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- •Contents
- •1. Thyroid Embryology, Anatomy, and Physiology: A Review for the Surgeon
- •2. The Assessment of Thyroid Nodules
- •3. Thyroid: Fine-Needle Aspiration Biopsy
- •4. Thyroid Imaging
- •5. Multinodular Goiter
- •6. Thyrotoxicosis and Thyroiditis: Causes, Investigation, and Management
- •7. Molecular Biology of Thyroid Cancer
- •8. Well-Differentiated Thyroid Cancer: An Overview and the Chernobyl Effect
- •9. Poorly Differentiated and Undifferentiated Thyroid Cancer
- •10. Postoperative Management of Well-Differentiated Thyroid Cancer
- •11. Medullary Thyroid Cancer
- •12. Technique of Thyroidectomy
- •13. Lymph Node Dissection in Thyroid Cancer
- •14. Management of the Laryngeal Nerves and Voice
- •15. Embryology, Anatomy, and Physiology of the Parathyroid Glands
- •16. Presentation and Diagnosis of Primary Hyperparathyroidism
- •17. Parathyroid Localization and Imaging
- •18. Intraoperative PTH Monitoring
- •19. Focused Parathyroidectomy
- •20. Parathyroid: Bilateral Neck Exploration
- •21. Reoperative Parathyroid Surgery
- •22. Management of Secondary and Tertiary Hyperparathyroidism
- •23. Parathyroid Carcinoma
- •24. Adrenal Embryology, Anatomy, and Physiology
- •25. Adrenal Imaging
- •26. Adrenal Venous Sampling
- •27. Primary Hyperaldosteronism
- •29. Pheochromocytoma and Paraganglioma
- •30. Adrenocortical Carcinoma
- •31. Incidentaloma
- •32. Adrenal Metastases and Rare Adrenal Tumors
- •33. Technique of Open and Laparoscopic Adrenalectomy
- •34. Laparoscopic Retroperitoneal Adrenalectomy
- •35. Pancreas: Embryology, Anatomy, and Physiology
- •36. Pancreatic Imaging: The Value for Surgery of Neuroendocrine Pancreatic Tumors

Fig. 32.1. Bilateral adrenal metastases in a patient with
metastatic melanoma.
Fig. 32.2. Lymphoma metastasis to the adrenal gland.
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metastatic melanoma, metastasis from lymphoma, and from unknown primary malignancy, respectively.
Magnetic Resonance Imaging
MRI also exploits the intralesional fat content to
exclude malignancy. With the advent of
dynamic gadolinium-enhanced and chemicalshift imaging (CSI), MRI has become a very
useful diagnostic method in the characterization of adrenal masses. The chemical-shift MRI
technique is used often with demonstrated sensitivity of 81–100% and specificity of 80–100%
for differentiating adenomas from nonadenomas
[33–37]. Korobkin et al. [38] and Outwater et al.
[39] showed that the presence of histologic lipid
in many of the examined adenomas accounted
for the low attenuation on unenhanced CT, causing a loss in SI on chemical-shift MRI. The low
attenuation values of adenomas on nonenhanced
CT and the lower SI in opposed phase compared
to in-phase MRI result from intratumoral fat
content [38–41]. Adrenal MRI should include
T1-weighted axial images for anatomic detail
and T2-weighted axial images [41]. Fat suppression is useful to prevent degradation of heavily
T2-weighted images by periadrenal fat. MRI also
has the added advantage of avoiding patient
exposure to radiation and is useful in patients
with allergy to iodine-containing contrast media.
Both CT and MRI are useful in excluding malignancy within an adrenal lesion; however, they
fall short of confirming adrenal malignancy.
The definitive diagnosis of malignancy usually
requires percutaneous biopsy or surgical pathology. However, noninvasive imaging can reduce
the need for percutaneous biopsy of adrenal
lesions found in oncologic patients [42, 43].
