Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

03 April 2021: Articles  Japan

Mucinous Cystic Adenoma of the Liver: A Thought-Provoking Case of an Uncommon Hepatic Neoplasm

Rare disease

Hikotaro Katsura1BCDEF, Tomohide Hori1ABCDEF*, Hideki Harada1DF, Kazuyoshi Matsumura2CD, Michihiro Yamamoto1DF, Masahiro Yamada1DF, Takefumi Yazawa3DF, Ben Sasaki1DF, Katsutoshi Kuriyama2CD, Masaki Tani1DF, Asahi Sato1DF, Yudai Sasaki1DF, Hidekazu Yamamoto1DF

DOI: 10.12659/AJCR.931368

Am J Case Rep 2021; 22:e931368

0 Comments

Abstract

BACKGROUND: Mucinous cystic neoplasm (MCN) of the liver is a rare hepatic neoplasm: a cystic, mucus-producing tumor. Histopathologic examination reveals ovarian-like stroma. The origin of MCN of the liver is still unknown, although ectopic ovarian-like stroma in the liver has been suggested as a possibility. We document a thought-provoking case of MCN of the liver, and intratumoral fatty tissue may support the opinion that ectopic ovarian-like stroma in the liver is a possible origin for both MCN and ovarian teratoma.

CASE REPORT: An expansive 10.5-cm cystic tumor was incidentally detected in a 71-year-old woman. Imaging studies revealed that the tumor was multiloculated, with cyst contents comprising mucus, muddy-looking fluid (inspissated bile), and hematoma. Imaging studies revealed fatty tissue and calcifications in the cyst walls. The diagnosis of MCN of the liver was made, although MCNs have never been reported to include fatty tissue. Extended left lobectomy was performed, and the tumor was curatively removed without any rupture. A multilocular cyst, mucus, calcifications, and fatty tissue were clearly observed on gross inspection. Histopathological examination revealed ovarian-like stroma. Evidence of malignancy was not detected. Her postoperative course was uneventful. To the best of our knowledge, our patient is the first case of MCN of the liver with intratumoral fatty tissue. This case may support the hypothesis that MCN originates from ectopic ovarian-like stroma in the liver.

CONCLUSIONS: We documented a thought-provoking case of MCN of the liver in detail, and this MCN accompanied with fatty tissue might originate from ectopic ovarian-like stroma.

Keywords: Adenoma, Cysts, Liver, Liver Neoplasms, Mucus, Ovary, Aged, Cystadenoma, Mucinous, Pancreatic Neoplasms

Background

Mucinous cystic neoplasm (MCN) of the liver is a rare hepatic neoplasm, and this cystic, mucus-producing tumor is histopathologically associated with ovarian-like stroma [1–3]. Radical resection is curative treatment [4–7], and the postoperative prognosis is acceptable [2,3,7,8]. The origin of MCN of the liver is still unknown, although ectopic ovarian-like stroma in the liver has been suggested [8–10].

We report a thought-provoking case of MCN of the liver in detail. In general, MCN of the liver does not involved fatty tissue [3,11,12]. In particular, to the best of our knowledge, our patient is the first case of MCN of the liver having fatty tissue inside the tumor. This case may support the hypothesis that ectopic ovarian-like stroma in the liver is the origin of MCN [8–10]. Moreover, we discuss a possible origin of an extremely rare MCN in the liver accompanied with fatty tissue, with a literature review.

