Annals of Surgical Oncology Cite Track
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
10.1245/ASO.2004.03.006
Annals of Surgical Oncology 11:619-628 (2004)
© 2004 Society of Surgical Oncology
This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Bentz, B. G.
Right arrow Articles by Kraus, D.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Bentz, B. G.
Right arrow Articles by Kraus, D.

ORIGINAL ARTICLES

Head and Neck Soft Tissue Sarcomas: A Multivariate Analysis of Outcomes

Brandon G. Bentz, MD, Bhuvanesh Singh, MD, James Woodruff, MD, Murray Brennan, MD, Jatin P. Shah, MD and Dennis Kraus, MD

From the Department of Surgery, Division of Otolaryngology-Head & Neck Surgery, University of Utah School of Medicine and the Huntsman Cancer Institute, Salt Lake City, Utah (BGB); Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York (BS, MB, JPS, DK); and Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York (JW).

Correspondence: Address correspondence and reprint requests to: Dennis Kraus, MD, Head & Neck Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Ave., New York, NY 10021; Fax: 212-717-3302; E-mail: krausd{at}mskcc.org


   ABSTRACT
TOP
 ABSTRACT
INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Background: Soft tissue sarcomas of the head and neck region represent a rare group of tumors of which a limited number of published individual- and institution-based experiences exist.

Methods: We performed an analysis of head and neck sarcoma patients identified from our institution between 1973 and 1999. Exclusion criteria included pediatric rhabdomyosarcomas, sarcomas of the neuromeningeal axis or non–head and neck primary disease sites, and bone sarcomas. All cases underwent pathologic re-review before statistical analysis.

Results: After pathologic review, 111 head and neck sarcoma patients remained (mean age, 47 ± 20 years). The median duration of follow-up was 51 months; the actuarial 5-year relapse-free, disease-specific, and overall survivals were 55%, 52%, and 44%, respectively. Forty-six percent remained free of recurrence at the most recent follow-up, and the most common site of recurrence was local followed by distant sites. By multivariate analysis, size and grade significantly influenced relapse-free, disease-specific, and overall survivals, whereas margin status additionally influenced relapse-free survival. Subset analysis of the fibrosarcoma/malignant fibrous histiocytoma and desmoid/dermatofibrosarcoma protuberans histologies was undertaken.

Conclusions: Size >5 cm and high-grade histology are considered poor prognostic indicators. Patients with either of these characteristics should be considered for adjuvant trials.

Key Words: Head and neck • Sarcoma • Multivariate analysis • Retrospective • Single institution


   INTRODUCTION
TOP
 ABSTRACT
 INTRODUCTION
MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Sarcomas of the head and neck region are rare. Of the patients admitted and treated for a soft tissue sarcoma from 1973 to 1999 in the Memorial Sloan-Kettering Cancer Center database, 5% of tumors were located in the head and neck region. The rare nature of these tumors has resulted in a limited number of published individual- or institution-based series in the recent literature. Clinicians must therefore make treatment decisions based on the insight gained from the collective experience of only a few published series. These decisions are difficult as a result of several common features: (1) disagreement among clinicians regarding the utility of current classification or grading schemes, (2) inconsistencies among pathologists as to definitions, (3) benign lesions or unrelated tumors that may appear similar to soft tissue sarcomas, and (4) changes in the diagnosis as a consequence of molecular analysis. For these reasons, rigorous histological review is considered essential for meaningful analysis.1

Common means used by published series to compensate for the rare nature of these tumors and to increase study numbers to achieve a level meaningful for statistical analysis have been the accrual of patients across a number of decades and the inclusion of such entities as pediatric sarcomas, osteosarcomas, or chondrosarcomas, which are known to have very different biological behavior. Our study is a review of the most recent experience at Memorial Sloan-Kettering Cancer Center with adult soft tissue sarcomas of the head and neck. The resulting group of patients was analyzed by multivariate analysis to define specific prognostic features that predict outcome and can guide treatment approaches.


