The chondromyxoid fibroma (CMF), first identified in 1948 by Jaffe and Lichtenstein, is a cartilaginous origin tumor that makes up less than 0.5 percent of all bone cancers [1-2] and is somewhat more common in men [3]. Between 1 and 5% of CMF instances are documented to occur in the head and neck, despite the condition being more frequently detected in the metaphysis of long bones [3-4].

In a review of 278 cases, 15 were in the skull or facial bones [7], and 1 of 189 cases in a study of 76 cases of chondromyxoid fibroma, which included additional cases from the literature, were in the skull [6]. A chondroid, fibrous, and myxoid material intermingled in lobulated patches is a chondroid fibroma [5]. This benign tumor can be seen in several anatomic regions, such as the long bones, flat bones, and cranio-facial bones [5]. It is particularly common in young individuals. We report the case of a 47-year-old female patient who had chondromyxoid fibroma in the sphenoid sinus.

A 47-year-old woman, with no particular pathological antecedents, presented to the otorhinolaryngology - head and neck surgery department with chronic bilateral nasal obstruction for 1 year with anosmia and no other associated rhinological signs, in particular no epistaxis and no facial pain. Physical examination, including nasal endoscopy, revealed a large mass in the right and left nasal cavity pushing the turbinates towards the septum. There were no associated symptoms such as visual complaints, paresthesia, facial pain or cephalea. A biopsy was taken in the operating theatre, and histopathological evaluation revealed a lobular growth pattern with stellate or spindle cells on a chondroid background (Figure 1). There was no well-developed hyaline cartilage. The pathology report indicated that the morphological features were consistent with CMF.

Prior to definitive resection, preoperative computed tomography (CT) and magnetic resonance imaging (MRI) imaging were completed for anatomic mapping and surgical planning the CT scan revealed the presence of an osteolytic tissue lesion process centered on the body of the isodense sphenoid, the seat of popcorn calcification measuring approximately 48*37 mm and extending over 49 mm, which invades the nasal septum, turbinates and posterior ethmoidal cells at the front, laterally, it invades the cavernous sinus and comes into contact with the intracavernous portion of the internal carotid arteries; posteriorly, it invades and lyses the left carotid duct and the clivus; superiorly, it is responsible for lysis of the sella turcica; and batternally, it invades the soft palate.

Figure 1: 47-year-old woman with chondrdomyxoid fibroma involving the sphenoid sinus. Axial (A), coronal (B) and sagittal (C) CT images demonstrate a locally advanced sphenoid tumor process with posterior invasion of the left carotid duct and clivus.

MRI with and without contrast supported the CT findings and demonstrated cystic components (Figure 2B) with relative T2 hypointensity concerning for high cellularity or a high nuclear-to-cytoplasmic ratio.

Figure 2: MR imaging from (a) coronal Plane, (b) Axial plane and (c) sagittal plane, demonstrating locally advanced sphenoid sinus tumor process, with scattered calcifications, with heterogeneous uptake.

Because the patient in our case did not exhibit any symptoms and because total resection was expected to be complicated, and according to the wishes of the patient who has refused surgery to curettage the tumor mass, follow-up was decided upon instead of excision. This case was discussed at the multidisciplinary consultation meeting.

Chondromyxoid fibroma (CMF) is a rare benign primary tumor accounting for 1.6% of the benign tumors in the series that Unni and Inwards (5) investigated. A lytic lesion that is round or oval in shape and less than 5 cm in diameter, chondromyxoid fibromas are typically found in the metaphysis of long tubular bones, however they can also occasionally affect the nasal bone, mandible, base of the skull, or frontal bone. Early adulthood is when chondromyxoid fibroma typically manifests [5].

Due to its uncommon incidence rate, CMF is a difficult disease to directly examine, and the current study's related drawback is the presentation of only one case. A review of the literature finds several studies that describe involvement of large portions of the skeleton. Cases have been found in the sella turcica, clivus, mastoid process, and paranasal sinuses of the head and neck [9]. Table 1 contains additional information about all reported instances of paranasal CMF, including patient demographics, location, therapy, and follow-up.

