copyright © 2004 pearson education, inc., publishing as benjamin cummings endocrine surgery

Copyright © 2004 Pearson Education, Inc., publishing as Benjamin Cummings

Endocrine Surgery


Primary Hyperparathyroidism (PHPT)

iPTH

Ca

PHPT


PHPT

• 0.1-3% of population.

• Common , over 100 000 new cases

diagnosed/y in US.

mm


PHPT

• Single parathyroid adenoma (80%-90%)

• Double adenomas (2%-15%)

• Asymmetric 4-gland hyperplasia (10%-15%)

• Carcinoma (<1% of patients)

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Symptoms and Signs

• Moans, groans, stones, and bones

• Hypercalcemia

• Bone disease

• Nephrolithiasis

• Hypophosphatemia

• Proximal renal tubular acidosis

• Hypomagnesemia

• Hyperuricemia

• Gout

• Anemia

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Manifestations of Hypercalcemia

• CNS dysfunction

• Muscle weakness

• Bowel hypomotility and constipation

• Peptic ulcer disease

• Pancreatitis

• Acute and chronic renal insufficiency

• Nephrogenic diabetes insipidus

• Distal renal tubular acidosis

• Nephrolithiasis

• Shortening of the QT interval

• Corneal calcium deposition (band keratopathy)

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Imaging

A.J. Coakley, A.G. Kettle and C.P. Wells et al., 99mTc sestamibi a new agent for parathyroid imaging, Nucl Med Commun 10 (1989), pp. 791–794

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• commonly used for cardiac imaging

• Avidly taken up by parathyroid tissue

• Reoperative setting


2002 NIH Workshop on Asymptomatic PHPT

• Serum Ca > 1.0 mg/dL above the upper limit of normal.

• Hypercalciuria (urinary Ca excretion > 400 mg/day) .

• Creatinine clearance that is <30% than that of age-matched normal subjects.

• Bone density at the hip, lumbar spine, or distal radius that is T score <-2.5.

• <50 years old.

• Difficult periodic follow-up.

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IOPTH MEASUREMENTS

• Recorded before resection of any enlarged glands

• Just before excising the gland.

• Postexcision IOPTH levels were measured 10 min or longer after resection of the enlarged parathyroid gland(s).

• A decrease of 50% or more in the IOPTH level was used to define successful parathyroidectomy.

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Why should virtually all patients be considered for surgical intervention?

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•Surgery offers the only cure

•Surgery is of benefit to symptomatic patients

•Surgery is of benefit to “asymptomatic” patients

•Increases bone density and decreases fracture rates


•Prolongs survival

•Improves neurocognitive symptoms

•92% of patients claim to feel better after parathyroidectomy, even when only 75% claim they felt "bad" before the operation.

•Regression of left ventricular hypertrophy.

•Cure of diabetes.

•Surgical cure rates are high

•Surgery is safe

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Why should virtually all patients be considered for surgical intervention?


Familial HPTH

• MEN type 1

• MEN type 2A

• Familial Isolated PHPT

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Surgical approach for MEN1

• Parathyroidectomy

• Thymectomy

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Subtotal Vs Total with autotransplantation

• Subtotal parathyroidectomy • Total with autotransplantation

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MEN 2A

• HPT 20-35%

• MTC > 95%

• Pheochromocytoma 42-50%

• Cutaneous lichen amyloidosis

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FIHPT

• Carefully screened for MEN, including a thorough FHx, ret oncogene testing, and exclude Pit &Panc neuroendocrine tumors.

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FIHPT

• Profound hypercalcemia, Nephrolithiasis, and severe osteoporosis.

• No definite genetics have been identified

• Jaw tumors (FIH-JT)

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PHPT in MEN1

• Concurrent ZE syndrome

parathyroidectomy (reducing ca levels can reduce gastrin secretion)

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PHPT in MEN2A

• MEN-2A must be biochemically screened for the presence of pheochromocytoma before parathyroidectomy.

• If present, parathyroidectomy should be delayed until the pheochromocytoma is addressed.

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Demographics of Thyroid Cancer

• ACS est 20,700 new cases of thyroid cancer in U.S. in 2002

• Cause-specific deaths in 2002 est to be 1300

• Occult cancer in autopsy studies reported to 4-35% worldwide


The New End Point

• The gland should be removed en bloc

• Care should be taken to remove the entire superior pole

• The RLN should be identified and followed to the point where it enters the larynx


Nodular hyperplasia

Adenoma

Cyst

Hashimoto thyroiditis

Malignancy

Differential Diagnosis of a Thyroid Nodule


Risk Factors for Thyroid Cancer

• History of head and neck radiation

• Male sex

• Family history of medullary carcinoma (MEN syndromes)

• Family history of papillary carcinoma

• Hoarseness (vocal cord paralysis)

• Fixation to adjacent structures

• Single, firm, cold nodule

• Nodule> 4cm

• Rapid tumor growth

• Enlarging thyroid nodule(s) on thyroid suppression

• Cervical lymphadenopathy


AGES AMES DAMES SAG MACIS

Age Age DNA Size Metastasis(Distant)

Grade

Metastasis Age Age Age

Extent Extent Metastasis(Distant)

Grade Completeness of resection

Invasion

Size


TYPES

Tumors of Follicular Cell Origin

Differentiated

Papillary

Follicular

Hürthle Cell

Undifferentiated

Anaplastic

Tumors of Parafollicular or C-cell Origin

Medullary

Other

Lymphoma

Squamous cell carcinoma,secondary tumors


MEN 2

• MEN 2A :

• MTC > 95% of the patients

• Pheochromocytoma 42-50%

• Hyperparathyroidism 20-35%

• Cutaneous lichen amyloidosis

• MEN 2B:

• MTC 100%

• Pheochromocytomas 50%

• Ganglioneuromas of the lips, tongue, eyelids, and gastrointestinal tract

• Marfanoid physical (but not cardiac) features

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Biochemistry

• Parafollicular C cells

• CTN

• CEA

• Corticotropin

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Pathology: Amyloid


CLINICAL PRESENTATION

• Most has already metastasized at the time of diagnosis.

