大韓放 射線醫學 會 誌 第 27 卷 第l號 pp.45-48, 1991 Journal of Korean Radiological Society, 27(1) 45-48, 1991

Primary Chondroid Chordoma arising from the Petrous Temporal Bone

: A Case Report

Young Uk Lee, M.D., Eun Kyung Youn, M.D.

Department o[ Radiology, Koryo General Hospital

〈국문초록〉

측두골 추체부에 발생한 원발성 연을양 척색종

-1 례 보고-

고려병원 진단방사선과

01 여 c:>

c:> c:> ':11 • L!!"

c:> '- 경

척색종은 잔여 원시척색으로부터 발생하는 흔치 않은 종양이며， 이중 원발성 연골양 척색종은 매 우 드물어 2예가 문헌상

에 보고되고 있다. 저자들은 좌측 측두골 추체부에 발생한 원발성 연골양 척색종 1 예 를 경험하였기에 문헌고찰과 함께 보고

하는 바이다.

Index Words: Petrous temporal bone. ch ordoma. 213.327

Petrous temporal bone. CT. 213. 1211

Chordomas are uncommon tumors which arise

from remnants of the primitive notochord. They are

situated chie f1y in the anterior spinal axis with a

predilection for the sacrococcygeal region and the

basioccipu t. About 50% of chordomas are sacrococ­

cygeal. 35% are intracranial. and 15 % arise from a

vertebral body (1 .6). As a histologic variant of chor­

doma. ‘ chondroid chordoma ’ was first described by

Heffelfinger et al (2) . We present a rare case ofprimary

chondroid chordoma arising from the petrous tem­

poral bone. To our knowledge ‘ only two other cases

of this type have been reported earlier (7.8) .

Case Report

A 43-year-old female presented with a thirty

month history of continuous headache and pro­

gressive difficulty in hearing , and with a two month

history of progressive dysphagia. dysarthria. a nd left

sided facial weakness. Neurologic examination reveal-

ed a group of left sided crania l nerve pa lsies involv­

ing the VIIth . VIIIth . IXth . Xth. XIth. and XIlth

cranial nerves

The plain films of the skull revealed irregula r

destruction with subtle calcifications a long th e

superomedial aspect of the left petrous temporal

bone. Routine axial brain computed tomography (CT)

revealed a 4x4cm s ized hypodense mass with

peripheral enhancem ent and internal calcified foci at

the left cerebellopontine angle (CPA) region (Fig. 1) .

On corona l CT. the mass was downward extended

through the skull base into the left parapharyngeal

space. The lower exten t of the growth was at the level

of the soft palate (Fig. 2) . On high-resolution temporal

bone CT , the petrous portion 와ld a part of the

m astoid portion of the left temporal bone were

destroyed. Superior a nd posterior walls ofthe left in­

ternal auditory canal (IAC) were a lso destroyed. but

th e re was no actual widening of the IAC (Fig. 3).

A suboccipital craniectomy was done , and left

이 논문은 1990년 10월 1 5일 접수하여 1 99 1년 1 월 4일에 채택되었음 Received October 15. accepted J anuary 4. 199 1

- 45-

大韓放射線醫學會誌 : 第 27 卷 第 l 號 1991

a b

Fig. 2. a. Coronal CT shows mass exten­sion through the skull base into the left parapharyngeal space. Intralesional calcifications are also seen on the parapharyngeal area as well as on the CPA region. b . On axial neck CT , the mass is well ‘ demonstrated. The left styloid process is I laterally displaced ‘

a

cerebellar hemisphere was m edially tra cted. Then the

part of da rk.reddish tumor mass was exposed . It was

surrounded by cerebella r dura mater a nd ha d a mix.

ed consistency composed of gelatinous and fragile

components. It was pa rtially removed

Microscopic examination showed that the tumor

was composed of two areas with more cellula r and

less cellular components. The more cellular a rea was

composed of monotonous physaliferous cells which

had abundant vacuolated cytoplasm. A bluish chon.

droid m a trix with s evera l chondrocyte -like cells was

also d emonstrated in the cell s parse r egion (Fig. 4) .

There was no atypical spindle cell . This appearance

was compatible with chondroid chordoma .

The patien t was given ex ternal ra diothera py at a

Fig. 1. Precontrast (a) and postcontrast (b) brain CT scans: A 4 x 4 cm sized left CPA mass shows internal calcifications and peripher외 enhancçment. Mass effect on the fourth ventricle is a1so seen .

b

dose of 5000 rads over a period of five weeks. She is

be ing on follow-up .

