Alveolar echinococcosis localized in the liver, lung and... : Chinese Medical Journal (original) (raw)
Echinococcosis is a parasitic disease caused by the larval forms of echinococci. It has two main forms as the unilocular cystic form that is more commonly seen and caused by E. granulosus and the alveolar form that is rarely seen and caused by E. multilocularis. E. multilocularis usually contaminates from foxes and sometimes contaminates from domestic animals as dogs and cats to humans and usually affects the liver. Progression of alveolar echinococcosis is more severe and agressive. It can metastise to the lungs, brain and bones. We present a case of alveolar echinococcosis that was initially thought to be a lymphomatous infiltration but then determined to infect the liver, lung and brain in a patient with a large T-cell lymphoma.
CASE REPORT
A 70-year-old female patient was admitted to our emergency unit because of complaints of fever, nausea, vomiting and confusion. Five years ago, she had been diagnosed with a non-Hodgkin's lymphoma with large T-cells in cervical lymph nodes. Either lymph node or mass had not been found by thoracal or abdominal CT. Bone marrow had not been affected. After chemotherapy, she had been in remission. One year ago, some nodular lesions had been determined by thoracal CT, including a lobulated lesion of 3.5 cm in diameter at the border of the apicoposterior segment of the left lung superior lobe and the superior segment of the left lung inferior lobe, a lesion of 1.5 cm × 1.0 cm at the posterobasal segment of the left lung inferior lobe, two lesions at the lingular segment of the left lung, two nodular lesions of 1.5 cm × 1.0 cm at the anterior segment of the right lung superior lobe (Figure 1).
A hypodense lesion of 11 cm × 10 cm ×7 cm containing calcifications had been determined at the anterior segment of the liver right lobe by abdominal CT (Figure 2). Biopsy specimen from the lesion had not been examined before CT showing lyphoma metastasis but malignancy. Cyclophosphamide (150 mg/d) therapy had been-prescribed.
Lesions at the right and left lung.
The patient was admitted to our emergency unit because of the presence of symptoms such as weight loss (10 kg in a month), weakness, night sweating, headache, trembling fever, nausea, vomiting, whole body spasm and confusion. She was unconscious and only responded with limb movements to painful stimulation. Babinski reflex was bilaterally positive. Muscle strength in the lower extremities was 3/6. Severe sensorial loss was determined. A painless, smooth surfaced, 7–8 cm hepatomegaly was palpated. Multiple lymph nodes (maximum 1–1.5 cm in size) were palpated in the axillary and inguinal regions bilaterally. Alcaline phosphatase was 501 U/L, gamma-glutamyl transferase (GGT) was 209 U/L, bilirubins were in normal ranges. Other values included leukocyte: 7400/ml, neutrophil: 4800/ml (66%), lymphocyte: 1500/ml (20%), monocyte: 700/ml (9%), eosinophil: 250/ml (3%), basophil: 150/ml (2%), Hb: 108 g/L, hematocrit: 30.3%, and trombocyte: 140 000/ml. Cranial CT showed regularly contoured cystic lesions excluding solid component but edematous effect on environmental tissues of the cerebral and cerebellar hemispheres (Figure 3).
Lesions at the right lobe of the liver.
Cyst infection was thought because of complaints as fever, nausea and vomiting and determination of cystic lesions on the lung and liver on CT that had been performed one year ago. After administration of ciprofloxacin 2×400 mg/d and metronidazole 4×500 mg/d, fever subsided but general condition did not recover. Indirect hemagglutination test for hydatid cyst was positive at dilutions up to 1/64. Spectrometric cranial MR imaging was better for the determination of the cyst content. Determined were multiple regular contoured pure cystic lesions (maximum 1.6 cm in size) that had environmental edema, that were isotense with cerebrospinal fluid in all sequences, and that were localised in the cortical deep white matter of the left parietal lobe, the deep white matter of the left frontal lobe, the left periventricular deep white matter, the periventricular deep white matter at the level of the left lateral ventricular trigon, right temporal lobe, left cerebellar hemisphere, and left inferior cerebellar pedincule (Figure 4). Contrast involvement on the wall of cystic lesion was seen on postcontrast images.
