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A patient presents to a physician because of troubling "heartburn" and difficulty swallowing. Esophageal

motility studies demonstrate a near absence of smooth muscle peristalsis and lower esophageal

sphincter tone. No mass lesions are noted and the esophagus is not dilated. Which of the

following findings would most likely also be present?

A. Anemia

B. Atrophic glossitis

C. Hourglass-shaped stomach on barium swallow

D. Massively dilated colon

E. Thick skin

Explanation:

The correct answer is E. Near complete absence of muscle tone and peristalsis is characteristic

of involvement of the esophagus with scleroderma, which causes replacement of muscle by dense

connective tissue. Similar changes in the dermis cause the skin to be thickened. The thickened,

shiny skin of the hands may cause them to resemble claws.

Anemia (choice A) and atrophic glossitis (choice B) are associated with esophageal webs in

Plummer-Vinson syndrome.

An hourglass-shaped stomach within the thoracic cavity (choice C) is a feature of a sliding

hiatal hernia.

A massively dilated esophagus (megaesophagus) can be caused by Chagas' disease, a trypanosomal

disease that can also cause massive dilation of the colon (choice D).

A strict vegetarian is not getting sufficient vitamin D in his diet, and he develops osteomalacia. This

disease is characterized by which of the following changes in his bones?

A. Decreased osteoblasts

B. Increased osteoclast activity

C. Increased osteoid

D. Marrow fibrosis

E. Sparse bony trabeculae

Explanation:

The correct answer is C. The hallmark of osteomalacia is widened osteoid seams. Although the

trabeculae are normal in number and size, they do not mineralize effectively, and the rim of

uncalcified osteoid is much larger than normal.

Osteoclast activity is normal in osteomalacia. Increased osteoclast activity (choice B) is

typical of hyperparathyroidism and Paget's disease of bone.

There are increased numbers of osteoblasts in osteomalacia (compare to choice A), which lay

down increased quantities of osteoid in an effort to strengthen the bone. Unfortunately, the

increased osteoid is not mineralized and the bone remains soft and weak.

The bone marrow space is normal in osteomalacia. Marrow fibrosis (choice D) is more typical of

hematopoietic disorders, although hyperparathyroidism may also lead to bone marrow fibrosis.

Although poorly mineralized, the bony trabeculae are of normal abundance in osteomalacia.

Sparse trabeculae (choice E) is characteristic of osteoporosis.

A 35-year-old woman notices a change in the appearance of a mole on her neck. Physical examination

reveals that the lesion is an irregular, nodular, superficial mass with a variegated

appearance. Biopsy demonstrates a primary malignant tumor. Which of the following factors is

most predictive of the patient's long term prognosis?

A. Circumference of lesion

B. Darkness of lesion

C. Degree of color variation

D. Depth of lesion

E. Sharpness of border between lesion and adjacent skin

Explanation:

The correct answer is D. The lesion is a malignant melanoma. Melanomas can develop either de

novo or in an existing mole. Sunlight exposure is a significant risk factor and fair-skinned

persons are at increased risk of developing melanoma. The most significant factor for long term

prognosis is the depth of the lesion, since the superficial dermis lies about 1 mm under the

skin surface, and penetration to this depth is associated with a much higher incidence of

metastasis than is seen with a more superficial location.

The circumference of the lesion (choice A) is much less important than depth, since one form of

melanoma (superficial spreading) can still have good prognosis despite large size, if it has

not extended to the depth of the superficial dermal lymphatic bed.

The darkness (choice B) or degree of variation in color (choice C) do not have prognostic

significance once melanoma is diagnosed.

Irregularity, or fuzziness at the border (choice E) of a mole-like lesion is a good clue to

potential malignancy, but does not affect prognosis once a melanoma is diagnosed.

A 45-year-old man presents to a physician with flank pain and hypertension. Serum chemistries

demonstrate slightly increased blood urea nitrogen and creatinine. Hematuria is noted on

urinalysis. Ultrasound studies demonstrate markedly enlarged kidneys with irregular margins and

many fluid-filled spaces of varying sizes. Excessive secretion of which of the following

hormones would most likely account for the patient's hypertension?

A. ACTH

B. Cortisol

C. Parathormone

D. Renin

E. Thyroxine

Explanation:

The correct answer is D. The disease is adult polycystic kidney disease, which is an autosomal

dominant condition that typically manifests in middle age. While the kidneys appear very badly

deformed, they function surprisingly well because the cystic spaces actually only affect 10% or

less of the nephrons. Pressure exerted by the cysts can somewhat compromise blood flow to some

glomeruli, which is probably why hypertension, as a consequence of renin secretion, is such a

problem for these patients.

ACTH (choice A) can stimulate cortisol (choice B) secretion, and the cortisol (in high amounts)

can have enough mineralocorticoid activity to cause hypertension. However, this would be more

likely with a pituitary tumor, adrenal tumor, or with exogenous corticosteroid use.

Parathormone (choice C) regulates calcium metabolism and does not usually affect blood

pressure.

