Pulmonary Tumor Thrombotic Microangiopathy with Cor Pulmonale due to Desmoplastic Small Round Cell Tumor

Sadimin, Evita; Collier, Adrienne G.; Gaffney, Joseph W.; Fyfe, Billie

doi:10.1016/j.jpeds.2011.12.010

Cited by 5 publications

(3 citation statements)

References 16 publications

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“…Pulmonary tumor thrombotic microangiopathy occurs mostly in adults, with carcinoma of the stomach being the most common associated malignancy in 60%-70% cases, followed by the pancreas [15][16][17]. Only 3 cases of PTTM are known to date in children, 2 of which occurred in children with gastric carcinomas (Table 1) [2,3,5].…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare pathologic phenomenon whose diagnosis is recognized by the presence of small vessel emboli and a fibrocellular intimal proliferation with the presence of neoplastic cells [1]. Fewer than 80 cases have been reported in the literature, with only 3 in the pediatric age group [2][3][4][5]. Carcinoma of the stomach is the most common malignancy in adults with PTTM [4].…”

Section: Introductionmentioning

confidence: 99%

“…Carcinoma of the stomach is the most common malignancy in adults with PTTM [4]. The initial clinical manifestations are often vague and include respiratory distress with or without acute right-sided heart failure followed by death in a high proportion of cases [1][2][3][4][5][6]. The ultimate diagnosis is more often than not established at autopsy.…”

Section: Introductionmentioning

confidence: 99%

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Myelodysplastic Syndrome with Pulmonary Tumor Thrombotic Microangiopathy in an 11-Year-Old Male Patient

et al. 2014

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Myelodysplastic syndrome (MDS) and pulmonary tumor thrombotic microangiopathy (PTTM) are independently rare in the pediatric population. This report describes an 11-year-old male patient who initially presented with respiratory distress and cardiovascular collapse. A large left main pulmonary artery embolus and multiple, smaller pulmonary thromboemboli were widely dispersed throughout both lungs. Despite aggressive supportive care, he died within seven hours of admission. A complete postmortem examination was performed, leading to the diagnoses of primary MDS and microthrombi in the lungs, including the characteristic fibroproliferative lesions seen in PTTM. Individually, both conditions are extremely uncommon, and therefore the coincidence of these 2 conditions in a child is singularly unique.

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