Pulmonary tumor thrombotic microangiopathy
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Title
was changed:
Pulmonary tumouraltumour thrombotic microangiopathy
Body
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Pulmonary tumour thrombotic microangiopathy (PTTM) occurs when there are non-occlusive tumour cell microemboli ("non-occlusive" microscopic tumour emboli2) "occlusive" fibrointimal remodelling in small pulmonary arteries, veins and lymphatics. It is considered one of the processes of microscopic pulmonary tumour embolism. It can be a rare cause ofrarely causes pulmonary hypertension.
Epidemiology
Associations
At autopsy, approximately 25% patients with pulmonary tumour thrombotic microangiopathy are found to have an extrathoracic malignancy. Most commonly 6:
- hepatocellular carcinoma
- breast cancer
- renal cell carcinoma
- gastric adenocarcinoma
- prostate carcinoma
- choriocarcinoma
Diagnosis
The diagnosis of pulmonary tumour thrombotic microangiopathy rests upon histological confirmation, most commonly from a lung biopsy 6.
Clinical presentation
Progressive breathlessness is the key symptom of pulmonary tumour thrombotic microangiopathy.
Laboratory tests
- hypoxaemia
- high A-a gradient
- raised D-dimer
Pathology
Thrombotic microangiopathy is a consequence of tumour cells metastasising to the distal tiny arterioles, venules and lymphatics of the lungs. The embolised cells induce an intravascular procoagulant effect resulting in fibrocellular overgrowth of the intima and vasoconstriction. Occasionally, this proceeds to overt pulmonary hypertension.
Radiographic features
Structural imaging is usually unremarkable in pulmonary tumour thrombotic microangiopathy:
- chest radiography is usually normal
-
CT pulmonary angiography
- unremarkable for pulmonary emboli
- lymphangitis and/or vessel beading may be seen
-
V/Q scintigraphy
- many subsegmental perfusional defects
Treatment and prognosis
Pulmonary tumour thrombotic microangiopathy heralds a dismal prognosis. Although treatment of the primary tumour improves prognosis 6.
History and etymology
It was first described by Von Herbay et al. in 1990 by Von Herbay et al.
See also
-<p><strong>Pulmonary tumour thrombotic microangiopathy (PTTM) </strong>occurs when there are tumour cell microemboli ("non-occlusive" microscopic tumour emboli<sup>2</sup>) with "occlusive" fibrointimal remodelling in small pulmonary arteries, veins and lymphatics. It is considered one of the processes of microscopic pulmonary tumour embolism. It can be a rare cause of <a href="/articles/pulmonary-hypertension-1">pulmonary hypertension</a>.</p><h4>History and etymology</h4><p>It was first described in 1990 by <strong>Von Herbay</strong> et al.<sup> 2</sup></p><h4>See also</h4><ul><li><a href="/articles/pulmonary-tumour-embolism">pulmonary tumour embolism </a></li></ul>- +<p><strong>Pulmonary tumour thrombotic microangiopathy </strong>occurs when there are non-occlusive tumour cell microemboli with fibrointimal remodelling in small pulmonary arteries, veins and lymphatics. It rarely causes <a href="/articles/pulmonary-hypertension-1">pulmonary hypertension</a>.</p><h4>Epidemiology</h4><h5>Associations</h5><p>At autopsy, approximately 25% patients with pulmonary tumour thrombotic microangiopathy are found to have an extrathoracic malignancy. Most commonly <sup>6</sup>:</p><ul>
- +<li><a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a></li>
- +<li><a href="/articles/breast-neoplasms">breast cancer</a></li>
- +<li><a href="/articles/renal-cell-carcinoma-1">renal cell carcinoma</a></li>
- +<li><a href="/articles/gastric-adenocarcinoma">gastric adenocarcinoma</a></li>
- +<li><a href="/articles/prostate-cancer-3">prostate carcinoma</a></li>
- +<li><a href="/articles/choriocarcinoma">choriocarcinoma</a></li>
- +</ul><h4>Diagnosis</h4><p>The diagnosis of pulmonary tumour thrombotic microangiopathy rests upon histological confirmation, most commonly from a lung biopsy <sup>6</sup>. </p><h4>Clinical presentation</h4><p>Progressive <a href="/articles/dyspnoea">breathlessness</a> is the key symptom of pulmonary tumour thrombotic microangiopathy.</p><h5>Laboratory tests</h5><ul>
- +<li><a href="/articles/hypoxaemia">hypoxaemia</a></li>
- +<li>high A-a gradient</li>
- +<li><a href="/articles/d-dimer-1">raised D-dimer</a></li>
- +</ul><h4>Pathology</h4><p>Thrombotic microangiopathy is a consequence of tumour cells metastasising to the distal tiny arterioles, venules and lymphatics of the lungs. The embolised cells induce an intravascular procoagulant effect resulting in fibrocellular overgrowth of the intima and vasoconstriction. Occasionally, this proceeds to overt <a href="/articles/pulmonary-hypertension-1">pulmonary hypertension</a>.</p><h4>Radiographic features</h4><p>Structural imaging is usually unremarkable in pulmonary tumour thrombotic microangiopathy:</p><ul>
- +<li>
