|Year : 2011 | Volume
| Issue : 3 | Page : 284-288
Adult soft-tissue sarcomas of the head and neck
KT Sidappa, A Krishnamurthy
Department of Surgical Oncology, Cancer Institute (WIA), 36, Sardar Patel Rd, Adyar, Chennai - 600 020, India
|Date of Web Publication||14-Sep-2011|
Department of Surgical Oncology, Cancer Institute (WIA), 36, Sardar Patel Rd, Adyar, Chennai - 600 020
Source of Support: None, Conflict of Interest: None
Background: Head and neck sarcomas are relatively rare tumors, accounting for only about 2% of all head and neck malignancies and for 4 to 10% of all soft tissue sarcomas. The rarity and more over the heterogeneity of these tumors make rigorous study of their clinical behavior difficult. Aims: We analyzed our single center experience of adult soft tissue sarcomas of the head and neck. Settings and Design: Our study included 27 patients of adult soft-tissue sarcomas of the head and neck who presented to our center between January 1996 and December 2005. Materials and Methods: Patient characteristics, clinical features, treatment modalities, clinical course, and long-term outcomes and its determinants were analyzed. Results: Surgery in the form of wide excision aimed at achieving negative margins was the main modality of treatment and adjuvant radiation was used in selected patients. The five-year disease-free survival was 64.5% and overall survival (OS) was 68%. Of the multiple potential factors analyzed, univariate analysis showed grade, margin status, and adjuvant radiation to be the important prognostic factors for the survival. The multivariate analysis however only showed margin status and adjuvant radiation to be prognostic. Conclusions: Surgery with negative margins is a key to long-term survival. Complete resection with adjuvant radiation in selected cases potentially decreases local recurrences and improves OS.
Keywords: Adult soft tissue sarcomas, head and neck, prognosis
|How to cite this article:|
Sidappa K T, Krishnamurthy A. Adult soft-tissue sarcomas of the head and neck. Indian J Cancer 2011;48:284-8
| » Introduction|| |
Soft-tissue sarcomas are rare heterogeneous mesenchymal neoplasms which account for less than 1% of all malignancies.  Soft tissues include muscles, tendons, fat, fibrous tissue, synovial tissue, vessels, and nerves. Approximately 60% of soft-tissue sarcomas arise in the extremities, the lower extremities involved three times more often than the upper extremities. The other sites being the trunk (19%), the retroperitoneum (15%), and the head and neck (9%).  Currently, more than 50 histological subtypes of soft-tissue sarcoma have been identified, the most common are malignant fibrous histiocytoma (MFH) (28%), leiomyosarcoma (12%), liposarcoma (15%), synovial sarcoma (10%), and malignant peripheral nerve sheath tumor (MPNST) (6%). 
| » Materials and Methods|| |
This was a retrospective study of all adult soft-tissue sarcomas of the head and neck that presented to the Division of Head and Neck Oncology between January 1996 and December 2005. The median follow-up period was 59 months. All the patients in the present study underwent a detailed history and physical examination of the head and neck. One-third of the patients had undergone unplanned excisions for presumably benign lesions at an outside center; the relevant operative and histopathology details were collected and confirmed at our center. All patients underwent a staging computerized tomography (CT) scan of the head and neck to localize the lesion and determine its extent. A few patients were additionally evaluated with magnetic resonence imaging scans. A CT scan of the chest was done for all patients after a biopsy confirmation of a sarcoma as a part of the metastatic work-up.
The lesions were surgically approached as per their anatomical location with an aim of achieving a tumor-free margin. The margins of excision were classified according the criteria as described by Enneking et al.  Adjuvant radiotherapy was considered for high-grade sarcomas, large tumors (>5 cm), close or positive surgical margins. Patients were followed up at timely intervals and recurrences were addressed as appropriate.
An analysis of multiple potential prognostic factors namely size, grade, age group, sex margin status, adjuvant treatment, and histology was done.
| » Results|| |
A total of 27 cases of adult head and neck soft-tissue sarcomas were analyzed. The median follow-up period was 59 months (ranging from 14-144 months).
Age and sex distribution
Majority of the cases were between the second and the fourth decade; the median age at presentation being 35 years (ranging from 16 to 76 years). There was a significant male preponderance (1.7 : 1).
The most common presenting feature was as a painless swelling. The average size of tumor at presentation was 4.8 cm (range, 2-12 cm). Spindle cell sarcomas, MPNST, and MFH constituted more than three-fourth of the histological subtypes [Table 1]. The sites of origin of these tumors are as described in [Table 2].
Surgery in the form of wide excision aimed at achieving negative margins was the main modality of treatment. One-third of patients (9 of 27) had unplanned excision at outside centers, six of whom underwent re-excision, two received adjuvant radiation only, and one kept under follow-up. Four of six patients who underwent re-excision had residual disease. Adjuvant radiation was given to 21 patients in all. Primary closure was possible in 12 patients, while 15 patients required some form of reconstruction. Local flaps were used in five patients, pedicled flaps in four, and microvascular free flaps in six patients.