   MATERIALS AND METHODS
TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
RESULTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Patient Data
We undertook a review of patients who had undergone inpatient treatment for a soft tissue sarcoma of the head and neck region at Memorial Sloan-Kettering Cancer Center between 1973 and 1999. This was performed as a retrospective chart review of patients between 1973 and 1981 and a review of the prospectively collected patient database from 1982 to 1999.

Pathologic Review
All pathologic slides were reviewed to confirm the diagnosis. Immunohistochemical studies were performed to clarify the exact subclassification of sarcoma on the basis of the most likely histopathologic differential diagnosis, as necessary. Histological grading was determined on the basis of the two-grade system of Hajdu.2 The grade of an individual lesion was based on the number of mitoses per high-power field, the amount of nuclear pleomorphism, and the presence or absence of necrosis. For the purposes of this study, angiosarcomas were classified as high-grade lesions, whereas dermatofibrosarcoma protuberans (DFSP) and desmoid tumors were classified as low-grade lesions.

Because of the distinct clinical behavior of adult patients with rhabdomyosarcoma (nine cases), these were included in analysis. With respect to the entire study population analysis, the diagnoses of desmoid tumors (n = 9) and DFSP (n = 13) were included in the analysis of the relapse-free survival (RFS) but were subsequently excluded from analysis of disease-specific survival (DSS) and overall survival (OS) because of the universally favorable outcome. One case of DFSP with fibrosarcomatous change was included in the DSS and OS analysis because of the ability of this subclassification to metastasize. Chondrosarcoma, osteogenic sarcoma, and rhabdomyosarcoma in the pediatric population were excluded from analysis because of the dramatically different biological behavior of these tumors. Additionally, excluded from analysis were sarcomas of the neuromeningeal axis or non–head and neck sites with metastasis to the head and neck region.

Because of the universally favorable cause-specific outcomes of desmoid tumors and DFSP, analysis for factors affecting disease recurrence was undertaken separately for this combined subset. Additionally, because the clinical courses of the fibrosarcoma and malignant fibrous histiocytoma (MFH) categories are similar and the current pathologic trend is to avoid the diagnosis of MFH,3 subset analysis of factors affecting outcome for this combined group was undertaken separately.

A total of 180 patients who met the inclusion criteria were identified from our patient database and were subjected to a detailed histopathologic review by the reference pathologist (J.W.). Clinical tumor, treatment, and outcome data were collected and analyzed. After pathologic review, 111 cases of head and neck soft tissue sarcoma form the basis of this report. The 69 cases were excluded from analysis because of a lack of available pathologic tissue (39 cases; 57% of excluded cases), outside pathologic consultation in which pathology slides were returned to the parent institution (8 cases; 12%), a misdiagnosed benign (7 cases; 10%) or nonsarcomatous (6 cases; 9%) malignant pathology, inability to make the definitive immunohistochemical diagnosis (5 cases; 7%), or a sarcoma of a primary disease site other than the head and neck region (4 cases; 6%).

Statistical Analysis
RFS was defined as the time from initial treatment to the diagnosis of a local, regional, or distant recurrence. Patients with persistent disease were designated as having an RFS of 0 months. Patients who presented with distant metastasis did not have a relapse-free interval and were excluded from initial RFS analysis. DSS was defined as the time in months from the initial diagnosis to death as a result of disease or treatment-related causes, whereas OS was defined as time to death from any cause.

Statistical analysis was performed with JMP 4.0 statistical software (SAS Institute, Inc., Cary, NC). Statistical comparison of RFS, DSS, and OS was determined according to the method of Kaplan and Meier.4 The log-rank test was used for univariate survival comparisons.5 Multivariate analysis of factors affecting survival was performed according to the Cox proportional hazard model.6


   RESULTS
TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Patient Demographics
The mean age of the entire study population was 47 ± 20 years. There were nearly equal number of men (51%) and women (49%). The median duration of follow-up for the entire group was 51 months. After elimination of those patients who presented with distant metastatic spread (n = 37), 74 patients remained for analysis of RFS. The mean age of this group was 49 ± 12 years, with a median follow-up duration of 56 months. Elimination of the cases of DFSP and desmoid tumors from analysis of DSS and OS left 89 cases. The mean age of this group was also 47 ± 20 years. The median duration of follow-up for our adjusted group was 44 months.

