Endocrinology 1

1. Endocrinology 1 Path picks Pituitary Adrenal Pancreas

2. Pituatory Infarction What is Sheehan syndrome? Pituitary infarction sometimes occurs after severe post partum haemorrhage. If the patient survives, she fails to lactate and develops signs and symptoms of pan hypo pituitarism. This is called Sheehan’s syndrome Histology of pituitary infarction with a surviving rim of pituitary on the outer surface of the gland in one corner (arrows).

3. Pituatory Infarction What is Sheehan syndrome? Pituitary infarction sometimes occurs after severe post partum haemorrhage. If the patient survives, she fails to lactate and develops signs and symptoms of pan hypo pituitarism. This is called Sheehan’s syndrome Histology of pituitary infarction with a surviving rim of pituitary on the outer surface of the gland in one corner (arrows).

4. Pituitary adenoma A large adenoma (arrows) has filled and expanded the pituitary fossa A large adenoma (arrows) of the pituitary compressing the optic chiasma. Some pituitary adenomas cause symptoms by their excessive hormonal secretion while others do not secrete hormones but cause symptoms by pressure effects on adjacent structures as they slowly increase in size.

5. Pan hypopituitarism. From top to bottom: pituitary fossa with a recurrent pituitary gland adenoma filling and expanding the pituitary fossa, gross atrophy of thyroid, adrenal cortex and testis. The tumour had originally been resected 24 years previously. At that time he had presented with decreased libido, shaving only every third day, cold sensitivity, tiredness, headaches and bitemporal hemianopia. He had been on long-term replacement therapy with cortisone, thyroxine, methyl testosterone and pitressin. Death was due to cerebral infarction.

6. Craniopharyngioma Craniopharyngioma is a type of brain tumor derived from pituitary glandembryonic tissue,[1] that occurs most commonly in children but also in men and women in their 50s and 60s.[2] It arises from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region and, therefore, contains deposits of calcium, which are evident on an x-ray. Histologically, craniopharyngiomas resemble adamantinomas (the most common tumors of the tooth). Patients may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopia, as the tumor may compress the optic chiasm. It has a point prevalence of approximately 2/100,000.[3] Craniopharyngiomas are also known as Rathke pouchtumors, hypophyseal duct tumors, or adamantinomas. Histology of craniopharyngiomashowing nests of squamous cells merging into columnar cells surrounding cystic spaces.

7. Adrenals Bisected adrenals. Extensive necrosis and granulomatous inflammation due to Cryptococcus neoformansinfection. This is one cause of Addison’s disease. Tuberculosis also causes destruction of the adrenal medulla.

8. Adrenal Haemorrhage What is Waterhouse-Friderichsen syndrome? Extensive haemorrhage into both adrenals in a male aged 10 months with meningococcal septicaemia (Waterhouse- Friderichsensyndrome). On admission to hospital he was febrile, shocked and comatose, and had a widespread haemorrhagic rash. Lumbar puncture yielded turbid C.S.F. from which N. meningitidiswas grown. Death occurred ½ hour later. The haemorrhage (arrows) is best seen in the 3 cut sections of the adrenal gland.

9. Adrenal Atrophy When you see a small adrenal gland what are the two options you should think? Child or atrophy. What are the main causes of adrenal atrophy? Long-term corticosteroid use Autoimmune destruction Adrenal cortical atrophy in a woman aged 57 with aplastic anaemia who was given large doses of prednisone (40- 90mg/day) during the last 4 months of life. Note how thin the cortex (blue arrows) is compared with the medulla (red arrows). Chronic autoimmune adrenalitisin a woman of 83 with Addison’s disease. Microscopy showed cortical atrophy, fibrosis and lymphoid infiltration of the residual epithelial islands. Hashimoto’s disease and chronic atrophic gastritis were also present.

10. Adrenal Hyperplasia Adrenal cortical hyperplasia in a patient with bronchogenic carcinoma and Cushing’s syndrome. Bilateral adrenal cortical hyperplasia in a woman aged 42. She was given 80 units of A.C.T.H. a day by intramuscular injection for 11 days before death. What are the main causes of Cushing Syndrome? due to excess levels of plasma cortisol, may be due to: (1) Cushing's disease (see above) in which there is bilateral hyperplasia of the adrenal cortex, (2) benign or (3) malignant neoplasms of the adrenal cortex, (4) ectopic production of ACTH (e.g. by a bronchogenic carcinoma) or (5) iatrogenic (steroid therapy).

