path endocine system - formatted Flashcards

Question

27. 20.02.61 A 16yr old female 140cm in height and weight 57kg has primary amenorrhoea. Most likely cause ? 1. Testicular feminisation 2. Prolactinoma 3. Anorexia 4. Polycystic ovaries 5. Turners syndrome

Answer 1

5. Turners syndrome • The most common cause of primary amenorrhea is delayed puberty due to some genetic factor that delays physical development. Being short is the most common sign of this, although sometimes a family history of delayed menstruation can indicate this situation. Time usually resolves the problem eg Turners

Answer 2

1: Paraganglioma, chemodectoma, carotid body tumours are interchangeable (with respect to paragangliomas within the carotid body location). *LW: Radiopaedia states, "Carotid body tumor, also known as a chemodectoma or carotid body paraganglioma, is a highly vascular glomus tumor that arises from the paraganglion cells of the carotid body", which is further confirmed in Large Robbins. Large Robbins states: - Paraganglia related to great vessels of head and neck.... infrequently release catecholamines, but because the cells that make up these lesions sense O2 and CO2 tensions within adjacent vessels, the tumours are also sometimes referred to as chemodectomas. - Tumors arising in the carotid body are designanted carotid body tumours - Tumors in the jugulotympanic body are referred to chemodectomas as these paraganglia sense oxygen and CO2 levels. Robbins: Carotid body tumours: frequently recur after incomplete resection, with many metastasising. Shamblin classification: types 2 and 3 are adherent to vessels. 2. often adherent to vessels resulting in incomplete excision and recurrence of 10% * I think that it may have stated 10-40% in the exam see quote from robbins below: * Despite the infrequency and small size of paragangliomas, they are of great clinical importance because from 10 to 40% are malignant and recur after resection * Despite encapsulation, well developed or scant, they are often densely adherent to adjacent vessels and difficult to excise

Answer 3

5. Reidel’s Thyroiditis – F - rare disorder of unknown etiology characterised by extensive fibrosis; hard, firm thyroid mass. Thyroid function depends on the extent to which the normal thyroid gland has been replaced by fibrotic tissue. Most patients are euthyroid. Hypothyroidism is noted in approx 30% of cases. Very rarely can hyperthyroidism occur, but this is probably secondary to a coexisting condition. 29. PATH2004 Least likely to cause hyperthyroidism. (TW) (--) 1. Graves disease - T - most common cause of endogenous hyperthyroidism. Autoimmune disorder produced by autoantibodies to the TSH receptor. 2. Toxic multinodular goitre - T 3. Toxic adenoma - T - in a minority of cases adenomas may be hyperfunctional with autonomous secretion of thyroid hormone. 4. Hashimoto’s thyroiditis - T - 5% do get initial hyperthroidism. In some cases it may be preceded by transient thyrotoxicosis. Autoimmune destruction of thyroid gland. 5. Reidel’s Thyroiditis – F - rare disorder of unknown etiology characterised by extensive fibrosis; hard, firm thyroid mass. Thyroid function depends on the extent to which the normal thyroid gland has been replaced by fibrotic tissue. Most patients are euthyroid. Hypothyroidism is noted in approx 30% of cases. Very rarely can hyperthyroidism occur, but this is probably secondary to a coexisting condition. 6. Subacute - T - both Granulomatous (de Quervain) and Lymphocytic forms of subacute thyroiditis have transient hyperthyroidism. Principle clinical manifestation of painless thyroidits is hyperthyroidism. Increased T3, T4, and reduced TSH. 7. Viral thyroiditis- T - transient hyperthyroidism. Common • Grave’s (85%) • Toxic Mutinodular Goitre (10%) • Toxic adenoma (4%) Uncommon (1%) • Acute/subacute thyroiditis • Hyperfunctioning thyroid carcinoma • Choriocarcinoma or hydatidiform mole • TSH-secreting pituitary adenoma • Neonatal thyrotoxicosis- a/w maternal Grave’s • Struma ovarii (ovarian teratomatous thyroid) • Iodide-induced hyperthyroidism • Iatrogenic (exogenous)

Answer 4

3. Bilateral or unilateral enlarged lobes T – the gland may be unilaterally or bilaterally enlarged 30. PATH2004 DeQuervain macroscopic appearance (--) (TW) 1. Diffuse smooth symmetrical goitre - F - Hashimotos / Graves 2. Multiple nodules - F - MNG 3. Bilateral or unilateral enlarged lobes T – the gland may be unilaterally or bilaterally enlarged 4. Only microscopic changes - F - subacute lymphocytic (painless) thyroiditis ("except for possible mild symmetric enlargement, the thyroid appears normal on gross inspection"). • The gland may be unilaterally or bilaterally enlarged and firm, with an intact capsule. It may be slightly adherent to surrounding structures. • In active inflammatory phase, scattered follicles may be disrupted and replaced by neutrophils forming microabscesses • Later = more classical aggregations of lymphocytes, histiocytes & plasma cells around damaged follicles • Multinucleated giant cells enclosing fragments of colloid, hence the designation granulomatous thyroiditis

Answer 5

1. T2hyperintense

Answer 6

Hashimoto’s - T - HT = chronic autmoimmune thyroid disorder characterised by slowly destructive lymphoid infiltrate of thyroid gland (and high circulating titres of antithyroid Abs). Occurs with increased frequency in patients with Down's syndrome and Turner's syndrome. Hürthle cells (oncycytes) are observed in both neoplastic and nonneoplastic conditions of the thyroid gland (eg, Hashimoto thyroiditis, nodular and toxic goiter).Note- increased risk in-> down's-> Turners- Hurstle cells **SCS added options to make this a question. Correct answer is Hashimoto. - Warthins - oncocytes present but not ass/w Turners. - Cystic hygroma are associated w Turners on antenatal imaging.