Regularly contoured cystic lesions and edematous effect on both cerebral and cerebellar hemispheres.
Biopsy specimen was taken from the lesion. PAS-positive granular membrane pieces concordant with alveolar echinococcosis were seen in necrotic areas of the specimen (Figure 5).
Cerebral and cerebellar lesions on spectrometric cranial MR image.
PAS-positive granular membrane pieces concordant with alveolar echinococcosis in the lesion (Original magnification × 125).
Surgical treatment could not be performed because of poor general condition of the patient. Albendazole 2× 400 mg/d was given, but the patient died five months later.
DISCUSSION
It is an infection that appears in humans after eating food contaminated with faeces of foxes, dogs and cats.1,2 When the contaminated material is taken orally, embryophores attach themselves to the duodenal mucosa, penetrate into the duodenum, pass to the portal blood circulation, and localise in the little sinusoids or capillaries of the liver (60%). Embryophores that can not attach to the liver (30%) are transported by the inferior vein cava and suprahepatic veins to the heart and, by pulmonary veins, reach the lung. Embryophores that can not attach to the lung (10%) are retransported by the pulmonary artery to the heart and then disseminate to the whole body.3 The occupational and environmental contact history was not determined in our patient.
The frequently encountered clinical symptoms are headache, nausea, vomiting and abdominal pain. Jaundice is rare however. Sometimes hepatomegaly can be the only symptom. Our patient presented with headache, vomiting and confusion. A 7–8 cm, smooth surfaced, painless hepatomegaly was palpated.
Serological tests are helpful in the diagnosis of this disease. The frequently used serological tests include antibody tests, ELISA and indirect hemaglutination (IHA). Monoclonal antibodies develop against the glycoprotein layer.4 In a study, determination of antibodies against Em2 and Emc antigens of E. alveolaris has been quite sensitive and specific.5 An intradermal allergic reaction test (Casoni test) also helps the diagnosis. But if the lesion does not have a real capsule, this test can be negative.3,4 Indirect hemaglutination (IHA) test for hydatid cyst was positive at dilutions up to 1/64 in our patient.
The liver is the most likely affected organ. There is a diffuse infiltration accompanied by invasive and destructive changes in the liver imitating a neoplasma.1,2 As the cyst does not have a fibrous capsule outside, it grows towards outside and constitutes a honeycomb structure formed of hundreds of little vesicules. It can disseminate to the biliary tract and can cause obstruction and jaundice in addition to portal hypertension and Budd-Chiari syndrome.3,6 It disseminates usually to the lung and brain3 but rarely to the pericardium, skin and bone.
Hydatid cysts are frequently seen in the right inferior lobe of the lung. Localisation in both lung and liver is seen in 6%-13% of cysts. It has been reported that in lung localisation 51% of cysts are solitary, 25% are multiple (12% unilateral, 13% bilateral), 24% are ruptured or infected.7 In our patient, bilateral multiple solid lesions were observed in the lung on CT.
Alveolar echinococcus cyst is frequently localised in the frontal lobe of the brain.3,8 Calcified, round, solid and regularly contoured lesions are observed on radiological examinations in cases of cerebral localisation. The lesions take contrast and there is edema in the surrounding tissue.9 Also the brain lesions in our patient were regularly contoured, round, isotense with cerebrospinal fluid, took contrast and caused edema in the surrounding tissue. The symptoms induced by cerebral involvement can not be observed in the early period. It progresses in years. The increase of intracranial pressure can be tolerated for a long time. But, a sudden increase can lead to coma.3 In our patient, symptoms appeared in the early period owing to multiple infection foci in the brain, cerebellar localisation, and increase of intracranial pressure due to ventricular obstruction.
Although serological tests and radiological examinations may be helpul, certain diagnosis is established by pathological examination.10 Percutaneous needle biopsy is not recommended because scolexes hinder the diagnosis by the remnant agent on the needle, which also infect the clean compartments.