11. Adrenal Tumours Adrenal Cortical Adenoma. The distinctive yellow colour, the small size and the similar texture to that of the adrenal cortex are all clues that this is an adrenal cortical adenoma. Carcinoma of the adrenal cortex in a male aged 11 months who resented with virilization. Operative surgical specimen. (The hole in the cut surface of the specimen is an artefact.) Carcinomas of the adrenal cortex are generally much larger than adrenal adenomas. Haemorrhage and necrosis are also characteristic of carcinomas and not adenomas

12. Adrenal Cortical Hyperplasia Nodular adrenal cortical hyperplasia: an incidental autopsy finding in a woman aged 54 who died of suppurative pyelonephritis. Mild nodularity of the adrenal cortex is common in middle aged and elderly patients and it is quite unusual for it to result in over production of cortisone. Nodular hyperplasia is however one cause of excessive production of cortisone.

13. Phaeochromocytoma Phaeochromocytomaof the adrenal. Operative surgical specimen. Note its location in the medulla – see the residual cortex at the edge of it (blue arrows). The redbrown colour is characteristic and quite different to the yellow of adrenal cortical hyperplasia and neoplasms. Histological appearance of a phaeochromocytoma. The cells are similar to those of the normal adrenal medulla but are arranged in broad trabeculae or islands. As with many types of endocrine neoplasm, including those of the adrenal, there are no reliable distinguishing features either gross or microscopic of benign or malignant other than the presence of metastasis.

14. Adrenal mets Bilateral adrenal secondaries from a primary lung cancer (arrows). Adrenal secondaries are frequently seen in lung cancer. Sometimes they cause so much destruction of the gland that the patient becomes hyper pigmented in the terminal stages of the disease.

15. Neuroblastoma Neuroblastomasoccur within the adrenal medulla. Sometimes they occur elsewhere along the sympathetic chain. This is one of the most common solid tumours of childhood. Secondary spread is mainly to regional lymph nodes, liver and lungs. Large neuroblastomashowing patchy necrosis and haemorrhage excised surgically from a female aged 16 months.She had a four week history of general ill health. The mother discovered an abdominal mass while she was bathing the child.

16. Normal Pancreas

17. Pancreatitis Relatively mild acute pancreatitis. The pancreas appears mildly congested and foci of calcification (arrows) are present within the gland. Over 80% of cases of acute pancreatitis are associated with either biliary calculi or alcohol abuse. Other less common causes include: infection, ischaemia, drugs, trauma, extension from adjacent inflamed tissues and metabolic causes such as hypercalcaemia, uraemia and Hypothermia . The key pathogenic feature of acute pancreatitis is the destructive effect of pancreatic enzymes released from acinarcells. Proteases, lipases and elastases are activated and released within the pancreas. Trypsin is probably the major activator of these. Possible ways pancreatic enzymes are activated and released include: obstruction of pancreatic duct, acinar cell injury and deranged intracellular transport of pancreatic enzymes. What causes the calcification? Pancreatic lipase release  fat supponification  fatty acid release  calcification

18. Pancreatitis Severe acute haemorrhagic pancreatitis. This was from a 48 year old female. Four days after elective cholecystectomy for cholelithiasis and chronic cholecystitis, she became shocked and developed ascites and her serum calcium fell. (Why would that be?) She died 2 days later. Note the obvious haemorrhage and the widespread deposits of calcium (arrows). Acute pancreatitis. The pre-existing architecture has been essentially destroyed. Haemorrhage and necrosis of acute pancreatitis, with some preserved pancreatic parenchyma on the left (arrow).

19. Complication of pancreatitis Focal acute necrosis of perirenalfat in a patient who had severe acute pancreatitis. The patient was oliguric due to acute renal tubular necrosis of ischaemic type. See the calcium deposition (arrows) highlighting foci of enzymatic fat necrosis.

