Abstract
Approximately 15% of soft-tissue sarcomas occur in head and neck area and sarcoma should be considered in the differential diagnosis of any tumor mass in this anatomic region, especially in younger patient.
This is a retrospective study that reviewed all head and neck sarcoma cased registered at Imam Khomeini Hospital and Institute of Cancer during the period of 1987-1996. Using medical records, relevant information including demographic, medical and risk factors were collected. Analysis of data on 40 eligible cases revealed age range of 31-40, male sex and living in urban areas as risk factors. Osteosarcoma was the most frequent pathology and painless mass was the most frequent sign. In 80% of the cases the tumors were primary and in 42.5% the disease recurred after treatment.
Key words: Sarcoma; Head and neck; Soft tissue sarcoma; Tehran
Introduction
Cancers are the second most cause of death after cardiovascular disease (1). A very important mortality cause is delay in diagnosis and treatment. Soft tissue sarcomas include a large expect rum of malignant tumors that can origin from any mesenchymal tissue in any anatomic organ. Sarcomas are uncommon but include various histologic types (2). In head and neck area sarcomas make less than 1% of all cancers. On the other hand 15% of soft tissue sarcomas and 1.5% of osteosarcomas are located in head and neck. Although there is a clear difference in their incidence rate in different sites for example synovial sarcoma is rare in head and neck, all types of sarcomas could occur in head and neck. Head and neck sarcoma most commonly occur in adults. Average age is 40-50 and male to female ratio is 1-2/1 (3). In most cases there isn't any certain etiology; however radiation exposure and trauma are the most important related factors according to different references (2, 3). 50% of sarcomas of head and neck are low grade. The tumor does local invasion. Lymph node metastasis is rare. Hematological metastasis is less than sarcomas of other sites. In general metastasis happens in 1/3 of cases which is mostly pulmonary. The most important diagnostic methods are CT scan and MRI.
In local tumors with no evidence of metastasis, treatment is tumor resection with enough resection of clean tissue. Recurrence can happen which is due to insufficient tissue resection.
Site of the tumor, patient's age, tumor invasion, grade, histologic type and treatment method are factors that are important regarding the prognosis. The roles of these factors are different in different types of sarcoma.
Method and material
This study was a retrospective study using the files of the admitted patients, who were admitted in Imam Khomeiny Hospital and Institute of Cancer between 1987 and 1996. The files were screened according to the pathologic results. The main objectives of the study included finding the incident rate of head and neck sarcomas, clinical manifestations of different types of sarcoma and the predisposing factors related to head and neck sarcoma. The fallowing factors were considered as possible predisposing factors: age, sex, occupation, residence, smoking, family history of cancer, radiation exposure, and trauma.
Factors that could affect the patient's prognosis and survival including the tumor's site, the disease expansion at the time of diagnosis, metastasis, and recurrence were also studied. The retrieved information was organized in pre- designed forms which were analysed consequently.
In some cases pathologic result included spindle cells. We couldn't put these under a specific pathology because spindle cells are found in many types of sarcoma. On the other hand we couldn't exclude them from the study otherwise we would have to exclude a large number of the cases. Therefore we studied these cases as a separate group under the title of Spindle Cell Sarcoma.
Findings
We studied 40 patients in this study from which 15 were admitted in Imam Khomeiny Hospital and 25 were cases of the Institute of Cancer.
Patients were from 0 to 69 years old (average 31.95 years). (Table 1)
The frequency of different types of sarcoma is shown in table 2. In terms of sex distribution, there were 28 men (70%) and 12 women (30%). The frequency of types of sarcoma in two sex groups is represented in table 3.