Fig. 32.3. Adrenal metastasis in a patient with unknown
primary carcinoma.
Positron Emission Tomography
PET isnow the most common imaging technique
for surveillance of patients with a history of
malignancy [44]. As such, many incidental adrenal lesions in oncology patients are initially discovered on PET scan. The most commonly used
technique uses fluorodeoxyglucose (FDG/PET),
which relies on uptake of FDG by metabolically
active cells as a method of identifying metastatic
lesions. PET has high sensitivity for identifying
adrenal lesions; however, the specificity for
malignant adrenal masses is poor [44–47]. In an

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ADRENAL METASTASES AND RARE ADRENAL TUMORS
effort to avoid false-positive results from adrenal
adenomas (which also take up FDG to a varying
degree), some have modified the PET criteria for
a malignant lesion. Yun et al. [44] defined lesions
with subjective uptake (SUV) greater than or
equal to the liver as positive for metastasis and
less uptake than the liver as negative. Using these
criteria, they reported 100% sensitivity and 94%
specificity in 50 adrenal lesions. In the rare cases
in which an adrenal adenoma does showelevated
metabolic activity on PET, the activity may
reflect inflammation within the lesion [48]. Several studies also highlight that the addition of CT
scan to PET has improved the sensitivity for
detection of malignant lesions [49, 50]. FDG/
PET is unable to distinguish adrenal cortical carcinoma (ACC) from metastatic disease in the
adrenal glands. Nor can it reliably diagnose
pheochromocytomas, metastatic disease, and
lymphomas, which generally exhibit high glycolytic activity [51].
Percutaneous Biopsy
Although adrenal biopsy is of limited utility in
patients with incidentally discovered adrenal
masses, CT-guided adrenal biopsy is an important diagnostic tool in patients with a personal
history of extra-adrenal malignancy. Cytologic
evidence of adrenal tissue excludes metastatic
adrenal malignancy and prevents unnecessary
evaluation and patient anxiety, whereas a
biopsy result positive for malignancy facilitates
therapeutic planning in patients with no evidence of other metastases. Percutaneous fineneedle aspiration (FNA) biopsies of the adrenal
gland that demonstrate malignancy have a positive predictive value of 100% and a negative
predictive value for malignancy of 92% [52].
Complication rates vary from 8 to 13%,
although most are mild and self-limiting [53,
54]. The overall sensitivity of core biopsy for
malignancy is reported 99% and the specificity
as 96% [52]. However post-procedure bleeding
is more common with core biopsies. It is
imperative to exclude adrenal medullary
hyperfunction prior to biopsy, so as to avoid
hemodynamic and vascular complications
such as severe hypertension, myocardial
infarction, or cerebrovascular incidents. There
are also reports of tumor seeding of the needle
tract [55].
Management of Adrenal
Metastases
The appropriate management of adrenal metastases depends on the type and extent of the
primary malignancy, patient comorbidity and
disease-free interval (DFI, the interval between
diagnosis of the primary malignancy and
the recognition of the adrenal metastases).
Figure 32.4 serves as a guideline in the manage-
ment of adrenal metastases. Since adrenalectomy is usually not curative, one must balance
the risk of surgery with potential benefit of
prolonging the time to recurrence.
Of all the outcome predictors in adrenal
metastases, DFI may be the most predictive.
Synchronous lesions are described as metastases that are recognized within 6 months of
the primary malignancy (DFI less than 6
months), while metachronous lesions are recognized more than 6 months after diagnosis of the
primary tumor. Surgery and chemotherapy
have been shown to increase survival in synchronous adrenal metastases from lung cancer
[56]. Metachronous metastases are extremely
rare, possibly because of the short life span of
patients with lung cancer [57]. In a review of 18
solitary metachronous adrenal metastasis (15
unilateral, 3 bilateral), identified in patients
with operable nonsmall-cell lung cancer
between 1965 and 1999, the median interval
between pulmonary resection and treatment of
adrenal lesion was 11.5 months. The median
survival after adrenalectomy and postoperative
chemotherapy was 19 months, compared with
15 months after chemotherapy alone, 14months
after adrenalectomy alone, and 8 months after
palliative radiation therapy.
