Case Reports in Hepatology

Case Reports in Hepatology / 2019 / Article

Case Report | Open Access

Volume 2019 |Article ID 4734606 |

Prachi Rana, Shida Haghighat, Hyosun Han, "Endometrioma of the Liver: A Case Report and Review of the Literature", Case Reports in Hepatology, vol. 2019, Article ID 4734606, 8 pages, 2019.

Endometrioma of the Liver: A Case Report and Review of the Literature

Academic Editor: Melanie Deutsch
Received11 Mar 2019
Accepted14 May 2019
Published04 Jun 2019


Hepatic endometriosis is a rare form of endometriosis first described by Finkel in 1986. A thorough review of the literature revealed 28 cases of hepatic endometriosis. This unusual condition offers several diagnostic challenges due to its variable appearance on imaging and need for histologic analysis to establish a definitive diagnosis. We present a 42-year-old female initially treated for presumed hydatid cyst that was later found to be endometriosis in the liver. The case highlights the importance of considering endometriosis in the differential for a patient presenting with a solitary liver mass regardless of age and previous history of endometriosis.

1. Introduction

Endometriosis is characterized by the presence of endometrial tissue outside of the uterine cavity. It is a benign condition most commonly noted in the uterus, fallopian tubes, ovaries, and local pelvic peritoneum. Atypical endometriosis, when the condition is found in extrapelvic regions, is rare [1]. While uncommon, atypical endometriosis has been described in remote sites including the GI tract, diaphragm, skin, lung, pleura, kidney, and pancreas. The only organ in the abdomen that is refractory to endometriosis is the spleen [2].

Hepatic endometriosis is one of the most rare forms of extrapelvic endometriosis, first described in 1986 [3]. Only 28 cases have been reported in the English literature. We herein present the 29th case of hepatic endometriosis, a 42-year-old female initially treated for presumed hydatid cyst that was later found to be endometriosis in the liver. This rare condition offers several diagnostic challenges. We offer an exhaustive review of the literature focusing on advances in the clinical manifestation, patient characteristics, pathogenesis, and diagnostic workup of the condition.

2. Case Presentation

A 42-year-old multiparous woman presented with episodic, severe right upper quadrant pain associated with nausea and vomiting. Her past surgical history included a hysterectomy and left oophorectomy for unclear reasons. Several months prior she presented to another hospital for similar symptoms and was diagnosed with a hepatic mass. Physical examination demonstrated right upper quadrant tenderness without any palpable masses. Liver function and viral serologies for hepatitis B and C were normal. Tumor markers demonstrated normal CA 19-9 and AFP, with mildly elevated CA-125 40 U/mL (normal <38U/mL).

Computed tomography with intravenous contrast showed a 3.2cm x 4cm x 1.8cm multiseptated cystic lesion in the left hepatic lobe and an ill-defined heterogeneous hyperdensity within the peripheral right hepatic lobe measuring 3cm x 1.3cm (Figure 1). Ultrasound-guided fine needle aspiration and core biopsy of the left hepatic lesion were inconclusive.

Further workup revealed a positive Echinococcal IgG antibody and she was started on Albendazole for a presumed hydatid cyst. After completion of therapy, she was scheduled for complete left lateral hepatic resection. However, she presented again several weeks later with progressive right upper quadrant pain. At this time repeat computed tomography redemonstrated the left hepatic mass which was unchanged in size but did not show the right hepatic lesion. Imaging also revealed a new pericardial effusion that was not present on previous imaging (Figure 2). Her liver tests were the following: AST 485 U/L (normal 10-40 U/L), ALT 308 U/L (normal 5-40 U/L), ALP 50 U/L (normal 35-104 U/L), and total bilirubin 0.5 mg/dL (normal <1.0 mg/dL). Given the concern for pericardial involvement, she urgently underwent a laparoscopic left partial hepatectomy (segment II and partial segment III). The postoperative course was uneventful. Final pathology was consistent with hepatic endometriosis (Figures 3 and 4). After 2 months of follow-up, the patient was asymptomatic and liver tests normalized. She was started on medroxyprogesterone acetate and remains well to date.

