A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Bone Cysts Presenting with a Pathological Fracture

MOJ Orthopedics & Rheumatology

Abstract Case Report

Non-ossifying fibroma is a benign tumor primarily of childhood and adolescence.

index of suspicion should always be maintained in order to make the correct Volume 10 Issue 1 - 2018 It is the most common tumor encountered in the pediatric population. A high

differentialdiagnosis. Although diagnosis the owing expected to its adulthood characteristic hypersclerosis radiological usually appearance takes place and naturalin the primary history. lesion,The occurrence its absence of shouldsuch entity never in eliminate an adult thiswith entityalready from closed the 1Department of Orthopaedics, King Fahad Hospital in Hofuf, Kingdom of Saudi Arabia 2Department of Orthopaedic Surgery, King Hamad University ofphysis; the pathophysiology and in less common of the locations; disease wille.g. certainlydistal tibia, enhance raises ourthe approach.challenge Wefor Hospital, Kingdom of Bahrain appropriate interpretation of the radiological features. A better understanding 3College of Medicine, King Faisal University, Kingdom of Saudi aneurysmal bone cyst changes of the distal tibia that was complicated by a Arabia present a unique case of an adult-type non-ossifying fibroma with secondary *Corresponding author: pathologic fracture. Although described in literature, to our knowledge and +97333366903; Email: Eyad Alqasim, King Hamad Keywords:following extensive research, it is rarely well-documented. University Hospital, P.O. Box 24343, Muharraq, Bahrain, Tel: Benign tumor; Non-ossifying fibroma; Pathological fracture; Received: | Published: Aneurysmal bone cyst; Fibrous cortical defect; Cortical desmoid January 25, 2018 February 05, 2018

Introduction Case Presentation Various terms have been suggested to describe non-ossifying emergency department complaining of pain in the right lower leg andA ankle 23-year-old following healthy a trivial twisting male presented injury to the to right the accidentankle while & fibroma including fibrous cortical defect, cortical desmoid, osteoclastogenesismetaphyseal fibrous at the defect metaphyseal and non-osteogenic region. Non-ossifying fibroma. and mild swelling to the right lower leg and ankle with no visible Regardless of the term used, they probable all refer to abnormal training in the gym. On examination, the patient had severe pain and adolescents. It is the most common childhood benign tumour status. Initial radiologic examination revealed a distal third tibia infibroma which isit isa benignfound in fibrogenic 30% of children tumor primarilywith open affecting physis. It children initially deformity, normal skin condition and intact distal neurovascular

lyticand fibulalesion longwith obliquehypersclerotic displaced margins fractures and athat narrow were transitional associated affects the metaphysis of long bones, most commonly the distal zonewith a(Figure roughly 1).3cm Routine x 2cm well-definedlaboratory investigationsexapnsile, lobulated including and closefemur to followed skeletal maturity by the proximal it transforms and into distal a hypersclerotic tibia. However, scar. as growth takes place, the lesion extends into the diaphysis and once Clinical features are usually unremarkable and the patient pathologicala complete bloodfracture count, with a liver differential function diagnosis test and of renal non-ossifying function test did not reveal any abnormalities. An initial diagnosis of is asymptomatic, unless the lesion is complicated by a fracture. to its characteristic metaphyseal lytic lobulated pattern that is fibroma versus chondromyxoid fibroma was, therefore, made. The The lesion may easily be diagnosed by plain film radiographs due patient was, accordingly, planned for bone curettage and primary fixation of the distal tibia and fibula. surrounded by a well-defined sclerotic rim. Although not required connectivefor the diagnosis, tissue histopathologicalmatrix with abundant examination lipophages may giant demonstrate cells and by aThe 1/3 tubular fracture plate was and first screws. approached This was followed via an by anterolateral a complete hemosiderincharacteristic pigmentation. spindle cells with elongated nuclei in a fibroblastic curettageapproach toof thethe distal lesion fibula and inbone which grafting the fibula with fracture cancellous was bonefixed The mainstay of treatment is conservative. In secondary chips allograft. Finally, the tibia fracture was fixed by minimally invasive percutaneous osteosynthesis (MIPO) technique using pathological fractures, however, the approach depends on 3.5mm locked plate medially (Figure 2). The excised bone material patient age. In very young patients, a simple full cast may suffice (Figureswas then 3- sent 5). for histopathological examination which confirmed to stabilize the fracture, while in older children curettage and a non-ossifying fibroma with secondary aneurysmal bone cysts surgical fixation may be necessary.

