A 19-year-old man presents with multiple lymph nodes in his neck. He reports no current infectious symptoms, and his monospot test is negative. On examination, he has blanching skin lesions on his face and conjunctivae, multiple large lymph nodes in his neck, and poor coordination when tested by tandem walking. He has past history of poor coordination and walking difficulty as well as recurrent sinopulmonary infections. A biopsy of the lymph node is positive for lymphoma. He is diagnosed with a hereditary disorder that is autosomal dominant, and is associated with defective deoxyribonucleic acid (DNA) repair mechanisms. Which of the following is the most likely diagnosis?
Ataxia-telangiectasia is inherited in an autosomal recessive manner. It is associated with non-Hodgkin’s lymphoma, acute lymphocytic leukemia, and stomach cancer. Associated immunoglobulin A (IgA) (± immunoglobulin E [IgE]) deficiency predisposes to infection as well. All the other conditions listed are inherited in an autosomal dominant manner, and a positive family history is much more likely
A 33-year-old male immigrant from Taiwan presents with increasing right upper quadrant (RUQ) pain. The pain is dull, and it does not radiate or change with eating. On examination the abdomen is soft, there is a mass in the RUQ, and no ascites is clinically detected. He has aprior history of hepatitis B. His laboratory investigations reveal hepatitis B surface antigen (HBsAg) positive, hepatitis B surface antibody (HBsAb) negative, aspartate amino transferase (AST) 60 U/L, alanine amino transferase (ALT) 72 U/L, and an elevated alpha-fetoprotein level. Which of the following is the most likely diagnosis?
Ataxia-telangiectasia is inherited in an autosomal recessive manner. It is associated with non-Hodgkin’s lymphoma, acute lymphocytic leukemia, and stomach cancer. Associated immunoglobulin A (IgA) (± immunoglobulin E [IgE]) deficiency predisposes to infection as well. All the other conditions listed are inherited in an autosomal dominant manner, and a positive family history is much more likely
A 42-year-old man received radiation exposure at a nuclear power plant in Eastern Europe. He is now concerned about his future cancer risk. Which of the following statements is correct?
Radiation-induced malignancies tend to occur at the age where that particular malignancy would normally occur. Therefore, the latency period can be 40 years or more. The latency period tends to be shortest (5–7 years) for leukemia. The risk for most malignancies is greatest with early-life radiation, and evidence suggests that therapeutic radiation confers excess risk as well. The amount of exposure determines the likelihood of developing malignancy, not its severity
A 25-year-old woman has recently moved to a new city. She comes today for her first routine visit and annual physical assessment. She asks you about screening tests for cancer at her age. Which of the following cancer screening tests are recommended for her age group?
There is universal agreement on the need for regular Pap smears in young women. There is no need to screen for colon cancer (fecal occult blood) or lung tumors (CXR), particularly at this age. Mammography, if indicated for screening, would be only for older women. Many authorities recommend breast selfexamination as well as physical examination by a physician.
A 25-year-old woman presents with intermittent double vision and fatigue. Her symptoms are worse at the end of the day. She reports no other focal muscle weakness or sensory symptoms. On examination her eye movements, motor strength, and reflexes in the upper and lower limbs are normal. Repetitive handgrip exercises cause loss of strength in the grip. ACXR reveals an anterior mediastinal mass. Which of the following is the most likely diagnosis of the anterior mediastinal mass?
All of the given responses are potential anterior mediastinal masses, but when an anterior mediastinal mass presents with myasthenia gravis the diagnosis of thymoma is essentially established. Myasthenia gravis occurs as a paraneoplastic process of the thymoma. The ocular and muscle fatigue symptoms are characteristic of myasthenia. About 5–10% of patients with thymoma will also have hypogammaglobulinemia. About 5% of patients with thymoma will have autoimmune pure red cell aplasia.
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A 64-year-old man presents with symptoms of difficulty swallowing and weight loss of 10 lb. He has no prior history of heartburn, stomach ulcers, or difficulty swallowing. He smokes one pack a day for the past 45 years and drinks approximately 5 oz of alcohol a day. He is thin appearing, there are no oral lesions, and the remaining examination is normal. Esophagoscopy reveals a midesophageal narrowing with ragged ulcerating, and biopsies are taken. Which of the following is the most likely diagnosis?
The history of weight loss and dysphagia suggests carcinoma of the esophagus, and squamous cell carcinoma is a disease that occurs in older men who drink and smoke heavily. Smoking and or excessive drinking are considered etiologic factors in the development of squamous cell carcinoma. Adenocarcinomas arise within dysplastic columnar epithelium in the distal esophagus, usually in the presence of chronic gastric reflux.