Tumor Characteristics at Presentation
Site of tumor origin was grouped into the following anatomical locations (Fig. 1): face (n = 23), scalp (n = 18), neck (n = 35), oral cavity (n = 9), pharynx (n = 1), sinus (n = 8), nasopharynx (n = 5), orbit (n = 2), and other (n = 5). In an additional five cases, the exact site of origin was indeterminate after initial treatment at an outside institution, and these cases were excluded from analysis of the effect of site on outcomes. For outcomes analysis, the presenting anatomical locations were grouped into face and scalp (n = 41), neck (n = 35), and deep facial cavities and viscera (n = 25).



View larger version (25K):
[in this window]
[in a new window]
  		FIG. 1. Location of the soft tissue sarcomas at presentation. Note that the most common site of presentation was the neck, followed by the face.

 
After pathologic re-review, the most common benign lesion to be misdiagnosed as a sarcoma was a spindle cell lipoma (four cases), followed by two cases of fasciitis and one case of a benign lymphoproliferative lesion. Malignant lesions that were misdiagnosed as a sarcoma included five carcinoma cases (three could not be classified further, and the other two were a basal cell carcinoma and a sarcomatoid carcinoma) and one melanoma. A total of 37 sarcoma cases (33%) had their sarcoma subtype reclassified. Of these cases, 28 (76%) either started with or were reclassified as either an MFH or fibrosarcoma. The most common histopathologic diagnosis within our group was fibrosarcoma, followed by angiosarcoma and various other diagnoses. Figure 2 depicts the pathologic classifications within our study group. Sixty-two patients (56%) had a high-grade sarcoma, whereas 49 patients (44%) had low-grade lesions. Seventy-six patients (72%) had tumors <=5 cm in greatest diameter, whereas 29 patients (28%) had tumors >5 cm in greatest diameter, and tumor size was not documented in 6 patients.



View larger version (39K):
[in this window]
[in a new window]
  		FIG. 2. Proportion of the present series with each individual histopathologic diagnosis. Numbers in parentheses represent the number of patients with each diagnosis and the percentage of the entire series. DFSP, dermatofibrosarcoma protuberans; MFH, malignant fibrous histiocytoma; Rhabdo, rhabdomyosarcoma; HPC, hemangiopericytoma.

 
Patient Characteristics at Presentation
At presentation, 56 patients were asymptomatic except for an enlarging mass, whereas 55 patients reported pain or neurological dysfunction at presentation. Overall, 55 patients presented with previously untreated lesions, whereas 56 patients had undergone prior treatment. The margin status on prior treatment was recorded in only 19 patients (34%); 7 had negative margins, 6 had microscopically positive margins, and 6 had grossly positive margins.

Treatment
The primary mode of treatment was surgery: 96 patients (87%) underwent surgical intervention. The other 15 patients were treated by nonsurgical means or the exact nature of their treatment modality was indeterminate after primary treatment at an outside institution. Surgical intervention consisted of gross complete excision in 86 patients (90%), subtotal excision in 3 patients (3%), enucleation in 1 patient (1%), and biopsy only in 6 patients (6%). Microscopic margin status was negative in 50 patients (52%) and positive in 40 patients (42%), and 6 (6%) patients lacked documentation of margin status.

Seventy-four patients (77%) received surgery alone, 15 patients (16%) underwent surgery plus adjuvant radiotherapy, and 7 patients (7%) had surgery plus chemotherapy. Five patients underwent chemotherapy and radiotherapy without surgery, one had chemotherapy alone, and in nine patients the exact treatment modality was not recorded. The 20 patients undergoing radiotherapy consisted of 19 patients receiving external beam radiotherapy with a mean dose of 5415 cGy (range, 2000–6600 cGy) and 1 patient receiving brachytherapy.

