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Neoplasia by Mind Map: Neoplasia

1. Pancreas

1.1. Pancreatic Adenomacarcinoma

1.1.1. 96% arise from ductal epithelium. Origin from the acinar cells are rare. Usually occurs in 70-80y/o. Located mostly at the head of the pancreas. Overall 5yr survival is 3%.

1.1.2. Pathogenesis: assoc w/ K-ras mutation and p16 and p53 mutations. Histology shows moderately differentiated adenocarcinoma. Well formed malignant glands and stromal fibrosis. Key feature: perineural invasion is present. Pancreatic cancer along a NERVE.

1.1.3. Patients present w/ epigastric pain accompanied by unintentional weight loss (90%). Common bile duct (CBD) obstruction leading to jaundice, Light colored stools (from absent Urobilli (UBG)), Palpable gallbladder (Courvoisier's Sign; 30%). Key Feature: Trousseau's Syndrome-Superficial migratory thrombophlebitis is assoc w/ pancreatic carcinoma. C19-9 is the gold standard marker. Virchow's Node and Sister Mary Jospeh's Node

1.1.4. Dx: w/ C sign on CT (cancer pushed against the duodenum making the "C" shape on CT. Rx: Surgery- Whipple's procedure, radiation, and chemo.

1.2. Islet Cell Tumors

1.2.1. Isulinoma Benign tumor of Beta islet cells; 80% assoc w/ MEN1 syndrome. Fasting hypoglycemia will cause mental status issues. increased serum insulin and decreased C-peptide. Rx: surgery

1.2.2. Glugagonoma malignant tumor of alpha islet cells; hyperglycemia, rash (necrolytic migratory erythema) Rx: surgery; octerotide (somatostatin analogue).

1.2.3. VIPoma (pancreatic cholera) malignant tumor w/ excessive VIP. Secretory diarrhea, achlorhydria. Hypokalemia, normal anion gap metabolic acidosis (loss of bicarb in stool). Rx: surgery; octerotide

1.2.4. Zollinger-Ellison (Gastrinoma) Syndrome malignant islet cell tumor that secretes gastrin; Assoc. with MEN 1 (20-30% of cases). Peptic ulcers, diarrhea, maldigestion of food. Dx: serum gastrin (>1000pg/mL) Rx: PPIs, surgery; octreotide

1.2.5. Somatostatinoma malignant tumor of delta islet cells; producing the inhibitory hormone somatostatin. Presents with achlorhydria, cholelithiasis and steatorrhea, and diabetes mellitus. Rx: Surgery; stretozotocin

1.2.6. General: Islet cell tumors are rare. Associated w/ 1. hyperinulism w/ hypoglycemia, 2. Zollinger-Ellison syndrome or ulcer and gstrinoma, 3. MEN syndromes. 70% of tumors are solitary adenomas, 10% are multiple adenomas, while another 10% metastasize. NOTE: malignancy is determined by the biological behavior and can't be predicted from histology

1.2.7. Histology: shows nests of malignant islet cells in the pancreas. The nest resemble the normal islets mostly; however, a large mass of tumor cells have a trabecular architecture that can be appreciated.

2. Kidney

2.1. Renal Cell Carcinoma

2.1.1. RCC accounts for 90% of all renal cancers in adults. Elderly men are at highest risk. Called hypernephroma/clear cell due the abundant clear cytoplasm. The tumors; however, grossly are yellow, grey, and white and bulge through the upper pole of the kidney. 5yr survival is 45-70% unless invasion into the renal vien (15%).

2.1.2. Histologically, a thin strip of fibrovascular connective tissue separates the normal kidney from the tumor. many polygonal cells with clear cytoplasm. The cells stain w/ PAS (w/ distase). Nuclei are round and regular. This cancer can metastasizes to the thyroid.

2.1.3. Presentation: hematuria, fever, weakness, and unintentional weight loss. Key feature: Associated w/ polycythemia. Early metastasis is common especially to lung and bone.

2.1.4. Associated w/ vonHippel Lindau Syndrome patients. (which have hemangioblastomas of the CNS and retina). 2/3 of the develop RCC due to male of the VHL gene function.

2.2. Angiomyolipoma

2.3. Wilms Tumor

2.4. Urothelial (transitional cell) carcinoma

2.5. Squamous Cell Carcinoma, Kidney

2.6. Adenocarcinoma, Kidney

3. Prostate

3.1. Adenocarcinoma, prostate

3.1.1. Usually occurs in the periphery of the prostate or in the subcapsular location. Common in the US esp among Black men. Precursor lesions are common (PIN; prostatic intraepithelial neoplasia).