3. Discussion

Endometriosis is a common gynecologic disease characterized by the presence of endometrial glands and stroma outside of the uterus. It affects 5-15% of women of reproductive age. Pelvic endometriosis involves the ovaries, fallopian tubes, uterine ligaments, Pouch of Douglas, and surrounding peritoneum [4]. A more rare form of endometriosis, extrapelvic, includes involvement of gastrointestinal tract, urinary system, thoracic cavity, kidneys, and pancreas. The exact prevalence of extrapelvic endometriosis is unknown but is thought to present in an older population with a median age of 34-40 years [4].

There is no clear consensus or unifying theory of the exact pathophysiology of extrapelvic endometriosis. Several theories have been proposed; however no theory alone accounts for the development of extrapelvic endometriosis, suggesting a multifactorial nature to the disease.

The classic theory of retrograde menstruation proposes that reflux of endometrial fragments through the fallopian tubes during menstruation results in implantation of the peritoneal cavity. Though retrograde menstruation is a common phenomenon seen in up to 90% of healthy women, not all of these women develop extrapelvic endometriosis [5].

The coelomic metaplasia theory suggests that endometriosis develops from metaplasia of the peritoneal epithelium possibly due to environmental or genetic factors [5, 6]. The induction theory suggests that defects in embryogenesis give rise to endometrial like tissue. The Müllerian ducts give rise to the female genitourinary tract. In males, this structure dissolves under the influence of anti-Müllerian hormone. However remnants of this structure may persist and differentiate later in life into endometriotic like tissue due to the presence of excesses in endogenous or exogenous estrogen as is seen in men with chronic liver disease and prostate cancer [5, 7].

While these theories account for endometriosis within the peritoneal and pelvic cavity and provide some insight into its pathogenesis in men, they do not account for the cases of disseminated endometriosis seen in cases of lymph nodes, thoracic cavity, and liver involvement, as seen in our patient. Whether these original cells originate in the uterus or the peritoneal cavity, the theory that endometriotic tissue disseminates through lymphatic spread offers a plausible explanation for the manifestation of hepatic endometriosis [5].

A thorough review of the literature revealed 28 cases of hepatic endometriosis. Our report adds one case to this rare clinical finding; herein we present the twenty-ninth case of hepatic endometriosis. Tables 1 and 2 summarize the previously reported cases and ours, comparing the presentation, imaging, treatment, and pathologic features. In this review, the patient age ranged from 21 to 62 years, with a mean of 41.5 years. Of the 19 cases that reported parity, ten were nulligravid and nine were either uni- or multiparous, thus demonstrating that pregnancy and childbirth have no bearing on hepatic endometriosis. Six of 29 (21%) patients were postmenopausal, thus showing this condition is not limited to women of reproductive age and that the diagnosis should be considered in postmenopausal women. Twelve of 29 (41%) had a prior history of endometriosis; thus the diagnosis should not be limited only to patients with a known history of endometriosis. A significant portion of these patients had prior abdominopelvic surgery—at least half (51%) had prior pelvic surgery, and 41% had a hysterectomy, suggesting that endometrial tissue seeding during surgery later resulted in the development of hepatic endometriosis. The majority (90%) of patients described in the literature had epigastric or right upper quadrant pain; only two patients complained of characteristic cyclic pain related to menses. Only three patients were asymptomatic and their condition was diagnosed incidentally. In one peculiar case, however, the patient presented with flu-like symptoms and right shoulder pain, misdiagnosed as pneumonia initially [8].

