Ann Otol 84: 1975

CHORDOMAS: NASOPHARYNGEAL PRESENTATION AND ATYPICAL LONG SURVIVAL HARRY

J.

RICHTER, JR., M.D.

JOHN G. BATSAKIS, M.D.

ROGER BOLES, M.D.

ANN ARBOR, MICHIGAN

SUMMARY - Atypical clinical and pathological features of cranio-eervical chordomas have prompted a review of these neoplasms at the University of Michigan Medical Center. Contrary to prior investigations, 92% of the spheno-occipital chordomas reported in this study clinically presented with a nasopharyngeal or intranasal mass. This high incidence indicates the requirement for an otorhinologic examination on all patients presenting with the neuro-ophthalmologic syndrome and roentgenologic findings characteristic of chordomas in this anatomical region. Special attention is called to a histologically distinctive variant of chordoma - the "chondroid chordoma." Apparently restricted to the cranio-cervical region, this variant has been associated with long survival.

Chordomas are relatively uncommon dysontogenetic neoplasms arising from notochordal remnants. Nearly 50% of all chordomas occur in the cranio-cervical region where, like their counterparts in the sacrococcygeal and vertebral areas, their poor prognosis is a result of their location, locally aggressive behavior, and high recurrence (more aptly, persistence) rates. 1 •2 Wright'' reports that only a small pro.portion of cranio-cervical chordomas have a clinical presentation in the nasopharynx and the majority of these show evidence of intracranial involvement. Other cases which present in the nasopharynx may be difficult to distinguish from those with a high cervical origin arising from the region of the altantooccipital membrane, atlas, and axis. Periodic clinical-pathological assessments Of the otolaryngologic aspects of chordomas have previously alerted the head and neck surgeon to this lesion, but recent reports clearly indicate there is still much to be learned from a clinical and pathological study of this unusual group of neoplasms.v"

In the last 20 years (1953-1973), 12 patients with chordomas of the craniocervical region have been seen at the University of Michigan Medical Center. Eleven of these (92%) had their clinical presentation in the nasopharynx. Additionally, three of these tumors have been classified as belonging to the recently recognized chondroid chordoma subgroup, a diagnosis which may be unfamiliar to otolaryngologists and yet have potentially considerable pathogenic and prognostic implications." CASE REPORTS

There was no apparent sex difference with 5 of the 11 patients being males. The age at time of clinical presentation ranged from 11 to 69 years with a mean of 53 years. Nine of the patients manifested varying degrees of cranial nerve involvement at the time of primary examination. The most striking example was a patient with headache, hearing loss, dysphagia, dysphonia, and deficits of III, VI, IX, X, XI, and XII cranial nerves. A mass in the nasopharynx was visualized in eleven of the patients. Surgical removal was attempted in eight

From the Departments of Otorhinolaryngology and Pathology, University of Michigan, Ann Arbor, Michigan.

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into the frontal and sphenoid sinuses (Fig. 1). A diagnosis of chordoma was made after biopsy of the nasal mass. The tumor was "removed" through an eyebrow incision and an external ethmo-fronto-sphenoidectomy. Thirteen years later, a recurrence in the frontal sinus was "removed" via a frontal sinusotomy using an osteoplastic bone flap. At that time there was some suggestion of intracranial extension. Persistence of the neoplasm produced displacement of the right orbit and diplopia. Symptomatic persistence of the neoplasm in 1971 and 1973 required bilateral frontal sinus ablations and craniotomy with subtotal removal of the tumor. The patient is alive at present but has persistent intracranial neoplasm and is blind in the right eye.

Fig. 1. (Case 1) Destruction of the ethmoid air cells and invasion of the frontal sinuses. Also evident in this Towne view is obstruction of the maxillary sinus.

patients, biopsy only in two patients, and biopsy and irradiation (6630 rads) in one patient. One of the patients underwent transpalatal resection and a CI-C3 laminectomy followed by 6000 rads of external radiation. Seven patients died within two years as a direct consequence of their neoplasm; two of these may be considered as postoperative deaths. All had persistent chordoma. Five patients remain alive, only one without evidence of neoplasm (Case 2). The longest survivor has had five "recurrences" over an 18lh year period. His neoplasm was classified as a chondroid chordoma. Greater detail of four of our patients is presented below because of their clinical and/or pathological features.

