1.
A 57-y/o man is brought to the emergencydepartment because of blurry vision, difficulty standing, and mental confusion. On physical examination the patient appears malnourished, he had diffuse crackles bilaterally, and on standing his gait is wide-based and unsteady. The patient’s friend, who accompanies him, says the patient “hasn’t been himself lately,” and is having difficulty remembering to do everyday tasks. Which neuropathologic findings are most consistent with this patient’s symptoms?
A. 
Atrophy of the caudate nucleus
B. 
Depigmentation within the substantia nigra
pars compacta
C. 
Neurofi brillary tangles and widening of
ventricles
D. 
No neuropathologic fi ndings
E. 
Symmetric lesions in the paraventricular
regions of the thalamus and hypothalamus,
mammillary bodies, and periaqueductal
region of the midbrain
2.
A 41-y/o man visits his doctor because of increasingly painful headaches. CT of the head is shown in the image. If a biopsy of this tumor were obtained, what would the pathologist likely see under the microscope
A. 
Densely packed cells with halos of cytoplasm
surrounding large round nuclei
B. 
Perivascular pseudorosettes with tumor
cells surrounding vessels
C. 
Pseudopalisading tumor cells surrounding
necrotic regions
D. 
Sharply demarcated areas of tumor cells
located at the grey-white matter junction
E. 
Whorled pattern of concentrically arranged
spindle cells with psammoma bodies
3.
After a 63-y/o man with a progressive, chronic movement disorder dies, the family requests an autopsy. On gross inspection of the patient’s brain, the pathologist notes the presence of a deep brain stimulator electrode. The pathologist then obtains a tissue specimen of the basal ganglia for histologic analysis (see image) that stains positive for α-synuclein. From which disease did the decedent most likely suffer?
A. 
B. 
C. 
D. 
E. 
4.
A newborn girl is diagnosed as dysmorphic by a pediatrician in the newborn nursery. On physical examination the girl has a broad neck, wide-spaced nipples, and a systolic ejection murmur. An echo-cardiogram is performed and demonstrates coarctation of the aorta. The echocardiography technologist also runs his transducer across the patient’s abdomen and notices a renal abnormality associated with this patient’s syndrome. The most likely observed renal abnormality increases this patient’s risk for developing which disease?
A. 
B. 
C. 
Transitional cell carcinoma
D. 
E. 
5.
A 65-y/o man presents to the office with complaints regarding his urine. He states that he has recently had bloody urine, but does not have any urinary pain, hesitation, dribbling, or increased frequency. He also says he has lost 4.5 kg over the past 2 months. A biopsy of the patient’s bladder wall is shown in the image. Which of the following risk factors has the strongest association with this patient’s disease?
A. 
History of aniline dye exposure
B. 
History of cyclophosphamide treatment
C. 
History of heavy cigarette smoking
D. 
History of pelvic irradiation
E. 
History of schistosomiasis infection
6.
A 2-year-old girl is brought to the ED because of fevers that have been occurring daily for 3 weeks. Physical examination reveals a 1.2-kg (2.6-lb) weight loss since her last doctor’s visit 1 month earlier. She also has pallor, hepatomegaly, and splenomegaly. She is admitted to the hospital and undergoes bone marrow aspiration; results are shown in the image. Which of the following is the most likely diagnosis?
A. 
Acute lymphoblastic leukemia
B. 
C. 
Hemophagocytic lymphohistiocytosis
D. 
E. 
7.
Following a course of chemotherapy, a 5-y/o male oncology patient develops altered mental status, tachycardia, decreased blood pressure, flushing of the extremities, and decreased urine output. After 48 hours in the pediatric intensive care unit, the patient dies from complications secondary to overwhelming gram-negative sepsis. At autopsy, the pathologist notes the abnormal appearance of the decedent’s kidneys, as shown in the image. What process most likely caused the findings demonstrated in the image?
A. 
B. 
Diffuse cortical necrosis
C. 
D. 
E. 
8.
A 26-year-old woman visits her physician with complaints of vaginal bleeding after sexual intercourse. She started menses at age 14 years and has 32-day cycles. She acknowledges having unprotected sex with multiple partners. Cytologic specimens are taken from the cervix and vagina. On microscopy, cervical cells have large nuclei with open chromatin; several cells have mitotic figures. What would most likely be present in the specimens that account for these findings?
A. 
Double-stranded DNA virus
B. 
C. 
D. 
E. 
Squamous cells covered with bacteria
9.
A 36-year-old woman with a family history of hereditary nonpolyposis colon cancer (HNPCC) presents to her gastroenterologist for her annual examination. Because of a strong family history of colon cancer, she undergoes yearly colonoscopy. However, she has shown no signs and symptoms of HNPCC and has no significant past medical history. Other than colorectal carcinoma, what is another condition for which she is at increased risk given a family history of HNPCC?
A. 
Carcinoma of the endometrium
B. 
Cholelithiasis causing cholecystitis
C. 
Melanin spots of the buccal mucosa
D. 
Pseudopolyps of the small or large bowel
E. 
Vitamin B12 defi ciency due to malabsorption
10.
A 35-year-old woman presents with dull, persistent flank and abdominal pain, polyuria, nocturia, and frequent urinary tract infections. Physical examination is notable for blood pressure of 150/90 mm Hg and multiple bilateral abdominal masses. Urinalysis is notable for microscopic hematuria and 1+ protein. CT of the abdomen reveals multiple cysts in the kidneys. The patient notes that she was adopted and knows nothing about her family medical history. What is the most likely etiology of this patient’s illness?