Recurrences were noted in nine of the 27 patients, of which six were local, one nodal, and two distant (lung) metastases. Their subsequent management, salvage rates, and final outcomes are as shown in [Table 3]. Three of the local recurrences were in patients who had positive/close margins, while two others were in patients with negative margins but did not receive adjuvant radiation, one had a 3-cm low-grade tumor over face and other was a patient who had an 8-cm high-grade tumor over the scalp and declined radiation. We also noted that all but one of the recurrences were associated with high-grade tumors.
The five-year disease-free survival was 64.5% and overall survival (OS) was 68%. Multiple potential prognostic factors pertaining to the patient, tumor, and treatment were analyzed. The univariate analysis showed grade, margin status, and adjuvant radiation to be the important prognostic factors for the survival [Table 4]. The multivariate analysis however only showed margin status (P = 0.038) and adjuvant radiation (P = 0.022) to be prognostic for survival.
Head and neck sarcomas are relatively rare tumors, accounting for a mere 2% of all head and neck malignancies and 4 to 10% of all soft tissue sarcomas.  The rarity of these tumors makes rigorous study of their clinical behavior difficult.  As a result, treatment paradigms are drawn from retrospective case series [Table 5] rather than from large clinical trials. Their study is also made more difficult by the diversity of histological subtypes and their varied clinical behavior.
Surgery in the form of wide excision is the primary modality of management of all adult soft-tissue sarcomas. In extremity sarcomas, wider resections are generally possible due to adequate soft tissue around the tumors and the same is generally achieved without significant cosmetic and function deficits. The local recurrences are less than 10% and majority of the deaths in non-head and neck sites are due to metastatic disease.
In comparison, because of the relatively small space of the head and neck region and proximity to vital structures, surgical removal of head and neck sarcomas often cannot achieve the ideal "wide" resection margins that are preferred in other anatomic sites. The other important considerations include the potential for morbidity related to breathing, swallowing, and speaking, and the significant cosmetic deformity that may follow facial and neck resections. Patients with head and neck sarcomas therefore frequently experience local relapses and succumb to it, many times without distant metastatic disease ,, ; 50% of the deaths in our series were due to progressive local disease, while metastatic disease progression accounted for 37.5% of the deaths.
Positive surgical margins are associated with a poor prognosis. ,,,, In our study the presence of positive/close margins was an independent prognostic factor in multivariate quality of the tissue forming the margin is a more important indicator of the risk for local recurrence rather than the physical distance from the tumor to the resection margin. , The generally accepted recommendation for surgical margins of head and neck sarcomas is to obtain a complete tumor resection with as wide a cuff of normal tissue as is feasible, with as little morbidity as possible.
Re-excision removes the unappreciated residual tumor that extends beyond the pseudocapsule of the primary tumor after unplanned excision. Residual disease after re-excision reported in previous studies varies from 30 to 60%. Local control is improved with repeated wide-margin resections after incomplete primary surgery. ,, In our study, 66% (4/6) of the re-excised specimens harbored residual disease. Since local failures are a common cause of death in head and neck sarcomas, re-excision should be considered in all unplanned excisions.
Furthermore, in the absence of demonstrable metastases, local recurrences should be treated aggressively, as they can occasionally be salvaged by further surgery. , Our local failure salvage rate was 50%.
The other potentially important factors for local recurrence are tumor size (>5 cm) ,, and grade of tumor. ,,,, In our series, high-grade tumors were not only associated with increased local, regional, and distant recurrences, but also found to be an independent prognostic marker.
Adjuvant radiotherapy improves local control and should be recommended for all high-grade tumors, tumors more than 5 cm, and for tumors with close or positive margins. , Although adjuvant radiation has shown to improve local control in soft-tissue sarcomas of the extremity, its impact in patients with positive microscopic margins has not been clearly defined. Data from the Princess Margaret Hospital, Massachusetts General Hospital, and MSKCC have shown higher 5-year local recurrence rates in patients with positive margins than in those with negative margins despite the use of adjuvant irradiation, although radiotherapy did reduce the risk of recurrence compared with no adjuvant treatment. , The other role for radiation in the management of soft-tissue sarcomas is in cases of unresectable tumors, where in it is used as a primary modality and at times with chemotherapy. , More often, such treatments result only in palliative benefit rather than cure.
The role of adjuvant chemotherapy is unclear. The benefit of adjuvant chemotherapy is shown in a subgroup analysis of the sarcoma meta-analysis collaboration trial, mainly for high-grade extremity sarcomas.  This however does not mean that there is no benefit of adjuvant chemotherapy in resectable head and neck soft-tissue sarcomas; it is just that the numbers of such patients in the trials included are few to make any specific recommendations.
As with head and neck carcinomas, reconstructions with microvascular free flaps have greatly improved the cosmetic and functional results following resection of head and neck sarcomas. Such flaps have the theoretical potential to cover large and critical bone and soft-tissue deficits with good to excellent results.
In conclusion, surgery with negative margins is the key to long-term survival. Complete resection with adjuvant radiation and possibly chemotherapy in selected cases can potentially decrease local recurrences and improve OS.
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[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]
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