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Review
. 2022 Dec 21;13(1):9.
doi: 10.3390/diagnostics13010009.

A Rare Case of a Primary Leiomyoma of the Clivus in an Immunocompetent Patient and a Review of the Literature Regarding Clival Lesions

Jacek Kunicki  1 , Natalia Rzewuska  1   2 , Michał Kunicki  3 , Piotr Wiśniewski  4
Affiliations
Review

A Rare Case of a Primary Leiomyoma of the Clivus in an Immunocompetent Patient and a Review of the Literature Regarding Clival Lesions

Jacek Kunicki et al. Diagnostics (Basel). .

Abstract

Leiomyomas are common lesions that are usually located in the genitourinary and gastrointestinal tracts. Primary leiomyomas at the skull base are uncommon. They are composed of well-differentiated smooth muscle cells without cellular atypia. The diagnosis of a leiomyoma has to be confirmed by immunohistochemistry. The tumor tissue is immunoreactive for SMA, S100 and cytokeratin. Leiomyomas mainly occur in immunocompromised patients. Most tumor tissues are positive for EBV. The presented case is that of a 56-year-old immunocompetent woman with a tumor on the clivus. The radiological images suggested chordoma or fibrous dysplasia. Transnasal transsphenoidal surgery was performed. The tumor tissue consisted of well-differentiated smooth muscle cells with elongated nuclei. Immunohistochemistry revealed a positive reaction for desmin, SMA and h-Caldesmon and a negative reaction for S100, beta-catenin, PGR and Ki67. The leiomyoma diagnosis was subsequently established. To the best of our knowledge, the case of a primary leiomyoma on the clivus of an immunocompetent patient is the first to be described. We also extensively reviewed the literature on the immunohistopathological and radiological differential diagnosis of clival lesions.

Keywords: chordoma; clival diseases; clivus; fibrous dysplasia; primary leiomyoma.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
(A,B) The left and right ovaries, with their diameters. The right ovary measured 25 × 15 mm and the left ovary 24 × 14 mm. (C) The transverse plan of the uterus at its widest dimensions. In this plane, the uterine width (Ut-W) was measured. The uterus was of normal size in anterior flexion. (D) The midsagittal plane of the uterus showing the uterine fundus, myometrium, endometrium (6 mm thick), isthmus, cervix and cul-de-sac.
Figure 1
Figure 1
(A,B) The left and right ovaries, with their diameters. The right ovary measured 25 × 15 mm and the left ovary 24 × 14 mm. (C) The transverse plan of the uterus at its widest dimensions. In this plane, the uterine width (Ut-W) was measured. The uterus was of normal size in anterior flexion. (D) The midsagittal plane of the uterus showing the uterine fundus, myometrium, endometrium (6 mm thick), isthmus, cervix and cul-de-sac.
Figure 2
Figure 2
(A) The magnetic resonance postcontrast T1-weighted sagittal image, demonstrating the isointense lesion with hyperintense borders (arrow). The image suggested chordoma or fibrous dysplasia (B). The magnetic resonance T2-weighted dark fluid sagittal image, showing the isointense clival lesion (arrow) (C). The computed tomography image, showing the centrally located lesion with a hypersclerotic rim (arrow). The tumor did not infiltrate the sphenoid sinuses.
Figure 3
Figure 3
(A) The leiomyoma is composed of cells with elongated nuclei without cellular atypia (hematoxylin and eosin staining at a magnification of ×40 and ×200). (B) The immunohistochemistry for h-Caldesmon, which is positive in the leiomyoma cells (at a magnification of ×200). (C) The immunohistochemical staining for nuclear marker beta-catenin, which is negative in the leiomyoma cells (at a magnification of ×200). (D) The immunohistochemistry for smooth muscle antigen (SMA), which shows reactivity (at a magnification of ×200).
Figure 4
Figure 4
(A,B) The postoperative magnetic resonance T1-weighted and T2-weighted sagittal images and the postoperative magnetic resonance T2-weighted coronal image (C) showing some minimal residual lesions within the clival region (arrows).
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