CASO CLÍNICO

 

Radiological follow-up of a patient with chondromyxoid fibroma of the frontal sinus. Differential diagnosis, therapeutic alternatives and a revision of the literature

Seguimiento radiológico en paciente con fibroma condromixoide del seno frontal. Diagnóstico diferencial, alternativas terapéuticas y revisión de la literatura

 

 

I. Zubillaga Rodriguez¹, S. Heredero Jung², M. Castrillo Tambay², G. Sánchez Aniceto¹ , J.J. Montalvo³

1 Médico Adjunto
2 Médico Residente
3 Jefe de Servicio
Servicio de Cirugía Oral y Maxilofacial.
Hospital Universitario 12 de Octubre, Madrid. España

Dirección para correspondencia

 

 

 

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ABSTRACT

Introduction. Chondromyxoid fibroma is a slow growing and rare benign bone tumor. Its location in the craniofacial skeleton is not common, especially in the frontal sinus.
Objectives. We present a long-term follow-up of a patient with a frontal chondromyxoid fibroma who was operated four years ago. We discuss the various differential diagnoses and possible treatments.
Discussion. The differential diagnosis includes chondrosarcoma, myxosarcoma, myxoma and chordoma. From the radiological point of view, chondromyxoid fibroma must be always considered as a diagnostic possibility when a solitary bone lesion appears with expansion or cortical osteolysis, lobulated margins and intralesional septi. Treatment includes curettage or monoblock resection with immediate bone graft reconstruction. Radiation therapy alone must not be considered as the first therapeutic possibility.
Conclusions. The benign nature of the tumor does not justify radical, unnecessary treatment. The subcranial approach is appropriate for lesions located in the frontal sinus. A clinical and radiological follow-up is needed to identify tumor relapse.

Key words: Chondromyxoid fibroma; Frontal sinus; Subcranial approach.

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RESUMEN

Introducción. El fibroma condromixoide es un tumor benigno de crecimiento lento y poco frecuente. Su incidencia en la región craneofacial es rara, especialmente en el seno frontal.
Objetivos. Presentamos el seguimiento a largo plazo de un paciente intervenido en relación a un fibroma condromixoide del seno frontal. Se discuten los distintos diagnósticos diferenciales y las alternativas terapéuticas.
Discusión. Desde el punto de vista de anatomía patológica es necesario hacer el diagnóstico diferencial con condrosarcoma, mixosarcoma, mixoma y cordoma. Desde el punto de vista radiológico, el diagnóstico de fibroma condromixoide ha de ser considerado en toda lesión ósea aislada con expansión u osteolisis, márgenes lobulados y septos intralesionales. El tratamiento del fibroma condromixoide incluye principalmente curetaje o resección en bloque, mientras que la radioterapia aislada no se considera de primera elección.
Conclusiones. La naturaleza benigna del fibroma condromixoide implica la importancia de evitar tratamientos radicales. El abordaje subcraneal se presenta como una alternativa válida para el tratamiento de lesiones localizadas en el seno frontal. Es fundamental un seguimiento clínico-radiológico adecuado para identificar precozmente posibles recidivas.

Palabras clave: Fibroma condromixoide; Seno frontal; Abordaje subcraneal.

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Introduction

Chondromyxoid fibroma is a slow-growing benign tumor first described by Jaffe and Lichtenstein¹ in 1948. It is a relatively rare tumor, with an incidence rate of less than 1% of all bone tumors.² It is generally located in the proximal metaphyses of the long bones, principally the tibia and femur.³

Approximately 2% of all the cases described are located in the craniofacial skeleton, mainly in the mandible,³ and they could originate from Meckel’s cartilage remains. Chondromyxoid fibroma has a certain predilection for secondary ossification centers, or near these.

These tumors commonly arise during the second and third decade in life, although cases have been described in patients at 20 days of life4 until the age of 79.⁵ In general there is no sex predilection although, of the tumors located in the mandible, there is a 2:1 female predominance.¹

The object of this paper is to present a long-term followup of a patient with chondromyxoid fibroma of the frontal sinus, published previously as a case report from an anatomopathological point of view.⁶ In addition, a review of the literature and of the differential diagnoses and therapeutic alternatives to be taken into account is carried out.

