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Pathology 2 - Block 3 - Part 3

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davidwurbel7's version from 2016-08-01 00:51

Neoplastic Disorders of the Bone

Metastasis
Question Answer
The most common malignancy of bone is thisMetastasis to the Bone
Nature of metastasis of breast cancer to the boneOsteolytic
Nature of metastasis of prostatic cancer to the bone Osteoblastic
Osteoblastic metastasis will show this marker elevatedAlkaline Phosphatase
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Bone Forming Tumors
Question Answer
Benign tumor of bone. Most commonly arises on the surface of facial bones. Associated with Gardner syndromeOsteoma
Benign tumor of osteoblasts surrounded by a rim of reactive bone. Occurs in young adults < 25 years of age (more common in males). Arises in cortex of long bones. Presents as bone pain that resolves with aspirin. Imaging reveals a bony mass (< 2 cm) with a radiolucent coreOsteoid Osteoma
Bone pain tend to occur at night and the pain is relieved on administration of aspirinOsteoid Osteoma
Malignant proliferation of osteoblasts. Peak incidence is seen in teenagers; less commonly seen in the elderly. Arises in the metaphysis of long bones, usually the distal femur or proximal tibiaOsteosarcoma
Genetic mutation that can predispose a person to developing osteosarcomaRb Gene Mutation
Presents as a pathologic fracture or bone pain with swelling. Imaging reveals a destructive mass with a 'sunburst ' appearance and lifting of the periosteum. Biopsy reveals pleomorphic cells that produce osteoidOsteosarcoma
Periosteum, which has been lifted, has laid down a proximal triangular shell of reactive boneCodman triangle
Osteoid that is calcified in lines with maybe a large area of osteoid calcifiedSunburst Appearance
Tumor comprised of multinucleated giant cells and stromal cells. Occurs in young adults (more common in females. Arises in the epiphysis of long bones, usually the distal femur or proximal tibia(region of the knee) and distal radius. 'Soap-bubble' appearance on x-ray. Locally aggressive tumor; may recurGiant Cell Tumor
The cortex is thin, but not destroyed. The tumor is located within the epiphysis. Tumor destroys bone, and hence, it appears that the tumor is cysticGiant Cell Tumor
Malignant proliferation of poorly-differentiated cells derived from neuroectoderm. Arises in the diaphysis of long bones; usually in male children (< 15 years of age). 'Onion-skin' appearance on x-ray. Biopsy reveals small, round blue cells that resemble lymphocytes. (11;22) translocation is characteristic. Often presents with fever, pain, swelling (similar to osteomyelitis) and metastasis; responsive to chemotherapyEwing Sarcoma
Ewing sarcoma is also called thisPrimitive Neuro-Ectodermal Tumor
Retinoblastoma, Medulloblastoma, Ewing Sarcoma, Acute lymphoblastic leukemia, Neuroblastoma and Rhabdomyosarcoma are examples of a group of tumors call thisSmall Round Blue Cell Tumors
Reactive bone formation around a tumor laid down in layersOnion Skin Appearance
The reactive bone formation which causes the onion skin appearance in bones is to thisCompression Stress of Osteoblasts
Arrangement of small round blue cells around a small empty spaceHomer Wright Rosettes
Ewing sarcoma cells contain abundant glycogen, as seen by the reddish granular cytoplasmic stained by thisPAS Stain
Benign tumor of cartilage. Usually arises in the marrow cavity of small bones of the hands and feet. Can be single or multipleChondroma
Multiple chondromas are called thisOllier’s disease
Tumor of bone with an overlying cartilage cap. Most common benign tumor of bone. Arises from a lateral projection of the growth plate (metaphvsis); bone is continuous with the marrow space. Overlying cartilage can transform (rarely) to chondrosarcomaOsteochrondroma
Malignant cartilage-forming tumor that arises from diaphysis of the pelvis or central skeletonChondrosarcoma
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Question Answer
5-15 yrs. Diaphysis Femur , pelvis, tibiaEwing's Sarcoma
10-20 yrs. Metaphysis. Femur, tibia, humerusOsteosarcoma
20-40 yrs. Epiphysis. Femur, tibia radiusGiant cell tumor
40-60 yrs. Diaphysis. Pelvis, ribs, shoulder bonesChondrosarcoma
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Question Answer
Benign non-neoplastic process of bones. Characterized by replacement of marrow by fibrous tissue. Presence of poorly formed woven bone arranged in Chinese letter pattern. Primarily targets ribs, femur, or cranial bones of children and young adultsFibrous Dysplasia
Fibrous dysplasia involving a single bone and is usually asymptomaticMonostotic Fibrous Dysplasia
Fibrous dysplasia involving multiple bones. Often symptomatic. Deformities and fractures of craniofacial bones. Shepherd Crook deformity of proximal femurPolystotic Fibrous Dysplasia
Polystotic Fibrous dysplasia with endocrine abnormalitiesMcCune-Albright Syndrome
Features include - Polystotic Fibrous dysplasia. Precocious sexual development. Irregular skin pigmentation (café-au-lait spots) (akin to “coast of Maine”)McCune-Albright Syndrome
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Pituitary Disorders 1

 

