Abstract
Abstract
Introduction
E
This article reports a case of intraparenchymal endometriosis of the liver in a premenopausal female, presenting with a 1-year history of intermittent right shoulder pain.
A literature search revealed only 19 cases of hepatic endometriosis in English-language publications.
A preoperative diagnosis of hepatic endometriosis is extremely difficult, because of the absence of specific clinical and radiological characteristics. A diagnosis of hepatic endometriosis is usually established after surgery.
Case
A 38-year-old female, was referred to the Aretaieion Hospital, University of Athens, in Athens, Greece, because she had a 3.5×4.4 mass in segments 7 and 8 of the liver. She had a 5-year history of pelvic endometriosis, and her medical history included a cesarian 7 years prior. During the last 16 months, she had right-shoulder pain, which was treated by orthopedics specialists with painkillers and anti-inflammatory agents. A detailed history revealed that this patient's shoulder pain was associated with menstruation.
A chest computed tomography (CT) scan showed a mass in the liver and, subsequently, magnetic resonance imaging (MRI) of the liver was performed. The latter revealed a 4.4×3.8 cystic mass in segments 7 and 8 of the liver infiltrating the right diaphragm (Figs. 1 and 2). Therefore, the right-shoulder pain was attributed to the infiltration of the right diaphragm and thus the irritation of the phrenic nerve. The patient's blood test results and and tumor markers were unremarkable. At exploratory laparotomy, a tumor at the diaphragmatic portion of the liver infiltrating the diaphragm was found. A wedge excision of the tumor was performed with clear margins en bloc with part of the right diaphragm (Figs. 3 and 4). The diaphragm was closed with 2-0 nonabsorbable continuous sutures and a suction drain was placed under the diaphragm.
Intraoperative view of the tumor (white arrow).
Part of the diaphragm and wedge-liver excision performed.
The histology report confirmed a mixed cystic and solid mass, with an endometrial-type epithelium, positive to estrogen receptors and prostaglandin receptors, and cholangian differentiation in some sites. The diaphragm excised was infiltrated by endometrial tissue, while the liver margins were free of pathology.
Results
The patient was discharged on the second postoperative day and remains free of symptoms 6 months later, as of this writing.
Discussion
Endometriosis, a common clinical condition most commonly found in the pelvis, has been reported in ∼ 6%–10% of women of reproductive age 7 and in ∼ 2.5% of postmenopausal women. 8 Atypical endometriosis, in which endometrial tissue is found in extrapelvic regions, 9 is rare. Endometrial tissue deposits have been described in almost all organs of the human body and, even very rarely, in males. 10 Several remote sites of endometriosis have been described—including the omentum, GI tract, peritoneum, operative scars, lymph nodes, umbilicus, skin, lungs, pleura, urinary bladder, kidneys, and pancreas.2–6 The cause of endometriosis, first described by Rokitansky in 1860 11 in the pelvis, remains largely uncertain. The two major theories involve either the implantation of endometrial cells (implantation theory) 12 or the metaplasia of the peritoneal epithelium (coelomic metaplasia theory) 13 in the region of occurrence. Each theory, individually, fails to explain all cases of endometriosis.
Intraparenchymal hepatic endometriosis is an extremely rare clinical entity. Finkel et al. described the first case in 1986 in a 21-year-old woman who complained of epigastric pain, nausea, and vomiting, and, was found to have an endometrial cyst measuring 13 cm located in the left liver lobe. 14
There are altogether 19 cases of hepatic endometriosis previously described in the literature. 15 The current case had right-diaphragm infiltration. In all previously reported cases, only 1 of the patients presented with cyclical pain in the upper right abdominal quadrant accompanying menstruation. In all other cases, patients presented with tenderness or pain, even jaundice, but no obvious connection to the menstrual cycle.
This lack of a “typical” clinic presentation makes it difficult to diagnose extragonadal endometriosis without histopathology. Twelve cases (68%) reported a previous history of endometriosis, mostly in a typical pelvic location, while 7 patients (24.1%) had no history of endometriosis at all. Thus, the diagnosis of this condition should not be limited to patients with known histories of endometriosis. In addition, 6 of 19 patients (31%) were postmenopausal, thus showing that this condition is not limited to women of reproductive age. Only 7 (36%) of the previously described 19 cases were diagnosed preoperatively. In the current case, there was a known history of endometriosis with a past history of a cesarian, and the patient's symptoms were associated with menstruation. The condition was misdiagnosed initially and was only revealed by the chest CT and confirmed preoperatively by MRI.
Conclusions
Intrahepatic endometriosis is a rare condition. The diagnosis may only be reached preoperatively by high clinical suspicion of the condition, when symptoms are associated with menstruation. Radiographic imaging techniques and transhepatic biopsy will confirm the diagnosis. Surgery with resection of any endometriosis lesion is the treatment of choice for patients and leads to permanent relief of symptoms.
Footnotes
Disclosure Statement
All of the authors declare that they have no conflicts of interest.