Published online Apr 24, 2024. doi: 10.5306/wjco.v15.i4.566
Peer-review started: November 16, 2023
First decision: December 31, 2023
Revised: January 20, 2024
Accepted: March 20, 2024
Article in press: March 20, 2024
Published online: April 24, 2024
Low-grade myofibroblastic sarcoma (LGMS) is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts. LGMS most commonly develops in adults, predominantly in males, in the head and neck region, oral cavity, especially on the tongue, mandible, and larynx. This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.
Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery. Case 1: A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus (rT4aN0M0), with symptoms of pain in the suborbital area, watering of the right eye, thick discharge from the right nostril, and augmented facial asymmetry. After open biopsy-confirmed LGMS, the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm. The patient qualified for adjuvant radiotherapy for the postoperative bed, with an additional margin. Currently, the patient is under 1.5 years of observation with no evidence of disease. Case 2: A 45-year-old man was admitted to our clinic with facial asymmetry, strabismus, exophthalmos, and visual impairment in the right eye. Six months earlier, the patient had undergone partial jaw resection at another hospital for fibromatosis. A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure. An open biopsy confirmed low-grade fibrosarcoma (rT4aN0M0). The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap. The patient subsequently underwent adjuvant radiotherapy to the postoperative area. After 9 months, recurrence occurred in the right mandibular arch below the irradiated area. The lesion infiltrated the base of the skull, which warranted the withdrawal of radiotherapy and salvage surgery. The patient qualified for palliative chemotherapy with a regimen of doxorubicin + dacarbazine + cyclophosphamide and palliative radiotherapy for bone metastases. The patient died 26 months after surgical treatment. The cases have been assessed and compared with cases in the literature.
No specific diagnostic criteria or treatment strategies have been developed for LGMS. The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision; adjuvant radiotherapy or chemoradiotherapy should also be considered. They have low malignant potential but are highly invasive, tend to recur, and metastasize to distant sites. Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage. Patients should be treated and observed at the highest referral centers.
Core Tip: Low-grade myofibroblastic sarcomas are tumors of low malignant potential; however, they are highly invasive and a high tendency to recur and metastasize to distant sites. Since only 55 cases of low-grade myofibroblastic sarcoma have been described, it is impossible to establish guidelines. As there are no specific diagnostic criteria, it is necessary to consider the occurrence of myofibroblastic sarcoma more often than reported in the literature.