Alveolar
echinococcosis mimicking malignant hepatic
tumor
<Chief
Complaint>
A 28-year-old male with progressive jaundice for
one month
<Brief
History>
A 28-year-old male Tibetan (西藏人) suffered from
poor appetite, malaise, and progressive jaundice for one
month. He also reported to have a weight loss of six kilogram
in two months, associated with The stool was clay-colored
stoool. There was no history of fever, abdominal pain,
diarrhea, or tuberculosis. He denied history of close contact
with neither foxes nor other animals. He was a preacher (喇嘛)
and had traveled to Nepal, India, Singapore, Malaysia, and
Hong Kong in recent six years. He stayed in Taiwan most of the
time in recent two years.
<Physical
Examination>
Physical examination revealed marked icteric
sclera and yellowish skin discoloration of the face, trunk,
and proximal limbs. The heart rate was 84 bpm, temperature was
36.9 ℃, blood pressure was 116/70 mmHg. His breathing sound
was clear and there were no heart murmurs. Palpation of
abdomen disclosed hepatosplenomegaly. There was no palpable
lymphadenopathy of the neck, axilla, and inguinal
areas.
<Laboratory and
Image Study>
1. CBC/DC
WBC |
Hb |
PLT |
Seg |
Mono |
Eos |
Baso |
Lym |
PT |
PTT |
K/μL |
g/dL |
K/μL |
% |
% |
% |
% |
% |
sec |
sec |
8.8 |
12 |
141 |
61 |
5 |
11 |
3 |
20 |
19.2/11.2 |
35.2/33 |
2. Biochemistry
BUN |
Cr |
Na |
K |
Alb |
Globulin |
Glu |
CRP |
mg/dL |
mg/dL |
meq/L |
meq/L |
g/dL |
g/dL |
mg/dL |
mg/dL |
20 |
1.2 |
142 |
3.9 |
4.0 |
3.6 |
102 |
1.4 |
AST |
ALT |
ALP |
r-GT |
Bil (T/D) |
CEA |
CA19-9 |
AFP |
mg/dL |
mg/dL |
meq/L |
meq/L |
mg/dL |
ng/ml |
U/ml |
|
71 |
44 |
1040 |
45 |
22.5/20 |
0.92 |
47.9 |
< 20 |
3. Urine analysis:
Appearance |
Sp.gr |
PH |
Protein |
Glu |
Ketone |
Clear |
1.006 |
6.0 |
-- |
-- |
-- |
OB |
Urobilinogen (EU/dL) |
WBC (/HPF) |
RBC (/HPF) |
Cast (/LPF) |
Crystal (/LPF) |
-- |
1.0 |
0-1 |
0-1 |
-- |
-- |
An abdominal sonography ( Fig. 1
) : a huge heterogeneous and ill-defined hepatic mass ( up to
13 centimeter). The internal echo pattern of the lesion was
inhomogeneous. The mass contained dense calcification with
acoustic shadowing. The intrahepatic bile ducts were dilated.
Stool and bile : no parasite Serology test
for B. abortus and B.melitensis : negative Bile and liver
specimen: AFS: negative; TB
culture : negative ;TB-PCR was negative
CT scan of abdomen ( Figure2A. 2B ):
a huge well-defined lobulated low density soft tissue mass
(measured as 12x12x11cm) with central amorphous, extensive
calcification.
MRCP: a huge hypovascular tumor
causing dilatation of the IHDs. The GB and CBD were collapsed.
T1W images revealed mostly low signal intensity with patches
of slight high signal intensity scattered in the posterior
aspect. The tumor was iso to slightly hyperintense in mild T2
pulse sequence but became hyperintense in heavy T2W image with
scattered cystic changes.
Angiography showed tumor blood
supply from celiac trunk and vascular invasion of right portal
vein, intrahepatic IVC and infra-hepatic
IVC.
<Course and
Treatment >
His prothrombin time (PT) was prolonged (19.2 sec, control
11.2 sec), which was corrected by vitamin K1 administration.
