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Flashcards in Endocrine: Pathoma, BRS, FA Deck (248):
1

Benign tumor of anterior pituitary cells

Pituitary Adenoma

2

May be functional (Hormone-producing) or non-functional

Pituitary Adenoma

3

The nonfunctional version of this presents with mass effect.

Pituitary Adenoma

4

Presents with bitemporal hemianopsia due to compression of optic chiasm

Non-functional Pituitary Adenoma

5

Presents with hypopituitarism due to compression

Non-functional Pituitary Adenoma

6

Presents as galactorrhea and amenorrhea in females

Prolactinoma

7

Presents as decreased libido and headache in males

Prolactinoma

8

Most common pituitary adenoma type

Prolactinoma

9

Treatment is dopamine agonists

Prolactinoma

10

Stains chromophobic

Prolactinoma

11

Tumor with hypersecretion of growth hormone

Somatotropic adenoma

12

Presents as gigantism in children with increased linear bone growth

Somatotropic adenoma

13

Presents as acromegaly in adults (enlarged bones of hands, feet and jaw) and growth of visceral organs

Somatotropic adenoma

14

Cardiac failure most common cause of death

Somatotropic adenoma

15

Presents with enlarged tongue

Somatotropic adenoma

16

Staining is acidophilic

Somatotropic adenoma

17

Results in increased IGF-1 and somatomedin C

Somatotropic adenoma

18

Secondary diabetes mellitus is often seen

Somatotropic adenoma

19

Diagnosed by high GH and IGF-1 levels along with lack of GH suppression by oral glucose

Somatotropic adenoma

20

Treatment is octreotide, GH receptor antagonists or surgery

Somatotropic adenoma

21

Tumor secreting ACTH leading to Cushing syndrome

ACTH cell adenoma

22

Insufficient production of hormones by the anterior pituitary gland

Hypopituitarism

23

Symptoms arise with 75% of parenchyma is lost

Hypopituitarism

24

Generalized pan-hypopituitarism

Pituitary Cachexia - Simmonds Disease

25

Caused by pituitary tumors (adults - adenomas and children - craniopharyngioma) and Sheehan Syndrome

Pituitary Cachexia - Simmonds Disease

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Pregnancy related infarction of pituitary gland

Sheehan Syndrome

27

Gland doubles in size during pregnancy but blooo supply does not increase.

Sheehan Syndrome

28

Presents as poor lactation, loss of pubic hair, and fatigue

Sheehan Syndrome

29

Congenital defect of the sella

Empty Sella Syndrome

30

Herniation of the arachnoid and CSF into the sella compresses pituitary gland

Empty Sella Syndrome

31

Results in growth retardation (dwarfish) in children

Growth Hormone Deficiency

32

Results in increased insulin sensitivity (hypoglycemia), weakness, and anemia in adults

Growth Hormone Deficiency

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Results in retarded sexual maturation in children

Gonadotropin Deficiency

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Results in loss of libido, impotence, loss of muscle & facial hair in men, and amenorrhea and vaginal atrophy in women