Submit Manuscript | http://medcraveonline.com

MOJ Orthop Rheumatol 2018, 10(1): 00387 A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Copyright: 2/4 Bone Cysts Presenting with a Pathological Fracture ©2018 Alsaleem et al.

Figure 1:

hypersclerotic Initial borders. injury AP and lateral x-rays - distal tibia and fibula Figure 4: fractures with an associated expansile, lobulated and lytic lesion with Histopathological slide of the lesion-high-power magnification showing characteristic spindle cell fibroblastic proliferation consistent with non-ossifying fibroma.

Figure 2:

Immediate post-operative AP and lateral x-rays - fibula andfracture screws. fixed with a 1/3 tubular plate and screws with the lesion curretaged and filled with allograft; and the tibia fixed by MIPO plate Figure 5: Histopathological slide of the lesion-high-power

secondary aneurysmal bone cysts. magnification demonstrating blood-filled spaces consistent with The patient was initially placed on a non-weight bearing protocol for eight weeks with only crutch and touch-toe

ambulation. Follow-ups were carried out at two weeks, four weeks, eight weeks, sixteen weeks, eight months and one year post-operatively. At eight weeks, the ankle range of motion was regained and a healing callous started to form. The patient was, atherefore, painless allowedfull range to of partially ankle motion bear weight. and x-rays At 4 revealed months post-good incorporationoperatively, full of weight the allograft bearing (Figurewas started 6). Examinationwhen the patient at eight had months and 1 year post-operatively revealed full ankle strength

Figure 3: defect and total fracture healing (Figure 7). revealing abundant osteophytes and hemosiderin pigmentation. and range of motion restoration with a complete filling of the Histopathological slide of the lesion-high-power magnification

Citation:

Alsaleem MK, Alqasim E, Aleid ZM, Alhathloul HS, Alharbi NM et al. (2018) A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Bone Cysts Presenting with a Pathological Fracture. MOJ Orthop Rheumatol 10(1): 00387. DOI: 10.15406/mojor.2018.10.00387 A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Copyright: 3/4 Bone Cysts Presenting with a Pathological Fracture ©2018 Alsaleem et al.

lobulated lesions without sclerotic margins. These are usually classification, stage A includes small, oval, possible slightly includes lesions that are both lobulated and sclerotic. The location ofeccentrically these lesions located varies independing the cortex on the near rate the of physis.growth. StageIn stage B

rarityC, the oflesion occurrence becomes of mineralized.the classical earlyFinally, and stage primitive D includes features fully of thesclerotic lesion lesions.in adulthood This as classification was demonstrated system in indeed our patient. proves the

The lesion is usually asymptomatic, but it is not uncommon ifto the encounter lesion is non-ossifyinglarge or associated fibroma with patients some degree presenting of trauma with at least minimal symptoms such as mild discomfort, especially surrounding tissue and/or weakening of the bone [5]. [1,4]. The presence of pain may be related to compression to the

the risk of developing a pathological fracture in a non-ossifying Figure 6: The authors agree with Arata et al. [6] who suggested that osteosynthesis and good incorporation of the allograft within the long or occupies more than 50% of the transverse diameter of the lesion. AP and lateral x-rays 4 months post-operatively-maintained bonefibroma bone increases if the primary lesion is more than 33mm

[6]. This conclusion may be applied adequately to our patient whoseThe initialdiagnosis radiographs is usually perfectly incidental demonstrated during such radiological findings. evaluation for other reasons

not necessary [4]. Due to its characteristic demonstratesradiological appearance, characteristic pathological features such confirmation as a longitudinal is usually [5,7]. This is especially true when the lesion

growth pattern, lobulated and bubbly appearance with sclerotic border, and intact cortex [7]. If however, any of these features is breached or in complicated cases, biopsy may be indicated xanthoma[7]. Histologically, cells the lesion reveals storiform fibrous tissue notwith present spindle in cells, the primary multinucleated lesion. The giant presence cells, hemosiderin of such changes and is probably attributed[8]. Secondary to the aneurysmalcomplicated bone pathological cysts are fracture usually