After initial treatment, 15 patients (14%) experienced a complication in the posttreatment setting. Three patients experienced an infectious or septic complication, two patients each had a postoperative hematoma or a deep venous thrombosis, and one patient each had pancreatitis, hematochezia, thrombocytopenia, mucositis, and pneumonia.

A total of 49 patients underwent treatment at our institution for recurrent sarcoma. For these recurrent tumors, 27 patients (55%) underwent surgery plus radiotherapy, 7 patients (14.3%) each had surgery plus chemotherapy and radiotherapy or chemotherapy as a single modality of treatment, 3 patients (6.1%) each had radiotherapy alone or surgery plus chemotherapy, and 2 patients (4.9%) had chemotherapy and radiotherapy.

Outcome
RFS, DSS, and OS were the worst for angiosarcomas—17%, 24%, and 20%, respectively (Table 1). Aside from desmoid and DFSP tumors, the best prognosis was found for hemangiopericytomas, with a 100%, 83%, and 71% RFS, DSS, and OS, respectively (Table 1). The other subclassifications of sarcomas fell between these two extremes. Those who experienced a complication of their primary treatment modality had a significantly worse RFS (P = .02), DSS (P = .005), and OS (P = .006) on univariate analysis. These results probably reflect the high proportion of high-grade lesions (14 patients, or 82.4% of those experiencing complications of their treatment) in this group.


View this table:
[in this window]
[in a new window]
  		TABLE 1. Relapse-free (RFS), disease-specific (DSS), and overall survival (OS) with respect to histopathologic classification
 
Of the 74 patients who presented without distant metastasis, 34 patients (46%) did not experience a recurrence and were free of disease at final follow-up. If the patient experienced a recurrence, the most common location of this recurrence was local (30 patients; 41% of the recurrent cases). Within this group of patients, 24 had a local recurrence as their sole site of recurrence, 2 had recurrence at local and regional sites, 3 had recurrence locally and at distant sites, and 1 patient had local, regional, and distant recurrence. Distant recurrences accounted for 11 cases, or 15% of all relapses. Of these patients, seven had a distant recurrence as the sole site, three patients had local and distant recurrences, and one patient had local, regional, and distant recurrence. Six patients had a regional recurrence, of which three had a regional recurrence alone, two experienced a local and regional recurrence, and, as stated previously, one experienced a local, regional, and distant recurrence.

Fifteen patient and tumor characteristics were evaluated with respect to their effect on survival by univariate analysis (Table 2). After exclusion of patients who presented with distant metastasis, analysis found that the age of the patient (P = .005), tumor size (P = .009), type of surgery (P = .02), margin status (P = .006), grade of the lesion (P = .0003), bone involvement (P = .008), and treatment complications (P = .02) all significantly affected RFS (Table 2). Excluding DFSP without fibrosarcomatous change and desmoids from univariate analysis of DSS found age (P = .03), size (P = .04), tumor grade (P = .0005), and treatment complications (P = .004) to significantly affect DSS (Table 2). Univariate analysis of OS in this group found that the patient’s age (P = .005), treatment modality (P = .02), tumor grade (P = .003), and occurrence of treatment complications (P = .006) affected OS.


View this table:
[in this window]
[in a new window]
  		TABLE 2. Univariate analysis of factors affecting relapse-free (RFS), disease-specific (DSS), and overall survival (OS)
 
After grouping the location of the primary lesion, anatomical location was found by univariate analysis to significantly affect the 5-year RFS (P = .04). Lesions of the face and scalp were found to have the best 5-year RFS (72%), whereas lesions of the deep cavities and viscera faired much worse, with a 5-year RFS of 38%. The effect on recurrence did not maintain its significant influence on RFS after evaluation by multivariate analysis.

The 5-year RFS for the group of 74 patients who did not present with distant metastases was 55%, whereas the DSS at 5 years for the group that excluded DFSP and desmoids was 52% and the OS was 44% (Fig. 3). The actuarial 5-year DSS for patients who underwent treatment for their recurrent disease was 51%. Multivariate analysis based on the four factors significant by univariate analysis found that the size and grade of the lesion and the margin status significantly influenced RFS in these patients. The size of the tumor and the tumor grade had a significant prognostic effect on DSS and OS on the basis of multivariate analysis (Table 3).



