Impact of Nrf2 overexpression on cholangiocarcinoma treatment and clinical prognosis

Purpose@#Nrf2 regulates antioxidant protein expression and protects against drug toxicity and oxidative stress, whereas Keap1 controls Nrf2 activity. The Keap1-Nrf2 pathway affects the prognosis of various cancers, however, its effect on cholangiocarcinoma chemoresistance and prognosis remains unclear. This study aimed to determine whether the Keap1-Nrf2 pathway affects chemoresistance and prognosis of distal cholangiocarcinoma. @*Methods@#We investigated the correlation between Nrf2 and Keap1 expression and clinical characteristics and prognosis in 91 patients with distal cholangiocarcinoma who underwent curative surgery. Immunohistochemical staining was performed on paraffin blocks using primary antibodies against Nrf2 and Keap1. The relationship between Keap1 and Nrf2 protein expression levels, and clinical characteristics and prognosis was examined. @*Results@#Nrf2 expression was not associated with overall survival in patients who did not receive adjuvant chemotherapy (P=0.994). Among patients receiving adjuvant chemotherapy, the Nrf2 low expression group had a significantly longer median overall survival than the Nrf2 high expression group in Kaplan-Meier survival analysis (P=0.019). In multivariate analysis, high expression of Nrf2 was confirmed as an independent poor prognostic factor in the group receiving adjuvant chemotherapy (P=0.041). @*Conclusion@#This study suggests that Nrf2 overexpression reduces the efficacy of adjuvant chemotherapy in distal cholangiocarcinoma.

Risk factors of recurrence following common bile duct exploration for choledocholithiasis

Purpose@#The purpose of this study was to investigate the recurrence factors of choledocholithiasis after common bile duct (CBD) exploration. @*Methods@#From January 2000 to December 2018, we retrospectively reviewed 253 patients who underwent CBD exploration surgery. We excluded 100 cases who had residual stone, combined major surgery, or follow-up loss after surgery. Total of 153 patients were included, and we investigated the recurrence factors of choledocholithiasis. Various variables such as patients’ demographics, gallstones, preoperative endoscopic treatment, and laboratory data were analyzed to find factors related to recurrent choledocholithiasis. @*Results@#The median follow-up period was 20.6 months (range 4.7–219 months), and 27 patients (17.6%) had experienced recurrent choledocholithiasis. Univariate analysis showed that the following variables were associated with recurrence of choledocholithiasis; preoperative leukocytosis (white blood cell ≥ 11,000/µL), open procedure, T tube insertion, long hospital duration, and long operation time. Logistic regression multivariate analysis identified preoperative leukocytosis (odds ratio [OR], 3.43; 95% confidence interval [CI], 1.21–9.73; p = 0.021), open procedure (OR, 5.54; 95% CI, 4.73–6.35; p = 0.037), and T-tube insertion (OR, 2.82; 95% CI, 1.04–7.65; p = 0.042) as independent predictors of recurrent choledocholithiasis. @*Conclusion@#Because of delayed recurrence of choledocholithiasis, it is recommended to continue follow-up of patients after CBD exploration surgery. Laparoscopic surgery was observed to be associated with a reduction in recurrence. The preoperative leukocytosis and clinical conditions in which open surgery is performed could be associated with recurrence of choledocholithiasis. However, further study is necessary to validate the result.

Nutritional risk factors are associated with postoperative complications after pancreaticoduodenectomy

PURPOSE: The purpose of this study was to investigate whether the indicators of nutritional risk screening tool are associated with postoperative complications following pancreaticoduodenectomy (PD). METHODS: We investigated whether nutritional risk is associated with postoperative complications based on the medical records of 128 patients who underwent PD from 2010. The tool was composed of 6 risk factors: albumin, total lymphocyte count, body mass index, weight loss, dietary intake loss, and nutritional symptoms. The patients were divided into 2 groups: a nutritional risk group and a nonrisk group. The rates of general complications and postoperative pancreatic fistula (POPF) were investigated according to this nutritional status. RESULTS: There were 65 patients who did not have any risk factors. However, 63 patients had one risk factor or more. In the nonrisk group, the overall complication rate and serious complication rate were 30.8% and 15.4%, respectively. If there were one or more risk factors, the overall and serious complication rates were 59.5% and 41.3%, respectively (P = 0.001 and P = 0.001, respectively). The rate of clinically relevant POPF (grade B or C) was 9.2% in the nonrisk group. However, this rate was 23.8% in the NRS risk group (P = 0.029). In multivariate analysis, the NRS risk group was a significant factor of clinically relevant POPF (odds ratio, 9.878; 95% confidence interval, 1.527–63.914; P = 0.016). CONCLUSION: There were statistically significant associations between complications and nutritional indicators. A comprehensive analysis of nutritional parameters will help predict postoperative complications.

