World J. Surg. 16, 1010-1016, 1992

World Journal of Surgery © 1992 by lhe Soci~l~ Intemationale de Chirurgie

Chondrosarcomas of the Head and Neck D e b o r a h S. R u a r k , M . D . , U w e K. S c h l e h a i d e r , M . D . , and Jatin P. S h a h , M . D . Head and Neck Service, Memorial Sloan-Kettering Cancer Center, New York, New York, U.S.A. Chondrosarcomas of the head and neck are uncommon. Over a 35-year period (1950-1985) at our institution, 557 patients had an estabfished diagnosis of chondrosarcoma. In 28 (5%) of these patients, the tumor was located in the head and neck region. A retrospective analysis was performed in an attempt to accurately define clinical characteristics, management, and outcome in these 28 patients. The patient population consisted of 15 males and 13 females whose ages ranged from 10 to 72 years, with a median of 38 years. Ten (36%) patients were untreated and 18 (64%) previously treated patients were referred for further management. The primary sites were maxilla (11), cervical vertebrae (7), mandible (3), skull (2), sphenoid and ethmoid sinuses (2), frontal sinus (1), nasal septum (1), and orbit (1). Lesions arising in the larynx were excluded from this study. The most "common presenting symptom was a painless mass. Surgery was the definitive treatment in all patients. However, adjuvant radiation therapy or chemotherapy was utilized for residual disease in almost half of the cases (13 patients). Follow-up ranged from 5 to 35 years. Twelve (43%) of the 28 patients were known to be alive and free of disease more than 5 years after surgery. The most common cause of death was uncontrollable local disease (88%). However, only 3 of the 10 previously untreated patients developed local recurrence. For all patients, the median interval from time of first recurrence until death was 25 months (range 6 to 70 months). Positive margins in 8 of 12 patients resulted in local recurrence and eventual death. Distant metastases were documented in 18% of patients, most often involving the lungs. Analysis of a subgroup of patients treated more recently (1975-1985) reveals 7 (78%) of 9 patients to be alive and free of disease at 5 years postoperatively.

Chondrosarcomas are a heterogeneous group of malignant tumors of cartilaginous origin. Although chondrosarcoma is the second most common primary spindle cell tumor of bone, it is uncommon in the head and neck region. Various reports estimate that between 6.4% and 12% of all chondrosarcomas will be located in the head and neck area [1-3]. This review of all patients with chondrosarcoma treated at our institution over the past 35 years was performed in order to accurately define clinical characteristics, management, and outcome. Presented at the Soci6t6 Internationale de Chirurgie and the Collegium Internationale Chirurgiae Digestivae in Stockholm, Sweden, August, 1991. Reprint requests: Deborah S. Ruark, M.D., Department of Surgery, Monmouth Medical Center, 300 Second Avenue, Long Branch, New Jersey 07740, U,S.A.

Materials and Methods

Tumor registry data from 1950 to 1985 were examined to identify all patients admitted to Memorial Sloan-Kettering Cancer Center (MSKCC) with a final discharge diagnosis of chondrosarcoma. A total of 557 patients were identified. A retrospective chart analysis was performed on 28 patients with chondrosarcoma of the head and neck. Lesions arising in the larynx were excluded from this study. Data collection included vital statistics, presenting symptoms, site of origin, radiologic examination, treatment, pathology, local recurrence and metastasis, follow-up, and results of therapy. Survival curves were plotted using the Kaplan-Meier method. Two-sided tests of significance were performed by using the Wilcoxon test. Results

The distribution of all sites are shown in Figure 1. The most common sites of origin of chondrosarcoma are the pelvis, femur, and humerus, followed by the tibia, ribs, scapula, head and neck, and sternum. Individual categories of all other sites have an incidence of < 2 % each. In this series, the population of 28 patients with chondrosarcoma of the head and neck consisted of 15 males and 13 females (male to female ratio 1.2:l). Patient ages ranged from l0 to 72 years, with a median age of 38 years. Approximately 21% of the group (6 patients) were children 5 years postoperatively. In addition, 2 patients survived >5 years (7 and 10 years) with Stable residual disease and 1 patient with initial positive margins died at 5 years from a motor vehicle accident with no evidence of disease. The overall 5 year survival rate was 54%. The most COmmon cause of death was uncontrollable local disease (15 of 17 patients, 88%). In all patients, the median interval from time

