Malignant Fibrous Histiocytoma/Undifferentiated High-Grade Pleomorphic Sarcoma of the Maxillary Sinus

Abstract

Malignant fibrous histiocytoma (MFH) also known as undifferentiated high-grade pleomorphic sarcoma (UHPS) is a soft tissue sarcoma, composed of undifferentiated mesenchymal tumors possessed fibrohistiocytic morphology without definite true histiocytic differentiation. Head and neck localization is very rare, showing an incidence ranging from 4 % to 10 % in different series of investigations. The most frequent involved sites in UHPS are the neck and parotid, followed by the scalp, face, anterior skull base and orbit. Upper aerodigestive tract, lateral skull base and ear are rare locations. The incidence of the lymphatic metastases is also rare. The aim of this article is to report a case of UHPS in the maxillary sinus with palatal, orbital and ethmoidal involvement, with lymphatic metastasis and its surgical treatment. In addition, we review the literature of similar cases of the past 12 years.

Malignant fibrous histiocytoma: Database review suggests a favorable prognosis in the head and neck

“…This may be due to its presentation near vital structures and subsequent difficulty with surgical excision . The larynx, maxillary sinus, and mandible have the worst prognosis in this region, with death often due to advanced local disease rather than distant metastasis . Due to the rarity of this tumor, most of the data on its prevalence and treatment outcomes are based on small retrospective studies or management of similar sarcomas.…”

Maxillary undifferentiated high-grade pleomorphic sarcoma: A case report and review of the literature

“…[2,8] It is a soft tissue sarcoma composed of undifferentiated mesenchymal cells. [1] It usually occurs in extremities, being more common in lower extremities. [4] Its location in the head and neck is rare, with an incidence of 4% to 10%.…”

“…The most frequent location in head and neck is the neck and parotid gland, followed by the scalp, face, anterior skull base and orbit, there are few reports in upper aerodigestive tract, lateral skull base and ears. [1,7,9,11] Their presence in oral cavity is extremely rare. There have been reports of predisposing factors such as history of recurrent trauma, surgery, radiation, fractures, osteonecrosis, Paget's disease, non-ossifying fibroma and fibrous dysplasia.…”

“…[20] Combination therapy is critical to success, being the 5-year survival of 50%-60%. [1] 4. CONCLUSION Undifferentiated high-grade pleomorphic sarcoma of oral cavity is a rare neoplasm of mesenchymal cells in head and neck.…”

“…[1] They represent a diagnosis of exclusion. [2,3] They correspond to 5% of soft tissue sarcomas in adults, occurring mainly in the lower extremities in patients during the sixth and seventh decade of life.…”