A 57-year-old man with rapidly progressive pulmonary hypertension

Submitted: May 15, 2021
Accepted: July 13, 2021
Published: August 9, 2021
Abstract Views: 1042
PDF: 426
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Pulmonary Tumor Thrombotic Microangiopathy (PTTM) is a rare condition associated with neoplastic disorders, predominantly gastric cancer, leading to pre-capillary Pulmonary Hypertension (PH). The pathologic mechanism involved is a fibrocellular intimal proliferation of small pulmonary vessels sustained by nests of carcinomatous cells lodged in pulmonary vasculature. Clinical presentation is nonspecific, including progressive dyspnea and dry cough. Diagnosis of PTTM is extremely challenging ante-mortem and prognosis is poor. Here we describe the case of a middle-aged man, without known previous cancer history. The clinical course was rapidly unfavorable, with progressive dyspnea and PH associated with hemodynamic instability, eventually culminating in patient’s death. PTTM diagnosis was made post-mortem. PTTM should be considered in any patient presenting with unexplained PH, especially if it is rapidly progressive, poorly responsive to standard approaches or there is suspected history of malignancy. A prompt diagnosis of PTTM could help in bringing light into this still under-recognized condition.



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Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J 2019;53:1802148. DOI: https://doi.org/10.1183/13993003.02148-2018
Von Herbay A, Illes A, Waldherr R, Otto HF. Pulmonary tumor thrombotic microangiopathy with pulmonary hypertension. Cancer 1990;66:587-92. DOI: https://doi.org/10.1002/1097-0142(19900801)66:3<587::AID-CNCR2820660330>3.0.CO;2-J
Chinen K, Tokuda Y, Fujiwara M, Fujioka Y. Pulmonary tumor thrombotic microangiopathy in patients with gastric carcinoma: An analysis of 6 autopsy cases and review of the literature. Pathol Res Pract 2010;206:682–9. DOI: https://doi.org/10.1016/j.prp.2010.05.002
Price LC, Wells AU, Wort SJ. Pulmonary tumour thrombotic microangiopathy. Curr Opin Pulm Med 2016;22:421–8. DOI: https://doi.org/10.1097/MCP.0000000000000297
Godbole RH, Saggar R, Kamangar N. Pulmonary tumor thrombotic microangiopathy: a systematic review. Pulm Circ 2019;9:2045894019851000. DOI: https://doi.org/10.1177/2045894019851000
Okubo Y, Wakayama M, Kitahara K, et al. Pulmonary tumor thrombotic microangiopathy induced by gastric carcinoma: Morphometric and immunohistochemical analysis of six autopsy cases. Diagn Pathol 2011;6;27.
Uga S, Ikeda S, Matsukage SI, Hamada M. An autopsy case of acute cor pulmonale and paradoxical systemic embolism due to tumour cell microemboli in a patient with breast cancer. Case Reports 2012;2012:bcr2012006682.
Buser M, Felizeter-Kessler M, Lenggenhager D, Maeder MT. Rapidly progressive pulmonary hypertension in a patient with pulmonary tumor thrombotic microangiopathy. Am J Respir Crit Care Med 2015;191:711-2. DOI: https://doi.org/10.1164/rccm.201501-0004IM
Gru AA, Pai RK, Roma AA. Pulmonary tumor thrombotic microangiopathy in patients with low-grade ovarian serous neoplasm: A clinicopathologic review of 2 cases of a previously unknown association. Int J Gynecol Pathol 2012;31:438-42. DOI: https://doi.org/10.1097/PGP.0b013e318249287d
Tanaka K, Nakasya A, Miyazaki M, et al. A case of hepatocellular carcinoma with respiratory failure caused by widespread tumor microemboli. Hukuoka Acta Medica 2011;102:298-302.
Malani AK, Gupta C, Kutty AVP, Betlej T. Pulmonary tumor thrombotic microangiopathy from metastatic gallbladder carcinoma: An unusual cause of severe pulmonary hypertension. Digest Dis Sci 2007;52:555-7. DOI: https://doi.org/10.1007/s10620-006-9232-z
Uruga H, Fujii T, Kurosaki A, et al. Pulmonary tumor thrombotic microangiopathy: A clinical analysis of 30 autopsy cases. Internal Med 2013;52:1317–23. DOI: https://doi.org/10.2169/internalmedicine.52.9472
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2015;46:903–75. DOI: https://doi.org/10.1183/13993003.01032-2015
Minatsuki S, Miura I, Yao A, et al. Platelet-derived growth factor receptor-tyrosine kinase inhibitor, imatinib, is effective for treating pulmonary hypertension induced by pulmonary tumor thrombotic microangiopathy. Int Heart J 2015;56:245-8. DOI: https://doi.org/10.1536/ihj.14-220
Ogawa A, Yamadori I, Matsubara O, Matsubara H. Pulmonary tumor thrombotic microangiopathy with circulatory failure treated with imatinib. Internal Med 2013;52:1927-30. DOI: https://doi.org/10.2169/internalmedicine.52.0718
Kayatani H, Matsuo K, Ueda Y, et al. Pulmonary tumor thrombotic microangiopathy diagnosed antemortem and treated with combination chemotherapy. Internal Med 2012;51:2767–70. DOI: https://doi.org/10.2169/internalmedicine.51.7682
Higo K, Kubota K, Takeda A, et al. Successful antemortem diagnosis and treatment of pulmonary tumor thrombotic microangiopathy. Internal Med 2014;53:2595-9. DOI: https://doi.org/10.2169/internalmedicine.53.2379

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How to Cite

Raimondi, Federico, Caterina Conti, Luca Novelli, Francesco Tarantini, Giuseppe Ciaravino, Piermario Scuri, Aurelia Grosu, Daniela Chinaglia, Lorenzo S.C. Grazioli, Andrea Gianatti, Ferdinando L. Lorini, Michele Senni, and Fabiano Di Marco. 2021. “A 57-Year-Old Man With Rapidly Progressive Pulmonary Hypertension”. Monaldi Archives for Chest Disease 92 (1). https://doi.org/10.4081/monaldi.2021.1930.