three

She was well until 25 years earlier, when multiple pedunculate skin tumors and café au lait spots develop. Four years before entry, one tumor localized on right thigh became painful, a rapid increase in size was also informed( 1 to 15 cm.) Biopsy reported was neurofibrosarcoma.

Fig.1

Fig.2

The treatment was hemipelvectomy. Evolution : no tumor activity.

Family history was notable for the presence of multiple neurofibromatosis in one parent, one brother and three sons, but without history of pheochromocytoma or multiple endocrine neoplams. There was no history of previous hypertension, hypotension, seizures, dyspnea, rheumatic heart disease, tuberculosis or use of tabacco or alcohol.

Thereafter the patient felt improved until two days defore admission, when headaches,anxiety, nervousness, sudden prechordial palpitations, back pain, dyspnea, and chest disconfort developed.

Physical examination showed an obese woman, whose blood pressure was 140 to 180 / 100 to 120. ( normal 90/60) mm Hg. Her Pulse 160 beats/ min. Her height was 1.50 m and her weight 40 kg. Weight loss 10 kg., the patient was palid, with multiple neurofibromas. Heart with tachycardia without murmur. Lungs with rale on lower base. Hepatomegaly 7-5-4 cm. Hands with achrocyanosis. She recieved treatment with isorbide, diazepam, chlorotiazide, heparine. No responded at treatment and the patient expired by arrest cardiac.

The Laboratory data: Hb: 9.8 gr. LeuKocytes count : 8000/ml, platelets count 250 000 mm3. Glucose 210 mg/100 ml. No catecholamines levels . Rx of chest was normal. An electrocardiogram reveled an anormal rhythm a rate of 160x min ( paroxymal tachycardia) and left ventricle hypertrophy.

Clinical diagnosis:

Lung thromboembolism Vs Miocardium acute infarct and neurofibromatosis.

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