20. Chronic Pancreatitis Severe chronic pancreatitis in a male alcoholic aged 45. The pathogenesis of chronic pancreatitis relates to: (1) ductal obstruction by concretions (2) decreased secretion of lithostathine, (3) oxidative stress, and (4) interstitial fat necrosis and haemorrhage with secondary fibrosis. The morphological features of chronic pancreatitis vary. They can include a shrunken fibrotic pancreas with foci of cystic change, and sometimes focal calcification. Sometimes there is less fibrosis and relatively more replacement of the parenchyma by adipose tissue. What is the main cause of chronic pancreatitis? Chronic pancreatitis with atrophy of the exocrine component and replacement by fibrous tissue.

21. CF Pancreatic abnormalities are also present in up to 90% of patients with cystic fibrosis. In mild cases, there is accumulation of mucus in small ducts, while in more severe cases, the ducts are blocked with secondary atrophy of exocrine glands and fibrosis. With severe atrophy of the exocrine component, only ducts and islets remain within a fibrofattystroma. Squamous metaplasia may occur in the duct epithelial lining. Cystic fibrosis (fibrocytic disease of the pancreas) in a boy aged 13 years. See duodenum (red arrows). The pancreas is not greatly reduced in size. Most of the parenchyma however has been lost and replaced by fibrofatty tissue (blue arrows). Bronchiectasis (black arrows), widespread suppurative pneumonia, patchy carnification, and hyperplasia of peribronchial lymph nodes (blue arrows) in a female aged 4½ years with cystic fibrosis. Meconium ileus had necessitated surgical intervention on the first day of life.

22. More CF Focal biliary fibrosis in a boy aged 13 years with fibrocystic disease of the pancreas. Histological examination shows that small bile ducts are obstructed by eosinophilic material. Note that while this superficially resembles cirrhosis, you cannot see rounded nodules each surrounded by fibrous tissue. The scarring here is far more irregular. Patients with this complication of cystic fibrosis usually present with signs of portal hypertension and liver failure at about this age. Meconium ileus and focal intestinal atresia (arrows) in a male aged 8 days with cystic fibrosis. Operative surgical specimen to treat the intestinal obstruction with which the patient presented. The atresia was an unassociated finding.

23. Cystadenoma of the pancreas Large benign cystadenoma of the pancreas causing obstruction of the duct and atrophy of the exocrine elements in the pancreatic tail (red arrows). Man aged 79 who had been aware of the presence of an abdominal mass for 2 years before he died of cerebral haemorrhage. Histology: Benign cystadenoma. Note the flat inconspicuous lining cells (arrows). Neoplasms: Cystic tumours of the pancreas account for about 5% of pancreatic neoplasms. They are usually present in the body or tail. They present as painless, slow growing masses. Many are multiloculated.

24. Pancreatic Carcinoma Carinoma (blue arrows) of the head of the pancreas with obstruction of ducts and atrophy of the body (red arrow) and the tail (black arrow).The dilated pancreatic duct can be seen for the whole length of the pancreas Carcinoma of the pancreas generally refers to carcinoma of the exocrine pancreas. The great majority of these arise from the ductal epithelium. The incidence of pancreatic cancer is increasing and as it usually presents late, the prognosis is generally poor. 60% arise in the head of the pancreas, thus producing obstructive biliary symptoms; 15-20% in the body, and about 5% in the tail.

25. Islet-Cell Tumour Gastrin-secreting islet cell tumour of the body of the pancreas in a man aged 45. He presented with duodenal ulceration, and then stomal ulceration following gastrojejunostomy. Blood gastrin level was raised. Pancreatic tumour resected. (The entire specimen comprises the tumour.) What syndrome may this patient have? Zollinger-Ellison

26. Haemochromatosis Haemochromatosis of the pancreas. The organ has become a uniform brown colour due to the deposition of iron. Haematochromatosisis an autosomal recessive condition in which excessive amounts of iron accumulate as ferritin and haemosiderinin various tissues of the body, including liver, pancreas, skin and myocardium. The pancreas becomes deeply pigmented and interstitial fibrosis is seen. Some parenchymal atrophy may be present. Haemosiderin is seen in both the exocrine and the endocrine components. Diabetes may result from the islet cell damage. The clinical condition of the ‘bronzed diabetic’ refers to diabetes in a patient with haematochromatosiswhose skin is a brown colour.