Neoplasms/Tumors

Cystic hygroma

-cavernous lymphangioma
-Occurs most often in neck or axilla

GLomangioma (glomus tumor)

-small, purple, painful subungual nodule on finger or toe

Hemangiosarcoma/Angiosarcoma

-Rare vascular tumor
-Occurs in skin, MSK, breast, liver
-Assoc. with exposures to arsenic, Thorotrast
-Polyvinyl chloride assoc. with hemangiosarcoma of the liver

Myoxoma

-Most common primary cardiac tumor
-90% occur in atria (LA usually)
-Described as "ball-valve" obstruction in the LA
-Associated with multiple syncopal episodes

Rhabdomyoma

-Most frequent primary cardiac tumor in kids
-Association with tuberous sclerosis

Retinoblastoma

-malignant retinal tumor in kids
-homozyg. deletion of Rb gene
-Prototypical "two-hit" hypothesis

CNS tumors in general

-Most are intracranial (SC less common)
-In adults, maj. are supretentor.
-In kids, infratentorial
-Primary malignant tumors rarely metastasize
-Metastatic brain tumors more frequent than primary

Glioblastoma Multiforme

-Most common primary brain tum
-Peak in late middle-age
-Marked anaplasia and pleomorphism; areas of necrosis/hem surrounded by "pseudopalisade" of tumor cells
-Most often in cerebrum
-Prognosis bad. Death in 1 year

Oligodendroglioma

-Slow-growing tumor of middle age
-Fried egg closely packed cells
-Cerebral hemispheres
-Tumor divided into groups of cells by capillary strands
-Foci of calcification

Ependymoma

-Usually in 4th ventricle
-Peak in kids/teens
-Tubules/rosettes

Meningioma

-2nd most common primary BT
-Benign, slowly growing
-Usually after 30, more women
-Originates in arachnoid cells of meninges - external to brain; can be removed
-Most common location: convexities of hemispheres and parasagittal region

Medulloblastoma

-common childhood neoplasm
-Highly malignant tumor of cerebellum
-sheets of closely packed cells arranged in rosette

Neuroblastoma

-Much less common than peripheral neuroblastoma
-Amplification of N-myc gene

Hemangioblastoma

-Most frequently in cerebellum
-may be part of von H-L
-Sometimes produce EPO

Neurilemmoma (Schwannoma)

-Benign, slowly growing
-Can be assoc. with acoustic n. or in posterior nerve roots and peripheral nerves.

Squamous cell carcinoma (Bronchogenic)

-Centrally located
-Hilar mass with cavitation
-Linked to smoking!
-may have PTH-rp and hypercalcemia
-Keratin pearls and intercellular bridges
*Squamous, Sentral, Smoking

Small cell (oat cell) carcinoma

Undifferentiated tumor
-Most aggressive lung CA
-Unlikely to be cured by surgery, usu. metastatic at dx
-assoc. with ectopic production of ACTH or ADH; Lambert-Eaton
-Linked to smoking!
-Small dark blue cells on histo

Large cell carcinoma (lung)

-Peripherally located
-Undifferentiated tumor
-May have features of squamous cell or adenocarcinoma

Bronchial adenocarcinoma

-Peripheral
-Develops in site of prior pulmonary inflamm. or injury
-Most common lung CA in nonsmokers and women
-Clara cells

Pancoast tumor

-Carcinoma that occurs in apex of lung and may affect cervical sympathetic plexus (can cause Horner's syndrome)

Nasopharyngeal carcinoma

-Most common in SE Asia, E Africa
-Cause is EBV

Laryngeal papilloma

-Benign; located on vocal cords
-In adults, usu single and may become malignant
-In kids, multiple lesions caused by HPV. Often recur after resection

Squamous Cell Crna of Larynx

-Most common malignant tumor of the larynx
-Usually men over 40
-Often assoc. with combo of Etohism and smoking
-Initially presents as hoarseness
-Glottic is most common and best prognosis

Pleomorphic adenoma

-most common salivary gland tumor
-mix of epithelial and mesenchyme-like elements
-women b/w 20-40
-usu benign and frequently recurs
-Rarely becomes malignant
-Most often parotid gland
-Difficult to resect d/t proximity to facial nerve.

Carcinoma of the esophagus

-In US, half adeno half squamous
-squamous more worldwide
-Adenocarcinoma linked with Barrett's
-SCC upper/middle thirds
-Adeno in lower 1/3

Hepatic adenoma

-related to use of OCPs
-when adenoma is subscapular, may rupture, leading to severe intraperitoneal hemorrhage.

