PMC9638724_01
Coronal (A) and axial (B) contrast-enhanced CT images show a large pelvic tumor, pushing the uterus to the left (arrow). The tumor is mostly necrotic, especially on its upper part, presenting with peripheric solid enhancing areas (*).
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The abdominal and pelvic computed tomography (CT) featured a large heterogeneous pelvic tumor, mostly necrotic, presenting with peripheric solid enhancing areas (Fig. 1).
Sagittal (A) and coronal (B) T2-weighted images show a large heterogeneous pelvic tumor (*) anterior to the uterus (arrow). Pelvic ascites is depicted (arrowhead).
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It revealed an ill-defined, voluminous, and intraperitoneal pelvic tumor, arising from the right parametrium, measuring 24 x 15 x 21 cm of greatest diameters (Figs. 2 and 3).
(A, B) Axial T2-weighted images shows an ill-defined heterogeneous tumor in the pelvis. The tumor features central cystic areas (*) consistent with necrosis and solid peripheral nodules with intermediate signal (arrow).
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It revealed an ill-defined, voluminous, and intraperitoneal pelvic tumor, arising from the right parametrium, measuring 24 x 15 x 21 cm of greatest diameters (Figs. 2 and 3).
Axial diffusion-weighted image b-1000 (A) showing peripheral solid areas (arrow) with high signal intensity and corresponding low signal intensity on apparent diffusion coefficient (ADC) map (B), compatible with restriction.
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T2-W images. The solid areas featured diffusion restriction (Fig. 4) and avid contrast enhancement (Fig. 5). The lower part of the tumor was almost completely solid, presenting with avid enhancement on contrast-enhanced sequences.
Time signal-intensity curve of the solid areas showed a type 3 pattern (rapid rising followed by plateau), suggesting malignancy.
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A time signal-intensity curve was performed, revealing a type 3 pattern (rapid rising followed by plateau) (Fig. 6), suggestive of malignancy.
(A) The tumor is arranged in fascicles with a marbled appearance due to alternating hypocellular and hypercellular areas. (H&E); (B) The neoplasm is composed of neural fusiform cells and rhabdomyoblasts with large vesicular nuclei with nucleoli and abundant deeply eosinophilic cytoplasm. Mitotic activity is noted. (H&E); (C) Tumor cells demonstrating nuclear loss of H3K27me3. (H3K27me3 antibody); (D) Rhabdomyoblasts showing a strong nuclear myogenin expression. (myogenin antibody).
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Histologically, the tumor showed alternating hypercellular and hypocellular areas (Fig. 7A). It was composed of fusiform cells and rhabdomyoblasts with a large vesicular nuclei and abundant deeply eosinophilic cytoplasm (Fig. 7B). Immunohistochemical analysis showed diffuse nuclear loss of H3K27me3 and myogenin staining in rhabdomyoblasts (Figs. 7C and D).
Representative image of interphase FISH analysis with Vysis LSI CDKN2A SpectrumOrange/VysisCEP9 SpectrumGreen Probes showing CDKN2A gene deletion. White arrows indicate nuclei with just one copy of CDKN2A probe e 2 copies of the control probe for centromere of chromosome 9 (CEP9) and yellow arrows indicate nuclei with just one copy of CDKN2A probe and 3 copies of CEP9 probe.
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Complementary FISH analysis was performed, revealing deletion on CDKN2A gene (Fig. 8).