Coincidence of Tracheobronchomegaly (Mounier-Kuhn Syndrome) and Juvenile Idiopathic Arthritis.

Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly includes clinical and radiographic findings of tracheobronchial dilatation and recurrent respiratory infections. MKS is a very rare pathology, especially in the paediatric age group which makes it a diagnostic challenge. A 4-year-old girl suffered from dyspnea, recurrent respiratory infections and joint pain. Chest radiography detected peribronchial reinforcement and CT-scan revealed extended tracheal dilatation and bronchiectasis. In addition to MKS our patient was diagnosed with juvenile idiopathic arthritis (JIA) and scleroderma. MKS can be caused by congenital disorder or acquired aetiology. Several connective tissue diseases have been associated with MKS but no cases of JIA or scleroderma are described previously. Our case illustrates that patients who suffer from recurrent respiratory infections with unsatisfactory evolution and unspecific chest X-ray alteration, MKS always has to be considered in the differential diagnosis particularly in patients who suffer from connective tissue diseases.

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[Bronchial mucoepidermoid carcinoma]. / Carcinoma mucoepidermoide bronquial.

We submit the case of a child afflicted with a mucoepidermoid bronchial tumor. The patient is a boy, aged seven, who after undergoing antibiotic treatment for six weeks because of a fever and atelectasia-condensation in the right lower lobe showed no signs of clinical improvement and was sent to our department to undergo further study and treatment. A bronchoscopy performed shows a polypoid mass that partially blocks the main bronchial tube a few milimiters under the access to the right upper lobe. A biopsy is carried out and the anatomopathological test shows there is a low degree epidermoid carcinoma. We decide to perform a lobectomy which for the tumor location and the lung condition has to be medium and lower right. We proceed to remove the adenopaty of hilium not affected by the tumor. The postoperative period develops without incidents. A check-up bronchoscopy performed three months later shows two polypoid masses in the right bronchial tube which, once a biopsy is performed, proved to be granulation tissue. Twelve months after undergoing surgery, the patient's condition is good, there is no evidence of tumor relapse and the breathing capacity is adequate, though there is an obstructive restrictive pattern in the espirometry. Even taking into consideration that lung tumors are extremely unusual, the epidermoid carcinoma is the one which most frequently occurs. The tumor's low malignancy is a sign that points to a good prognosis. Performing conservative surgery by means of bronchoplasty should be taken into account so as to keep the sequelae on the lung condition to a minimum, even though in this case the tumor location made it impossible.

[Thymus in posterior mediastinum]. / Timo en mediastino posterior.

It is presented the clinical case of a mass of posterior mediastinum with diagnostic by C.T.Scan, and ratified by Pathologic Anatomy, as posterior prolongation of thymus. Comments of the importance of the diagnostic by image and the rarity of this entity are made.

[Genitourinary rhabdomyosarcoma in childhood]. / Rabdomiosarcomas genitourinarios en la infancia.

We present the result obtained by GICOP in the treatment of 12 genitourinary rhabdomyosarcoma, located in vagina-uterus (5), bladder-prostate (4) and paratesticular (3). The age of the patients ranging from 1 to 15 years. Embryonal rhabdomyosarcoma was the most common histologic type (58 for 100) being the major number of cases in stadium II. The treatment was based in the combination of chemotherapy protocolised with vincristine, d-actinomycin, cyclophosphamide (VAC), vincristine, adriamycin (VAC-VAD) and/or iphosphamide, vincristine, d-actinomycin (IVA), use of radiotherapy, marrow auto-transplantation and radical surgery avoidance. Complete remission was achieved in 11 out of 12 cases (92 for 100), relapsing in 5 cases (38 for 100) of which 4 (80 for 100) achieved a second remission. An 82 for 100 disease free survival was achieved in a 5 year period.

[Rhabdomyosarcoma of the bile ducts]. / Rabdomiosarcoma de vías biliares.

Two children of two and three years old affected with biliary tract rhabdomyosarcoma are presented. In both cases the diagnostic was confirmed by anatomopathologic studies of the tumoural piece obtained by surgical extraction. Clinic evolution, surgical technic and cytostatic treatment are described been noticeable a favourable clinic development in both cases. Authors had made a bibliographic revision on the subject, pointing a minor incidence of the biliary tract in comparison with other tumour locations.