PMC5836187_01
Pathological aspects of the pulmonary metastasis from the pulmonary artery intimal sarcoma. a Hematoxylin-eosin-saffron (HES) staining of the pulmonary biopsy showing a poorly differentiated necrotic lesion. x10. b The spindle tumour cells are severely atypical with numerous mitoses. HES. x40. c Immunohistochemistry staining showing expression of MDM2 by tumour cells. d FISH analysis showing numerous red signals in tumour cells revealing an amplification of the 12q15 region, which contains the MDM2 gene.
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F1
She was included in our high-throughput molecular screening trial (PERMED-01 trial, NCT02342158) and a pulmonary biopsy was performed, confirming the diagnosis of metastasis from the PAIS (Fig. 1) with MDM2 amplification, associated with CDK4 amplification.
Radiological response to carboplatin-vinorelbine chemotherapy. Chest CT scans during 4 successive periods of treatment with carboplatin-vinorelbine. a-d January 2017, before the 1st cycle. Four lesions are shown (red arrows): 2 intraparenchymal lesions (a, b), 1 lesion in contact with the second rib (c), and 1 lesion of the right posterior inframediastinal space (d). e-h April 2017, after 3 cycles. Regression of all 4 lesions. i-l June 2017, after 6 cycles. Continued regression of all 4 lesions. m-p October 2017, after 9 cycles. Stability of the intraparenchymal lesions (m, n), but progression of the inframediastinal lesion (p) and the lesion in contact with the second rib (o).
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F2
After 3 cycles (April 2017), a CT scan revealed objective regression of the subdiaphragmatic lesions (Fig. 2a-h). After six cycles (June 2017), the lesions further decreased at the mediastinal and pleuropulmonary levels, with stability of the other pulmonary lesions and no new lesion (Fig. 2i-l). After the 9th cycle, in October 2017, the PS was zero without any clinical symptom, but the CT scan showed disease progression with an increase in size of two lesions (Fig. 2m-p) and stability of the other lesions.