There is now evidence that resection of isolated adrenal metastases may offer a survival
benefit [56, 58, 59]. Kim et al. [60] and Lo et al.
[58] recommend aggressive treatment in
patients who undergo complete resection of
the primary lung tumor and have a DFI more
than 6 months. Adrenal metastases from lung
cancer and melanomas represent the bulk of
these patients. Kim et al. conducted a retrospective review of 37 patients who had undergone adrenalectomy for metastatic disease at
their institution between 1986 and 1996. Fiveyear survival was 24%. DFI >6monthsand
complete resection were the only predictors of

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ENDOCRINE SURGERY
Fig. 32.4. Flowchart for management of adrenal metastases.
improved survival. Lung cancer was the most
common primary tumor, followed by renal and
colorectal cancer [60]. Sarela et al. [61] with a
retrospective review of 41 patients who underwent adrenalectomy during 1997–2002 at this
institution showed an overall 5-year survival
of 29%.
Mercier et al. described the management of
23 patients, who underwent complete resections
of solitary adrenal metastasis after surgical
treatment of nonsmall-cell lung cancer. The
diagnosis of adrenal metastasis was synchronous with the diagnosis of nonsmall-cell lung
cancer in 6 of the 23 patients and metachronous
in 17 patients. The median DFI for the patients
with metachronous adrenal metastasis was 12.5
months (range 4.5–60.1 months). The overall
5-year survival of these 23 patients was 23%
after adrenalectomy for solitary adrenal metastasis. Univariate and multivariate analysis
showed that a DFI greater than 6 months was
an independent and significant predictor of
increased survival in patients after adrenalectomy. All patients with a DFI of less than
6 months died within 2 years of the operation.
In contrast, the 5-year survival rate was 38%
after resection of a solitary adrenal metastasis
that developed more than 6 months after lung
resection. Findings from other studies also support the concept that a DFI less than 6 months

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ADRENAL METASTASES AND RARE ADRENAL TUMORS
and synchronous metastasis were associated
with significantly worse survival rates and suggested either tumor aggressiveness or advanced
tumor stage that was undetected when the
primary lung carcinoma was resected [62].
Luketich and Burt reported a much better
median survival (31 months) in patients with
synchronous metastases after neoadjuvant chemotherapy and adrenal resection than did Mercier and associates. Other factors, such as the T
and N staging of the primary tumor, histology,
administration of adjuvant therapy, or size of
the metastases, did not affect survival.
A retrospective study of 24 patients with melanoma metastatic to the adrenal glands [63]
showed that eight patients underwent resection
for cure, two patientsunderwentpartialresection
of large unilateral adrenal metastases, and 14
patients with unresectable tumors had chemotherapy or were treated symptomatically.
Mean survival in the group that underwent resection for cure was 59 months (with four of the
eight patients living more than 5 years), whereas
survival in the unresectable group was 15
months. An important caveat is that many of
these studies consist of highly selected patients;
therefore these results may not be easily extrapolated to all patients with adrenal metastases.
We could find no convincing evidence supporting adrenalectomy for patients in whom the
source of the primary malignancy is unknown.
Technique of Adrenalectomy
Open adrenalectomy has been the preferred surgical approach in patients with primary or metastatic
adrenal cancer. This technique ensured en bloc
excision of tumor by the tumor. However complication rates associated with open adrenalectomy
are high. With increasing laparoscopic experience
many surgeons nowadvocate laparoscopic adrenalectomy for metastases and malignantlesions of the
adrenal gland [64]. In selected patients laparoscopic adrenalectomy can be performed in the
absence of local invasion [65]. Patients who require
a more radical excision may be best served by open
adrenalectomy [66]. Although laparoscopic resection of large tumors (>15 cm) has been reported
[67], this is more technically challenging because of
the limited visibility, increased vascularity, and the
difficulty with manipulation and retraction of a
large tumor. Therefore many surgeons still recommend open adrenalectomy for very large tumors.
