Abstract

We would like to describe and report a 72-yrars-old Japanese male patient case with extramammary Paget's disease. The clinical course commenced with the appearance of exanthema on the pubic region and three and a half years later, systemic and multi-organ involvement was observed. The patient passed away due to respiratory failure caused by Paget's cell embolism which originated from the male external genitalia.Autopsy results revealed metastasis to both lungs, liver, both adrenals, superior sagittal sinus, vertebrae, sternum, ribs and lymph nodes: both pulmonary hili, paraaortic and both inguinal regions. Examination of pulmonary histopathological preparations revealed the presence of metastatic cells in the pulmonary vessels (various sizes), within the alveolar walls and in some regions thrombus was observed.The patient sought medical attention for exanthema on the external genitalia but was observed to have respiratory failure, elevated levels of myoenzymic activity and bleeding tendencies. The patient passed away 14-days after the initial examination and during hospitalization, the changes in blood gases (deterioration and/or improvement) due to metastatic cell embolism or thrombosis were observed. Embolism and/ or thrombosis is suggestive as the cause of DIC and in addition, increase in myoenzymic activity was simultaneously observed with deterioration of blood gases due to metastatic embolism and/or thrombosis with this particular case. It is suggested that metastatic thrombosis occured within the vascular vessels of the skeletal muscles even thought we were unable to determine this from the autopsy findings.Conclusively, it is suggestive that cases with metastatic intravascular embolism, simultaneously increases the probability of concurrent occurrence of multi-organ metastatic embolism.

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