3.1.2. Malignant glands are small (microacinar) and packed. All glands are lined by a single cell layer. Larger glands are hyperplasic, and nerves are prominent beneath the capsule. Perineural invasion is common. Cancer invades the NERVE.

4. Germ-cell Tumors

4.1. Seminoma

4.1.1. Male germ cell tumors peak at ages 15-43 yrs. Mixtures of two or more histologic patterns are most common. Accounts for 30% of germ cell tumors and has a 90% cure rate. Radiosensitive.

4.2. Non-Seminoma

4.2.1. Embryonal Carcinoma, Testis Testis w/ extensive hemorrhage and necrosis. Tumor cells form tubular, alveolar, papillary, and sheet like patterns. Nuclei are hyperchromatic w/ prominent nucleoli. Mitoses are also common. Pure embryonal carcinoma is very rare and most tumors show other elements such as yolk sac.

4.2.2. Teratoma, Ovary

4.2.3. Serous Cystadenoacarcinoma, Ovary

4.2.4. Yolk Sac (endodermal sinus) tumor make AFP

4.2.5. Choriocarcinoma make hCG

4.2.6. Teratoma, Testis

4.2.7. Mixed germ cell tumor make hCG and AFP

4.2.8. Sex cord stromal tumors Stromal tumor (benign) Leydig cell tumor Sertoli cell tumor

4.2.9. Lymphoma most common cause of testicular mass in males >60 y/o; often bilateral. Usually Diffuse large B-cell type.

5. Bladder

5.1. Papillary Transitional Cell Carcinoma, Urinary Bladder

6. Liver

6.1. Metastatic Prostate Cancer of the Liver

6.2. Metastatic Small Cell Lung Carcinoma of the Liver

6.3. Cavernous Hemangioma

6.4. Hepatocellular Carcinoma/ Hepatoma

7. Larynx

7.1. Squamous Cell Carcinoma, Larynx

8. Lung

8.1. Small Cell Carcinoma, Lung

8.2. Non-Small Cell Carcinoma

8.2.1. Metaplasia, Dysplasia, Squamous Cell Carcinoma In Situ

8.2.2. Ewing's Sarcoma Metastasis to Lung

8.2.3. Metastatic Osteosarcoma, Lung

8.2.4. Brochogenic Squamous Cell Carcinoma, Lung

9. Bone

9.1. Benign

9.1.1. Giant cell tumor (osteoclastoma)

9.1.2. Osteochondroma

9.2. Malignant

9.2.1. Chrondrosarcoma

9.2.2. Metastatic Osteosarcoma

9.2.3. Ewing's Sarcoma

9.2.4. Ductal Carcinoma of Breast, metastatic to Femur

10. Brain

10.1. Meningioma

10.2. Glioblastoma multiforme (grade IV astrocytoma)

10.3. Schwannoma

10.4. Oligodendroglioma

10.5. Pitutiary Adenoma

10.6. Childhood Tumors

10.6.1. Pilocytic (low-grade) astrocytoma

10.6.2. Medulloblastoma

10.6.3. Ependymoma

10.6.4. Hemangioblastoma

10.6.5. Carniopharyngioma

11. Skin

11.1. Nevus

11.2. Malignant Melanoma

11.3. Basal Cell Carcinoma

12. Heart

12.1. Rhabdomyoma

12.2. Myxoma

12.2.1. Benign mesenchymal tumor w/ gelatinous appearance and abundant ground substance.

12.2.2. Most common primary cardiac tumor in adults.

12.2.3. Usually forms a pedunculated mass in the left atrium that causes syncope due to obstruction of the mitral valve.

13. Soft Tissue

13.1. Rhabdomyosarcoma

13.2. Ewing's Sarcoma

14. Tumor Nomenclature

14.1. Definitions

14.1.1. Neoplasm: new growth that will no stop growing. Clonal proliferation of cells from one cell that went awry.

14.1.2. Metaplasia: change of one adult cell type to another adult cell type usually in response to injury.

14.1.3. Dysplasia: Disordered growth. Cells will not look as uniformed as the usual architecture of the tissue.

14.1.4. Desmoplasia: malignant cells invade and stimulate surrounding cells passing the basement membrane to secrete fibrin. It is blue in hue. I leads to fibrotic scar. (typical of adenocarcinomas).

14.1.5. Pleormorphism: variable cell/nuclear size and shape. This is an indicator of malignancy.

14.1.6. Monomorphic: cells/nuclei are the same shape and size.

14.1.7. Differentiated: the degree to which the cells within the neoplasm resemble the primary structure from wince it originated. If the cells of the neoplasm originated in the colon and the colonic features are still noted in the neoplasm then the neoplasm is considered to be "well" differentiated. However, if they neoplasm in colon was completely pleomorphic with no resemblance to tissue origin then it is poorly differentiated.

