QUESTION: 189

A 32-year-old man comes to the office for his annual checkup. He is asymptomatic and his physical exam is normal. He reports that his father died of colon cancer at age 46 and his older brother was recently diagnosed with colon cancer at age 37. His paternal aunt was previously diagnosed and treated for endometrial cancer. He is concerned about his family history of malignancy and wants to discuss cancer screening.

What would be the most appropriate recommendation at this time?

flexible sigmoidoscopy
fecal occult blood testing, with referral for endoscopy if positive
screening colonoscopy
screening colonoscopy starting at age 50
prophylactic colectomy

Answer(s): C

Explanation:

The patient appears to be at risk for hereditaty nonpolyposis colon cancer (HNPCC) or Lynch syndrome. This autosomally dominant inherited cancer predisposition is characterized by colorectal cancer involving at least two generations, with one or more cases being diagnosed before age 50, and patients may have multiple primary cancers (affected women often also have endometrial or ovarian cancer). It is recommended that HNPCC family members undergo screening colonoscopy every two years beginning at age 25. The colon cancers in HNPCC often involve the proximal colon, so flexible sigmoidoscopy would be an insufficient tool for screening the at-risk bowel. HNPCC should be differentiated from familial adenomatous polyposis (FAP), another inherited colon cancer predisposition. This wellstudied and described autosomal dominant inherited condition is much less common than HNPCC. Affected patients develop thousands of adenomatous premalignant polyps, which are generally evenly distributed from cecum to anus and usually become evident between puberty and age 25. Because the polyps are so widespread and evenly distributed, proctosigmoidoscopy is usually a sufficient screening procedure for at- risk family members. When diagnosed with FAP, it is recommended that patients under prophylactic colectomy. If not treated surgically, almost all patients will develop colorectal cancer by age 40. Colonoscopy beginning at age 50 would be recommended for persons at average risk for colon cancer.

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QUESTION: 190

Which of the following risk factors has the strongest association with the development of malignant melanoma?

dark skin and hair color with tendency to tan easily and not to burn easily
personal history of sunburn, especially early in life
pigmented lesion with asymmetric irregular borders, color variegation, and diameter 8 mm
family history of non-melanoma skin cancer
development of actinic keratosis

Answer(s): C

Explanation:

History of excess sun exposure and sunburn early in life is associated with increased incidence of skin cancers, including melanoma, but the highest risk would be the development of a suspicious pigmented lesion. Clinicians can be guided by the "ABCD" rules: asymmetry, irregular borders, color variegation within the same lesion, and diameter >6 mm. Other risk factors for melanoma would include fair skin and hair with tendency to burn easily and a family history of melanoma. Actinic keratoses are premalignant lesions, but can develop into cutaneous squamous cell malignancies, not melanoma.

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QUESTION: 191

A54-year-old man without significant past medical history presents to his primary care physician complaining of epigastric discomfort and early satiety. He subsequently undergoes an endoscopic procedure revealing an ulcerated mucosal lesion. The biopsy of this lesion is interpreted as a well- differentiated lymphoma.

Which of the following statements regarding his treatment and prognosis is most accurate?

His prognosis is poorer than if he were diagnosed with a gastric adenocarcinoma.
This lymphoma is not associated with Helicobacter pylori infection.
Antibiotic therapy may induce regression of the lesion in the majority of cases.
Treatment will not offer curative potential, so he should be referred for hospice care.
Gastric resection is recommended for well-differentiated, bbut not higher grade, lymphomas.

Answer(s): A

Explanation:

Although gastric lymphomas are less common than adenocarcinomas, they are much more treatable with a more favorable prognosis. Gastric lymphomas, especially well-differentiated mucosa-associated lymphoid tissue (MALT), are associated with Helicobacter pylori infection, and antibiotic therapy to eradicate H. pylori has been associated with regression of 75% of such tumors. Higher-grade gastric lymphomas may require chemotherapy with a standard regimen, such as CHOP, and consideration for surgical resection with curative intent.

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QUESTION: 192

A 72-year-old man with a diagnosis of prostate cancer was recently seen in the clinic for restaging and re- evaluation. His bone scan showed development of widespread osseous metastases and his PSA was rising. He was started on leuprolide acetate, a gonadotropin releasing-hormone (GnRH) agonist. He now returns to the clinic complaining of new severe mid-thoracic back pain, which is worse with recumbency and worse with Valsalva maneuver. He also reports that he has a brief but intense electric shock sensation in his lower extremities when he bends over to tie his shoes. On physical exam, he had localized tenderness over the mid-thoracic spine, but his motor strength, sensation, and deep tendon reflexes are all intact.

What is the most appropriate next step?

Obtain an MRI of the thoracic spine.
Refer for neurosurgical evaluation.
Initiate radiation therapy to the affected thoracic spine.
Start the patient on scheduled narcotics for relief of the back pain and follow up in 1 week.
Stop the leuprolide and schedule the patient to return to clinic in 1 week for re-evaluation.

Answer(s): A

Explanation:

The patient has symptoms of spinal cord compression and needs an urgent MRI to establish the diagnosis. Spinal cord compression usually develops when patients have metastases to the vertebral body with epidural extension of the tumor, displacing the underlying thecal sac, and causing cord edema and injury. Patients with cord compression usually experience new or worsening pain symptoms days or weeks before the development of motor weakness below the level of compression. Loss of sensation and loss of bowel or bladder control occur even later. Clues that the pain symptoms may represent cord injury include pain that is worse with recumbency or Valsalva and the occurrence of Lhermitte's sign, an electric sensation down the back and into the extremities with extension or flexion of the neck or spine.

Initiation of therapy, such as radiation therapy or neurosurgical intervention, might be necessary later but would be premature before the diagnosis is established with an imaging study. If the patient's history or physical exam suggests spinal cord compression, initiation of corticosteroids should be started immediately while diagnostic imaging is pending. Pain control with adequate narcotic analgesia is important and may be instituted while the appropriate diagnostic studies are being obtained. Delay of 1 week would be inappropriate due to the urgent nature of the problem and risk of neurological compromise. The patient's neurological status at the time of diagnosis is the most important prognostic factor: 7580% of patients who are ambulatory at the time of diagnosis will retain locomotion. But, if already paraplegic, only 10% will regain the ability to walk. While this patient appeared neurologically intact, the development of neurological deficits can progress over a period of days, making rapid diagnosis and institution of appropriate therapy such as corticosteroids and radiotherapy an urgent consideration. Other factors such as age, presence of co-morbid medical conditions, functional status, and tumor androgensensitivity are important to the patient's overall cancer prognosis.

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QUESTION: 189

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QUESTION: 191

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QUESTION: 192

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