Answer 7

5. Shithouse - Malignant thyroid tumors: Papillary 75-85%, Follicular 10-20%, Medullary 5%, Anaplastic <5%. Papillary - young (esp 20-40yo), female, lymphatic spread, calcification, orphan annie nuclei, irradiation, pasmmoma bodies. Px 10y survival 98%. Follciular - any age (peak 40s-50s), associated with goitre, not an FNA diagnosis (can look like follicular adenoma), hematogenous spread. Px 92% 10y survival. Worse if larger lesion. Medullary - any age (peak 20-30s for MEN, 40-50s sporadic), tumor of parafollicular (C) cell origin, produces calcitonin, MENIIa, MENIIb, Familial MTC, NF, vHL, <50% show broad sheets of amyloid, lymphoid and hematogenous spread. Px 90% 10y survival. Mor aggressive MENIIb. Anaplastic - >60yo, locally invasive, 50% have goitre, 20% has Hx or previously more differentiated tumor (?develops from this ?due to p53 tumor suppressor gene loss). Px approx 100% mortality.

Answer 8

2. Warthins - T - Warthins: 5th – 7th decades; M>F; 8 x risk in smoking, oncocytes p 772 old Robbins. Oncocytes are epithelial cells stuffed with mitochondria that impart the granular appearance to the cytoplasm. 3. 55yo man - Oncocytes in biopsy specimen from solitary parotid cyst (TW) 1. Benign pleomorphic adenoma - F - great histologic heterogeneity. Epithelial elements resembling ductal cells or myoepithelial cells. 2. Warthins - T - Warthins: 5th – 7th decades; M>F; 8 x risk in smoking, oncocytes p 772 old Robbins. Oncocytes are epithelial cells stuffed with mitochondria that impart the granular appearance to the cytoplasm. 3. HIV - F 4. Type 1 branchial cleft cyst - F - fibrous walls, usually lined by stratified squamous or pseudostratified columnar epithelium underlaid by an intesne lymphocytic infiltrate. 5. Mucoepidermoid - F - cords, sheets, or cystic configurations of squamous, mucous, or intermediate cells

Answer 9

3. Bilateral or unilateral enlarged lobes 4. DeQuervain macroscopic appearance (TW) 1. Diffuse smooth symmetrical goiter - F - Hashimotos / Graves 2. Multiple nodules - F - MNG 3. Bilateral or unilateral enlarged lobes 4. Only microscopic changes - F - subacute lymphocytic (painless) thyroidits. AKA lymphocytic thyroiditis. DeQuervains (AKA subacute (granulomatous) thyroiditis): - Self-limited granulomatous thyroiditis - Hyperthyroid then hypothyroid then euthyroid. - distinct histology characterised by GC formation / granulomata. - Recent viral infection (mumps, measles, flu adenovirus, coxsackievirus, echovirus). - Lobes can be normal sized or enlarged (uni-/bilateral). Regions of decreased echogenicity and decreased vascularity on USS. - Reduced uptake on NM study. - Typically middle aged women.

Answer 10

1. Adrenal cortex - F - adrenal medulla 1. Phaeochromocytoma not a common location? (TW) 1. Adrenal cortex - F - adrenal medulla 2. Adrenal medulla - T - Approx 95% of catecholamine-secreting tumors are in the abodmen, of which 85-90% are intraadrenal (phaeochromocytoma) UTD. 3. Mediastinum - T 4. Bladder - T 5. Organ of Zuckerkandal - T - Most common extraadrenal location Pheochromocytoma arises from pheochromocytes the predominant cells of the adrenal medulla, these cells were widespread in the fetal sympathetic chain but typically degenerate after birth. Radiographics: More than 90% of phaeochromocytomas are located within the adrenal glands, and 98% occur within the abdomen. Extraadrenal pheochromocytomas develop in paraganglionic chromaffin tissue of the sympathetic nervous system. They may occur anywhere from the base of the brain to the urinary bladder and are considered to be paraganlgiomas. Common locations for extraaadrenal pheochromocytomas include the organ of Zuckerkandl, bladder wall, retroperitoneum, heart, mediastinum, and carotid and glomus jugulare bodies. 1% located in thorax, most paravertebral. Have been described in pericardium. Female reproductive organs have been described but extremely rare. AJR

