International Clinical and Medical Case Reports Journal (ISSN: 2832-5788) | Volume 3, Issue 6 | Case Report | Open Access DOI

Clivus Undifferentiated Pleomorphic Sarcoma. A Case Report

Dr. Martha Lilia Tena-Suck*

Pedro Duran1, Christian Edward Sánchez-Sánchez2, Eliezer Villanueva3, Citlaltepetl Salinas-Lara4, Carlos Sanchez-Garibay5, Martha Lilia Tena-Suck6*

1Department of Neuropathology. National Institute of Neurology and Neurosurgery, Mexico

2Imagen Service. National Institute of Neurology and Neurosurgery, México

3Service of Neurosurgery. National Institute of Neurology and Neurosurgery, México

4Department of Neuropathology. National Institute of Neurology and Neurosurgery, México

5Department of Neuropathology. National Institute of Neurology and Neurosurgery, México

6Department of Neuropathology. National Institute of Neurology and Neurosurgery, México

*Correspondence to: Dr. Martha Lilia Tena-Suck 

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Abstract

Undifferentiated pleomorphic sarcoma, previously called malignant fibrous histiocytoma, is a prevalent tumor, and it is usually associated with trauma and radiotherapy. They are found anywhere in the body, but those reported with localization at the skull base are very rare. The case of a 19-year-old girl who began with headache, vertigo, dysphagia to solids and liquids, and dysphonia, with a deviation of the tongue to the right, loss of balance to the right, and incontinence of urinary sphincters, is presented. A brain computed tomography was performed, which showed a lesion in the clivus with a diagnosis of chordoma. She underwent surgery and a small tissue fragment for intraoperative crushed study was performed and reported as a malignant neoplasia suggestive of sarcoma. In the definitive study, fibrohistiocytic neoplasia formed irregular bundles with a storiform appearance, identifying large atypical giant pleomorphic cells that show cellular inclusions. Immunohistochemistry was positive for Vimentin, alpha 1-antitrypsin, fascin, CD68, and focally in fibrillar pattern for smooth muscle actin and CD34. Based on the histological and IHC findings, an undifferentiated pleomorphic sarcoma was diagnosed. Very few cases of undifferentiated pleomorphic sarcoma in the skull base have been reported.

Keywords:

Undifferentiated pleomorphic sarcoma; Malignant fibrous histiocytoma; Mesenchymal stromal or stem cells; Clivus tumors

Citation:

Duran P, Sánchez-Sánchez CE, Villanueva E, Salinas-Lara C, Sanchez-Garibay C, Tena-Suck ML. Clivus Undifferentiated Pleomorphic Sarcoma. A Case Report. Int Clinc Med Case Rep Jour. 2024;3(6):1-13.