INTRODUCTION

Chordomas are malignant bone neoplasms that originate from embryonic notochordal remains, and they affect the skull base and the spine (1). They are usually of slow growth, with a strong trend towards local invasion and recurrence. It is a rare type of tumor, making up for about 5% of all malignant bone tumors (2).

As to their location, 50% of these tumors are found on the sacrococcygeal region, 35% in the skull base and 15% in other sites along the spine. It affects more males than females in a 2:1 ratio. Its incidence peak falls on the 5th and 6th decades of life (3).

It has a varied biological behavior, without correlation to age, gender, hystology or tumor site, still bearing unexplained occurrence of fast growth tumors of aggressive recurrence in some patients, and slow growth in others (2). The clinical presentation varies according to tumor size and site, and the patient may not show symptoms for over one year before diagnosis (2).

Differential diagnosis of skull base tumors include: meningioma, schwannoma, pituitary adenoma, naso-pharynx and sphenoid sinus carcinomas; while those of the spine may include: chondrosarcoma, myeloma or metastasis (4).
The goal of this paper is to present a case of cervico-pharyngeal chordoma and review its diagnosis and treatment.

CASE REPORT

A 65 year old female seen at the ER complaining of moderate dyspnea, cough, sialorrhea and pharyngeal discomfort in the past 3 months. She complained of a worsening of symptoms in the previous month, with neck pain, full cranial headache episodes, exclusive mouth breathing and a 4 Kg weight loss. She also complained of feeling like she had a "pharyngeal bolus", but, she denied symptoms of odynophagia, dysphagia, or dysphonia. She had a previous history of hypothyroidism and psychiatric disorder, being under the use of Levothyroxin, Carbolythium and Haloperidol. She had a negative family history of neoplasms.

The otolaryngology exam revealed a large submucosal smooth mass occupying the postero-lateral left wall of the nasopharynx, with medial extension (Figure 1). No abnormalities were found in the indirect laryngoscopy, rhinoscopy and otoscopy. No nodules or masses were found on the neck palpation. She was admitted for exams and clinical observation.

A cervico-facial CT scan showed a left pre-vertebral and retropharyngeal mass, which destroyed C2 and extended into the C2-C3 foramen, involving the left vertebral artery (Figures 2 and 3) without lymph node involvement.
The oropharynx lesion was biopsied and investigated by fine needle puncture, having the microscopy showing broad citoplasm cells with round nuclei and myxoid edema, producing the diagnosis of chordoma (Figure 4).

The neurosugery team was consulted and they were against surgery because of the degree of involvement of the cervical spine and the lesion intrarrhachidian extension.

There was a worsening of the respiratory obstruction with frequent dyspnea. Traqueostomy was carried out on the 5th day of hospital stay, and the patient was discharged after 4 days, being then referred to the Department of Onchology.
Onchological treatment started with chemo and radiotherapy. The patient died 1 month later due to pulmonary tromboembolism secondary to the neoplastic disease.

DISCUSSION

It is rare to find cervical spine chordomas, and its extension to the pharyngeal cavity, specially with respiratory obstruction, is even more rare. There are but a few cases reported in the literature (5,6).
The neck lesions may cause symptoms related to: 1) compression of extravertebral structures such as the upper digestive and respiratory tracts, leading to dysphagia or dyspnea; 2) root or spine compression and vertebral bone destruction; 3) palpable mass (in the neck or in the retropharyngeal space).

Pain and sensorial alterations are the most common symptoms, and they are due to bone destruction and nerve compression (4,7). In our case, the extra-vertebral (pharyngeal) manifestations were not only the most important ones, but also the earlier ones to appear because the tumor grew towards the naso-pharynx region, and this initially led the patient to seek medical help.

Intracranial chordomas of the clivus cause mainly cerebral compression and cranial nerve lesions, causing headaches and visual alterations. However, its extension towards the nasal cavity, nasal sinuses, pharynx and neck is not uncommon, presenting symptoms similar to the cervical chordoma (8,9). When involving the sacrum, they may cause local pain, compressive signs of cauda equina and rectal mass (9).

Histologically, they are commonly characterized by cells with large vacuoles in their cytoplasm called physaliphores (Figure 4). These cells are grouped and involved in abundant myxoid matrix (8,10). Biopsy is essential for both the diagnosis and therapy guidance. Because of the difficulty in accessing most of the vertebral or intravertebral lesions, the fine needle aspiration biopsy (FNA) has been one important method to collect cytopathological material (10). GUPTA et al used CT-Scan guided FNA (11). Cytology may be complemented with immunohistochemical analysis in order to diagnose differentiated forms of chordoma and other types of tumors such as chondromas and chondrosarcomas (12).

Chordomas express cytokeratin makers, epithelial membrane antigens, vimentin and S-100 protein (5,13). Both FNA and biopsy were carried out because of ease of access considering both the size and location of the lesion.

For radiology assessment, CT shows bone destruction associated to a large paravertebral soft tissue mass, with internal calcifications. CT-scan is useful in studying the degree and extension of the bone lesion. MRI has helped in improving the diagnosis of these lesions in relation to the CT-Scan because it maps out the whole tumor. The typical MRI image is a T2 hyperintensity, and we may even see heterogenous highlights when gadolinnium is used (4,8,14).