[7]. Figure 7: which results in bleeding and the formation of blood-filled spaces resolution of the fracture. AP and lateral x-rays 1 year post-operatively. Complete surgery for symptomatic cases or cases complicated by a pathologicalWe strongly fracture agree with Andreacchio et al. [5] in prescribing Discussion useful tool that when combined with the clinical presentation can provide reliable [5].guidelines We believe to appropriatelythat Arata’s criteria determine are a very the tumor occurring mainly in childhood and adolescence [1] It is well recognized that non-ossifying fibroma is a benign been implemented in our patient who accordingly constituted necessity of surgical intervention. These guidelines have, therefore, . Although exists on the need for surgical treatment in pathological fractures. Mostuncommon, literature we stateagree that with there Glockenberg is a higher et al.male who predominance described in an excellent surgical candidate. Some controversy, however, still their paper that the age range for this condition is 3 to 42 [1]. because the primary lesion would persist and not be obliterated most commonly affects the metaphyseal region of bone [1] Drennan et al. [9] recommended curettage and bone grafting to females; and this was estimated to be 2:1 [1,2]. The condition as the fracture heals the distal femur followed by the proximal tibia , namely lesion regression occurs as the fracture heals [9]. Gitelis et al. on the other hand, stated that Ritschl et al. [3] carried out extensive studies[1,3]. to determine [10]. The authors, however, believe that curettage, bone grafting and fracture the insertionsite of evolution sites of oftendons non-ossifying or ligaments fibroma in the[3]. metaphysis Accordingly, [3]. it fixation is inevitable. The choice of bone grafting is various with was concluded that non-ossifying fibroma probably arises from calcium sulphate [5], autogenous bone marrow grafts [11] and, in Conflictour case, cancellous of Interest bone chips being used. Ritschl et al. [3] have also developed a radiological classification how the lesion transforms from its primitive lobulated and The authors declare that they have no competing interests. system for non-ossifying fibromawhich adequately demonstrates lytic pattern to its well healed sclerotic form. According to this

Citation:

Alsaleem MK, Alqasim E, Aleid ZM, Alhathloul HS, Alharbi NM et al. (2018) A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Bone Cysts Presenting with a Pathological Fracture. MOJ Orthop Rheumatol 10(1): 00387. DOI: 10.15406/mojor.2018.10.00387 A Unique Adult-Type Distal Tibia Non-Ossifying Fibroma with Secondary Aneurysmal Copyright: 4/4 Bone Cysts Presenting with a Pathological Fracture ©2018 Alsaleem et al.

Acknowledgement 5. None. extremityAndreacchio with A, calcium Alberghina sulfate F, graftsTesta in G, skeletally Canavese immature F (2018) patients. Surgical treatment for symptomatic non-ossifying fibromas of the lower Consent 6. Eur J Orthop Surg Traumatol 28(2): 291-297. The patient presented in this case report has given his/her full informed consent for the case report to be published. Arata MA, Peterson HA, Dahlin DC (1981) Pathological fractures through non-ossifying fibromas. Review of the Mayo Clinic 7. References experience. J Bone Joint Surg 63(6):980-8. 1. Hoeffel C, Panuel M, Plenat F, Mainard L, Hoeffel J-C (1999) Pathological fracture in non-ossifying fibroma with histological 66-69.Glockenberg A, Sobel E, Noel Jf (1997) Nonossifying fibroma: four features simulating aneurysmal bone cyst. Eur Radiol 9(4): 669-671. cases and review of the literature. J Am Podiatr Med Assoc 87(2): 8. Mizukawa N, Nishijima Y, Nishijima K (1997) Metaphyseal fibrous - determination of their origin and natural history using a defect (nonossifying fibroma) in the mandible. Int J Oral Maxillofac 9. 2. Ritschl P, Karnel F, Hajek P (1988) Fibrous metaphyseal defects Surg 26(2): 129-130.

3. Drennan DB, Maylahn DJ, Fahey JJ (1974) Fractures Through Large radiomorphological study. Skeletal Radiol 17(1): 8-15. 10. Non-Ossifying Fibromas. Clin Orthop Relat Res 103: 82-88.

Ritschl P, Lintner F, Pechmann U, Brand G (1990) Fibrous metaphyseal Gitelis S, Wilkins R, Conrad EU (1995) Benign Bone Tumors. J Bone defect. Int Orthop 14(2). 11. Joint Surg 77(11): 1756-1782. 4. Herget GW, Mauer D, Krauß T, Tayeh AE, Uhl M, et al. (2016) Non- Hase T, Miki T (2000) Autogenous bone marrow graft to non- ossifying fibroma: natural history with an emphasis on a stage- ossifying fibroma with a pathologic fracture. Arch Orthop Trauma related growth, fracture risk and the need for follow-up. BMC Surg 120(7-8): 458-459. Musculoskelet Disord 17(1).

Citation: