A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery

Assi, Tarek; Kattan, Joseph; Rassy, Elie El; Moussa, Tania; Nassereddine, Hussein; Honoré, Charles; Adam, Julien; Terrier, Philippe; Dumont, Sarah; Mir, Olivier; Cesne, Axel Le

doi:10.1016/j.critrevonc.2020.102889

Cited by 39 publications

(47 citation statements)

References 87 publications

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“…The clinical presentation ranges from the complete absence of symptoms to nonspecific symptoms like dyspnoea, cough, haemoptysis, syncope, weight loss and hypotension [ 5 , 7 ]. B-natriuretic peptide levels may be elevated in acute, chronic pulmonary embolism or PAS [ 9 , 10 ], while D-dimer may be elevated either in the setting of the longstanding inflammatory response induced by PAS or by an overlaying thrombus encasing the tumour. Concurrent lower extremity thrombosis has also been reported [ 10 ].…”

Section: Clinical Discussionmentioning

confidence: 99%

“…Concurrent lower extremity thrombosis has also been reported [ 10 ]. It has been reported that echocardiographic evidence of pulmonary hypertension indicates bilateral pulmonary artery disease even in the case of a unilateral CT pattern [ 9 ].…”

Section: Clinical Discussionmentioning

confidence: 99%

“…Diagnosis can take months from the onset of symptoms [ 7 ], affecting the treatment options and overall prognosis. After diagnosis, the reported median survival is 1.5 months in untreated patients (range 1.5–5.5 months) [ 8 , 9 ], but may be extended to 17–26 months with systemic chemotherapy [ 6 , 9 ]. In contrast, surgical resection improves PAIS survival to a reported median survival of 26.8–36.5 months [ 9 ].…”

Section: Clinical Discussionmentioning

confidence: 99%

“…After diagnosis, the reported median survival is 1.5 months in untreated patients (range 1.5–5.5 months) [ 8 , 9 ], but may be extended to 17–26 months with systemic chemotherapy [ 6 , 9 ]. In contrast, surgical resection improves PAIS survival to a reported median survival of 26.8–36.5 months [ 9 ]. Most patients die due to disease recurrence and/or pulmonary circulation occlusion [ 8 ].…”

Section: Clinical Discussionmentioning

confidence: 99%

“…Most CT scans performed for suspected pulmonary emboli or masses did not include unenhanced imaging, preventing evaluation of mass enhancement, whereas MRI studies typically have both unenhanced and multiple post-contrast images [ 12 ]. CT enhancement (>25 HU) has been shown to differentiate between bland and tumour thrombus in various vessels [ 9 ]. A tailored study that includes pre-and post-contrast images may provide diagnostic information in cases of suspected PAS.…”

Section: Radiological Discussionmentioning

confidence: 99%

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A 51-year-old man with chronic cough and left hilar prominence

Kourouni

Aesif

Tamarkin

et al. 2021

Breathe

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Section: Clinical Discussionmentioning

confidence: 99%

Section: Clinical Discussionmentioning

confidence: 99%

Section: Clinical Discussionmentioning

confidence: 99%

Section: Clinical Discussionmentioning

confidence: 99%

Section: Radiological Discussionmentioning

confidence: 99%

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A 51-year-old man with chronic cough and left hilar prominence

Kourouni

Aesif

Tamarkin

et al. 2021

Breathe

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A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature

Alizadehasl,

Najdaghi,

Mohseni Salehi

et al. 2024

Clinical Case Reports

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Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that presents significant diagnostic and therapeutic challenges due to its nonspecific symptoms and propensity for late detection. This case report aimed to elucidate the diagnostic journey, surgical intervention, and multidisciplinary management of this rare entity. In September 2023, a 42‐year‐old male presented with dyspnea on exertion and retrosternal chest pain, classified as NYHA FC II. Initial investigations, including ECG and lab tests, indicated tachycardia and elevated troponin and NT‐pro‐BNP levels. Transthoracic and transesophageal echocardiography identified a multilobulated mass in the right ventricular outflow tract and main pulmonary artery. Cardiac MRI and CT angiography confirmed a high‐grade pleomorphic spindle cell tumor, leading to surgical resection in October 2023. Histopathology confirmed intimal sarcoma. Postsurgery, the patient underwent chemotherapy and radiotherapy, showing significant clinical improvement and no recurrence on follow‐up PET‐CT. This case highlights the importance of a multidisciplinary approach in diagnosing and managing primary intimal sarcoma of the pulmonary artery, emphasizing the role of advanced imaging, timely surgical intervention, and combined chemotherapy with radiotherapy in improving patient outcomes.

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Percutaneous endovascular biopsy for the diagnosis of pulmonary artery masses: A preliminary study of single‐center

et al. 2023

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Percutaneous endovascular biopsy (PEB) including forceps biopsy and catheter aspiration has been used to make a pretreatment diagnosis for pulmonary artery (PA) masses. This retrospective study aims to describe the procedure of PEB and compare the diagnostic yield of forceps biopsy and catheter aspiration for a definite diagnosis in patients with PA masses. All consecutive 22 patients (53 ± 14 years), 11 males and 11 females, who underwent PEB for pathologic confirmation between November 2018 and November 2022 were enrolled. All 22 patients performed computed tomography pulmonary angiography or positron emission tomography‐computed tomography to confirm the filling defects suspicious for PA malignancy before intervention. And then, all patients underwent PEB successfully without acute or fatal complications, including both forceps biopsy and catheter aspiration in 15 cases, only forceps biopsy in 5 cases, and only catheter aspiration in 2 cases. Histopathological analysis provided a definite diagnosis in all PEBs with a clinical success of 91.0% (20/22). Among them, in 15 patients who underwent both forceps biopsy and aspiration biopsy, the technical success using forceps biopsy was 93.3% (14/15), and aspiration biopsy was 6.7% (1/15), and there was a significant difference in diagnostic accuracy when comparing two techniques. Twenty‐one out of 22 PA masses (95.5%) were malignant, of which, the most frequent malignant lesion observed was PA sarcoma (66.7%, 14/21). Benign lesion included one thrombus (4.5%, 1/22). In conclusion, PEB is an effective and safe diagnostic method for differentiating benign and malignant PA masses and could be peformed when PA masses appeared clinically malignant.

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A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery

Cited by 39 publications

References 87 publications

A 51-year-old man with chronic cough and left hilar prominence

A 51-year-old man with chronic cough and left hilar prominence

A Comprehensive Insight Into Primary Intimal Sarcoma of the Pulmonary Artery; From Diagnosis to Management: A Case Report and Review of the Literature

Percutaneous endovascular biopsy for the diagnosis of pulmonary artery masses: A preliminary study of single‐center

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