TROUSSEAU SYNDROME AS AN INITIAL

MANIFESTATION OF PANCREATIC

ADENOCARCINOMA: CASE REPORT

A. Kaprelyan1, Al. Tzoukeva

1, M. Atanasova

2,

D. Kalev3, B. Balev

4, R. Georgiev

4

1Dept. of Neurology,

2Clinic of Gastroenterology,

3Clinic of Oncology,

4Dept. of Radiology

Medical University – Varna

Varna, Bulgaria

al_tz@abv.bg

A. Kaprelyan1, Al. Tzoukeva

1, M. Atanasova

2,

D. Kalev3, B. Balev

4, R. Georgiev

4

1Dept. of Neurology,

2Clinic of Gastroenterology,

3Clinic of Oncology,

4Dept. of Radiology

Medical University – Varna

Varna, Bulgaria

al_tz@abv.bg

Abstract—Multiple ischemic cerebral strokes as the

first manifestation of pancreatic carcinoma are infrequent. In

recent times the term Trousseau’s syndrome must be reserved

for unexplained thrombotic problems that precede or appear

concomitantly with visceral malignancy. We report a case of 55-

year-old female with malignancy – related thromboembolism,

presented with multiple and recurrent ischemic strokes, from a

recently diagnosed pancreatic adenocarcinoma in the tail. We conclude that patients with multiple, repeated

cerebral infarction, with minimal risk factors for stroke, must be

further investigate for an underlying malignancy, possibly of

pancreatic origin.

Keywords—Trousseau’syndrome, stroke, malignancy, pancreas

I. Introduction

Multiple ischemic cerebral strokes as the first

manifestation of pancreatic carcinoma are infrequent

[1,7,9,10]. In recent times the term Trousseau’s syndrome

must be reserved for unexplained thrombotic problems that

precede or appear concomitantly with visceral malignancy

[2,6,8,12]. Migratory thromboses, which occurred in about

10% of patients with pancreatic adenocarcinomas, may be

associated with chronic disseminated intravascular

coagulopathy, cancer – related hypercoagulability syndrome,

or tumor embolism [5,13,15,16]. Pancreatic tumor of the body

and the tail tended to grow relatively silently and metastasized

before diagnosis [11].

We reported a case of 55-year-old female with

malignancy – related thromboembolism with multiple and

recurrent ischemic strokes, from a recently diagnosed

pancreatic adenocarcinoma in the tail.

II. Case report

A 55-year-old female, previously healthy, presented

with sudden-onset speech difficulties, dizziness, and right-

sided leg weakness resulting in unsteady gait. Prior to

admission to neurology clinic, a medical consult with

gastroenterologist was done due to intermittent epigastric pain,

noted after meal. She had normal vital signs and was fully

awake, conversant with Glasgow Coma Scale (GCS) at 20.

Neurological examination revealed partial sensory and motor

aphasia, right-sided latent lower monoparesis, and hemi-

hyperreflexia. The patient did not have any cranial nerves

dysfunction, sensory deficit or coordination disorder.

Laboratory data demonstrated anemia (Hb 116 g/l) and

elevation of erythrocyte sedimentation rate 40 mm,

Leucocytes 16.2 g/l, blood glucose 7.1 mmol/l, ASAT 49 U/l,

ALAT 40 U/I, CRP 176 nmol/l, tumor markers: CEA - 328

ng/ml, CA 15-3 - 91.3 U/ml, CA 19-9 >1000 U/ml, CA

125(OV) >500 U/ml, proteinuria and hematouria.

Hypercoagulability studies were negative, coagulation factors,

and D – dimers were within normal.

Fig.1 Non-enhancing hypodense area (12.5x11 mm, 25 HU) in the left parietal

and occipital lobes, and second one in the left parietal lobe.

Later on brain CT (25.09.2012) revealed one

hypodense area (44x22 mm, 29 HU) in the left parietal lobe

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and second one located near the posterior corn of the left

lateral ventricle (fig.2).