Gonadotropin Deficiency

35

Results in secondary hypothyroidism

TSH Deficiency

36

Results in secondary adrenal failure

ACTH Deficiency

37

ADH deficiency

Central Diabetes Insipidus

38

Due to hypothalamic or posterior pituitary pathology

Central Diabetes Insipidus

39

Signs and symptoms all due to loss of free water

Central Diabetes Insipidus

40

Presents with polyuria, polydipsia, hypernatremia, and low urine osmolality

Central Diabetes Insipidus

41

Water deprivation test fails to increase urine osmolality

Central Diabetes Insipidus

42

Treatment is desmopressin

Central Diabetes Insipidus

43

Impaired renal response to ADH

Nephrogenic Diabetes Insipidus

44

Due to inherited mutation to ADH receptor or drugs such as lithium

Nephrogenic Diabetes Insipidus

45

Presents with polyuria, polydipsia, hypernatremia, and low urine osmolality

Nephrogenic Diabetes Insipidus

46

No response to desmopressin

Nephrogenic Diabetes Insipidus

47

Normal ADH levels

Nephrogenic Diabetes Insipidus

48

Water deprivation test fails to increase urine osmolality

Nephrogenic Diabetes Insipidus

49

Due to excessive ADH secretion

Syndrome of Inappropriate ADH Secretion

50

Most often due to ectopic production especially small cell carcinoma of the lung

Syndrome of Inappropriate ADH Secretion

51

Clinical features are based on retention of free water

Syndrome of Inappropriate ADH Secretion

52

Presents with hyponatremia and low serum osmolality

Syndrome of Inappropriate ADH Secretion

53

Presents with mental status changes, seizures and cerebral edema

Syndrome of Inappropriate ADH Secretion

54

Treatment is free water restriction, demeclocycline, conivaptan, tolvaptan

Syndrome of Inappropriate ADH Secretion

55

Serum aldosterone is low

Syndrome of Inappropriate ADH Secretion

56

Development of large pituitary adenomas following bilateral adrenalectomy due to loss of feeback inhibition on growth of pre-existing pituitary microadenomas

Nelson Syndrome

57

Cystic dilatation of thyroglossal duct remnant

Thyroglossal Duct Cyst

58

Presents as anterior neck mass

Thyroglossal Duct Cyst

59

Most common thyroid congenital anomaly

Thyroglossal Duct Cyst

60

Persistence of thyroid tissue at base of tongue

Lingual Thyroid

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Presents as base of tongue mass

Lingual Thyroid

62

Increased level of circulating thyroid hormone

Hyperthyroidism

63

Presents with increase in basal metabolic rate (increased ATPase synthesis)

Hyperthyroidism

64

Presents with increase symapthetic nervous system activity (Increased Beta1-receptors)

Hyperthyroidism

65

Autoantibody IgG stimulates TSH receptor (Type II HS)

Graves Disease

66

Most common cause of hyperthyroidism

Graves Disease

67

Presents most commonly in women of childbearing age (20-40)

Graves Disease

68

Increased incidence in HLA-DR3 and HLA-B8 positive individuals

Graves Disease

69

Presents with a diffuse goiter

Graves Disease

70

Presents with exophthalmos and pretibial myxedema due to TSH receptors in orbit and shin that produce glycosaminoglycan

Graves Disease

71

Irregular follicles with scalloped colloid seen on histology

Graves Disease

72

Lab findings: Increased total and free T4 with Hypocholesterolemia, Increased serum glucose
decreased TSH

Graves Disease

73

Fatal complication of Grave's Disease

Thyroid Storm

74

Due to elevated catecholamines and massive hormone excess in response to stress.

Thyroid Storm

75

Presents as arrhythmia, hyperthermia, and vomiting with hypovolemic shock

Thyroid Storm

76

Treat with 3 P's: Propranolol, Propylthiouracil, and Prednisolone

Thyroid Storm

77

Combination of hyperthryoidism, nodular goiter, and absence of exophthalmos

Plummer Disease

78

Teratoma made up of thyroid tissue

Struma ovarii

79

Due to relative iodine deficiency

Multinodular Goiter

80

Nontoxic, but can become TSH-independent over time.

Multinodular Goiter

81

Follicular cells working independent of TSH due to mutation in TSH receptor

Toxic Multinodular Goiter

82

Increased release of T3 and T4

Toxic Multinodular Goiter

83

Thyrotoxicosis if a patient with iodine deficiency goiter is made iodine replete

Jod-Basedow Phenomenon

84

Hypothyroidism in neonates and infants

Cretinism

85

Presents as 6 P's: Pot-bellied, Pale, Puffy face, Protruding umbilicus, Protuberant tongue, and Poor Brain development

Cretinism

86

Causes: Maternal hypothyroidsm during pregnancy, thyroid agenesis, dyshormonogenetic goiter, and iodine deficiency

Cretinism

87

Most common cause in US is thyroid dysgenesis

Cretinism

88

Congenital defect in thyroid hormone production, usually with thyroid peroxidase

Dyshormonogenetic goiter

89

Hypothyroidism in older children or adults

Myxedema

90

Presents with myxedema, weight gain, cold intolerance, slow mentally

Myxedema

91

Common causes: Iodine deficiency, Hashimoto Thyroiditis, therapy for hyperthyroidism, and primary idiopathic