MANDIBLE 3

SKULL 2

ORBIT 1 NASAL SEPTUM 1 RON TAL SINUS 1 -R SINUSES 2

Fig. 2. Primary sites of chondrosarcoma of the head and neck in 28 patients.

of first recurrence until death was 25 months (range 6 to 70 months). Ten patients of the entire group were previously untreated. Three of these patients developed local recurrence and died within 5 years, giving a survival rate of 70%, which remained unchanged thereafter. Eighteen patients of the entire group were previously treated elsewhere. Data in this group revealed 39% survival at 5 years, with ultimate survival of only 22% for length of follow-up of this study. Local recurrence occurred in 8 (67%) of 12 patients with positive margins, resulting in 5-year survival rate of 42%. All patients with negative margins survived a minimum of 5 years, free of disease. This was statistically significant (p = 0.019) (Fig. 3). Examination of patient survival based on tumor grading revealed a 5-year survival rate of 77% for low-grade lesions and only 33% for high-grade lesions. Statistical analysis revealed p = 0.035 by the Wilcoxon test (Fig. 4), Analysis of a subgroup of patients treated more recently (1975-1985) showed 78% survival compared with those treated in the earlier period (19501975) with a 5-year survival rate of 42% (p = 0.094) (Fig. 5). For purposes of survival analysis, patients were categorized into different anatomic sites of maxilla, cervical vertebrae, and all others. Although minimal survival improvement was seen in comparison of maxilla to cervical vertebrae (64% vs 43%, respectively), no significance was delineated. Distant metastases were documented in 18% of patients, most often involving the lung. Discussion

Chondrosarcoma can be one of the most difficult of the malignant bone tumors to distinguish from the standpoint of the histopathologist [1]. The spectrum ranges from a well-differentiated growth resembling a benign cartilage tumor to a highgrade malignancy with aggressive local behavior and the potential to metastasize [4]. In this study 9 (32%) patients had been previously diagnosed as having benign chordoma, chondroma, or osteochondroma before the establishment of accurate diagnosis of chondrosarcoma. The time interval between these two events ranged from 6 to 120 months, with a median interval of 12 months. In 2 of these patients, the diagnosis was changed from benign to

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World J. Surg. Vol. 16, No. 5, Sept./Oct. 1992

Table 1. Clinical characteristics of 28 patients with chondrosarcoma of the head and neck. Pt. no.

Local Recurrence (Times)

Age (yrs)

Sex

Site of Origin

Symptoms

Definitive Treatment

I.

45

F

Maxilla

28

M

Mandible

Maxillectomy & orbital exenteration Partial mandibulectomy

Yes

2.

Nasal obstruction & discharge Chin numbness, painful teeth

3.

31

F

Mandible (lower gingiva)

Painless mass × 2 mos

Yes

4.

26

M

Painless mass × 2 mos

5.

35

F

6.

72

M

7.

29

F

Maxilla, alveolar ridge Sphenoid & ethmoid sinuses Anterior ethmoid & nasal bone Maxilla

Marginal mandibulectomy, testosterone Egypt 21, virus, radioactive thallium Subtotal maxillectomy

8.

15

M

Orbit (retrobulbar)

Blurred vision, proptosis

9.

56

M

Vertebra C5

Pain in shoulder arm, hand

10.

46

2F

Nasal septum

Nasal obstruction, loss of smell

I1.

56

F

Maxilla (hard palate)

12.

53

F

Maxilla

13.

l0

F

Maxilla

Nasal obstruction, mass in hard palate Painless enlargement of upper gingiva & hard palate Painless mass, on dental exam

14.

42

M

Vertebra

Painless mass for 1 year, neck discomfort

15.

38

M

Vertebrae C7T3

16.

44

M

Maxilla

17.

18

M

18.

59

F

Maxilla (alveolar ridge) Vertebrae C7, TI

19.