 

Case report

The patient was a 46-year-old male that was sent to the Oral and Maxillofacial Department of the Hospital Universitario 12 de Octubre (Madrid) as a result of a frontal sinus tumor-like mass above the right eyelash, which was slowgrowing and that had been developing for approximately 18 months (Fig. 1). There was no history of local trauma nor was his medical history of interest. During the physical examination a soft lesion was noted on palpation, which appeared to be a cystic neoformation with a diameter of 3 cm. It was non-tender and there were no local signs of infection. Hypoesthesia was not appreciated in the area of the supraorbital or supratrochlear nerves.

The conventional plain radiography showed a rounded and radiolucid lesion in the right frontal sinus with a diameter of approximately 2.5-3 cm. A CAT scan was later requested of the craniofacial skeleton, and again a radiolucid lesion that was well-defined could be appreciated. It was occupying the upper half of the right frontal sinus. It had a cystic appearance and it had led to osteolysis of the anterior as well as posterior wall of the frontal sinus (Fig. 2). The initial diagnostic impression was of mucocele with aggressive local behavior and involvement of the frontal dura mater.

The surgical intervention was carried out under general anesthesia. The lesion was approached by means of a bicoronal incision. The mass appeared to be solid and with no capsule (Fig. 3). Biopsies were taken intraoperatively of the lesion margins, which was categorized as a locally aggressive mesenchymal tumor. Exeresis was carried out of the tumor lesion by means of a subcranial approach that included part of the surrounding frontal bone and the pericranium with preservation of the frontal dura mater (after bipolar coagulation) (Fig. 4). Later a galeopericranial flap was used for sealing the frontal sinus and for isolating the cranium defect (Fig. 5). A monocortical bone graft of parietal calvarium was used for the bone defect of the anterior wall of the frontal sinus. The bone graft as well as the osteotomy carried out for the subcranial approach, were fixed with lowprofile titanium plates (1.0 mm) (Fig. 6).

The macroscopic examination described the tumorlike mass as a purplish and nodular lesion measuring 2.5 x 2.5 x 1 cm. When sectioned it appeared fibrous and it had a cystic formation in its interior. It had a diameter of 0.4 cm. Microscopically the lesion had a polylobulated morphology. The lobules were separated one from the other by connective tissue that was richly vascularized, but the interior of the lobules was not vascularized. In addition, the lobules were irregular in shape and they were made up of myxoid stroma that was positive for colloidal iron. In the center of the stroma, polygonal cells could be observed in round sheets as well as fusiform and stellate cells. These cells were more abundant around the edges than in the centers of the lobules. Mitoses were not observed, although there were small calcifications and necrotic foci.

Electron microscopy showed multipolar stellate cells in the center of fibromyxoid stroma with cotton woollike material surrounding the cell membranes. The cell membranes did not have surface structure and in the cytoplasm there was no evidence of any of the organelles increasing. The diagnosis was of mesenchymal cell proliferation with chondral differentiation.

The immunohistochemical study finally gave the diagnosis of neoformation with chondral phenotype, which coincided with the histopathological diagnosis of chondromyxoid fibroma.

After the surgical intervention and a postoperative period that was complication-free, the patient was discharged and monitored periodically. A follow- up CAT scan was carried out after a year (Fig. 7) and there were no radiological changes suggestive of recurrence. Two years after the intervention, the patient was clinically asymptomatic (Fig. 8), with CAT scans showing no sign of recurrence (Fig. 9). In the last check-up by CAT scan (Fig. 10) carried out four years after the surgical treatment, lesions were not observed radiologically that were suggestive of recurrence. In addition, a graft of calvarium fixed with titanium can be observed, and no intolerance to this material has arisen to date. The patient continues being asymptomatic.

 

Discussion

Revision of the literature

With regard to bone tumor classification, it was Lichtenstein⁷ who first described in 1951 the chondromyxoid fibroma as a pathologic entity belonging to the group of benign tumor arising from the cartilage. Later Jaffe⁸ in 1958 recorded more than 30 cases with this diagnosis.

