SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Pulmonary tumor thrombotic microangiopathy is an uncommon disorder in which tumor cells embolize to the pulmonary vasculature, causing activation of coagulation and fibrocellular subintimal proliferation. Vascular stenosis causes pulmonary hypertension (PH), right heart failure, and death. We present a case of PH and respiratory failure in a young woman with tumor emboli from high risk type human papillomavirus (HPV)-associated cervical cancer. CASE PRESENTATION: A 30 year old woman with asthma but in good health presented with dyspnea and was diagnosed with pneumonia. When antibiotics did not help, lower extremity ultrasound identified deep venous thrombi. Despite anticoagulation with Apixaban, dyspnea worsened, and she was hospitalized for suspected CTEPH. Initial vitals were BP 103/61 mmHg, HR 117/min, RR 28/min, T 36.4C, and SaO2 95% (15 LPM O2). Lungs were clear, heart rhythm was regular with increased P2, and edema was absent. Labs included AST 64 U/L and BNP 450 pg/mL. Hypercoagulable testing, rheumatologic studies, pregnancy testing, and infectious work-up were negative. ECHO showed a severely dilated RV, reduced function, and RVSP 40-50 mmHg. RHC showed RAP 14 mm Hg, PAP 59/33 (mean 43) mmHg, PCWP 7 mmHg, PA saturation 38%. CT angiography showed bilateral ground-glass opacities and consolidation with a dilated pulmonary trunk but no significant lymphadenopathy or pulmonary embolism. Despite vasodilators, PH and heart failure worsened, and veno-arterial ECMO was initiated. CT abdomen, part of the transplant evaluation, showed pelvic adenopathy and an abnormal cervix. A 2-cm cervical mass was palpated, but biopsy showed benign squamous mucosa. A subsequent true-cut cervical biopsy showed moderately to poorly differentiated squamous cell carcinoma with lymphovascular invasion. Surgical lung biopsy, performed to help define the lung disease, showed metastatic squamous cell carcinoma with arterial and lymphatic tumor emboli resulting in pulmonary infarction. Chemotherapy (Taxol/Cisplatin) was given, but she did not improve. She opted for palliative care and died from malignancy-related complications. Of note, cervical cells were positive for type 18-HPV DNA. DISCUSSION: This case highlights several important considerations. A thorough PH clinical assessment is warranted when symptoms progress despite therapy. Among the etiologies of PH, malignancy should also be considered, regardless of age or the absence of apparent risk factors. Finally, it is noteworthy that vaccination might have prevented disease in this instance: available vaccines protect against several HPV types, and all protect against HPV types 16 and 18, which have the highest cervical cancer risk. CONCLUSIONS: Though uncommon, pulmonary tumor thrombotic microangiopathy should be considered in patients with respiratory failure and PH of unclear etiology, particularly when symptoms progress despite medical therapy. Reference #1: Price LC et al, Pulmonary tumour thrombotic microangiopathy. Curr Opin Pulm Med. 2016 Sep;22(5):421-8. Reference #2: Godbole R et al. Pulmonary Tumor Thrombotic Microangiopathy: Clinical, Radiologic, and Histologic Correlation. J Clin Imaging Sci. 2015 Jul 31;5:44. Reference #3: Martínez-Gómez X et al. Multidisciplinary, evidence-based consensus guidelines for human papillomavirus (HPV) vaccination in high-risk populations. Euro Surveill. 2019 Feb;24(7). DISCLOSURES: no disclosure on file for Charles Hoopes; No relevant relationships by Timothy Lewis, source=Web Response No relevant relationships by Tyler Reynolds, source=Web Response No relevant relationships by Victoria Rusanov, source=Web Response no disclosure on file for Jose Tallaj; no disclosure on file for Vincent Valentine; No relevant relationships by Raymond Wade, source=Web Response No relevant relationships by Keith Wille, source=Web Response
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