Youn Wha Kim Sung Wan Kim YongKoo Park Chondromyxoid fibroma CMF is a relatively rare bone tumorthat was first described by Jaffe and Lichtensteinin 1948 Thistumor makes up less than 1 of all bon ID: 953661
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Chondromyxoid fibroma (CMF) is a relatively rare bone tumor that was first described by Jaffeand Lichtenstein in 1948. CMF of the sinonasal tract is very rare. A 28-year-old male present-edwith long-standing, intermittent, pulsatile pain in the right temporal area. A computed tomog- Youn Wha Kim Sung Wan Kim Yong-Koo Park Chondromyxoid fibroma (CMF) is a relatively rare bone tumorthat was first described by Jaffe and Lichtensteinin 1948. Thistumor makes up less than 1% of all bone tumors and often in-CMF of the craniofacial bones is very rare,with the mandible and maxilla being the most common sitefor CMF of the craniofacial bones. bone tumor was performed. Subsequently, a frontostomy wasalso performed, and a pus-like discharge was aspirated from thefrontal sinus. The curettage specimen was fragmented, darkbrownish, capsuleless, glassy, soft, and resembled fibro-chon-lobular configuration of the tumor. The lobules were separatedby a fibrous band composed of spindle cells (Fig. 3). The lob-ules were composed of a chondoid or myxoid matrix with calci-fication in the central area and polyhedral, round, and stellateshaped cells in the periphery (Fig. 4). The tumor cells had occa-and the nucleoli were inconspicuous. One mitotic figure wascalcification pattern was a fine, granular or dense, plaque-likeformation. Immunohistochemical staining for Sox9 was posi-tive for the nuclei of about 20% neoplastic cells. A few neo-plastic cells were positive for S-100. These findings were con-sistent with CMF. The patientÕs pain was relieved after surgery. The World Health Organization defines CMF as Òa benigntumor characterized by lobules of spindle-shaped or stellate cellswith abundant myxoid or chondroid intercellular material.ÓThese tumors have a predilection for the long bones of childrenand adults in their 20s, while involvement of the craniofacialbones is found in only 5.4% of patients.ed in many craniofacial region sites; however, the tumor is extre-mely rare in the ethmoid sinus, with only eight cases reportedin the English literature (Table 1).described in neonates and adults of both genders. There is no Cystic mass-like lesion with surrounding edema of the right Lobulated mass of the right ethmoid sinus is seen on a par- Tum
or lobules are composed of a chondoid or myxoid ma-trix with calcification in the central area and polyhedral or round or Fig. 4.Polyhedral, round, or stellate shaped cells in the peripheryof the tumor lobules. established radiological pattern for CMF due to its rarity. In allreviewing previous reports, a CMF of the ethmoid sinus com-plicated by a brain abscess has not been reported previously. A differential diagnosis of a CMF is difficult histologically aswell as clinically. It is very important to distinguish between aCMF and chondrosarcoma because the management of these twoentities differs. Histologically, a CMF is usually well-demarcat-ed, even when it erodes the bone. The lesions are nodular withmyxoid lobules separated by thin, fibrous septa. Importantly, aCMF has a paucicellular center rather than the uniform cellulararrangement observed in chondrosarcoma, and mitotic figuresWhen compared to a CMF, chondrosarcomadiffers in that the tumor encases the preexisting bony trabecu-Chondrosarcoma reveals well-differentiated hyaline carti-with individual cells show-ing nuclear pleomorphism and atypia. Additionally, the differ-ential diagnosis includes fibrous dysplasia and chordoma. Fib-rous dysplasia lacks the epithelioid cells and lobulation patternseen in a CMF.Chordoma, especially in its chondroid form,can be distinguished from a CMF by its infiltrative margin andClinically, CMFs arising in the paranasal sinuses can presentwith pain, swelling, nasal obstruction, or an asymptomatic ma-the brain abscess arose at this time. We believe that the CMFofthe ethmoid sinus may have been one of the causes of the brainabscess, as the CT scan showed that the ethmoidal bone lesionextended into the frontal sinus. Pus from the narrowed frontalsinusopening was drained by a frontostomy. Blockage of mucusflow in the paranasal sinus was a possible source of infection.From these findings, it is reasonable to suggest that the CMFSox9 is a transcription factor that was recently described as aphases of chondrocyte differentiation and is useful for distingui-shing between undifferentiated tumors in the small, blue, round-cellcategory and tumors of true cartilaginous origin.Sox9 reactivity does not differentiate CMF from a low-gradechondr
osarcoma because both are cartilaginous, it could assistin establishing a diagnosis of CMF when histological featuresare suggestive of a different cell lineage.bone and the dura mater of the ethmoid sinus has been report-Thus, complete surgical resection is considered to be theoptimal means for treating a CMF.However, a CMF of the cran-iofacial bones is best treated by curettage because of the func-tional and cosmetic deformities that result from complete sur-gical resection. Curettage is ordinarily successful but increasestherisk of recurrence.The recurrence rate from curettage is ap-AuthorsAge (yr)/SexSize (cm)Radiologic findingsClinical presentationTreatment57/F2.5 50/FNA20/MNA32/M6.0 34/F2.5 10/FNA0/MNA8/F7 Our study28/M2 Mass on the nasal bridge extending into theOsteolytic lesion in the ethmoid bone invad-Mass with bone erosion, scalloping, exten-Large mass in the ethmoidal sinus erodingSolid, well-defined mass in the ethmoidSuggestive benign tumor without destruc-Round, bony density in the right ethmoidSurgical excision withEndoscopic Slowly expanding, painlessFrontal headache, pain, nasalPainless frontal swelling andOcclusion of the nasal cavity, Table 1.Nine cases of ethmoid CMF: a clinicopathological and radiographical literature review is recommended for patients who have received curettage. Someauthors have propose using radiotherapy for local relapses fol-lowing surgical excision, particularly at the base of the skull.In conclusion, a CMF of the ethmoid sinus is very rare. Alth-ough a CMF is a benign bone tumor, serious medical conditionssuch as a brain abscess may be associated with a CMF, depend-ingon the tumor location. Patients who receive curettage of theCMF should be checked periodically for recurrence. *+ ! , - . / / 01 %0*(230*1212456 +7 8/9 + ! !9 : &8 ; ., &#x=-1;.3;!; )?: &#x=-1;.3; ;
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