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Primary non-parasitic splenic cyst: a case report

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Cystic disease of the spleen is a relatively rare disease. It is classified either as a true primary cyst or as a secondary pseudocyst. Most splenic cysts are pseudocysts, which have non-epithelial lining, and are caused by previous abdominal blunt trauma. Conversely, primary splenic cysts have epithelial lining and are subdivided into parasitic and non-parasitic cyst. Non-parasitic primary splenic cyst is considered congenital and comprises about 10% of all splenic cysts. Total or partial splenectomy is the treatment of choice, but parasitic infection must be excluded prior to an operation. In this present report, we described a symptomatic, large primary non-parasitic splenic cyst, which was surgically treated with partial splenectomy.
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Korean J Hepatobiliary Pancreat Surg 2013;17:139-141 Case Report
Primary non-parasitic splenic cyst: a case report
Sung Il Kang, and Sung Yoon Jeon
Department of Surgery, Seogwipo Medical Center, Jeju, Korea
Cystic disease of the spleen is a relatively rare disease. It is classified either as a true primary cyst or as a secondary
pseudocyst. Most splenic cysts are pseudocysts, which have non-epithelial lining, and are caused by previous abdomi-
nal blunt trauma. Conversely, primary splenic cysts have epithelial lining and are subdivided into parasitic and non-para-
sitic cyst. Non-parasitic primary splenic cyst is considered congenital and comprises about 10% of all splenic cysts.
Total or partial splenectomy is the treatment of choice, but parasitic infection must be excluded prior to an operation.
In this present report, we described a symptomatic, large primary non-parasitic splenic cyst, which was surgically treat-
ed with partial splenectomy. (Korean J Hepatobiliary Pancreat Surg 2013;17:139-141)
Key Words: Spleen; Cyst
Received: August 5, 2013; Revised: August 20, 2013; Accepted: August 26, 2013
Corresponding author: Sung Il Kang
Department of Surgery, Seogwipo Medical Center, 1530-2, Donghong-dong, Seogwipo, Jeju 697-804, Korea
Tel: +82-64-730-3253, Fax: +82-64-733-4320, E-mail: sungiry@naver.com
Copyright 2013 by The Korean Association of Hepato-Biliary-Pancreatic Surgery
Korean Journal of Hepato-Biliary-Pancreatic Surgery ISSN: 1738-6349
INTRODUCTION
Cystic disease of the spleen is relatively rare, with an
incidence of 0.07%.1 The splenic cysts categorized either
as primary (true cysts) or as secondary (pseudocysts).
The latter is mostly caused by abdominal trauma and has
no epithelial lining in the cystic lumen. Conversely, a
primary splenic cyst has epithelial lining of the lumen.
Additinoally, primary splenic cysts are further subdivided
into parasitic and non-parasitic cyst. Non-parasitic pri-
mary splenic cyst is considered congenital and comprise
of about 10% of all splenic cysts.2 We present here a
rare case of primary non-parasitic splenic cyst that is
confirmed by pathologic diagnosis after partial splenec-
tomy.
CASE
A 20-year-old female immigrant worker with a dull ab-
dominal pain of several days duration was referred to our
hospital from a local clinic, where an abdominal ultra-
sonography (US) had revealed a large cystic mass in the
spleen. On abdominal examination, a soft non-tender mass
was palpable in the left upper quadrant. Her medical his-
tory was notable only for appendectomy performed sev-
eral years ago. She had no other traumatic event in the
abdomen. Her vital signs were stable and all laboratory
findings were within the normal range. Abdominal com-
puted tomography (CT) scan revealed the splenic cyst to
measure 14 cm in diameter with scoliosis most likely due
to mass effect (Fig. 1). The patient was offered open or
laparoscopic partial resection of the spleen, and the patient
opted for open surgery for economic reasons.
On laparotomy, the cyst was found to arise from the
spleen. It was adhered to the left lateral side of the liver
and left stomach wall (Fig. 2). The cystic fluid was care-
fully aspirated to avoid rupture into the operation field.
After this, the whole cyst was excised in a partial
splenectomy. A closed drain was left near the splenec-
tomy site.