36

M

Lower neck & upper back pain Painless mass in upper jaw Painless mass in upper jaw 2-3 wks. Pain for I year in shoulder, fingers Enlarging space between incisors

Maxilla

Yes

No

Pathology

Follow-up

Low grade, margins positive 8 cm mass, microscopic margins positive Low grade initially, 1.4 cm mass, high grade with skull base & brain invasion at recurrence Grade 1, margins clear

DOD, 49 mos Postop death 9 mos, mets to lung DOD, 28 mos, mets to sternum Alive, NED at 35 yr

Impaired vision, right eye × 2 weeks Impaired vision, painless mass

Transfrontal craniotomy, excision of tumor, postop RT Wide local excision of forehead

Yes

Low grade, skull base invasion

DOD, 6 yr 9 mos

Yes

Low grade

DOD, 11 yr

Nasal obstruction & sinusitis

Orbital exenteration, partial maxillectomy, partial skull base removal, postop RT Orbital exenteration, partial maxillectomy, excision of zygoma, postop RT Excision of spinous process C5-6, postop RT Excision of hard palate, maxillary antrum, ethmoids, sphenoids, postop RT Excision of hard palate, partial bilateral maxillectomy

Yes

Invasion of skull base

DOD, 9 mos, postop complication

Yes (3)

Resection incomplete, gross margins positive

DOD, 54 mos, mets to lung

Yes (3)

No grade

Yes (1)

Tumor margins positive, resection incomplete

DOD, 39 mos, mets to lymph node DOD, 6 yr l mos

Yes

5 cm mass, low grade, all tumor grossly resected

DOD, 46 mos

Simple maxillectomy

No

Low grade, 3.5 cm mass, margins clear

Alive, NED 27 yr

Radical bilateral maxillectomy, excision of hard palate & nasal cavity, preop RT Preop RT, excision tumor, cervical laminectomy C2, partial C3 laminectomy, postop RT Laminectomy, postop chemo and RT

Yes (2)

No grade

DOD, 23 mos

Yes (2)

Low grade, 5 cm mass

DOD, 33 mos

Yes (2)

High grade, incomplete resection, anaplastic at autopsy Low grade, margins clear Low grade, 1 cm mass, margins clear

DOD, 51 mos, mets to lung

DOD, 16 mos postop meningitis Alive, NED 16 yr

Subtotal maxillectomy

No

Partial maxillectomy

No

T1 vertebral body resection, laminectomy, postop RT Partial maxillectomy

Yes

Grade I1, margins positive

Yes

Low grade, 1.5 cm mass, margins positive

Alive, NED 25 yr Alive, NED 20 yr

D,S. Ruark et al.: Chondrosareomas of the Head and Neck

1013

Table 1. Continued.

Local Recurrence (Times)

Pt. no.

Age (yrs)

Sex

Site of Origin

Symptoms

Treatment

20.

20

F

Vertebrae C5-C7

21.

28

F

Frontal sinus

Arms numb, tingling, I yr, mass x 3 too. Forehead mass diplopia

22.

51

M

Mandible

Painless mass

Cervical laminectomy, C5-C7 with removal of tumor, postop RT Bilateral radical frontal sinus excision, brachytherapy, ethmoidectomy, sphenoidectomy, postop RT & chemo Marginal mandibulectomy

23.

18

M

Skull (parietal)

Skull mass, headache

24.

48

M

Maxilla

25.

48

F

Skull (parietal occiptal)

26.

15

F

27.

50

M

Posterior pharyngeal wall & cervical spine Vertebrae C3C5

28.

18

M

Maxilla (upper alveolus)

Pathology

Follow-up Alive, NED 5 yr 4 mos

Yes

Low grade, 5 cm mass, margins clear Grade ll, incomplete resection

No

No grade

Parietal craniectomy, postop chemo and RT

Yes

Progressive cheek swelling

Total maxiUectomy

Yes (2)

Headaches, diplopia, memory loss paresthesias Hoarseness, dysphagia, 1 mo

Occipital craniotomy, postop chemo and RT

Yes (2)

Mesenchymal chondrosarcoma incomplete resection Grade II, initially high grade at final resection, initial incomplete resection Mesenchymal (necrotic) chondrosarcoma

Anterior pharyngotomy, resection of retropharyngeal tumor

Yes

Grade II, 4 cm mass, margins positive at initial resection

Auto death, NED 5 yr

Resection of tumor of cervical spine, cryotherapy Partial maxillectomy.

