Cases from the Osler medical service at Johns Hopkins University

Abstract

Presenting features A 45-year-old African American woman with a history of idiopathic cardiomyopathy, heart failure, hypertension, substance abuse, and multiple abdominal masses was admitted to the Osler medical service with 3 weeks of increasing shortness of breath, worsening dyspnea on exertion, increasing weight, increased lower extremity edema, and abdominal distention. The patient had an episode of chest tightness for 5 minutes on the day before presentation. The pain did not radiate and was not associated with nausea or diaphoresis. Medications on admission included furosemide for the week before admission; however, the patient had not been taking any of her prescribed medications for 4 to 5 months before admission. Aside from increasing abdominal distention, the patient had no abdominal complaints and denied any constitutional symptoms. One year before admission, during evaluation of hypertension, a computed tomographic scan and ultrasound demonstrated uterine fibroids. These studies showed multiple large uterine leiomyomata with the possibility of peritoneal carcinomatosis. The patient did not follow up for further outpatient evaluation. The patient was afebrile on presentation. She had a heart rate of 98 beats per minute, blood pressure of 178/124 mm Hg, a respiratory rate of 16 breaths per minute, and an oxygen saturation of 95% on room air. She was in mild respiratory distress and appeared well nourished. Jugular venous pulsation was present at the angle of the mandible, and further neck examination revealed “shotty” cervical lymphadenopathy and a firm, mobile left supraclavicular lymph node approximately 0.5 cm in diameter. Chest examination revealed bibasilar crackles with occasional wheezes. Cardiovascular examination revealed a regular rate and rhythm, a II/VI systolic murmur heard best at the left lower sternal border, and an S 3. Abdominal examination revealed a firm, distended abdomen with active bowel sounds. Multiple, firm, nontender masses ranging from 2 cm to 6 cm in diameter were easily palpable. An umbilical hernia was present, and 3+ pitting edema was noted bilaterally in the lower extremities. Laboratory values were notable for a white blood cell count of 6800 × 10 3/μL, a hemoglobin level of 11.8 g/dL, and a negative result for urine human chorionic gonadotropin. Tumor markers during the initial evaluation 1 year previously showed a carcinoembryonic antigen level of 3.3 ng/mL (reference, 0 to 5.0 ng/mL), a carbohydrate antigen (CA) 19-9 level of 45.4 U/mL (reference, 0 to 36.0 U/mL), and a cancer antigen (CA)-125 level of 13 U/mL (reference, 0 to 35.0 U/mL). Repeat markers drawn in on admission showed a carcinoembryonic antigen level of 4.1 ng/mL, a CA 19-9 level of 127.3 U/mL, and a CA-125 level of 50 U/mL. The patient was admitted for an exacerbation of heart failure in the setting of hypertensive urgency. After management of her hypertension and heart failure, she underwent computed tomographic imaging of the chest, abdomen, and pelvis ( Figure 1) and an ultrasound-guided percutaneous biopsy of the largest periumbilical mass ( Figure 2). What is the diagnosis?