Answer 11

1. increased VMA - F - (least correct) pheos seen with MEN 2A and 2B but not MEN 1. Can get carcinoids with MEN 1, and for this would test for urinary 5-HIAA (hydroxyindoleacetic acid) which is end product of serotonin metabolism - note however that carcinoids can produce other biogenic amines (ie Norepinephrine / dopamine) whose break down product is VMA. 2. CT request for patient with MEN 1 which are features (TW) 1. increased VMA - F - (least correct) pheos seen with MEN 2A and 2B but not MEN 1. Can get carcinoids with MEN 1, and for this would test for urinary 5-HIAA (hydroxyindoleacetic acid) which is end product of serotonin metabolism - note however that carcinoids can produce other biogenic amines (ie Norepinephrine / dopamine) whose break down product is VMA. 2. Increased parathormone - T - primary hyperparathyroidism is the most common manifestation of MEN 1. Multiple gland involvement is typical. Hyperplasia more common than adenoma. 3. GI mass - T - (this was previous remembered as false) pancreatic islet cell or gastrointestinal endocrine cell tumors become clinically apparent in approx 1/3rd patients with MEN1. Most common cause for symptomatic disease is Zollinger-Ellison. Duodenum is a common site for tumors (gastrinomas). 4. Pituitary mass - T - clinically apparent (anterior) pituitary adenomas develop in approximately 15-20% of patients with MEN1 when sought by CT or MRI. Most common type of pituitary tumor in MEN1 is a prolactinoma, but a GH, ACTH, gonadotroph, and non-functioning tumors can also occur.

Answer 12

4. Increased lymphoma in Gastric wall. F - complications include: malignant islet cell tumour (in 60%), liver mets with continued gastric secretion, perforated DU/jejunal ulcer, oesoph stricture with reflux, obstruction, GI bleeding, gastric carcinoids (esp. in MEN1). 3. Which is not a feature of Zollinger-Ellison? (GC) 1. Hypervascular GI mass. T 2. Gastrinoma. T - non-beta cell gastrin-secreting tumour of the pancreas 3. Increased ulcers. T - multiple in 10%; solitary ulcer in 90%; recurrent or intractable; unusual locations (postbulbar). 4. Increased lymphoma in Gastric wall. F - complications include: malignant islet cell tumour (in 60%), liver mets with continued gastric secretion, perforated DU/jejunal ulcer, oesoph stricture with reflux, obstruction, GI bleeding, gastric carcinoids (esp. in MEN1). 5. Atrophic Gastritis. - ? - hypergastrinaemia causes gastric gland hyperplasia (mainly parietal cells in fundus). [Robbins; eMedicine] The symptoms of Zollinger-Ellison syndrome are secondary to hypergastrinemia, which causes hypertrophy of the gastric mucosa and leads to an increase in the number of parietal cells (as many as 6 times the reference range value) and increased maximal acid output. On its own, gastrin also stimulates acid secretion, resulting in increased basal acid secretion. The increased acid production leads to mucosal ulceration of the GI tract, diarrhea, or malabsorption. Malabsorption in patients with Zollinger-Ellison syndrome is usually multifactorial and is caused by direct mucosal damage (caused by increased stomach acid), inactivation of the pancreatic enzymes, and the precipitation of bile salts.

Answer 13

2. Fibrosing nodules - ?F - indicating Reidel thyroiditis? however there is a fibrosing variant (rare) of hashimotos * *LJS - Can have fibrosis (fibrosing variant) - but not extending beyond capsule of thyroid gland (ddx Reidel thyroiditis) 4. Autopsy of the thyroid in Hashimotos shows? (TW) 1. Hurthle cells - T - This is presumable the best single answer. Thyroid follicles are small and are lined in many areaas by epithelial cells with abundant eosinophilic, granular cytoplasm, termed Hürthle cells. Hürthle cells (oncycytes) are observed in both neoplastic and nonneoplastic conditions of the thyroid gland (eg, Hashimoto thyroiditis, nodular and toxic goiter). 2. Fibrosing nodules - ?F - indicating Reidel thyroiditis? however there is a fibrosing variant (rare) of hashimotos. * *LJS - Can have fibrosis (fibrosing variant) - but not extending beyond capsule of thyroid gland (ddx Reidel thyroiditis) 3. Psammoma bodies - T - Thyroid microcalcifications are psammoma bodies and are one of the most specific features of thyroid malignancy. They are found in all primary thyroid carcinomas, most commonly papillary. Also seen in benign conditions such as follicular adenoma and Hashimoto thyroiditis (Radiographics 2007). **LJS - Papillary carcinoma ass/w Hashimotos, might be getting at this.

Answer 14

3. Bilateral or unilateral enlarged lobes 5. Macroscopic features of Dequervians Thyroiditis: (TW) 1. Diffuse smooth symmetrical goiter - F - Hashimotos / Graves 2. Multiple nodules - F - MNG 3. Bilateral or unilateral enlarged lobes 4. Only microscopic changes - F - subacute lymphocytic (painless) thyroidits. AKA lymphocytic thyroiditis. DeQuervains (AKA subacute (granulomatous) thyroiditis): Self-limited granulomatous thyroiditis with distinct histology characterised by GC formation / granulomata. Probably viral links with previous viral infection (mumps, measles, flu adenovirus, coxsackievirus, echovirus). 30-50yo. Women 5x > men.