Fig.2. a hypodense zone left parietal 44/42mm, with mean density 29 HU,

acute ischaemic insult; b small hypodense zone 9mm left parietal above the

posterior horn of the left ventricle-subacute ischemic insult

One week later the patient demonstrated progression of

her neurologic deficit. Brain magnetic resonance imaging

(MRI) (28.09.2012) revealed large infarction localized in the

left temporal and parietal lobes, multiple punctiform areas in

the right parietal lobe, both cerebellar hemispheres, and corpus

callosum, as well as two encephalomalatic areas with perifocal

gliosis in the right frontal and temporal lobes. MRI data

confirmed multiple acute embolic ischemic strokes and two

old ischemic cerebrovascular accidents (fig.3).

Fig.3.a AX T2 FLAI- multiple hyperintense zones right parietal and left parieto-temporal, which present multiple acute ischemic insults; b DWI-

multiple hyperintense zones right parietal, left parieto-temporal, with diffusion

restriction, due to multiple ischemic insults; c ADC map, these lesions are correspondingly hypointense on ADC map-acute ischemic insults

Fundoscopic examination discovered findings of

hypertonic retinal angiopathy. Transcranial Doppler

utrasonography was normal. Trans-thoracic echocardiography

was normal. Chest findings were normal. Enhanced abdominal

CT (26.09.2012) demonstrated multiple, different sized oval-

shaped metastatic lesions in the both liver lobes, the biggest

one 60x50 mm, one hypodense area in the upper pole of the

spleen, and necrotic tumor mass (50x39x25) located in the

pancreatic tail (fig.4).

Fig. 4 Contrast-enhanced CT of abdomen shows hypodense zone in the

pancreas tail – Tu –carcinoma; multiple hypodense zones in the liver, and one hypodense area in the spleen upper pole – meta

Fine-needle aspiration liver biopsy (27.09.2012)

detected cytological findings of metastases from low-

differentiated adenocarcinoma. The consult with oncologist

and abdominal surgeon confirmed data relevant to advanced

stage IV adenocarcinoma of pancreatic tail with liver and

spleen metastases.

We diagnosed Trousseau’s syndrome accompanying

pancreatic cancer and performed anticoagulation (Heparin)

and antiplatelet (Clopidogrel and Aspirin) treatment. Brain CT

monitoring (18.10.2012) revealed hemorrhagic transformation

in the previous ischemic areas (fig.5).

Fig.5 a hemorrhagic transformation, hyperdense linear foci, in the zones of the known insults; b hemorrhagic transformation, hyperdense linear foci right

parietal in the new discovered insult.

The patient died of cerebral edema 1 month later.

III. Discussion

In 1865, Armand Trousseau (1801 – 1865) described

that some patients presented with unexpected, unusual, or

migratory thrombosis, later or concomitantly manifested a

visceral malignancy [2,3,8,12,13,14]. In review of the findings

– our patient suffered from multiple bihemispheric progressive

ischemic stroke. Atherosclerosis, hypertension, diabetes

mellitus, or nonneoplastic hypercoagulable states appear to be

conventional risk factor for cerebrovascular disease, but she

was not known hypertensive and/or diabetic. Ischemic stroke

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with no overt vascular risk factors should be considered for

cancer screening [1,4,5,6]. For this patient CA19-9 was

extremely high and a large silent necrotic mass in pancreatic

tail were seen on abdominal CT. The combination of

radiology with a best serum tumor marker for pancreatico-

billiary cancer CA19-9, significantly increases the diagnosis

sensitivity to 97.2% and the specificity to 88.7% [7]. The

presented patient has necrotic stage IV adenocarcinoma of

pancreatic tail with hepatic and splenic metastases and

multiple acute cerebral ishemic stroke as the first symptom,

wich is very rare. Adenocarcinoma of pancreatic tail often

grows silently and the most common clinical manifestation is

glucose tolerance disorder [11]. The underlying

pathophysiological mechanisms for the presence of the

cerebrovascular diseases, due to the pancreatic cancer itself,

are unclear [9,10,15,16]. For this patient, except the elevated

serum pancreatic carcinoma antigen and mild hepatic

pathology, the coagulation factors and all of the rest blood

tests were within normal limits. Ultrasonographic duplex

study, trans–thoracic echocardiography and chest findings

were normal. We speculate that in our case the cause of the

multiple cerebral infarctions may have been due to migratory

arterial tumor emboli from necrotic pancreatic cancer.

IV. Conclusion

We conclude that patients with multiple, repeated cerebral infarction, with minimal risk factors for stroke, must be further investigate for an underlying malignancy, possibly of pancreatic origin.

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