Question Answer
Often involves tumor (usually benign). May be due to ectopic productionToo much (hormone)
Due to enzyme deficiencies (required for synthesis). Autoimmune disease (gland destruction). Infection, hemorrhage, infarction etc.Too little (hormone)
Benign tumors (majority) and Malignant tumorsMass lesions
Due to involvement of organ that produces the hormonePrimary
Due to involvement of pituitary that produces trophic hormonesSecondary
Due to involvement of hypothalamusTertiary
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Question Answer
Benign tumor that is the most common overall pituitary adenoma. Produces prolactinProlactinoma
Clinical finding include elevated prolactin levels, Female: secondary amenorrhea, galactorrhea, and infertility. Male: Infertility and impotenceProlactinoma
Treatment for a prolactinomaBromocriptine (Dopamine analogue)
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(Insert 18)
Question Answer
Produced in the liver due to stimulation of GH. Stimulates growth of bone (linear and lateral), cartilage and soft tissueIGF-1
Clinical findings include increased lateral bone growth, Prominent jaw, Frontal bossing. Enlargement of frontal sinuses: increases hat size. Macroglossia, cardiomyopathyAcromegaly
This is diagnosed by an increased GH and IGF-1. Lack of GH suppression and HyperglycemiaAcromegaly
Treatment for this is Octreotide (somatostatin analog), GH receptor antagonist (pegvisomant) or surgeryAcromegaly
Refers to ↑↑cortisol due to ACTH secreting adenoma of pituitaryCushing Disease
Can be due to Cushing’s disease or due to ectopic production of ACTH or lesions of adrenal gland producing excess cortisolCushing Syndrome
Prolactinoma cells stain this wayAcidophilic
Somatotropic adenoma cells stain this wayAcidophilic
Corticotropic adenoma cells stain this wayBasophilic
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Pituitary Disorders 2

Question Answer
The most common cause of SIADH is due to thisSmall Cell Cancer of the Lung
The life-threatening condition due to SIADH is thisCerebral Edema
The cerebral edema caused by thisDilutional Hyponatremia
Clinical findings include Hyponatremia - Serum Na is usually below 120 mEq/L. Low serum osmolality. Mental status changes - irritability, confusion & seizures. neuronal swelling and cerebral edemaSIADH
SIADH can be treated with this drug that is an antibiotic with anti-ADH propertyDemeclocycline
The most common cause of hypopituitarism in adultsNon-functioning (null) pituitary adenomas
Ischemic necrosis of pituitary due to post-partum hemorrhage which results in the lost of function of the pituitarySheehan Syndrome
Tumor occurring in children that is a remnant of Rathke’s pouchCraniopharyngioma
A small adenoma has a blood vessel ruptures causing hemorrhage in the adenoma causing it to increase in size which will then compresses the normal pituitary causing the pituitary to become non-functionalPituitary Apoplexy
Refers to presence of an empty sella turcica that is not filled by pituitaryEmpty sella syndrome
Nonfunctioning pituitary adenoma is the most common cause of hypopituitarism in adults and is associated with this conditionMultiple Endocrine Neoplasia (MEN) I Syndrome
Clinical findings include failure to lactate, amenorrhea, pallor, hypothyroidismSheehan Syndrome
Most common cause of hypopituitarism in childrenCraniopharyngioma
Benign tumor derived from remnants of Rathke’s pouch. Located above sella turcica and extends into sella turcica and destroys the pituitary gland. Age at presentation: 5-15 yrsCraniopharyngioma
Cystic tumor filled with fluid that looks like machinery oil. Shows areas of calcification. Stratified squamous cells with keratin formationCraniopharyngioma
Clinical findings include Secondary Amenorrhea, Decreased libido. Diminished secondary sex characteristics.Adult Female Hypogonadotropinism
Clinical findings include Impotence (failure to sustain an erection). Diminished sec. sex characteristics.Adult Male Hypogonadotropinism
Clinical findings include growth and sexual maturation retardation. Delayed fusion of epiphysesChild Hypogonadotropinism
Lab workup shows Low FSH and LH and Low estradiol/ testosteroneHypogonadotropinism
No effect on growth. hypoglycemia due it being gluconeogenicAdult GH Deficiency
Pituitary dwarfism and delayed growth and sexual maturationChild GH Deficiency
Secondary hypothyroidism. Decreased serum T4 and TSHTSH Deficiency
Secondary adrenal insufficiency. Hypoglycemia (cortisol is gluconeogenic)ACTH Deficiency
Failure of lactation in post-partum patientsProlactin Deficiency
The most important deficiency to recognize in a hypopituitary patient is thisAdrenal Insufficiency
Condition that results due to deficiency of ADHCentral Diabetes Insipidus
Failure of the kidney to respond to ADHNephrogenic Diabetes Insipidus
Causes of this include Hypokalemia: vacuolar nephropathy of Collecting tubules, Drugs: Lithium, demeclocycline and Nephrocalcinosis: calcification of BM of collecting tubuleNephrogenic Diabetes Insipidus
Clinical features include polyuria, polydipsia with risk of life threatening dehydration. Hypernatremia and high serum osmolality. Low urine osmolality and specific gravityDiabetes Insipidus
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