Radical operation should be performed for suitable alveolar echinococcosis cases. Antibiotherapy should be continued for at least two years after surgical treatment. Resection as much as possible or continous antibiotherapy for years are required in unsuitable cases for radical surgery. In surgical treatment, hydatid cyst is removed completely as unruptured and the cavity is washed with hypertonic saline solution after intervention.1,4 But removal of the whole cyst is difficult as it does not have a real capsule restricting itself. It should be behaved so carefully during the surgical treatment, because if the cyst ruptures the disease agent can disseminate to the whole body by hematogen. The agent can also cause patient's death by sensitive reaction.
Various treatments have been tried for alveolar echinococcosis. Long lasting antibiotherapy can prolong the life of inoperable patients.11 Albendazole and mebendazole are most frequently used at the present time. The most effective drug has been albendazole as demonstrated in animal experiments.11 It is used frequently at a dose of 10 mg/kg per day.
The prognosis of the disease is similar to that of liver tumours. Its mortality has been as high as 90% in untreated cases.11 Drug treatment of the disease has been successful only in some patients. Liver transplantation is suitable for patients in whom the medication fails or who have symptoms of liver insufficiency.11 The prognosis of the disease was mortal in our patient in spite of drug treatment as surgical treatment could not be performed.
Association of lymphoma with alveolar echinococcosis has not been reported in the literature. When diffuse, solid and cystic lesions in the liver develop in cancer patients on remission, lymphomatous infiltration is considered first. But when case record, physical and radiological findings are not concordant with those of the existing disease, other diseases should also be taken into consideration. The prognosis of alveolar echinococcosis is mortal in spite of antimicrobial therapy as surgical treatment could not be performed.
REFERENCES
1. Czermak BV, Unsinn KM, Gotwald T, Waldenberger P, Freund MC, Bale RJ, et al. Echinococcus multilocularis revisited. Am J Roentgenol 2001; 176: 1207–1212.
2. Choji K, Fujita N, Chen M, Spiers AS, Morita Y, Shinohara M, et al. Alveolar hydatid disease of the liver: computed tomography and transabdominal ultrasound with histopathological correlation. Clin Radiol 1992; 46: 97–103.
3. King CH. Cestodes (tapeworms). In: Mandell GL, Bennett JE, Dolin R, eds. Mandell, Douglas and Bennett's principles and practise of infectious disease. 4th ed. New York: Churchill Livingstone; 1995: 2544–2553.
4. Duncan GJ, Tooke SM. Echinococcus infestation of the biceps brachii. A case report. Clin Orthop Relat Res 1990; 261: 247–250.
5. Gottstein B, Deplazes P, Eckert J, Muller B, Schott E, Helle O, et al. Serological (Em2-ELISA) and parasitological examinations of fox populations for Echinococcus multilocularis infections. Zentralbl Veterinarmed B 1991; 38: 161–168.
6. Gillet M, Bresson Hadni S. Hepatic alveolar echinococcosis. Rev Prat 1991; 41: 1805–1811.
7. Saksouk FA, Fahl MH, Rizk GK. Computed tomography of pulmonary hydatid disease. J Comput Assist Tomogr 1986: 10: 226–232.
8. Bilge T, Barut S, Bilge S, Aydım Y, Aksoy B, Senol S, et al. Primary multiple hydatid cysts of the brain: case report. Surg Neurol 1993: 39: 377–379.
9. Tuzun M, Altinors N, Arda IS, Hekimoğlu B. Cerebral hydatid disease CT and MR findings. Clin Imaging 2002; 26: 353–357.
10. Gottstein B. Molecular and immunological diagnosis of echinococcosis. Clin Microbiol Rev 1992; 5: 248–261.
11. WHO Informal Working Group on Echinococcosis. Guidelines for treatment of cyctic and alveolar echinococcosis in humans. Bull WHO 1996; 74: 231–242.
Keywords:
alveolar echinococcosis; lymphoma
© 2008 Chinese Medical Association