ABSTRACT
Introducción. El fibroxantoma atípico (FXA) es un tumor poco frecuente de histogénesis incierta, considerado por la mayoría de los autores como la variante superficial del histiocitoma fibroso maligno (HFM). El objetivo principal de este trabajo es presentar las características clínicas e histológicas de una serie de 10 pacientes. Material y métodos. Los datos fueron recogidos retrospectivamente de la historia clínica informatizada. Se analizan variables clínicas (edad de aparición, tiempo hasta el diagnóstico, localización, patología acompañante, evolución), histológicas (patrón arquitectural, celularidad, ulceración, invasión vascular o perineural, afectación tejido celular subcutáneo, pleomorfismo, mitosis, infiltrado inflamatorio acompañante) e inmunohistoquímica. Casos clínicos. Las características clinicoepidemiológicas coinciden en general con las publicadas en la literatura especializada: edad avanzada de aparición, poco retraso hasta el diagnóstico, afectación de piel con daño actínico y buena evolución. Histológicamente todos los casos presentaban un predominio fusocelular, dispuesto en un patrón vagamente estoriforme junto con células gigantes y células poligonales eosinófilas. Discusión. El diagnóstico es siempre de exclusión, y deben diferenciarse mediante inmunohistoquímica de otros tumores fusocelulares como carcinomas epidermoides, melanomas, leiomiosarcomas o dermatofibrosarcoma protuberans entre otros. A pesar de ser un tumor poco frecuente es preciso conocerlo para evitar tratamientos agresivos e innecesarios
Introduction. Atypical fibroxanthoma (AFX) is a rare tumor of unknown histogenesis, considered by most authorities as a superficial form of malignant fibrous histiocytoma (MFH). The aim of this work is to report the clinicopathological features of 10 cases of AFX. Material and methods. Data were retrospectively collected of the computerized medical history. Clinical (age, onset-diagnosis time, location, accompanying pathology, outcome), histological (architectural pattern, cell type, ulceration, vascular or perineural invasion, subcutis involvement, pleomorphism, mitosis, inflammatory infiltrate) and immunohestochemical variable were analyzed. Cases report. Clinical and epidemiological features coincide with those previously reported: onset late in life, short time onset-diagnosis, involvement of skin with notable sun damage and a good outcome. Pathologically all the cases showed a spindle-cell prevalence arranged in a vaguely storiform pattern, along with both, multinucleated and eosinophilic cells. Discussion. The diagnosis of AXF is always of exclusion. Other spindle-cell tumors such as squamous cell carcinoma, malignant melanoma, leyomiosarcoma or dermatofibrosarcoma protuberans must be ruled out by immunohistochemical techniques. In spite of its rarity, the recognition of AFX is important in order to avoid inappropriately aggressive treatment
Subject(s)
Middle Aged , Male , Female , Adult , Humans , Immunohistochemistry/methods , Immunohistochemistry , Melanoma/diagnosis , Melanoma/etiology , Melanoma/pathology , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/surgery , Histiocytoma, Benign Fibrous/pathology , Histiocytoma, Benign Fibrous/epidemiology , Histiocytoma, Benign Fibrous/etiologyABSTRACT
INTRODUCTION: Atypical fibroxanthoma (AFX) is a rare tumor of unknown histogenesis, considered by most authorities as a superficial form of malignant fibrous histiocytoma (MFH). The aim of this work is to report the clinicopathological features of 10 cases of AFX. MATERIAL AND METHODS: Data were retrospectively collected of the computerized medical history. Clinical (age, onset-diagnosis time, location, accompanying pathology, outcome), histological (architectural pattern, cell type, ulceration, vascular or perineural invasion, subcutis involvement, pleomorphism, mitosis, inflammatory infiltrate) and immunohistochemical variable were analyzed. CASES REPORT: Clinical and epidemiological features coincide with those previously reported: onset late in life, short time onset-diagnosis, involvement of skin with notable sun damage and a good outcome. Pathologically all the cases showed a spindle-cell prevalence arranged in a vaguely storiform pattern, along with both, multinucleated and eosinophilic cells. DISCUSSION: The diagnosis of AXF is always of exclusion. Other spindle-cell tumors such as squamous cell carcinoma, malignant melanoma, leyomiosarcoma or dermatofibrosarcoma protuberans must be ruled out by immunohistochemical techniques. In spite of its rarity, the recognition of AFX is important in order to avoid inappropriately aggressive treatment.
