Sipple's Syndrome (Multiple Endocrine Neoplasia) in Pregnancy … Case Report

Abstract

A case of Sipple's syndrome (multiple endocrine neoplasia) in a 24-year-old pregnant Hindu patient is described. Classical features included multiple carcinoma of the thyroid and adrenal glands. The patient died shortly after admission with hypertensive encephalopathy/preecIampsia.Two types of multiple endocrine neoplasia (MEN) are described. Type 1 is associated with adenomas of the parathyroid glands, pancreas, anterior pituitary gland, together with peptic ulceration. Type 2 includes phaeochromocytoma of the adrenal gland and medullary carcinoma of the thyroid gland: the tumours are often bilateral and multiple. The latter syndrome may be associated with parathyroid adenomas or Von Recklinghausen's neurofibromatosis. There is often a familial tendency (Robbins and Cotran, 1979). Over 20 families with the Type 2 multiple endocrine syndrome have been described in the recent literature (William, 1965; Jackson et a]., 1973 andBeagie et al., 1975). However, we have not come across a case of M E N with the features described in the present report. CASE REPORTA 24-year-old, para 3 Hindu female was admitted to the Hospital in comatose state. On examination, she was deeply unconscious, with a pulse rate of 90/min. and a blood pressure of 280/150. There was bilateral pitting oedema of the feet. The uterus was 32 weeks in size, and fetal heart sounds were present. Systemic examination did not reveal any apparent abnormality. A clinical diagnosis of cerebrovascular accident with severe preeclampsia was made. Resuscitative measures failed to revive the patient and she died shortly after admission. The only investigations carried out were ward testing of urine which showed protoeinuria and a haemoglobin estimation which was 9 g/dl. Salient Autopsy FindingsExternal examination did not reveal any neurofibromatosis. There was pitting oedema of both lower 1. Reader. 2. Associate Professor. 3. Professor and Head. 4. Hon. Professor. Obstetrics and Gynaecology.extremities. Internal examination of the brain revealed oedema, congestion and petechial haemorrhages throughout the white matter. Microscopically, the brain showed extensive oedema and medial hypertrophy of the cerebral blood vessels. The pituitary gland was soft, but revealed no necrosis or tumour. The thyroid gland was enlarged, well encapsulated, firm in consistency and weighed 150 g. The right lobe measured 5 x 4 x 3 cm, the cut surface showing multiple, firm, white, well circumscribed nodules ranging from 0.2 to 2 cm in diameter; the left lobe showed similar nodules measuring 0.2 to 1 cm (figure 1). Medullary carcinoma was shown on histological examination. There was a positive stain for amyloid with crystal violet and Von Gierke's method; there was also birefringence with polarized light. The parathyroids did not show any adenomatous changes. The left adrenal gland was enlarged, well encapsulated, and the cut section showed a well circumscribed tumour, 5 cm in diameter, which was reddish-brown in colour, soft in consistency and Figure 1. Autopsy appearance of the thyr...

Multiple endocrine neoplasia presenting as phaeochromocytoma during pregnancy

“…Parathyroid adenomas develop in 10 to 20% of patients (Gagel et al 1988). Reports of MEN 2a during pregnancy are rare (Chodankar et al 1982). Since phaeochromocytoma can be life threatening, early diagnosis and treatment during pregnancy is of primary concern.…”