Case Report

Imatinib Alleviated Pulmonary Hypertension Caused by Pulmonary Tumor Thrombotic Microangiopathy in a Patient With Metastatic Breast Cancer

We describe a case of pulmonary tumor embolism (PTE) from breast cancer diagnosed by selective aspiration cytology using a Swan-Ganz catheter. A 60-year-old woman was referred to Hamanomachi Hospital because of increased levels of tumor markers. The patient complained only of slight exertional dyspnea and a dry cough. Due to breast cancer, she had undergone a mastectomy followed by radiation and chemotherapy one year earlier. Positron emission tomography scanning with CT images revealed no evidence of malignancy. Repeated chest CT images showed emerging wedge-shaped nodules in the subpleural zones of the left lower lobe with diffuse ground-glass opacities in the bilateral lower lobes. The D-dimer level was negative. Pulmonary perfusion scintigraphy showed multiple small wedge-shaped defect areas on the peripheral sides of the bilateral lungs. Suspecting PTE, we performed selective aspiration cytology from the left pulmonary arteries. Cancer cells were detected from selected branches of left A8 and A9. Morphology and immunostaining led to a final diagnosis of PTE of recurrent breast cancer. Pulmonary embolism of cancer is a progressive, fatal condition with challenging diagnosis. Selective aspiration cytology with a Swan-Ganz catheter is a useful, less invasive option in patients with suspected PTE.

Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but fatal cancer-related disease. Owing to its non-specific findings, aggressive course, and lack of established treatment guidelines, only a few cases of antemortem diagnosis in long-term survivors have been reported. We aimed to report a case of uterine cervical cancer induced PTTM that was suspected based on pulmonary hypertension and successfully treated using combination chemotherapy despite of delayed diagnose. It is important to be aware that PTTM should be suspected when respiratory failure occurs in patients with unexplained pulmonary hypertension. Multidisciplinary treatments including molecular targeted therapies might be effective treatment options.

Although pulmonary tumor embolism (PTE) is a well-recognized end-stage form of pulmonary metastases at postmortem examination, the entity is rarely the first clinical sign of prostate cancer. Diagnosis of this condition in patients who have no previous history of malignancy is a challenge. Herein, we reported a 79-year-old man presented with progressive, unexplained dyspnea on exertion. Microscopic PTE coinciding with pulmonary lymphangitic carcinomatosis were readily recognized based on the presence of multifocal dilatation and beading of the peripheral pulmonary arteries with thickening of the bronchial walls and interlobular septa on the initial thin-section chest CT images. Pathologic examination of the transbronchial lung biopsy specimen revealed tumor emboli occluding both the small muscular pulmonary arteries and lymphatic vessels. These tumor cells were positive for prostatic specific antigen on immunohistochemical staining. The final diagnosis of prostatic adenocarcinoma was confirmed. Remarkable clinical and radiographic improvement was achieved following bilateral orchiectomies and anti-androgen treatment.

Pulmonary Tumor Thrombotic Microangiopathy (PTTM) is a fatal complication of malignancy characterized by embolization of tumor cells to the pulmonary vasculature leading to a vascular reaction resulting in stenosis and pulmonary hypertension. Because the clinical manifestations of PTTM overlap with those of other entities, premortem diagnosis is challenging. We describe an unusual case of PTTM as the only clinical manifestation of recurrent metastatic breast cancer. A 50 year-old woman presented with hypoxemia and echocardiographic findings consistent with pulmonary hypertension and cor pulmonale. Correlation of premortem pulmonary imaging with autopsy histopathologic findings revealed that ill-defined ground-glass opacities identified on CT angiogram corresponded to areas of cellular interstitial fibrosis and widespread intrapulmonary tumor emboli involving predominantly small-sized arteries with associated florid intimal fibrosis. The radiologic nodularities and scattered peripheral wedge-shaped consolidations corresponded to evolving pulmonary infarcts on histopathology. Although retrospectively, the imaging findings were concordant with a spectrum of increasing severity of tumor embolization and vascular remodeling, the diagnosis of PTTM was not made premortem. PTTM is a rare entity that must be considered in cancer patients with unexplained hypoxemia, pulmonary hypertension and lung opacities on imaging.

Le syndrome de microangiopathie thrombotique tumorale pulmonaire est une entité clinicopathologique rare où des micro-emboles de cellules tumorales dans la microcirculation pulmonaire induisent une microangiopathie thrombotique, une insuffisance respiratoire et un tableau de cœur pulmonaire aigus ou subaigus. Ses caractéristiques histologiques comprennent des micro-emboles tumoraux dans les petites artères et artérioles du poumon, associés à une prolifération fibrocellulaire et fibromusculaire de l’intima et parfois à la formation de thrombus. Le diagnostic est extrêmement difficile avant le décès. Ainsi, un grand nombre des observations rapportées reposent sur des données autopsiques. De très rares observations de diagnostics réalisés avant le décès suggèrent l’éventuelle efficacité d’une chimiothérapie. De nombreux détails restent à élucider, la recherche interdisciplinaire étant une priorité avec une étroite collaboration entre pathologistes et cliniciens afin de mieux comprendre ce syndrome le plus souvent mortel. Grâce à cette collaboration, il est éventuellement possible que ce syndrome ne soit plus considéré comme toujours ultimement fatal, l’utilisation de thérapeutiques ciblées permettant des survies de plusieurs mois après le diagnostic.

Pulmonary tumor thrombotic microangiopathy syndrome is a rare clinicopathological entity in which tumor cell micro-emboli in the pulmonary microcirculation induced thrombotic microangiopathy. This can cause respiratory failure, and acute or sub-acute right heart failure. Histological features include micro tumor emboli in the small arteries and arterioles of the lung associated with thrombus formation and fibro-cellular and fibro-muscular intimal proliferation. The diagnosis is however extremely difficult to make before death. Thus, most of the observations reported are based on autopsy data. Very rare diagnostic observations made before death suggest the potential effectiveness of chemotherapy. Many details remain to be elucidated, interdisciplinary research is a priority with close collaboration between pathologists and clinicians to better understand this, often fatal, syndrome. It may be that the use of targeted therapies will improve the very poor prognosis allowing survival of several weeks or months after diagnosis.