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Neglected Desmoplastic Fibroma of calcaneum with collapsed hindfoot, restored with allograft transplantation: A rare case report

Published:December 28, 2022DOI:https://doi.org/10.1016/j.jcot.2022.102088

      Abstract

      Desmoplastic fibroma of the calcaneum is a rare, locally aggressive tumour. A 24-year-old female presented with long-standing heel pain, with the collapse of the calcaneum (hindfoot) that was untreated for 7 years. Eradication of this locally aggressive lesion by adjuvant therapy and restoration of calcaneal bony morphology by allograft was an arduous and challenging task. At the final follow-up, the heel was painless, and the patient could walk normally without support.

      Conclusion

      Restoration of calcaneal height is challenging in young patients, and using structural allograft restores calcaneal and hindfoot morphology. Patient education about the high chances of recurrence despite surgical clearance is essential, and intraoperative adjuvant usage can reduce the recurrence.

      Keywords

      1. Introduction

      Desmoplastic fibroma is one of the rare bone tumours, reported in 0.06% of all bone tumours and 0.3% of benign bone tumours.
      • Said-Al-Naief N.
      • Fernandes R.
      • Louis P.
      • Bell W.
      • Siegal G.P.
      Desmoplastic fibroma of the jaw: a case report and review of the literature.
      • Böhm P.
      • Kröber S.
      • Greschniok A.
      • Laniado M.
      • Kaiserling E.
      Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.
      • Rabin D.
      • Ang L.C.
      • Megyesi J.
      • Lee D.H.
      • Duggal N.
      Desmoplastic fibroma of the cranium: case report and review of the literature.
      • Ikeshima A.
      • Utsunomiya T.
      Case report of intra-osseous fibroma: a study on odontogenic and desmoplastic fibromas with a review of the literature.
      Jaffe,
      • Jaffe Henry L.
      Tumors and Tumorous Conditions of the Bones and Joints.
      in 1958, first described this benign tumour identified from large collagen fibre formation by the tumour cells and termed it as "desmoplastic fibroma". Around 200 cases of desmoplastic tumours of bone have been published in the world literature. Reported incidences are in the mandible, femur, pelvic bones, radius and tibia,
      • Said-Al-Naief N.
      • Fernandes R.
      • Louis P.
      • Bell W.
      • Siegal G.P.
      Desmoplastic fibroma of the jaw: a case report and review of the literature.
      • Böhm P.
      • Kröber S.
      • Greschniok A.
      • Laniado M.
      • Kaiserling E.
      Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.
      • Rabin D.
      • Ang L.C.
      • Megyesi J.
      • Lee D.H.
      • Duggal N.
      Desmoplastic fibroma of the cranium: case report and review of the literature.
      and only 5 cases
      • Gebhardt M.C.
      • Campbell C.J.
      • Schiller A.L.