Myxedema

92

Decreased serum free T4 & T3. Increased serum TSH. Hypercholesterolemia

Hypothyroidism

93

Most common cause of hypothyroidism in regions with normal iodine nutrition

Hashimoto Thyroiditis

94

Autoimmune destruction of thyroid gland associated with HLA-DR5

Hashimoto Thyroiditis

95

Initially presents as hyperthyroidism due to follicle damage

Hashimoto Thyroiditis

96

Anti-TSH-R, Anti-thyroglobulin and antithyroid peroxidase antibodies are often present

Hashimoto Thyroiditis

97

Chronic inflammation with germinal centers and Hurthle cells are seen

Hashimoto Thyroiditis

98

Increased risk for B-Cell (Marginal zone) Lymphoma

Hashimoto Thyroiditis

99

Finding: Nontender but enlarged thyroid

Hashimoto Thyroiditis

100

Follows a viral infection

Subacute Granulomatous Thyroiditis (De Quervain)

101

Presents as tender thyroid with transient hyperthyroidism

Subacute Granulomatous Thyroiditis (De Quervain)

102

Self-limited hypothyroidism is seen

Subacute Granulomatous Thyroiditis (De Quervain)

103

More common in women

Subacute Granulomatous Thyroiditis (De Quervain)

104

Chronic inflammation with extensive fibrosis of thyroid gland

Reidel Fibrosing Thyroiditis

105

Presents as hypothyroidism with 'hard as wood' non tender thyroid gland

Reidel Fibrosing Thyroiditis

106

Fibrosis may extend to local structures such as an airway

Reidel Fibrosing Thyroiditis

107

Manifestation of IgG4-related systemic disease

Reidel Fibrosing Thyroiditis

108

Clinically mimics anaplastic carcinoma, but patients are younger with this and malignant cells are absent.

Reidel Fibrosing Thyroiditis

109

Benign proliferation of follicles surrounded by a fibrous capsule

Follicular Adenoma

110

Nonfunctional usually but can secrete thyroid hormone

Follicular Adenoma

111

Most common type of thyroid carcinoma

Papillary Carcinoma

112

Exposure to ionizing radiation in childhood is a major risk factor

Papillary Carcinoma

113

Comprised of papillae lined by cells with clear "Orphan-Annie eye" or "Ground-glass" nuclei and nuclear grooves.

Papillary Carcinoma

114

Often associated with psammoma bodies

Papillary Carcinoma

115

Often spreads to cervical lymph nodes, but prognosis is excellent

Papillary Carcinoma

116

Associated with a change in chromosome 10 forming a RET-PTC fusion gene that acts as tyrosine kinase

Papillary Carcinoma

117

Associated with mutations in B-type Ras Kinase (BRAF)

Papillary Carcinoma

118

Malignant proliferation of follicles (uniformly shaped) surrounded by a fibrous capsule with invasion through the capsule

Follicular Carcinoma

119

Cannot be distinguished from follicular adenoma by FNA

Follicular Carcinoma

120

Metastasis through the blood

Follicular Carcinoma

121

Prognosis is good but not as good as papillary carcinoma

Follicular Carcinoma

122

Malignant proliferation of parafollicular C Cells

Medullary Carcinoma

123

Results in high levels of Calcitonin being produced causing hypocalcemia

Medullary Carcinoma

124

Biopsy reveals sheets of malignant cells in an amyloid stroma

Medullary Carcinoma

125

Familial cases are due to multiple endocrine neoplasia 2A and 2B

Medullary Carcinoma

126

Associated with medullary carcinoma, pheochromocytoma and parathyroid adenomas

Multiple Endocrine Neoplasia 2A

127

Associated with medullary carcinoma, pheochromocytoma, and ganglioneuromas of oral mucosa

Multiple Endocrine Neoplasia 2B

128

Sporadic and familial forms are both associated with RET oncogene mutation

Medullary Carcinoma

129

Undifferentiated malignant tumor thyroid in elderly

Anaplastic Carcinoma

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Often invades structures leading to dysphagia or respiratory compromise

Anaplastic Carcinoma

131

Poor prognosis.

