Pulmonary Artery Intimal Sarcoma: A Case Report

Kriz, Joseph P; Munfakh, Nabil A.; King, Gregory S.; Carden, Juan O

doi:10.1159/000445498

Cited by 15 publications

(16 citation statements)

References 14 publications

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“…Therefore, MRI has strength for distinguishing combined thrombus along with PAIS. In PET-CT, all PAIS patients showed FDG hypermetabolism, as in previous reports ( 23 24 ). PET-CT also has the advantage of demonstrating the presence of other foci of PAIS and identifying metastases distant from the PAIS.…”

Section: Discussionsupporting

confidence: 84%

Pulmonary Artery Intimal Sarcoma versus Pulmonary Artery Thromboembolism: CT and Clinical Findings

Kim

Kang

et al. 2018

Korean J Radiol

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ObjectiveTo describe CT and clinical findings of pulmonary artery intimal sarcoma (PAIS) compared with those of pulmonary thromboembolism (PTE), to investigate MRI and positron emission tomography (PET)-CT findings of PAIS, and to evaluate the effect of delayed diagnosis of PAIS on survival outcomes.Materials and MethodsTwenty-six patients with PAIS were retrospectively identified and matched for sex, with patients with PTE at a ratio of 1:2. CT and clinical findings of the two groups were compared using Student's t test or chi-square test. The effect of delayed diagnosis on survival was investigated using Kaplan-Meier analysis.ResultsThe most common tumor pattern in PAIS was tumoral impaction. Heterogeneous attenuation, wall eclipse signs, intratumoral vessels, acute interphase angles, single location, presence of lung ischemia, and central location were significantly more common in PAIS than in PTE (all p < 0.01). Levels of D-dimers and brain natriuretic peptide were lower in PAIS than in PTE (p < 0.05). In three patients of PAIS, long inversion time sequence MRI showed intermingled dark signal intensity foci suggestive of intermingled thrombi. All nine patients who had undergone PET-CT displayed hypermetabolism. Diagnosis was delayed in 42.3% of the PAIS patients and those patients had a significantly shorter overall survival than patients whose diagnosis was not delayed (p < 0.05).ConclusionThe characteristic CT and clinical findings of PAIS may help achieve early diagnosis of PAIS and make better survival outcomes of patients. MRI and PET-CT can be used as second-line imaging modalities and could help distinguish PAIS from PTE and to plan clinical management.

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Section: Discussionsupporting

confidence: 84%

Pulmonary Artery Intimal Sarcoma versus Pulmonary Artery Thromboembolism: CT and Clinical Findings

Kim

Kang

et al. 2018

Korean J Radiol

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“…A multimodal approach, utilizing several imaging modalities to visualize the thorax and vasculatures via CT, PET/CT, and MRI, is the best way to rapidly diagnose PAIS [ 1 , 2 ]. The diagnostic challenge lies in the complexity of distinguishing: (1) whether the mass is intravascular versus extravascular, (2) whether this mass is malignant or benign, and (3) if the mass is intravascular, whether it is a tumor thrombus, tumor mass, or pseudoaneurysm.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Artery Intimal Sarcoma: A Diagnostic Challenge Using a Multimodal Approach

Nakrani

Yeung

Kim

et al. 2020

Case Reports in Medicine

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Pulmonary artery intimal sarcoma (PAIS) is a rare tumor without clear syndromic presentation other than nonspecific symptoms of cough, dyspnea, and weight loss. This diagnosis is difficult due to challenging radiographic interpretations of multiple imaging modalities. We present a case of a 60-year-old male, who presented to his pulmonologist and underwent a CT chest with IV contrast that initially suggested primary lung carcinoma. CT angiogram showed significant vascular filling defects suspicious of an intravascular mass, rather than vascular invasion by lung lesions. The PET/CT scans further suggested a malignant process, but indistinguishable between an extravascular or intravascular etiology. Taking these results together, they suggested an intravascular malignancy, prompting a tissue biopsy, which ultimately led to a diagnosis of PAIS with metastases. Establishing a definitive diagnosis is essential as treatment and prognosis are different for sarcoma compared to carcinoma. There is no standard treatment to date, and management often includes a multidisciplinary approach involving surgery, radiation, chemotherapy, and targeted therapy. PAIS is a rare entity that cannot be diagnosed clinically and needs a multimodality approach for its diagnosis.

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“…In patients with PAIS, they have been used either alone or more frequently in combination. Our literature review identified 11 patients who were informative for their tumour response after such a regimen [ 2 , 3 , 5 , 7 , 8 , 9 , 10 ]. There were 3 objective responses (1 complete and 2 partial) and 8 had progressive disease, mirroring the ∼20% objective response rate observed with anthracycline-based regimens in soft tissue sarcomas.…”

Section: Discussionmentioning

confidence: 99%

“…Given the scarcity of the disease, only case reports and small retrospective series are reported in the literature. The prognosis is very poor, with median overall survival below 18 months [ 2 ]. In early stages, the treatment of choice is complete surgical resection, and the role of adjuvant chemotherapy and radiotherapy remains controversial.…”

Section: Introductionmentioning

confidence: 99%

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

Cantaloube¹,

Moureau-Zabotto²,

Mescam³

et al. 2018

Case Rep Oncol

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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.

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Pulmonary Artery Intimal Sarcoma: A Case Report

Cited by 15 publications

References 14 publications

Pulmonary Artery Intimal Sarcoma versus Pulmonary Artery Thromboembolism: CT and Clinical Findings

Pulmonary Artery Intimal Sarcoma versus Pulmonary Artery Thromboembolism: CT and Clinical Findings

Pulmonary Artery Intimal Sarcoma: A Diagnostic Challenge Using a Multimodal Approach

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

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