      • Mankin H.J.
      Desmoplastic fibroma of bone. A report of eight cases and a review of the literature.
      • Rastogi S.
      • Varshney M.K.
      • Trikha V.
      • Khan S.A.
      • Mittal R.
      Desmoplastic fibroma: a report of three cases at unusual locations.
      • Park J.W.
      • Kang H.G.
      • Lim K.M.
      • Kim J.H.
      • Kim H.S.
      Three-dimensionally printed personalized implant design and reconstructive surgery for a bone tumor of the calcaneus: a case report.
      of the calcaneal desmoplastic lesion have been reported in the literature until 2018. Desmoplastic fibroma in bone tissue is a locally aggressive and invasive benign lesion. The fibroblasts and collagenous matrix tissue in desmoplastic fibroma resemble a desmoid soft tissue tumour.
      • Said-Al-Naief N.
      • Fernandes R.
      • Louis P.
      • Bell W.
      • Siegal G.P.
      Desmoplastic fibroma of the jaw: a case report and review of the literature.
      ,
      • Böhm P.
      • Kröber S.
      • Greschniok A.
      • Laniado M.
      • Kaiserling E.
      Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.
      The recurrence rates after surgical clearance have been reported to be between 37% and 72%.
      • Böhm P.
      • Kröber S.
      • Greschniok A.
      • Laniado M.
      • Kaiserling E.
      Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.
      ,
      • Inwards C.Y.
      • Unni K.K.
      • Beabout J.W.
      • Sim F.H.
      Desmoplastic fibroma of bone.
      ,
      • Stefanidis K.
      • Benakis S.
      • Tsatalou E.
      • Ouranos V.
      • Chondros D.
      Computed tomography and magnetic resonance imaging of desmoplastic fibroma with simultaneous manifestation in two unusual locations: a case report.
      Treatment options like intra-lesional curettage, wide excision, microsurgical transfers, etc., have been advocated. Thus, the need for attention from the treatment perspective is essential for managing these rare lesions.
      • Böhm P.
      • Kröber S.
      • Greschniok A.
      • Laniado M.
      • Kaiserling E.
      Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.
      • Rabin D.
      • Ang L.C.
      • Megyesi J.
      • Lee D.H.
      • Duggal N.
      Desmoplastic fibroma of the cranium: case report and review of the literature.
      • Ikeshima A.
      • Utsunomiya T.
      Case report of intra-osseous fibroma: a study on odontogenic and desmoplastic fibromas with a review of the literature.
      However, to our knowledge, this is the first report of calcaneal lesion presenting with pathological fracture and collapse of calcaneum in a young patient. Restoration of calcaneal and hindfoot height is paramount in young patients to revamp hindfoot biomechanics to achieve an excellent outcome and patient satisfaction. We present a case of desmoplastic fibroma in calcaneum, the relevant diagnostic evaluation and treatment challenges.

