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Year : 2020  |  Volume : 63  |  Issue : 4  |  Page : 656-657
Chondromyxoid fibroma of the nasal cavity - A rare tumor at an unusual site

1 Department of Pathology, Seth G.S. Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India
2 Department of ENT, Seth G.S. Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India

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Date of Submission05-Nov-2019
Date of Decision23-Dec-2019
Date of Acceptance27-Dec-2019
Date of Web Publication28-Oct-2020

How to cite this article:
Sathe P, Agnihotri M, Joshi A, Marfatia H. Chondromyxoid fibroma of the nasal cavity - A rare tumor at an unusual site. Indian J Pathol Microbiol 2020;63:656-7

How to cite this URL:
Sathe P, Agnihotri M, Joshi A, Marfatia H. Chondromyxoid fibroma of the nasal cavity - A rare tumor at an unusual site. Indian J Pathol Microbiol [serial online] 2020 [cited 2022 May 18];63:656-7. Available from: https://www.ijpmonline.org/text.asp?2020/63/4/656/299333

Chondromyxoid fibroma (CMF) is a rare benign tumor, accounting for less than 0.5% of all bone tumors.[1] It has a predilection for bones of lower extremity and is typically metaphyseal in location.[2] It rarely affects the craniofacial bones.[2] Although CMF has a distinct histomorphology, the ones arising in craniofacial bones show unusual features, thus posing a diagnostic difficulty.

A 16-year-old female, presented with bilateral nasal obstruction and mass in the right nasal cavity since seven months. She also complained of anosmia, nasal deformity, and change in voice. Local examination showed an external nasal deformity in the form of widened nasal bridge and hypertelorisn. A pinkish mass was seen obliterating the right nasal cavity. Magnetic resonance imaging showed a well-defined lobulated mass measuring 8.7 × 3.6 × 4.8 cm in the right nasal cavity causing compression of inferior and middle turbinate without bony destruction [Figure 1]a. The lesion was extending into the nasopharynx posteriorly and the sphenoid sinus posterior-superiorly. Radiologically, the possibilities of rhinoscleroma or fibrous tumor of the nasal septum were considered.
Figure 1: (a) MRI showed a well-defined lobulated mass completely occupying the right nasal cavity. (b) Grossly, multiple grey-white firm irregular bits aggregating to 13 × 10 × 2 cm were received

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At surgery, the tumor was found to completely occupy the nasal cavity causing the expansion of the vestibule and the right lateral nasal wall. The nasal septum was deviated to the left. The tumor was removed by piecemeal method.

Grossly, multiple grey-white firm irregular bits aggregating to 13 × 10 × 2 cm were received [Figure 1]b. Histology showed a characteristic lobulated lesion showing paucicellular centers and peripheral cellularity. It was composed of spindle and spiculated star-shaped cells with myxoid and chondroid intercellular material [Figure 2]a and [Figure 2]b. The interlobular area showed fibrovascular bundles and few scattered multinucleated giant cells [Figure 2]c. Many nodular deposits of calcified and osseous material were seen in the center of the lobules [Figure 2]d. No hyaline cartilage was identified. There was no atypia, mitosis, or necrosis. The differentials included the spectrum of lesions with chondromyxoid matrix occurring in nasal cavity, i.e., benign fibroosseous lesion, low-grade chondrosarcoma, chondroblastoma, chondroid chordoma, and chondromesenchymal hamartoma. Among the benign fibroosseous lesions, the possibility of cement-ossifying fibroma was considered as there were spindle cells with myxoid matrix and acellular basophilic mineralized material resembling cementum but absence of lobulation ruled it out. Chondrosarcomas show lobulation with spindling of nuclei but the entire lobule should be hypercellular although in the present case there was zonation showing hypocellular centers and peripheral cellularity. Also, there was no atypia and the patient was young. The absence of chicken wire calcification and physaliphorous cells ruled out chondroblastoma and chondroid chordoma, respectively. Chondromesenchymal hamartoma occurs in patients less than three years of age and usually shows hyaline cartilage. Thus, the morphology of lesion was not compatible with any of the diagnoses enumerated above. Reviewing the case, the histomorphology of the lesion showed the characteristics for CMF but due to rarity in the sinonasal location and the presence of unusual features like increased calcification and less number of giant cells, the diagnosis of CMF was not thought of before. The literature search showed that craniofacial CMFs are extremely rare and are known to show such variations in morphology. Finally, the tumor was signed out as CMF.
Figure 2: (a) Histology showed a lobulated lesion characteristically showing paucicellular centers and peripheral cellularity (H and E ×40). (b) Lobule was composed of spindle and spiculated star-shaped cells with chondromyxoid background material (H and E ×400). (c) Interlobular area showed fibrovascular bundles and very few multinucleated giant cells (H and E ×600). (d) Many nodular deposits of calcified and osseous material were seen in the center of the lobules (H and E ×400)

