Dermatofibrosarcoma protuberans. 1995

C K Koh, and C B Ko, and H P Bury, and E H Wyatt
Department of Dermatology, Hull Royal Infirmary, England.

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant tumor of the skin, the diagnosis of which can be difficult not only clinically but also histologically. The tumor has a low metastatic potential, but tends to recur locally if removal is incomplete. METHODS We reviewed the clinical and histologic features of 19 cases of DFSP, seen at the Department of Dermatology (14 cases) and Department of Surgery (5 cases) at the Hull Royal Infirmary between 1975 and 1992. RESULTS There were only 19 cases of DFSP diagnosed from a population of 500,000 over a period of 17 years. None of the cases were diagnosed clinically prior to histologic diagnosis. The highest incidence was in the 4th decade of age. The most frequent site was on the head and neck. None of the lesions metastasized but there were a total of eight incidents of local recurrence in five patients. Histologically all the cases showed presence of storiform-pattern spindle-shaped cells and new collagen formation. CONCLUSIONS Dermatofibrosarcoma protuberans is a soft tissue tumor that is often not recognized clinically due to its rarity and variable appearance. Local recurrence is frequent. A wide surgical excision is the treatment of choice.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D008938 Mitosis A type of CELL NUCLEUS division by means of which the two daughter nuclei normally receive identical complements of the number of CHROMOSOMES of the somatic cells of the species. M Phase, Mitotic,Mitotic M Phase,M Phases, Mitotic,Mitoses,Mitotic M Phases,Phase, Mitotic M,Phases, Mitotic M
D009361 Neoplasm Invasiveness Ability of neoplasms to infiltrate and actively destroy surrounding tissue. Invasiveness, Neoplasm,Neoplasm Invasion,Invasion, Neoplasm
D009364 Neoplasm Recurrence, Local The local recurrence of a neoplasm following treatment. It arises from microscopic cells of the original neoplasm that have escaped therapeutic intervention and later become clinically visible at the original site. Local Neoplasm Recurrence,Local Neoplasm Recurrences,Locoregional Neoplasm Recurrence,Neoplasm Recurrence, Locoregional,Neoplasm Recurrences, Local,Recurrence, Local Neoplasm,Recurrence, Locoregional Neoplasm,Recurrences, Local Neoplasm,Locoregional Neoplasm Recurrences,Neoplasm Recurrences, Locoregional,Recurrences, Locoregional Neoplasm
D002467 Cell Nucleus Within a eukaryotic cell, a membrane-limited body which contains chromosomes and one or more nucleoli (CELL NUCLEOLUS). The nuclear membrane consists of a double unit-type membrane which is perforated by a number of pores; the outermost membrane is continuous with the ENDOPLASMIC RETICULUM. A cell may contain more than one nucleus. (From Singleton & Sainsbury, Dictionary of Microbiology and Molecular Biology, 2d ed) Cell Nuclei,Nuclei, Cell,Nucleus, Cell
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003094 Collagen A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH). Avicon,Avitene,Collagen Felt,Collagen Fleece,Collagenfleece,Collastat,Dermodress,Microfibril Collagen Hemostat,Pangen,Zyderm,alpha-Collagen,Collagen Hemostat, Microfibril,alpha Collagen
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260 Female Females

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