No

Low grade, 4 cm mass

Alive, NED 9 yr 6 mos

No

Low grade, 1 cm mass, margins clear

Alive, NED 7 yr

Shoulder pain 2 mos Painless mass

No

.._._

DOD, 13 mos

Alive, NED I1 yr Alive, 7 yr l0 mo with residual disease Alive, 10 yr disease status unknown DOD, 37 mos

F: Female; M: Male; NED: No evidence of disease; DOD: Died of disease; RT: Radiotherapy; Mets: Metastasis; Postop: Postoperatively; Chemo: Chemotherapy; Mos: months; Yr: years.

malignant chondrosarcoma after the original slides were reviewed. A high index of suspicion should be maintained beCause of the infrequent incidence of benign chondroma in the head and neck region. The majority of lesions in this patient population were histologically classified as low grade (68%) which is comparable to other series reported in the literature [3, 5]. The overall 5"Year survival rate of 54% is a reflection of a large proportion of previously treated patients in this series. The subgroup of Primary cases had a 70% 5-year survival in comparison to Secondary cases (those treated before) in which a lower survival of 39% was noted. Survival ranging from 44% to 81% is reported in the literature [5-7]. It has been stated previously that contrary to earlier reports, ehondrosarcoma seems to be no more aggressive in children than adults [8]. Approximately one-fifth (21%) of our patients Were 2 years. Metastases were not uncommon and occurred in 5 (18%) patients with specific sites being the lung, sternum, and regional lymph nodes. A trend toward improved survival was observed during the last 10 years of this study when compared with the previous 25 year period (78% vs. 42%) with the difference approaching significance ~ = 0.094). Analysis of factors such as histology or site or origin were not found to contribute to the improved survival as there was no difference from the previous time period. It was recently reported by Hertzanu [11] from Johannesburg that computer tomography (CT) plays an important role in delineating the extent of the tumor with visualization of bony destruction, associated soft-tissue mass, and calcification, information which is essential for surgical and therapeutic planning. It is our impression that accurate preoperative assessment by CT scan facilitated more complete surgical resection and occasional use of adjuvant therapy may have influenced the long-term outcome. Surgery remains the most effective primary modality in the treatment of chondrosarcoma. An aggressive approach with the intent of obtaining histologically clear margins has been shown in this series to prevent local recurrence. Radiotherapy was administered principally in patients with inoperable recurrence

I

~

I

I

I

I

I

I

12

18

24

so

a5

42

48

54

so

Time (Months)

Time (Monthe) Low Grade (n - 13)

I

s

1950 - 1976 (n-19)

----i--1975 - 1986 (n-g)

p • 0.094 Fig. 5. Survival of patients treated from 1950-1975 versus 1975-1985.

or inadequate surgical margins. Data presented in the Princess Margaret Hospital series suggests that postoperative radiotherapy can achieve local control in a significant number of cases (47%) [12, 13]. McNaney and coworkers [14] at M.D. Anderson Hospital reported similar results. Only 2 patients in this series received pre-operative radiotherapy, both resulting in either no improvement or progression of disease. Subsequent therapy administered included multiple modalities, however, both patients died at 23 and 33 months postoperatively. We recognize that this is limited experience, but most other series as well indicate that radiotherapy is more frequently utilized postoperatively or for palliation [3, 7, 12-14]. Chemotherapy combined with radiation therapy was utilized as adjuvant treatment in selected cases of high-grade tumors [2], mesenchymal chondrosarcomas [2], or rapid local recurrence with aggressive behavior and potential for metastasis [ t ]. Regimens varied from 1 to 8 cycles, most frequently used agents being cyclophosphamide, vincristine, methotrexate, doxorubicin, and actinomycin D. Survival in this group of 5 patients ranged from 13 months to 94 months, with a median of 37 months. Little information appears in the literature regarding benefit of adjuvant chemotherapy. The number of patients in this series receiving chemotherapy does not permit conclusions regarding its value as adjuvant therapy. In our opinion it may offer palliation in some select cases, particularly high-grade or mesenchymal chondrosarcoma. R~sum~

