The Endocrine System Flashcards
Main histologic feature of pituitary adenomas.
Monomorphic cells without a significant reticulin network
Most common pituitary adenomas.
Prolactin cell adenoma; followed by Somatotroph (GH) cell adenoma
The main pathology of Cushing disease (not syndrome).
Corticotroph cell adenoma
Associated with mass effects and hypopituitarism (secondary to destruction of the normal pituitary parenchyma).
Nonfunctioning pituitary adenomas
The only definitive criterion for diagnosis of pituitary carcinoma.
Metastases
Atypical adenomas with metastases; usually functional, with ACTH as the most common hormone produced, followed by prolactin.
Pituitary carcinoma
Posterior pituitary syndromes.
Diabetes insipidus and SIADH
The origin of this tumor is from vestigial remnants of Rathke pouch; with bimodal age incidence (5-15 years; 65 years); has adamantinomatous, and papillary types.
Craniopharyngioma
Most common cause of primary hyperthyroidism.
Diffuse toxic hyperplasia (Graves disease) (85%)
Most common cause of congenital hypothyroidism worldwide.
Iodine deficiency
Most common cause of hypothyroidism in iodine-sufficient areas.
Hashimoto thyroiditis
Hypothyroidism in infancy and childhood, impaired development of CNS (Mental retardation) and skeletal system (short stature), coarse facial features, protruding tongue, and umbilical hernia.
Cretinism
Hypothyroidism in late childhood and adults; and slowing of physical and mental activity; overweight, hypercholesterolemia, nonpitting edema, coarse facial features, macroglossia and deepening of voice; accumulation of matrix substances, such as glycosaminoglycans and hyaluronic acid, in skin, subcutaneous tissue, and a number of visceral sites.
Myexedema
Histologically characterized by dense lymphocytic infiltrate with germinal centers; follicles are atrophic with Hurthle cell change (cells with eosinophilic granular cytoplasm); associated with antibodies against thyroglobulin and thyroid peroxidase; Type IV hypersensitivity with Type II component.
Hashimoto thyroiditis
Granulomatous inflammation of the thyroid and viral infections; clinically presents as painful thyroiditis.
Subacute Granulomatous (De Quervain) thyroiditis
Lymphocytic inflammation of the thyroid (morphologically similar to Hashimoto but without Hurthle cell change, fibrosis); commonly seen in postpartum patients; clinically presents as painless thyroiditis.
Subacute Lymphocytic thyroiditis
Characterized by extensive fibrosis of the thyroid gland; associated with primary retroperitoneal fibrosis (Ormond disease) and autommune IgG4-related disease; pathology is unknown, but postulated to be of autoimmune type.
Reidel thyroiditis
Triad of Graves disease.
Thyrotoxicosis, ophthalmopathy and dermopathy
Main pathology of Graves disease.
Presence of thyroid stimulating immunoglobulin (TSIs); binding to TSH receptors causes activation of follicular cell function
Histologic features of Graves disease.
Diffuse hypertrophy and hyperplasia of the follicles with scalloped colloid (moth-eaten) (because of active reabsorption of follicular cells)
Usually hypothyroid (and compensatory TSH increase leads to proliferation of the gland)
Diffuse and multinodular goiter
Goiter that has 2 phases: 1. Hyperplastic phase (due to TSH influence), and 2. Colloid phase (involution of gland due to sufficient iodine intake or increased thyroid hormone demand)
Colloid goiter
Repeated hyperplastic and colloid phases results in irregular involvement of the thyroid gland, leading to a condition known as:
Multinodular goiter
Multinodular toxic goiter characterized by development of autonomously functioning nodules; presents with hyperthyroidism not associated with ophthalmopathy and dermopathy.
Plummer syndrome
Clinical characteristics favoring malignancy in thyroid nodules.
Solitary, young, male, history of radiation therapy, “cold” nodules
Normal looking follicular cells surrounded by an intact capsule; can also be composed of follicular cells that underwent Hurthle cell change (Hurthle cell adenoma); solitary; Note: adenomatous nodule, which is a part of multinodular goiter, is the more appropriate diagnosis for multiple nodules showing adenomatous change.
Follicular adenoma
Main distinguishing feature of follicular carcinoma from follicular adenoma.
Capsular and vascular invasion
Most common form of thyroid cancer; histologically characterized by papillary architecture with dense fibrovascular cores; nucleus has finely dispersed chromatin (ground-glass or Orphan Annie nuclei); with Psammoma bodies; spreads by lymphatic route (vs. follicular carcinoma (hematogenous)).
