Acute intermittent porphyria in pregnancy: a common misdiagnosis.

Acute intermittent porphyria (AIP) is inherited in an autosomal dominant fashion. Only 10% to 15% of the gene carriers have the clinical syndrome. The prevalence of AIP in Europe is 1/20,000. Pregnancy represents an essential risk factor in patients suffering from AIP. The clinical syndrome in AIP presents mainly with acute attacks, especially during the first trimester. Misdiagnosis of AIP unfortunately is very common. Pregnancy in women with AIP is associated with higher rates of spontaneous abortion, hypertension, low birth weight infants and considerable mortality (2-42%). Pregnancy, despite the major hormonal alterations it causes, is seldom associated with porphyric symptoms. There are only limited reports supporting the use of hemin during pregnancy, but experience indicates that it can be safely administered in pregnant women. Until clinical improvement is achieved, symptomatic treatment is recommended. Despite the fact that pregnancy in women suffering from AIP is related to higher rates of morbidity and complications, close management throughout the pregnancy could ensure a good outcome.

Laparoscopic surgery for malignant adrenal tumors.

Advances in imaging have improved early detection of primary and metastatic adrenal tumors. The laparoscopic approach, the gold standard for benign adrenal diseases, is controversial for malignant adrenal tumors. A prospective randomized study of the role of laparoscopic surgery in adrenal cancer is not feasible because of the rarity of the disease. A review of the literature demonstrates the safety and efficacy of laparoscopic adrenalectomy for solitary adrenal tumors. In primary adrenal malignancies, the laparoscopic approach should be considered cautiously, only when it can achieve complete tumor resection with an intact adrenal capsule. Conversion to an open procedure should be an early decision, prior to tumor morcellation or fracture of the tumor capsule. Patients who have local invasion, tumors that are too large, or require organ resection require an open procedure.

Laparoscopic adrenalectomy for large adrenal metastasis from contralateral renal cell carcinoma.

We present herein the case of a patient with solitary metachronous contralateral adrenal metastasis from renal cell cancer. The patient had undergone left radical nephrectomy and adrenalectomy for localized renal cancer 7 years previously. Laparoscopic transperitoneal right adrenalectomy was performed. The postoperative period was uneventful. Histology showed right adrenal metastasis from renal cancer. At 6-month follow-up, there was no evidence of recurrence.