Case 1. A 39-year-old man was admitted to the University of Michigan Medical Center in June 1955 after a two month history of vertigo. A large fleshy mass protruded from the right nasal chamber. Radiological studies demonstrated destruction of the posterior ethmoid air cells and extension

Comments. Microscopic examination of the neoplasm removed at the initial operation and from subsequent excisions has demonstrated a progressive chondroid component to the tumor, until the last specimen which is nearly indistinguishable from a chondrosarcoma. Figure 2 illustrates the chordoma adjacent to an area of chondroid change. Figure 3, from the first recurrence, depicts a transition of chordoma to a chondroid lesion with lacunar cells. Pathological diagnosis: Chondroid chordoma with progressive chondrification. Case 2. A 69-year-old man presented to the hospital with severe neck pain in August 1962. Relief was achieved by resection of the greater occipital nerve. One year later he was readmitted with clinical evidence of involvement of the III, IV, VI, IX, X, XI, and XII cranial nerves and a large soft mass in the nasopharynx. The clivus was poorly delineated on radiological examination of the skull (Fig. 4). Biopsy of the nasopharyngeal mass revealed a chordoma and the patient received 6630 rads. At first. there was no apoarent response, but gradually the cranial neuropathies abated. and on the last follow-up visit (April 1973) there was no evidence of cranial nerve deficit or of a tumor recurrence.

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Fig. 2. (Case 1) Chordomatous tissue lies next to a group of cells that is surrounded by a cartilaginous matrix (lower left). Pathological diagnosis is chondroid chordoma with progressive chondrification. Fig. 3. (Case 1) Recurrent lesion. Atypical cartilaginous areas in chordoma.

Comments. The neoplasm was a typical chordoma characterized by progressive inter- and intracellular vacuolization. No chondroid areas were noted. Pathological diagnosis; chordoma. Case 3. A 68-year-old woman was admitted to the hospital in February 1973 with what appeared clinically and radiologically to be sinusitis. In September 1973 unilateral epistaxis occurred and examination revealed a mass protruding from the ethmoid region into the left nasal cavity. Biopsy was performed and a diagnosis was made. She manifested no cranial neuropathies or evidence of intracranial extension of the tumor. She returned to her referring physician who planned an external frontoethmoidectomy. The pathological diagnosis of the tumor was chondroid chordoma. Case 4. A 47-year-old woman presented to the hospital with a two-year

history of neck pain and hyponasal speech. Cervical spine and skull roentgenograms showed demineralization of the sella and a large mass low in the nasopharynx (Fig. 4). She underwent a CI-C3 laminectomy with subtotal removal of her tumor. This was followed by a transpalatal removal of residual tumor on the posterior pharyngeal wall. Within four months, there was considerable clinical deterioration and the onset of multiple cranial nerve involvement. Cervical decompression and irradiation with 6000 rods of cobalt therapy did not alter the progression of the disease, and she died of intracranial complications within one year of the primary diagnosis.

Comments. The distinctly biphasic appearance of the neoplasm is clearly seen in Figures 5 and 6. The distinctive chondroma appearance is present in Figure 5. Figure 6 is highlighted by cartilaginous metaplasia in the intercellular stroma and beginning lacunar

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Fig. 4. ( Case 2) Lateral view of the skull showing nasopharyngeal soft tissue mass and demineralization of the clivus.

formation in the cells. Pathological diagnosis: chondroid chordoma. DISCUSSION

Chordomas presenting in the nasopharynx may have no apparent extension outside of this region and may be considered as true primary nasopharyngeal tumors, but these are uncommon. Such tumors are more likely the result of a downward extension from a craniooccipital tumor or as an upward extension of a high cervical growth.P-" As exemplified by our patients, base of skull (cranio-cervical) chordomas are usually associated with a characteristic neuro-ophthalmologic and otologic symptom pattern, highlighted by multiple cranial nerve involvement and localized headaehes.v" Destruction of bone (clivus, sphenoid), intracranial masses,' and nasopharyngeal masses are characteristic roentgenographic findings. 2 - 4 Diagnosis is rarely made on clinical grounds, and the correct histological diagnosis often evades the pathologist on his first or even subsequent examinations. Several factors relate to this difficulty, The appearance of a chor-

doma varies according to the biological age of the neoplasm and to the site of the biopsied tissue." Even with a prepared mind, however, chordomas and chondromatous tumors of this region are distinguished with difficulty. The recently recognized chondroid chordoma! and chordoid sarcoma" offer some explanation for the variable appearances of some of the chordomas at the base of the skull. Three of our cases (1,3,4) represent chondroid chordomas as described by Heffelfinger et al." Two of the three manifested calcification, a feature not often displayed by the typical chordoma. The calcification may also serve as a distinguishing radiological sign." The chondroid chordoma may contain such an abundant cartilaginous component that the diagnosis of chondroma or chondrosarcoma is suggested or even made. Most often there seems to be an admixture of chondroid and chordoma elements that range from small scattered areas of hyaline cartilage in a chordoma background to the reverse in which the chondroid component dominates.' Merger between typical chordoma and the cartilaginous foci should