Papillary carcinoma
Carcinoma arising from parafollicular C cells (secretes calcitonin); histologically characterized by amyloid deposits; known for its multicentricity and association with MEN2 syndromes.
Medullary Thyroid Cancer (MTC)
Highly lethal thyroid cancer (mortality rate of 100% in 6 months) with a highly pleomorphic morphology; clinically presents with rapidly enlarging thyroid mass; immunostaining for thyroglobulin is negative.
Undifferentiated carcinoma (Anaplastic carcinoma)
Most common cause of primary hyperparathyroidism; almost always confined to one gland (compared to hyperplasia that involves multiple glands); may sometimes show atypia.
Parathyroid Adenoma (85-95%)
Definitive criteria for diagnosis of parathyroid carcinoma.
Metastases and local invasion
Most common cause of secondary hyperparathyroidism.
Renal failure
Most common cause of hypoparathyroidism.
Iatrogenic
Absolute deficiency of insulin secondary of beta cell destruction by an autoimmune process; accounts for only 10% of cases of DM; Type IV hypersensitivity with Type II component; histologically characterized by mononuclear infiltration of the islets with reduction of islet size and number (insulitis).
Type I DM
Peripheral resistance to insulin and relative insulin deficiency is the main pathology; most common form of DM; histologically characterized by amyloid replacement of islets.
Type 2 DM
The hallmark of diabetic macrovascular disease, which explains its association with coronary artery disease and cerebrovascular disease.
Accelerated atherosclerosis
Histologic characteristics of diabetic microangiopathy.
Diffuse thickening of the basement membrane, with more leaky capillaries
Nodular glomerulosclerosis (Kimmelsteil-Wilson lesion); Hyaline arteriolosclerosis; and necrotizing papillitis from pyelonephritis are lesions encountered in:
Diabetic nephropathy
Retinal hemorrhages, microaneurysms, venous dilations, edema, exudates and thickening of retinal capillaries are lesions encountered in what form of diabetic retinopathy?
Nonproliferative (background)
Neovascularization and fibrosis are lesions encountered in what form of diabetic retinopathy?
Proliferative
Most common PanNET; associated with Whipple’s triad: 1. Hypoglycemia <50 mg/dL; 2. Neuroglycopenic symptoms; and 3. Relief with feeding or parenteral administration of glucose; Biologically favorable behavior; characterized by deposition of amyloid.
Insulinoma
Associated with Zollinger-Ellison syndrome: 1. Pancreatic islet cell tumor; 2. Hypersecretion of gastric acid; and 3. Peptic ulceration; also associated with MEN-1; biologically aggressive behavior.
Gastrinoma
Most common cause of Cushing syndrome (hypercortisolism).
Exogenous administration of steroids
Most common cause of endogenous hypercortisolism.
ACTH-secreting pituitary adenoma (Cushing disease)
Morphologic change in adrenal cortex of patients with ACTH-dependent Cushing syndrome.
Diffuse hyperplasia
Morphologic change in adrenal cortex of patients with ACTH-independent Cushing syndrome. (Cortisol-secreting adrenal adenoma).
Cortical atrophy
Most common cause of primary hyperaldosteronism.
Bilateral idiopathic hyperplasia (60%)
The most common enzymatic defect in congenital adrenal hyperplasia.
21-hydroxylase deficiency
Most common cause of primary adrenal insufficiency (Addison disease).
Autoimmune adrenalitis (60-70%)
The only criterion to distinguish adrenocortical carcinoma from an adrenocortical adenoma.
Metastases (adenomas tend to have atypia as well)
Tumor of chromaffin cells; histologically composed of polygonal to spindle-shaped cells with finely granular cytoplasm, arranged in nests called zellballen; only criteria for malignancy is metastases; 10% extraadrenal; 10% bilateral; 10% biologically malignant; 10% are not associated with hypertension.
Pheochromocytoma
MEN with 3Ps: Parathyroid (Primary hyperparathyroidism either secondary to hyperplasia or adenoma), Pancreas (either Gastrinoma or Insulinoma), and Pituitary (most common is Prolactinoma, followed by Somatotroph adenoma); Autosomal dominant; associated with mutations in MEN1 gene on 11q13.
MEN 1 (Wermer syndrome)
MEN with MTC, Pheochromocytoma and primary hyperparathyroidism; Autosomal dominant; associated with gain of function mutations on RET proto-oncogene in 10q11.2
MEN 2A (Sipple syndrome)
MEN with MTC, Pheochromocytoma, ganglioneuromas and Marfanoid habitus; associated with different mutations on RET proto-oncogene.
MEN 2B