View larger version (21K):
[in this window]
[in a new window]
  		FIG. 3. Relapse-free (RFS), disease-specific (DSS), and overall survival (OS) by Kaplan-Meier survival curves for the patient population after exclusion of patients who presented with distant metastases (RFS) and desmoid or dermatofibrosarcoma protuberans tumors (DSS and OS).

 

View this table:
[in this window]
[in a new window]
  		TABLE 3. Multivariate analysis of factors affecting relapse-free, disease-specific, and overall survival
 
Desmoid/DFSP Subset Analysis
Twenty-three patients had a diagnosis of head and neck desmoid or DFSP tumors, of which 9 (39%) were male and 14 (61%) were female. The mean age of this subset population was 45 ± 19 years. The median duration of follow-up for this subset was 93 months.

Nine patients were diagnosed with desmoid tumors, whereas 14 patients had DFSP tumors. All patients presented with an enlarging mass, whereas two patients also had some neurological dysfunction. Six patients had a tumor of the face, 3 patients had scalp tumors, 12 tumors presented in the neck, and 2 were found in the oral cavity. Seventeen patients had tumors that were <=5 cm in diameter, whereas six patients had tumors >5 cm. All patients underwent surgical removal of their tumors, and two patients underwent postoperative radiotherapy. These two patients had undergone prior surgical excision at an outside institution.

Survival analysis for this subset demonstrated a 5-year DSS of 100%, whereas the 5-year RFS and OS were 81% and 80%, respectively. Univariate analysis of the 14 patient- and tumor-related factors found that only tumor size >5 cm approached a significant effect on RFS (P = .06). No other factors were found to affect recurrence.

Fibrosarcoma/MFH Subset Analysis
Thirty-four patients had the diagnosis of either fibrosarcoma or MFH, of which 19 (56%) were male. The mean age of this subset population was 47 ± 20 years. The median duration of follow-up was 47 months.

Twenty-two patients had the diagnosis of fibrosarcoma, of which 13 (59%) were low-grade lesions and 9 (41%) were high-grade histology. The other 12 patients were diagnosed with MFH, of which 3 (25%) were low-grade and 9 (75%) were high-grade histology. Four patients presented with pain, whereas five patients presented with neurological dysfunction. Eight tumors were located in the face, four in the scalp, eight in the neck, two in the oral cavity, four in the sinus, three in the nasopharynx, one in the orbit, and two at other sites. In an additional two cases, the exact site was indeterminate. Twenty-three patients (68%) had tumors <=5 cm, eight (24%) had tumors >5 cm, and three did not have the size of their tumor recorded.

Almost the entire subset population had complete surgical excision (31 patients; 91%), whereas only 1 patient each had a subtotal excision and an enucleation. In one patient the exact nature of the surgical intervention was not recorded. Sixteen of these patients did not experience a recurrence at final follow-up, whereas 11 had a local recurrence, 2 had regional recurrence, 3 had distant recurrence, and 1 patient each had local and distant, and local, regional, and distant recurrences.

At presentation, 10 patients presented with distant metastatic disease and were therefore eliminated from analysis of RFS. Analyzing the remaining 24 patients, the 5-year RFS was 38%. Analysis of the subset population (n = 34) demonstrated a 5-year DSS and OS of 57% and 54%, respectively.

Analysis of the 14 patient and tumor characteristics for effect on RFS demonstrated that patients with tumors >5 cm (n = 6; 0% RFS) faired significantly worse when compared with patients with a tumor <=5 cm (n = 18; 50% RFS; P < .001). Additionally, low-grade lesions (n = 12) had a significantly better RFS (58%) when compared with high-grade lesions (17%; P < .02). Finally, patients who did not experience a complication of their treatment (n = 22) had a significantly better RFS (41%) when compared with patients who did have a treatment complication (n = 2; 0%; P < .002). High-grade histology (n = 16) also significantly affected DSS (P < .004) and OS (P = .001) when compared with low-grade tumors (n = 17). Moreover, OS was affected by the occurrence of treatment complications (n = 5) when compared with patients who did not experience a complication of their treatment (n = 28; P = .04).