Answer 15

4. Reidel’s Thyroiditis – F - rare disorder of unknown etiology characterised by extensive fibrosis; hard, firm thyroid mass. Thyroid function depends on the extent to which the normal thyroid gland has been replaced by fibrotic tissue. Most patients are euthyroid. Hypothyroidism is noted in approx 30% of cases. Very rarely can hyperthyroidism occur, but this is probably secondary to a coexisting condition. 6. Least likely to cause Hyperthyroidism? (TW) 1. Graves disease - T - most common cause of endogenous hyperthyroidism. Autoimmune disorder produced by autoantibodies to the TH receptor. 2. Toxic adenoma - T - in a minority of cases adenomas may be hyperfunctional with autonomous secretion of thyroid hormone. 3. Hashimotos thyroiditis - T - 5% do get initial hyperthroidism. In some cases it may be preceded by transient thyrotoxicosis. Autoimmune destruction of thyroid gland. 4. Reidel’s Thyroiditis – F - rare disorder of unknown etiology characterised by extensive fibrosis; hard, firm thyroid mass. Thyroid function depends on the extent to which the normal thyroid gland has been replaced by fibrotic tissue. Most patients are euthyroid. Hypothyroidism is noted in approx 30% of cases. Very rarely can hyperthyroidism occur, but this is probably secondary to a coexisting condition. 5. Subacute thyroiditis – T - principle clinical manifestation of painless thyroidits is hyperthyroidism. Increased T3, T4, and reduced TSH.

Answer 16

1. In regards to thyroid cancer: (TW) 1. 20% of medullary thyroid cancer associated with MEN - T - ~80% of MTC are sporadic, the rest are familial as part of the MEN2 syndrome.

Answer 17

3. Sarcoidosis - F 2. Phaeochromocytoma not associated with: (TW) 1. MEN - T - MEN2A: P-M-P (pheochromocytoma, medullary carcinoma thyroid, phyerparathyroidism due to primary parathyroid hyperplasia). MEN2B / 3: M-P-MN (medullary carcinoma thyroid, pheo, mucosal neuromas / intestinal ganglioneuromas, marfanoid). Chromosome 10 2. VHL - T - two primary familial disorders associated with pheochromocytoma, both autosomal dominant inheritance: vHL and MEN2. vHL phenotype: pheochromocytoma (frequently bilateral), paraganglioma (rarely), retinal angioma, cerebellar hemangioblastoma, epididymal cystadenoma, renal and pancreatic cysts, pancreatic neuroendocrine tumors, and renal cell carcinoma. Chromosome 3p25 3. Sarcoidosis - F 4. NF - T - AD, chromosome 17, Approx 2% of pts with NF1 develop catecholamine-secreting tumors (usually a solitary benign adrenal pheochromocytoma). 5. Can’t remember e

Answer 18

1. Anaplastic - T - surgery often not indicated as usually advanced disease. Locally advanced inoperable disease - combined XRT and chemotTX. If localized, complete resection should be attempted with postoperative combined modality therapy with radiation and chemotherapy for tumors that are resected completely (no extrathyroid spread). Hyperfractionated XRT (high 'radical' doses >Gy) appears to be associated with improved local control of disease. 3. 76 year old male for radiotherapy of thyroid lesion. Most likely? (TW). Presuming they mean external beam and not I131. 1. Anaplastic - T - surgery often not indicated as usually advanced disease. Locally advanced inoperable disease - combined XRT and chemotTX. If localized, complete resection should be attempted with postoperative combined modality therapy with radiation and chemotherapy for tumors that are resected completely (no extrathyroid spread). Hyperfractionated XRT (high 'radical' doses >Gy) appears to be associated with improved local control of disease. 2. Follicular - F - Surgery + I131 3. Medullary - F - Best treatment for MC is thyroidectomy and lymphadenectomy. Note that early studies found no survival benefit from ext beam XRT (ie path notes), however retrospective analysis showed XRT may prolong the interval until disease progression or recurrence in some patients. XRT useful for primary therapy if resection incomplete, and for adjuvant therapy. ChemoTx, transarterial chemoembolisation also can be used for advanced / residual disease. 4. Papillary - F - surgery + I131 5. Lymphoma - ?F (but also potentially true as well) - Pts with thyroid lymphoma should be staged like any other patient with lymphoma. Stage I or limited stage II - choid is between radiotherapy alone for indolent lymphoma, or combined XRT and chemoTx for aggressive histologies. Rate of local contral with 35-40Gy given in 4 weeks is excellent. Radiation therapy alone is adequate Tx for localized indolent lymphomas, such as MALT lymphoma or follicular lymphoma. Combined Tx for all patients with aggressive histiology thyroid lymphoma.