• CLN

• 15% symptoms of upper aerodigestive tract compression or invasion such as dysphagia or hoarseness

• 5% distant disease

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CLINICAL PRESENTATION

• Calcitonin:

• Diarrhea

• facial flushing

• ACTH:

• Cushing's syndrome.

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Genetic screening

• Patients identified as carriers of a RET mutation will develop MTC and should undergo prophylactic Thyroidectomy.

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Treatment

• Total thyroidectomy

• CLN Dissection:

• from the level of the hyoid to the innominate vessels and laterally to the carotid arteries.

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MRND

• Preserve:

• SCM

• Spinal Accessoy N.

• IJV

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Pheo & Para in MEN2A

• MEN-2A must be biochemically screened for the presence of pheochromocytoma before parathyroidectomy.

• If present, parathyroidectomy should be delayed until the pheochromocytoma is addressed.

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Causes of Cushing’s Syndrome

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Cushing’s disease 68

Ectopic ACTH syndrome12

12

Ectopic CRH syndrome<1

<1

Adrenal adenoma 10

Adrenal carcinoma 8

Adrenal cortical hyperplasias

1

Pseudo-Cushing’s Syndrome

1

Major depression

Alcoholism

1


S & S

• General Central obesity Proximal muscle weakness Hypertension Headaches Psychiatric disorders

Skin Wide (>1 cm) purple striae Spontaneous ecchymoses Facial plethora Hyperpigmentation Acne Hirsutism Fungal skin infections

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S & S• Hypokalemic alkalosis

• Osteopenia Delayed bone age in children

• Menstrual disorders, decreased libido, impotence

• Glucose tolerance, diabetes mellitus

• Kidney stones Polyuria

• Elevated white blood cell count


Carcinoid Tumors


Products of carcinoid tumors

Dopamine Neurokinin A

Histamine Neurokinin B

Polypeptides Corticotropin (ACTH)

Kallikrein Gastrin

Pancreatic polypeptide Growth hormone

Bradykinin Peptide YY

Motilin Glucagon

Somatostatin Beta-endorphin

Vasoactive intestinal peptide Neurotensin

Neuropeptide K Chromogranin A

Substance P Prostaglandins

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5-HIAA

• Definitive diagnosis can be confirmed by the finding of elevated urinary 5-HIAA levels

• Asymptomatic patients ,only 75% sensitive for detecting the presence of 1ry tumors without metastatic disease.

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Chromogranin A

• Elevated in > 80% of patients with carcinoid tumors.

• Elevated level is an independent predictor of an adverse prognosis.

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Diagnostic Modalities

• CT :

• hepatic and lymph node metastases

• SB follow-through:

• kinking of bowel

• smooth luminal filling defect

• Sensitivity:30- 40%.

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Capsule Endoscopy


Diagnostic Modalities

• Somatostatin receptor scintigraphy:

• successfully localize primary and metastatic tumors in approximately 80% of patients

• useful staging procedure before surgical exploration to detect the extent of tumor burden and allow surgical planning.

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Surgery

• 1ry< 1 cm in diameter without evidence of regional lymph node metastasis:

• segmental intestinal resection is adequate.

• Lesions of TI or appendiceal lesios >2cm are best treated by R hemicolectomy.

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Gastrinoma

• Sporadic 75%

• MEN 1 25%

mm

mm

• Peptic acid hypersecretion:

• abdominal pain

• Diarrhea


MEN 1

• 12% manifest all three Ps

• Screen in patient with PHPT who <50y

• PHPT

• Pancreatic neuroendocrine tumors:

• ZES (54%)

• Insulinoma (21%)

• Pituitary tumors

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Secretin test in gastrinoma

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Insulinoma distribution

• Location:• Virtually all are intrapancreatic.

• Most are solitary (10% multiple)• Diameter:• 10% >2cm• 10% malignant. • 10% MEN-1 (Higher risk of recurrence).

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Another Rule of 10%


Symptoms

• Whipple triad:• symptoms of hypoglycemia (catecholamine release)• low blood glucose level (40 to 50 mg/ dL)• Relief of symptoms after intravenous administration of

glucose • Neuroglycopenic :confusion, visual change. • Sympathoadrenal : palpitations, diaphoresis, and tremulousness. • Seizure disorder is another common misdiagnosis.

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Localization Techniques True Positives(%)

• Ultrasonography 23

• Octreotide radioimaging (SRS) 86(50%)

• CT 43

• MRI 26

• Endoscopic ultrasonography 82

• Selective angiography 56

• Provocative angiography 65

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Surgery

• Benign:

• Enucleation:

• Malignant(10%):

• Cancer-type operation

• Metastatic, attempt to remove all primary and metastatic tumor to minimize persistent hyperinsulinism.

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Medical therapy

• Indications:

• insulinoma was missed during pancreatic exploration

• Pt not a candidate for or refuses surgery

• Metastatic disease.

• Diazoxide

• Octreotide

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copyright © 2004 pearson education, inc., publishing as benjamin cummings endocrine surgery

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