Case Report

A 71-year-old woman with diabetes and deep venous thrombosis was referred to our hospital for treatment of meningitis. She had no history of smoking or excessive alcohol consumption. Treatment for meningitis resulted in drug-induced liver damage, and the liver was checked by ultrasound as part of our routine protocol in such cases. An expansive 10.5-cm cystic tumor was incidentally detected in the medial lobe of the liver. Plain and dynamic computed tomography and enhanced and fat-suppressed magnetic resonance imaging revealed that this tumor was multilocular (ie, cyst-in-cyst), contained mucus, muddy-looking fluid, and hematoma (Figure 1A–1D), and was associated with fatty tissue (Figure 2) and calcifications (Figure 1A). The cyst wall did not show enhancement, but a partial solid component was observed (Figure 1D). The sharply marginated cyst wall touched the middle hepatic vein. Tumor markers were all within the reference range. Splenomegaly indicated portal hypertension, although collateral development was not observed. We suggest that intratumoral bleeding, which resulted in hematoma, might secondarily cause communication with the bile duct. Hence, a diagnosis of MCN of the liver was made, although MCN has not been reported to include fatty tissue. In fact, intraductal papillary neoplasm of the bile duct (IPNB) was a differential diagnosis in our patient. Three-dimensional volumetry showed that the remnant liver volume in an extended left lobectomy was estimated to be 76.9%. The demarcation line was clearly confirmed after ligation of the left portal vein, and then, the middle and left hepatic arteries were ligated. Extended left lobectomy with resection of the middle hepatic vein and Spiegel lobe was performed, and the cystic, mucus-producing tumor was curatively removed without any rupture (Figure 3A). In order to keep a safe surgical margin and to avoid an intraoperative rupture, we employed intraoperative ultrasound. Contrast agent (Sonazoid; GE Healthcare, Oslo, Norway) and color Doppler were also used. Hence, tran-section of hepatic parenchyma and the middle hepatic vein were guided in real-time during hepatectomy. The operative time was 241 minutes. Blood loss was 702 mL, and blood trans-fusion was not required. Gross pathologic inspection of the resected specimen revealed inspissated bile within the cystic tumor, corresponding to the muddy-looking fluid seen in pre-operative imaging studies (Figure 1C, 1D). Multiloculation, mucus, calcifications, and fatty tissue were clearly observed (Figures 3B, 4). Histopathological assessment revealed ovarian-like stroma (Figure 5), that was positive for estrogen and progesterone receptors in immunohistochemical studies (Figure 5). Evidence of malignancy was not detected. The diagnosis was MCN of the liver. Histopathologic examination of the nontu-moral liver revealed cirrhosis, presumably related to metabolic disease. Her postoperative course was uneventful, and she was discharged from the hospital on postoperative day 14.

Discussion

MCN of the liver was first documented in 1958 by Edmondson [13], and its incidence is rare, at <5% of cystic diseases of the liver [7,13]. In 1985, it was reported that some cystic and mucus-producing neoplasms in the liver were histopathologically associated with ovarian-like stroma [1], and thereafter, there was a period of confusion between the clinical assessment and histopathological findings. Clinical symptoms, serologic markers, and imaging modalities are unreliable for diagnosing cystic and mucus-producing neoplasms of the liver, resulting in misdiagnosis in 55–100% of these patients [14,15]. Therefore, there was a great change in the disease concept and diagnostic criteria for these hepatic neoplasms. The World Health Organization (WHO) classification of digestive system tumors continually reflects important advancements in the field of clinical oncology and tumor pathology [16]. In 2010, the WHO clarified the classification of cystic and mucus-producing neoplasms in the liver and put an end to physicians’ confusion and muddled pathological criteria (Table 1) [2]. Thereafter, the WHO classification is widely used for classification of epithelium-lined cystic lesions of the liver [2,3].

MCN is a cystic, mucus-producing neoplasm; it has the following clinical features and pathological characteristics: (i) significant sex-based difference in incidence (ie, occurrence mainly in women), (ii) multilocular (so-called “cyst-in-cyst”) or unilocular cystic form, (iii) no communication with the bile duct, (iv) mucosal fluid, (v) wall lined by cuboidal or columnar mucus-producing epithelial cells, and (vi) histopathological confirmation of ovarian-like stroma [1–3,14,16]. In our case, inspis-sated bile in the MCN indicated communication with the bile duct, which we suggested might occur secondarily because of bleeding into the MCN, supported by the coexistence of hematoma. In contrast, IPNB, which is important to include in the differential diagnosis, is a biliary epithelial neoplasm that shows papillary growth on the axis of the vascular stroma into the bile duct. IPNB has the following clinical features and pathological characteristics: (i) no sex-based difference in incidence, (ii) lobulated shape, (iii) changes in the bile duct (eg, distal dilation), (iv) independent of mucus production, (v) noninvasive papillary or villous tumor, and (iv) absence of ovarian-like stroma [1–3,14,16].