      2. Case report

      A 24-year-old female came to our clinic with severe pain in the heel on weight-bearing and walking. She had pain in her left heel for 7 years, which was insidious onset, less painful and intermittent to start with, then gradually progressed until 2 weeks before presentation in our clinic. Her pain worsened, and she could not walk without the support of crutches. A biopsy of the calcaneal lesion was done in a different institution 4 years before the presentation and was diagnosed as Desmoplastic fibroma of the calcaneum. However, the patient was hesitant for further treatment due to personal reasons and fear of prognosis. She presented with severe pain, limp, and antalgic gait with forefoot loading and walking with elbow crutches. Clinical examination of her left heel revealed healed surgical scar over the lateral aspect of the old biopsy (Fig. 1J). The heel was tucked up, shortened and broadened, loss of normal contour of tendoachilles, associated with flat feet and hindfoot valgus, when inspected from a posterior aspect with prevailing too many toes sign. The palpation – of the lateral side of the heel revealed boggy swelling compared to the unaffected side; the heel was tender to palpate on all three sides, and posterior bony prominences of calcaneum were less prominent than the right side (unaffected side). Palpation of the hindfoot aspect revealed a tiny heel and tendo-achilles tucked up (Fig. 1J). The clinical measurement of the heel on the lateral surface from the tip of the lateral malleolus to the sole showed a 22mm loss of heel height compared to the opposite side (Fig. 1H and I) and movements of ankle and subtalar joint on the affected side was painful.
      Fig. 1
      Fig. 1Pre-operative radiological picture of the lesion since 7 years of symptomatic phase to untill presentation in 2018, 1A) X-ray of the patient 7 years prior to admission (taken in 2011) showing well defined osteolytic lesion with clear zone of transition in the body, anterior process with thinning out of lateral cortex and posterior part of calcaneum with intervening thick trabeculae separating the body lesion and posterior lesion, 1B,C)X-ray taken in 2012 showing with expansion and ballooning of lateral cortex and increase in osteolytic lesion and thinning out of intervening intra-lesional trabeculae, MRI showing increased signal intensity with loss of bony architecture of calcaneum, 1D,E) X-ray taken in 2014 and 2016 showing similar findings as the previous x-rays but with further loss of intervening intra-lesional trabecular, clear matrix and expansion of posterior calcaneal lesion, 1F,G,) X-ray and CT scan (2018) of the patient showing complete collapse of the calcaneum with destruction of bony architecture, loss of body, superior surface, lateral wall and part of anterior process. 1H, I) clinical picture at the time of presentation in 2018- Loss of heel height compared to the opposite side, 1J) Outer view/lateral border of the left foot while patient on standing shows old biopsy surgical scar, 1K) Inner view/medial border of the foot shows flat foot due to loss of calcaneal inclination/calcaneal height.
      At the time of presentation in our clinic, the patient shared her old x-rays (Fig. 1) and MRI scan that were taken from her onset of symptoms till date (2011–2018). Old x-rays showed an expansile osteolytic lesion in the calcaneum with clear margins. The successive x-rays showed progress in size, expanding into the lateral wall, anterior process, body of calcaneum, posterior aspect sparing medial wall and posterosuperior prominence. These radiological findings were from the previous x-rays (Fig. 1A, B, C, D, E).
      At the time of presentation, it was seen that the collapse of the calcaneum with the superior cortex and subtalar articular surface of the calcaneum fell within the bony confinement of the calcaneum (Fig. 2F). The patient was further evaluated with weight-bearing x-rays in lateral view and Saltzmann's (weight-bearing axial view of hindfoot). Various measurements of the Hindfoot (Fig. 2A, B, C, D, E, F) were quantified to describe calcaneal patho-anatomy and for pre-operative workup and planning. CT scan was done to evaluate the amount of destruction of the calcaneum and to measure the height, width and length of calcaneum of normal and affected sides (Fig. 2D). The patient's previous biopsy was reported as a desmoplastic fibroma. Hence, a detailed discussion was carried out with the patient - about the surgical clearance, potential lesion recurrence after intra-lesional curettage and the need for restoration of hindfoot height, width and alignment with structural allograft. Given the high recurrence of Desmoplastic fibroma, option of complete enblock excision of the lesion with the entire calcaneum was discussed with the patient. Further, the drawback in calcaneal preservation surgery, the need for repeat curettage and excision of native bone – for future recurrence, was also discussed in detail. The patient, young and unwilling to complete the excision of calcaneum, consented to curettage and allograft insertion to restore calcaneal bony morphology.
      Fig. 2
      Fig. 2Pre-operative x-rays of affected side (left) and normal side comparing the radiological parameters of amount of collapse, 2A, B, C, D) showing loss of hindfoot height by 22mm when compared to the right side in x-ray and CT scan, 2E, F,) showing the calcaneal inclination of 14.40 (A), reversed Bohler's angle of −34.40 (B), loss of 5th metatarsal base height (C) -on comparing the normal side, 2G) Showing the loss of 15mm of hindfoot bony calcaneal width due to destruction of lateral cortex by the lesion.