Answer 19

3. Calcification - T - coarse calcifications are the most common type of calcification in MTCs (Radiographics). Thyroid microcalcifications are psammoma bodies and are one of the most specific features of thyroid malignancy. They are found in all primary thyroid carcinomas, most commonly papillary. 1. Which is true regarding medullary carcinoma? (TW) 1. 10-15% are associated with BRCA 1 or 2 - F - think "Boobs, Bums, Booze, Babies". Increased risk in Boobs: Breast (male and female); Bums: Colon (only minimal increase); Booze: Pancreatic cancer; Babies: Ovarian and Prostate cancer. 2. Age >65 - F - generally peak incidence is 40-50yo, or younger (20-30yo) for familial types. Papillary (20-40yo). Follciular (40-50yo). Anaplastic (>60yo). 3. Calcification - T - coarse calcifications are the most common type of calcification in MTCs (Radiographics). Thyroid microcalcifications are psammoma bodies and are one of the most specific features of thyroid malignancy. They are found in all primary thyroid carcinomas, most commonly papillary. In most patients with MTC, the disease has already metastasized at the time of diagnosis.

Answer 20

2. Solitary adenoma - T - most common subtypes of primary adlosternoism are: aldosterone-producing adenoma; bilateral idiopathic hyperaldosteronism. Less common forms include: unilateral adrenal hyperplasia; bilateralhyperplasia; familial hyperaldosteronism; pure aldosterone-producing adrenocortical carcinoma and ectopic aldosterone-secreting tumors. 2. Which is true regarding primary hyperaldosteronism? (TW) 1. Hyperkalaemia - F - hypo. Aldosterone in kidney: increases number of open sodium channels in luminal membrane leading to increased sodium (cations) resorption, which makes the lumen electronegative, thereby creating an electrical gradient that favors secretion of cellular potassium into the lumen - resultant hypokalemia (however is an inconsistent finding). 2. Solitary adenoma - T - most common subtypes of primary adlosternoism are: aldosterone-producing adenoma; bilateral idiopathic hyperaldosteronism. Less common forms include: unilateral adrenal hyperplasia; bilateralhyperplasia; familial hyperaldosteronism; pure aldosterone-producing adrenocortical carcinoma and ectopic aldosterone-secreting tumors. 3. Hyponatraemia - F - increased sodium resorption (see ans. 1.). There may be mild hypernatremia only, due to the bodys regulatory mechanism: ADH released + thirst which maintains a stable sodium concentration. 4. Metabolic acidosis - F - alkalosis. Increased urinary H= secretion mediated by hypokalemia (which directly increases bicarbonate resorption) and direct stimulatory effect of aldosterone on distal acidification. In a low K+ state: get a shift of K+ out of cells / exchanged for H+ which moves into cells to compensate. Classic presenting signs of primary aldosteronism are hypertension and hypokalemia. Added option 4.

Answer 21

1. Papillary RCC - F - RCCs of predominant papillary, chromophobe, or oncocytic histology are not associated with vHL disease (UTD). pRCC is the 2nd most common subtype, and most common multifocal or bilateral renal tumor. Most pRCCs are sporadic. 3. VHL is associated with all of the following except: (TW) 1. Papillary RCC - F - RCCs of predominant papillary, chromophobe, or oncocytic histology are not associated with vHL disease (UTD). pRCC is the 2nd most common subtype, and most common multifocal or bilateral renal tumor. Most pRCCs are sporadic. 2. Clear cell RCC - T - all vHL-associated RCCs are clear cell tumors, although these clear cell tumors may contain minor papillary components (usually <25% of the lesion). 3. 3p deletions - T - vHL gene Ch 3p25. Specific germline mutations or deletions of the vHL gene can influence the clinical manifestations of vHL disease. 4. Retinal angiomas - T - see below 5. Pheochromocytomas - T - vHL phenotype: pheochromocytoma (frequently bilateral), paraganglioma (rarely), retinal angioma, cerebellar hemangioblastoma, epididymal cystadenoma, renal and pancreatic cysts, pancreatic neuroendocrine tumors, and renal cell carcinoma.

Answer 22

1. Nephritic syndrome - F - proteinuria (usually not severe), hematuria (RBC casts), hypertension, uremia and variable renal insuffciency (azotemia, oliguria). 4. Phaeochromocytoma is not associated with: (TW) 1. Nephritic syndrome - F - proteinuria (usually not severe), hematuria (RBC casts), hypertension, uremia and variable renal insuffciency (azotemia, oliguria). 2. Elevated VMA - T - VMA: vanillylmandelic acid is metabolite of norepinephrine, elevated in phaeo and neuroblastoma. If high clinical suspicion test 24hr urine and plasma metanephrines (plasma metanephrines probably superior test); if low clinical suspicion test 24hr urine. If positive then MRI or CT. 3. Headache - T - classic triad of pheochromocytoma is 1) episodic headache, 2) sweating, 3) tachycardia. About 50% have paroxysmal hypertension; most of rest have what appears to be essential hypertension. Symptoms of phaeochromocytoma are cause by tumoral hypersecretion of norepinephrine, epihephrine and dopamine, although increased central sympathetic activity may also contribute.