In a retrospective review of 170 patients
comparing laparoscopic and open adrenalectomy for ACC [68], tumor fracture occurred
during attempted bag extraction in one patient,
while another patient underwent open conversion due to uncontrolled hemorrhage. There
are at least two independent reports of the
rapid development of peritoneal carcinomatosis after laparoscopic adrenalectomy for ACC
[69, 70]. On the other hand, a number of studies show that laparoscopic adrenalectomy is
associated with minor postoperative discomfort, reduced hospital stay, and reduced complication rate [71–74]. Some authors report no
port site metastases or loco-regional recurrences after long-term follow-up of patients
who underwent laparoscopic adrenalectomy
for malignant tumors [75, 76]. Despite favorable results from major centers, several isolated cases of local tumor recurrence have
been reported by other groups. The recurrences developed in conjunction with the
appearance of metastases at other sites in the
body; however, in some cases, the pattern of
recurrence suggested that tumor spread was by
the laparoscopic dissection, pneumoperitoneum, or both [69, 77, 78].
Sarela et al. [61] performed a retrospective
study on 41 patients undergoing open or laparoscopic adrenalectomy for adrenal gland metastases. The overall 5-year survival rate was 28
months. The size of the adrenal metastases
removed by laparoscopic adrenalectomy was significantly smaller than those removed by the open
approach (median diameter 4.5 vs 7.4 cm). The
authors found that a DFI exceeding 6 months was
the only significant predictor of improved survival. Taken together, these results suggest that
well-selected patients with adrenal metastases
can safely undergo laparoscopic adrenalectomy
with no oncological disadvantage [65, 66, 79].
Ablation of Adrenal Metastases
Given the palliative nature of interventions for
adrenalmetastasesitisnotsurprisingthat
attempts have been made to ablate these
lesions operatively or percutaneously. CTguided radiofrequency ablation may be the
most popular technique, especially in patients
who are poor surgical candidates [80, 81]. Both

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the adrenal mass and the location of the RF
electrode are reliably seen on CT [81]. Radiofrequency ablation uses alternating RF current
to generate heat and induce tissue necrosis. RF
electrodes placed in the tumor cause local ion
agitation and heat to cause local tissue destruction. There is minimal morbidity associated
with this procedure and the follow-up imaging
and biochemical results indicate that RF ablation effectively destroys both native adrenal
tissue and adrenal metastases, particularly
those smaller than 5 cm in diameter [81]. RF
treatment can also be performed on an outpatient basis with minimal morbidity [81, 82].
Alpha and beta blockers may be considered in
patients undergoing RF ablation of adrenal
lesions to avoid hypertensive crisis as this
has been reported as a complication of RF
performed on a liver metastasis adjacent to a
normal adrenal gland [81]. Other palliative
techniques include selective arterial embolization and injection of alcohol or acetic acid.
Rare Adrenal Tumors
Adrenal Hemorrhage
Focal or diffuse adrenal hemorrhage is seen in
0.1–1.1% of autopsy cases [83]. Adrenal hemorrhage commonly occurs in association with
trauma [84], surgery [85], anticoagulant therapy
[86], septicemia [87], hypotension, or tumor
(metastases, carcinoma, pheochromocytoma, or
adenoma). In such cases, bilateral adrenal
hemorrhage usually develops and acute adrenal
insufficiency is clinically present [88]. Increased
adrenocorticotropic hormones have been implicated in adrenal hemorrhage, and there are
reports of adrenal hemorrhage in patients with
inflammatory bowel disease treated withintravenous adrenocorticotropic hormone. Animal studies confirm that adrenocorticotropic hormones
cause the adrenal glands to enlarge and become
hyperemic, eventually leading to necrosis and
hemorrhage [89]. Other mechanisms for adrenal
hemorrhage include stress or adrenal medullary
venous thrombosis [90–93]. Adrenal hemorrhage is best managed by correcting clinically –
evident coagulopathy and replacing blood
components as necessary. These lesions are
usually self-limiting. Hemorrhagic pseudocysts
are the most common adrenal cysts.
