PMC9637945_01
MRI of the abdominal cavity and morphological assessment of the liver tumor biopsy (UESL; hematoxylin-eosin, HE). (A, B) Images reveal a massive cystic formation in the right lobe of the liver, encompassing the segments S1, S5, S7, S8, and partially S4 and S6, measured 19 x 18.8 x 19.3 cm (3580 cm3), with the signs of periportal infiltration. The inferior vena cava at the level of tumor mass is compressed and pushed aside. The right and middle hepatic veins, as well as the arteries, pass through the mass. The portal vein at the level of the bifurcation and its right branch pass through the mass. Classified as PRETEXT stage III C1, E0, H0, F1, N0, M0, P2, V2; (C) a T2-weighted image shows a solitary tumor node, 8 x 6 x 8 cm. Microscopically the lesion was presented by short bundles and sheets of spindle-shaped or irregularly shaped cells and collagenous stroma with myxomatous areas (D). Neoplastic cell exhibited moderate nuclear-cytoplasmatic ratio, nuclei varied in site and shape from ovoid to polygonad. The cytoplasm of tumor cell was eosinophilic or optically empty (E).
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Magnetic resonance imaging (MRI) of the abdominal cavity ( Figures 1A, B ) revealed a massive tumor in the right lobe of the liver, about 3580 cm3 and rapidly growing (by 441% in 2 months). A solitary node observed in the projection of the left adrenal gland at the time of the diagnosis was regarded as a metastasis to lymph nodes ( Figure 1C ). The tumor tissue was represented by short bundles and sheets of spindle-shaped or irregularly shaped cells with a moderate nuclear-cytoplasmic ratio; the tumor cells had nuclei of varying size and shaped from ovoid to polygonal, the chromatin slumpy or hyperchromic, the cytoplasm weakly eosinophilic or optically empty; the stroma presented with low collagen content and myxomatous areas ( Figures 1D, E ).
Angiographic imaging of the UESL-supplying vessels and MRI images showing favorable dynamics of the liver tumor volume. (A) Proximal portion of the right diaphragmatic artery supplying UESL; (B) distal portion of the right diaphragmatic artery supplying UESL; (C) hepatic artery proper before chemoembolization; (D) hepatic artery proper after chemoembolization, with the catheter left in place for chemoperfusion. (E) before the interventional procedures; (F) after the first session of chemoembolization; (G) after the second session of chemoembolization.
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The transcatheter oil chemoembolization applied a mixture of lipiodol and doxorubicin (50 mg) to fill the branches of the right diaphragmatic artery ( Figures 2A, B ) and the collateral arteries nourishing the tumor from the superior mesenteric artery. The procedure resulted in tumor response [a 20% reduction in the tumor volume according to computed tomography; ( Figures 2E, F )], which served an indication for the second session. The second chemoembolization used microspheres 200-400 microm in diameter loaded with oxaliplatin (50 mg) infused in the collateral arteries stemming from a branch of the right diaphragmatic artery, the superior mesenteric artery, and the right renal artery nourishing the sarcoma, and lipiodol with doxorubicin (50 mg) infused in the hepatic artery proper ( Figures 2C, D ). After the second session, the tumor decreased by another 55% to a volume of 2144 cm3, though the periportal infiltration persisted ( Figure 2G ); noteworthy, the solitary tumor node on the left, interpreted as a paraaortic lymph node metastasis, lacked significant dynamics.
Intraoperative appearance and morphological assessment of the retroperitoneal tumor node and mesenteric tumor foci. (A, B) The solitary tumor node in the retroperitoneum (MPNST); (C) metastatic foci in the mesentery (MPNST); (D) MPNST spindle-shaped cells with scanty cytoplasm with hyperchromatic; (E) MPNST cells showed retained H3K27Me expression; (F) diffuse immune reactivity for S-100.
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The patient was operated by "J" laparotomy; examination in the retroperitoneal space on the left revealed a tumor node 10 x 10 cm, hardly resembling a lymph node metastasis by gross appearance, removed surgically ( Figures 3A, B ). Intraoperative examination revealed multiple diffuse lesions in the mesentery of the small intestine, reaching 5.2 cm in diameter ( Figure 3C ); with regard to the baseline diagnosis of neurofibromatosis type 1, these lesions were preliminary identified as neurofibromas. The tumor also contained cellular elements with shapeless hyperchromic anaplastic nuclei and zones of chondroid differentiation ( Figure 3D ). TRK, and Myogenin ( Figures 3E, F ). Thereby, morphological examination confirmed the presence of synchronous tumor process identified as malignant peripheral neural sheath tumor (MPNST) grade 2 with peritoneal metastasis.