ENDOCRINE Flashcards
What is the origin and consequence of a craniopharyngioma?
Origin: Above sella turcica from remnants of Rathke Puoch
Consequence: cystic mass with compression of adjacent tissues causing destruction of the posterior lobe of the pituitary
Imaging: mass replaces midline structure sin the region of the hypothalamus
SIADH is associated with what carcinomas?
small cell carcinoma of lung prostate carcinoma GI carcinoma pancreatic carcinoma thymoma lymphoma Hodgkin disease
What are the major clinical manifestations of Sheehan syndrome?
PALLOR (decreased MSH) HYPOTHYROIDISM (decreased TSH) OVARIAN FAILURE (decreased FSH and LH) FAILURE OF LACTATION (decreased Prolactin) ADRENAL INSUFFICIENCY
Acromegaly symptoms
thickened skin Facial Changes Goiter Barrel Chest abnormal glucose tolerance male sexual dysfunction menstrual disorders in women degenerative arthritis peripheral neuropathy
What are the symptoms of functional lactotrope adenoma?
Amenorrhea
Galactorrhea
Infertility
What is most commonly used goitotrogenic drug?
Lithium (used in the management of manic-depressive states)
What is Endemic goiter?
Goitrous hypothyroidism of dietary iodine deficiency in locales with a high prevalence of the disease
somatotrope adenoma
growht hormone-secreting tumor of the pituitary
What are some pathological processes that result from a somatotrope adenoma?
Acromegaly (many with neurological and msk symptoms/ some with HTN) Gigantism (in children) Menstrual irregularities in women Diabetes Mellitus Headache Optic Chiasm compression
What is used to treat lactotroph microadenomas
Dopamine agonists
What is Adrenogenital syndrome? what is it’s cause?
Virilization of external genitalia in female infants (pseudohermaphroditism)
Appearance: hypertrophic clitoris and parital fusion of labioscrotal folds in a genetic female
caused by congenital deficiency of 21-hydroxylase
What hormones increase in 21 Hydroxylase deficiency?
Adrenal androgens and progesterone
What congenital anomalies are seen in DiGeorge Syndrome?
Failure to develop 3rd and 4th Branchial pouches result int he following
Parathyroid Agenesis/Hypoplasia
Thymus Agenesis/Hypoplasia
Congenital Heart Defects
Dysmorphic faces
What is characteristic phenotype of Albright hereditary Osteodystrophy?
Short stature obesity mental retardation subcutaneous calcification congenital anomalies of the bone
What genetic abnormality and consequent pathology is seen in Albright Hereditary Osteodystophy?
Mutations in a gene (GNAS1) whose product, the protein, Gs, couples hormone receptors on the stimulation of adenylyl cyclase.
Consequently, in the renal tubular epithelium, the production of cAMP in response to PTH is impaired, and inadequate resorption of calcium from the glomerular filtrate. This causes Psuedohypoparathyroidism
What mental changes are often observed in cases of hyperparathyroidism?
Depression
Emotional Liability
Poor Mentation
Memory Defects
Describe a parathyroid adenoma microscopically and macroscopically/on gross exam.
Micro: Sheets of neoplastic CHIEF CELLS in a rich capillary network. A rim of normal parathyroid tissue is usually evident outside the tumor capsule and distinguises adenoma from parathyroid hyperplasia
Macro: Circumscribed, reddish brown, solitary mass, measuring 1 to 3 cm in diameter
What are common causes of secondary hyperparathyroidism?
Most common cause: Renal failure
- Diabetic glomerulosclerosis is a major cause of renal Insufficiency
Other causes: Vitamin D Deficiency
Intestinal malabsorption
Fanconi Syndrome
Renal Tubular Acidosis
What diseases are seen with MEN-2A affected patients? What is cause?
C-cell derived medullary thyroid carcinoma
Chromaffin cell-derived pheochromocytoma
Hirschspring Dieases (Congenital Megacolon)
Nueroal Crest Tumors (eg. glioma)
Cause: Mutations of the RET protooncogene, a transmembrane receptor of the tyrosine kinase family
What diseases are seen with MEN-1 affected patients? What is cause?
Pituitary Adenoma
Parathyroid Hyperplasia or Adenoma
Islet Cell Tumors of the Pancreas (Insulinoma and gastrinoma)
Cause: Mutation of the MEN 1 tumor suppressor gene
What is the most common cause of neonatal hypothroidism today?
Thyroid Agenesis
What are symptoms of cretinism in a newborn?
Early Weeks: Umbilical Herniation and Large Abdomen Sluggish Low body temperature Refractory Anemia
Later Weeks:
Mental Retardation Stunted Growth
Charactersitic facies
Describe Osteitis Fibrosa Cystica. What are the symptoms?
Severe bone deformities
“Brown Tumor” Formation(of Hyperparathyroidism)
Symptoms: Bone Pain Bone Cysts Pathologic Fractures Localized Bone Sewllings (brown tumors)
What are dominant clinical manifestations of hypothyroidism?