Table1: Age frequency of patients with head and neck sarcoma, admitted in Imam Khomeiny Hospital Complex. 1987-1996
|
Age group (years) |
frequency |
Percentage |
|
0-10 |
4 |
10 |
|
11-20 |
7 |
17.5 |
|
21-30 |
8 |
20 |
|
31-40 |
12 |
30 |
|
41-50 |
4 |
10 |
|
51-60 |
3 |
7.5 |
|
6169 |
2 |
5 |
|
Total |
40 |
100 |
Table 2: Frequency of different pathologic types of head and neck sarcoma, Imam Khomeiny Hospital Complex 1987-1996
|
Pathology type |
Spindle Cell S. |
Rabdomyosarcoma |
Osteosarcoma |
Chondrosarcoma |
Fibro sarcoma |
Others |
|
percentage |
20 |
22.5 |
25 |
10 |
7.5 |
15 |
Others: Pleomorphic, Synovial cell, undifferentiated, dermato fibro sarcoma
Table3: Frequency of types of sarcoma in two different sex groups, Imam Khomeiny Hospital Complex
|
Pathology type |
Spindle Cell S. |
Rabdomyosarcoma |
Osteosarcoma |
Chondrosarcoma |
Fibro sarcoma |
Others* |
|
Male% |
37.5 |
77.8 |
70 |
100 |
66.7 |
83.3 |
|
Female% |
62.5 |
22.2 |
30 |
0 |
33.3 |
16.7 |
* Others: Pleomorphic, Synovial cell, undifferentiated, dermato fibro sarcoma
31 of the patients were city residents (77.5%) and 19 the patients lived in rural area (22.5%). 85% of the patients had lived in a city for more than 10 years.
In terms of occupation, the most of the patients were office worker, house wife or student. The next frequent jobs were construction, agricultural or industrial respectively.
There wasn't any information about radiation exposure or trauma history in any of the patient's files.
19 patients were cigarette smokers (47.5%); in 6 of them there the length of smoking was apparent, which was 7-50 years (average: 27.3). There wasn't any information regarding amount of smoking. Family history of cancer was negative in 92.5% of the patients. The frequency of signs and symptoms are shown in tables 4 and 5 respectively. The anatomic site of the tumor is represented in table 6.
Table 4: Frequency of signs in head and neck sarcomas, Imam Khomeiny Hospital Complex, 1987-1996
|
Pathology type Sign (%) |
Spindle Cell S. |
Rabdomyosarcoma |
Osteosarcoma |
Chondrosarcoma |
Fibro sarcoma |
Others * |
|
Mass |
80 |
53.3 |
76.9 |
83.3 |
75 |
62.5 |
|
Nerve palsy |
10 |
0 |
0 |
0 |
0 |
0 |
|
LAP |
0 |
20 |
7.7 |
16.7 |
0 |
0 |
|
Others** |
10 |
26.7 |
15.4 |
0 |
25 |
37.5 |
* Others: Pleomorphic, Synovial cell, undifferentiated, dermato fibro sarcoma
** Others: ulcer, trismus, proptosis, malocclusion …
Table 5: Frequency of symptoms in head and neck sarcomas, Imam Khomeiny Hospital Complex, 1987-1996
|
Pathology type Symptom (%) |
Spindle Cell S. |
Rabdomyosarcoma |
Osteosarcoma |
Chondrosarcoma |
Fibro sarcoma |
Others * |
|
Mass |
70 |
50 |
71.4 |
66.7 |
100 |
66.7 |
|
Weight loss |
0 |
0 |
7.1 |
0 |
0 |
0 |
|
Fever |
0 |
0 |
16.7 |
0 |
0 |
0 |
|
Pain |
20 |
21.4 |
0 |
0 |
0 |
11.1 |
|
Others** |
10 |
28.6 |
21.4 |
16.7 |
0 |
22.2 |
* Others: Pleomorphic, Synovial cell, undifferentiated, dreamt fibro sarcoma
**Others: hemorrhage, dysphagia, malocclusion, respiratory distress, hearing loss
Table 6: Frequency of the site of involvement of head and neck sarcomas, Imam Khomeiny Hospital Complex, 1987-1996
|
Pathology type Site (%) |
Spindle Cell S. |
Rabdomyosarcoma |
Osteosarcoma |
Chondrosarcoma |
Fibro sarcoma |
Others * |
|
Neck |
12.5 |
44.4 |
0 |
0 |
33.3 |
0 |
|
Maxilla |
12.5 |
11.1 |
40 |
0 |
0 |
0 |
|
Mandible |
25 |
0 |
50 |
100 |
33.3 |
16.7 |
|
Scalp |
37.5 |
11.1 |
10 |
0 |
0 |
33.3 |
|
Others ** |
12.5 |
33.3 |
0 |
0 |
33.3 |
50 |
* Others: Pleomorphic, Synovial cell, undifferentiated, dermato fibro sarcoma