Definitive diagnosis of MCN in the liver is challenging [14,15]. MCN of the liver does not involve fatty tissue [3,11,12], and generally has no communication with the bile duct. The most common differential diagnosis is IPNB [2,3,12,15]. IPNB also is accompanied by mucus production, but generally shows communication with the bile duct and/or biliary changes [1–3,14,16]. In our patient, the cystic tumor contained mucus, muddy-looking fluid (ie, bile), and hematoma. Intratumoral bleeding easily results in hematoma [17], and a bleeding episode may secondarily cause unexpected communication with the bile duct [17,18]. Even though intratumoral bleeding and biliary communication are rare in MCN, patients with MCN in the liver accompanied with intratumoral bleeding [17] and biliary communication [18] have been reported. In our patient with MCN in the liver, we considered that intratumoral bleeding might secondarily cause biliary communication. Hence, we made a preoperative diagnosis of not IPNB but MCN, although MCN in the liver never involves fatty tissue [3,11,12],

MCN of the liver has malignant potential; therefore, radical re-section is the first-choice therapeutic strategy [4–7,19]. Even in cases that are malignant, MCN of the liver generally grows into its own cystic lesion [2,3]. Hence, curability can be achieved by optimal surgery even in malignant MCNs in the liver [2,3,19], and oncological and prognostic outcomes are good after radical resection [2,8,19]. Needless to say, even a subtle rupture during hepatectomy should be avoided to prevent iatrogenic dissemination, and hepatectomy procedures for surgical curability should be carefully decided on a case-by-case basis. In benign MCN, curative resection is mandatory [7,8,19] because patients with a partial remnant often have persistent symptomatic disease [2,8]. The prognosis of benign MCN after surgery is excellent [7,8,19], and no oncological deaths have been documented. Even in malignant MCN, the 5-year survival rate after surgical resection was reported to be 68.9–100% [8,19].

MCN and IPNB are clearly classified on the basis of the presence or absence of ovarian-like stroma [1–3], and a histopathological characteristic of MCN is the presence of ovarian-like stroma in the cyst wall as aggregated spindle-shaped cells. This stroma expresses estrogen and/or progesterone receptors, although the positivity rate in immunohistochemistry is low. However, epithelial cells of the MCN produce mucus, which is the main component. From the viewpoint of immunohistochemistry of the core protein of mucin, the meaning of a specific profile of mucin expression (eg, MUC1) is controversial in MCN of the liver [20], although this expression reflects the malignant potential of cystic, mucus-producing neoplasms of the pancreas [21].

The origin of MCN of the liver is still unknown. Some hypotheses have been suggested; for example, endoblast (the source of the hepatic parenchyma and bile ducts) and ectopic ovarian-like stroma in the liver are reported to be potential sources [8–10]. MCN of the liver morphologically and embryologically recapitulates ovarian stroma [8–10]. MCN in the liver may arise from ectopic stromal cells or primitive gonadal tissue [8–10], and these may have an ability to transdifferentiate to ovarian cortical cells [8–10]. In general, teratomas arise from germ cells that originate in the mature genital glands [22,23], and extremely rare cases of teratomas at extragonadal site and/or origin have been reported [24–26]. In particular, the unique finding of fatty tissue and rare finding of calcifications in the tumor were clearly observed in our patient. To the best of our knowledge, our patient is the first case of MCN of the liver that had associated intratumoral fatty tissue (Figure 2). Calcifications were also observed in our case. In contrast, ovarian teratomas often involve teeth, bone, skin, and hair, in addition to fatty tissue [22,23]. However, we speculate that ectopic ovarian-like stroma in the liver is a possible origin for both MCN and ovarian teratoma [8–10,22,23], and the unique finding of intratumoral fatty tissue in our case supports the possibility that ectopic ovarian-like stroma is the origin of MCN of the liver [8–10,23].

