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Chondroblastoma and Chondromyxoid Fibroma

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Chondroblastoma and Chondromyxoid Fibroma See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/236096923 Chondroblastoma and Chondromyxoid Fibroma Article in The Journal of the American Academy of Orthopaedic Surgeons · April 2013 DOI: 10.5435/JAAOS-21-04-225 · Source: PubMed CITATIONS READS 8 90 4 authors, including: Camila Bedeschi Rego De Mattos Chanika Angsanuntsukh Hospital Estadual da Criança Ramathibodi Hospital 5 PUBLICATIONS 23 CITATIONS 8 PUBLICATIONS 58 CITATIONS SEE PROFILE SEE PROFILE Alexandre Arkader Children's Hospital Los Angeles 57 PUBLICATIONS 474 CITATIONS SEE PROFILE All content following this page was uploaded by Camila Bedeschi Rego De Mattos on 10 July 2015. The user has requested enhancement of the downloaded file. All in-text references underlined in blue are added to the original document and are linked to publications on ResearchGate, letting you access and read them immediately. Review Article Chondroblastoma and Chondromyxoid Fibroma Abstract Camila B. R. De Mattos, MD Chondroblastoma and chondromyxoid fibroma are benign but Chanika Angsanuntsukh, MD locally aggressive bone tumors. Chondroblastoma, a destructive lesion with a thin radiodense border, is usually seen in the Alexandre Arkader, MD epiphysis of long bones. Chondromyxoid fibroma presents as a John P. Dormans, MD bigger, lucent, loculated lesion with a sharp sclerotic margin in the metaphysis of long bones. Although uncommon, these tumors can be challenging to manage. They share similarities in pathology that could be related to their histogenic similarity. Very rarely, From the Department of chondroblastoma may lead to lung metastases; however, the Orthopaedic Surgery, The Children’s Hospital of Philadelphia, mechanism is not well understood. Philadelphia, PA (Dr. De Mattos and Dr. Dormans), the Department of Orthopaedic Surgery, Ramathibodi hondroblastoma is a rare, be- Hospital, Mahidol University, Epidemiology Bangkok, Thailand Cnign bone tumor, usually lo- (Dr. Angsanuntsukh), Children’s cated in the epiphysis or apophysis Hospital Los Angeles, Los Angeles, Chondroblastoma represents 1% to of long bones. It was first described CA (Dr. Arkader), and the University 2% of all primary bone tumors and of Pennsylvania School of Medicine, by Kolodny in 1927 as a cartilage- approximately 5% of benign bone Philadelphia (Dr. Dormans). containing giant cell tumor (GCT) tumors.4-6 The ratio of male to fe- Dr. Arkader or an immediate family but was better characterized by Cod- male patients is approximately member serves as a paid consultant man in 1931, who believed it to be 4,5,7-10 to or is an employee of Biomet 2:1. Although chondroblastoma an “epiphyseal chondromatous giant Trauma. Dr. Dormans or an has been reported in patients ranging cell tumor” involving the proximal immediate family member serves as in age from 2 to 73 years, most pa- a board member, owner, officer, or 1 humerus. In 1942, Jaffe and Lich- tients are aged <20 years.7-9,11,12 committee member of the Pediatric tenstein,2 after a comprehensive re- Orthopaedic Society of North The bone most affected by chon- view, included tumors in locations America, the Scoliosis Research droblastoma is the femur, followed Society, the International Society of other than the proximal humerus by the humerus and tibia.7,9-12 Re- Orthopaedic Surgery and and designated the tumor as benign Traumatology (SICOT) Foundation, ports in the literature fluctuate be- chondroblastoma of bone, that is, as SICOT USA, and the World tween identifying the proximal hu- Orthopaedic Concern. Neither of the a different, separate entity from merus and proximal femur as the following authors or any immediate GCT. Historically, because of Cod- most affected site.7-9,11,12 In the foot, family member has received man’s great contribution, chondro- anything of value from or has stock chondroblastoma is located espe- or stock options held in a blastoma of the proximal humerus cially in the talus and calcaneus, in commercial company or institution was referred as “Codman tumor.” an apophysis or near the articular related directly or indirectly to the 13 subject of this article: Dr. De Mattos Chondromyxoid fibroma (CMF), a surface. Chondroblastoma can also and Dr. Angsanuntsukh. rare mixture of benign cartilage and fi- occur in flat bones, such as the scap- brous and myxoid tissue that generally J Am Acad Orthop Surg 2013;21: ula, patella, sternum, and skull 5 225-233 develops in long bones of the lower ex- bones. The average age of patients tremity, was described in 1948 by Jaffe with chondroblastoma in small or http://dx.doi.org/10.5435/ 3 JAAOS-21-04-225 and Lichtenstein. Prior to their de- flat bones is higher than that of pa- scription, the lesion was thought to tients with chondroblastoma of long Copyright 2013 by the American 6,13 Academy of Orthopaedic Surgeons. be a myxoma of the bone, enchon- bones. About 0.5% to 1% of droma, or chondrosarcoma. chondroblastomas present on verte- April 2013, Vol 