Table 1 indicates that clinical sequelae arise secondary to the mass effect rather than specific to the acute pathology of CMF. The literature review indicates inconsistent presenting symptoms. The lack of specificity of symptoms highlights the significance of a thorough evaluation and workup in obtaining an accurate diagnosis.

Table 1: location-based outcome statistics for all documented instances of CMF in the paranasal sinuses. Abbreviations: NA, not available or not covered in the article; DF, disease-free; M, male; F, female; y, year; mo, month; d, day; CMF: Chondromyxoid fibroma.

On radiography, CT, and MRI, chondromyxoid fibroma resembles these other cartilage tumors. Everybody shows a diverse rise in signal on T2-weighted imaging and a decrease in signal on T1-weighted images. They frequently show calcification of the chondroid matrix on radiography and CT [10,11]. T2-weighted scans show heterogeneity because the tumor's chondroid, myxoid, and fibrous components differ. All of these lesions will often brighten when exposed to gadolinium [10,11]. The margins of chondromyxoid fibromas are typically well-defined [5].

Both chordomas and chondrosarcomas frequently show obvious bone loss [10,11]. Chordomas are generally regarded to arise in the midline, while chondrosarcomas are thought to arise off the midline, along the petro-occipital fissure [11]. However, this orthodoxy was not supported by a recent study that examined 38 chordomas and 4 low-grade chondrosarcomas that were located in the base of the skull [10].

Because the imaging results from different lesions overlap, a thorough histopathologic assessment is essential to determining the correct diagnosis. Zilmer and Dorfman noted a 22% first misdiagnosis rate in their cohort of 36 CMF patients and the possibility of more harsh therapy being administered than is necessary, such as amputating the affected limb for a benign lesion [12-13]. According to the World Health Organization, CMF is "a benign tumor characterized by zones of more cellular tissue rich in spindle-shaped or round cells with a varying number of multinucleated giant cells of different sizes" and "lobules of spindle-shaped or stellate cells with abundant myxoid or chondroid intercellular material" [14]. The central nuclei of stellate or spindle-shaped cells have eosinophilic cytoplasm and black chromatin that is either homogenous or coarsely distributed. Atypia and nuclear pleomorphism are less prevalent in CMF and more indicative of chondrosarcoma [15-17]. Moreover, chondrosarcoma lacks the myxochondroid tissue's crossing bands. In addition to a "bubbly appearance to the stroma along with degenerative and liquefactive changes," Castle et al. list this as one of the diagnostic features of chondrosarcomas [16]. On the other hand, chordomas have the almost pathognomonic physaliferous cells (those with copious, bubbly, or vacuolated eosinophilic cytoplasm) associated with the lesion [18].

Excision is a common treatment for chondromyxoide fibromatosis [19]. However, the recurrence following excision is common since tumors may not fully disappear and may resurface [20-22]. This reaction is often limited, and a harmful conversion is unlikely [19]. However, radiation therapy is typically avoided due to cases of malignant transformation [23].

While surgical excision is the usual course of treatment for craniofacial lesions (CMF), some authors propose curettage specifically for facial CMF with strict monitoring due to the functional and esthetic repercussions of extensive resection. By citing the high recurrence rate following curettage, Baujat claimed that broad surgical resection is the most effective method for achieving long-term control [24]. Certain situations, like the one mentioned above, might be candidates for endoscopic resection.

The possibility of malignant transformation of remaining tissue is another factor in favor of surgery. Despite a low transformation incidence [25], instances of malignant transformation have been documented, and radiation therapy has been associated with an increased risk of transformation [6,28,26]. However, getting total excision is more challenging in a position at or near the base of the skull [10]. In an effort to lessen recurrence, several writers have proposed postoperative irradiation [27].

A review of the literature shows that CMF has been recorded to arise in the sinonasal cavity, despite being an extremely rare tumor that accounts for less than 0.5 percent of all bone tumors.  Histopathologic distinction is necessary for appropriate treatment. Stellate cells are abundantly found within a myxoid or chondroid matrix, as revealed by a biopsy. Inaccessible tumors may require curettage, but total surgical resection is the ideal treatment to reduce the chance of recurrence.

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