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330
ENDOCRINE SURGERY
Prognosis
The prognosis of parathyroid carcinoma is quite variable. No one characteristic correlates with outcome. The best prognosis depends upon early recognition and complete excision of the tumor at initial surgery. The mean time to recur­renceis usually3 years,although intervals of up to 20 years have been reported. When the tumor recurs, complete cure is unlikely, although pro­longed survival is still common with palliative surgery. Five-year survival rates vary from 40 to 86%. The National Cancer Database survey reported a 10-year survival of approximately 49% [14] and the MD Andersen Cancer Center reported survival rates of 85 and 77% at 5 and 10 years, respectively [82]. The National Surveil­lance, Epidemiology, and End Results database recently reported a 10-year survival of 67.8% [96].
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Section III
Adrenal
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24

Adrenal Embryology, Anatomy, and Physiology

Donal Shanahan and Thomas William Jay Lennard
Embryology
The paired adrenal (suprarenal) glands are flattened retroperitoneal endocrine glands closely applied to the medial aspect of the super­ior pole of each kidney. The internal structure of these pale yellow glands are incongruous in that the adrenal gland is composed of two discrete parts, namely an outer cortex enveloping a cen­tral medulla. The adrenal cortex and medulla contain distinct endocrine tissues that secrete different hormones and are regulated by separate control systems. As such, the parts of the adrenal gland have different embryological origins, in that the cortex is derived from coelomic (body cavity) epithelium whereas the medulla is ectodermal in origin arising from the neural crest (Fig. 24.1).
In the sixth week of intrauterine life the adrenal fetal cortex develops from the body cavity epithelium. The fetal cortex is thick and spherical in shape, a second group of cells surround the fetal cortex and forms the definitive cortex. The outer part of the fetal cortex gives rise to the zona reticularis whereas the definitive cortex differentiates into zona glomerulosa and zona fasciculate (Fig. 24.2). The inner part of the fetal cortex persists throughout intrauterine life but it degenerates shortly after birth.
The neural crest is the name given to the band of cells at the outermost edges of the
neural band. Migration of neural crest cells leads to the formation of the dorsal root ganglia and sympathetic ganglia. Neural crest cells accumulate in a mass that will become the adrenal medulla on either side of the body cav­ity (Figs. 24.1 and 24.2). Relative to body weight the fetal adrenal glands (Fig. 24.3) are 10–20 times larger than the adult adrenal gland [1] (Figs. 24.4 and 24.5).
Shortly after formation of the adrenal cortex, neural crest cells migrate into the center of this accumulation of cells to develop into the adrenal medulla. The cortex engulfs the medulla until the medulla is completely enclosed. During intrauterine life two cortical layers surround the medulla, first the zona glomerulus and then the zona fasciculate, a final layer the zona reticularis is formed after birth at around 3 years of age (Fig. 24.6). Therefore, the formation of the adre­nal gland is not completed until the end of three years of age.
During fetal development the migration of adrenocortical and medullary cells can result in accessory or ectopic tissue. Adrenocortical tissue has been found around the kidney, along gonadal vessels and uterus. This tissue can be responsible for the recurrence of Cush­ings post adrenalectomy secondary to excess ACTH secretion. Adrenal medullary cells can persist anywhere along the path of neural cell migration and that explains the occurrence of extra adrenal pheochromocytoma.
J.G.H. Hubbard et al. (eds.), Endocrine Surgery, Springer Specialist Surgery Series, DOI 10.1007/978-1-84628-881-4_24, Ó Springer-Verlag London Limited 2009
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Fig. 24.1. Development of the adrenal gland. Transverse
section of developing fetal abdomen. Adrenal cortex develops before medulla. (A) At 6 weeks of development adrenal cortex cells migrate from coelomic epithelium into body cavity. (B)At 7 weeks of development neural crest cells migrate into adrenal cortex. 1 ¼Spinal cord, 2 ¼Aorta, 3 ¼ Surface ectoderm, 4 ¼ Adrenal cortex, 5 ¼Coelomic epithelium, 6 ¼Hindgut, and 7 ¼Adrenal medulla.
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ENDOCRINE SURGERY
Fig. 24.2. Development of the adrenal gland. (A) 7 weeks; (B)
8 weeks; (C) 20 weeks; (D) newborn.
Anatomy
Due to the large size and position of the liver, the right adrenal gland and kidney lie inferior to the left adrenal gland and kidney. Therefore, the upper pole of the left kidney rises to the level of the eleventh rib but the right kidney is slightly lower [2]. In external appearance the right and left adrenal glands are dissimilar in that the former is pyramidal in shape whereas the left adrenal gland is larger and lunate in shape.
Anterior to the right adrenal gland is part of the right lobe of the liver and the inferior vena cava. Anterior to the left adrenal gland is part of the body and pylorus of the stomach, pancreas, and sometimes the spleen. Posterior to both adrenal glands is the diaphragm later­ally and the psoas fascia covering the psoas major muscles medially. Lateral to the adrenal gland is the superior pole of the kidney.
Fig. 24.3. White female, premature, stillborn. Length, 43 cm;
weight, 2.2 kg. 1 ¼Left kidney, 2 ¼Left adrenal gland, 3 ¼ Abdominal aorta, 4 ¼Inferior vena cava, 5 ¼Right adrenal gland, 6 ¼Left kidney. (Courtesy of Mr. Albert Van Schoor, Department of Anatomy, School of Medicine, Faculty of Health Sciences, University of Pretoria, South Africa).
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ADRENAL EMBRYOLOGY, ANATOMY, AND PHYSIOLOGY
Fig. 24.4. Relations of the right adrenal gland. 1 ¼Inferior
vena cava, 2 ¼Right adrenal gland, 3 ¼Right dome of dia­phragm, 4 ¼ Right kidney, 5 ¼Right psoas major muscle. (Photographed by Mr. Frank Addison, Walton Library, Newcastle University, England).
Superior to the right adrenal gland is the liver and superior tothe leftadrenal glandis the left dome of the diaphragm and spleen (Figs. 24.4 and 24.5).
The adrenal glands like other endocrine glands receive a rich blood supply. The blood supply to the adrenal glands arises from the inferior phrenic arteries, aorta, and renal arteries (Figs. 24.4 and 24.5). The superior adrenal artery arises from the inferior phrenic artery, the middle adrenal artery arises directly from the abdominal aorta, and the inferior adrenal artery arises from the renal artery (Fig. 24.5). These three groups of arteries form a plexus within the capsule of the adrenal gland. This capsular plexus gives rise to small arteries that descend through the cortex into the medulla where they branch into an
Fig. 24.5. Relations of the left adrenal gland. 1 ¼ Left adrenal
gland, 2 ¼Left adrenal vein, 3 ¼ Left renal vein, 4 ¼Left kidney, 5 ¼Spleen, 6¼Body of pancreas, 7¼Abdominal aorta, 8 ¼Left psoas major muscle. (Photographed by Mr. Frank Addison).
elaborate network of dilated capillaries sur­rounding the medullary secretory cells (Fig. 24.6). The capsular plexus also supplies the cortex by forming a network of capillary sinusoids.
The capillary sinusoids descend between the secretary cells of the cortex to drain at the corticomedullary junction into small veins that then drain into the central vein of the medulla. The medullary capillaries also drain into the central vein of the medulla [3]. Therefore, the venous return from the adrenal glands consists of a single vein that on the right drains directly into the inferior vena cava and on the left drains into the left renal vein (Fig. 24.5).
Lymphatic drainage from the cortex and medulla of adrenal glands is directly into the
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