Thyroxine excess (choice E) can cause hypertension as a consequence of its effects on the heart

and vasculature; this might be seen in the setting of hyperthyroidism due to thyroid disease.

A 45-year-old woman with a history of thyroid disease presents to her physician with a anterior neck

mass. Biopsy demonstrates non-Hodgkin's lymphoma. Which of the following thyroid conditions

most likely preceded the development of lymphoma in this patient?

A. Follicular thyroid carcinoma

B. Graves disease

C. Hashimoto's thyroiditis

D. Nodular goiter

E. Papillary thyroid carcinoma

Explanation:

The correct answer is C. Hashimoto's disease (autoimmune thyroiditis) confers a 60- to-80 fold

increased risk of developing thyroid lymphoma compared to normal controls. This increased risk

is not surprising, considering the histology exhibited in Hashimoto's, in which sheets of

benign but activated lymphocytes infiltrate and eventually destroy the thyroid gland, producing

eventual thyroid failure.

Neither follicular (choice A) nor papillary (choice E) thyroid carcinomas predispose for

lymphoma.

Lymphoma is not particularly related to Graves disease (choice B) or benign nodular goiter

(choice D).

A patient with intestinal malabsorption is found to markedly improve when flour products (bread,

noodles, etc.) are removed from his diet. At the height of the patient's disease, marked

histologic changes would be seen at which of the following sites?

A. Distal large bowel

B. Distal small bowel

C. Entire large bowel

D. Entire small bowel

E. Proximal small bowel

Explanation:

The correct answer is E. The patient has celiac disease, which is apparently an acquired

hypersensitivity to the gluten (such as gliadin) in wheat. Unlike tropical sprue (which may be

related to enterotoxigenic E. coli infection), which involves the entire small bowel, celiac

sprue is usually limited to the proximal small bowel. This may occur because the gluten

antigens have not yet been digested at this point in their journey through the bowel. A gluten-

free diet usually restores the small bowel mucosa.

Accumulation of which of the following substances indicates aging at a cellular level?

A. Beta-carotene

B. Bilirubin

C. Hemosiderin

D. Lipofuscin

E. Melanin

Explanation:

The correct answer is D. Lipofuscin is a brown pigment that accumulates with aging. It is

believed to be produced from the peroxidation of lipids. Lipofuscin accumulation does not

necessarily impair the ability of the cell to function, and can be found in the hearts and

livers of healthy elderly patients.

Beta-carotene (choice A) is a carotenoid ingested in the diet (found in yellow vegetables such

as squash, pumpkins, and carrots) and converted to vitamin A. Excessive beta-carotene can cause

a benign yellow-orange discoloration of the skin in a condition known as carotenemia.

Bilirubin (choice B) is a pigment derived from the metabolism of the heme group of hemoglobin.

As hemoglobin is broken down, it first forms biliverdin, which is subsequently converted to

bilirubin. Bilirubin can be conjugated (to glucuronide) or unconjugated. The conjugated form

(also called the direct reacting portion) accumulates in biliary obstructions. The unconjugated

form of bilirubin (indirect-reacting) accumulates in hemolytic processes.

Hemosiderin (choice C) is the storage form of iron and stains blue with Prussian blue.

Hemosiderin accumulation from breakdown of red cells is seen in chronic passive congestion of

the lung (inside hemosiderin-laden macrophages called "heart failure cells"). Hemosiderin

deposition is also seen in hemochromatosis, a disorder characterized by abnormal iron storage.

Hemochromatosis is seen in patients with increased iron uptake from the GI tract, and in

patients receiving repeated blood transfusion therapy.

Melanin (choice E) is a brown-black pigment made by melanocytes in the skin. Melanin is also

found in the iris, giving the eye its color. Neuromelanin is a type of melanin found in

catecholamine neurons in the brain.

A 25-year-old man presents to a physician because of multiple small nodules on his lips. The clinician

notes that the patient has a marfanoid habitus. Biopsy of one of these nodules demonstrates a

mucosal neuroma. Which of the following screening tests is indicated?

A. Pentagastrin-stimulated calcitonin

B. Serum gastrin

C. Serum insulin

D. Serum parathyroid hormone

E. Serum vasoactive intestinal peptide

Explanation:

The correct answer is A. The presence of mucosal neuromas, particularly when multiple and when

the patient has a marfanoid syndrome, is a marker for multiple endocrine neoplasia type IIb (MEN

IIb). MEN IIb predisposes for medullary carcinoma of the thyroid and pheochromocytoma.

Pentagastrin-stimulated calcitonin studies may suggest the presence of thyroid C cell

hyperplasia, which may precede frank medullary carcinoma.

High serum gastrin (choice B) suggests pancreatic or duodenal gastrinomas, which may be a

component of MEN I.

High serum insulin (choice C) or vasoactive intestinal peptide (choice E) also suggest

pancreatic endocrine tumors, which may be a component of MEN I.

Parathyroid hyperplasia or adenomas with parathyroid hormone secretion (choice D) can be seen in

MEN I and MEN IIa, but are not part of MEN IIb.