Multivariate analysis failed to demonstrate any significant effect of tumor size or grade on RFS, whereas these parameters did significantly influence DSS (Table 4). The only parameter found by multivariate analysis to influence OS was high-grade histology.


View this table:
[in this window]
[in a new window]
  		TABLE 4. Univariate and multivariate fibrosarcoma/malignant fibrous histiocytoma subset analysis
 

   DISCUSSION
TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
CONCLUSIONS
 REFERENCES
 
The overwhelming majority of cancers in the head and neck region (80%–90%) are of epithelial origin. Lymphoma is the next most common head and neck malignancy. Sarcomas of the head and neck are relatively rare tumors, accounting for 1% to 11% of all head and neck neoplasms.1 The rare nature of these tumors accounts for the difficulties encountered in making definitive treatment decisions based on the present literature. Furthermore, it is well known that certain benign7 and unrelated malignant lesions can be mistaken for sarcomas.8 Lesions such as melanoma, lymphoma, or carcinoma may all have a very similar histological appearance and thus may be misdiagnosed and mistreated.

This underscores the necessity of careful examination of the hematoxylin and eosin slides with immunohistochemical confirmation to avoid misdiagnosis. Given recent advances in the understanding of lesions such as MFH and the increasing use of molecular diagnosis, including the now-routine use of immunohistochemistry, some pathologic diagnoses were changed from the original entry into our prospective database by an experienced pathologist with dedicated expertise in sarcoma pathology. This resulted in changes in a significant number of diagnoses rendered. This finding underscores the often subjective nature of subclassification of sarcoma pathology and the nuances that exist in differentiating between categories of sarcomas. The increased use of the immunohistochemistry and molecular oncology markers will further our ability to definitively subclassify sarcomas in the future. More importantly, grade did not change.

Significant prognostic effect has been most consistently attributed in the literature to grade, size, and margin status for sarcomas of the head and neck (Table 5). Very few studies have examined prognostic factors affecting survival in head and neck soft tissue sarcomas by multivariate analysis. In our series, we found that size >5 cm and high-grade histology were the two variables that negatively affected RFS, DSS, and OS. Positive surgical margins were found to affect RFS as well. When subset analysis was performed for fibrosarcomas and MFH, size and grade continued to demonstrate an effect on survival.


View this table:
[in this window]
[in a new window]
  		TABLE 5. Prior literature of soft tissue sarcomas of the head and neck
 
For the studies that have used multivariate analysis, the findings are very similar. Indeed, prior reports from our institution concurred with the importance of these prognostic indicators.9 In another study of 65 patients from the University of California at San Francisco,10 tumor size and grade were found to significantly affect local control, whereas age, grade, adequacy of surgery, and margin status significantly affected survival. They found a trend toward improved local control with surgery and radiotherapy when compared with other treatment modalities. Radiotherapy alone in doses <=65 Gy was insufficient for disease control. Another study11 found that the radicality of surgery and tumor histology were independent factors affecting recurrence, whereas the same factors and age were significantly related to the risk of death on multivariate analysis. The effect of age is probably related to the inclusion of pediatric patients in the analysis. However, another study,12 which eliminated pediatric patients from analysis, also found age—in addition to margin status, grade, and the presence of distant metastasis on presentation—to be a significant independent prognostic indicator.

A study from Tata Memorial Hospital in India13 retrospectively examined 72 adult head and neck sarcoma patients. Their multivariate analysis also found that tumor size >5 cm and high-grade histology significantly affected survival. They pointed out that the high rates of locoregional failure warrant the need for improved treatment strategies. Addressing the role of adjuvant treatment, a study from the University of California at Los Angeles14 advocated the selective use of adjuvant radiotherapy for incompletely resected and high-grade tumors. Their recommendations were that in low-grade lesions with close or positive margins or for all high-grade tumors, postoperative radiotherapy should be undertaken. Conversely, in low-grade tumors with adequate surgical margins, radiotherapy need not be used. In this study, osteosarcomas and chondrosarcomas were included in the analysis. These recommendations are supported in our series by results such as those seen with our DFSP tumors. Despite these tumors being considered well differentiated, it is well known that these tumors are locally aggressive with a high rate of local recurrence.15 Our data demonstrated an 84% RFS associated with a 100% DSS. The addition of radiotherapy to those patients in our series who had positive margins allowed for this favorable disease-specific outcome.