14.1.8. Undifferentiated: poorly differentiated. Cells have gone awry and it is difficult to determine their origin. Another name for undifferentiated cells is "anaplastic".

14.2. Lineage

14.2.1. Epithelium Ademona, Adenocarcinoma Papillom, Paillary Carcinoma

14.2.2. Mesenchyme Lipoma, Liposcarcoma

14.2.3. Lymphocyte Lymphoma, Leukemia

14.2.4. Melanocyte Nevus (Mole), Melanoma

15. Vascular

15.1. Hemangioma

15.2. Angiosarcoma

15.3. Kaposi Sarcoma

16. Thyroid

16.1. Follicular Adenoma, Thryoid

16.2. Papillary Carcinoma, Thyroid

16.3. Medullary Carcinoma, Thyroid

16.4. Anaplastic Carcinoma, Thyroid

17. Adrenal Medulla

17.1. Pheochromocytoma

18. GI

18.1. Colon

18.1.1. Adenomatous Polyp, Colon General Tubular- small and pedunculated; most common polyp (60%). Usually found in the sigmoid colon. Looks like a mushroom, and it has branches of glands. Villous- large and sessile more likely to be malignant. Usually found in the rectosigmod area. They secrete protein and potassium rich mucus. This can produce hypoalbuminemia and hypokalemia. Cancer risk correlated w/ size, architecture, dysplasia Tubulovillous- (20-30% of polyps) usually has a stalk, contains ademomatous and villous change that resembles small bowel vili. Microscopic The epithelial surface is focally elevated. Increased glands (adenomatous change). The crowed glands also show nuclear hyperchromicity and decreased mucin. FYI Ras gene is the most commonly observed oncogene in adenomas. Adenomas >2cm have a 40% risk for malignancy.

18.1.2. Familial Adenomatous Polyposis (FAP) AD patients develop tubular adenomas and cancer. Polyps develop // 10 and 20 y/o. Caused by inactivation of adenomatous polyposis coli (APC) suppressor gene. Presents w/ malignant transformation // 35 and 40 y/o. All patients will develop colon cancer (100%) therefore, colectomy is recommended prophylactically. Unique: associated with congenital hypertrophy of the retinal pigment epithelium.

18.1.3. Adenocarcinoma, Colon Risk Factors: Age>50y/o, smoking, obesity, heavy alcohol intake, Hereditary polyposis syndromes, HPNCC, Family cancer syndrome, first degree relatives with colon cancer. IBD (ulcerative colitis> Crohn's Disease), low fiber diet, increased saturated fats and reduced vegetable intake. Mutations in APC, K-ras, and P53 (AKP mnemonic). 80% of sporatic cancers. Most commonly found in the rectosigmoid region (50%) of cases; however is found on all other areas of the large bowel. Screen with fecal occult blood test and colonscopy. Can be found on digital rectal exam in some cases. Colonscopy is gold standard- start at age 50 if no risk factors. Every 3-5yrs if history of polyp removal. Begin at age 40 if first degree relative has polyps or colorectal cancer. TMN staging is the most important prognostic factor. Left vs. Right sided Colon Cancer Left Side: Tend to obstruct, lesions will have annular napkin ring appearance/ apple core lesion, constipation or diarrhea w/ or w/o bleeding, bright red blood (BRB) coating the stool, Uniquely Strept. Bovis endocarditis. Right Side: Tend to bleed, more polypoid in appearance, blood mixed in w/ stool, iron deficiency is more likely than in left sided cancer of the colon.

18.1.4. Gardner's Syndrome AD polyposis syndrome characterized by colon polyps and benign osteomas and desmoid tumors usually in the jaw bone.

18.1.5. Turcot's Syndrome AR polyposis syndrome characterized by colon polyps and the finding of a malignant brain tumor (astrocytoma and medulloblastoma)

18.1.6. Hereditary Nonpolyposis Colorectal Cancer (HNPCC/ Lynch Syndrome)

18.2. Stomach

18.2.1. Adenocarcinoma, Stomach is common in Japan, China, and South American, but rare in US&UK. 5yr survival 10%. Most commonly found on the lesser curvature of the antropyloric region. A variant of gastric carcinoma is Linitis Plastica (Brinton's Disease) w/ a diffuse cancer (leather bottle cancer) that has flattened rugae. Unlike most gastric cancers Linitis Plastica is NOT associated w/ H. Pylori infection; however, it is associated with an E-Cadherin mutation. Histologically, shows normal gastric epithelium w/ area of ulceration surrounding the underlying adenocarcinoma. Tumor is diffuse w/ mucin secreting from the malignant glands causing "signet ring" morphology. These cells penetrate the muscularis propria. However, the morphologic type is not prognostic.