Anaplastic Carcinoma

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Excess PTH due to a disorder of a parthyroid gland

Primary Hyperparathyroidism

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Most common cause is a parathyroid adenoma

Primary Hyperparathyroidism

134

SPoradic parathyroid hyperplasia and parathyroid carcinoma are less common causes

Primary Hyperparathyroidism

135

MOst often presents as asymptomatic hypercalcemia

Primary Hyperparathyroidism

136

Can also present with groans (constipation), stones (hypercalciuria), bones (osteitis fibrosa cystica), and psychiatric overtones (depression)

Primary Hyperparathyroidism

137

Lab findings: Increased PTH, increased serum calcium, decrease serum phosphate, increase urinary cAMP, and increase serum alkaline phosphatase

Primary Hyperparathyroidism

138

Also will present with ab or flank pain due to kidney stones or acute pancreatitis and also peptic ulcers

Primary Hyperparathyroidism

139

Cystic bone spaces filled with brown fibrous tissue

Osteitis fibrosa cystica

140

Also known as Von Recklinghausen disease of bone

Osteitis fibrosa cystica

141

Excess production of PTH due to a disease process extrinci to parathyroid gland

Secondary Hyperparathyroidism

142

Most common cause is chronic renal failure

Secondary Hyperparathyroidism

143

Lab findings: Increased PTH, decreased serum calcium, increased serum phosphate, and increase alkaline phosphatase.

Secondary Hyperparathyroidism

144

This occurs in spite of correction of hypocalcemia and pre-existing secondary hyperparathyroidism from chronic renal disease

Tertiary Hyperparathyroidism

145

Lab findings: Extremely high PTH and increased Calcium

Tertiary Hyperparathyroidism

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Cause is development of an adenoma in a previously hyperplastic gland

Tertiary Hyperparathyroidism

147

Impaired hydroxylation of 25-hydroxycholecalciferol

Vitamin D-dependent rickets

148

Decreased absorption of calcium and increased PTH secretion

Vitamin D-dependent rickets

149

Causes include autoimmune damage, surgical excision, and DiGeorge Syndrome

Hypoparathyroidism

150

Findings: Muscle spasms when using blood pressure cuff (Trousseau sign) or tapping on facial nerve (Chvostek sign)

Hypoparathyroidism

151

Labs reveal decreased PTH and decrease serum calcium

Hypoparathyroidism

152

Also known as Albright Hereditary Osteodystrophy

Pseudohypoparathyroidism

153

Autosomal dominant unresponsiveness of kidney to PTH

Pseudohypoparathyroidism

154

Findings: Shortened fourth and fifth metacarpals and metatarsals, short stature, and hypocalcemia

Pseudohypoparathyroidism

155

Due to a mutation in GNAS1 that encodes G proteins that respond to hormones

Pseudohypoparathyroidism

156

Selective paternal imprinting results in a mother passing on a mutated GNAS1 to her offspring

Pseudohypoparathyroidism

157

Skeletal abnormalities without hormone dysfunction due to transmission of mutant paternal GNAS1 allele

Pseudopseudohypoparathyroidism

158

Excess Cortisol

Cushing Syndrome

159

Findings: Muscle weakness, moon facies, buffalo hump, and truncal obesity, abdominal striae, osteoporosis, hypertension, hyperglycemia

Cushing Syndrome

160

Causes: Exogenous corticosteroids (bilateral adrenal atrophy), primary adrenal tumor, ACTH-secreting tumor (bilateral adrenal hyperplasia), and paraneo-plastic ACTH secretion (bilateral adrenal hyperplasia)

Cushing Syndrome

161

When caused by a adrenal tumor, this can't be suppressed by exogenous adrenal steroids

Cushing Syndrome

162

When caused by a pituitary tumor, this can be suppressed by exogenous adrenal steroids

Cushing Syndrome

163

Hypercorticism due to a corticotropic adenoma of pituitary or multiple very small pituitary adenomas.

Cushing Disease

164

Excess Aldosterone

Hyperaldosteronism

165

Presents as hypertension due to sodium retention, hypkalemia,a nd metabolic alkalosis

Hyperaldosteronism

166

Aldosterone Secreting Adrenal Adenoma

Conn Syndrome

167

Most commonly due to sporadic adrenal hyperplasia. But also can be caused by adrenal adenoma (aldosteronoma) and adrenal carcinoma

Primary Hyperaldosteronism

168

Characterized by high aldosterone and low renin

Primary Hyperaldosteronism

169

Seen in activation of RAS due to CHF or renovascular hypertension

Secondary Hyperaldosteronism

170

Characterized by high aldosterone and high renin

Secondary Hyperaldosteronism

171

Excess specific adrenal hormones with bilateral adrenal hyperplasia

Congenital Adrenal Hyperplasia

172

Deficiency in cortisol and mineralcorticoids with an increase in sex hormones.