Answer 23

4. Sarcoidosis - F 5. Phaeochromocytomas are not associated with: (TW) 1. VHL - T - two primary familial disorders associated with pheochromocytoma, both autosomal dominant inheritance: vHL and MEN2. vHL phenotype: pheochromocytoma (frequently bilateral), paraganglioma (rarely), retinal angioma, cerebellar hemangioblastoma, epididymal cystadenoma, renal and pancreatic cysts, pancreatic neuroendocrine tumors, and renal cell carcinoma. Chromosome 3p25. 2. MEN 2 - T - MEN2A: P-M-P (pheochromocytoma, medullary carcinoma thyroid, phyerparathyroidism due to primary parathyroid hyperplasia). MEN2B / 3: M-P-MN (medullary carcinoma thyroid, pheo, mucosal neuromas / intestinal ganglioneuromas, marfanoid). Chromosome 10. Sipple syndrome (MEN 2). 3. Neurofibromatosis - T - AD, chromosome 17, Approx 2% of pts with NF1 develop catecholamine-secreting tumors (usually a solitary benign adrenal pheochromocytoma). 4. Sarcoidosis - F Most catecholamine-secreting tumors are sporadic, however 15-20% have the disease as part of a familial disorder; in these patients it is more likely to be bilateral adrenal phaeo's or paragangliomas. MEN 1 (Wermer): PPP (primary hyperparathyroidism, pituitary tumors, pancreatic neuroendocrine tumors.

Answer 24

a. Follicular overlapping features with papillary - F - follicular has variable architecture: typically microfollicular architecture with uniform cuboidal cells. No nuclear features of papillary carcinoma and no psamomma bodies. * *note papillary is allowed to have some follicular features, but if any there are papillary features, gets called papillary (Robbins) (RY) 1. Cancer Thyroid: (TW) a. Follicular overlapping features with papillary - F - follicular has variable architecture: typically microfollicular architecture with uniform cuboidal cells. No nuclear features of papillary carcinoma and no psamomma bodies. b. Medullary Cancer amyloid deposits - T - Medullary - any age (peak 20-30s for MEN, 40-50s sporadic), tumor of parafollicular (C) cell origin, produces calcitonin, MENIIa, MENIIb, Familial MTC, NF, vHL, <50% show broad sheets of amyloid, lymphoid and hematogenous spread. Px 90% 10y survival. More aggressive MENIIb c. Paillary lymph node mets - T - Papillary - young (esp 20-40yo), female, lymphatic spread, calcification, orphan annie nuclei, irradiation, pasmmoma bodies. Px 10y survival 98%. d. Follicular Hematogenous - T - Follciular - any age (peak 40s-50s), associated with goitre, not an FNA diagnosis (can look like follicular adenoma), hematogenous spread. Px 92% 10y survival. Worse if larger lesion e. Female more common than male - T: female predominance in early and middle aged adults presenting with thyroid cancer, and equal distribution in childhood and late adult years (LW).

Answer 25

d. Granulosa - F - ?bad recall - not sure if this meant 'granuloma' in which case referring to subacute (granulomatous thyroiditis) AKA DeQuervain thyroiditis. 2. Hashimotos – False (TW) a. Lymphocyte infiltrate is sine qua non - T - extensive infiltration of parenchyma by a mononuclear inflammatory infiltrate containing small lymphocytes, plasma cells, and well-developed germinal centers. The sine quq non is stromal infiltration by lymphocytes and variable numbers of plasma cells. b. Hurthle cell - T - Thyroid follicles are small and are lined in many areas by epithelial cells with abundant eosinophilic, granular cytoplasm, termed Hürthle cells. Hürthle cells (oncycytes) are observed in both neoplastic and nonneoplastic conditions of the thyroid gland (eg, Hashimoto thyroiditis, nodular and toxic goiter). c. Autoimmune thyroiditis - T - chronic autoimmune thyroid disorder characterised by a slowly destructive lymphoid infiltrate of the thyroid gland, variable thyroid function, and high circulating titres of antithyroid antibodies, d. Granulosa - F - ?bad recall - not sure if this meant 'granuloma' in which case referring to subacute (granulomatous thyroiditis) AKA DeQuervain thyroiditis. e. Hyper functioning early - T - transient hyperthyroid with increased T3/T4 and reduced TSH, later have reduced T3/T4 and elevated TSH.

Answer 26

a and e 12. CA Thyroid (JS) a. 20% MEN with medullary - T - 80% arise sporadically, the remaining 20% occur in the setting of MEN IIA or IIB or as familial tumours without an association with MEN b. Follicular growth - ??? c. Papillary Hematogenous spread - F - foci of lymphatic invasion are often present but involvement of blood vessels is uncommon. Mets to local LN in half of cases. d. Follicular ill defined mass - F - present as a slowly enlarging painless nodule e. Medullary calcitonin - T - neuroendocrine neoplasms derived from parafollicular (C) cells which secrete calcitonin

