Adult polyglucosan body disease: the diagnostic value of axilla skin biopsy.

The diagnostic value of axilla skin biopsy has been investigated in a patient with adult polyglucosan body disease. The biopsy data have been compared with those of control subjects and with those from previously reported patients with Lafora's disease. In a patient with adult polyglucosan body disease and in patients with Lafora's disease, an abundance of polyglucosan bodies was found in the myoepithelial cells of the axillary apocrine glands. In the control group of subjects, polyglucosan bodies were only sporadically seen. Axilla skin biopsy is, therefore, an easy and reliable method for confirming the diagnosis of adult polyglucosan body disease.

Primary liposarcoma of the mediastinum in a child.

A case of primary anterior mediastinal liposarcoma occurring in a child is reported. A 5-year-old male child complained of exertional dyspnea and left chestpain. The chest roentgenogram showed a complete opacity of the left pleural space. After thoracotomy, the histological examination revealed mediastinal myxoid-pleomorphic liposarcoma. Surgical resection was performed and chemotherapy was given; 10 months later a recurrence was noted in the mediastinum. The child died 17 months after establishing the diagnosis.

Heart failure and hepatitis in a patient taking tocainide.

We report, for the first time to our knowledge, heart failure and liver abnormalities which developed after 2 months of treatment with tocainide.

[The triad: mediastinal chemodectoma, chondroma of the lung and esophageal leiomyoma]. / La triade: chémodectome du médiastin, chondrome du poumon et léiomyome de l'oesophage.

In 1958 a mediastinal tumor was discovered in an asymptomatic patient. The tumor was resected and diagnosed as a nonchromaffin chemodectoma. Additionally an oesophageal abnormality was discovered but not treated. At subsequent follow-ups the mediastinum never appeared normal and the heart size progressively increased. In 1974 a small opacity appeared in the left lung. In 1975 the patient was operated: a pulmonary osteochondroma, a pericarditis and an intrapericardial aortopulmonary chemodectoma were discovered. The oesophagus was not explored. In july 1979 an inoperable epidermoid carcinoma of the main bronchus of the left lung was discovered and the patient died in september 1979. There was no hypertension and no catecholamine excess. A post-mortem section was refused. After the recent description by Carney of the triad: extra-adrenal paraganglioma, lung chondroma and gastro-intestinal leiomyo(sarco)ma, we feel that our patient could present this syndrome and that the oesophageal tumor could be a benign leiomyoma. However the exact diagnosis of the oesophageal lesion is still unknown.

Intrathoracic chemodectoma with multiple localisations.

In 1958 a mediastinal tumour was discovered in an asymptomatic woman with a history of vomiting and an oesophageal anomaly which had not been treated. A tumour of the anterosuperior mediastinum in relation to the aortic arch was extirpated and proved to be a chemodectoma or non-chromaffin paraganglioma. At subsequent follow-ups the mediastinum was never normal and the heart size progressively increased, the oesophageal anomaly remaining unchanged. A small opacity appeared in the left lung in 1974. An operation performed in 1975 revealed an osteochondroma in the lung, pericarditis, and an intrapericardial chemodectoma. The oesophagus was not explored. An intrathoracic chemodectoma is rare. The importance of angiography in its diagnosis is emphasised. Malignant degeneration is seldom observed. Therapy is surgical, the tumour being radioresistant. The possibility to be considered in our patient was either relapse of the tumour with degeneration or multiple localisations of the tumour.

The alveolar soft-part sarcoma. A clinically malignant, neuro-endocrine tumor, possibly originating from the tissue composing the paraganglia.

The cases of two young women with alveolar soft-part sarcomas (ASPS) in one of the upper extremities are reported. The ASPS is a relatively rare malignant tumor of the soft parts that grows slowly and shows a predilection for the extremities; it occurs most often in young women. The tumor is deceptive in that the clinical impression is benign. So far, the only method of treatment is ample surgical excision. The tumor appears to be scarcely radio-sensitive and primary cytostatic therapy gives very little response. Histogenetically, the tumor is related to the neuro-endocrine paraganglion system. The long-term prognosis of the tumor is unfavorable.