18.2.2. Leiomyoma, Stomach

18.2.3. Primary gastric malignant lymphoma

18.2.4. Metastatic Gastric Adenocarcinoma

18.3. Small Bowel

18.3.1. Carcinoid Tumor most common small bowel malignancy. However, the small bowel is the least common site for primary malignancy in the entire GI tract. (So this tumor does not arise very often.) BRIGHT YELLOW TUMOR Neuroendocrine tumor (salt&pepper chromatin), metastatic potential correlates w/ size and depth. Foregut and Hindgut invade but don't metastasize and midgut does both. Found on the vermiform appendix in most cases (40%). Commonly metastasize to liver at which the patient is then diagnosed w/ Carcinoid Syndrome. Key feature: This tumor produces serotonin and it is delivered to the liver via portal vein. Presents: flushing of skin, diarrhea (>70%), intermittent wheezing and dyspnea, facial telangiectasia, tricuspid regurgitation and pulmonary stenosis (b/c serotonin causes increased collagen production in valves). Dx: increased urine Serotonin, CT scan of liver for mets. Rx:Avoid alcohol, resect the tumor, chemo, and somatostatin analogue.

18.3.2. Gastric Polyps FA12 p358 Hyperplastic Juvenile Peutz-Jeghers

18.4. Oral

18.4.1. Squmous Cell Carcinoma, mouth

18.5. Salivary Gland

18.5.1. Pleomorphic Adenoma, salivary gland

18.5.2. Warthin Tumor

18.5.3. Mucoepidermoid carcinoma

18.6. Esophagus

18.6.1. Barrett's Esophagus

18.6.2. Esophageal carcinoma

18.6.3. Squamous cell carcinoma, esophagus

19. Gall Bladder

19.1. Gall bladder carcinoma

20. Blood

20.1. Hodgkin's Lymphoma

20.2. Non-Hodgkin's Lymphoma

20.2.1. Burkitt's Lymphoma

20.2.2. Diffuse Large B-cell lymphoma

20.2.3. Mantle cell Lymphoma

20.2.4. Follicular lymphoma

20.2.5. neoplasms of mature T-cells Adult T-cell lymphoma Mycosis fungoides/ Sezary syndrome

20.3. Multiple Myeloma

20.4. Monoclonal Gammopathy of Undetermined Significance (MGUS)

20.4.1. monoclonal plasma cell expansion w/o the symptoms of multiple myeloma.

20.5. Leukemias

20.5.1. Lymphoid neoplasms Small Lymphocytic lymphoma (SLL)/ Chronic Lymphocytic Leukemia (CLL) Acute Lymphoblastic leukemia/lymphoma (ALL) Hairy Cell Leukemia

20.5.2. Myeloid Acute Myelogenous Leukemia (AML) Acute Promyelocytic Leukemia (M3) Chronic Myelogenous Leukemia (CML)

20.6. Langerhans Cell Histiocytosis (LCH)

21. Female

21.1. Uterus

21.1.1. Leiomyoma, Uterus

21.1.2. Endometrial Carcinoma, Uterus

21.1.3. Endometrial Poylp

21.2. Cervix

21.2.1. Severe Dyslasia, Cervix

21.2.2. Carinoma In Situ, Cervix

21.2.3. Cervical Carcinoma

21.3. Breast

21.3.1. Invasive Ductal Carcinoma, Breast

21.3.2. Infiltrative (Invasive) Lobular Carcinoma, Breast

21.3.3. Metastatic Breast Ductal Adenocarcinoma

21.3.4. Gynecomastia, male breast

21.3.5. Inflammation and Fat Necrosis, Breast

21.3.6. Fibroadenoma

21.3.7. Lobular Carinoma in Situ (LCIS)

21.3.8. [MALE]-breast cancer subareolar mass in older males. High density of breast tissue under the nipple. may produce discharge. Most common histological subtype is invasive ductal carcinoma. Lobular is rare since men have very few lobules. Assoc w/ BRCA2 mutations and Klinefelters syndrome.

21.3.9. Ductal Carcinoma In-Situ Can be treated by recsection unlike LCIS

21.4. Vulva

21.4.1. Condyloma

21.4.2. Vulvar Carcinoma

21.5. Vagina

21.5.1. Clear Cell Adenocarcinoma, vagina

21.5.2. Embryonal Rhabdomyosarcoma, vagina

21.5.3. Vaginal carcinoma

21.6. Ovary

21.6.1. Surface Epithelial Tumor

21.6.2. Endometrioid Tumor

21.6.3. Brenner Tumor

22. Eye

22.1. Retinoblastoma

22.2. Metastatic Melanoma to the Eye

23. Pediatric Neoplasia

23.1. leukemia