Answer 27

b. Carcinoid occur in alveolar cells and deviates rather than invading adjacent bronchi - F - arise from K cells of bronchial mucosa and penetrate the bronchial wall to fan out producing the collar-button lesions 18. Neuroendocrine neoplasm of the lungs – FALSE (JS) a. Small foci of neuro endocrine cells in a scar are inconsequential - T - can have benign tumorlets in areas of scarring or chronic inflammation b. Carcinoid occur in alveolar cells and deviates rather than invading adjacent bronchi - F - arise from K cells of bronchial mucosa and penetrate the bronchial wall to fan out producing the collar-button lesions c. Mitotic rate, lymph node invasion and differentiation important to differentiate between typical and atypical carcinoid but this is difficult with a FNA - T - I presume but can't find anything about FNA diagnosis... d. Small cell is neuroendocrine tumour - T - contain neurosecretory granules similar to those found in Kulchitsky cells

Answer 28

c. Solid pseudopapillary tumour - T - most common in <35yo, 90% are female. Gross: large, usually encapsulated, hemorrhagic, necrotic, rarely multifocal; most common in tail of pancreas. Excellent prognosis after excision; mets in 4% (omentum, liver, LNs). [Path outlines; Dahnert] 26. 18 year old with a pancreatic neoplasm is most likely to be: (GC) a. Intraductal mucinous papillary tumour - F - typically older patients. b. Cystadenocarcinoma - F - 40-60yo; mucinous tumour - large, thick-walled, usually in body + tail; eggshell Ca2+. c. Solid pseudopapillary tumour - T - most common in <35yo, 90% are female. Gross: large, usually encapsulated, hemorrhagic, necrotic, rarely multifocal; most common in tail of pancreas. Excellent prognosis after excision; mets in 4% (omentum, liver, LNs). [Path outlines; Dahnert] d. Cystadenoma - F - >60yo, 70% are female, associated with VHL.

Answer 29

b. Near the origin of the inferior mesenteric artery - T - 2nd most common site of extra-adrenal pheochromocytoma (2-5%), after the paraaortic sympathetic chain (8%). 29. The organ of Zuckerkandl is located: (GC) a. In the bladder wall - F - accounts for 1% of extra-adrenal pheochromocytomas. b. Near the origin of the inferior mesenteric artery - T - 2nd most common site of extra-adrenal pheochromocytoma (2-5%), after the paraaortic sympathetic chain (8%). c. At the carotid artery bifurcation - F - carotid body d. In the adrenal gland - F - pheochromocytomas occur in adrenal medulla. [Dahnert]

Answer 30

c. Follicular carcinoma appears as an ill-defined nodule - F - Follciular - Encapsulated tumor sometimes very difficult to differentiate from adenomas. Any age (peak 40s-50s), associated with goitre, not an FNA diagnosis (can look like follicular adenoma), hematogenous spread. Px 92% 10y survival. Worse if larger lesion. US: indistinguishable from benign follicular adenoma. 32. Which of the following is false regarding thyroid carcinoma? (TW) a. Papillary carcinoma is associated with lymph node metastases - T - Papillary - young (esp 20-40yo), female, lymphatic spread, calcification, orphan annie nuclei, irradiation, pasmmoma bodies. Px 10y survival 98%. b. Medullary carcinoma is associated with MEN (? % attached to this stem) - T - Medullary - any age (peak 20-30s for MEN, 40-50s sporadic). Sporadic 80%. Tumor of parafollicular (C) cell origin, produces calcitonin, MENIIa, MENIIb, Familial MTC, NF, vHL, <50% show broad sheets of amyloid, lymphoid and hematogenous spread. Px 90% 10y survival. More aggressive MENIIb. Penetrance of medullary thyroid cancer in MEN2A is nearly 100%. MTC occurs in almost all MEN2b patients. c. Follicular carcinoma appears as an ill-defined nodule - F - Follciular - Encapsulated tumor sometimes very difficult to differentiate from adenomas. Any age (peak 40s-50s), associated with goitre, not an FNA diagnosis (can look like follicular adenoma), hematogenous spread. Px 92% 10y survival. Worse if larger lesion. US: indistinguishable from benign follicular adenoma.

Answer 31

*SCS: depends on if asking about breast ca or Thyroid cancer. If thyroid: C If breast: A * LW: Assuming thyroid medullary carcinoma: - Answer = C: calcification, occurs in medullary thyroid carcinoma, along with amyloid sheets. - No association with BRCA, and peaks 20-30s. If in relation to medullary breast carcinoma: a. 10-15% are associated with BRCA 1 or 2 - T - More frequent in women who inherit mutations of BRCA-1 gene. Up to 20% or breast cancers in BRCA1 carriers had Medullary histology, and up to 3% in BRCA2 carriers (JAMA 1998). 35. Which is true regarding medullary carcinoma? (TW) a. 10-15% are associated with BRCA 1 or 2 - T - More frequent in women who inherit mutations of BRCA-1 gene. Up to 20% or breast cancers in BRCA1 carriers had Medullary histology, and up to 3% in BRCA2 carriers (JAMA 1998). b. Age >65 - F - medullary and medullary-like carcinomas occur more frequently in younger patients than other types of breast cancer (~35yo). c. Calcification - F - rounded soft tumor that grows rapidly. Necrosis is common and may result in cyst formation. Calcification is uncommon and DCIS is either very limited or not present.

