PMC9986328_01
(A) A round mass in the right lower lobe adjacent to the bronchus, with a well-defined boundary and thickened adjacent lung texture (blue arrow). (B) A mediastinal window on the same plane as the image above shows a round soft tissue mass adjacent to the bronchus in the lower lobe of the right lung, with a well-defined boundary and thickened adjacent lung texture (blue arrow). (C) Contrast-enhanced mediastinal window scan on the same plane shows obvious enhancement of a round soft tissue mass adjacent to the bronchus in the lower lobe of the right lung, with uniform enhancement, a well-defined boundary, no burr or satellite foci, and an irregular soft tissue mass near the lung texture (blue arrow). (D) Coronal reconstruction, showing irregular density shadow in the lower lobe of the right lung with a well-defined boundary, surrounding the bronchial branches of the lower lobe. (E) PET-CT: soft tissue mass in the right middle bronchus, the right lower lobe bronchus, the basal trunk, and the medial basal segmental bronchus, along with elevated FDG metabolism and combined with pathology results, this led soft tissue sarcoma to be considered. FDG: fluorodeoxyglucose; PET-CT: Positron emission tomography/computed tomography.
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F1
On postoperative day 2, the chest was examined by enhanced CT (Figures 1A-D): (1) the middle bronchus of the right lung changed postoperatively, with the wall being slightly rough, and the upper portion of the lower bronchus was visible; (2) multiple paratracheal nodules in the lower lobe of the right lung were found, so enlarged lymph nodes were considered; (3) emphysema and inflammation in the middle and lower lobes of the right lung and local thickening of the right lung pleura were observed. Positron emission tomography/computed tomography (PET-CT) (Figure 1E) revealed a soft tissue mass in the lumen of the right middle bronchus, right lower lobe bronchus, basal trunk, and medial basal segmental bronchus, and increased fluorodeoxyglucose (FDG) metabolism; given these findings combined with pathology results, soft tissue sarcoma was considered.
(A,B) (HE staining x100) Tumor cells are arranged in a nest-like and alveolar manner, with abundant cytoplasm, some of which are eosinophilic and transparent. The nuclei are round in shape with obvious nucleoli and abundant blood supply in the tumor. (C) (Immunohistochemical TFE3 x200) Tumor cells TFE3 positive. (D) (Immunohistochemical CD56 x200) The cytoplasm and membrane of tumor cells positive. (E) (Next-generation sequencing) TFE3 rearrangement, ASPSCR1-TFE3 fusion.
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F2
Postoperative pathology (Figures 2A,B) revealed a grayish-white mass with a size of 1.8 cm x 1.5 cm x 1 cm in the section of the lower lobe of the right lung. CD56 + (Figure 2D), SMA -, CK (Pan) -, EMA -, SALL4 -, MyOD1 -, presence of INI1, desmin -, synaptophysin -, S-100 partial +, Ki-67 close to 10% +, MelanA -, CgA -, PHOX2B -. Combining these findings with morphology and immunohistochemistry, ASPS was first considered; no tumor metastasis was observed. Next-generation sequencing (Figure 2E, on August 26, 2020) revealed TFE3 rearrangement and ASPSCR1-TFE3 gene fusion.
Treatment flow chart. Chemotherapy1-4: vindesine 3 mg/m2, d1 + cyclophosphamide 1.2 g/m2, d1 + epirubicin37.5 mg/m2, d1-d2 (first cycle, May 11-May 12); ifosfamide 1.8 g/m2, d1-d5 + etoposide 100 mg/m2, d1-d5 (second cycle, June 3-June 7); vindesine 3 mg/m2, d1 + cyclophosphamide 1.2 g/m2, d1 + epirubicin 37.5 mg/m2, d1-d2 (third cycle, June 27-June 28); paclitaxel 260 mg/m2, d1 + cisplatin 20 mg/m2, d2-d5 + ifosfamide 1.2 g/m2, d2-d5 (fourth cycle, July 21-July 25). Chemotherapy5: ifosfamide 1.5 g/m2, d1-d5 + irinotecan 90 mg/m2, d1-d3 + carboplatin 450 mg/m2, d2 (fifth cycle: August 21-August 25). Chemotherapy6-12: irinotecan 90 mg/m2, d1-d3 + carboplatin 450 mg/m2, d2 (6th-12th cycles; September 18-September 20, October 15-October 17, November 6-November 8, November 27-November 29, December 25-December 27 of 2020; January 21-January 23, February 17-February 19 of 2021).
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F3
Subsequently, the 6th to 12th cycles of chemotherapy (irinotecan + carboplatin: September 18-September 20, October 15-October 17, November 6-November 8, November 27-November 29, and December 25-December 27 of 2020; January 21-January 23 and February 17-February 19 of 2021) were performed, for a total of 12 cycles of chemotherapy throughout the course of treatment (Figure 3) and a total of 28 cycles of radiotherapy (October 6-November 11, 2020).