title: Histiocytic sarcoma of the cavernous sinus: case report and literature review.
abstract: Histiocytic sarcoma is a rare malignant neoplasm of hematopoietic origin composed of cells showing morphologic and immunophenotypic evidence of histiocytic differentiation. We describe the 2nd case of primary histiocytic sarcoma of the cavernous sinus/Meckel's cave, and the 8th case involving the CNS. A 61-year-old Caucasian man presented with numbness on the entire left side of his face, shooting pain in the left frontal region, and headaches. Imaging revealed an enhancing extra axial soft tissue mass located in the left cavernous sinus and left Meckel's cave. Diagnosis was established through open biopsy, after failed attempts via CT-guided trans-foramen ovale fine-needle aspiration biopsy and keyhole biopsy. The tumor was composed of large non-cohesive epithelioid cells invading nerves and ganglion cells. Tumor cells were immunopositive for CD68, CD163, and immunonegative for the anaplastic large cell lymphoma marker ALK-1 as well as other lymphoid, myeloid, and dendritic cell markers. Histiocytic sarcoma has strong potential for systemic spread; early diagnosis and treatment are important. Our patient was initially treated with radiation therapy but subsequently developed metastases.
authors.affiliation: Department of Neurological Surgery, University of Virginia Health System, Charlottesville, VA, USA, cc5hx@virginia.edu.
authors.forename: Ching-Jen
authors.initials: CJ
authors.lastname: Chen
authors.forename: Erik A
authors.initials: EA
authors.lastname: Williams
authors.forename: Taylor E
authors.initials: TE
authors.lastname: McAneney
authors.forename: Brian J
authors.initials: BJ
authors.lastname: Williams
authors.forename: James W
authors.initials: JW
authors.lastname: Mandell
authors.forename: Mark E
authors.initials: ME
authors.lastname: Shaffrey
mesh_terms: D030301:Activin Receptors, Type II / Q000032:analysis; D015703:Antigens, CD / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D044963:Biopsy, Fine-Needle; D002426:Cavernous Sinus; D016326:Extracellular Matrix Proteins / Q000032:analysis; D054747:Histiocytic Sarcoma / Q000175:diagnosis* / Q000473:pathology / Q000532:radiotherapy; D006801:Humans; D018960:Hyaluronan Receptors / Q000032:analysis; D061705:Image-Guided Biopsy; D008297:Male; D008875:Middle Aged; D019043:Vascular Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000532:radiotherapy; D000097382:CD68 Molecule
chemical_list: D030301:Activin Receptors, Type II; D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D014408:Biomarkers, Tumor; D016326:Extracellular Matrix Proteins; D018960:Hyaluronan Receptors; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C098946:hyaluronan-mediated motility receptor; C425364:ACVRL1 protein, human