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| CASE REPORT |
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| Year : 2020 | Volume
: 17
| Issue : 2 | Page : 72-76 |
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Symptomatic nonossifying fibroma: Is prophylactic fixation warranted?
George Shaji, Ronald Joseph Menezes
Department of Orthopaedics, Father Muller Medical College and Hospital, Mangalore, Karnataka, India
| Date of Submission | 29-Jan-2021 |
| Date of Acceptance | 30-Jan-2021 |
| Date of Web Publication | 08-Mar-2021 |
Correspondence Address:
George Shaji
Department of Orthopaedics, Father Muller Medical College and Hospital, Kankanady, Mangalore - 575 002, Karnataka
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/joasis.joasis_4_21
Nonossifying fibroma (NOF) is a nonneoplastic lesion of the long bones which are rarely seen after skeletal maturity. The disorder is most often asymptomatic, discovered incidentally on plain radiographs and spontaneous regression over the years is typical. Nevertheless, larger lesions are at an increased risk of pathological fracture and together with atypical radiographic features can cause concern leading to consultations in orthopedic oncology clinics. A correct diagnosis can be established after a careful study of the patient's history and clinical status together with different imaging modalities and histologic characterization of the lesion. Surgical treatment may be required to forestall fractures in symptomatic lesions. Recurrence is rare after surgical intervention. Here, we report an interesting case of a pathological fracture through a symptomatic NOF with a 1-year follow-up, after surgical intervention.
Keywords: Nonneoplastic, nonossifying fibroma, pathological fracture, surgical interve
How to cite this article:
Shaji G, Menezes RJ. Symptomatic nonossifying fibroma: Is prophylactic fixation warranted?. J Orthop Assoc South Indian States 2020;17:72-6 |
How to cite this URL:
Shaji G, Menezes RJ. Symptomatic nonossifying fibroma: Is prophylactic fixation warranted?. J Orthop Assoc South Indian States [serial online] 2020 [cited 2023 Apr 1];17:72-6. Available from: https://www.joasis.org/text.asp?2020/17/2/72/310998 |
| Introduction | |  |
Termed by Jaffe and Lichtenstein as “nonosteogenic fibroma of the bone,” and later classified as a tumor-like lesion by the World Health Organization, the nonossifying fibroma (NOF) is a nonneoplastic bone lesion, which is histologically characterized by a benign fibroblastic proliferation admixed with osteoclast type giant cells.[1],[2] It belongs to the group of developmental abnormalities with a predilection for the metaphyseal region of long bones.[1],[3]
NOFs are typically asymptomatic and found incidentally on imaging for separate complaints. The affected sites include distal femur, proximal and distal tibia, fibula, and proximal humerus.[3] Although its real incidence is unknown, it is estimated that 30%–40% of children may have one or more undetected lesions.[4],[5] Lesions seen after skeletal maturity, though uncommon, typically have a good prognosis as spontaneous regression over the years is typical.[6],[7],[8]
However, larger lesions can predispose to pathological fractures.[5],[8],[9] Such lesions may require surgical intervention.[10],[11],[12] Correlation with histopathological features is imperative for diagnosis.[4],[10]
| Case Report | | |
A 35-year-old manual laborer had a trivial fall at his workplace, following which he had pain with deformity of his left thigh. Radiograph taken at a nearby hospital showed a subtrochanteric left femur fracture. Upon suspicion, magnetic resonance imaging of the extremity was done [Figure 1]. | Figure 1: Magnetic resonance imaging report of the pelvis with the left femur, suggesting a pathological fracture through a probably benign lesion in the subtrochanteric region of the left femur
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Subsequently, the patient was referred to our tertiary care hospital. On presentation to the casualty, the patient gave a history of left thigh pain, present for over a year, which had aggravated for the past 6 months affecting work, for which he got a radiograph done [Figure 2] and was treated with analgesics. | Figure 2: Anterior–posterior radiograph of the pelvis with bilateral proximal femurs, white arrow showing a solitary multiloculated lytic lesion involving more than 50% of the cortex with a thin sclerotic rim in the meta-diaphyseal region of the subtrochanteric region of the left femur
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In casualty, the limb was immobilized in a Thomas splint and a radiograph was taken [Figure 3]. Routine laboratory workup was done and after informed consent, the patient was posted for surgery the following day. | Figure 3: Anterior–posterior and lateral radiograph of the left hemipelvis with the proximal femur, the arrow showing a pathological subtrochanteric femur fracture, immobilized in a Thomas splint
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In the operation theater, after obtaining an adequate exposure of the fracture site, an intralesional curettage and lavage was done [Figure 4]a, [Figure 4]b, [Figure 4]c. The curetted sample was sent for histopathological analysis. An anterograde femoral nail was used to fix the fracture [Figure 5]a and [Figure 5]b. Cancellous bone graft harvested from the ipsilateral iliac crest was used to fill the defect from curettage [Figure 6]. A postoperative radiograph was taken [Figure 7]. | Figure 4: Intraoperative pictures of subtrochanteric fracture region of the left femur. (a and b) showing intralesional curettage and lavage using a high-speed burr; and (c) showing the defect post curettage prior to nail fixation
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 | Figure 5: (a and b) Fluoroscopic images showing nail fixation of the left femur
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 | Figure 7: Postoperative check radiograph showing anterior–posterior and lateral images of the left femur with anterograde femoral nail in situ
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Postoperative period was uneventful. The patient was mobilized using crutches, with a strict nonweight bearing on the left lower limb which was continued for 3 months. Staples were removed at 2 weeks. Partial weight-bearing was started by 4th month and full weight-bearing by 6th month, after clinicoradiological assessments at follow-ups.