La localisation au niveaude la trte et du cou du chrondrosarcome est rare. Entre 1950--1985 (30 ans), dans notre institution, le diagnostic de chrondosarcome a 6t6 6tabli chez 557 patients. Chez 28 de ces patients (5%), la tumeur 6tait localis6e dans la rrgion de la trte et du cou. Par une analyse rrtrospective, on a d6fini les caractrristiques cliniques, le traitement, et 1'6volution de ces 28 patients. I1 s'agissait de 15 hommes et de 13 femmes dont les ~ges allaient de 10 ~ 72 ans (m6diane = 38 ans). Dix patients (36%) n'avaient pas eu de traitement antrrieur alors que 18 (64%) avaient drjb. 6t6 trait6s ailleurs et avaient 6t6 adressrs chez nous pour traitement complrmentaire. Les local-

D.S. Ruark et al.: Chondrosarcomas of the Head and Neck

isations principales 6taient le maxillaire s u # r i e u r (n = 11), les vertSbres cervicales (n = 7), le mandibule (n = 3), le crfine (n = 2), les sinus sph6noi'de et 6thmoide (n = 2), le sinus frontal (n = 1), le septum nasal (n = 1) et l'orbite (n = 1). Les 16sions du larynx ont 6t6 exclues de cette 8tude. Le sympt6me principal 6tait la d6couverte d ' u n e masse indolore. Le traitement d6finitif propos6 a 8t6 la chirurgie dans t o u s l e s cas. L a radioth6rapie ou la ehimioth6rapie adjuvante ont 8t8 utilis6es pour traiter des r6sidus tumoraux chez environ la moiti6 des patients (n = 13). Pour I'ensemble des patients, le suivi est all6e de 5 et 35 ans. Au rnoins 12 des 28 patients (43%) sont en vie sans rfcidive h plus de 5 ans. L a cause de d6cSs la plus fr6quente a 8t6 la rScidive locale noncontr61able (88%). Cependant, seulement trois des 10 Patients n ' a y a n t pas eu de traitement ant6rieur ont rfcidiv6 localement. Pour l'ensemble des patients, l'intervalle (valeur m6diane) entre les premiers signes de r6cidive et la mort a 6t6 de 25 mois (extr6mes 6 h 70 mois). Huit des 12 patients ayant eu des marges des s6curit6 envahies ont eu une r6cidive locale et sont morts. Les m f t a s t a s e s h distance ont 6t8 observ6es chez 18% des patients, le plus souvent au niveau des poumons. L'analyse d ' u n sous-groupe de patients (n = 9) trait6s plus rScemment (1975-1985) montre que 7 (78%) sont en vie sans nlaladie 6volutive h 5 ans. Resumen

Los condrosarcomas de la cabeza y el cuello son neoplamas taros. En un periodo de 35 afios (1950-1985) se atendieron 557 Pacientes con el diagn6stico de c o n d r o s a r c o m a en nuestra instituci6n. En 28 de los pacientes (5%) el tumor estaba ubicado en la regi6n de la cabeza y el cuello. Se realiz6 un anAlisis retrospectivo con el objeto de definir las caracteristicas clfnicas, el manejo y el resultado obtenido en estos 28 pacientes. E! grupo i n c h y 6 15 hombres y 13 mujeres con edades entre 10 y 72 afios y una edad media de 38 afios. Diez pacientes (36%) no fueron tratados y 18 pacientes (64%) que habfan sido tratados previamente fueron referidos para terapia adicional. Los sitios prirnarios fueron el maxilar (11), las v6rtebras cervicales (7), la mandibula (3), el cffmeo (2), los senos esfenoidales y etmoidales (2), los senos frontales (1), el septo nasal (1) y la 6rbita (1). Las lesiones originarias en la laringe fueron excluidas de este estudio. El sfntoma m,'is comtin de presentaci6n fue una masa indolora. L a cirugia constituy6 el tratamiento definitivo en la totalidad de los casos. Sin embargo, se utiliz6 radioterapia o quimioterapia adyuvantes por enfermedad residual en casi la rnitad (13 pacientes). El seguimiento vari6 entre cinco y 35

Invited Commentary Thomas A. Mustoe, M.D. Department of Surgery, Northwestern University School of Medicine, Chicago, Illinois, U.S.A.