The result of serologic hydatid antibody test had been
notified several days after the operation and was negative.
Both carbohydronic antigen 19-9 (CA 19-9) and carci-
noembryonic antigen (CEA) of the cystic fluid were over
1,000 U/ml respectively. The pathologic report indicated
a primary splenic cyst with cystwalls containing stratified
squamous epithelial cells (Fig. 3). The patient was dis-
charged on the 10th postoperative day without compli-
cations.
140 Korean J Hepatobiliary Pancreat Surg Vol. 17, No. 3, August 2013
Fig. 1. Abdominal CT scan sho-
ws a large splenic cyst with sco-
liosis. (A) Axial view, (B) Coro-
nal view.
Fig. 2. The cyst was adhered to the left sides of the liver an
d
stomach wall. Fig. 3. The microscopic examination shows stratified squ-
amous epithelial cells lining the cyst (H&E, ×400).
DISCUSSION
Cystic diseases of the spleen are relatively rare with an
incidence of 0.07%1 and are categorized as either primary
or secondary cysts.3 Secondary splenic cysts are usually
results from previous abdominal trauma. These have no
epithelial lining of the cystic lumen and, as such, are
peudocysts. Conversely, primary splenic cysts contain epi-
thelial lining, and these true cysts are subdivided into par-
asitic and non-parasitic cyst.
Parasitic splenic cysts, or splenic hydatid disease
(SHD), results from infection by Echinococcus species. It
must be excluded before invasive procedure because spill-
age of the cystic contents may lead to anaphylactic shock
or intraperitoneal dissemination of Echinococcus species.4
Although Echinococcus is not endemic to South Korea,
we had performed the serologic hydatid antibody test be-
cause the patient herself had come from an endemic
country. However, the result was negative. During the op-
eration, we took special care not to rupture the cystic fluid
into the peritoneum, as the result of the serologic hydatid
antibody test had not been made reported.
Primary non-parasitic splenic cysts are considered con-
genital and are lined by mesothelial, squamous, or transi-
tional epithelium. The serum or cystic fluid is often pos-
itive for CA 19-9 and CEA, although these cysts do not
have malignant potential.5-8 One hypothesis is that the in-
ner epithelial cells produce these tumor markers, and these
can circulate systematically by stoma-like channel be-
tween the lumen of the cyst and sinus of the adjacent
splenic tissue.7 In our case, CA 19-9 and CEA levels from
cystic fluid were high but pathologic report revealed no
Sung Il Kang and Sung Yoon Jeon. Primary non-parasitic splenic cyst: a case report 141
malignant changes.
A large sized splenic cyst can be detected on physical
examination. Moreover, preoperative US and CT scan are
useful in understanding the size and relationship of the
cystic lesions to surrounding structures.3 This information
is helpful in the differential diagnosis and management.
The conventional approach to splenic cysts has been com-
plete total splenectomy. However, overwhelming post-
splenectomy syndrome (OPSS) is a serious concern after
total splenectomy, and partial splenectomy is increasingly
considered as the treatment of choice.9 Additionally, lapa-
roscopic approach has been performed successfully.6,9-11
Percutaneous drainage or injection of sclerosing agents
have been used in limited cases for small cysts, but these
procedures are associated with high recurrence rates.12,13
In summary, splenic non-parasitic true cyst is a rare
disease. It can be detected by ultrasonography or CT scan
and are confirmed by pathologic findings. The serologic
hydatid antibody is checked prior to an invasive procedure
to exclude parasitic infection. Cystic tumor markers can
be elevated in the serum, but it has no malignant
potential. Partial or total splenectomy is the treatment of
choice, and percutaneous drainage can be considered for
small cysts or as bridges to surgery.
REFERENCES
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125 and Carcinoembryonic Antigen. Case Rep Gastroenterol
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7. Trompetas V, Panagopoulos E, Priovolou-Papaevangelou M, et
al. Giant benign true cyst of the spleen with high serum level
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8. Yigitbasi R, Karabicak I, Aydogan F, et al. Benign splenic epi-
thelial cyst accompanied by elevated Ca 19-9 level: a case report.