In both sexes: Muscular Weakness Peripheral Edema "Myxedema Madness" Pallor Enlarged Tongue
In females:
Ovulatory Failure
Progesterone Deficiency
Irregular and Excessive Menstrual Bleeding
In males:
Erectile Dysfunction
Oligospermia
Nongoitrous Hypothyroidsm amy result from what pathology?
Antibodies that block TSH itself or the TSH receptor without activating the thyroid
What are the complications seen in Toxic Goiter
Usually cardiac complications, including atrial fibrillation and CHF in older patients (usually over 50 years of age)
What is HASHIMOTO Thyroiditis?
Chronic Autoimmune Thyroiditis
characterized by circulating antibodies to thyroid antigens and features of cell-mediated immunity to thyroid tissue commonly arising in the 40s/50s - mostly women
Describe a Thryoid gland of a patient suffering from Hashimoto Thyroiditis
An enlarged, firm gland weighing from 60-200 g
The cut surface is pale tan and fleshy and exhibits a vaugely nodular pattern
Micro: .1. a conspicuous infiltrate of lymphocytes and plasma cells
2. destruction and atrophy of the follicles
3. oxyphilic metaplasia of the follicular epithelial cells (Hurthle or Askanazy cells)
inflammatory infiltraes are cocallyarranged in lymphoid follicles, often with germinal centers
Reidel thyroiditis features
Fibrosis (retroperitoneum, mediastinum, orbit)
Involves soft tissues of the neck
“Woody” stony hard thyroid
Dequervain thyroidits features
Hyperplastic and vascular thyroid
Multinodular Goiter features
a nodular gland without inflammation
A nontoxic goiter microscopically features
Hypertophy and Hyperplasia
Variation in the size of follicles
Fibrosis
Evidence of Old Hemorrhage
Graves Disease features what thyroid pathology?
Hyperplastic and Vascular Thyroid
Most common thyroid tumor in young women is..
Papillary carcinoma
tends to metastasize to regional lymph nodes but only rarely distantly
What characterized papillary carcinoma
Branching papillae lined by epithelial cells with clear (ground glass or Orphan Annie_ nuclei and fibrovascular cores.
Sometimes calcospherites (psammoma body) is evident
What characterizes Subacute Thyroiditis?
Subacute Thyroiditis (deQuervain, granulomatous, or giant-cell thyroidits) is caused by a viral infection (ex. Influenza virus, adenovirus, echovirus, coxsackievirus)
It is self-limiting
Characterized by GRANULOMATOUS INFLAMMATION. Acute inflammation often with microabscesses
Thyroid is enlarged to 40-60g and th e cut surface is firm and pale
What is the cause of Graves Disease? What is the microscopic appearance?
IgG antibodies bind the TSH receptor expressed on the plasma membrane of thyrocytes
Follicles are lined by hyperplastic, tall columnar cells. Colloid is pink and scalloped at the periphery adjacent to the follicular cells
What pathology and clinical symptoms are seen in Medullary Thyroid Carcinoma?
MTC has polygonal, granular cells, spearated by a distincly vascular stroma.
MTC extends by direct invasion into soft tissues and metastasizes to the regional lymph nodes and distant organs.
Patients with MTC often suffer a number of symptoms related to endocrine secretion, including carcinoid syndrome (serotonin) and Cushing Syndrome (ACTH)
How does a Neuroblastoma appear on histologic examination?
Pseudorosettesfeaturing tumor cells clustered radially around small vessels
Tumor cells may secrete catecholamines (simlar to mature descendants sin the adrenal medulla). These compounds are metabolized and excreted as urinary vanillylmandelic acid in the urine
What does Dexamethasone accomplish in a Dexamethasone suppression test?
Dexamethasone suppresses pituitary secretion of corticotropin and hense, hypercotisolism whereas it is without effect in cases of adarenal hyperplasia or functional adrenal tumors. Thus, the demamethasone suppression test distinguishesACTH-dependent and ACTH-independent forms o fCushing Syndrome.
What are clinical manifestations of untreated Addison Disease
Weakness Weight Loss GI symptoms Hypotension Electrolyte Disturbances Hyperpigmentation
(90% of the adrenal gland must be destroyed for these symptoms to appear)
What are common causes of Addison Disease?
Most Common: Autoimmune destruction of adrenal gland
Other Causes: TB, Metastatic Carcinoma, Amyloidosis, Sarcoidosis, Adrenoleukodystrophy, and Congenital Adrenal Hypoplasia
What findings often are seen in CONN syndrome?
Muscle weakness and fatigue are produced by the effect of potassium depletion oon skeletal muscle.
Most patients with primary aldosteronism are diagnnoesd after the detection of asymptomatic DIASTOLIC HTN.
Syndrome can be associated with low or nomral renin levels
What are key clinical manifestations of Pheochormocytoma?
- Asymptomatic HTN discovered on a routine PE
- symptomatic HTN resitnat to antiHTNtherapy
- Malignant HTN (encephalopathy, papilledema, proteinuria)
- MI or Aortic Dissection
- Convulsions