- +<a href="/articles/chest-radiograph">chest radiography</a> is usually normal</li>
- +<li>
- +<a href="/articles/ct-pulmonary-angiogram-technique">CT pulmonary angiography</a><ul>
- +<li>
- +<a href="/articles/ct-pulmonary-angiogram-technique"></a>unremarkable for pulmonary emboli</li>
- +<li>
- +<a href="/articles/lymphangitic-carcinomatosis">lymphangitis</a> and/or <a href="/articles/vascular-beading">vessel beading</a> may be seen</li>
- +</ul>
- +</li>
- +<li>
- +<a href="/articles/vq-scan-2">V/Q scintigraphy</a><ul><li>many subsegmental perfusional defects</li></ul>
- +</li>
- +</ul><h4>Treatment and prognosis</h4><p>Pulmonary tumour thrombotic microangiopathy heralds a dismal prognosis. Although treatment of the primary tumour improves prognosis <sup>6</sup>.</p><h4>History and etymology</h4><p>It was first described by Von Herbay et al. in 1990<sup> 2</sup>.</p><h4>See also</h4><ul><li><a href="/articles/pulmonary-tumour-embolism">pulmonary tumour embolism </a></li></ul>
References changed:
- 1. Price L, Wells A, Wort S. Pulmonary Tumour Thrombotic Microangiopathy. Curr Opin Pulm Med. 2016;22(5):421-8. <a href="https://doi.org/10.1097/MCP.0000000000000297">doi:10.1097/MCP.0000000000000297</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27387102">Pubmed</a>
- 2. Kumar N, Price L, Montero M, Dimopoulos K, Wells A, Wort S. Pulmonary Tumour Thrombotic Microangiopathy: Unclassifiable Pulmonary Hypertension? Eur Respir J. 2015;46(4):1214-7. <a href="https://doi.org/10.1183/13993003.00052-2015">doi:10.1183/13993003.00052-2015</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26206876">Pubmed</a>
- 3. 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(10):682-9. <a href="https://doi.org/10.1016/j.prp.2010.05.002">doi:10.1016/j.prp.2010.05.002</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20554399">Pubmed</a>
- 4. Patrignani A, Purcaro A, Calcagnoli F, Mandolesi A, Bearzi I, Ciampani N. Pulmonary Tumor Thrombotic Microangiopathy: The Challenge of the Antemortem Diagnosis. J Cardiovasc Med (Hagerstown). 2014;15(11):828-33. <a href="https://doi.org/10.2459/JCM.0b013e328354e473">doi:10.2459/JCM.0b013e328354e473</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22710763">Pubmed</a>
- 5. Hutchinson J, Fulcher J, Hanna J, Ward M. Pulmonary Tumor Thrombotic Microangiopathy: Case Report and Review of Literature. Am J Forensic Med Pathol. 2018;39(1):56-60. <a href="https://doi.org/10.1097/PAF.0000000000000369">doi:10.1097/PAF.0000000000000369</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29232215">Pubmed</a>
- 6. Venn R, Parkes E, Joshi A, Caple E, Davies H. Respiratory Failure in a Cancer Patient: Pulmonary Thrombotic Microangiopathy. Clin Med (Lond). 2020;20(4):430-2. <a href="https://doi.org/10.7861/clinmed.2020-0102">doi:10.7861/clinmed.2020-0102</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32675152">Pubmed</a>
- 1. Price LC, Wells AU, Wort SJ. Pulmonary tumour thrombotic microangiopathy. (2016) Current opinion in pulmonary medicine. 22 (5): 421-8. <a href="https://doi.org/10.1097/MCP.0000000000000297">doi:10.1097/MCP.0000000000000297</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27387102">Pubmed</a> <span class="ref_v4"></span>
- 2. Neelam Kumar, Laura C. Price, M. Angeles Montero, Konstantinos Dimopoulos, Athol U. Wells, S. John Wort. Pulmonary tumour thrombotic microangiopathy: unclassifiable pulmonary hypertension?. (2015) European Respiratory Journal. 46 (4): 1214. <a href="https://doi.org/10.1183/13993003.00052-2015">doi:10.1183/13993003.00052-2015</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26206876">Pubmed</a> <span class="ref_v4"></span>
- 3. 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. (2010) Pathology, research and practice. 206 (10): 682-9. <a href="https://doi.org/10.1016/j.prp.2010.05.002">doi:10.1016/j.prp.2010.05.002</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20554399">Pubmed</a> <span class="ref_v4"></span>
- 4. Patrignani A, Purcaro A, Calcagnoli F, Mandolesi A, Bearzi I, Ciampani N. Pulmonary tumor thrombotic microangiopathy: the challenge of the antemortem diagnosis. (2014) Journal of cardiovascular medicine (Hagerstown, Md.). 15 (11): 828-33. <a href="https://doi.org/10.2459/JCM.0b013e328354e473">doi:10.2459/JCM.0b013e328354e473</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22710763">Pubmed</a> <span class="ref_v4"></span>
- 5. Hutchinson JC, Fulcher JW, Hanna J, Ward ME. Pulmonary Tumor Thrombotic Microangiopathy: Case Report and Review of Literature. (2018) The American journal of forensic medicine and pathology. 39 (1): 56-60. <a href="https://doi.org/10.1097/PAF.0000000000000369">doi:10.1097/PAF.0000000000000369</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29232215">Pubmed</a> <span class="ref_v4"></span>
Systems changed:
- Chest
- Oncology
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