We hope that our case report will be informative for physicians, surgeons, pathologists, and radiologists in the hepatobiliary field.

Conclusions

A thought-provoking case of MCN of the liver was documented in detail. In general, MCN of the liver never involves fatty tissue, and our extremely rare case of MCN may have originated from ectopic ovarian-like stroma in the liver.

Figures

Figure 1.. Hepatic CT and MRI findings. (A, B) Imaging findings of plain CT (A) and T2-weighted MRI (B). Calcifications were clearly detected on plain CT (white arrows). The multilocular cyst (ie, cyst-in-cyst) contained mucus (blue arrows). A hematoma was observed inside (red arrows). (C, D) Imaging findings of dynamic CT (C) and T1-weighted MRI (D). The cystic, mucus-producing tumor contained not only mucus (blue arrows), but also muddy-looking fluid (ie, inspissated bile) (green arrows) and hematoma (red arrows). The cyst wall did not show enhancement, and a partial solid component was observed (orange arrows). CT – computed tomography; MRI – magnetic resonance imaging. Figure 2.. Fatty tissue within the cyst. Imaging findings of plain CT (A), T1-weighted MRI (B), and fat-suppressed MRI (C). The cystic, mucus-producing tumor clearly included fatty tissue (yellow arrows). CT – computed tomography; MRI – magnetic resonance imaging. Figure 3.. Gross pathological examination. Examination of the resected specimen revealed that the tumor was 10.5 cm and was curatively removed without any rupture of the cyst (overhead view, A). The cyst was multilocular, with mucus, calcifications, hematoma (red arrows), and fatty tissue (yellow arrows) observed (coronary view, B). Inspissated bile was present within the tumor. Cut surface (white arrows) is presented (A, B). Figure 4.. Histopathological examination. (A) Microscopic section of the MCN cyst wall (hematoxylin-eosin staining, ×40 magnification). Cystic wall lined by epithelial cells, and mucosal fluid was involved. (B) Fatty tissue was also observed (hematoxylin-eosin staining, ×200 magnification). Figure 5.. Ovarian-like stroma. Ovarian-like stroma was observed (hematoxylin-eosin staining, ×80 magnification) (A). Ovarian-like stroma was positive for estrogen receptor (B) and progesterone receptor (C) in immunohistochemical studies.

References:

1.. Wheeler DA, Edmondson HA, Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. A clinicopathologic study of 17 cases, 4 with malignant change: Cancer, 1985; 56; 1434-45

2.. Bosman F, Carneiro F, Hruban R, Theise N: WHO classification of tumours of the digestive system, 2010, Lyon, International Agency for Research on Cancer

3.. : Digestive System Tumours (World Health Organization Classification of Tumours), 2019, Lyon, International Agency for Research on Cancer

4.. Vogt DP, Henderson JM, Chmielewski E, Cystadenoma and cystadenocarcinoma of the liver: a single center experience: J Am Coll Surg, 2005; 200; 727-33

5.. Lauffer JM, Baer HU, Maurer CA, Biliary cystadenocarcinoma of the liver: The need for complete resection: Eur J Cancer, 1998; 34; 1845-51

6.. Sang X, Sun Y, Mao Y, Hepatobiliary cystadenomas and cystadenocarcinomas: A report of 33 cases: Liver Int, 2011; 31; 1337-44

7.. Brittingham C, Tuma F: Hepatic Cystadenoma, 2021, Treasure Island (FL), StatPearls Publishing

8.. Devaney K, Goodman ZD, Ishak KG, Hepatobiliary cystadenoma and cyst-adenocarcinoma. A light microscopic and immunohistochemical study of 70 patients: Am J Surg Pathol, 1994; 18; 1078-91

9.. Akwari OE, Tucker A, Seigler HF, Itani KM, Hepatobiliary cystadenoma with mesenchymal stroma: Ann Surg, 1990; 211; 18-27