21-hydroxylase Congenital Adrenal Hyperplasia

173

Presents with hypotension and hyperkalemia and clitoral enlargement or precocious puberty

21-hydroxylase Congenital Adrenal Hyperplasia

174

Presents with increased mineralocorticoids and decreased cortisol and decreased sex hormones

17-alpha-hydroxylase Congenital Adrenal Hyperplasia

175

Presents with hypertension and Hypokalemia

17-alpha-hydroxylase Congenital Adrenal Hyperplasia

176

Presents with low-renin hypertension

11-Beta-hydroxylase Congenital Adrenal Hyperplasia

177

Presents with decreased aldosterone and cortisol, but an increase in aldosterone pre-cursors causing hypertension

11-Beta-hydroxylase Congenital Adrenal Hyperplasia

178

Functional and are components of MEN1, Carney Complex, and McCune-Albright Syndrome

Adrenocortical Adenoma

179

Functional and associated with Li Fraumeni and Beckwith-Wiedemann syndrome

Adrenocortical Carcinomas

180

Lack of adrenal hormones

Adrenal Insufficiency

181

Acute insufficiency of adrenal hormones

Waterhouse-Friderichsen Syndrome

182

Hemorrhagic necrosis of adrenal glands due to DIC in young children with N meningitidis infection (meningococcemia)

Waterhouse-Friderichsen Syndrome

183

Lack of cortisol exacerbates hypotension leading to death

Waterhouse-Friderichsen Syndrome

184

Progressive destruction of adrenal glands resulting in chronic insufficiency of adrenal hormones

Addison disease

185

Caused by autoimmune destruction most commonly in the West

Addison disease

186

Caused by TB most commonly in the developing world

Addison disease

187

Can be caused by metastatic carcinoma or infections

Addison disease

188

Clinical features: hypotension, hyponatremia, hypovolemia, hyperkalemia, weakness, and hyperpigmentation

Addison disease

189

Increased ACTH and MSH

Addison disease

190

Most common tumor adrenal medulla in children

Neuroblastoma

191

Originates from neural crest cells

Neuroblastoma

192

Presents as abdominal distention with firm, irregular mass that can cross the midline.

Neuroblastoma

193

Presents with Homovanillic acid in the urine

Neuroblastoma

194

Bombesin positive

Neuroblastoma

195

Associated with overexpression of M-Myc oncogene

Neuroblastoma

196

Tumor of chromaffin cells

Pheochromocytoma

197

Clinical features due to increased catecholamines: Hypertension, headache, palpitations, tachycardia and sweating

Pheochromocytoma

198

Diagnosed by increased serum metanphrines and increased 24-hour urine metanephrines and vanillylmandelic acid.

Pheochromocytoma

199

Rule of 10%'s: 10% malignant, 10% bilateral, 10% extra-adrenal, 10% calcify, 10% kids

Pheochromocytoma

200

Most common tumor of renal medulla in adults

Pheochromocytoma

201

Phenoxybenzamine is administered perioperatively to prevent hypertensive crisis

Pheochromocytoma

202

Insulin deficiency leading to a metabolic disorder characterized by hyperglycemia

Type 1 Diabetes Mellitus

203

Due to autoimmune destruction of beta cells by T lymphocytes

Type 1 Diabetes Mellitus

204

Characterized by inflammation of islets

Type 1 Diabetes Mellitus

205

Associated with HLA-DR3 and HLA-DR4

Type 1 Diabetes Mellitus

206

Manifests in childhood

Type 1 Diabetes Mellitus

207

Signs: High serum glucose, weight loss, low muscle mass, polyphagia, polyuria, polydipsia, glycosuria

Type 1 Diabetes Mellitus

208

Risk for ketoacidosis characterized by excessive serum ketones

Type 1 Diabetes Mellitus

209

Arises with stress in which glucagon release causes increased lipolysis to increase FFA's which are turned into ketone bodies

Diabetic Ketoacidosis

210

Results in hypoglycemia, anion gap metabolic acidosis, and hyperkalemia

Diabetic Ketoacidosis

211

Presents with Kussmaul respirations, dehydration, flu-like, and fruity smelling breath.

