These tumors are rare but result in dangerous pregnancy complications. Geelhoed (1983) provided an earlier review of 89 cases in which 43 mothers died. Maternal death was much more common if the tumor was not diagnosed antepartum-58 versus 18 percent. As seen in Table 58-6, maternal mortality rates are now lower but still formidable. In their review of 77 cases, Biggar and Lennard (2013) reported that antepartum diagnosis is the most important determinant of maternal mortality risk. That said, Salazar-Vega and colleagues (2014) described good outcomes in women diagnosed after delivery.
TABLE 58-6. Outcomes of Pregnancies Complicated by Pheochromocytoma and Reported in Four Contiguous Epochs
Diagnosis of pheochromocytoma in pregnancy is similar to that for nonpregnant patients. MR imaging is the preferred technique because it almost always locates adrenal and extraadrenal pheochromocytomas (Fig. 58-6). In many cases, the principal challenge is to diferentiate preeclampsia from the hypertensive crisis caused by pheochromocytoma. Grimbert and colleagues (1999) diagnosed two pheochromocytomas during 56 pregnancies in 30 women with von Hippel-Lindau disease.
FIGURE 58-6 Coronal magnetic resonance image taken in a 32-week pregnant woman shows a right-sided pheochromocytoma (arrow) and its position relative to the liver above it.
Immediate control of hypertension and symptoms with an .-adrenergic blocker such as phenoxybenzamine is impera tive. he dose is 10 to 30 mg, two to four times daily. After .-blockade is achieved, 3-blockers may be given for tachycar dia. In many cases, surgical exploration and tumor removal are performed during pregnancy, preferably during the second trimester (Biggar, 2013; Dong, 2014). Successfulrlaparoscopic removal of adrenal tumors has become the norm (Miller, 2012;
Zuluaga-G6mez, 2012). If diagnosed later in pregnancy, either planned cesarean delivery with tumor excision or postpartum resection is appropriate.
Recurrent tumors are troublesome, and even with good blood pressure control, dangerous peripartum hypertension may develop. We have cared for three women in whom recur rent pheochromocytoma was identiied during pregnancy.
Hypertension was managed with phenoxybenzamine in all three. Two newborns were healthy, but a third was stillborn in a mother with a massive tumor burden who was receiving phenoxybenzamine, 100 mg daily. In all three women, tumor was resected postpartum.
his syndrome is rare and the female:male ratio is 3: 1 (Arit, 2015). Most cases are iatrogenic from long-term corticosteroid treatment. Cushing disease refers to bilateral adrenal hyperplasia stimulated by corticotropin-producing pituitary adenomas. Corticotropin is also called adrenocorticotropic hormone (ACTH). Most adenomas are microadenomas measuring < 1 em, and half measure <5 mm. Rarely, abnormal secretion of hypothalamic corticotropin-releasing factor may cause corticotropic hyperplasia. Such hyperplasia may also be caused by nonendocrine tumors that produce polypeptides similar to either corticotropin-releasing factor or corticotropin. Less than a fourth of cases of Cushing syndrome are corticotropin independent, and most of these are caused by an adrenal adenoma. Tumors are usually bilateral, and half are malignant. Occasionally, associated androgen excess may lead to severe virilization.