Spleen Preservation in Laparoscopic Distal Pancreatectomy for Solitary Pseudopapillary Tumors Is Oncologic Safe

Pancreatic solid pseudopapillary neoplasm (SPN) is a rare tumor that is mostly benign, but a surgical resection is recommended. Pancreatic SPN has a relatively well-demarcated margin and lymph node metastases are rare. The prognosis of pancreatic SPN is relatively good. Therefore, minimally invasive surgery (MIS) is often available. When a distal pancreatectomy is performed for SPN located at the pancreatic body or tail, it is believed to preserve the spleen. MIS with preservation of spleen not only helps the patient recover after surgery, but also does not worsen the oncology results compared to open surgery with or without a splenectomy. Recently, robotic surgery has expanded gradually. Therefore, it is expected that MIS with spleen preservation will become more common for pancreatic SPN.

Bile duct segmental resection versus pancreatoduodenectomy for middle and distal common bile duct cancer

PURPOSE: To compare survival outcomes between bile duct segmental resection (BDR) and pancreatoduodenectomy (PD) for the treatment of middle and distal bile duct cancer. METHODS: From 1997 to 2013, a total of 96 patients who underwent curative intent surgery for middle and distal bile duct cancer were identified. The patients were divided into 2 groups based on the type of operation; 20 patients were included in the BDR group and 76 patients were in the PD group. We retrospectively reviewed the clinical outcomes. RESULTS: The number of lymph nodes (LNs) was significantly greater in patients within the PD group compared to the BDR group. The total number of LNs was 6.5 ± 8.2 vs. 11.2 ± 8.2 (P = 0.017) and the number of metastatic LNs was 0.4 ± 0.9 vs. 1.0 ± 1.5 (P = 0.021), respectively. After a median follow-up period of 24 months (range, 4–169 months), the recurrence-free survival of the PD group was superior to that of the BDR group (P = 0.035). In the patients with LN metastases, the patients undergoing PD had significantly better survival than the BDR group (P < 0.001). CONCLUSION: Surgeons should be cautious in deciding to perform BDR for middle and distal common bile duct cancer. PD is recommended if LN metastases are suspected.

Pancreatitis-Mimicking Pancreatic Cancer with Duodenal Obstruction: A Case Report

Pancreatic cancer tends to be delayed in diagnosis because of the lack of early symptom and less than 20% of patients present with resectable masses. A 95-year-old male visited due to recurrent abdominal pain and vomiting. About 2 years ago, a polypoid lesion was detected at the post-bulbar area on esophagogastroduodenoscopy for medical check-up. Endoscopic biopsy noted chronic inflammation with glandular atypia. On the CT scan, there was an intraluminal polypoid mass lesion with mixed hypodensity at the duodenal second portion. Ultrasound guided biopsy targeting the hypodense lesion was performed and revealed chronic pancreatitis. The vomiting persisted and the patient received a palliative gastrojejunostomy. Twenty-five days after gastrojejunostomy, jaundice occurred and an ill-defined mass at the pancreas head was noted on the CT. Pylorus preserving pancreatoduodenectomy was performed and a 3.5 cm sized, moderate to poorly differentiated ductal adenocarcinoma of pancreas head was diagnosed. Nineteen days after operation, the patient was discharged in good condition.

