Microangiopatía trombótica tumoral pulmonar en un adolescente con adenocarcinoma gástrico

Background: Pediatric malignant tumors occur in 9 to 12 per 100,000 habitants; fewer than 5% are gastrointestinal tract neoplasms and 0.05 are gastric adenocarcinomas. Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare condition seen in 0.9 to 3.3% in adults with extrathoracic malignant tumors; one case has been reported with symptom remission after treatment. Only 2 cases have been reported in children. Case report: We report a thirteen year-old, male adolescent who presented with a month and a half of unproductive cough, weight loss, abdominal pain, vomiting and progressive dyspnea. Physical examination: Cachexia, respiratory distress, hepatomegaly and rales. A chest CT scan showed multiple adenopathies and lymphangitic infiltrate. A gastric biopsy reported diffuse gastric signet-ring cells adenocarcinoma. Abdominal CT scan showed thickening of the stomach layers and upper and lower cluster nodes. There was no response to chemotherapy. He died 3 months later. The autopsy showed gastric plastic linitis and PTTM. Conclusions: The PTTM is a condition associated with gastric carcinomas, especially in signet ring cell carcinoma. The combination of PTTM and gastric cancer is very rare in childhood, however it should be considered in the differential diagnosis of pulmonary hypertension and progressive dyspnea. The PTTM may be susceptible to treatment, but it requires a high index