The main therapy for soft tissue sarcomas of the head and neck is en-bloc resection with a margin of normal tissue. The adequacy of surgical resection seems to be a major determinant of outcome for sarcomas.1 The difficulty with en-bloc resection in these tumors relates to the proximity of important neurovascular structures and vital organs, as well as the cosmetic and functional morbidity resulting from such radical extirpative procedures. It is known that some soft-tissue sarcomas develop a pseudocapsule as a result of compression of adjacent tissues. This may obscure the true invasive nature of these neoplasms. Sarcomas tend to extend outside this pseudocapsule and invade tissues that may seem uninvolved during surgery. Because of the anatomical challenges of en-bloc resection for soft tissue sarcomas of the head and neck, positive margins should prompt the addition of adjuvant radiotherapy.16

After removal of all patients previously treated for their sarcomas from our study, too few patients remained for evaluation of the effects of adjuvant radiotherapy on outcome. However, some insight may be gained from our data. In our series, final pathologic evaluation demonstrated that 42% of our population had microscopically positive margins. Moreover, 63% (12 of 19) of patients who presented after prior treatment demonstrated positive margins. This rate of margin positivity is not surprising in light of the close proximity to numerous vital structures within the head and neck region. Of those patients whose disease recurred, the most common site of recurrence was local. In light of the fact that 77% of our patient population was treated by surgery alone, our high rate of local recurrence may be linked to a high rate of microscopically positive margins without the addition of adjuvant radiotherapy. These results translated into a 5-year RFS and DSS of 55% and 52%, respectively, underscoring the close relationship between local recurrence and survival in soft tissue sarcomas of the head and neck region.17

Despite our inability to establish a survival benefit of adjuvant radiotherapy, these data suggest that the addition of adjuvant radiotherapy may improve local control rates, which may translate into improvement in DSS. In the absence of large series of head and neck sarcomas, treatment algorithms can only be extrapolated from published series of sarcomas of other sites. Our institution has a significant experience with soft tissue sarcomas in other sites and has shown the influence of microscopically positive margins on outcomes18 and the value of radiotherapy in randomized trials.19

The addition of chemotherapy is controversial and has yet to demonstrate definitive benefit.1 Our data were unable to determine any survival benefit with the addition of chemotherapy, but it was used in a sporadic fashion and without the benefit of a clinical trial. Further research is needed to find treatment modalities that may affect survival for these patients.


   CONCLUSIONS
TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSIONS
REFERENCES
 
Soft-tissue sarcomas of the head and neck region represent a heterogeneous group of tumors with distinct prognostic implications. Patients who present with low-grade lesions are good candidates for wide local excision alone (securing histologically negative margins); in these patients, withholding adjuvant radiotherapy seems justified. High-grade tumors require wide surgical excision with adequate margins, when feasible, followed by adjunctive radiotherapy. This approach has improved local control rates but has yet to demonstrate an effect on OS.


   FOOTNOTES
 
Soft tissue sarcomas of the head and neck region represent a rare group of tumors. We performed an analysis of 111 head and neck sarcoma patients. Size >5 cm and high-grade histology were found to significantly affect survival.

Received for publication March 6, 2003. Accepted for publication February 25, 2004.


