Primary extradural leiomyosarcoma involving cavernous sinus in an immunocompetent patient

Abstract

C A S E R E P O R Tcranial nerve palsy. Routine laboratory studies were within normal limits. Serology was negative for HIV. Her magnetic resonance imaging brain [ Figures 1 and 2] revealed an isointense lesion on T2-weighted images with brilliant enhancement on contrast involving right middle cranial fossa base with cavernous sinus involvement and destruction of petrous bone. A possibility of skull base meningioma was considered. Operative detailsPatient underwent right temporal craniotomy, zygomatic osteotomy, interdural approach and tumor decompression under intra-operative neuronavigation. Intra-operatively, tumor was soft to firm, highly vascular, and extra-axial. The portion extending into the cavernous sinus was left behind due to significant bleeding. Resected tumor was sent for histopathology examination. Pathology findingsHistological examination revealed a tumor with pushing borders that was adhered to the nonneoplastic nerve bundle. Tumor was composed of spindle shaped cells that were arranged in interweaving fascicles. The tumor cells had cigar shaped nuclei with pointed ends and indistinct cytoplasmic borders [ Figure 3a and b]. There was brisk mitotic activity with several bizarre and giant nuclei. The stroma of tumor was richly vascular with areas of hyalinization. On immunohistochemistry (IHC), the tumor cells were positive for desmin, smooth muscle actin and vimentin [ Figure 4a]. The tumor cells were negative for S-100, epithelial membrane antigen (EMA), glial fibrillary acidic protein and CD117. Ki-67 staining revealed a high proliferation index of 25-30% [ Figure 4b].

Low-grade Leiomyosarcoma of the Cavernous Sinus in an HIV Positive Patient: Case Report

“…According to Zhang et al report, out of 37 patients, 18 (48.6%) had the dura as the tumor's site of origin, 10 (27.0%) had it in the convex set, five (13.5%) at the base of the skull and three (8.1%) in the tentorium cerebelli [8]. In the available reports we only found two patients who had, as in our case, the cavernous sinus as a site of origin [10,11]. This is particularly important because it limits the possibilities of treatment, since of these three patients, one was treated exclusively with 15 Gy radiosurgery in five fractions [11], and the other reported patient, like ours, was taken to surgery but a complete resection was not achieved [10].…”

“…In the available reports we only found two patients who had, as in our case, the cavernous sinus as a site of origin [10,11]. This is particularly important because it limits the possibilities of treatment, since of these three patients, one was treated exclusively with 15 Gy radiosurgery in five fractions [11], and the other reported patient, like ours, was taken to surgery but a complete resection was not achieved [10].…”

Giant primary intracranial multi-fossa leiomyosarcoma involving the frontal sinus, ethmoid air cells, anterior fossa, middle fossa, and intraventricular space: A case report and literature review

“…[ 38 ] Table 1 illustrates a cumulative literature review of LMS as well as the unique manifestation of LMS in the ethmoid air cells and frontal sinus within the context of an immunocompetent patient presented in this case report. [ 1 , 3 , 5 , 6 , 8 , 10 , 13 , 16 , 19 , 20 , 22 - 24 , 26 , 29 , 33 , 35 - 37 ]…”

“…[38] Table 1 illustrates a cumulative literature review of LMS as well as the unique manifestation of LMS in the ethmoid air cells and frontal sinus within the context of an immunocompetent patient presented in this case report. [1,3,5,6,8,10,13,16,19,20,[22][23][24]26,29,33,[35][36][37] Examination of Table 1 highlights the fact that no reported LMS has been reported with the same spatial distribution. Furthermore, the total number of reported primary intracranial LMS is 52 and the average age of these patients was 41.98 ± 17.99 years.…”