      3. Surgical technique

      The patient was taken up for the surgical procedure under regional anaesthesia. Through an extended lateral approach, the sural nerve and peroneal tendons were identified and protected (Fig. 3A, B, C). Tendoachillies Z-plasty was done with the same surgical approach (Fig. 3D and E). The subtalar joint was exposed, and a laminar spreader was inserted; greyish brown tissue was thoroughly curetted from the anterior process, body, medial wall and posterior wall of the calcaneum. Sample tissue was sent to two different onco-pathologists for histopathological examination. Adjuvant therapy like high-speed burring of the calcaneal bony walls and chemical cauterization with surgical spirit (methyl-salicylate −80% alcohol) was instituted. Surgical spirit was copiously irrigated, left instilled for 4–5minutes, and a thorough wash was given to the cavity. Tibial bone allograft (Fig. 3F) was taken based on our pre-operative measurements of normal side calcaneum from the CT scan; the allograft was prepared and trimmed to fill the void in the calcaneum (Fig. 3I and J). Multiple drill holes (Fig. 3G) were made on the surface for better incorporation of allograft into the talus and calcaneal wall. The subtalar articular surface of the calcaneum and talus was denuded, and allograft was placed in the void. Then under an Image intensifier, a guidewire was passed into the calcaneum, allograft and talar bony for subtalar fusion through the posterior approach. Fixation was completed with 7mm cannulated screws of appropriate length. Gain in the approximate length, width and height were appreciated in the image intensifier. (Fig. 3J and K).
      Fig. 3
      Fig. 3Operative pictures of the patient, 3A, B) showing through extensile lateral approach lesion was exposed, sural nerve and peroneal tendons were identified and protected, 3C) Showing opening of the subtalar joint with the laminar spreader and the collapsed calcaneum was opened up, 3D, E) due to tucked up heel with loss of calcaneal inclination, 'z-plasty' of the Tendo-Achilles was done, 3F) Appropriate pre-operative CT scan measurement (length, width and height) is marked, and the allograft is cut, 3G) Multiple drill holes are being made in the allograft to improve the bone ingrowth/osteoconduction and better incorporation of the allograft; 3H, I) Intra-operative C-arm picture showing the collapsed calcaneum is being opened up and held by the laminar spreader instrument, 3J, K) Allograft was placed inside the void in the calcaneum after thorough curettage of the lesion, and fixed by 7mm cannulated cancellous screws across the subtalar joint.
      The wound was closed in the layers, and a below-knee cast was applied. Suture removal was done after 2 weeks, and the cast was maintained for 3 months. The patient was asked to follow strict non-weight bearing walking for 3 months, then partial weight-bearing with a protective cam boot for another 3–4 weeks. Full weight-bearing is allowed only after 4months from the index surgery. The patient was regularly followed up with x-rays and an AOFAS Hindfoot score. Patient's x-rays were assessed clinically for symptoms and radiologically for loss of height, calcaneal width, incorporation of allograft and any recurrence (Fig. 4 C, D, Table 1). At the time of the final follow-up of 2 years, the patient showed well-incorporated allograft and 3–4mm of loss of height in lateral and Saltzman's view. There was no change in calcaneal width at the time of the final follow-up. However, there were minimal lytic changes around the anterior and inferior aspect of the calcaneum in final follow-up x-rays and CT scans. The patient was asymptomatic and able to walk without pain. There were no other complications like infection/implant cut-out in this patient.
      Fig. 4
      Fig. 4Postoperative follow-up x-ray and histological picture of the patient (Hematoxylin and Eosin with a magnification of 10x and 100x) 7A) Immediate postoperative x-ray, 7B) Histological picture of the lesion showing bone trabeculae and proliferating fascicles of plump spindle cells in interlacing fascicles and entrapping an osteoblast lined bone trabecula, where these features are compatible with a benign desmoplastic fibroma.7C, D) postoperative follow-up measurement of the hindfoot height and heal width.
      Table 1Tabular column showing progressive calcaneal hindfoot height measurement in the follow-up x-ray. At the final follow-up, hindfoot height is reduced by 3–4mm in both the views, however heel width is being maintained.
      Hind foot HeightRight side (Normal side)Left side (Affected side)
      PreopPostop3months9 months2 yr 4 months
      Lateral view70.9748.2058.9658.8957.6555.52
      Axial view83.9761.3778.8978.5676.5474.66
      Heal width26.6611.3024.9724.924.924.9

