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Clinico-Pathological Conference
Case Study

CPC #1: Tuesday, September 11, 2007
Hurd Hall, The Johns Hopkins Hospital
Neoplasia at 12:00 PM

Radiologist : Stanley Siegelman, MD
Clinical Discussant: Luis Diaz, MD
Pathology Resident: Amy Duffield, MD PhD
Pathologist: Pedram Argani, MD
Moderator: Derek Fine, MD
Chief Complaint

Surgical biopsy and possible resection of anterior mediastinal mass.

History of Present Illness

The patient is a 67 year old African-American man who presented to the Johns Hopkins Hospital for further evaluation of an anterior mediastinal mass. The mass was first noted on chest radiograph approximately one year prior to admission. A fine needle aspiration revealed scant fragments of dense fibrous tissue. The tissue was negative for cytokeratin, actin and S100 protein by immunohistochemistry. Over the next 10 months, there were extensive discussions with the patient about a surgical biopsy and possible excision. Radiologic imaging suggested compression of the heart by the mass. The patient had significant surgical risks and had expressed the wish to not have aggressive surgery if the mass was malignant. After a number of months of discussions, he was admitted for surgical diagnosis and possible resection. It was felt that if the lesion were benign, it could likely be resected. The patient had a significant cardiac and renal history including coronary artery disease status post three myocardial infarctions and a coronary artery bypass graft 3 years prior to admission. He also had end-stage kidney disease due to chronic hypertensive renal disease and diabetes. He was receiving hemodialysis 3x/week. At the time of admission the patient denied chest pain, cough, fever, night sweats, or chills. He has chronic short of breath and dyspnea on exertion.

Past Medical History

End Stage Renal disease
Diabetes Mellitus
Hypertension
Hypertriglyceridemia
Coronary artery disease with extensive three vessel disease
Myocardial infarction (x3)
Coronary artery bypass graft (2002)
Familial adenomatous polyposis, status post total abdominal colectomy (1992)
Repair ventral abdominal hernia (1994)
Hemorrhagic pancreatitis (1994)
Transanal polypectomy (1995 & 1996)
Small bowel obstruction (1994, 1997, 2002)

Family History

The patient's mother has diabetes, hypertension, "heart trouble" and colon polyps. The patient has seven siblings; one of his siblings died of colon cancer in his 30s, and one other sibling has colon polyps.

Social History

The patient is a disabled truck driver who is now working as a minister. He is married and has three children and five foster children. He lives with his wife and his foster children. The patient does not smoke, drink or use any illicit drugs.

Medications

Dialysis every Tuesday, Thursday, Saturday; Hectorol 2.5 mg every Monday, Wednesday, Friday; Lisinopril 5 mg daily, Renagel 800 mg before meals, Protonix 40 mg daily, Plavix 75 mg daily, Lipitor 40 mg daily, Aspirin 81 mg daily, Nifedipine XL 60 mg daily, Toprol-XL 200 mg daily, Motrin as needed.

Allergies

No known drug allergies.

Review of Systems

"Heaviness in the chest" and night sweats since the coronary artery bypass graft in 2002.

Physical Exam on Admission
  • Weight: 199 lbs Height: 73 inches T: 96.2 BP: 174/91 P: 80
  • General: African-American male in no acute distress.
  • HEENT: Sclera anicteric. Extraocular movements intact.
  • CV: Regular rate and rhythm with no murmurs appreciated. Surgical scars from prior chest surgeries noted.
  • Lungs: Clear to auscultation bilaterally. No wheezes, rales or rhonchi.
  • Abdomen: Diffuse tenderness to palpation. Surgical scars from prior abdominal surgeries noted.
  • Lymph Node Exam: Two 0.5 mm, inguinal lymph nodes on the left side, otherwise no lymphadenopathy in the inguinal, axillary or cervical regions.
  • Extremities: No cyanosis, clubbing, or edema. Right arm arteriovenous fistula noted.
Laboratory Values on Transfer
  • Na 137; K 5.1; Cl 100; BUN 55; Cr 8.3; Glucose 112; Calcium 9.4; TP 7.1; Albumin 3.9; TBili 0.3; AST 6; ALT 11; Alk Phos 82
  • WBC 6,470; RBC 4.18; Hemoglobin 12.6; Hct 40.4; MCV 96.7; Platelet 326
  • PT 11.3; APTT 24.5
Radiologic Studies

Chest X-ray 1/20/2005 (approximately year prior to admission) (not shown): Cardiomegaly, evidence of prior anterior chest surgery with possible anterior mediastinal mass, minimal tortuosity and arteriosclerosis of the thoracic aorta.

Clinical Course

The patient was taken to the OR for possible resection of the mass. The mass was found to be compressing the left ventricle and right atrium. It surrounded the right ventricle and was tethered to the diaphragm. During an extensive dissection, the right ventricle was torn and the patient was placed on cardiopulmonary bypass. Unfortunately, even after the mass was removed from the heart and a patch was placed over the right ventricle, the patient could not be taken of bypass due to global left ventricular and right ventricular dysfunction. He expired in the operating room.

Questions

What is your differential diagnosis and most likely diagnosis of the mediastinal mass?

How does the family history and past medical history help with the likely diagnosis?

Serial Radiologic Studies

Images Click on an image below to enlarge.

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