MFS of the head and of the neck are extremely rare. To the best of our knowledge, only 19 cases have been previously described in the head and neck so far (Table
1), our case being the first observed within the pterygopalatine fossa. Apart from the general histologic features, MFS demonstrates wide histologic variability, based on its grade of malignancy. These tumors have been subdivided into three or four grades based on the degree of cellularity, nuclear pleomorphism, and mitotic activity [
5,
6]. Low-grade MFSs are considered to have low malignancy, and rarely show distant metastasis, implying a good short-term prognosis. Mentzel
et al.[
5], in their study, reviewed 75 patients and documented local recurrence in 55% of cases and distant metastases in 33%. More recently, Nishio
et al.[
7] reported an overall 5-year survival rate of 60 to 70%; however, the local recurrence rate of the low-grade type is as high (50% to 60%) as that of the high-grade type. Sanfilippo
et al. [
8], in a review of 178 patients, stated that, while local recurrences were predicted by the quality of surgical margins, the distant metastases were observed based on histological grade. Zhu Qiubei
et al.[
9] reviewed, from 1981 until 2012, 18 cases. The average age of onset reported was 53 years with male prevalence (66.7%). The average follow-up after treatment for all the cases of MSF of head and neck described was 17 months (range 3 to 39 months). None of the patients reported developed locoregional lymph node or systemic metastases at the latest follow-up. Surgery followed by adjuvant radiotherapy was the most common form of treatment strategy, and only two articles dealing with radio/chemotherapy exist, due to the small number of cases. Cante
et al.[
10] reported one case of MSF of maxillary sinus with a complete remission at 18-month follow-up after a combined radio-chemotherapy (RT/CHT) without surgery (Table
2). The results obtained with combined RT/CHT treatment for head and neck MFS should encourage further studies to confirm the efficacy in terms of long-term disease-free survival. According to these considerations, a complete tumor resection with adequate resection margins, followed by adjuvant radiotherapy, remains the mainstay for treatment of MFS. A possible re-excision of recurrent lesions is considered the mainstay of therapy for disease control.
Table 1
Cases of myxofibrosarcoma in the head and neck region
Maxillary sinus | 3 |
Sphenoid sinus | 3 |
Orbit | 2 |
Maxilla | 2 |
Esophagus | 1 |
Hypopharynx | 1 |
Neck | 1 |
Vocal folds | 1 |
Parotid | 3 |
Mandible | 1 |
Infratemporal space | 1 |
Total of cases reported in head and neck
|
19
|
Table 2
Head and neck myxofibrosarcoma: review of cases
Male | 66 | Radiotherapy | 3 months |
Male | 58 | Surgery | Unknown |
Female | 67 | Surgery, radiotherapy | 8 months |
Female | 52 | Surgery | Unknown |
Male | 55 | Surgery | 8 months |
Male | Unknown | Unknown | Unknown |
Male | 40 | Surgery | Unknown |
Male | 69 | Surgery | 16 months |
Male | 55 | Surgery | 27 months |
Female | 36 | Surgery | 24 months |
Male | 79 | Surgery | Unknown |
Female | 37 | Surgery, radiotherapy | 8 months |
Female | 27 | Surgery, radiotherapy | 6 months |
Male | 23 | Surgery, radiotherapy | 39 months |
Male | 69 | Radiotherapy, chemotherapy | 12 months |
Female | 78 | Surgery, radiotherapy | 24 months |
Female | 42 | Surgery, radiotherapy | 26 months |
Male | 52 | Surgery, radiotherapy | 20 months |
Male | 65 | Radiotherapy, chemotherapy | 18 months |