In North America, Memorial Sloan-Kettering Cancer Center (along with M.D. Anderson Hospital and Princess Margaret blospitai) stand alone in their reporting of sufficient numbers of Uncommon tumors of the head and neck in careful retrospective

1015

afios. Doce de los 28 pacientes (43%) estaban vivos y libres de enfermedad a los 5 o m~s afios de seguimiento. L a causa de muerte m~s comtin fue !a extensi6n incontrolada de la enfermedad local (88%). Sin embargo, s61o 3 de 10 pacientes no previamente tratados desarrollaron recurrencia local. E1 intervalo medio de sobrevida entre Ia primera recurrencia y ta muerte fue de 25 meses (rango 6 a 70 meses) para et grupo total. M a r # n e s positivos dieron como resultado recurrencia local y eventualmente muerte en 8 de 12 pacientes. Met,'istasis distantes fueron detectadas en 18% de los casos, siendo m~is frecuente en los pulmones. El an~ilisis del subgrupo de pacientes tratados m~s recientemente, en el periodo 1975-1985, revel6 que 7 de 9 pacientes (78%) se hallaban vivos y libres de enfermedad a los 5 afios. References

1. Dahlin, D.C.: Bone Tumors: General Aspects and Data on 8542 cases, 4th edition, Springfield, Illinois, 1986, pp. 227-259 2. McKenna, R.J., Schwin, C.P., Soong, K,Y., Higenbotham, N.C.: Sarcomata of the osteogenic series. J. Bone Joint Surg. 48A: i, 1966 3. Burkey, B.B., Hoffman, H.T., Baker, S.R., Thornton, A.F., McClatchy, K.D.: Chondrosarcoma of the head and neck. Laryngoscope 100:1301, 1990 4. Batsakis, J.G., Solomon, A.R., Rice, D.H.: The pathology of head and neck tumors: Neoplasms of cartilage, bone, and notochord, Part 7. Head Neck Surg. 3:43, 1980 5. Fu, Y.S., Perzin, K.H.: Non-epithelial tumors of the nasal cavity, paranasal insues, and nasopharynx: A clinicopathologic study. 111. Cartilaginous tumors. Cancer 34:453, 1974 6. Aden, M., Tollefsen, H.R., Huvos, A.G., Marcove, R.C.: Chondrosarcoma of the head and neck. Am. J. Surg. 120:456, 1970 7. Finn, D.C., Goepfert, H., Batsakis, J.G.: Chondrosarcoma of the head and neck. Laryngoscope 94:1539, 1984 8. Chou, P., Mehta, S., Conzalez-Crussi, F.: Chondrosarcoma of the head in children. Pediatr. Pathol. •0:945, 1990 9. Hyams, V.J., Rabuzzi, D.D.: Cartilaginous tumors of the larynx. Laryngoscope 80:755, 1970 10. Gorenstein, A., Neel, H.B., Weiland, L.H., Devine, K.D.: Sarcoma of the larynx. Arch. Otolaryngol. •06:8, 1980 11. Hertzanu, Y., Mendelsohn, D.B., Davidge-Pitts, K., Cohen, M.: Chondrosarcoma of the head and neck: The value of computed tomography. J. Surg. Oncol. 28:97, 1985 12. Harwood, A.R., Krejbich, J.I., Fornasier, V.L.: Radiotherapy of chondrosarcoma of bone. Cancer 45:2769, 1980 13. Krochak, R., Harwood, A., Cummings, B., Quirt, I.C.: Results of radical radiation for chondrosarcoma of bone. Radiother. Oncol. 1:109, 1983 14. McNaney, D., Lindberg, R., Ayala, A., Barkley, H.T., Hussey, D.H.: Fifteen year radiotherapy experience with chondrosarcoma of bone. Inter. J. Radiat. Oncol. Biol. Phys. 8:197, 1982

analyses to allow meaningful analysis. Senior author Shah, recent President of the Society of Head and N e c k Surgeons, and his two co-authors, Ruark and Sch!ehaider, report on a 35 year analysis of chondrosarcomas of the head and neck at Memorial Sloan-Kettering, and only find 28 tumors, a statement on their rarity. The average head and neck surgeon might encounter one or two in his/her lifetime. As in many retrospective analyses, this paper is basically a report of previous empiric therapy and therefore any comparison of therapies would be of limited value. The prolonged 35 year time period of the study accentuates this problem. However, this report represents the largest number of head and neck