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CMF is a rare cartilaginous benign tumor, composed of chondroid, fibrous, and myxoid tissues in varying proportions.[3] Approximately, 5.4% of all CMFs occur in the skull and facial bones, predominantly present in the mandible and maxilla.[4] The nasal bones and paranasal sinuses are extremely rare locations, with only 21 cases reported in literature.[5] Although the peak of incidence is in the second and third decades with a male prevalence, when it comes to cranium and face bones, women prevail.[5]

The distinct histological features of CMF include a lobular pattern with stellate or spindle-shaped cells in a chondromyxoid background, presence of osteoclastic type giant cells, and infrequent calcification.[6] Lobule characteristically demonstrates paucicellular centers and peripheral hypercellularity.[6] Literature also describes few unusual features like microlobulation, extensive calcification, reactive bone formation, and permeation of bone and sparse multinucleated giant cells, usually seen with craniofacial CMF.[7] The reported case also showed these features with extensive calcification and sparse giant cells. Thus, relative rarity and variation in the characteristic morphology pose diagnostic difficulties. Thus, CMF may not occupy the primary place in the differential diagnosis of chondroid rich skull lesions, in spite of having the characteristic lobular pattern as happened in our case. The lesions, which are common at this location like chondrosarcoma, benign fibroosseous, or chordoma are usually considered.[7]

The choice of treatment is excision. It has a recurrence in 25% of the cases, generally due to the incomplete removal of the lesion.[8]

The case is presented for the rarity of occurrence of CMF in craniofacial bones and also to highlight the unusual histological features at this location which will help in the diagnosis of future cases.

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Nazeer T, Ro JY, Varma DG, Hermosa JRDL, Ayala AG. Chondromyxoid fibroma of paranasal sinuses: Report of two cases presenting with nasal obstruction. Skeletal Radiol 1996;25:779-82.  Back to cited text no. 1
Sass SMG, Pinto MC, Maeda CAS, Campos DS, Maranhão ASA. Chondromyxoid fibroma of the nasal bone. Intl Arch Otorhinolaryngol 2009;13:117-20.  Back to cited text no. 2
Armah HB, McGough RL, Goodman MA, Gollin SM, Surti U, Parwani AV, et al. Chondromyxoid fibroma of rib with a novel chromosomal translocation: A report of four additional cases at unusual sites. Diagn Pathol 2007;2:44.  Back to cited text no. 3
Isenberg SF. Endoscopic removal of chondromyxoid fibroma of the ethmoid sinus. Am J Otolaryngol 1995;16:205-8.  Back to cited text no. 4
Wang J, Zhu J, HuangM, Lu A. Chondromyxoid fibroma of the inferior turbinate: A case report. Otolaryngology Case Reports 2019;11:100118.  Back to cited text no. 5
Azorín D, Gil A, Sánches-aniceto G, Ballestín C, Martínez-Tello J. Chondromyxoid fibroma of the frontal sinus. Br J Oral Maxillofac Surg 2003;41:418-20.  Back to cited text no. 6
Wu CT, Inwards CY, O'Laughlin S, Rock MG, Beabout JM, Unni KK. Chondromyxoid fibroma of bone: A clinicopathologic review of 278 cases. Hum Pathol 1998;29:438-46.  Back to cited text no. 7
Shek TWH, Peh WCG, Leung G. Chondromyxoid fibroma of skull base: A tumor prone to local recurrence. J Laryngol Otol 1999;113:380-5.  Back to cited text no. 8

Correspondence Address:
Mona Agnihotri
Department of Pathology, Seth G.S. Medical College and K.E.M. Hospital, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_865_19

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