case_images: PMC10864579_01
case_images: Enhanced computed tomography (CT) results: (A) CT before treatment showed a 8.6*11.4*12.4 cm tumor in pelvic, prostate, seminal vesicle gland and bladder was squeezed; (B) Follow-up CT examination at 5 weeks after oral imatinib therapy showed that the tumor volume doubled in size (10.0*12.4*20.5cm), rectum squeezed newly; (C) Follow-up CT examination at 1 weeks after laparoscopic ileostomy showed that no growth in tumor size, with overfilled bladder and massive blood accumulation; (D) Follow-up CT examination at 3 months after total pelvic organ resection showed multiple hypodense nodules and masses in the liver S4/8, larger ones (14.4*12.5*11.6 cm).
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case_images: During clinical examination, a large mass measuring 8.6cm*11.4cm*12.4 cm ( Figure 1A ) was seen in the pelvic cavity. TPM3-NTRK1 was found in the results with 41.59% mutation abundance, and this result was validated by IHC ( Figure 3L ) and Sanger sequencing ( Supplementary Figure 1 ). The TPM3-NTRK1 fusion involving exons 1-6 of TPM3 and exons 11-17 of NTRK1 ( Supplementary Figure 1B  show the fusion mode). The results suggested that the patient may benefit from targeted agents such as entratinib, larotinib, lopatinib and sitravatinib.
case_images: Histopathological features: (A) Large mass removed by total pelvic resection. (B) Overall the histopathology. (C) The tumor was composed of ovoid or spindle-shaped cells, arranged in a bundle or woven pattern (HE-100X). (D) There are some distinct areas of interstitial collagen fiber proliferation, some of which are visible as hemorrhagic necrosis (HE-100X).
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case_images: Total pelvic resection specimen showed a huge mass (29cm*17cm*11cm) in the pelvis, the cut surface showed cystic solid mass, tough texture, focal necrotic pattern ( Figure 2A ). Microscopically, the tumor cells were more uniformly short spindle-shaped and diffusely distributed, with high cell density and infiltrative growth ( Figure 2B ). Some of the tumor had vacuolated cytoplasm, and the tumor cells were obviously heterogeneous, with large deep-stained nuclei and a mitotic rate of >5/10 high-power fields ( Figure 2C ). Additionally, interstitial collagen fibers were obviously proliferated, and hemorrhagic necrosis was seen in some areas ( Figure 2D ).
case_images: IHC markers staining of the surgical tissue cells showed (A) Ki-67 proliferation index in most of the tumor areas was around 60%, (B, C) Vimentin and CDK4 positivity, (D, E) CD34 and SMA partial positivity, (F-K) ALK, EMA, S100, Desmin, CD117 and DOG1 protein negative expression, (L) Pan-TRK positive expression.
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case_images: The IHC analysis of surgical specimen showed that the proliferation index (Ki67) is roughly 60% ( Figure 3A ). Besides, Vimentin and CDK4 were positive in the case ( Figures 3B, C ), while the expression of CD34, S100, Dog1, Desmin, CD117, MDM2, SMA, EMA and ALK were all lost in the tumor ( Figures 3D-K ). TPM3-NTRK1 was found in the results with 41.59% mutation abundance, and this result was validated by IHC ( Figure 3L ) and Sanger sequencing ( Supplementary Figure 1 ).