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Indian Journal of Cancer, Vol. 48, No. 3, July-September, 2011, pp. 282-283 Commentary Sarcomas of head and neck region N Tandon, K Prabhash Department of Medical Oncology, Tata Memorial Hospital, Mumbai, India Correspondence Address: K Prabhash, Department of Medical Oncology, Tata Memorial Hospital, Mumbai, India, kp_prabhash@rediffmail.com Code Number: cn11077 PMID: 21921323 DOI: 10.4103/0019-509X.84910 Soft tissue sarcomas of the head and neck are rare mesenchymal malignant neoplasms, accounting for less than 10% of all soft tissue sarcomas and approximately 1% of all head and neck neoplasms. [1],[2] Soft tissue sarcoma is relatively rare and only few series have been reported. The case series with the maximum number of patients is of 110 patients. In this context, this report becomes very important. This article has 27 patients with a median follow-up of 59 months. We need to appreciate the efforts of the authors for the same. Soft tissue sarcomas which show a relative predilection for head and neck are cutaneous angiosarcoma, capillary hemangioma of juvenile/cellular type, atypical fibroxanthoma, pediatric rhabdomyosarcoma and solitary fibrous tumor, while nasopharyngeal angiofibroma and sinonasal hemangiopericytoma are unique to their site of origin. [3] Soft tissue sarcomas of the head and neck are not related to smoking unlike squamous cell carcinomas. In fact, they have been found to be associated with genetic syndromes like neurofibromatosis and retinoblastoma, as well as irradiation. Malignant fibrous histiocytoma is probably the most frequent type of radiation-induced soft tissue sarcoma of the head and neck. Fibrosarcomas have also been reported as being related to scar formation. Adult soft tissue sarcomas display a diverse array of histologies and clinical activity ranging from relatively slow-growing lesions to aggressive locally destructive lesions with a potential for systemic metastasis. Lymph node metastasis occurs only in 3-10%, while distant metastases occur in 28% of the patients, with the most frequent sites being lung followed by bone, central nervous system and liver. [4],[5] In a review of 110 patients at Alberta, Canada, [2] who were treated between 1974 and 1999, the median age was 66 years (range 16-97 years) with a peak between 70 and 89 years. The most common sign was a painless lump of the subcutaneous tissue in almost 80% of cases. Tumor size was greater than 5 cm in only 12 (11%) patients. In 71%, the tumor was localized superficial to the investing fascia. Malignant fibrous histiocytoma was the most common histologic diagnosis (41 of 110), followed by Dermatofibrosarcoma Protuberans (DFSP) and fibrosarcoma. Most of the tumors were low grade (44/61), followed by high-grade tumors (11/61), and intermediate grade formed the smallest fraction (6/61). In the article by Siddapaa et al, what is different as compared to published literature? These include younger median age, male preponderance and spindle cell as the commonest histology. On the other hand, the presenting features are similar to those found in earlier reports. The management of soft tissue sarcomas in the head and neck is primarily surgical. Sarcoma growth is expansive with pseudocapsule formation and a tendency to grow along fascial planes. For these reasons, a wider resection is required respecting fascial compartments. Unfortunately, in the head and neck area, adequate margins are difficult to obtain because of anatomic constraints. Because of the low incidence of lymph node metastasis, elective lymph node dissection is not indicated unless clinically involved. [7] Regional lymph node dissection is necessary if there is nodal metastases. [8],[9] The reported series highlights the role of surgery as the most important mode of treatment. This also highlights that nodal metastases are rare and neck dissection can usually be avoided. Adjuvant radiotherapy is generally recommended after excision of high-grade and large sarcomas and when surgical margins are close or positive. This series also emphasizes that recurrence pattern of adult head and neck cancer is different from that of extremity sarcoma, with a lesser likelihood for distant metastasis. This would have been more evident if the grade of sarcoma and its correlation with patterns of recurrences and metastasis was also mentioned in the article. It also appears that the role of systemic therapy in non-extremity sarcoma is very limited. The best results are achieved with the combination of doxorubicin and ifosfomide. [10] This series also highlights that distant metastases are uncommon but not negligible. Role of adjuvant chemotherapy may be explored in future to decrease the mortality. Nicolas Penel et al[11] studied the prognostic value of surgery in a retrospective database of soft tissue sarcomas of head and neck and found that young age (P = 0.03) and inadequate resection (P = 0.00003) were most likely to have local and metastatic failure. Singh et al[6] showed that high-grade deep tumors >5 cm and the ones with intralesional margin have the worse prognosis. Willers et al[12] analyzed 57 patients with head and neck soft tissue sarcomas and suggested that angiosarcomas of the head and neck have a considerably poorer prognosis in addition to tumor size, grade and extension. In a multivariate analysis by Sidappa and Krishnamurthy, margin status (P = 0.038) and adjuvant radiation (P = 0.022) were found to be of prognostic importance for survival. [13] We think sample size was small and multivariate analysis would not give a reliable result. There were nine patients who underwent unplanned surgery outside, out of whom six underwent re-excision and two received adjuvant radiation. Reasons for different management for the same have not been mentioned in the article. Also, the impact of these unplanned excisions on the outcomes of the patients remains less well defined. But this highlights that one needs to have high index of suspicion for these tumors in appropriate patients and the condition needs to be managed by experts to avoid unnecessary treatment and better outcome. References
Copyright 2011 - Indian Journal of Cancer |
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