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Fig. 5. ( Case 4) Typical chordoma appearance. Compare with Fig. 6. Fig. 6. (Case 4) Same tumor as in Figure 5. Cartilaginous melaplasla in the intercellular stroma and beginning lacunar formation in the cells. Pathological diagnosis is chondroid chordoma.

be looked for. Short of this, the identification of syncytial chordoma cells in a cartilaginous matrix will identify the lesion as a chondroid chordoma. To facilitate histological screening for chordomatous areas in a predominately chondroid tumor, we applied phosphotungstic acid hematoxylin (PTAH) and reticulin stains as described by Crawford." Confirming our earlier experience, however, these special stains were not helpfuJ.2 Heffelfinger's- study suggests a considerable difference between the typical spheno-occipital chordomas and the chondroid chordomas: an average survival time for 19 patients with chondroid chordomas of 15.8 years as compared to 4.1 years for 36 patients with typical chordomas. This difference exists despite similar treatment programs. Case 1 illustrates the better prognosis of a prominent chondroid element in a chordoma. Case 4, on the

other hand, while possessing a similar histological appearance, died from intracranial extension soon after diagnosis was made. Case 3, the third example of chondroid chordoma, has had insufficient follow-up for judgment. The typical (nonchondroid) chordoma of the cranio-cervical region does not carry a good prognosis. The experience of Dahlin and MacCarty" is fairly' representative. Only two of their fifteen patients survived longer than five years, one living 18.7 years after subtotal surgical removal and radiation therapy. The atypical behavior of Case 2 with a similar treatment program would seem to indicate that irradiation has a potential benefit in extending prognosis in the typical chordoma. Metastases from craniocervical chordomas are rare but have been documented.s Wright" reported that only a small number of cranio-occipital chor-

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domas have a nasopharyngeal presentation. We record a 92% frequency. He also indicated that all nasopharyngeal

chordomas had evidence of an intracranial extension. Four of our cases presented without such evidence.

DEPT. OF OTORHINOLARYNGOLOGY, UNIV. OF MICHIGAN MEDICAL CENTER, MICH. 48104.

ANN

ARBOR,

REFERENCES 1. Utne JR, Pugh DG: The roentgenologic aspects of chordoma. Am J Hoentgenol Radium Ther Nucl Med 74:593-608, 1955

2. Batsakis JG, Kittleson AC: Chordomas: Otorhinolaryngologic presentation and diagnosis. Arch Otolaryngol 78: 168-175, 1963 3. Wright D: Nasopharyngeal and cervical chordoma - some aspects of their development and treatment. J Laryngol81: 1337-1355, 1967 4. Heffelfinger MJ, Dahlin DC, MacCarty

CS, et al: Chordomas and cartilaginous tumors at the skull base. Cancer 32:410-420, 1973 5. Martin RF, Melnick PJ, Warner NE, et al: Chordoid sarcoma. Am J Clin Path 59: 623-635, 1973 6. Crawford T: The staining reactions of chordoma. J Clin Path 11: 110-113, 1958 7. Dahlin DC, MacCarty CS: Chordoma: A study of fifty-nine cases. Cancer 5: 11701178,1952

DEAFNESS RESEARCH FOUNDATION GRANTS The Deafness Research Foundation announces that applications for research grant support for the calendar year 1976 must be submitted prior to August 15, 1975, for consideration by the Scientific Review Committee, Only those applications on the new grant application forms will be accepted for consideration. For further information and updated application forms, write to: The Deafness Research Foundation, 366 Madison Avenue, New York, New York 10017.

MODERN TRENDS IN OTOLOGIC SURGERY A course including seminars, discussions, and surgical demonstrations will be presented Oct. 28 - Nov. 1, 1975, by the Department of Surgery, Section of Otolarvngoloy, Notre-Dame Hospital, Montreal, Quebec. Guest lecturers will include Professor Ugo Fisch, Juan Marquet, M.D., Brian McCabe, M.D., and George Shambaugh, Jr., M.D. For further information write: Robert A. Bertrand, M.D., Vestibular Laboratory, NotreDame Hospital, 1560 Sherbrooke St. East, Montreal, Quebec, Canada,H2L 4 MI.

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Chordomas: nasopharyngeal presentation and atypical long survival.

Atypical clinical and pathological features of a cranio-cervical chordomas have prompted a review of these neoplasms at the University of Michigan Med...
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