   REFERENCES
TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 

  1. Patel SG, Shaha AR, Shah JP. Soft tissue sarcomas of the head and neck: an update. Am J Otolaryngol 2001; 22: 2–18.[CrossRef][Medline]
  2. Hajdu S. Pathology of Soft Tissue Tumors. Philadelphia: Lea & Febiger, 1979.
  3. Fletcher CD, Gustafson P, Rydholm A, Willen H, Akerman M. Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol 2001; 19: 3045–50.[Abstract/Free Full Text]
  4. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958; 53: 457–61.[CrossRef]
  5. Peto R, et al. Design and analysis of randomized clinical trials requiring prolonged observation of each patient. II. Analysis and examples. Br J Cancer 1977; 35: 1–39.[Medline]
  6. Cox D. Regression models and life tables. J R Stat Soc B 1972; 34: 187–220.
  7. Allen PW, Allen LJ. Perce the permissive pathologist: a cautionary tale of one who misdiagnosed a pseudosarcoma, killed the patient and was found out. Aust N Z J Surg 1994; 64: 273–4.[Medline]
  8. Flens MJ, et al. The contribution of immunocytochemistry in diagnostic cytology. Comparison and evaluation with immunohistology. Cancer 1990; 65: 2704–11.[CrossRef][Medline]
  9. Kraus DH, et al. Prognostic factors for recurrence and survival in head and neck soft tissue sarcomas. Cancer 1994; 74: 697–702.[CrossRef][Medline]
  10. Le QT, et al. Prognostic factors in adult soft-tissue sarcomas of the head and neck. Int J Radiat Oncol Biol Phys 1997; 37: 975–84.[CrossRef][Medline]
  11. Kowalski LP, San CI. Prognostic factors in head and neck soft tissue sarcomas: analysis of 128 cases. J Surg Oncol 1994; 56: 83–8.[Medline]
  12. Dijkstra MD, et al. Survival of adult patients with head and neck soft tissue sarcomas. Clin Otolaryngol 1996; 21: 66–71.[Medline]
  13. Dudhat SB, et al. Prognostic factors in head and neck soft tissue sarcomas. Cancer 2000; 89: 868–72.[CrossRef][Medline]
  14. Tran LM, et al. Sarcomas of the head and neck. Prognostic factors and treatment strategies. Cancer 1992; 70: 169–77.[CrossRef][Medline]
  15. Stojadinovic A, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol 2000; 7: 696–704.[Abstract]
  16. Lewis JJ, Benedetti F. Adjuvant therapy for soft tissue sarcomas. Surg Oncol Clin North Am 1997; 6: 847–62.[Medline]
  17. Stojadinovic A, et al. Primary adult soft tissue sarcoma: time-dependent influence of prognostic variables. J Clin Oncol 2002; 20: 4344–52.[Abstract/Free Full Text]
  18. Pisters PW, et al. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 1996; 14: 1679–89.[Abstract/Free Full Text]
  19. Pisters PW, et al. A prospective randomized trial of adjuvant brachytherapy in the management of low-grade soft tissue sarcomas of the extremity and superficial trunk. J Clin Oncol 1994; 12: 1150–5.[Abstract/Free Full Text]
  20. Weber RS, et al. Soft tissue sarcomas of the head and neck in adolescents and adults. Am J Surg 1986; 152: 386–92.[CrossRef][Medline]
  21. Greager JA, et al. Soft tissue sarcomas of the adult head and neck. Cancer 1985; 56: 820–4.[CrossRef][Medline]
  22. Farhood AI, et al. Soft tissue sarcomas of the head and neck in adults. Am J Surg 1990; 160: 365–9.[CrossRef][Medline]
  23. Wanebo HJ, et al. Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research. Head Neck 1992; 14: 1–7.[Medline]
  24. Eeles RA, et al. Head and neck sarcomas: prognostic factors and implications for treatment. Br J Cancer 1993; 68: 201–7.[Medline]
  25. Le Vay J, et al. An assessment of prognostic factors in soft-tissue sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg 1994; 120: 981–6.[Abstract]
  26. Willers H, et al. Adult soft tissue sarcomas of the head and neck treated by radiation and surgery or radiation alone: patterns of failure and prognostic factors. Int J Radiat Oncol Biol Phys 1995; 33: 585–93.[CrossRef][Medline]




This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Bentz, B. G.
Right arrow Articles by Kraus, D.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Bentz, B. G.
Right arrow Articles by Kraus, D.

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS