Ante-mortem diagnosis of pulmonary tumour thrombotic microangiopathy in a patient with unrecognised extramammary Paget's disease

Abstract

Pulmonary tumour thrombotic microangiopathy (PTTM) resulting in fatal pulmonary hypertension is a rare complication of malignancy. Patients with PTTM generally suffer rapid deterioration of hypoxaemia, and a diagnosis is often only made at autopsy. We report a case of extramammary Paget's disease associated with PTTM. An ante-mortem diagnosis was made based on cytology of blood aspirated from a pulmonary artery catheter in a wedged position. Together with a review of the literature, this case highlights the potential diagnostic value of blood cytology in patients with cancer with rapidly progressing pulmonary hypertension.

The effect of the lymphovascular plugging metastasis pattern in extramammary Paget disease on identifying metastatic lesions using imaging tests: an autopsy case series

“…Although the common metastatic sites of EMPD have been reported, no autopsy case series has described the incidence, pattern and distribution of metastasis. We found all patients had developed multiple lymph node metastases and liver and lung metastases with lymphovascular invasion.…”

Pulmonary tumor thrombotic microangiopathy associated with extramammary Paget's disease: An autopsy case report

“…A few cases of antemortem diagnosis have mostly been made by pathological examinations such as transbronchial lung biopsy (TBLB), video-assisted thoracic surgery (VATS), and cytological analysis. 4 In order to clarify the definition of PTTM from a pathological view point, Sato et al recently proposed the following criteria to discern PTTM from incidental pulmonary tumor embolism: the number of PTTM-like lesions (vessels composed of fibrocellular intimal thickening, organized thrombi and varying number of cancer emboli) exceed 17 vessels in a 1-cm 2 area of lung specimen without pulmonary metastatic nodule. 6 Alveolar areas composed of abundant alveolar hemorrhage or pulmonary edema did not fulfill the criteria but those in the peripheral normal-looking alveolar areas.…”

“…3 Only one case of EMPD with PTTM has been reported so far. 4 Herein, we report a secondary case of EMPD associated with PTTM and review the associated literature.…”

Case of extramammary Paget’s disease causing pulmonary tumor embolism

“…Yuma WAKI, 1 Yoshimasa NOBEYAMA, 2 Tomohiro OGAWA, 1 Osamu FUKUCHI, 1 Nei FUKAZAWA, 3 Akihiko ASAHINA 2…”

“…Powell et al 2 proposed that local ischemia due to the compression of small vessels by corticosteroid-dependent hypertrophied fat cells induces fat necrosis and alkaline phosphatase release from damaged lysosomes, leading to phosphate binding to the denatured proteins and subsequent formation of calcification. Supporting this idea, Ammouri et al 3 documented the clinical features of SLE-associated calcinosis cutis by reviewing 41 cases as follows: (i) it occurred at a mean of 9.8 years after the diagnosis of SLE; and (ii) all of the patients were on…”