Diabetic Ketoacidosis

212

Insulin treatment is always necessary

Type 1 Diabetes Mellitus

213

Severe glucose intolerance but high insulin sensitivity

Type 1 Diabetes Mellitus

214

End-organ insulin resistance leading to a metabolic disorder characterized by hyperglycemia

Type 2 Diabetes Mellitus

215

Most common type of diabetes

Type 2 Diabetes Mellitus

216

Arises in middle-aged obese adults (obesity decreases receptors)

Type 2 Diabetes Mellitus

217

Strong genetic pre-disposition exists

Type 2 Diabetes Mellitus

218

Insulin levels are increased early, but later due to beta-islet cell exhaustion, will be decreased

Type 2 Diabetes Mellitus

219

Amyloid (amylin) deposition in islets.

Type 2 Diabetes Mellitus

220

Low glucose intolerance and low insulin sensitivity

Type 2 Diabetes Mellitus

221

Risk for hyperosmolar non-ketotic coma (super high glucose)

Type 2 Diabetes Mellitus

222

Autosomal dominant syndrome characterized by mild hypglycemia and hyposecretion of insulin bu no loss of beta cells

Maturity-Onset Diabetes Mellitus of the Young

223

Insulitis marked by lymphocytic infiltration

Type 1 Diabetes Mellitus

224

Nonenzymatic glycosylation of large vessel vascular basement membranes leads to atherosclerosis

Diabetes

225

Nonenzymatic glycosylation of small vessel vascular basement membranes leads to hyaline arteriolosclerosis

Diabetes

226

Preferential involvement of efferent arterioles leads to glomerular hyperfiltration injury that progresses to nephrotic syndrome (Kimmelstiel-Wilson nodules)

Diabetes

227

Cataract formation, peripheral neuropathy, xanthomas, and abscesses are common

Diabetes

228

Component of MEN1 along with parathyroid hyperplasia and pituitary adenomas

Islet Cell Tumor

229

Present as episodic hypoglycemia with mental status changes that are relieved by administration of glucose (Whipple triad)

Insulinoma

230

Diagnosed by decreased serum glucose levels, increased insulin, and increased C-peptide

Insulinoma

231

Present as treatment resistant peptic ulcers that extend to duodenum and jejunum.

Gastrinoma

232

Known as Zollinger-Ellison syndrome

Gastrinoma

233

Known as an alpha cell tumor

Glucagonoma

234

Results in secondary diabetes mellitus

Glucagonoma

235

Causes skin lesion called necrolytic migratory erythema

Glucagonoma

236

Endocrine tumor marked by secretion of vasoactiv eintestinal peptide

VIPoma

237

Associated with "Watery diarrhea, Hypokalemia, and Achlorhydria (WDHA)"

VIPoma

238

Also known Verner-Morrison syndrome or pancreatic cholera

VIPoma

239

Also known as Wermer Syndrome

Multiple Endocrine Neoplasia 1

240

Hyperplasias or tumors of pituitary, parathyroid, or pancreatic islets (3P's)

Multiple Endocrine Neoplasia 1

241

Pancreatic portion presents as Zollinger-Ellison, hyperinsulinism, or pancreatic cholera

Multiple Endocrine Neoplasia 1

242

Linked to mutations in MEN I gene

Multiple Endocrine Neoplasia 1

243

Commonly presents with kidney stones and stomach ulcers

Multiple Endocrine Neoplasia 1

244

Known as Sipple Syndrome

Multiple Endocrine Neoplasia 2A

245

Presents with Parathyroid hyperplasia and Pheochromocytoma (2P's) Also includes medullary thyroid carcinoma

Multiple Endocrine Neoplasia 2A

246

Linked to mutations in ret oncogene

Multiple Endocrine Neoplasia 2A

247

Pheochromocytoma (1P) and also medullary thyroid carcinoma and mucosal neuromas.

Multiple Endocrine Neoplasia 2B

248

Linked to mutations in ret oncogene

Multiple Endocrine Neoplasia 2B