Undifferentiated Sarcoma of the Sellar Region

Abstract

Malignancies lacking specific features of cellular maturation are termed "undifferentiated" and represent 5-10% of all human tumors. They are encountered at a variety of sites but do not, as a rule, arise in the sellar region. A 39-year-old male with a history of testicular seminoma and an unsuccessful biopsy of a third ventricular neoplasm, presented with visual disturbances and memory loss. Light microscopically, the tumor consisted entirely of undifferentiated spindle cells. No germ cell component was noted. An exhaustive immunohistochemical study found immunoreactivity for vimentin and desmin, but for no other myoid markers. Polymerase chain reaction showed no X;18 translocation. Based upon these studies, a diagnosis of "undifferentiated sarcoma" was made. Our case, being highly unusual among reported sellar neoplasms, underscores the difficulties inherent in the differential diagnosis of undifferentiated neoplasms.

Primary undifferentiated spindle-cell sarcoma of sella turcica: successful treatment with adjuvant temozolomide

“…To the best of our knowledge, our case represents the second reporting of a sellar undifferentiated spindle cell sarcoma. Interestingly, in the previous report of undifferentiated sellar spindle cell sarcoma, a consideration was that the tumour in some way may have been related to the patient’s previous testicular seminoma, either as a metastasis or as a separate de novo lesion 16. Our case represented a true form of primary intrasellar undifferentiated tumour, as the patient had no history of malignancy and his PET scan did not show any evidence of other tumours.…”

“…Our extensive review of the Pubmed and Medline literatures have revealed only three case reports to date of primary intrasellar spindle cell sarcomas without prior radiation exposure 2 15 16. Two of these reports identified the primary sellar tumour as fibrosarcoma,2 15 and one report was of an undifferentiated sarcoma 16. That one case represented the first sellar example of an undifferentiated spindle cell sarcoma in medical literature, which showed desmin reactivity alone, without other immunohistochemical or ultrastructural features of rhabdomyosarcoma 16.…”

“…Our extensive review of the Pubmed and Medline literatures have revealed only three case reports to date of primary intrasellar spindle cell sarcomas without prior radiation exposure 2 15 16. Two of these reports identified the primary sellar tumour as fibrosarcoma,2 15 and one report was of an undifferentiated sarcoma 16.…”

“…Two of these reports identified the primary sellar tumour as fibrosarcoma,2 15 and one report was of an undifferentiated sarcoma 16. That one case represented the first sellar example of an undifferentiated spindle cell sarcoma in medical literature, which showed desmin reactivity alone, without other immunohistochemical or ultrastructural features of rhabdomyosarcoma 16. Our case exhibited histopathological features of a poorly differentiated spindle cell neoplasm which showed largely non-reactive immunohistochemistry profile except BCL-2 positivity, suggesting an undifferentiated sarcoma possibly of mesenchymal origin.…”

“…In both the cases of primary sellar fibrosarcoma, chemotherapy was not used as an adjuvant, as its role for these tumours was not well-defined 2 15. In the case of undifferentiated sellar sarcoma previously described, the patient underwent subtotal tumour resection and radiotherapy and was planned for chemotherapy (mesna, adriamycin, ifosfamide); however, post-therapy follow-up was not provided16 The patient in our case, who had undifferentiated sellar sarcoma, was treated with subtotal resection, postoperative radiotherapy and chemotherapy with temozolomide. To the best of our knowledge, this is the second report of primary sellar undifferentiated spindle cell sarcoma and the first report of the use of temozolomide in this setting.…”