Author, YearAgeParityPre/Post-MenopausalPrior Pelvic SurgeryHysterectomyHistory of EndometriosisSymptomsMethod of DiagnosisTreatment

Asran, 201061UnknownPostSalpingo-oophorectomyYesYesPost prandial epigastric painCT guided percutaneous liver biopsyN/A
Bouras, 201335NulliparousPreNoNoNoRecurrent, intermittent epigastric painSurgeryL lateral hepatic sectionectomy by laparotomy
Chung, 199840MultiparousPreL ovarian cystectomyNoYesAsymptomaticSurgeryCyst enucleation
De Riggi, 201627NulliparousPreNoNoNoPainless abdominal massSurgeryL hepatectomy by laparotomy
Finkel, 198621UniparousPreL fallopian tube cyst removalNoNoEpisodic sharp, epigastric pain associated with nausea and vomiting not related to mensesSurgeryCyst enucleation + Danazol
Fluegen, 201332NulliparousPreNoNoNoRUQ painSurgeryUltrasonic pericystectomy
Goldsmith, 200948NulliparousN/ASalpingo-oophorectomyYesYesRelapsing/remitting chronic RUQ painSurgeryNonanatomic resection, laparotomy, ultrasonic cyst aspiration
Groves, 200352N/AN/AOophorectomyYesNoRUQ painSurgeryR hepatectomy
Hertel, 201244N/AN/AOophorectomyPartialnoSudden onset upper abdominal painSurgeryPartial hepatectomy
Huang, 200256N/APostSalpingo-oophorectomyYesYesIntermittent epigastric pain not associated with mensesSurgeryL hepatic lobectomy by laparotomy
Inal, 200025N/APreNoNoYesPelvic pain, mass and rectal hemorrhagePercutaneous CT guided biopsyDanazol
Jelovsek, 200452UniparousPostSalpingo-oophorectomyYesYesFlu like symptoms, pleuritic chest painSurgeryLeuprolide, then resection via laparotomy
Keramidaris, 201840MultiparousPreNoNoNoAsymptomatic, incidentalSurgeryCystectomy by laparotomy
Khan, 200231N/AN/AHysterectomy, salpingo-oophorectomyYesYesMalaise, abdominal distentionSurgeryR hepatectomy + goserelin
Khan, 200261N/APostNoNoYesRUQ painSurgeryR hepatectomy
Liu, 201536UniparousPreNoNoNoRUQ pain prior to menstruationSurgeryPericystectomy
N'Senda, 200254UniparousPostHysterectomy, oophorectomyYesNoRUQ pain for 1 yearSurgeryRight hepatectomy by thoracolaparotomy
Nezhat, 2005 (1)36NulliparousN/ANoNoNoCyclic epigastric pain for 1 yearSurgeryCyst removal by CO2 laser laparoscopically
Nezhat, 2005 (2)30NulliparousN/ANoNoYesChronic pelvic pain, dysmenorrhea, and painful bowel movementsSurgeryLaparoscopic removal of liver mass
Reid, 200346NulliparousN/AOophorectomyYesyesRUQ painSurgeryR hepatectomy + goserelin
Rivkine, 201351MultiparousN/AHysterectomyYesnoEpigastric pain, vomitingSurgeryL lobectomy by laparotomy
Roesch-Dietlan, 201125NulliparousPreNoNoNoRelapsing/remitting RUQ painSurgeryIncidentally found during laparoscopic cholecystectomy, treated with danazol
Rovati, 199037NulliparousPreNoNoYesChronic, acyclic epigastric painSurgerySegmentectomy by laparotomy + Danazol
Schuld, 201139UniparousPreNoNoNoRUQ pain, coughSurgerySegmentectomy, transdiaphragmatic pulmonary wedge resection
Sherif, 201644N/APreHysterectomyYesYesRUQ pain and vomitingCT guided core biopsySegmentectomy
Tuech, 200342NulliparousN/ANoNoNoChronic, acyclic epigastric painSurgeryDeroofing & cystectomy
Verbeke, 1996 (1)34N/APreNoNoNoAcute abdomenSurgeryR hemihepatectomy
Verbeke, 1996 (2)62N/APostYesNoNoRUQ painSurgeryCholecystectomy, L hepatectomy
Rana, 201942MultiparousPreHysterectomy, L oophorectomyYesNoSevere RUQ pain, N/VSurgeryL partial hepatectomy

