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  P22 - Mesothelioma, Thymoma and Other Thoracic Malignancies - Case Reports (ID 136)

  • Event: WCLC 2020
  • Type: Posters
  • Track: Mesothelioma, Thymoma and Other Thoracic Malignancies
  • Presentations: 1
  • Moderators:
  • Coordinates: 1/28/2021, 00:00 - 00:00, ePoster Hall

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    P22.01 - A Case of Intimal Sarcoma with Osteosarcomatous Differentiation (ID 859)

    00:00 - 00:00  |  Presenting Author(s): Etsuko Satoh
    • Abstract
    • Slides

    Introduction
    

    Intimal sarcoma is very rare malignant mesenchymal tumor, which originates from the intima of large blood vessels. Histologically, it often shows areas with rhabdomyosarcomatous or osteosarcomatous differentiation. Intimal sarcoma is associated with poor prognosis. In addition, standard treatment for this disease is not fully established in either adjuvant or metastatic setting. Here we report a case of intimal sarcoma with osteosarcomatous differentiation.

    Methods
    

    A 25-year-old woman was taken by ambulance to Juntendo University Hospital due to dyspnea on exertion, hyposthenia, and fainting. She was initially diagnosed as neurally mediated syncope. One month later, her dyspnea on exertion worsened. She was immediately admitted to the hospital. Contrast-enhanced CT (CE CT) showed tumor in her pulmonary arteries, and emergency surgery was performed. She was diagnosed as sarcoma through intraoperative rapid pathological diagnosis. She was referred to our hospital for postoperative adjuvant chemotherapy. Her left arm was swollen at her first visit, because she had thrombus from her left brachiocephalic vein to her subclavian vein and to her internal jugular vein. After the operation, her CE CT and positron emission tomography did not show any tumor but thrombus. Her tumor was localized in the lumen of pulmonary artery. Later, histopathological results showed the proliferation of spindle cells with tumor osteoid and chondroid formation. The final diagnosis was “intimal sarcoma with osteosarcomatous differentiation (MDM2 amplification was negative) arising from pulmonary artery.”

    Results
    

    Standard therapy for intimal sarcoma is not established; however, as her tumor showed osteosarcomatous differentiation, we treated her with cisplatin and doxorubicin, recommended as category 1 for the first-line therapy by NCCN clinical practice guidelines in oncology, bone cancer. Before starting adjuvant chemotherapy, she underwent oocyte cryopreservation. Treatment regimen was cisplatin 100 mg/m²/5 hours day 1, doxorubicin 30 mg/m²/24 hours day 1 and day 2. From day 2 to day 4 during the first course, she had grade 3 nausea; from day 3 she had grade 1 ototoxicity and grade 2 tinnitus. At day 7, her ototoxicity was worsened to grade 3. From the next course, we reduced cisplatin dose from 100 mg/m² to 60 mg/m². Since then, her ototoxicity has not worsened. She completed 4 courses as adjuvant chemotherapy.

    Conclusion
    

    We experienced a case of intimal sarcoma with osteosarcomatous differentiation. The detailed pathological diagnosis, the appropriate choice of adjuvant chemotherapy regimen, and the consideration for adolescence and young adulthood (AYA) were all important. We treated her with cisplatin and doxorubicin based on her pathological diagnosis as intimal sarcoma with osteosarcomatous differentiation. If her tumor had not had osteosarcomatous differentiation, we would not have treated her with this regimen. However, rationality of this strategy requires to be further clinical investigation. Especially because of her AYA status, we gave extra consideration to oocyte cryopreservation before chemotherapy.

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