Tumor-related thrombotic pulmonary microangiopathy: Review of pathologic findings and pathophysiologic mechanisms

Pinckard, J. Keith; Wick, Mark R.

doi:10.1016/s1092-9134(00)90038-8

Cited by 70 publications

(43 citation statements)

References 12 publications

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“…Therefore, diffuse obliteration of distal pulmonary arterial vasculature by B-cell lymphoma remains the only cause of this PAH, and this mechanism is analogous to the previously described tumor-related thrombotic pulmonary microangiopathy associated with gastric and undetermined carcinoma [13,14]. Cells of IVL are in fact often described as being enmeshed in platelet-fibrin thrombi in pulmonary small vessels [5,6].…”

Section: Discussionsupporting

confidence: 53%

Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

et al. 2005

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Intravascular lymphoma (IVL) is a rare and usually fatal disease that belongs to the class of high-grade malignant lymphomas and which is characterized by proliferation of neoplastic lymphoid cells exclusively within the lumina of small blood vessels. Its polymorphic and nonspecific clinical manifestations make antemortem diagnosis very difficult. We report herein a case of IVL revealed by fatal, precapillary, pulmonary arterial hypertension and associated with long-lasting fever. Extensive investigation of the usual causes of pulmonary arterial hypertension was negative. The diagnosis of intravascular lymphoma was made on postmortem analysis, revealing diffuse and sometimes complete obliterations of the lumina of small blood vessels by large B-cell lymphoma, including pulmonary capillaries. Thus, we propose that IVL must be added to the spectrum of etiologies of subacute pulmonary arterial hypertension, notably in the context of associated fever, both entities requiring emergency diagnosis. For this purpose, blood collected via pulmonary capillary-wedge aspiration for cytologic examination may be associated with right-sided heart catheterization when this latter procedure is suitable. Am.

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Section: Discussionsupporting

confidence: 53%

Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

et al. 2005

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“…Most reported cases of PTTM have been described in Japan, which is likely to reflect the high prevalence of gastric carcinoma. PTTM occurs in 16-27% cases of gastric carcinoma [4,5,9], especially the mucinous, signet ring and poorly differentiated subtypes [3,5,9]. PTTM is less commonly associated with breast [10], bladder [11], ovarian clear cell [12], hepatocellular [13] and gallbladder carcinoma [14].…”

Section: Incidence/epidemiologymentioning

confidence: 99%

“…It may be difficult to diagnose, and is difficult to distinguish from pulmonary tumour embolism except following histological examination of lung tissue. Until recently, PTTM was universally a postmortem finding, with an appalling prognosis [3,4]. Recent advances, however, suggest improvements in short-term outcomes.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary tumour thrombotic microangiopathy

Price

Wells

Wort

2016

Current Opinion in Pulmonary Medicine

120

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Purpose of review Pulmonary tumour thrombotic microangiopathy (PTTM) describes tumour cell microemboli with occlusive fibrointimal remodelling in small pulmonary arteries, veins and lymphatics. Progressive vessel occlusion ultimately results in pulmonary hypertension, which is often severe and rapid in onset. PTTM is associated with carcinomas, notably gastric carcinoma, with vascular endothelial growth factor and platelet-derived growth factor signalling implicated in driving the intimal remodelling. PTTM is a rare cause of pulmonary hypertension, but given that up to a quarter of autopsy specimens from patients dying of carcinoma show evidence for PTTM, it is probably underdiagnosed AQ5 . Recent findingsUntil recently, prognosis in PTTM was universally abysmal from weeks to a few months. Diagnostic utilities include aspiration of tumour cells at wedged right heart catheterization, HRCT AQ6 findings and computed tomography-positron emission tomography (CT-PET), although definitive diagnosis requires histological analysis. Reports of PTTM treated with a combination of targeted pulmonary vasodilator therapies, anticoagulation, specific chemotherapy and platelet-derived growth factor inhibition, for example using imatinib, suggest that these approaches can prolong survival.Summary PTTM is increasingly recognized as an important cause of pulmonary hypertension, often in patients presenting with new-onset pulmonary hypertension and as yet undiagnosed malignancy. Prospects of survival are improving with targeted combination therapy, and early recognition and diagnosis are likely to be the key factors to improve outcome.