**Others: Larynx, Oral Cavity, Cheek, Parotid, Paranasal sinuses, EOM
Table 7: Frequency of different types of head and neck sarcoma in different age groups, Imam Khomeiny Hospital Complex, 1987-1996
|
Pathology |
Spindle Cell S. |
Rabdomyosarcoma |
Osteosarcoma |
Chondrosarcoma |
Fibro sarcoma |
Others * |
Total |
|
0-10 % |
0 |
33.3 |
0 |
0 |
0 |
16.7 |
|
|
11-20 % |
37.5 |
0 |
10 |
0 |
3303 |
33.3 |
|
|
21-30 % |
12.5 |
11.1 |
40 |
25 |
0 |
16.7 |
|
|
31-40 % |
37.5 |
0 |
40 |
75 |
33.3 |
16.7 |
|
|
41-50 % |
12.5 |
22.2 |
0 |
0 |
33.3 |
0 |
|
|
51-60 % |
0 |
22.2 |
0 |
0 |
0 |
16.7 |
|
|
61-69 % |
0 |
11.1 |
10 |
0 |
0 |
0 |
|
|
Total % |
100 |
100 |
100 |
100 |
100 |
100 |
100 |
* Others: Pleomorphic, Synovial cell, undifferentiated, dermato fibro sarcoma
At the time of admission, 32 patients were in primary stage (80%), 3 cases had local lymph node invasion (lymphadenopathy) (7.5%), 2 cases of metastasis (both to lung) (5%) and 3 had recurrence of the disease (7.5%).
29 of the patients didn't undergo any imaging diagnostic method (or at least there wasn't any report on their files), 8 patients (20%) had a CT scan report. MRI was done for 1 (2.5%) patient. Ultrasonography, plain radiography and radio nuclear scan were used for 1 patient each.
Recurrence had happened in 23 (57.5%) of the patients, from which 14 cases were in the original site, 2 cases in another site in head and neck and 1 case distant metastasis. 17 (42.5%) patients didn't have any recurrence.
The most common age range in which recurrence had happened was 31-40 (41.2% of recurrence cases) and there wasn't any recurrence in age range 51-60. The frequency of recurrence is shown in table 8.
Table 8: Frequency of recurrence in head and neck sarcomas, Imam Khomeiny Hospital Complex, 1987-1996
|
Recurrence
Sarcoma type |
Recurrence happened |
No recurrence |
Total | |||
|
frequency |
% |
frequency |
% |
frequency |
% | |
|
Osteosarcoma |
4 |
40 |
6 |
60 |
10 |
100 |
|
Rabdomyosarcoma |
2 |
22 |
7 |
78 |
9 |
100 |
|
Chondrosarcoma |
3 |
75 |
1 |
25 |
4 |
100 |
|
Fibro sarcoma |
2 |
66.6 |
1 |
33.3 |
3 |
100 |
|
Spindle cell sarcoma |
3 |
37.5 |
5 |
62.5 |
8 |
100 |
|
Others * |
3 |
50 |
3 |
50 |
6 |
100 |
|
Total |
17 |
|
23 |
|
40 |
100 |
* Others: polymorphic sarcoma, synovial sarcoma, dermatofibro sarcoma and undifferentiated sarcoma
Discussion
Head and neck sarcoma is rare. A study (1980-1995) only 15 cases of sarcoma (not including rhabdomyo sarcoma) was reported (5). In our study there were only 40 cases of head and neck sarcoma in 10 years in one of the referral centers of the country, which is in favor of the results of other studies.
The most common pathology was osteosarcoma and then rhabdomyo sarcoma. The last is the most common soft tissue sarcoma in under 15 years old and one of the most common sarcomas in adults and teenagers (2).
Angiosarcoma, neurosarcoma and liomyo sarcoma are the only sarcomas which are more common in females. The rest of the sarcomas are more common in males (2, 3). In our study only spindle cell sarcoma was more common in female.
The most common age range was 31-40. Considering the fact that osteosarcoma of jaw bones are more common in age 20-30 (3), and that it is also the most common pathologic finding in this study, it seems that including this sarcoma in this study has brought down the age range by one decade, comparing to what other references have mentioned as the age range of soft tissue sarcoma of head and neck.
According to table 7, the age distribution of different types of sarcoma in this study is the same as the other references (2, 3, 4).
The age distribution of spindle cell sarcoma is the same as chondrosarcoma and fibro sarcoma.
