[Pulmonary lymphangioleiomyomatosis (LAM). Diagnostic usefulness of immunohistochemical markers]. / Linfangioleiomiomatosis pulmonar (LAM). Utilidad diagnóstica de los marcadores inmunohistoquímicos.

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown cause affecting the lungs of female patients. Although clinical and radiological findings may suggest LAM, a firm diagnosis is usually made after lung biopsy. Cases of LAM (histochemical diagnosis) or "suggestive of LAM" at the "12 of October" Hospital are reviewed. We applied a battery of immunohistochemical tests not used to date--involving estrogen, progesterone, desmin and HMB45 receptors--that allowed us to classify specimens as having either LAM lesions or lesions with non-LAM muscle proliferation. Smooth muscle proliferation in LAM is a distinct phenotype, such that diagnosis is facilitated by analyzing for immunohistochemical markers such as HMB45. This marker can be detected on formalin-fixed paraffin-embedded sheets in specimens obtained by either open lung or transbronchial biopsy.

[Fetal cardiac tumors]. / Tumores cardíacos fetales.

INTRODUCTION AND OBJECTIVES: Fetal cardiac tumors are rare and have a different histology than in adults: in the fetus, rhabdomyoma is more prevalent, but myxoma has not been described. We report our experience with nine fetuses with prenatally diagnosed primary cardiac tumours. METHODS: This is an observational and descriptive study of fetuses investigated because of the prenatal and echographic diagnosis of heart tumors. RESULTS: There were nine fetuses with thirteen cardiac masses among more than 700 fetal echocardiographic studies performed by pediatric cardiologists. Histology was available in four of them; three rhabdomyomas and once cavernous hemangioma. In one of the cases, the parents elected to interrupt the pregnancy; three patients died in the neonatal period (two as a direct consequence of the tumors and one due to sepsis) and we have no histological information regarding any of the other five fetuses. The subjects who survived beyond the neonatal period are mostly doing well. However, one developed tuberous sclerosis and another developed a hypoplastic left heart syndrome, perhaps due to the massive restriction of flow across the foramen ovale. The tumors in the other cases were well tolerated and hemodynamic or arrhythmic consequences were minimal of absent. No women with risk factors such as tuberous sclerosis were included in our study. CONCLUSIONS: Fetal cardiac tumors are rare and display a different histology. We recommend a conservative approach to treatment.

Glomus tumour of the trachea: report of a case with microscopic, ultrastructural and immunohistochemical examination and review of the literature.

A case of glomus tumour of the trachea is reported. The patient, a 58-year-old man, complained of dyspnoea, cough and occasional haemoptysis for many years and had been misdiagnosed as having chronic bronchitis. The diagnosis of glomus tumour in a tissue sample taken by bronchoscopy was useful in planning adequate surgery. Light and electromicroscopy of the excised tumour confirmed the preoperative diagnosis. Immunohistochemical examination showed vimentin and actin in the tumour cells, and negativity for high and low molecular weight keratins, desmin, neurofilaments, and factor VIII-related antigen, findings similar to glomus tumours of other sites.