| Results | | |
Samples containing multiple fibrocollagenous tissues and bony bits, largest measuring 3 cm × 1 cm × 0.5 cm, were received for histopathological analysis. At × 100 magnification, the slides studied showed features suggestive of NOF [Figure 8]a, [Figure 8]b, [Figure 8]c. No cytological atypia was noted. | Figure 8: H and E-stained slides under high-power (×100) view with red arrows showing (a) spindle cells arranged in storiform pattern with foamy macrophages; (b) hemosiderin-laden macrophages; (c) osteoclast-like giant cells
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At 2nd, 7th, and 1-year follow-up, the progress was uneventful and the fracture had healed with no signs of recurrence on radiographs [Figure 9],[Figure 10],[Figure 11]. The patient resumed his routine work without difficulty. | Figure 9: Second-month follow-up radiographs of the left proximal femur showing uniting fracture
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 | Figure 10: Seventh-month follow-up check radiograph of the left proximal femur showing union at the fracture site
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 | Figure 11: One-year follow-up radiograph of the left proximal femur showing good union with no signs of recurrence at the fracture site
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| Discussion | | |
In view of its self-limiting behavior, several authors consider NOFs as a “no touch” lesion and recommend conservative management.[5],[7],[8]
Occasionally, NOFs can cause pain and swelling, especially when associated with athletic activity.[13] In addition, large NOFs are painful, particularly if a fracture has occurred.[3],[8]
Radiologically, NOF typically appears as a solitary, multiloculated, eccentric, and ovoid lesion, with a sclerotic rim and no associated periosteal reaction but often causes thinning and expansion of the overlying cortex. The margins can range from being densely sclerotic, or scalloped, to being hazy and indistinct.[3],[4],[5],[7]
Evolution of NOFs was classified by Ritschl into four progressive stages.[7] This variability in radiological presentation raises concerns leading to consultation in orthopedic oncology clinics.[7],[14] The initial radiograph of our patient prior to sustaining the fracture was in Stage-B of Ritschl evolution.
Correlation with histopathology is imperative to differentiate NOF from other benign lytic lesions of the bone.[10] The main entity in the histologic differential is the “fibrous cortical defect” (FCD), which is a small lytic intracortical lesion, typically eccentrically located and sharply outlined. Due to their small size, FCDs are asymptomatic and undergo spontaneous regression.[6],[7] Occasionally, they enlarge and separate from the growth plate to extend into the medullary cavity.[2]
NOF is distinguished by its larger size and extension into the medullary cavity.[1],[2],[3] Smaller lesions may be watched while enlarging lesions can cause pathological fracture from the cortical breach.[5],[6],[7],[8] The principal site where the limb may be at risk is the proximal femur.[3]
Herget et al. concluded that patients with stage-B lesions have an increased risk of suffering a fracture and therefore must reduce strenuous activities and must be closely monitored with a 6- to 12-month interval.[5]
A similar conclusion was drawn from a study by Arata et al., in which pathological fractures through NOF occurred when the percentage of bone occupied exceeded 50% in both the planes (anterior–posterior and lateral), and the vertical length exceeded 33 mm in all nonfibular lesions.[8]
However, in a study conducted by Easley and Kneisl, 59% cases had large NOFs that did not fracture. They concluded that most patients can be monitored closely without intervention, as majority of the lesions resolved spontaneously.[15]
Many surgical procedures have been described to forestall fractures such as internal metallic devices, autograft bone, or allograft chips with or without cement.[8],[11],[16] Curettage followed by autologous bone grafting in conjunction with internal fixation can correct the deformity as well as provide pain relief and allow individuals to resume their activities.[10],[11],[12]
In our case, the pathological fracture through the lesion in the proximal femur was managed surgically by curettage, nail fixation, and bone grafting. Histopathology confirmed the diagnosis. The patient resumed his routine work at the end of 1-year follow-up.