Mt Sinai J Med 2006;73:871-873.
9. Macheras A, Misiakos EP, Liakakos T, et al. Non-parasitic splen-
ic cysts: a report of three cases. World J Gastroenterol 2005;11:
6884-6887.
10. Iimuro Y, Okada T, Sueoka H, et al. Laparoscopic management
of giant splenic true cyst with partial splenectomy: A case report.
Asian J Endosc Surg 2013;6:226-230.
11. Kiriakopoulos A, Tsakayannis D, Papadopoulos S, et al. Laparo-
scopic management of a ruptured giant epidermoid splenic cyst.
JSLS 2005;9:349-351.
12. De Caluwé D, Phelan E, Puri P. Pure alcohol injection of a con-
genital splenic cyst: a valid alternative? J Pediatr Surg 2003;38:
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cutaneous drainage in non-parasitic splenic cysts? Case report. G
Chir 2012;33:343-345.
... Total or partial splenectomy is the accepted treatment; however, parasitic infections must be excluded preceding operation [4]. Still, improved perception of the significant immunological role of the spleen has led to alternative spleen-preserving options. ...
... In such these cases, the preoperative differentiation between parasitic and non-parasitic is vital due to their variations in diagnosis and treatment. Parasitic splenic cysts or splenic hydatid disease must be excluded before invasive procedure due to spillage of the cystic contents, which may lead to anaphylactic shock or intraperitoneal dissemination of Echinococcus species [4,24]. Accordingly, surgeons should keep in mind the possibility of a parasitic cyst when no reliable diagnosis has been made. ...
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Background Splenic cysts are quite rare and asymptomatic. They may result from infection by a parasite, especially Echinococcus granulosus (hydatid cyst), or from non-parasitic causes. Since primary splenic cysts are not common, simple cysts can be misdiagnosed with a hydatid cyst in endemic areas. Case presentation We reported a 14-year-old Iranian girl initially presented with a vague abdominal pain, which progressed to left shoulder pain, fullness, early satiety, and shortness of breath and remained undiagnosed for 7 months despite seeking medical attention. Finally, imaging revealed a massive splenic cyst measuring 220 mm × 150 mm × 160 mm raising concern for a hydatid cyst due to regional endemicity. Consequently, the patient underwent total splenectomy. However, histopathological examination surprisingly revealed a simple non-parasitic cyst. Conclusions Detecting rare simple spleen cysts requires early ultrasonography (US) and careful reassessment of diagnoses for non-responsive or worsening symptoms. Distinguishing them from splenic hydatidosis, especially in endemic areas, demands thorough paraclinical evaluations and patient history regarding potential parasitic exposure. While total splenectomy is the primary treatment for these huge cysts, the optimal surgical approach should be tailored case by case. These insights emphasize a comprehensive diagnostic approach to enhance accuracy and optimize patient care for these uncommon cysts.
... 6 Los pacientes suelen referir molestias en el cuadrante superior izquierdo acompañadas de otros síntomas debidos a la compresión de órganos adyacentes, como: sensación de plenitud, náuseas, vómito, flatulencia o diarrea. 7 La exploración física revela, en la mitad de los casos, esplenomegalia o una masa palpable, más o menos dolorosa, en el mismo flanco. ...
... Aún no se dispone de una prueba específica de laboratorio pero hace poco se descubrió que el contenido quístico puede ser positivo para el marcador tumoral Ca 19-9, que pasa a la circulación sistémica, aun sin potencial maligno. 7 Cuando los quistes se sobreinfectan, como en nuestro caso, aparece fiebre, leucocitosis y dolor abdominal intenso que, en ocasiones, puede referirse hacia el hombro ipsilateral o la parrilla costal por afectación diafragmática o derrame pleural asociado. 8 Sin embargo, son raros los niños que aquejan, desde el principio, como el nuestro, síntomas respiratorios e insuficiencia pulmonar, por implicación directa del parénquima. ...