Adrenal Cysts
Adrenal cysts are rare with an incidence of less
than 0.1% [94]. They comprise 4–22% of adrenal incidentalomas [8].The most common presentation is an incidentaloma; however, some
patients may also develop abdominal pain and
mass [95]. Adrenal cysts may be benign or
malignant. The endothelial cysts are lymphangiomatous or angiomatous. These are small and
multiple. The epithelial cysts are most likely
derived from embryonic rests and include cystic
adenomas, embryonal cysts, and glandular
retention cysts. The benign adrenal cysts have
a reported incidence at autopsy of 0.064–0.18%
[96]. With the advent and widespread use of
imaging cystic diseases of the adrenal gland
are being found more frequently today. Due to
its low incidence there are few reports of laparoscopic management of adrenal cysts [97, 98].
Adrenal cysts are more common in women
and in patients in fourth and fifth decade of life
[99]. Although most adrenal cysts are nonfunctioning and asymptomatic, they can become large
enough to cause nonspecific abdominal or flank
pain, or hypertension [100, 101]. Pseudocysts
often arise from hemorrhage within the adrenal
gland, sometimes secondary to stress, birth,
trauma, and surgery. Adrenal cysts can also
occur in association with benign and malignant
tumors. The overall incidence of malignancy in
adrenal cysts is estimated to be about 7% [95].
For adrenal cysts 5 cm or greater confirmed
by the imaging or smaller ones in which malignancy is suspected, a complete endocrine evaluation is recommended [102]. It should include
serum potassium, renin, cortisol, and 24-h urinary catecholamines, metanephrines, vanillylmandelic acid, 17-hydroxycorticosteroids,
and aldosterone. Small, asymptomatic, or nonfunctioning cysts can be followed clinically without intervention [103].When adrenal cysts are
6 cm or greater, symptomatic or functioning or
malignancy is suspected on imaging, surgical
removal is recommended [103]. Surgical intervention can be open [104] with cyst enucleation or en
bloc adrenalectomy, or laparoscopic [97, 105, 106]
with cyst decortication and adrenalectomy.

435
ADRENAL METASTASES AND RARE ADRENAL TUMORS
Whilethe endothelial cysts have been reported
to be the most common subtype of adrenal cysts
in some reports [107], there is evidence that adrenal pseudocysts are more common in three
different reports [103]. The separation into
endothelial cysts and pseudocysts might be clinically insignificant since some pseudocysts are
believed to represent endothelial cysts which
have lost the endothelial lining [108].
Lymphangioma
Lymphangiomas represent 16% of all adrenal
cysts. They are usually asymptomatic and small,
and discovered incidentally during abdominal
imaging for other reasons. Cyst wall calcification
mayalsobepresent.Nointerventionisnecessary
if the cyst is simple and has no solid component
and if the patient is asymptomatic. Adrenalectomy is indicated if the cyst enlarges or if the
patients develop symptoms related to the cyst.
Fig. 32.5. Myelolipoma of the right adrenal gland.
Myolipomas
Pseudocysts
A pseudocyst lacks an epithelial lining and often
is a result of hemorrhage or infarction. An adrenal pseudocyst is a cystic lesion arising within the
adrenal gland that is surrounded by a fibrous
tissue wall devoid of a recognizable lining layer.