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“…Pulmonary thrombotic microangiopathy is uncommon, reported in only 3% to 26% of autopsied patients with solid tumors (19)(20)(21)(22), and is usually only diagnosed postmortem (23). In this condition, embolic tumor cells obstruct pulmonary vessels, activate the coagulation cascade (24), and produce infl ammatory mediators promoting thrombosis and intimal proliferation (23). Th is disorder results in increased vascular resistance, secondary pulmonary hypertension (24,25), and right-sided heart failure.…”

Section: Baylor University Medical Center Proceedingsmentioning

confidence: 99%

“…In this condition, embolic tumor cells obstruct pulmonary vessels, activate the coagulation cascade (24), and produce infl ammatory mediators promoting thrombosis and intimal proliferation (23). Th is disorder results in increased vascular resistance, secondary pulmonary hypertension (24,25), and right-sided heart failure. Such patients generally have widely disseminated cancer, and their prognosis is dismal.…”

Section: Baylor University Medical Center Proceedingsmentioning

confidence: 99%

Malignant Rhabdoid Tumor of the Kidney Arising in an Adult Patient

Podduturi¹,

Campa-Thompson²,

Zhou³

et al. 2014

Baylor University Medical Center Proceedings

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Malignant rhabdoid tumors (MRT) of the kidney are rare in children and even less common in adults, with only six previously reported adult cases. We present the case of a 60-year-old man with an MRT arising in the left kidney with extensive pulmonary micrometastases and thromboembolism resulting in thrombotic pulmonary microangiopathy (pulmonary tumor embolism syndrome). MRT is an extremely aggressive neoplasm with a short survival time.M alignant rhabdoid tumor (MRT) of the kidney is a highly aggressive, extremely rare neoplasm that is usually seen in children. Th e term rhabdoid is used because the tumor cells resemble rhabdomyoblasts but lack myogenic markers, and pathologic diagnosis requires familiarity with these microscopic features plus awareness that adult onset is possible. We describe clinical and necropsy fi ndings in an adult with primary renal MRT. CLINICAL HISTORYA 60-year-old white man with known hypothyroidism, hypertension, and chronic obstructive pulmonary disease had progressive fatigue and abdominal distension for 6 months and worsening dyspnea for 2 weeks. Imaging showed bilateral pulmonary infi ltrates consistent with pneumonia, and he was treated at home with antibiotics and prednisone. Th ree days later, he presented to an outside emergency department with gross hematuria, left calf pain, hemoptysis, painful testicular swelling, and nontraumatic ecchymoses. He was then transferred to Baylor University Medical Center at Dallas and admitted to the intensive care unit. Examination disclosed bilateral lower-extremity edema and ecchymosis over the left calf and ankle. His body mass index was 30.4 kg/m 2 , blood pressure, 170/90 mm Hg; heart rate, 96 beats/minute; respiratory rate, 18 breaths/minute; white blood cell count, 31.9 k/μL; hemoglobin, 9.3 g/dL; hematocrit, 25.9%; platelets, 81 k/μL; prothrombin time, 18.0 seconds (reference range 9.0-12.0 seconds); blood urea nitrogen, 46.0 mg/dL; creatinine, 1.6 mg/dL; and arterial blood gas pH, 7.38. He was given heparin and multiple units of red blood cells and cryoprecipitate. Chest radiograph showed bilateral basilar and right upper lobe infi ltrates. Computed tomography showed a thrombus within the left renal vein that extended into the inferior vena cava and a mass in the mid to lower pole of the left kidney (Figure 1).Over the next few days, the patient's oxygen requirements increased, his abdomen became more distended, and his urine output decreased. A left radical nephrectomy and retroperitoneal lymph node dissection was performed. At surgery, there was no residual thrombus in the left main renal vein. Postoperatively,

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Tumor-related thrombotic pulmonary microangiopathy: Review of pathologic findings and pathophysiologic mechanisms

Cited by 70 publications

References 12 publications

Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

Pulmonary tumour thrombotic microangiopathy

Malignant Rhabdoid Tumor of the Kidney Arising in an Adult Patient

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