10.. Van Treeck BJ, Lotfalla M, Czeczok TW, Molecular and immunohisto-chemical analysis of mucinous cystic neoplasm of the liver: Am J Clin Pathol, 2020; 154; 837-47

11.. Kania L, Guglielmo F, Mitchell D, V Interpreting body MRI cases: Classic findings in abdominal MRI: Abdom Radiol, 2018; 43; 2790-808

12.. Anderson MA, Dhami RS, Fadzen CM, CT and MRI features differentiating mucinous cystic neoplasms of the liver from pathologically simple cysts: Clin Imaging, 2021; 76; 46-52

13.. Edmondson H, Tumors of the liver and intrahepatic bile ducts: Atlas of tumor pathology armed forces institute of pathology, 1958; 24-28, Washington, D.C., Fascicle

14.. Simo KA, McKillop IH, Ahrens WA, Invasive biliary mucinous cystic neoplasm: A review: HPB (Oxford), 2012; 14; 725-40

15.. Shyu S, Singhi AD, Cystic biliary tumors of the liver: diagnostic criteria and common pitfalls: Hum Pathol, 2020 [Online ahead of print]

16.. Nagtegaal ID, Odze RD, Klimstra D, The 2019 WHO classification of tumours of the digestive system: Histopathology, 2020; 76; 182-88

17.. Andrade DC, Chassaing C, Mamodaly M, A giant bleeding liver mucinous cystic neoplasm: Liver Int, 2019; 39; 1999-2000

18.. Ferreira R, Abreu P, Jeismann VB, Mucinous cystic neoplasm of the liver with biliary communication: case report and surgical therapeutic option: BMC Surg, 2020; 20; 328

19.. Marcacuzco Quinto AA, Nutu OA, Rodríguez Gil Y, Cystic liver neoplams: A single centre experience and literature review: Cir Esp, 2021; 99; 27-33

20.. Usyaky PV, Kubyshkin VA, Vishnevsky VA, Mucinous cystic liver tumors: Diagnosis and surgical treatment: Khirurgiia (Mosk), 2016; 10; 27-40

21.. Nagata K, Horinouchi M, Saitou M, Mucin expression profile in pancreatic cancer and the precursor lesions: J Hepatobiliary Pancreat Surg, 2007; 14; 243-54

22.. Saleh M, Bhosale P, Menias CO, Ovarian teratomas: Clinical features, imaging findings and management: Abdom Radiol (NY), 2021 [Online ahead of print]

23.. Taylor EC, Irshaid L, Mathur M, Multimodality imaging approach to ovarian neoplasms with pathologic correlation: Radiographics, 2021; 41; 289-315

24.. Sethi P, Purkait S, Mature cystic teratoma of Douglas’ pouch: A rare entity: Cureus, 2019; 11; 5515

25.. Miyazaki H, Yokoyama S, Ito K, [A case of mature teratoma in the hepatoduodenal ligament.]: Gan To Kagaku Ryoho, 2020; 47; 2239-41 [in Japanese]

26.. He C, Yang Y, Yang Y, Teratoma of the adrenal gland: clinical experience and literature review: Gland Surg, 2020; 9; 1056-64