Most of the series published include cases involving the lower extremities, as the most common location of the chondromyxoid fibroma is the metaphyseal area of the long bones of the lower extremities, principally the tibia and the femur. Feldman and col⁵ (1970) recorded 207 cases; Salzer and Salzer- Kuntschik9 (1965) 117 cases; Frank and Rockwood¹⁰ (1969) 91 cases, and Frank and Rockwood¹⁰ (1972) reviewed 76 cases. Other series with lower patient numbers are the 41 cases collected by the Bone Tumor Committee of the Japanese Orthopaedic Association National Cancer Center,¹² and the 32 cases collected by Schajowicz and Gallardo¹³ (1971).

The first cases of chondromyxoid fibroma with a craniofacial location were published in 1958 by Jaffé,⁸ who recorded a case in the parietal area. The craniofacial area most commonly affected is the mandible,2 and the first case was described by Paul.¹⁴ Generally, the integrity of the periosteum avoids extraosseous involvement, but Lingen¹⁵ presented in 1993 a case of mandibular chondromyxoid fibroma with extraosseous involvement at diagnosis. Other locations within the craniofacial skeleton that have been described are: premaxila,¹⁶ skull base,^17-19 occipital region,¹¹ and pterygoplatine space.²⁰

With regard to frontal bone involvement, it is very uncommon for the chondromyxoid fibroma to be located here. The cases described in the literature are set out in table 1.

Differential diagnosis

A careful correlation is necessary of the clinical, radiological and histological findings in order to reach a correct diagnosis.

According to the World Health Organization,²⁹ chondromyxoid fibroma is a benign tumor characterized by the presence of areas of spindle-shaped or stellate cells with abundant myxoid or chondroid intercellular material, separated by zones rich in rounded cells with a varying number of multinucleated giant cells of different sizes.

The presence of large pleomorphic cells can lead to it being confused with the chondrosarcoma. In fact, one of the greatest points of interest in the management of these tumors involves the differential diagnosis with the low-grade chondrosarcoma. This entity can have prominent myxoid areas, peripheral cells with bizarre nuclei and pleomorphic cells with hyperchromatic nuclei.³⁰ The histopathological findings of both lesions are very similar, however, low-grade chondrosarcoma typically lacks well-circumscribed borders around the lesion, as well as the fibrous septa that divide the chondromyxoid fibroma into (pseudolobulated) areas. In addition, a greater amount of cellularity can be observed in the chondromyxoid fibroma together with more multinucleated giant cells around the tumor edges.

Furthermore, S-100 positivity supports the cartilaginous origins of the chondromyxoid fibroma, as the myxomatous areas are negative for this protein. However, it should be considered that a positive result for S-100 does not exclude the lesion being a chondrosarcoma, given the lack of value and low sensitivity in the differential diagnosis of these lesions.¹⁵

Preliminary studies of the chemical composition of the chondromyxoid fibroma reveal the presence of high levels of glutamic acid in the tumor matrix,² which is not observed in the chondrosarcoma.

Analysis of cellular DNA content by flow cytometry can be of help for distinguishing benign types of neoplasms from malignant ones. Benign chondroid lesions have a normal DNA content (diploic range). Approximately 60% of chondrosarcomas have an abnormal DNA content, which is not observed in benign neoplasms. Thus, while a normal content of DNA does not exclude a diagnosis of chondrosarcoma, an abnormal content of DNA (aneuploidy) in a chondroid neoplasm identifies a chondrosarcoma.³¹

When the matrix is less chondroid and predominantly myxoid, the differential diagnosis with myxosarcoma should be established. The diagnosis of myxoma can be excluded because of the absence of a reaction to the mucin of this tumor and in addition, the condensation of tumor cells in the periphery of the lesion goes against a diagnosis of myxoma.

A differential diagnosis has to be established including chordoma, a tumor lesion that like the chondrosarcoma is more aggressive and capable of producing distant metastasis. In order to differentiate the chordoma from the chondromyxoid fibroma it is important to take into consideration that the former has infiltrative margins. It has a composition of wide epithelial cells, with vacuolated or eosinophilic cytoplasm that form cellular nests or cords, and there is an absence of the fibrous component found in the chondromyxoid fibroma.

