Low-grade myofibroblastic sarcoma of maxillary sinus and buccal mucosa: Two rare cases and review of the literature

Low-grade myofibroblastic sarcoma (LGMS) represents an atypical tumor composed of myofibroblasts with a predilection for the head and neck, especially in the tongue and oral cavity, with a high tendency to local recurrence and metastasis, even after a long period. LGMS arising from maxillary sinus and buccal mucosa are not very common. To the best of our knowledge, only 55 cases of low-grade myofibroblastic sarcoma have been reported and only four cases of LGMS of maxillary sinus and three cases of LGMS of buccal mucosa have been reported in world literature. We report two cases of LGMS of the maxillary sinus and buccal mucosa, discussing clinical, histological, inmunohistochemical and therapeutic features.

Myofibrosarcoma-two cases report and review of literature / 中国矫形外科杂志

[Objective]To investigate the clinical and histopathological characteristics of myofibrosarcoma,and study its treatment and prognosis.[Method]The clinical features,histopathological characteristics,diagnosis,treatment and prognosis of two rare myofibrosarcoma patients were observed,and relative literatures were reviewed.[Result]After biopsy,the frist patient was treated with amputation in her middle thigh.postoperative chemotherapy was performed in the local hospital,but the drug and its dose didn't know in detail.one year latter,the patient died because of lung metastasis.The second patient was treated with tumor resection,but three months latter,the tumor recurred and was resected again,and the patient accepted chemotherapy,eleven nonthns latter,lung metastasis was found.then the patient died.About 54 patients with myofibrosarcoma were found in the literatures published in recent years.This neoplasms often involved the head and neck region,trunk and extremities in male patient A expandable and painless mass is the chief clinical features.Myofibrosarcoma can be identified by its specific morphologic features and positive myoid markers.[Conclusion]Myofibrosarcoma is a rare spindle cell neoplasm,Its morbidity dosen't know in detail.When making diagnisis,it is important to distinguished from synovial sarcoma,fibrosarcoma and leiomyosarcoma et al,Surgical resection is the mainstay of therapy,but the result of chemotherapy is not affirmed,The tumor is lower grade malignant one,and is invadable.The rate of local recurrence is high,and distant metastasis could be found.Its prognosis is not affirmed.

Myofibrosarcoma of the Nostril Sill after Excision of a Wart

Myofibroblasts are mesenchymal spindle cells that share morphologic and functional features with fibroblasts and smooth muscle cells, especially in relation to matrix production and contractility. Myofibroblastic sarcomas have been described recently and such lesions are relatively rare and almost unknown, especially developed in the skin. Most examples of myofibrosarcoma occur de novo. We herein report a case of solitary myofibrosarcoma occurred after several laser and pharmacological treatment of a wart. A 28-year-old woman had a 10-month history of small firm painless papule on the lower margin of her nostril. Her lesion was once treated with laser at a local clinic. At first visit to our hospital, the lesion was 0.4 x 0.3 cm solitary skin colored keratotic flat papule and it was diagnosed as a wart and ablated with carbon dioxide laser. Two subsequent recurrences occurred within 6 months and repeatedly ablated with the same laser. One year later the patient appeared with 1.5 x 1.5 cm pale red rubbery-firm nodule on the same site. On punch biopsy, it showed thickened collagen with mild inflammatory infiltrate. We performed intra-lesional triamcinolone injection every three weeks under the impression of hypertrophic scar after the wart removal, and no improvement was made and the lesion's growth was accelerated. On exicisional biopsy of lesion, a myofibroblastic tumor of uncertain malignant potential was suggested and subsequent wide excision and lateral lip advancement flap was done. The patient has been free of recurrence 14 months after wide excision. This report emphasizes the possibility that iatrogenic manipulation of the normal wound healing process may well contribute to the development of myofibrosarcoma.

Myofibrosarcoma of the Breast: A case report

Myofibrosarcoma of the breast is a rare malignant mesenchymal tumor that has been reported in only four well documented cases so far. We report a case of myofibrosarcoma of the breast in an 88-year-old man who complained of a palpable mass for 1 year. Microscopically, the tumor consisted mainly of spindled cells, arranged in irregular fascicles and embedded in broad bands of dense hyalinized collagen. It showed ill-defined border infiltrating the adjacent adipose tissue, moderate cellular pleomorphism, and high mitotic rate (8~9/10 HPF). Immunohistochemical study confirmed myofibroblastic differentiation of the tumor cells with diffuse strong reaction for vimentin, smooth muscle actin, and fibronectin.