Clinical Topics Conference: Chapter 21, Endocrine Pathology

Click on a hyperlink.

  1. 32 year-old female with weight gain
  2. 35 year-old female with back pain
  3. 37-year-old woman with chronic renal failure
  4. 16-year-old female with weight loss and palpitations

Clinical vignette 1

The patient was a 32 year old woman with a two year history of weight gain and swelling of face and neck. Examination showed a round, hyperemic, somewhat swollen-appearing face. She had cutaneous striae along the lateral aspects of her abdominal wall. She stated that she had been diagnosed with diabetes some time in the past. Of the lab values it seemed important that she has a serum cortisol level of 8.6, considered elevated. Her baseline urine cortisol was 103 (normal 0-50) and 17-hydroxycorticoids were 15.1 (normal 2-8). These findings were considered to be consistent with Cushing disease or Cushing syndrome, and MRI scans of the head were taken. These showed a mass within a slightly enlarged sella turcica. A transphenoidal operation found a small adenoma of the pituitary gland. Histologically, this tumor was basophilic on H&E stain and showed tumor cells positive for ACTH on immunostaining.

Discussion topics

  1. What is the difference between Cushing disease and Cushing syndrome?

  2. What is the cause of the former and what causes exist for the latter?

  3. What are the principal findings on physical examination in these entities?

  4. What are the pertinent laboratory findings?

  5. Is malignant transformation to carcinoma within the realm of possibilities?
  • Go to top

    Clinical vignette 2

    This 35-year-old white woman was admitted with the complaint of severe shooting pain in the left scapular region of the back. Hirsutism had been evident since the menarche, which had occurred at nine years of age. Menstruation ceased six months previous to admission.

    Physical examination: revealed a masculine type of escutcheon and growth of hair on the face, neck, axillae, pubis, chest, arms and legs. Her voice was masculine and her breasts underdeveloped. A mass was palpated in the left upper quadrant of the abdomen. Lab and X-ray: Urine analysis disclosed 17-ketosteroids, 53.2 mg. per 24 yours (three times normal for females) and 11-oxysteroids, 3.0 mg. per 24 hours (the upper limits of normal). There was no glycosuria. Retrograde pyelogram demonstrated downward displacement of the left kidney and backward displacement of its upper pole. A tumor was seen on CT in the region of the left adrenal.

    Hospital course: On the 17th hospital day, a soft, brown, ovoid adrenal tumor, which weighed 2,300 gm. and measured 20 X 18.5 X 10 cm. was removed. The specimen had a vascular capsule which was smooth except for two areas 6.0 X 4.0 cm. which had no capsule. On section, necrotic and hemorrhagic tumor had penetrated the fibrous capsule.

    Pathology: On microscopic examination the tumor cells resembled those of the adrenal cortex. Nuclei were atypical and the cytoplasm was often vacuolated, suggesting the presence of hormonally active lipoid. Necrosis and hemorrhage were extensive.

    Follow-up: The patient was readmitted five weeks after surgery complaining of back pain. The masculine characteristics were still evident. It was thought that the adrenal tumor had recurred. She rapidly developed ascites and extreme jaundice and became disoriented and apprehensive. Laparotomy revealed a tremendously enlarged liver which was almost completely replaced with a rapidly growing, diffuse tumor. Histological examination disclosed a structure similar to that of the previously biopsied adrenal tumor. She expired on the 15th hospital day. At autopsy, metastatic carcinoma involved the liver, lungs, tail of the pancreas, mesenteric, peripancreatic, portahepatic and tracheobronchial lymph nodes and mesentery.

    Discussion topics

    1. Why are the 17-ketosteroids increased and the 11-deoxycortisol ketosteroids almost normal?

    2. Compare the clinical picture seen in this case with that seen in cortical adrenal hyperplasia (CAH, formerly called adrenogenital syndrome).
  • Go to top

    Clinical vignette 3

    This 37-year-old woman had had acute glomerulonephritis at age 13 which progressed to chronic renal failure. she was hospitalized several times for uremia and anemia and died in severe renal failure. Laboratory studies during life showed low serum Ca and high serum P.

    At autopsy, all parathyroids were enlarged, the aggregate weight being 6 g. Bones of the skull and vertebral bodies showed focal areas of softening. Metastatic calcifications were present in the aortic and mitral valve rings, in coronary, splenic and uterine arteries and in the kidneys.

    Discussion topics

    1. Explain the metabolic relationships between chronic renal failure, vitamin D, and low serum Ca levels in this patient.

    2. Why was there metastatic calcification in this case when serum Ca was low?

    3. In addition to chronic renal disease, what other conditions lead to secondary hyperparathyroidism.
  • Go to top

    Clinical vignette 4

    This 16-year-old female had experienced nervousness, paroxysmal tachycardia and exertional dyspnea for two months. She had lost a moderate amount of weight. One week before admission it was noted that the thyroid was enlarged. Physical examination revealed a diffuse, non-nodular enlargement of the thyroid, lid lag, a fine tremor, tachycardia and an increased pulse pressure. Serum T3 and T4 were elevated. The surgically removed thyroid gland looked beefy red and weighed 45 grams.

    Discussion topics

    1. What is the pathogenesis of Graves disease?

    2. What is the significance of lid lag?

    3. What are the most important complications of thyrotoxicosis?
  • Go to top