Calcification in the wall of a cyst is suspicious for
a pseudocyst or a parasitic cyst. An adrenal
tumor can also undergo cystic degeneration and
form a pseudocyst. Adrenal cysts may become
secondarily infected. Surgical excision is recommended in the presence of symptoms or if there
is suspicion of malignancy.
Parasitic Infections
Hydatid cyst can present as a cyst in relation to
the adrenal gland. Serology for Echinococcus is
performed for diagnosis. Most sensitive screening
tests are ELISA and indirect hemagglutination
tests. Detection of antibody to Echinococcal antigen and a CT showing peculiar morphology of
daughter cysts will confirm the diagnosis. Aspiration is not recommended if parasitic cyst is suspected because of the risk of dissemination or
anaphylaxis [109]. Adrenalectomy with care to
avoid disruption of the cyst is the treatment of
choice.
Adrenal myolipomas are rare tumors (0.08–2%
of the population), benign, nonfunctional, and
asymptomatic. Symptomatic cases often manifest with abdominal pain attributable to spontaneous rupture of the mass, intratumoral hemorrhage, or compression of peritumoral tissues
[110]. The presence of the fat in the tumor is
the key to the diagnosis of the myelolipoma.
Currently, there is no consensus regarding the
appropriate management of adrenal myelolipomas. Surgical removal is indicated for symptomatic lesions or if malignancy is suspected
[110]. Figure 32.5 demonstrates myelolipoma
of the right adrenal gland.
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33
Technique of Open and Laparoscopic Adrenalectomy
Dina M. Elaraj and Quan-Yang Duh
Introduction
Evaluation of an adrenal mass is one of the most
unique and interesting problems in the field of
surgery. It entails determining whether the adrenal mass is functional or nonfunctional, benign
or malignant, and primary (i.e., arising within
the adrenal gland) or metastatic from another
site. The work-up consists of biochemical testing,
imaging studies, and, rarely, invasive testing
such as selective venous sampling. In the case
of a nonfunctional adrenal mass, patients should
undergo age- and gender-appropriate risk factor-screening tests in order to exclude common
malignancies. Rarely, in the case of a patient with
a history of cancer or in the case of a patient with
bilateral adrenal masses, a fine-needle aspiration
biopsy (after excluding the possibility of pheochromocytoma) may be necessary.
Indications for adrenalectomy are listed in
Table 33.1. Details regarding the work-up of a
patient with an adrenal mass or the diagnosis of
patients with functioning adrenal tumors are found
in Chapter 31. The presentchapter isfocused onthe
various techniques of adrenalectomy.
In addition to the usual steps taken to prepare a
patient for a major operation, patients with functional tumors require special consideration.
Pheochromocytoma
Preparation for surgery in a patient with pheochromocytoma consists of alpha blockade for
1–3 weeks before operation. The most commonly used agent is phenoxybenzamine, a
long-acting alpha adrenergic antagonist. In
addition, if the patient has a history of arrhythmias, or if after adequate alpha blockade the
patient experiences persistent tachycardia or
extra-systoles, a beta blocker is added. The
most commonly used agent is propranolol. In
addition, because patients with pheochromocytomas tend to have intravascular volume depletion due to chronic vasoconstriction, volume
expansion is an essential part of the preoperative preparation [1]. Furthermore, as 25–75% of
patients with pheochromocytoma may have
impaired glucose tolerance [2], appropriate
blood glucose control is also important.
Aldosteronoma
Preoperative Preparation
Since all patients with aldosteronoma have
The preoperative preparation of the patient
depends on the results of the hormonal testing
as well as the clinical and biochemical diagnosis.
J.G.H. Hubbard et al. (eds.), Endocrine Surgery, Springer Specialist Surgery Series,
DOI 10.1007/978-1-84628-881-4_33, Springer-Verlag London Limited 2009
hypertension, appropriate blood pressure con-
trol is important. Patients usually require multi-
ple agents to control their blood pressure. Most
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