Author, YearUSCTMRI

Asran, 2010N/AMultiple, irregularly shaped, heterogeneous, low density lesions scattered throughout the liverN/A
Bouras, 2013N/A10cm cystic lesion with a fatty component and calcifications10cm cystic lesion with a fatty component and calcifications
Chung, 19986.4 cm x 3 cm x 2.5 cm septated cystLow density hepatic cyst, with undulating wall but no obvious septationsN/A
De Riggi, 2016N/A30cm hepatic cyts in the L love reaching segments IV, V, VIII
Finkel, 198612.5 x12x9.5 cm cystic mass in L lobe with possible septations12cm smooth-walled cystic lesion without septationsN/A
Fluegen, 2013N/AN/A9.5cm x 12cm lobulated cyst in segments IV, V, VIII
Goldsmith, 20099 x 11cm cystic mass in segment IV. The wall appeared thick with complex septae.N/A11 x13 cm cystic mass in segments IV and VIII with incomplete septations
Groves, 2003Bilateral lesions, largest in R posterior lobe 12 x 9cmN/AN/A
Hertel, 2012N/AN/A9.5x9.1x11.2cm cystic mass with a thickened wall in R hepatic lobe
Huang, 2002N/A9x6cm well circumscribed cystic mass with irregular soft tissue componentsN/A
Inal, 2000Round, well defined and heterogeneous including anechoic cystic and echogenic solid components with septations and solid componentsRound, well circumscribed heterogeneous mass with septations. Fine punctate/nodular calcifications at the periphery of the lesionA lobulated but well-demarcated subcapsular mass in the posterior segment of R lobe of the liver
Jelovsek, 2004N/A11 x7cm massN/A
Keramidaris, 2018Large cystic lesion between L and R lobe of the livernoneMultiseptated cystic lesion 10.3x7.8x7.7 cm in the L lobe, segments IV, II, III
Khan, 2002Large mass in R lobe and small in L lobelarge non-enhancing lobulated mass in R lobe and mass in L lobe; portal vein thrombosisN/A
Khan, 2002N/ALarge mass occupying the entire R lobeN/A
Liu, 20156 cm lesion in L lobe (segment III)6.5 x6cm loculated cystic lesion in segment III, wall with thick complex septaeN/A
N'Senda, 2002N/AHuge heterogeneous hypodense mass partially enhanced after contrast injection; cystic changes w/ fluid levelsHeterogeneous mass on both T1-, T2- and T1-weighted image after gadolinium injection; cystic changes w/ fluid levels
Nezhat, 2005 (1)3-cm hepatic cyst in the far caudal aspect of the right lobe of the liver3-cm hepatic cyst in the far caudal aspect of the right lobe of the liverN/A
Nezhat, 2005 (2)Normal findingsN/ANormal findings
Reid, 200310cm mass with echogenic margins and internal debrisLow density lesionN/A
Rivkine, 201380 x 75 mm intraparenchymal hepatic necrotic tumorHypovascularized, cystic mass in the L liver lobe with hemorrhagic contents, no septationsCystic mass in segments II and III
Roesch-Dietlan, 2011No masses, multiple small gallstonesN/AN/A
Rovati, 199010cm cystic mass with septationsMultilocular 10cm cyst with fine calcifications in the wallN/A
Schuld, 2011N/AN/A6.8 x2.3 cm in diameter in the right basal lung and peripheral bile ducts
Sherif, 20163cm complex cyst in R lobes3cm well defined hypodense subcapsular lesion in R lobe with heterogeneous peripheral enhancement in the venous and delayed phasesSubcapsular partially cystic focal lesion with possible hemorrhagic content and heterogeneous peripheral enhancement
Tuech, 2003N/A24cm smooth walled cystic lesion without septations in the R lobeN/A
Verbeke, 1996 (1)N/ACystic tumor in R lobe of liver, with reactive enlargement of L hepatic lobeCystic tumor in R lobe of liver, with reactive enlargement of L hepatic lobe
Verbeke, 1996 (2)Cyst (12 x 10 x 7.5 cm) in the left liver lobe, located near the gallbladder and the liver hilus, which partially compressed the proximal ductus choledochus.Cyst (12 x 10 x 7.5 cm) in the left liver lobe, located near the gallbladder and the liver hilus, which partially compressed the proximal ductus choledochus.N/A
Rana 2019N/A3.2cm x 4cm x 1.8cm multi-septated cystic lesion in the left hepatic lobeN/A

