• Myxofibrosarcoma
• Prognosis
• Surgery

• Myxofibrosarcoma
• head and neck
• sinonasal

A myxofibrosarcoma (MFS) is a malignant fibroblastic tumor that tends to occur in the lower and upper extremities. The reported incidence of head and neck MFSs is extremely rare. We report a 46-year-old male with “a neoplasm in the scalp” who was hospitalized and diagnosed with an MFS (highly malignant with massive necrotic lesions) based on histologic and immunohistochemistry evaluations. The magnetic resonance imaging manifestations did not demonstrate the “tail sign” mentioned in several studies, which resulted in a great challenge to establish an imaging diagnosis. The treatment plan is closely associated with the anatomic location and histologic grade, and more importantly, aggressive surgery and adjuvant radiotherapy may be helpful. Hence, we report the case and share some valuable information about the disease.

A 46-year-old male with “a neoplasm in the scalp for 6 mo” was hospitalized. Initially, the tumor was about the size of a soybean, without algesia or ulceration. The patient ignored the growth, did not seek treatment, and thus, did not receive treatment. Recently, the tumor increased to the size of an egg; there was no bleeding or algesia. His family history was unremarkable. No abnormalities were found upon laboratory testing, including routine hematologic, biochemistry, and tumor markers. Computed tomography showed an ovoid mass (6.25 cm × 3.29 cm × 3.09 cm in size) in the left frontal scalp with low density intermingled with equidense strips in adjacent areas of the scalp. Magnetic resonance imaging revealed a lesion with an irregular surface and an approximate size of 3.55 cm × 6.34 cm in the left frontal region, with clear boundaries and visible separation. Adjacent areas of the skull were damaged and the dura mater was involved. Contrast enhancement showed an uneven enhancement pattern. Surgery was performed and postoperative adjuvant radiotherapy was administered to avoid recurrence or metastasis. The post-operative pathologic diagnosis confirmed an MFS. A repeat computed tomography scan showed no local recurrence or distant metastasis 19 mo post-operatively.

The case reported herein of MFS was demonstrated in an extremely rare location on the scalp and had atypical magnetic resonance imaging findings, which serves as a reminder to radiologists of the possibility of this diagnosis to assist in clinical treatment. Given the special anatomic location and the high malignant potential of this rare tumor, combined surgical and adjuvant radiotherapy should be considered to avoid local recurrence and distant metastasis. The significance of regular follow-up is strongly recommended to improve the long-term survival rate.

• Malignant fibrous histiocytoma
• Myxofibrosarcoma
• Scalp
• Magnetic resonance imaging
• Treatment
• Case report

• Defects
• Infratemporal region
• Repair
• Submandibular gland FLAP

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.