Answer 32

c. Lymphocytic hypophysitis - T - uncommon disorder that is initially characterized by lymphocytic infiltration and enlargement of the pituitary; this stage is followed by destruction of the pituitary cells. It most often occurs in late pregnancy or postpartum period. Affected patients typically present with headache. Preferential hypofunction of ACTH and TSH has been described, however diabetes insipidus, hyperprolactinemia, growth hormone excess, and associated autoimmune thyroiditis can occur. 37. A woman 3 months post partum presents with headache and hypopituitarism. Which is most likely? (TW) a. Empty sella - F - empty sella syndrome may be assoc with visual field defects and occasionally with endocrine anomalies, such as hyperprolactinemia. Only rarely is it associated with hypopituitarism because sufficient functioning parenchyma is maintained (Robbins). UTD says: although some papers claim deficiences of 1 or more pituitary hormones (in primary empty sella) - the evidence for such deficiencies is not convincing; ie, there is no consistent evidence of an "empty sella syndrome". If considering secondary ESS and Sheehan's, see below. b. Rathke cyst - F c. Lymphocytic hypophysitis - T - uncommon disorder that is initially characterized by lymphocytic infiltration and enlargement of the pituitary; this stage is followed by destruction of the pituitary cells. It most often occurs in late pregnancy or postpartum period. Affected patients typically present with headache. Preferential hypofunction of ACTH and TSH has been described, however diabetes insipidus, hyperprolactinemia, growth hormone excess, and associated autoimmune thyroiditis can occur. Sheehan's syndrome: Less common in developed countries. Typically pts have Hx of postpartum hemorrhage so severe as to cause hypotension and require transfusion of multiple units of blood. Severe hypopituitarism can be recognized during the 1st days or weeks after delivery by development of lethargy, anorexis, weight loss, and inability to lactate. Less severe hypopituitarism may be manifested in the wks and months after delivery. The mildest degrees of hypopituitarism may not be recognized for may years after the inciting event. Most patients have a small pituitary within a sella of normal size, sometimes read as an "empty sella" on MRI. More selective loss of the anterior pituitary lobe (supplied from low-pressure venous system) relative to the posterior pituitary which receives blood directly from arterial branches (less susceptible to ischaemic injury and is therefore usually not affected). Sheehan's syndrome, causing secondary empty sella (ie option a.) - I don't think would account for the 3/12 presentation (ie less severe Sheehan's) with both headache and hypopit. *** but open for discussion ***

Answer 33

b. Adenoma <2cm - T - 80% of cases are caused by an adenoma in one gland (Conn syndrome). These are almost always solitary and small (<2cm). Other causes include primary adrenocortical hyperplasia or glucocorticoid suppressible hyperaldosteronism 44. What is true regarding primary hyperaldosteronism? (JS) a. Hyperkaelaemia - F - Primary hyperaldosteronism is a group of syndromes characterised by excess aldosterone secretion, causing sodium retention and potassium excretion with resultant hypertension and hypokalaemia b. Adenoma <2cm - T - 80% of cases are caused by an adenoma in one gland (Conn syndrome). These are almost always solitary and small (<2cm). Other causes include primary adrenocortical hyperplasia or glucocorticoid suppressible hyperaldosteronism c. Adenoma >3cm

Answer 34

Both b and c b. Lymphoma in the gastric folds - T - complications include: malignant islet cell tumour (in 60%), liver mets with continued gastric secretion, perforated DU/jejunal ulcer, oesoph stricture with reflux, obstruction, GI bleeding, gastric carcinoids (esp. in MEN1). c. Atrophic gastritis - T causes a hypertrophic gastritis. 50. Which is not associated with Zollinger Ellison syndrome? (JS) a. Peptic ulcer disease - F - multiple in 10%; solitary ulcer in 90%; recurrent or intractable; unusual locations (postbulbar). b. Lymphoma in the gastric folds - T - complications include: malignant islet cell tumour (in 60%), liver mets with continued gastric secretion, perforated DU/jejunal ulcer, oesoph stricture with reflux, obstruction, GI bleeding, gastric carcinoids (esp. in MEN1). c. Atrophic gastritis - T - Causes hypertrophic gastritis. Hypergastrinaemia causes gastric gland hyperplasia (mainly parietal cells in fundus). d. Hypervascular pancreatic mass - F - may arise in the pancreas, peripancreatic region or wall of the duodenum e. Gastrinoma - F - Cause of ZE in 90% of cases = non-beta cell gastrin-secreting tumour of the pancreas * RY - note: Presume this is the old format of q where there are multiple correct answers. Zollinger Ellison specifically causes a hypertrophic gastritis (not atrophic). Although if question was high gastrin levels then high gastrin can be seen in autoimmune atrophic gastritis. Zollinger Ellison is not associated with lymphoma (classic association for gastric lymphoma is chronic H pylori gastritis). * AJL good explanantion. I agree.

Answer 35

d. Sarcoidosis

path endocine system - formatted Flashcards

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