Abdominal US, CT, and MRI are the imaging modalities most frequently used. Typical US findings include well-defined cystic masses with solid components and septations. The majority of CT reports show low density, heterogenous cystic lesions that are either nonenhancing or poorly enhancing. Calcifications have been reported along with irregular soft tissue components but can be variable. Finally, MRI usually demonstrates signal intensity on T1- and T2-weighted images, similar to that of normal endometrium. However, because endometrial implants can exhibit various degrees of hemorrhage due to hormonal stimulation, implants may demonstrate a spectrum of appearances depending on the age of the hemorrhage but can be variable [9].

Of the 19 cases that reported lab values, 79% had normal liver tests. Three cases exhibited mild transaminitis, and a fourth case, ours, had markedly elevated transaminases with AST 485 U/L and ALT 305 U/L.

Excluding two, all patients underwent surgery for treatment. The most common surgery was hepatectomy via laparotomy (59%). Other surgical techniques included ultrasonic cyst manipulation. In the two nonsurgical cases, those patients were treated with danazol alone [9, 10]. Tumor size ranged from 1 to 30cm, with mean tumor size 9.8 cm.

The final diagnosis can only be made by histopathologic analysis. The differential diagnosis includes both benign and malignant conditions, as echinococcal cyst, abscess, hematoma, cystadenoma, and malignant cystic neoplasm, such as cystadenocarcinoma or metastatic disease. Method of diagnosis was largely by histologic analysis after surgery in 90% of cases. Only four patients underwent CT-guided percutaneous biopsy prior to surgery, with only three yielding a diagnosis of endometriosis and one case, ours, yielding inconclusive results. Histopathologic examination of the tumors was consistent with endometriosis as evidenced largely by fibrous capsules with internal epithelial lining containing endometrial glands and stroma. Furthermore, although malignant transformation of endometriosis is a rare event, occurring commonly in the ovary, there were two cases of malignancy reported, one adenosarcoma and one low-grade endometrial stroma sarcoma [11, 12]. Of the eight cases that reported on immunostaining, all eight cases were positive for estrogen and progesterone receptor, consistent with endometriosis. Five cases reported on further immunohistochemistry markers that included CD10 (in endometrial tissue) and/or CK7 (in glandular tissue) [1317].

Hepatic endometriosis is a rare form of endometriosis. This unusual condition offers several diagnostic challenges but should be considered in the differential in any female presenting with a solitary hepatic mass, regardless of age and previous history of endometriosis.

Verbal informed consent was obtained from the patient(s) for their anonymized information to be published in this article.


Institutional Mailing Address: LAC + USC Medical Center, 1200 N. State St., CT A7D - GME, Los Angeles, CA 90033.

Conflicts of Interest

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Authors’ Contributions

Prachi Rana and Shida Haghighat equally contributed to the collection of the data and writing of the manuscript. Hyosun Han reviewed and edited the manuscript.


We thank Dr. Yangling Ma and Dr. Esteban Gnass for the review and interpretation of the pathologic reports.


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Copyright © 2019 Prachi Rana et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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