The safety and risk factors of major hepatobiliary pancreatic surgery in patients older than 80 years

PURPOSE: Recently, the number of elderly patients has increased due to a longer life expectancy. Among these elderly patients, more octogenarians will be diagnosed with major hepatobiliary pancreatic (HBP) diseases. Therefore, we need to evaluate the safety and risk factors of major HBP surgery in patients older than 80 years. METHODS: From January 2000 to April 2015, patients who underwent major HBP surgery were identified. The patients were divided into 2 groups according to their age at the time of surgery: Group O (≥80 years) and group Y (<80 years). The patient characteristics and intra- and postoperative outcomes were retrospectively investigated in the 2 groups. RESULTS: The median age was 84 years (range, 80–95 years) in group O and 61 years (range, 27–79 years) in group Y. group O had worse American Society of Anesthesiologists (ASA) physical status (ASA ≥ III: 23% vs. 7%, P = 0.002) and was associated with a higher rate of hypertension and heart problems as comorbidities. There were significant differences in albumin and BUN, favoring group Y. The length of intensive care unit stay was longer in group O, whereas the overall complication and mortality rates did not show statistical difference. But, there was a significant difference in systemic complication of both Clavien-Dindo classification grade ≥II and ≥III as complications were divided into surgical site complication and systemic complication. CONCLUSION: Major HBP surgery can be performed safely in patients older than 80 years if postoperative management is appropriately provided.

Incidentally Discovered Aldosterone and Cortisol Cosecreting Adrenal Cortical Adenoma / 이화의대지

A substantial proportion of adrenal incidentalomas demonstrates subtle hormonal hypersecretion; however, adenomas that cosecrete aldosterone and cortisol are rare. We here report a case of an adrenal mass that was incidentally detected on a computed tomography scan in a 57-year-old man. The patient had a 10-year history of diabetes mellitus and a 5-year history of hypertension. Evaluation revealed hyperaldosteronemia with an elevated plasma aldosterone-to-renin ratio, hypokalemia, unsuppressed cortisol after dexamethasone administration, and elevated urinary free cortisol concentration. The appearance of the right adrenalectomy specimen indicated adrenal adenoma. Postoperatively, the blood glucose and blood pressure control improved and the urinary cortisol and aldosterone-to-renin ratio normalized. A complete endocrine evaluation in patients with incidentally discovered adrenal masses should be performed, even if the patient has a long-standing history of hypertension and diabetes, to avoid any postoperative adrenal crises.

Long-term results of laparoscopic common bile duct exploration by choledochotomy for choledocholithiasis: 15-year experience from a single center

PURPOSE: The aim of this study is to assess the long-term results of laparoscopic common bile duct exploration (LCBDE) and validate its effectiveness as a primary treatment modality for CBD stone. METHODS: A retrospective review of the medical records of 157 patients who underwent LCBDE from 1997 to 2011 was conducted. All LCBDE were performed by choledochotomy. Clinical demographics, operative outcome, recurrence rate of CBD stones, and long-term bile duct complications were analyzed. The mean follow-up period was 51.9 months. RESULTS: LCBDE was completed in 152 patients (96.8%) and 5 patients (3.2%) had open conversion. The male/female ratio was 78/79 and mean age was 67.3 years. Stone clearance was successful in 149 of 152 patients (98.0%). Nonlethal complications were noted in 11 patients (7.2%), including bile leakage in 6 patients (3.9%). Recurrent CBD stones developed in 9 of 152 patients (5.9%). Preoperative endoscopic sphincterotomy (P = 0.492) and choledochotomy repair type (T-tube drainage vs. primary closure, P = 0.740) were not significantly related to stone recurrence. There were no signs of any type of biliary injury or stricture observed in any of the patients during the follow-up period. CONCLUSION: LCBDE can be performed without increased risk of long-term complications such as bile duct stricture and recurrent CBD stones. LCBDE is a safe and effective treatment option for choledocholithiasis in terms of long-term outcome as well as short-term outcome.

