Bronchocentric granulomatosis as a first clinical manifestation in an adult patient with p67phox deficiency.

We report on a case of adult chronic granulomatous disease which first manifested as a pulmonary mass, and was histologically diagnosed as bronchocentric granulomatosis associated with aspergillosis in a patient with a deficiency of p67phox and a low oxidative response. Antifungal treatment was required for clinical resolution.

[Infrequent clinical presentation of Askin's tumor]. / Tumor de Askin de presentación clínica infrecuente.

Malign small cell tumors in the thoracopulmonary region is a tumor of neuroectodermic origins with polymorphous and infrequent presentation. It is mostly found among young people, developing an aggressive and severe course. It is a small cell tumor involving small quantities of cytoplasm without glucogen, round or oval nucleus with disperse chromatin, and little prominent nucleolus without tendency of manifesting pseudo red spots, being PAS negative. Two cases which initially resembled pleuropulmonary infection are presented. Case 1: Sixteen year old male. Presented with a high fever, pleuritic thoracic pain, and a cough with little expectoration. He was diagnosed with severe pulmonary infection and parapneumonic right pleural discharge. His condition improved with antibiotic treatment, but the cough persisted. Thoracoabdominal echography showed right pleural discharge and possible hepatic mass. Surgical intervention was performed. Askin's tumor was detected by biopsy. He began treatment with chemotherapy. Case 2: Thirty-four year old woman. Presented with non-productive cough, pleuritic thoracic pain, and high fever. In the thoracic TAC, there was right pleural discharge along with images suggesting hypodense mass. Given the patient's lack of response to antibiotics, a thoracotomy was performed. The anatomopathological diagnosis was Atkin's tumor. After beginning treatment, the patient died after ten days.

[Inflammatory pseudotumor of the lung: presentation of 2 cases, one of them endobronchial]. / Pseudotumor inflamatorio de pulmón: presentación de dos casos, uno de ellos endobronquial.

Inflammatory pseudotumor (IP) is a rare pathological entity. Although, the pulmonary affection is the most frequent, any other organ can be involved. Its etiopathology is unknown. The disease is considered as a reactive process constituted by chronic inflammatory elements and a benign behavior. The lack of a specific clinical setting and the low profitability of the bloodless techniques makes mandatory the thoracostomy for a definitive diagnosis. We report two cases of pulmonary IP. One of them with an endobronchial localization which is a infrequent variety, scantily reported in the medical literature. We discuss the clinical and etiopathologic aspects of this rare pathological entity.

Intracerebral calcifications in childhood lymphoblastic leukemia. A new iatrogenic disease?

A 3-year-old child diagnosed as having acute lymphatic leukemia (ALL), developed meningeal leukemia 36 months after the onset of the disease. He was twice subjected to cranial irradiation plus intrathecal methotrexate (i.t. MTX). Skull radiology showed bilateral gyriform calcification of both cerebral hemispheres. Hematological relapse was first detected 5 years after diagnosis and the child died 5 months later. The most striking findings of a right frontal lobe biopsy and the postmortem examination were wide calcium deposits located in the cortex and in the adjacent white matter. Intense demyelination as well as areas of neuron poverty were apparent in the necropsy but in the biopsy specimen. The possible interrelationship between such deposition and cranial irradiation and/or i.t. MTX suggests a new iatrogenic disorder.