| Conclusion | | |
Large symptomatic NOF can place the patient at risk for developing a fracture requiring serial radiograph monitoring with a 6- to 12 month interval. Although majority of the cases can be monitored without intervention, surgical treatment may be justified to forestall fractures. Curettage and bone grafting are indicated in such cases with prophylactic fixation whenever needed.
Acknowledgment
The author is grateful to the department of pathology at his institution.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Ethical clearance
Case Report has been approved by the local institutional ethics committee [FMMCIEC].
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Jaffe HL, Lichtenstein L. Non-osteogenic fibroma of bone. Am J Pathol 1942;18:205-21.  |
| 2. | Nielsen GP, Kyriakos M. Fibrohistiocytic tumors. In: Fletcher CD, Bridge J, Hogendorn PC, Mertens F, editors. WHO Classifications of Tumours of Bone and Soft Tissue. Lyon: IARC Press; 2013. p. 301-4. |
| 3. | Mankin HJ, Trahan CA, Fondren G, Mankin CJ. Non-ossifying fibroma, fibrous cortical defect and Jaffe-Campanacci syndrome: A biologic and clinical review. Chir Organi Mov 2009;93:1-7. |
| 4. | Dahlin DC. Fibroma (nonosteogenic fibroma of bone, metaphyseal fibrous defect), myxoma, cortical desmoid, fibromatosis, and ''Xanthoma''. In: Thomas CC, editor. Bone tumors. General Aspects and Data on 6,221 Cases. 3 rd ed. Springfield, IL: Charles C Thomas; 1978. p. 122-36. |
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| 6. | Pitcher JD, Weber KL. Benign fibrous and histiocytic lesions. In: Schwartz HS, editors. Orthopaedic Knowledge Update Musculoskeletal Tumours. 2 th ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007, p. 121-32. |
| 7. | Ritschl P, Karnel F, Hajek P. Fibrous metaphyseal defects- determination of their origin and natural history using a radiomorphologic study. Skeletal Radiol 1988;21:179-89. |
| 8. | Arata MA, Peterson HA, Dahlin DC. Pathological fractures through non-ossifying fibromas. Review of the mayo clinic experience. J Bone Joint Surg Am 1981;63:980-8. |
| 9. | Shimal A, Davies AM, James SL, Grimer RJ. Fatigue-type stress fractures of the lower limb associated with fibrous cortical defects/non-ossifying fibromas in the skeletally immature. Clin Radiol 2010;65:382-6. |
| 10. | Moretti VM, Slotcavage RL, Crawford EA, Lackman RD, Ogilvie CM. Curettage and graft alleviates athletic-limiting pain in benign lytic bone lesions. Clin Orthop Relat Res 2011;469:283-8. |
| 11. | Hase T, Miki T. Autogenous bone marrow graft to non-ossifying fibroma with a pathologic fracture. Arch Orthop Trauma Surg 2000;120:458-9. |
| 12. | Andreacchio A, Alberghina F, Testa G, Canavese F. Surgical treatment for symptomatic non-ossifying fibromas of the lower extremity with calcium sulfate grafts in skeletally immature patients. Eur J Orthop Surg Traumatol 2018;28:291-7. |
| 13. | De Mattos CB, Binitie O, Dormans JP. Pathological fractures in children. Bone Joint Res 2012;1:272-80. |
| 14. | Stacy GS, Dixon LB. Pitfalls in MR image interpretation prompting refferals to an orthopaedic oncology clinic. Radio Graph 2007;27:805-28. |
| 15. | Easley ME, Kneisl JS. Pathologic fractures through nonossifying fibromas: Is prophylactic treatment warranted? J Pediatr Orthop 1997;17:808-13. |
| 16. | Drennan DB, Maylahn DJ, Fahey JJ. Fractures through large non-ossifying fibromas. Clin Orthop Relat Res 1974;103:82-8. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]
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