Hepatocellular carcinoma

-Most common primary malign of liver
-Almost always assoc. with cirrhosis of any kind, esp. HBV
-assoc. with aflatoxin B1 contam.
-Marked by inc. serum AFP
-Invasion of vascular channels with hematogenous dissemination.

Cholangiocarcinoma

-most frequent in Far east, where assoc. with liver fluke (clonorchis sinensis)
-Early invasion of vasculature
-Not assoc. with HBV or cirrhosis
-Can be assoc. with Thorotrast

Pancreatic carcinoma

-Common tumor (adeno usually)
-Smokers
-Usu in head of pancreas, causing obstructive jaundice
-distended, palpable gallbladder
-Abd pain radiating to back, weight loss, anorexia, migratory thrombophlebitis (Trousseau's sign)
-Usually death within a year.

Carcinoid Tumor

-Tumor of endocrine cells
-make up 50% of sm int. tumors
-"Dense core bodies" on EM
-Produce 5HT and carcinoid syndrome
-Classic sx: wheezing, R heart failure, murmurs, diarrhea, flushing

Angiomyolipoma

-Benign tumor of kidney
-Hamartoma of fat, smooth muscle and BVs
-Often assoc with tuberous sclerosis

Renal Cell Carcinoma

-Men, 50-70; smokers
-May be assoc. with von HL disease or chromo 3 deletion
-Often invades renal veins/IVC
-Early hematogenous spread
-Polygonal clear cells
-Triad of flank pain, palpable mass, hematuria
-Fever, polycythemia, Ectopic hormone production as well as paraneoplastic PTH

Wilm's tumor

-most common malignancy of early childhood (peak 2-4)
-Large palpable flank mass
-WAGR
-Wt-1, 2 genes on chromo 11
-Can be part of Beckwith-Wiedeman syndrome

Transitional cell carcinoma

-In renal pelvis, assoc. with phenacetin abuse
-Likely to recur after removal
-Presents with hematuria
-Spreads by local extension
-Industrial exposure, smoking, long-term cyclophosphamide

Squamous cell carcinoma of urinary tract

-minority of malignancies
-May result from chronic inflammation d/t chronic bacterial infection or Schistosoma
-May be associated with renal calculi

Papillary carcinoma of thyroid

-Most common thyroid CA
-Orphan Annie nuclei, psammoma bodies
-Good prognosis
-May be related to prior head/neck radiation
-Sometimes assoc with changes in chromo 10; ret-PTC gene

Follicular carcinoma

-Relatively uniform follicles
-Poorer prognosis than papillary

Medullary carcinoma of thyroid

-from parafollicular C cells; produces calcitonin
-Sheets of tumor cells in amyloid-containing stroma
-May be assoc. with MEN IIa/IIb

Undifferentiated/anaplastic thyroid carcinoma

-Tends to occur in elderly patients
-Poor prognosis

Pheochromocytoma

-From chromaffin cells of adrenal medulla
-Usually benign (90%)
-Urinary excretion of catecholamine metabolites is characteristic (VMA)
-Can cause hyperglycemia
-May be part of MEN IIa/b or neurofibromatosis or vHL

Neuroblastoma

-Highly malig. catechol-producing tumor of childhood
-HTN (less than with pheo), abdominal mass (usu in adrenal medulla)
-HVA in urine
-N-myc oncogene

Osteoma

-Assoc. with FAP
-Skull, facial bones -- paranasal sinuses
-new mature bones grows on another piece of bone
-males

Osteoid osteoma

-interlacing trabeculae of wovn bone surrounded y osteoblasts
-< 2 cm found in prox tibia and femur
Men under 25

Osteoblastoma

Same as osteoid osteoma but found in vertebrae

Enchondroma

Benign cartilaginous tumor found in intramedullary bone
Usually hands/feet

Giant cell tumor

-Multinucleated giant cells and fibrous stroma
-Epiphyses of long bones (often about the knee)
-soap bubble appearance
-locally aggressive
-women age 20-40

Osteosarcoma

-osteoid and bone-producing tum.
-metaphyses of long bones (knee region)
-men 10-20
-Codman's triangle or sunburst pattern from elevation of perios.
-Poor progn.

Ewing's Sarcoma

-Anaplastic small blue cell malignant tumor
-Boys < 15
-Aggressive with early mets, responds to chemo
-Onion-skin appearance in bone
Diaphysis of log bones, pelvis, scapula and ribs
-11;22 translocation

Chondrosarcoma

-Malignant cartilaginous tumor
-Men aged 30-60
-Pelvis, spine, scapula, humerus, tibia, or femur
-maybe of primary origin or from osteochondroma
Expansile glistening mass within the medullary cavity