Case report
A 67-year-old female patient with metastatic pancreatic carcinoma was
admitted to the department of gastro-oncology in our university medical
center for the initiation of palliative chemotherapy. The carcinoma was
first detected by CT and diagnosed by confirmatory histology using
ultrasound guided biopsy two weeks prior to the admission. In addition
to the primary tumor, CT-scans had detected multiple liver metastases
(Figure 1), so palliative chemotherapy with FOLFIRINOX (oxaliplatin,
irinotecan, leucovorin and fluorouracil) was recommended. Initially, the
therapy was planed in an outpatient setting, but due to dyspnea and
progressive thrombocytopenia, the patient was admitted to the hospital.
On admission, the patient reported worsening dyspnea for about two weeks
which now met the criteria of NYHA stage III. Furthermore, she reported
that a deep vein thrombosis of the left lower leg had been diagnosed 10
days before admission. Anticoagulant treatment with apixaban 5mg twice
daily had been initiated, but recently discontinued by her family
physician due to severe thrombocytopenia of so far unknown origin. The
patient had no history of cardiovascular disease. During physical exam,
we did not find typical clinical sings of heart failure or deep vein
thrombosis but petechiae on different sites of the skin on both lower
legs.
Laboratory findings confirmed severe thrombocytopenia and highly
elevated D-dimer levels reflecting a hypercoagulatory state. Given the
severe shortness of breath and the deep vein thrombosis, an immediate
CT-angiogramm of the chest was planned to rule out pulmonary embolism.
In order to apply intravenous contrast medium and after multiple
attempts at inserting a peripheral venous catheter had failed, a central
venous catheter was inserted into the right internal jugular vein.
Following insertion, bedside ultrasound was used for confirmation of the
central venous placement by visualizing bubble artifacts in the right
atrium after injection of agitated saline through the distal
port3.
Unexpectedly, during visualization of the right atrium and right
ventricle in subcostal view, transthoracic echocardiography (using a
Philips Affinity 70 ultrasound system) revealed large masses adherent to
the tricuspid valve leaflets (Figure 2 A/B). The differential diagnosis
included thrombi, bacterial vegetations and metastases. A CT was
obtained immediately and demonstrated a hypodense lesion of 28 x 20 mm
with predominantly intra-ventricular and only a small intra-atrial
proportion (Figure 3). The lesion was irregularly configured and showed
multiple stripy foothills to the ventricle walls. Furthermore, bilateral
segmental and subsegmental pulmonary embolisms were detected.
After an interdisciplinary discussion of the echocardiography and CT
results, we interpreted the tricuspid mass as most consistent with
non-bacterial thrombotic endocarditis. We explained the findings to the
patient and outlined the risks and benefits of initiating the planned
chemotherapy. Specifically, we were concerned that chemotherapy might
destabilize the intracardial mass, causing massive pulmonary embolism On
the other hand, anticoagulative therapy was deemed riskful because of
the marked thrombocytopenia. Finally, not acting at all would allow the
tumor to grow in an uncontrolled manner. The patient opted for the
chemotherapy which was initiated the next day. During the treatment,
several sets of blood cultures as well as serum procalcitonin were
negative indicating no hint for infectious endocarditis. Therapeutic
anticoagulation was not possible due to persistent severe
thrombocytopenia and spontaneous intracutaneous and catheter-related
bleeding.
Several days later, the patient developed arterial thromboses in both
lower extremities with critical ischemia, reflecting an overall deranged
coagulation with parallel hypercoagulation and bleeding disorder
consistent with disseminated intravascular coagulation. We performed a
short time echocardiographic control after the first course of
chemotherapy.
Unfortunately, the masses covering the tricuspid valve leaflets
increased with signs of valve dysfunction and severe tricuspid
insufficiency (Figure 4). Despite severe thrombozytopenia, continuous
heparin and alprostadil were started. Within a few days, the thrombocyte
count reached normal levels, and serum D dimer levels declined.
Naturally, toe necroses did not resolve. The patient was discharged on
low molecular-weight heparin in order to continue palliative
chemotherapy in the outpatient department.