A Rare Orbital Pathology: A Large Orbital Dermatofibrosarcoma Protuberans. 2022

Adar Aslan Kaya, and Zeynep Gürsel Özkurt, and Aylin Gül, and Mustafa Nacir
Sultanbeyli State Hospital, Clinic of Ophthalmology, İstanbul, Turkey.

Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma of the dermis. It is a malignant, locally aggressive, and infiltrative tumor with frequent recurrence. In this case, a 44-year-old woman presented with a 15-year history of a swelling in the medial canthus of the right eye that caused tearing. Imaging revealed a septated mass isodense to soft tissue that had eroded the medial wall of the orbit. Macroscopic examination showed an elastic, gray-brown, encapsulated, irregular mass measuring 45x35x22 mm. The surgical margins were positive, so adjuvant radiotherapy was started. The patient was followed for 2 years without recurrence. According to the literature, this mass is the largest orbital DFSP treated by globe-sparing primary resection.

UI MeSH Term Description Entries
D007765 Lacrimal Apparatus The tear-forming and tear-conducting system which includes the lacrimal glands, eyelid margins, conjunctival sac, and the tear drainage system. Lacrimal Gland,Nasolacrimal Apparatus,Conjunctival Sacs,Lacrimal Ducts,Lacrimal Punctum,Lateral Canthus,Medial Canthus,Apparatus, Lacrimal,Apparatus, Nasolacrimal,Canthus, Lateral,Canthus, Medial,Conjunctival Sac,Duct, Lacrimal,Gland, Lacrimal,Lacrimal Duct,Lacrimal Glands,Lacrimal Punctums,Punctum, Lacrimal,Sac, Conjunctival
D009915 Orbit Bony cavity that holds the eyeball and its associated tissues and appendages. Eye Socket,Eye Sockets,Orbits,Socket, Eye,Sockets, Eye
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D018223 Dermatofibrosarcoma A sarcoma of the deep layers of the skin. The tumors are locally aggressive tends to recur but rarely metastatic. It can be classified into variants depending on the cell type tumors are derived from or by its characteristics: Pigmented variant from MELANIN-containing DERMAL DENDRITIC CELLS; Myxoid variant, myxoid STROMAL CELLS; Giant cell variant characterized by GIANT CELLS in the tumors; and Fibrosarcomatous variant chracterized by tumor areas histologically indistinguishable from FIBROSARCOMA. Bednar Tumor,Bednar's Tumor,DFSP, Fibrosarcomatous,DFSP, Juvenile,DFSP, Myxoid,DFSP, Pigmented,Darier-Ferrand Tumor,Darier-Hoffmann Tumor,Dermatofibrosarcoma Protuberan,Dermatofibrosarcoma Protuberans, Familial,Dermatofibrosarcoma Protuberans, Fibrosarcomatous,Dermatofibrosarcoma Protuberans, Giant Cell,Dermatofibrosarcoma Protuberans, Myxoid,Dermatofibrosarcoma Protuberans, Pigmented,FS-DFSP,Familial Dermatofibrosarcoma Protuberans,Fibrosarcomatous Dermatofibrosarcoma Protuberans,Giant Cell Fibroblastoma,Giant Dermatofibrosarcoma Protuberans,Metastatic Dermatofibrosarcoma Protuberans,Bednars Tumor,Cell Fibroblastoma, Giant,Cell Fibroblastomas, Giant,DFSPs, Fibrosarcomatous,DFSPs, Juvenile,DFSPs, Myxoid,DFSPs, Pigmented,Darier Ferrand Tumor,Darier Hoffmann Tumor,Dermatofibrosarcoma Protuberan, Familial,Dermatofibrosarcoma Protuberan, Fibrosarcomatous,Dermatofibrosarcoma Protuberan, Giant,Dermatofibrosarcoma Protuberan, Metastatic,Dermatofibrosarcoma Protuberan, Myxoid,Dermatofibrosarcoma Protuberan, Pigmented,Dermatofibrosarcoma Protuberans,Dermatofibrosarcoma Protuberans, Giant,Dermatofibrosarcoma Protuberans, Metastatic,Dermatofibrosarcomas,Familial Dermatofibrosarcoma Protuberan,Fibroblastoma, Giant Cell,Fibroblastomas, Giant Cell,Fibrosarcomatous DFSP,Fibrosarcomatous DFSPs,Fibrosarcomatous Dermatofibrosarcoma Protuberan,Giant Cell Fibroblastomas,Giant Dermatofibrosarcoma Protuberan,Juvenile DFSP,Juvenile DFSPs,Metastatic Dermatofibrosarcoma Protuberan,Myxoid DFSP,Myxoid DFSPs,Myxoid Dermatofibrosarcoma Protuberan,Myxoid Dermatofibrosarcoma Protuberans,Pigmented DFSP,Pigmented DFSPs,Pigmented Dermatofibrosarcoma Protuberan,Pigmented Dermatofibrosarcoma Protuberans,Protuberan, Dermatofibrosarcoma,Protuberan, Familial Dermatofibrosarcoma,Protuberan, Fibrosarcomatous Dermatofibrosarcoma,Protuberan, Giant Dermatofibrosarcoma,Protuberan, Metastatic Dermatofibrosarcoma,Protuberan, Myxoid Dermatofibrosarcoma,Protuberan, Pigmented Dermatofibrosarcoma,Protuberans, Dermatofibrosarcoma,Protuberans, Familial Dermatofibrosarcoma,Protuberans, Fibrosarcomatous Dermatofibrosarcoma,Protuberans, Giant Dermatofibrosarcoma,Protuberans, Metastatic Dermatofibrosarcoma,Protuberans, Myxoid Dermatofibrosarcoma,Protuberans, Pigmented Dermatofibrosarcoma,Tumor, Bednar,Tumor, Bednar's,Tumor, Darier-Ferrand,Tumor, Darier-Hoffmann

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