      4. Discussion

      Untreated/neglected desmoplastic fibroma of the calcaneus can present with a pathological fracture and collapse of the calcaneum. The critical takeaway from this case report is adequate to incite for prevention of recurrence by the institution of adjuvant surgical measures and restoration of hindfoot height for optimum results. Most lytic lesions in the foot occur during adolescence.
      • Bernstein A.L.
      • Jacobs A.M.
      • Oloff L.M.
      • Gilula L.
      Cyst and cystlike lesions of the foot.
      The exact diagnosis of these benign tumours is not always definitive, and treatment depends on the aggressive behaviour of the lesion. In our case, the lytic lesion occupied a large area of the calcaneus with the collapse of the calcaneum, resulting in severe pain during walking. Presenting symptoms are pain and swelling for a long duration, which precedes functional disability and pathological fracture. Radio-graphically, it usually presents as a lytic meta-diaphyseal lesion without any mineralized matrix and often has a honey-combed/soap-bubble appearance.
      • Böhm P.
      • Kröber S.
      • Greschniok A.
      • Laniado M.
      • Kaiserling E.
      Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.
      ,
      • Rabin D.
      • Ang L.C.
      • Megyesi J.
      • Lee D.H.
      • Duggal N.
      Desmoplastic fibroma of the cranium: case report and review of the literature.
      ,
      • Crim J.R.
      • Gold R.H.
      • Mirra J.M.
      • Eckardt J.J.
      • Bassett L.W.
      Desmoplastic fibroma of bone: a radiographic analysis.
      Radiologically, the lesion shows a diffuse pattern of bone destruction with a very narrow transition zone which can be seen in our case. The cortical break is evident in 29% of cases of this tumour but should not be misdiagnosed as a sign of malignancy.
      • Crim J.R.
      • Gold R.H.
      • Mirra J.M.
      • Eckardt J.J.
      • Bassett L.W.
      Desmoplastic fibroma of bone: a radiographic analysis.
      In our case, the destruction of the lateral wall of the calcaneum from the time of the first x-ray.
      Further, serial x-rays showed gradual destruction of the bone matrix with an expansion of osteolytic lesions. Our case is a unique presentation with the collapse of the calcaneum and pathological fracture, which is not reported in the literature. Thus, managing collapsed calcaneum in a young patient was a challenging scenario, where the care of complete excision with adjuvant therapy needed to reduce the chances of recurrence and restoration of calcaneal anatomical morphology was essential to achieve the desired result. Biological calcaneal preservation and reconstruction using vascularized or pedicle osteo-fascio-cutaneous flaps with the fibula
      • Li J.
      • Wang Z.
      Surgical treatment of malignant tumours of the calcaneus.
      or the iliac crest
      • Scoccianti G.
      • Campanacci D.A.
      • Innocenti M.
      • Beltrami G.
      • Capanna R.
      Total calcanectomy and reconstruction with vascularized iliac bone graft for osteoblastoma: a report of two cases.
      or with allografts have been used.
      • Woźniak W.
      • Raciborska A.
      • Walenta T.
      • Szafrański A.
      • Szymborska A.
      • Bajor M.
      New technique of surgical treatment of malignant calcaneal tumours. Preliminary report.
      Calcanectomy without reconstruction
      • Geertzen J.H.B.
      • Jutte P.
      • Rompen C.
      • Salvans M.
      Calcanectomy, an alternative amputation? Two case reports.
      has also been advocated for malignant lesions. Recently, 3D printed implants
      • Park J.W.
      • Kang H.G.
      • Lim K.M.
      • Kim J.H.
      • Kim H.S.
      Three-dimensionally printed personalized implant design and reconstructive surgery for a bone tumor of the calcaneus: a case report.
      and calcaneal reconstructive surgery for a bone tumour of the calcaneus have been reported. However, the possible long-term complication of painful peri-implant arthritis, loosing, metalosis, wound problems due to end bone loading, and additional cost for staging and 3D printing of implant looms over its usage.
      The shortcomings of this case report are though the foot is salvaged, the long-term repercussions from the loss of height and ankle arthritis can be expected.
      In summary, the management of collapsed calcaneum in a young patient is a challenging scenario. Preservation of the calcaneum and restoration of the height of the calcaneum and hindfoot give reasonable patient satisfaction. The clinical and functional outcomes of the surgery were acceptable. In treating benign lesions, the structural allograft shaped to fill the void is a feasible skeletal reconstructive option for limb-salvage surgery. Patients must be explained the high chances of recurrence of these lesions after surgical clearance.

      Patient informed consent statement

      The authors declare that informed patient consent was taken from the patients.

      Declaration of competing interest

      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.

      Acknowledgement

      Nil.

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