From the radiological point of view, the findings are specific, and the differential diagnosis should include the following lesion: giant cell granuloma, bone cysts, enchondromas, benign chondroblastoma, benign fibrous lesions, aneurismatic bone cyst, monostotic fibrous dysplasia, hyperparathyroidism, myxoma and neurofibromatosis.³²

Therapy

Choosing the therapeutic approach most suitable for treating the chondromyxoid fibroma continues being a source of controversy. Basically, two treatment modalities have been used: curettage or en bloc resection, followed by immediate reconstruction with bone grafts. Radiotherapy on its own is not considered treatment of first choice.

The benign nature of the tumors and the possibility of treating them successfully should there be reoccurrence, as well as the aesthetic and functional deformity that can occur after en bloc exeresis of the lesion, are the principal points supporting curettage as first-choice treatment. Thus, curettage, with a close follow-up in order to treat any recurrence promptly, has been established as an excellent alternative by a large proportion of authors. The indication appears clear for those lesions that affect the jaws given that en bloc resection can be too radical if we take into consideration the potential aesthetic and functional residual deformity.

On the other hand, the considerable rate of recurrence after curettage (13-22%)³⁰ points to conservative en bloc resection being the first therapeutic possibility followed by immediate reconstruction with bone grafts. In this sense, it is important to point out that tumor recurrence is generally due to the incomplete resection of the lobular extensions of the tumor. Moreover, the rate of recurrence increases if the following factors are present: young patients, multinodular growth pattern and a mucosal content within the tumor. Thus, some authors also recommend en bloc resection as opposed to curettage, given the possibility of malignant transformation to chondrosarcoma that the chondromyxoid fibroma has, even though this is a very small possibility.

Traditionally, radiotherapy has been considered of dubious value for treating a chondromyxoid fibroma, as it is linked to a disposition for the malignant degeneration of the lesion. However, recent studies²⁸ suggest the usefulness of radiotherapy as coadjuvant treatment for lesions with a craniofacial location that cannot be resected completely, and these claim that there have only been two cases in the literature of malignant degeneration after radiotherapy, as opposed to one case of spontaneous malignant degeneration.

Radiological follow-up

In this article a case is presented of frontal sinus chondromyxoid fibroma with the longest follow-up in the literature. After the treatment, and the complete resection of the lesion, the patient continues being asymptomatic and there is no radiological evidence of disease recurrence. In addition, during the follow-up no complications in connection with the surgery were observed.

An adequate clinico-radiological follow-up is essential for identifying recurrence promptly. Given the low incidence rate of these lesions in the craniofacial region, setting out a management protocol is not necessary. However, most cases published in the literature show that recurrence occurs during the first postoperative year.²⁸ Therefore, making periodic revisions with a yearly CAT scan revision is necessary especially during the first years after the surgery.

 

Conclusions

The chondromyxoid fibroma should always be considered as a diagnostic possibility when a solitary bone lesion is evaluated with geographic destruction, lobulated margins, intralesional septa, and expansion or cortical osteolysis and/or sclerotic borders, especially in patients that are between the second and third decades in life.

Generally, if the periosteum is intact there will be no extraosseous involvement.

The benign nature of the chondromyxoid fibroma implies the importance of avoiding unnecessary radical treatment. Whenever possible, and if not too much morbidity is caused, it should be removed with sufficient margins to avoid tumor recurrence. Moreover, reconstruction with bone grafts, if necessary, should be carried out immediately after the resection.

The subcranial approach is a valid alternative for the treatment of lesions located in the frontal sinus.

A careful correlation of the clinical, radiological and histological finding should be established in order to obtain an adequate and definitive diagnosis, and treatment that will avoid any recurrence of the tumor.

Periodic radiological follow-up is essential for diagnosing promptly any possible recurrence.

 

 

Dirección para correspondencia:
Dr. I. Zubillaga Rodríguez
Servicio de Cirugía Oral y Maxilofacial
Hospital Universitario 12 de Octubre
Avda. de Andalucía km 5.400
28041 Madrid, España
Email: ignaciozubillaga@yahoo.es

Recibido: 19.10.05
Aceptado: 06.10.06

 

 

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