Velika retroperitonealna masa pri 56-letni ženski Large retroperitoneal mass in a 56-year old female
Avtor / Author
Ustanova / Institute
Nuhi Arslani1,2
"'Univerzitetni klinični center Maribor, Oddelek za abdominalno in splošno kirurgijo, Maribor, Slovenija; 2Univerza v Mariboru, Medicinska fakulteta, Maribor, Slovenija 'University Medical Centre Maribor, Department of Abdominal and General Surgery, Maribor, Slovenia; 2University of Maribor, Faculty of Medicine, Maribor, Slovenia
Izvleček
Abstract
Ključne besede:
Retroperitonealni prostor, kongenitalne napake, benigne ciste, ciste Müllerjevega voda
Key words:
Retroperitoneum, congenital abnormalities, benign cyst, Müllerian ductal cysts
Članek prispel / Received
8.11.2016
Članek sprejet / Accepted
20.4.2017
Naslov za dopisovanje / Correspondence
Dr. Nuhi Arslani, dr. med. Univerzitetni klinični center Maribor, Oddelek za abdominalno in splošno kirurgijo, Maribor, Slovenija. E-pošta: arslani.nuhi@gmail.com
Namen: Ciste Mullerjevega voda so redke kongenitalne napake regresije Mullerjevega sistema, ki se običajno nahajajo v medenici. Njihova prisotnost v retroperitoneju je zelo redka. In-cidenca retroperitonealnih cist raznolike morfologije in etiologije je približno 1 na 100 000 odraslih bolnikov. Razvrstimo jih lahko v limfne ciste, mezotelne, enteralne in urogenitalne ciste. Urogenitalne ciste so nadalje razvrščene v pronefrične, mezonefrič-ne, metanefrične in Mullerične tipe. Pri obeh spolih je najpogostejše mesto za Mullerjeve ciste medenica, pri moških večinoma v bližini prostatičnega utrikla in modih. Pri ženskah jih lahko pogosto zamenjujemo s primarnimi cistami jajčnikov in jajcevodov. Poročilo o primeru: V tem poročilu predstavljamo primer benigne retrope-ritonealne ciste Mullerjevega tipa pri 56-letni bolnici.
Purpose: Mullerian ductal cysts are rare congenital abnormalities of Mullerian system regression, typically located in the pelvis . Their presence in retroperitoneum is extremely rare, as the incidence of retroperitoneal cysts, even of diverse morphology and etiology, is approximately 1 in 100,000 adult admissions. Moreover, they can be classified into lymphatic, mesothelial, enteric, and urogenital cysts. Urogenital cysts are further categorized into pronephric, mesonephric, metanephric, and Mullerian types. However, in both sexes the most common location of the Mullerian cyst is in the pelvis; in men they reside mostly near the prostatic utricle and the appendix of the testis. In women, they may be often confused with primary ovarian or tubal cysts. Case Report: In this case report, we present a case of a benign retroperitoneal cyst of the Mullerian type in a 56-year old female.
CASE REPORT
A 56-year old female was admitted to the urological department for percutaneus biopsy of a kidney cyst. Further investigations showed that there was no connection between the kidney and the cyst; thus, a mesenteric cyst was suspected. She complained of nonspecific pain under the left costal margin, a digestive disorder, nausea, and flushing. In the past, she was anemic due to gynecological bleeding, for which she underwent endometrial ablation. She also had a history of uterine myomas. She was on medication for elevated blood pressure. Her physical examination was unremarkable.
Laboratory examinations, including total blood count, biochemical profile, and tumor markers were within normal range. A nativ computer tomografi (CT) scan and ultrasound of the abdomen were performed, which confirmed a round cystic lesion, likely mesenteric filled with 480 ml of sludge, located under the spleen with no connection to other organs. Moreover, a biliary hepatic cyst in the fifth lobe and a 6 cm large uterus myoma were described. Her urea breath test was positive for Helicobacter pylori, for which she underwent eradication therapy without consequence.
After one year, follow-up abdominal computer to-mografi scan showed enlargement of the abdominal
Figure 1. Transverse abdominal CT scan showing the retroperitoneal cyst on the left.
mass to 12 x 16 x 15 cm in size. The homogeneous hypodense mass was located between the lower margin of the spleen and lesser pelvis, and had radiolog-ic features of a mesenteric cyst with close relationship to and compression of other organs (Figures 1, 2). Extirpation of the cyst was offered.
A laparotomy was performed. After the mobilization of the line of Toldt, the cyst became apparent in the retroperitoneum (Figure 3). There were no attachments to other organs in the abdomen or pelvis. The cyst was evacuated in its entirity from the retroperitoneum, mostly with hand manipulation. Drainage was left in situ. Macroscopically, the cyst was homogenous, measuring 23 x 15 x 13 cm and weighing 2040 g. The epithelium was approximately 0.1 cm thick and the cyst contained yellow clear fluid (Figures 5, 6). Microscopic examination revealed Mullerian duct origin. The cystic wall was composed of tubular epithelium with connective tissue stroma. There were no complications in the early postoperative period or during follow-up.
DISCUSSION
Retroperitoneal cyst of Mullerian type is a rare entity (5, 6). The precise mechanism of its development is not known. One theory postulates that the retro-peritoneum includes aberrant, embryologic-derived
Figure 2. Coronal CT scan showing the retroperitoneal cyst on the left.
Figure 3. Exposure of the cyst after mobilization of the line of Toldt.
Figure 4. The macroscopically homogenous mullerian cyst containing yellow clear fluid
Mullerian duct tissue. This tissue might grow later in life under the influence of abnormal hormonal stimuli. The other theory suggests that the peritoneum might have undergone differentiation to become a serous/tubal-type with invagination of the underlying tissue, producing a cystic structure (7).
Retroperitoneal mMullerian cysts are mostly found in women after the third decade . In most cases, retroperitoneal cysts are clinically silent; but they can cause abdominal discomfort, pain, nausea, and vomiting (8, 9) In our case, epigastric discomfort and nausea were present, probably because of the H. pylori infection. Furthermore, Mullerian duct cysts
Figure 5. Removed cyst after the procedure, measuring 23 x 15 x 13 cm and weighing 2040 g.
can cause obstruction and irritation of the urinary system with the development of hydronephrosis and hydrourether (10,11). Retroperitoneal cysts can also be present as a palpable abdominal mass, in approximately 50% of cases (12).
The diagnosis of retroperitoneal cyst is difficult to establish preoperatively due to the lack of pathognomonic clinical features. Laboratory data are of no value, except for cancer antigen 125 that can indicate the benign nature of the cyst . Radiologic investigations such as ultrasound, CT scan, and magnetic resonance imaging of the abdomen can determinate the location, relationship to other organs, and the nature of the cystic lesion, whether serous or blood-filled. The definitive diagnosis can be confirmed after pathologic examination of the cyst. In our case, the cystic formation first resembled a renal cyst. Further radiologic diagnostic investigations proved there to be no connection between its formation, and the kidney and the pancreas.
Invasive treatment of Mullerian cysts is only indicated when symptoms appear. The best treatment choice is surgical extirpation with preservation of other normal surrounding structures. This procedure can be performed either laparoscopically or open. Open excision is recommended for large pelvic or abdominal masses. Segmental bowel resection may be necessary due to involvement of the mesen-teric vessels (12).
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