A 62-year-old man presents with dark tarry stools and light-headedness. Upper endoscopy finds an ulcerating lesion in his stomach and biopsies confirm gastric cancer. Which of the following is a risk factor for carcinoma of the stomach?
Low dietary vitamin C, and high salt and nitrate consumption predispose to gastric cancer, as does ingestion of smoked foods. Smoking is a risk factor, but alcohol is not.H. pylori is another important risk factor, since it causes gastritis that eventually leads to chronic atrophic gastritis, metaplasia, dysplasia, and then carcinoma.
A young man with leukemia is treated with methotrexate. Which of the following is the mechanism of action of this drug?
The most likely mode of action of methotrexate is by tightly binding dihydrofolate reductase (DHFR), which maintains the intracellular folate pool in its fully reduced form as tetrahydrofolates. These compounds are required in the de novo synthesis of pyrimidines and purines.
A 52-year-old man presents with abdominal pain and weight loss. He describes a dull “gnawing” pain located in the epigastric region radiating to the back. The abdominal examination is normal with no hepatosplenomegaly or masses palpated. A computerized tomography (CT) scan of the abdomen reveals a 3-cm mass in the pancreas. Which of the following statements regarding cancer of the pancreas is true?
Adenocarcinoma is the most common cancer of the pancreas. The proximal pancreas is the most common site, with only 20% occurring in the body and 5–10% in the tail. About 95% of the tumors arise from the exocrine portion of the gland, and these are usually malignant. Most of the endocrine tumors are benign. Early development of metastases is characteristic of pancreatic adenocarcinoma
A73-year-old man was born in Taiwan and came to the United States 3 years ago. He is known to be HBsAg positive. Which of the following findings suggests the development of HCC?
For most patients, the development of HCC is the first manifestation of their underlying liver disease. The most common presentation is with RUQ pain, mass, and weight loss, but hepatic decompensation with jaundice and ascites is also common. About 25% of patients have hepatic bruits.
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A 47-year-old man presents with dark black stools and vague crampy abdominal pain. On examination he is pale, blood pressure 100/70 mm Hg, pulse 110/min, and the abdomen is soft and nontender. Rectal examination confirms melena, and the patient is transfused 2 units of packed red blood cells. Upper endoscopy does not identify the source of bleeding, so a small bowel barium study is ordered. It reveals a small bowel tumor. Which of the following statements concerning small bowel tumors is correct?
Adenocarcinoma, the most common malignancy of the small bowel, is most common proximally, particularly in the duodenum. Small bowel lymphomas are most common in the ileum, but the stomach is the most common site of GI lymphoma. Carcinoids usually present with local symptoms. Carcinoid syndrome is present only with hepatic metastases. Benign tumors bleed more frequently than malignant ones. Malignant adenocarcinomas can occur in Peutz-Jeghers syndrome.
A 58-year-old man is newly diagnosed with colon cancer and undergoes surgical resection of the tumor. The pathology result reports no lymph node involvement, the lesion is 4 cm in size and involves the muscularis layer. His preoperative evaluation for distal metastases was negative. Which of the following factors is most important in predicting prognosis and survival in patients with colon cancer?
Depth of tumor penetration (Dukes stage) is the most important prognostic factor in the 5-year survival in patients with colon cancer. Rectal bleeding is a good prognostic sign, perhaps because surface erosion manifests early. Young age, male gender, and location in the rectum all indicate a poorer prognosis. Unlikemost tumors, no correlation with tumor size and prognosis has been established for colon cancer.
A 64-year-old woman presents with abnormal vaginal discharge and postmenopausal bleeding. Pelvic examination reveals a bulky uterus and no adnexal masses. She undergoes a dilation and curettage (D&C), and the pathology is positive for adenocarcinoma. Which of the following is correct about carcinoma of the uterine endometrium?
Please select 2 correct answers
Carcinoma of the cervix is common in young women. For endometrial cancer, the peak is from age 55 to 60. Endometrial cancer is associated with nulliparity, diabetes mellitus, and obesity. Cervical cancer is associated with human papilloma virus (HPV) infection. There is tremendous variation in incidence of cervical cancer based on geography, ethnicity, and sexual history.
You are seeing a 62-year-old woman with a family history of breast cancer. She is worried about her future risk of breast cancer, since her mother died of breast cancer at the age of 63. Which of the following features is also a recognized risk factor for breast cancer?
Breast cancer risk is reduced by 20% for each year that menarche is delayed. Early menopause, natural or surgical, also decreases risk. Early (age 18 or 19) full-term pregnancy and multiparity decrease the risk. Radiation exposure is a risk factor primarily in adolescence and is marginal after the age of 40. In summary, there are three important dates in the assessing the risk of breast cancer: age of menarche, age of first full-term pregnancy, and age of menopause.
