Metastatic Intimal Sarcoma of the Pulmonary Artery Sensitive to Carboplatin-Vinorelbine Chemotherapy: Case Report and Literature Review

Abstract

Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour with a very poor prognosis. In advanced stages, chemotherapy and radiotherapy are poorly efficient, and no standard chemotherapy guideline is currently available. Here, we report on a 37-year-old woman with PAIS initially treated with surgical resection who developed metastatic relapse refractory to anthracycline-based chemotherapy, then trabectedin, then pazopanib. The patient was then given carboplatin-vinorelbine chemotherapy. The treatment was well tolerated, and, rapidly, a CT scan showed an objective response that lasted 8 months despite the 4th therapeutic line. We review the literature and show that our case is the second one that provides evidence of the efficacy of platinum-vinorelbine regimens in this aggressive tumour.

Systemic treatments in MDM2 positive intimal sarcoma: A multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network

“…The outcome for InS patients is poor, with a reported median overall survival (mOS) in the range of 8 to 13 months . Retrospective data on the activity of systemic therapies in InS are limited, and no prospective studies have been conducted . This lack of knowledge is increasingly important today, as new potentially active treatments are emerging.…”

“…4,5 Retrospective data on the activity of systemic therapies in InS are limited, and no prospective studies have been conducted. 4,[6][7][8][9][10] This lack of knowledge is increasingly important today, as new potentially active treatments are emerging.…”

A Case of Unresectable Pulmonary Artery Intimal Sarcoma with Prolonged Survival by Chemotherapy

“…In addition, there have been case reports showing good response of pulmonary artery intimal sarcoma to vinorelbine-based regimens [15, 16]. These agents could have been alternative choices in our case.…”

Reirradiation of pulmonary artery intimal sarcoma: A case report

“…The patient described here presented with all the above symptoms but had been diagnosed with pulmonary hypertension 4 years prior. This misdiagnosis is quite common as PAS starts with nonspecific symptoms and it can be easily confused with pulmonary embolism; however, the onset of the symptoms is usually more gradual with PAS than pulmonary embolism . In our patient, this missed diagnosis led to a delay in therapy that could have affected outcomes.…”

“…Most PAS are derived from the embryologic bulbus cordis area, mainly from the pulmonary artery. Intimal sarcoma of the pulmonary artery (PAIS) is a rare tumor with an estimated incidence between 0.001% and 0.003%, which is likely an underestimate due to frequent misdiagnosis and late detection during surgery or autopsy . Median overall survival (OS) of approximately 17 months is reported in the literature for PAIS patients .…”