A. 
A mutation on chromosome 3
B. 
A mutation on chromosome 6
C. 
A mutation on chromosome 9
D. 
A mutation on chromosome 16
11.
A 3-year-old developmentally delayed girl presents to the pediatric neurologist for evaluation of new onset seizures. The parents are also concerned because the child frequently exhibits inappropriate outbursts of laughter. Physical examination is signifi cant for abnormal facies marked by microcephaly, deep-set eyes, and a large mouth with a protruding tongue. The child’s gait is unstable. The most likely diagnosis is an example of which of the following genetic phenomena?
A. 
B. 
C. 
D. 
E. 
12.
A mother brings her 5-year-old son to his pediatrician’s office. The boy has been experiencing frequent falls as well as progressive difficulty with walking, jumping, and hopping. Laboratory testing reveals a creatine kinase level of 2840 U/L. Muscle biopsy reveals variation in fi ber diameter, an increased number of internalized nuclei, and muscle fiber degeneration and regeneration. Western blot analysis will most likely reveal the complete absence of which of the following?
A. 
Cystic fi brosis transmembrane conductance
regulator
B. 
C. 
Phenylalanine hydroxylase
D. 
E. 
13.
A 55-year-old recent immigrant from Taiwan presents to the clinic with a 3-month history of worsening nasal congestion, epistaxis, and recurrent ear infections. Physical examination reveals painless fi rm lymph node enlargement in the neck. CT of the head reveal a large mass situated in the upper nasopharynx. Biopsy of the lesion shows large epithelioid cells intermixed with numerous infi ltrating lymphocytes.
The infectious agent directly associated with this patient’s pathology is best described by which category?
A. 
B. 
C. 
D. 
E. 
14.
A 65-year-old male immigrant from Africa presents to the emergency department after an episode of gross hematuria. He states that he has seen small amounts of blood in his urine from time to time over the past several months. His physical examination is remarkable only for mild hepatosplenomegaly. A urology consult is called, and the urologist performs a bedside cystoscopy. A large fungating mass is seen adherent to the superior part of the bladder. Results of a biopsy are shown in the image.
What is the most likely environmental exposure associated with this disease in the patient?
A. 
B. 
C. 
D. 
Infection with a papovavirus
E. 
Long-term indwelling catheter
15.
A patient with AIDS and a CD4+ cell count <50/mm3 is suffering from an infection that affects his lungs, eyes, gastrointestinal tract, and central nervous system. Results of a biopsy are shown in the image. With what is the patient most likely infected?
A. 
B. 
C. 
D. 
E. 
F. 
16.
A 66-year-old woman has an autosomal recessive disease with multiple sequelae, including diabetes mellitus and arthritis. Physical examination reveals hepatomegaly and skin hyperpigmentation. A biopsy of her liver is shown in the image. What is the most likely explanation for her health problems?
A. 
Chronic ingestion of alcohol
B. 
Genetic defi ciency in the synthesis of
β-globin chains
C. 
Inappropriately high iron absorption
D. 
Mutation in RBC membrane protein
17.
A 37-year-old HIV-positive man presents for evaluation of anogenital lesions. He states that the lesions have been present for years, but have recently grown in size and become pruritic and tender. On examination he is circumcised and has multiple hyperkeratotic papules on his penis shaft, perineum, and anal area. He also has a palpable rectal mass with guaiacpositive stool and conjunctival pallor. On further questioning, he admits to recent unintentional weight loss, constipation, and bloating. His CD4+ cell count is 150/mm3 and his hematocrit is 26%. CT scan of the abdomen shows a 3 × 4-cm rectal mass with multiplemetastatic lesions in his liver.
What tumor-suppressor protein is targeted by the virus causing this patient’s rectal cancer?
A. 
B. 
C. 
D. 
E. 
18.
A 5-year-old boy presents with an unsteady gait and severe vertigo and nausea. A brain lesion is seen on CT scan; a biopsy of the lesion is shown in the image. Where in the brain is the patient's lesion?
A. 
B. 
Intermediate section of the cerebellar
hemisphere
C. 
Lateral section of the cerebellar hemisphere
D. 
E. 
Postcentral gyrus of the parietal lobe
19.
A 64-year-old retired shipyard worker has been experiencing shortness of breath, a cough, and chest pain for 5 months. In that time he has lost 14.5 kg (32 lb). He develops progressive ascites, and ultimately dies due to a pulmonary embolus. Autopsy results are shown in the image. Exposure to which substance is a risk factor for this patient’s disorder?
A. 
B. 
C. 
D. 
E. 
20.
A 59-year-old man is hospitalized after suffering
a severe myocardial infarction. He is initially
treated with nitrates, β-blockers, and aspirin,
and subsequently undergoes cardiac
catheterization with placement of two stents.
Following the procedure he is hemodynamically
stable without recurrence of chest pain.
However, 5 days after admission his heart rate
is 134/min, blood pressure is 72/35 mm Hg,
and respiratory rate is 29/min. Physical examination
reveals distant heart sounds and an elevated
jugular venous pressure. Which of the
following complications is most likely causing
this patient’s symptoms?
A. 
B. 
C. 
D. 
Rupture of the papillary muscle
E. 
Rupture of the ventricular free wall