Percutaneous biliary drainage tube was inserted to relieve the
obstructive jaundice. Bile was sent for analysis but no
parasite or eggs were found. Culture for bacteria, fungus and
tuberculosis were all negative. Liver biopsy was performed.
The histology showed necrotic tissue, plasma cell with some
eosinophil infilatration and cholestasis. No parasite could be
identified. Acid-fast stain was negative. Culture of liver
specimen for bacteria, fungus, and tuberculosis were all
negative. He underwent surgical resection of right hepatic
lobe (Figure3).
The postoperative course was uneventful. The histology
revealed multilaminated membrane without scolices, which was
compatible with alveolar echinococcosis of liver (Figure4A.
4B
). Albendazole was given for six
months.
<Analysis>
泡型包蟲病 (alveolar
echinococcosis) 是一種很罕見但致命力卻很高的寄生蟲疾病,它與較常見的囊型包蟲病 (cystic
echinococcosis) 不論在臨床的表現、影像學的特徵、治療的方式或是預後情形方面,都有許多不同之處。囊型包蟲病
(cystic echinococcosis)是由單房性包蟲(Echinococcus
granulosus)感染所導致,而泡型包蟲病 (alveolar
echinococcosis)則是由多房性包蟲(Echinococcus
multilocularis)感染所導致。後者會造成多房性囊腫,也可能會形成實質性腫塊,甚至會有類似惡性肝腫瘤侵犯至血管或附近的器官的表現。我們報告了一個表現類似惡性肝腫瘤之泡型包蟲病(alveolar
echinococcosis)的病例。一位28歲的男性西藏喇嘛,因為一個多月來漸進性黃膽伴隨體重減輕到醫院求診,理學檢查發現肝臟腫大及黃膽,抽血檢查發現嗜酸性白血球升高(
904/mm3),以及肝功能異常(Bil T/D= 22.5/20mg/dL, ALT=44U/L, AST=71U/L,
ALK-P=1040
U/L),腹部超音波發現肝臟右葉有一12x11x11cm之高回音腫瘤,腫瘤內部呈現部分鈣化及壞死的情況,肝內膽管因腫瘤壓迫而造成擴張,同時發現有侵犯至下腔靜脈、部分肝內靜脈、右側肝門脈以及右側腎上腺的情形。電腦斷層檢查呈現低密度腫瘤併部分鈣化,打入顯影劑後腫瘤部位並無顯影。核磁共振檢查在T1-造影下呈現低訊號腫塊,T2-造影下呈現高訊號腫塊,亦含有部分鈣化及少許小的囊腫,腫瘤之血管供應並不豐富。之後病患接受經皮穿肝膽汁引流。糞便及膽汁的檢查及培養沒有特別的發現,血清腫瘤指數並未明顯升高。肝臟切片檢查顯示淋巴球及嗜酸性白血球浸潤及部分肉芽組織及壞死,但並未發現惡性細胞、致病菌或寄生蟲。病人於黃膽改善之後接受右肝切除,病理檢查證實為多房性包蟲(Echinococcus
multilocularis)感染。術後病人復原狀況良好並接受albendazole 400mg bid
治療。泡型包蟲病(alveolar
echinococcosis)在台灣並沒有正式的報告病例,此一病例亦為境外移入之個案,但隨著兩岸之間交流日益頻繁,國內醫師在發現肝臟腫瘤但無惡性腫瘤證據或臨床上有寄生蟲感染之跡象時,必須把多房性包蟲(Echinococcus
multilocularis)感染也列入鑑別診斷之一。
References:
-
Bresson et al. A twenty-year history of
alveolar echinococcosis: analysis of a series of 117
patients from eastern France. Eur J Gastroenterol Hepatol
2000; 12: 327-336.
-
Sasaki F et al. Imaging diagnosis of
alveolar echinococcosis in young patients Pediatr Radiol
1997; 27:63-66
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Eckert et al. Echinococcosis: an emerging or
re-emerging zoonosis? International Journal for Parasitology
2000; 30: 1283 – 1294
-
Romig et al. An epidemiologic survey of
human alveolar echinococcosis in southwestern Germany. Am J
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566-573
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