A 67-year-old man complains of shortness of breath on exertion and right-sided chest pain. The pain is constant in nature; he has no fever or chills and no sputum production. On examination, air entry to the left lower lobe is reduced and the area is dull on percussion. The CXR reveals left lower lobe pleural effusion, pleural thickening with calcification (plaques), and lower lobe fibrosis. Further history from the patient is significant in that he is a lifetime nonsmoker, and that he worked as a pipe fitter until retiring 2 years ago. Which of the following is the most likely diagnosis?
The history of being a pipe fitter suggests asbestos exposure, and the CXR finding of pleural plaques and lower lobe fibrosis confirms prior asbestos exposure. The classic associated cancer is mesothelioma. However, in 30–50% of cases, no history of asbestos exposure is apparent. The average age on presentation is 60, and this is typically many years after the exposure. The most common presenting symptoms are dyspnea and nonpleuritic chest pain. Smoking injury in patients with asbestos exposure increases the risk of other lung cancers such as adenocarcinoma or squamous cell carcinoma.
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A 23-year-old notices left leg pain after returning from a ski trip. The pain persists for 2 months, and he presents for medical evaluation after noticing a lump on his calf. On examination, there is a 2-cm nonmobile mass in his anterior shin. A magnetic resonance imaging (MRI) scan suggests the lesion is arising from the tibia, and a biopsy reveals bone sarcoma. Which of the following statements about bone sarcoma is correct?
Overwhelmingly, the major prognostic factor in osteosarcoma is location of the tumor. Pelvic and axial lesions do worse than those in the extremities, and survival is better in tibial tumors than femoral tumors. Lung metastases are very common.
A 19-year-old woman notices axillary lymphadenopathy and presents for evaluation. She has noticed the lymph nodes for over a month. She has no fever, chills, weight loss, or night sweats. On examination, she has multiple mobile nontender lymph nodes in both axilla, and no cervical or inguinal lymph nodes. No spleen is palpable, and the rest of the examination is normal. A monospot test is negative, and a complete blood count (CBC) and biochemistry are normal. A biopsy of one lymph node reveals Hodgkin’s disease (HD), nodular sclerosing variety. A bone marrow biopsy is arranged. Which of the following tests is also part of the routine staging evaluation for patients with HD?
CT imaging of the chest, abdomen, and pelvis is part of the staging workup for patientswith HD. Other components of staging include CBC, lytes, LDH, CXR, and bone marrow biopsy. PET and gallium scanning are not always done, and are usually helpful at the completion of treatment to document remission. The purpose of staging laparotomy is to determine whether radiation alone will be used for treatment. As chemotherapy usage increases, the necessity for staging laparotomy decreases
A 23-year-old man is newly diagnosed with Hodgkin’s lymphoma. Which of the following is the most likely presenting symptom of this type of lymphoma?
Please select 2 correct answers
The most characteristic presentation of Hodgkin’s disease is that of enlarged, superficial cervical or supraclavicular lymph nodes in a young person. The nodes are usually freely moveable, nontender, and not painful. Occult presentation with intrathoracic or intraabdominal disease is unusual.
A 68-year-old man presents with left axillary adenopathy that on biopsy reveals a low-grade lymphocytic lymphoma. Which of the following statements is correct?
About 85% of low-grade lymphocytic lymphomas are widespread at the time of diagnosis. However, staging is still important as radiation therapy can be curative for localized (stage I, II) disease. Because the prognosis for this malignancy is measured in years, it has been difficult to demonstrate a survival benefit for aggressive chemotherapy. The poor prognosis for lymphoma in older patients might be a result of less-aggressive therapy.
A 63-year-old man presents with a new skin rash on his chest. The lesions are well-defined red-colored plaques. A skin biopsy of the lesion reveals lymphoma. Further diagnostic evaluation does not reveal any evidence of a visceral malignancy. Which of the following characteristics about this disease is correct?
Cutaneous lymphomas are of T-cell origin and are more common in other parts of the world, such as Japan. Patients with adult T-cell lymphoma-leukemia (ATLL) have acute fulminant courses characterized by skin invasion and leukemic cells. This syndrome is clearly related to human T-cell lymphotropic virus-I (HTLV-I), and there is a possibility that HTLV-I, or another retrovirus, might be the agent for mycosis fungoides and Sézary syndrome. ATLL responds poorly to treatment and therapy for the lowgrade malignancies controls symptoms but does not result in cure.
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