B Joel Tjarks1, Jennifer S Ko2,3, Steven D Billings2,3. 1. Department of Pathology, University of South Dakota - Sanford School of Medicine, Sioux Falls, South Dakota. 2. Department of Pathology, Cleveland Clinic, Cleveland, Ohio. 3. Department of Dermatology, Cleveland Clinic, Cleveland, Ohio.
Abstract
BACKGROUND: Myxofibrosarcoma classically presents as a painless mass in the proximal extremities. Cutaneous myxofibrosarcomas arising in the head and neck and distal extremities are extremely uncommon. We present a series of 6 cases of myxofibrosarcoma presenting in the head and neck and acral locations. METHODS: Archives were searched using the term "myxofibrosarcoma" over a 6-year period (2009-2015). The clinicopathologic features of myxofibrosarcoma were recorded. Cases in the head and neck or acral locations were retrieved. When available, the patient's medical records were reviewed. RESULTS: Ninety-five cases of myxofibrosarcoma were identified over a 6-year period. Six patients were identified with myxofibrosarcoma arising in the head (n = 4, M:F 3:1), hand (n = 1, F) and foot (n = 1, F). Each had typical features of myxofibrosarcoma. Two of the tumors on the head were high-grade and had multiple recurrences, while the remaining 2 were intermediate grade. Both acral tumors were intermediate grade and 1 recurred locally within a year of diagnosis. CONCLUSIONS: Myxofibrosarcoma may rarely involve the head and neck and acral locations, and presentation in these sites is a potential source of diagnostic difficulty. Recognition of the characteristic histologic features of myxofibrosarcoma in conjunction with judicial use of immunohistochemical stains should allow for accurate diagnosis.
BACKGROUND: Myxofibrosarcoma classically presents as a painless mass in the proximal extremities. Cutaneous myxofibrosarcomas arising in the head and neck and distal extremities are extremely uncommon. We present a series of 6 cases of myxofibrosarcoma presenting in the head and neck and acral locations. METHODS: Archives were searched using the term "myxofibrosarcoma" over a 6-year period (2009-2015). The clinicopathologic features of myxofibrosarcoma were recorded. Cases in the head and neck or acral locations were retrieved. When available, the patient's medical records were reviewed. RESULTS: Ninety-five cases of myxofibrosarcoma were identified over a 6-year period. Six patients were identified with myxofibrosarcoma arising in the head (n = 4, M:F 3:1), hand (n = 1, F) and foot (n = 1, F). Each had typical features of myxofibrosarcoma. Two of the tumors on the head were high-grade and had multiple recurrences, while the remaining 2 were intermediate grade. Both acral tumors were intermediate grade and 1 recurred locally within a year of diagnosis. CONCLUSIONS: Myxofibrosarcoma may rarely involve the head and neck and acral locations, and presentation in these sites is a potential source of diagnostic difficulty. Recognition of the characteristic histologic features of myxofibrosarcoma in conjunction with judicial use of immunohistochemical stains should allow for accurate diagnosis.
Authors: Chiara Pagnoni; Luca Improta; Rossana Alloni; Francesco Mallozzi Santa Maria; Irene Aprile; Beniamino Brunetti; Carlo Greco; Bruno Vincenzi; Alessandro Gronchi; Sergio Valeri Journal: J Med Case Rep Date: 2021-10-01