Pulmonary tumor thrombotic microangiopathy
(PTTM) is a rare clinicopathological entity in which the tumor cells embolize
to the pulmonary vasculature leading to fibro cellular intimal thickening and
arteriolar occlusion by cellular intimal proliferation. Sub-acute respiratory
failure, pulmonary hypertension, right sided heart failure and sudden death may
be seen due to consequences of stenosis of blood vessels.
We describe a case of a 23 year old man who presented with alleged
history of found unconscious and declared brought death. He was clinically
diagnosed as refectory sepsis with ventilator associated pneumonia with acute
kidney injury. Past history revealed Crohn’s disease and treatment with
Azathioprine and prednisolone. Mucicarmine was positive in colonic tumor as
well as in pulmonary tumor cell emboli with recanalization and intimal fibro
cellular proliferation of small arteries. A postmortem diagnosis of poorly
differentiated signet ring cell carcinoma of colon with PTTM was made based on
autopsy results.
Unfortunately, PTTM is difficult to diagnose and is mostly a
post mortem diagnosis with an extremely poor prognosis. Pulmonary hypertension
due to metastatic tumor emboli should be included in the differential diagnosis
of various causes of dyspnea in patients with cancer.
Pulmonary tumor thrombotic
microangiopathy (PTTM) is a rare clinicopathological entity in which the tumor
cells embolize, organize and recanalize to the pulmonary vasculature leading to
fibro cellular intimal thickening and vessel stenosis. Sub-acute respiratory
failure, pulmonary hypertension, right sided heart failure and sudden death may
be seen due to consequences of stenosis of blood vessels caused by PTTM. In
this report, we describe a rare case of PTTM associated with metastatic colon
carcinoma diagnosed on postmortem examination of a young man. We also aim to
review the literature related to PTTM and associated malignancies and how they
were diagnosed and managed.
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