Clinical manifestations of rhabdomyosarcoma differ according to its site of involvement. It's most common site is orbital cavity; therefore exophthalmia and double vision are frequent presenting signs. It also can present with hoarseness and dysphagia and respiratory difficulty, dependent on the site of involvement.
Osteosarcoma presents with a mass which is accompanied by pain and looseness of teeth; but chondrosarcoma is a painless mass with no loosened teeth. In fibro sarcoma mass which is painless in 1/3 of the cases and weight loss in advance illness, are frequent signs (2, 3, 4).
In our study the most common sign and symptom in all types was a mass. Fever and weight loss were only associated with some cases of osteosarcoma. The tumor mass was painless in osteosarcoma, chondrosarcoma and fibro sarcoma. Lymphadenopathy was present in osteosarcoma, chondrosarcoma and rhabdomyosarcoma. Facial nerve palsy was positive in 10% of spindle cell sarcoma. (Rhabdomyosarcoma of middle ear can cause facial nerve palsy (4).)
Place of residence was studied as a possible risk factor. 77.5% of our patients lived in a city so it was marked as a risk factor. However, since the study was done in Tehran and the two centers located in Tehran it should be considered that urban residents might have had easier access to them which might have affected the results.
60% of the patients were office workers, students or house wives. None of these has any significant environmental factor. Therefore occupation wasn't labeled as a risk factor.
The most common site of involvement was mandible for osteosarcoma, neck for rhabdomyosarcoma, neck and mandible in fibro sarcoma and only mandible for chondrosarcoma. Some anatomic sites were involved in couple of cases: larynx in synovial sarcoma, orbit in rhabdomyosarcoma, oral cavity in fibro sarcoma, cheeks in rhabdomyosarcoma, parotid gland in dermato fibro sarcoma and spindle cell sarcoma, sinuses in rhabdomyosarcoma and undifferentiated sarcoma. These were studied as one single group.
There wasn't any case involving ears, pharynx or thyroid gland. Sarcomas are rare in these areas anyway. In 1996 one case of fibro sarcoma of thyroid was reported in a patient who had survived the Chernobyl accident (6). In pharyngeal area sarcomas are very rare due to its rich mesenchymal tissue. They have reported only 6 cases of sarcoma of the hypopharynx between 1973 and 1987 (7). Most of the sarcomas in laryngeal area are chondrosarcoma and fibro sarcoma (4). In 1998 a case of parotid gland synovial sarcoma was reported (8).
Prognosis of our patients is promising, considering 80% of the cases haven't had any evidence of local or distant metastasis at the time of diagnosis. There are 2 cases of lung metastasis that have to be fallowed in order to determine the extent of the metastasis. In the cases of chondrosarcoma, rhabdomyosarcoma and osteosarcoma local metastasis to regional lymph nodes were identified.
Unfortunately CT scan and MRI weren't used sufficiently in the patients. (Only 8 cases had undergone CT scanning.) These two imaging techniques are very voluble in evaluating the existence and extent of the tumor (2,4) and should be considered more efficiently in future.
There was a 42.5% recurrence rate in studied cases which showed the importance of careful surgery with more clean tissue resection. The most recurrent sarcomas were chondrosarcoma and fibro sarcoma, whereas rhabdomyosarcoma had the least recurrence rate.
Recommendations
Most of the findings of this study are similar to those of the other references including the low incidence of sarcoma in head and neck, the high incidence of osteosarcoma and rhabdomyosarcoma among head and neck sarcomas, age and sex distributions, the most common signs and symptoms and anatomic sites of involvement. Similar to other studies there isn't any etiologic factors recognized for sarcoma, however in our study in our study sex, age, and place of residence showed considerable relationship with the incident of the disease.
What seems to be important to remember is a careful evaluation for any mass in head and neck area. It will guarantee the diagnosis of the disease in its early stages. It is also important to use CT scan and MRI methods more sufficiently in order to achieve an early diagnosis, in time operation and less rate of recurrence of the disease.
References:
Grayson, W; Nayler, SJ; Jena, GP. Synovial sarcoma of the parotid gland. A case report with clinicopathological analysis and review of the literature. South Africa Journal of Surgery. 1998. 36 (1):32-5.
Published in: The Journal of Faculty of Medicine, 57th year, Number 3, 1999
By: E. Razmpa, MD., M. Mohagheghy, MD., P. Mansoory, MD