent general state of health, without any concerns for systemic signs of occult malignancy including night sweats, recent weight loss or fatigue. Radiographs of the affected foot and ankle demonstrated an extensive mixed radiolucent and sclerotic lesion involving the entire body of the calcaneus with some cortical erosion laterally. No evidence of pathologic fracture was appreciated. As part of further work up, an MRI was obtained to evaluate the lesion and assess for potential soft tissue extension.
MRI confirmed the entire calcaneus was replaced with a T1 hypointense, T2 mixed hyperintense and hypointense expansile lesion with heterogeneous enhancement (Fig. 1). The anterior process of the calcaneus was spared. Cortical thinning was appreciated along the lateral calcaneus, however no extension into the adjacent soft tissue was appreciated. Based on the clinical and radiographic evaluation, differential diagnoses suggested at this point included giant cell tumor, chondromyxoid fibroma, desmoplastic fibroma, non ossifying fibroma, fibrous dysplasia, chondroblastoma, and other potentially aggressive lesions. Initial suggestion included proceeding with preliminary biopsy, however, the patient preferred to proceed directly with more definitive operative intervention.
Subsequently, the indication for operative management was established. Surgical intervention consisted of a curopsy type procedure, as described by Reddy et al, with evacuation of the material from the os calcis for biopsy and subsequent backfill with a combination autologous and allogeneic bone graft with the understanding this would serve as biopsy of the tumor as well as decreasing the tumoral burden, but not provide complete tumor resection.
Given the extent of involvement of the calcaneus, definitive treatment options in the present case would be major proximal amputation such as below knee amputation versus total calcanectomy with possible titanium implant, which would certainly be associated with significant morbidity. Given anatomic, functional, and biomechanical considerations, en bloc resection was not a viable option. Contents were evacuated using a unique orthopedics bone harvester system. The unique harvester utilized in this case is a suction powered curettage system with the ability to harvest both cancellous bone graft as well as bone marrow aspirate in the same device. The curette attached to the distal end of the system also allows for further debridement and physical capture of any infected, diseased or non viable bone. Pertinent intraoperative findings include altered density of the fascia and periosteum overlying calcaneus. The lateral wall of the calcaneus was noted to consist of a firm fibrous cortical cancellous bone (Fig. 2). During evacuation of the contents of the calcaneus and lesion, the area was noted to be extremely avascular and composed of a combination fibrous and myxoid tissue with some cancellous bone. Fully evacuating contents of the calcaneus and this lesion did involve the entire extent of the bone. Autologous bone graft was then obtained from the distal tibial metaphysis and was exponentially aided with an allograft demineralized bone matrix in order to fill the void created within the calcaneus. Final histopathologic examination of surgical specimens revealed a spindle cell neoplasm with abundant collagen. Neoplastic cells were focally arranged in a fascicular pattern with fragments of woven bone identified embedded within the tumor, determined by our pathology team to be most consistent with a desmoplastic fibroma (Fig. 3). Postoperative course involved a period of restricted weight bearing however otherwise the patient progressed uneventfully. At 16 months, the patient reported doing well with no recurrence of symptoms.
Desmoplastic fibroma is a rare, locally aggressive benign primary bone tumor usually affecting those in the first three decades of life, with no noted sexual predilection.
Differentials include giant cell, fibrous dysplasia, aneurysmal bone cyst, unicameral bone cyst, chondromyxoid fibroma, non ossifying fibroma, and potentially malignancies such as fibrosarcoma or osteosarcoma.
Radiographically, DF presents as a purely lytic lesion without any mineralized matrix. When seen in long bones, it is oval shaped and usually arises in the metaphysis and extends into the epiphysis and to the subchondral bone plate. DF characteristically has a narrow zone of transition without frank sclerosis. Centrally, the tumor demonstrates a lacelike appearance due to the reticular network of pseudo trabeculae. In recurrent DF, the trabeculation usually appears more dense and irregular.
CT findings are similar to radiographs but can provide a better picture in terms of overall osseous destruction. Magnetic resonance imaging should also be used as an adjunct imaging modality for diagnosis in order to evaluate the extent of soft tissue penetration.
Regarding treatment options for DF, due to a lack of available literature, treatment modalities often are subject to surgeon preference and experience. Wide resection, specifically en bloc resection, is supported by the literature as the treatment of choice, largely due to the high recurrence rates demonstrated by DF. In a 1996 study, Bohm et al reported a recurrence rate of 55-72% after non-resection procedures, and 17% after resection with a 3 year follow-up period. Within those that recurred, 25% progressed to a major amputation as treatment of recurrence. Based on these results, the authors suggested management with marginal or wide resections from the outset, provided it does not result in a significant functional deficit.
In the present case, as well as in many cases of the calcaneus, anatomic considerations precluded wide resection.
When lesion site or extent limits wide resection, aggressive intralesional curettage is another viable treatment option. Generally, this method has been reported to show higher rates of recurrence in comparison to wide resections, however some success has been shown. Nishida et al evaluated aggressive intralesional curettage extending beyond the sclerotic perilesional osseous shell with adjunct bone graft. Over a follow up period exceeding 5 years, no complications or recurrences were reported.
This is further supported by Stefanidis et al, who detailed no episodes of recurrence following curettage and bone grafting of two simultaneously growing lesions in the ilium and sacrum with more than 6 years of follow up.
While intralesional curettage was utilized in the present case in order to biopsy and mitigate tumor formation, given the extent of the tumor, this did not serve as definitive treatment but rather a form of symptom management. Although not used in the current case report, radiotherapy has been reported as another lesser studied, yet nevertheless successful option in the treatment of DF.
In conclusion, the above case report details a rare case of DF of the calcaneus in a 22 year-old female managed with curopsy technique biopsy and curettage with backfill with autologous bone graft augmented with allogeneic demineralized bone matrix. Limitations to this study include a relatively short follow-up period of 16 months, in the context of this neoplasm with quite high recurrence rates documented after up to 3 years follow up. Clinical and radiographic presentation regarding DF can be rather nonspecific, and as such it is rarely on the differential until diagnosed with histopathologic report. While DF in the calcaneus is rare and documentation is limited in the literature, cases are important to present and fill this void in the literature as anatomic considerations in this location will likely not allow for en bloc resection. As such, the presentation and management of DF of the calcaneus is critical to present due to the paucity of the literature surrounding this rare osseous neoplasm in the calcaneus, the high recurrence rates, and the high morbidity which would be associated with en bloc resection in this location.
Declaration of competing interests
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
Informed patient consent
Complete informed consent was obtained from the patient for the publication of this study and accompanying images.
Eva Patalas, MD and the pathology departments at Cambridge Health Alliance and Massachusetts General Hospital
Computed tomography and magnetic resonance imaging of desmoplastic fibroma with simultaneous manifestation in two unusual locations: a case report.