Discussion

Chordomas are neoplasms of notochordal origin

which represent about 0.1 to 0.2 % of all intracranial

n eoplasms (9). The notochord extends from the coc­

cyx to the basisphenoid and has the role in forma­

tion of the axial skeleton. So. most of chordomas have

predilection sites along the midline axis , such as

sacrococcygeal region and clivus. During the fifth

w eek of fetallife , in the region of the skull base , the

notochord pe rsues a sigmoid shaped path

Throughout its course , the notochord gives off

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Young Uk Lee , et al: Primary Chondroid Chordoma arising from the Petrous Temporal Bone

‘ a

b

Fig.3. High resolution tempora l bone CT : a. Axia l scan shows destructive change involving the petrous portion and a part of the mastoid portion of the left temporal bone. Note the extensive destruction of the left jugular fossa . b. Corona l scan shows destruction of the superior wall of the left IAC without definite widening

numerous d e ndrite-like branches. some of which

pen etrate the base of the skull anlage a nd others

whic h ramify under the nasophary ngeal mucosa(l 0 .1 l)

Therefore . it is possible that portions of these den­

dritic processes of the notochord become d e tached

during the development and afford a basis for th e

development of chordomas at unusual sites.

The term ‘ primary chordoma ‘ h as been coined for

those chordomas which arise from unusua l extraax­

ial sites by Shugar et al (10) The maxilla (10) and base

of the temporal bone (7 ,8) have been described as

such sites of origin in the literature.

The term ‘ chondroid chordoma' m eans a

histologic variant of chordoma which shows car­

tilaginous tissues in addition to the c lassical picture

of a chordoma. Embryologically. m esoderm sur­

rounds the notochord and eventually produces a car­

tilaginous model of the spine. These m esodermal

rests m ay become trapped in chordal vestiges and

produce a cartilaginous component in the adult

tumor. About 15% of all chordomas a re chondroid

a

b

Fig. 4. a. The lower power ::;llue of the pathologic specimen shows more ceIlu lar and less ceIlular areas . There is chondroid matrix with chondrocytelike cells in the ceIlspa rse region . b. The high power slide of the more cellular region: The physaliferous cells with abundant vacuolated cytoplasms are typical of chordoma

chordomas. (2).

The case that we report is unsua l in being both

a prima ry as w e ll as a chondroid chordoma. Our pa­

tient presente d with left sided low cra nia l nerve

palsies. The pla in skull film and the CT findings were

compatible with the radiologic characteristics of chor­

domas such as bony destruction and a n extraosseous

soft tissue mass with intralesional calcifica tions (12)

Bu t. the site of the lesion was unusual for c hordomas

With the location . other possibilities of schwannoma.

glomus jugulare. m etastasia , and cartilaginous tumor

such as chondrosarcoma were also considere d.

Cranial chordomas are usually diagnosed between

the fourth and fifth d ecades of life. And there is no

sexual predilection or a slight male predominance

But. chondroid chordomas present at a n earlier age

with female pre dilection. There is no d efinitely useful

radiographic findings to differentia te chondroid chor­

domas from typical chordomas. The inc ide n ce of

calcification a ppears to be higher with the former

than with the latter. The recent report a bout MR fin-

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大廳放射線醫學會끓‘ : 第 27 卷 第 l 號 1991

ding of the chordoma suggested that chondroid chor­

domas had shorter Tl and T2 relaxa tion times than

typical chordomas. pe rhaps because the watery and

gelatinous matrix was replaced by the cartilaginous

elements (13) . The pathologic diagnosis of chordoma

is based on the findings of large vacuolated

‘ physalife rous ce lls ’ Chondrid chordoma is

charaterized by areas resembling hyaline cartilage in

addition to typical chordomatous areas.

The treatm ent of choice is total surgical removal,

but cranial chordomas are seldom resectable because

of the proximity of vital structures a nd the relative

surgical inaccessibility of these areas (14). 80. the

treatment includes both surgical resection and

radiotherapy. Postoperative radiotherapy may help

to control the tumor over a longer period (3) and the

response is dose dependent (15). In our patient. the

surgical removal of the tumor was incomplete and

followed by radiotherapy.

Average survival rates of chordomas vary from 4.2

years (1 6) to 7 .7 years (4). Primary chordomas are

known to have a better prognosis than typical chor­

domas in the usual sites (10) . According to Heffelf­

inger et a\. (2). chondroid chordomas 외so have a

better prognosis with the mean survival of 15.6 years.

Further follow-up is required for our patient to con­

firm this.

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