21, No 4 225 Chondroblastoma and Chondromyxoid Fibroma Figure 1 mors, there is no single characteristic borders. The cells contain one or two abnormality or chromosomal break- round, oval, slightly indented, or ing point specific for chondroblas- even multilobulated nuclei with or toma or CMF. without nucleoli.8 Occasional cells The histogenesis of chondroblas- may have enlarged nuclei without toma and CMF is still uncertain. Ro- nuclear atypia.4,25 The presence of meo et al20 confirmed the active role mitotic figures is scarce.2,4,25 There of cartilage-signaling molecules, both are scattered multinucleated osteo- Indian Hedgehog/parathyroid hor- clast-type giant cells among the mone–related protein (IHh/PTHrP) chondroblasts.7,8 There may be foci and fibroblast growth factor, indicat- of chondroid matrix formed by the ing that chondroblastoma is a neo- chondroblasts.25 Dystrophic calcifi- Photomicrograph of chondroblastoma. Note the diffuse plasm that originates from a mesen- cation is occasionally present and and compact proliferation of chymal cell committed toward may surround individual cells, giving mononuclear cells with indented chondrogenesis via active growth the classic “chicken wire” appear- nuclei with abundant eosinophilic ance7,8 (Figure 1), although this is cytoplasm and distinct cell borders. plate signaling pathways. This con- There is presence of focal clusion supports the chondrogenic not mandatory for diagnosis. pericellular (ie, chicken wire) nature of this tumor and the close re- In 15% to 32% of cases, chondro- calcification (arrows) on the top left lationship between the physis and blastoma may be associated with corner (hematoxylin-eosin, original 21 magnification ×400). the tumor. CMF has myofibroblas- secondary aneurysmal bone cyst tic differentiation in its “fibrous” ar- (ABC).7-9,11 Although the reasons for eas driven by transforming growth this association are unclear, hypothe- brae, generally in the posterior ele- factor β-1.22 A strong expression of ses include mechanical stress, ments and/or the body.14 the Sox9 gene, which is responsible trauma, and hemorrhage.13 More ag- A prevalence of <0.5% of all bone tu- for chondrocytic differentiation as gressive chondroblastoma that can mors is reported in many series describ- well as regulation of the expression cause metastases or recurrence shows ing CMF.6,15,16 There is a slight male of cartilage-specific genes in mature no difference in histology compared predominance.17 CMF most com- chondrocytes, especially the synthe- with less aggressive chondroblas- monly arises in young patients in the sis of collagen type II, was found in toma.25 The histology is equivalent second or third decades of life.3,17,18 both chondroblastoma and CMF.23,24 to that of the primary site, and the Most of these tumors are located in This demonstrates that the expres- presence of atypical cells is rare.25 the metaphysis of long bones with sion of Sox9 in these tumors is con- Grossly, CMF appears as lobulated, variable distances from growth plate, sistent with its commitment to the well-circumscribed, and sharply demar- mainly in lower extremities.17 Rarely, early phases of cartilage differentia- cated from the adjacent bone marrow. the lesion involves the epiphysis. The tion, with chondroblastoma being a The lesion is firm and white. The cut diaphysis can be involved, especially more “immature” tumor than CMF surface shows a solid tumor mass that in large tumors. A lesion in small because of the greater presence of is yellow, grayish-white, or blue- bones, such as phalanges, may in- positive Sox9 in CMF cells.23 gray.6 Microscopic analysis of CMF volve the bone in its totality. The reveals three components: myxoma- proximal tibia is the most common tous zones, fibrous zones, and fields site, comprising 28% to 52% of all Pathology that appear chondroid. The classic lower extremity lesions in the litera- histologic features of CMF are lob- ture.17,19 This site is followed by the Grossly, a chondroblastoma is a ules of stellate or spindle-shaped cells ilium, ribs, distal femur, metatarsals, gray-white tumor with yellowish ar- in abundant myxoid background or and distal tibia.17 In contrast to eas, usually because of calcification, chondroid intercellular material. chondroblastoma, CMF is rarely en- which can be soft, rubbery, or fria- Scattered giant cells are found in ap- countered in the humerus.15,17 ble.2 Microscopically, chondroblas- proximately 50% of cases, usually at toma reveals proliferation of mono- the edge of the lobules16,17 (Figure 2). Etiology nuclear cells.10 The tumor is These lobules have a hypocellular characterized by compact areas of center and a condensation of the nu- Although cytogenetic abnormalities round, oval, or polygonal chondro- clei toward the periphery, creating a can be highly specific for some tu- blasts with well-defined cytoplasmic hypercellular periphery. The inter- 226 Journal of the American Academy of Orthopaedic Surgeons Camila B. R. De Mattos, MD, et al Figure 2 Figure 3 Photomicrograph of chondromyxoid fibroma.
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