Pulmonary artery sarcoma: An important mimic of pulmonary embolism—Case reports and literature review

Abstract

Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low d ‐dimer, troponin T or NT‐proBNP, as well as characteristic findings on CT pulmonary angiography such as the ‘wall eclipsing sign’ and an non‐dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2‐year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.

A 5‐year survivor of endarterectomy for sclerosing undifferentiated intimal sarcoma of the pulmonary artery: Importance of clinical suspicion and careful histologic evaluation

“…Sclerosing PA sarcoma is a rare type which results from fibrosis or hyalinization 8 . The most effective way of improving short‐term survival has been surgical intervention 9 . Improved survival with this disease can be accomplished with early diagnosis and radical surgical resection, possibly with adjuvant chemotherapy and radiation 9 .…”

“… 8 The most effective way of improving short‐term survival has been surgical intervention. 9 Improved survival with this disease can be accomplished with early diagnosis and radical surgical resection, possibly with adjuvant chemotherapy and radiation. 9 Today, immunohistochemical evidence of MDM2 overexpression in tumor cells may aid in diagnosing intimal sarcoma.…”

“… 9 Improved survival with this disease can be accomplished with early diagnosis and radical surgical resection, possibly with adjuvant chemotherapy and radiation. 9 Today, immunohistochemical evidence of MDM2 overexpression in tumor cells may aid in diagnosing intimal sarcoma. 10 This case underwent PEA with 5 years‐survival.…”

Neoplastic Ruptured Cerebral Aneurysm Caused by Intimal Sarcoma: A Case Report

“…Although total resection is recommended, it is difficult because the lesion is often the pulmonary artery or the aorta. 6) Anthracycline, gemcitabine, and pazopanib have been used as chemotherapy, but effective regimens are still unclear. 19) Therefore, currently, there is no sufficient treatment to prevent metastasis.…”

“…3) Currently, although a number of case reports and small case series have been reported, diagnosis and treatment of ISA remains difficult. 1,[4][5][6][7][8][9] ISA is highly invasive, and the mean overall survival time ranges from 5 to 18 months. 2) At the time of diagnosis, metastasis is found in approximately 75% of patients, with common locations including the bone, lung, liver, spleen, kidney, skin, and brain.…”