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INTRODUCCIÓN: Los quistes esplénicos aislados son raros en niños y potencialmente graves y casi siempre difíciles de diagnosticar. CASO CLÍNICO: Paciente de 5 años, traído a urgencias por fiebre alta, dolor abdominal y vómito. Reporte hematológico: leucocitosis con elevación de reactantes de fase aguda, hemocultivo negativo y coprocultivo positivo para Salmonella serogrupo D. En la radiografía de tórax se objetivó infiltrado basal izquierdo y derrame pleural; en la ecografía abdominal se detectó lesión quística intraesplénica de gran tamaño. Con el diagnóstico de absceso gigante del bazo se pautó tratamiento antibiótico intravenoso y drenajes percutáneos repetidos hasta la intervención quirúrgica definitiva con extirpación quística parcial y fenestración de la cápsula. En el cultivo del absceso creció una Salmonella del mismo serogrupo. El análisis anatomopatológico de la pieza fue compatible con quiste epitelial primario. CONCLUSIONES: Como parte del diagnóstico diferencial de cualquier paciente con signos sugerentes de afectación en el hipocondrio izquierdo deben considerarse los quistes esplénicos. En los últimos años ha habido grandes avances en su tratamiento; en pacientes pediátricos suele ser conservador. PALABRAS CLAVE: Quiste epitelial; absceso esplénico; esplenectomía; niños
... Medical and laboratory technologies have greatly advanced in recent years. However, the diagnosis of CE is still a challenge, which presents difficulty in determining whether a splenic cyst is parasitic or nonparasitic (Kang and Jeon, 2013;Kowe et al., 2015), until pathognomonic signs have been manifested as a result of the increase in size of the echinococcal cyst and the compression of the surrounding tissues. ...
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Cystic echinococcosis (CE) is a zoonotic disease caused by Echinococcus granulosus sensu lato, the metacestode of a tapeworm parasite of high medical importance. Infection of the parasite leads to the development of echinococcal cysts, and the spleen is a rarely infected organ. A 46-year-old woman who was born and who resides in Sardinia, Italy, was referred to the Echinococcosis outpatient clinic at the University Hospital of Sassari (Sardinia, Italy) for a pain in the left flank. She used to live in the countryside, in contact with several animals, and for 2 years, she had been working in a family garden, growing vegetables as a hobby. Ultrasounds and X-ray were performed, which evidenced a rounded formation in the upper third of the spleen, while a CT scan confirmed a parasitological cyst. Immunological examinations on serum samples did not detect specific antibodies against Echinococcus spp. Following surgical exportation, the whole spleen with the cystic lesion was delivered to the World Organisation for Animal Health (WOAH) and the National Reference Laboratory for Echinococcosis for further laboratory analyses. Moreover, characterization of the cyst fluid resulted dense and shiny. Observation under a light microscope at ×400 magnification revealed the formation of rectangular crystals and aggregates attributable to cholesterol molecules. Subsequently, through parasitological investigation, molecular biology investigations confirmed E. granulosus sensu stricto G1. We describe cholesterol crystals in a splenic echinococcal cyst for the first time. There is no clear explanation of how the cholesterol crystals formed in this case, but this was attributed to multifactorial causes, including atherosclerosis, chronic inflammation, parasite metabolism, and host responses.
... There may be pressure symptoms of constipation or difficulty in breathing. [5][6][7][8] Rarely giant splenic cyst can erode the anterior abdominal wall and present as splenocutenous fistula as in this patient. 3 Investigation that confirm presence of splenic cyst are Abdominal CT scan, Abdominal ultrasound and occasionally endoluminal ultrasound scan and MRI. ...
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Splenic cyst with splenocutaneous fistula is extremely rare. It is mainly caused by trauma or parasitic infestation. Splenic cyst mainly present with right upper quadrant abdominal pain and swelling. It can be complicated by rupture however presentation as splenecutaneous fistula is extremely rare as only one case was reported in the literature. Management of splenic cyst can be partial or total splenectomy and can be done open or laparoscopic. In this case we present a case of splenic cyst with splenocutaneous fistula that was managed surgically with resection.
... In general, the prevalence of splenic cysts is higher among women when compared to men, with the age group between the second and third decades of life, being the most affected [8]. The patient described is female and is 35 years old, which is in agreement with those found in literature. ...
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