Figures

Figure 1.. Hepatic CT and MRI findings. (A, B) Imaging findings of plain CT (A) and T2-weighted MRI (B). Calcifications were clearly detected on plain CT (white arrows). The multilocular cyst (ie, cyst-in-cyst) contained mucus (blue arrows). A hematoma was observed inside (red arrows). (C, D) Imaging findings of dynamic CT (C) and T1-weighted MRI (D). The cystic, mucus-producing tumor contained not only mucus (blue arrows), but also muddy-looking fluid (ie, inspissated bile) (green arrows) and hematoma (red arrows). The cyst wall did not show enhancement, and a partial solid component was observed (orange arrows). CT – computed tomography; MRI – magnetic resonance imaging.Figure 2.. Fatty tissue within the cyst. Imaging findings of plain CT (A), T1-weighted MRI (B), and fat-suppressed MRI (C). The cystic, mucus-producing tumor clearly included fatty tissue (yellow arrows). CT – computed tomography; MRI – magnetic resonance imaging.Figure 3.. Gross pathological examination. Examination of the resected specimen revealed that the tumor was 10.5 cm and was curatively removed without any rupture of the cyst (overhead view, A). The cyst was multilocular, with mucus, calcifications, hematoma (red arrows), and fatty tissue (yellow arrows) observed (coronary view, B). Inspissated bile was present within the tumor. Cut surface (white arrows) is presented (A, B).Figure 4.. Histopathological examination. (A) Microscopic section of the MCN cyst wall (hematoxylin-eosin staining, ×40 magnification). Cystic wall lined by epithelial cells, and mucosal fluid was involved. (B) Fatty tissue was also observed (hematoxylin-eosin staining, ×200 magnification).Figure 5.. Ovarian-like stroma. Ovarian-like stroma was observed (hematoxylin-eosin staining, ×80 magnification) (A). Ovarian-like stroma was positive for estrogen receptor (B) and progesterone receptor (C) in immunohistochemical studies.

SARS-CoV-2/COVID-19

04 July 2022 : Case report  Italy

Interleukin-6 Is a Promising Marker of COVID-19 in Children: A Case Series of 2 Brothers with Severe COVID-...

Am J Case Rep In Press; DOI: 10.12659/AJCR.934468  

29 June 2022 : Case report  USA

A Clinical Case of COVID-19 Vaccine-Associated Guillain-Barré Syndrome

Am J Case Rep In Press; DOI: 10.12659/AJCR.936896  

16 June 2022 : Case report  Ecuador

Multidrug-Resistant Klebsiella pneumoniae in a Patient with SARS-Cov-2 Pneumonia in an Intensive Care Unit ...

Am J Case Rep In Press; DOI: 10.12659/AJCR.936498  

13 June 2022 : Case report  Japan

Stenotrophomonas maltophilia Infection Associated with COVID-19: A Case Series and Literature Review

Am J Case Rep In Press; DOI: 10.12659/AJCR.936889  

In Press

04 Jul 2022 : Case report  USA

Unusual Case of Mirizzi Syndrome Presenting as Painless Jaundice

Am J Case Rep In Press; DOI: 10.12659/AJCR.936836  

04 Jul 2022 : Case report  Italy

Interleukin-6 Is a Promising Marker of COVID-19 in Children: A Case Series of 2 Brothers with Severe COVID-...

Am J Case Rep In Press; DOI: 10.12659/AJCR.934468  

04 Jul 2022 : Case report  Saudi Arabia

Relapsed Angioimmunoblastic T Cell Lymphoma with Fulminant Leukemic Involvement

Am J Case Rep In Press; DOI: 10.12659/AJCR.936448  

01 Jul 2022 : Case report  Poland

A 75-Year-Old Female Smoker with Advanced Small-Cell Lung Cancer and Eastern Cooperative Oncology Group Per...

Am J Case Rep In Press; DOI: 10.12659/AJCR.936536  

Most Viewed Current Articles

23 Feb 2022 : Case report  USA

Penile Necrosis Associated with Local Intravenous Injection of Cocaine

DOI :10.12659/AJCR.935250

Am J Case Rep 2022; 23:e935250

17 Feb 2022 : Case report  Oman

Myocarditis, Pulmonary Hemorrhage, and Extensive Myositis with Rhabdomyolysis 12 Days After First Dose of P...

DOI :10.12659/AJCR.934399

Am J Case Rep 2022; 23:e934399

06 Dec 2021 : Case report  Brazil

Lipedema Can Be Treated Non-Surgically: A Report of 5 Cases

DOI :10.12659/AJCR.934406

Am J Case Rep 2021; 22:e934406

09 Feb 2022 : Case report  Saudi Arabia

Extensive Cerebral Venous Sinus Thrombosis (CVST) After the First Dose of Pfizer-BioNTech BNT162b2 mRNA COV...

DOI :10.12659/AJCR.934744

Am J Case Rep 2022; 23:e934744

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923