Surgery - complete tumor removal remains the treatment of choice for the chordoma. This is not always possible due to the extension and location of the lesion (involvement of noble cranial or vertebral structures), let alone its major trend towards local recurrences (3,6).

Chordomas are considered relatively radio-resistants. Radiotherapy is indicated as adjuvant treatment used to reduce recurrences or even to treat the tumor; and for inoperable cases or incomplete tumor removals. Its results have very little influence on the survival increase, however, it may significantly soothe the pain (3,4). The proton source radiotherapy seems promising, showing improvements in local control and survival rates (15). Studies with radio-surgery, brachytherapy and chemotherapy have yet to show uniform results as to their efficacy (12). It is our opinion that in the treatment of chordomas it is important to resect the most tumor possible followed by radiotherapy. Notwithstanding, our patient was not operated upon because of both tumor size and invasion, thus chemo and radiotherapy were indicated by the Department of Onchology.

The incidence of metastasis varies between 10 and 20%, and it may occur between 1 and 10 years after initial diagnosis, having little impact on the patients' survival rate. They affect soft tissue, lymph nodes lungs, bones and liver (2,12).
Cure rates for this disease vary between 10 and 24% and the 5 and 10 year survival rate is of 50% and 35%, respectively. Better survival rates are obtained when there is complete tumor resection either with or without radiotherapy (6,12,15).


FINAL COMMENTS

The rare occurrence of chordomas in the neck, specially with oropharynx extension and the difficulty to establish a differential diagnosis with other regional neoplasms make it very difficult to diagnose it properly, specially by the Otolaryngologist. Decisive factors for a better prognosis would be early diagnosis and surgery with complete tumor resection. We stress the need for the ENT to familiarize him/herself with this type of neoplasms in order to instate a careful investigation, early diagnosis and, consequently, effective treatment.

REFERENCES

1. Gelabert GM, Pintos ME, Caparrini EA, Martinez RR. Intradural cervical chordoma. J Neurosurg Sci, 43(2):159-62, 1999.
2. Zacay G, Eyal A, Faibel M, Shacked I, Kronrnberg J, Hadani M, Talmi YP. Chordoma of the cervical spine. Ann Otol Rhinol Laryngol, 109(4):438-40, 2000.
3. Soyer J, Le Carrou T, Wager M, Lapierre F. Difficulty in treating spinal chordoma of the thoracic vertebrae. Neurochirurgie, 47(1):69-71, 2001.
4. Hester TO, Valentino J, Strottmann JM, Blades DA, Robinson MC. Cervicothoracic chordoma presenting as progressive dyspnea and dysphagia. Otolaryngol Head Neck Surg, 120(1):97-100, 1999.
5. Yamashita A, Hatakeyma K, Marutsuka K, Fujita S, Ono S, Torihara K, Tamura S, Komune S, Asada Y. Chordoma of cervical vertebra protruding into the oral cavity. Pathol Int, 52(1):59-62, 2002.
6. Marquez DF, Sanabria BJ, Rivas MC, Rodriguez BJM, Cenjor EC, Gutierrez FR, Martinez PD. Cordoma faríngeo. Acta Otorrinolaringol Esp, 48(1):61-3, 1997.
7. Nina P, Franco A, Barbato R, De Gregorio A, Schisano G. Extradural low cervical chordoma. Case report. J Neurosurg Sci, 43(4):305-9, 1999.
8. Menezes AH, Traynelis VC. In: Youmans JR. Neurological Surgery, 4a edição, Philadelphia, WB Saunders Company, 3043-9, 1996.
9. Zimmerman EA, Cohen BH. In: Rowland LP. Merritt's Textbook of Neurology, 9a edição, Philadelphia, Willians & Wilkins, 379-81, 1995.
10. Kay PA, Nascimento AG, Unni KK, Salomão DR. Chordoma. Cytomorphologic findings in 14 cases diagnosed by fine needle aspiration. Acta Cytol, 47(2):202-8, 2003.
11. Gupta RK, Cheung YK, Al Ansari AG, Naran S, Lallu S, Fauck R. Diagnostic value of image-guided needle aspiration cytology in the assessment of vertebral and intervertebral lesions. Diagn Cytopathol, 27(4):191-6, 2002.
12. Naufal RF, Tedeschi H, Oliveira E, Chaddad Neto FE. In: Campos CAH, Costa HOO. Tratado de Otorrinolaringologia, 1a edição, São Paulo, Roca, 689-700, 2002.
13. Sefiani S, Amarti A, Maher M, Benkiran L, Saidi A. Dedifferentiaded chordoma of the skull base. A case report. Neurochirugie, 48(5):436-9, 2002.
14. Smolders D, Wang X, Drevelengas A, Vanhoenacker F, De schepper AM. Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol, 32(6):343-50, 2003.
15. Noel G, Habrand JL, Jauffret E, de Crevoisier R, Dederke S, Mammar H, Haie-Meder C, Pontvert D, Hasboun D, Ferrand R, Boisserie G, Beaudre A, Gaboriaud G, Guedea F, Petriz L, Mazeron JJ. Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure. Strahlenther Onkol, 179(4):241-8, 2003.

Figure 1. Oropharynx lesion.
Figure 2. CT-Scan showing the extension of the lesion to the oropharynx.
Figure 3. CT-Scan showing vertebrae destruction and intrarrhachidian lesion invasion.
Figure 4. histopathological cross-section showing broad cytoplasm cells or physaliphore cells.