Myxofibrosarcoma of the sinus piriformis: case report and literature review
© Qiubei et al.; licensee BioMed Central Ltd. 2012
Received: 3 July 2012
Accepted: 21 September 2012
Published: 15 November 2012
Myxofibrosarcoma is a common sarcoma in the extremities of older people, but is rare in the head and neck region. Here, we report the case of a 42-year-old male patient in whom myxofibrosarcoma generated from the sinus piriformis. Histopathologically, the tumor was characterized by spindle cellular proliferation with moderate cellular density in fibromyxoid stroma. Immunohistochemically, the tumor cells showed positive reactivity for vimentin, Ki-67, smooth muscle actin, and CD34, but negative staining for S-100. Based on these results, the tumor was diagnosed as a low-grade myxofibrosarcoma. Resection of the tumor was performed via a transcervical approach. The patient’s postoperative clinical course was uneventful and no local recurrence or distant metastasis has been found so far. The pathology, clinical characteristics, and treatment of myxofibrosarcoma are also reviewed.
KeywordsMyxofibrosarcoma Sinus piriformis
malignant fibrous histiocytoma
smooth muscle actin.
As one of the most common sarcomas, myxofibrosarcoma (MFS) is originally described as a myxoid variant of malignant fibrous histiocytoma (MFH), the unique characteristic of which can be ascribed as its frequent occurrence in the subcutaneous tissues of the extremities of older people. Myxofibrosarcoma rarely occurs in the head and neck regions , and only a few corresponding cases in these areas have been reported, with 3% to 10% involvement, including the larynx , esophagus , sphenoid sinus [4, 5], mandible , maxillary sinus , parotid [8, 9], orbit [10, 11], and infratemporal space . To our knowledge, only one case has so far been reported regarding MFS generated from the hypopharynx .
The objective of this work was to report an additional case of MFS arising in the sinus piriformis. The clinical, radiological, and histopathological characteristics of this tumor were also reviewed. Three years after operation, the postoperative clinical course was uneventful and no evidence of recurrence or metastasis and symptoms in the throat were observed in this patient.
Cases of myxofibrosarcoma in the head and neck region
Blitzer et al. 
Died after 3 months
Pomerantz et al. 
Barnes and Kanbour 
Sphenoid sinus-cavernous sinus
Surgery, adjuvant radiotherapy
Alive after 8 months
Imai et al. 
Lam et al. 
Left sphenoid sinus
Alive after 8 months
Iguchi et al. 
Song and Miller 
Nishimura et al. 
Alive after 16 months
Udaka et al. 
Alive after 27 months
Enoz and Yusufhan 
Alive after 2 years
Gugatschka et al. 
Xu et al. 
Alive after 8 months
Zhang et al. 
Alive after 6 months
Zouloumis et al. 
Alive 39 months
Norval et al. 
Died after 1 year
Srinivasan et al. 
Died after 24 months
Krishnamurthy et al. 
Alive after 26 months
Nakahara et al. 
Alive after 20 months
Histologically, MFS may exhibit various proportions of myxoid matrix with varied cellularity. Thus, it is suggested that MFS be subdivided into four  or three  grades according to the degree of cellularity, pleomorphism of the nucleus, and mitotic activity. Low-grade MFS shows a hypocellular to moderately cellular architecture with a prominent myxoid matrix. Tumor cells are fusiform, round, or stellate, with ill-defined, slightly eosinophilic cytoplasm and atypical, enlarged, hyperchromatic nuclei (Figure 5). Mitoses can only seldom be seen [1, 26]. Most of the tumors have stretched and curved capillaries, and the tumor cells tend to be located along the vessel periphery. Another finding worth mentioning is the presence of prominent elongated, curvilinear, thin-walled blood vessels with a perivascular condensation of tumor cells or inflammatory cells (mainly lymphocytes and plasma cells). Although magnetic resonance imaging and CT scans evidently make a great contribution to the visualization of malignant features, such as local tissue invasion, histopathological examination is recognized as the gold standard, for its capability to provide a definitive diagnosis . Immunohistochemically, low-grade MFS is generally positive for CD-34, vimentin , and sometimes for SMA and Ki-67 , while negative for S-100 protein. The histopathological findings of the present case were further consistent with a diagnosis of low-grade myxofibrosarcoma and immunoreactivity to vimentin and CD34, probably reflecting the tumor’s primitive fibroblastic nature.
Complete tumor resection with adequate resection margin remains the mainstay for treatment of MFS. The radiotherapy is applied only for recurrent, unresectable lesions or tumors with positive resection margins, to suppress local recurrence and the risk of histologic progression, especially for low-grade MFS. The value of chemotherapy in MFS is still an issue for open debate . Low-grade MFS is considered to have low malignancy, and rarely shows distant metastasis, implying a good short-term prognosis. The overall 5-year survival rate is 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. It has continuity from low- to high-grade subdivision, showing low-grade areas in high-grade lesions, and a histologic progression of low- to high-grade tumors in recurrences, hence acquiring metastatic potential [15, 29]. Therefore, these patients should be placed under careful and long-term follow-up. Complete resection of the tumor was accomplished in our case and this patient showed no recurrence and metastasis three years after the operation.
In summary, we report a rare case of MFS that generated from the sinus piriformis. Histopathological examination is the gold standard for offering a definitive diagnosis, and the prognosis is accurate after complete resection and careful surveillance. Local recurrence may occur generally with progression of the tumor stage and risk of later metastasis. In this case, the hypothesis that the tumor is recurrent may be reasonable because of the insufficient resection in the previous operation. This emphasizes the recurrent character of MFS and the importance of sufficient resection.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor-in-chief of this journal.
Zhu Qiubei and Lin Cheng should be regarded as co-first authors.
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