An adult patient presents with persistent headaches. A CT scan of the head demonstrates a 2-cm spherical

mass at the junction of the white and gray matter of the lateral aspect of the cerebral

hemisphere. Which of the following would most likely produce this lesion?

A. Astrocytoma

B. Ependymoma

C. Glioblastoma multiforme

D. Meningioma

E. Metastatic carcinoma

Explanation:

The correct answer is E. 70% of adult brain tumors occur above the tentorium (70% of childhood

tumors occur below the tentorium). Statistically, metastases > astrocytomas (including

glioblastoma) > meningioma > pituitary tumor. Location at the junction of cortical gray and

white matter is also typical for metastatic disease, as is the round shape.

Astrocytomas (choice A) typically arise in the white matter and have an irregular shape.

Ependymomas (choice B) are uncommon and arise from the ependymal lining of the ventricles.

Glioblastoma multiforme (choice C) is an aggressive form of astrocytoma that can cause a

"butterfly lesion", crossing between the cerebral hemispheres.

Meningiomas (choice D) can cause spherical lesions, and are usually located on the surface of

the brain.

A 62-year-old female is evaluated for uterine bleeding. In the course of her workup, an endometrial

biopsy reveals marked endometrial hyperplasia. A left ovarian mass is identified, which appears

to be solid and about 4 cm in diameter on ultrasound. The ovarian tumor is most likely to be

a(n)

A. corpus albicans

B. endometrioid carcinoma

C. endometriotic cyst

D. granulosa cell tumor

E. teratoma

Explanation:

The correct answer is D. Granulosa cell tumors arise from the granulosa cells of the ovarian

follicle. Typically occurring in postmenopausal women, granulosa cell tumors are solid, yellow

masses that frequently produce estrogens. Consequently, granulosa cell tumors often present

with abnormal uterine bleeding, and they are considered a risk factor for endometrial

carcinoma.

Corpora albicantia (choice A) are the fibrotic remnants of corpora lutea. The corpus albicans

is small, usually less than 1 cm, and does not produce an ovarian mass or hormonal changes that

might cause bleeding.

Endometrioid carcinoma (choice B) of the ovary is an epithelial malignancy that resembles

endometrial carcinoma by light microscopy. It is a solid and/or cystic tumor that arises (like

serous or mucinous carcinoma) in the absence of any appreciable hormonal imbalance.

An endometriotic cyst (choice C) is a focus of endometriosis within an organ other than the

uterus. Endometriotic cysts are commonly called "chocolate" cysts due to the appearance of

brown, semi-solid hemorrhage with the lining wall of endometrial glands and stroma.

Endometriotic cysts do not appear solid, and they commonly regress after menopause.

Teratomas (choice E) usually appear solid and cystic on diagnostic studies, due to the multiple

tissue components present within. Most teratomas contain hair and sebaceous material among

other things, but they do not produce estrogens. There are rare cases of teratomas containing

predominantly thyroid tissue or carcinoids that may be hormonally active.

A 79-year-old man complains of pain in the upper portion of his neck on swallowing. He occasionally

regurgitates undigested food shortly after eating. Which of the following is the most likely

etiology of his problems?

A. Mallory-Weiss tears

B. Plummer-Vinson syndrome

C. Schatzki rings

D. Traction diverticula

E. Zenker's diverticulum

Explanation:

The correct answer is E. This is the classic presentation of Zenker's diverticulum, which is a

false diverticulum formed by herniation of the mucosa at a point of weakness at the junction of

the pharynx and esophagus in the posterior hypopharyngeal wall. Zenker's diverticulum is also

associated with halitosis, and if the diverticulum fills completely with food, it can cause

dysphagia or obstruction of the esophagus.

Mallory-Weiss tears (choice A) are mucosal tears at the gastroesophageal junction secondary to

repeated, forceful vomiting. They are often seen in alcoholics.

Plummer-Vinson syndrome (choice B) is the triad of dysphagia (due to esophageal webs in the

upper esophagus), atrophic glossitis, and iron-deficiency anemia.

Schatzki rings (choice C) are mucosal rings found in the distal esophagus at the squamocolumnar

junction.

In contrast to a Zenker's diverticulum, the usually asymptomatic traction diverticula (choice

D) are true diverticula involving all of the layers of the esophagus. They are typically caused

by adherence of the esophagus to a scarred mediastinal structure.

A 72-year-old woman complains of constipation and abdominal pain. Over a period of 48 hours, her

symptoms worsen, and she is transported to the hospital for laparoscopic evaluation of an acute

abdomen. At laparoscopy, a volvulus is noted. Which of the following is the most likely

location for the volvulus?

A. Appendix and cecum

B. Ascending colon

C. Descending colon

D. Sigmoid colon

E. Transverse colon

Explanation:

The correct answer is D. Typically, an elderly patient with a volvulus develops an "acute

abdomen", and is found at laparoscopy or laparotomy to have a twisted (and potentially

infarcted) segment of bowel, usually in the poorly supported sigmoid colon. Most other parts of

the colon are held in place by the posterior peritoneal membrane, and the transverse colon is

stretched so that it can not twist. In contrast, intussusception tends to occur either in