Long-term Clinical Results of Laparoscopic Splenectomy for Surgical Disease of the Spleen: Recent Outcomes

PURPOSE: Laparoscopic splenectomy (LS) is one method for treatment of various diseases of the spleen, especially hematological conditions. However, few recent long-term follow-up results have been reported. The purpose of this study is to evaluate the outcome of patients in a single institution who recently underwent LS and to analyze their long-term follow-up results. METHODS: Of 366 splenectomies, this study was conducted as a retrospective review of 52 patients who underwent LS for treatm ent of hematological or primary diseases of the spleen from January 1998 to October 2011. The data included age, sex, pathological diagnosis, operative time, postoperative hospital stay, rate to open conversion, perioperative transfusion, morbidity, mortality, and relapse. We analyzed outcomes of variable results through long-term follow-up. RESULTS: The mean follow-up period was 84 months (range, 4~147 months). The most common indication for LS was immune thrombocytopenic purpura (ITP). The median postoperative hospital stay was eight days (range, 3~28 days). Mean operative time was 203 minutes (range, 115~475 minutes). Two patients underwent open conversion. Thirty eight patients received perioperative transfusions. The mean spleen weight was 294.9 g (range, 31~2,564 g). The overall morbidity rate was 5.8% and one patient experienced relapse. Of the 28 patients with ITP, 89.3% responded to LS. CONCLUSION: LS should be one of the best treatment options regardless of splenomegaly and spleen-associated diseases. In particular, for patients with ITP, LS has shown very effective long-term follow-up results. Therefore, LS should be more actively considered as an early treatment option in surgical disease of the spleen, such as ITP.

Current Status of Laparoscopic Liver Resection in Korea

Since laparoscopic liver resection was first introduced in 2001, Korean surgeons have chosen a laparoscopic procedure as one of the treatment options for benign or malignant liver disease. We distributed and analyzed a nationwide questionnaire to members of the Korean Laparoscopic Liver Surgery Study Group (KLLSG) in order to evaluate the current status of laparoscopic liver resection in Korea. Questionnaires were sent to 24 centers of KLLSG. The questionnaire consisted of operative procedure, histological diagnosis of liver lesions, indications for resection, causes of conversion to open surgery, and postoperative outcomes. A laparoscopic liver resection was performed in 416 patients from 2001 to 2008. Of 416 patients, 59.6% had malignant tumors, and 40.4% had benign diseases. A total laparoscopic approach was performed in 88.7%. Anatomical laparoscopic liver resection was more commonly performed than non-anatomical resection (59.9% vs 40.1%). The anatomical laparoscopic liver resection procedures consisted of a left lateral sectionectomy (29.3%), left hemihepatectomy (19.2%), right hemihepatectomy (6%), right posterior sectionectomy (4.3%), central bisectionectomy (0.5%), and caudate lobectomy (0.5%). Laparoscopy-related serious complications occurred in 12 (2.8%) patients. The present study findings provide data in terms of indication, type and method of liver resection, and current status of laparoscopic liver resection in Korea.

Selection of surgical treatment types for intrahepatic duct stones

BACKGROUNDS/AIMS: Complete elimination of intrahepatic duct (IHD) stones is difficult and IHD stone disease is frequently associated with various complications, recurrence and sometimes cholangiocarcinoma. Therefore, we analyzed the long-term surgical results and evaluated the management currently considered appropriate. METHODS: Overall 110 patients who had been diagnosed with benign IHD stone disease and who underwent surgical treatment were enrolled in this study. The patients were categorized into three groups according to the type of surgery performed; liver resection (LR) group, intrahepatic duct exploration (IHDE) group and hepaticoenterostomy (HE) group. We compared and analyzed the results of these three groups. RESULTS: The number of cases in the LR group, IHDE group and HE group were 77, 25 and 8 respectively. The LR group required a longer operation time (p=0.000), more frequent transfusion (p=0.028) and had higher morbidity (p=0.049). However, the LR group had a higher clearance rate (90.9%) (p=0.000) than the other groups. In addition, there were a total of 22 cases of IHD stone recurrence during the follow-up, but there was no statistically significant difference among the three groups. The location of IHD stones was related to a risk factor for incomplete stone removal, but not for recurrence. CONCLUSION: The fundamental principle for the treatment of IHD stone disease should be liver resection. However, it can lead to a longer operative time and higher rate of complications than the other procedures. There is also no difference in the IHD stone recurrence rate among the procedures. Therefore, these alternative and minor procedures could also be taken into account for patients with poor preoperative condition.

Laparoscopic common bile duct exploration for elderly patients: as a first treatment strategy for common bile duct stones / 대한외과학회지

PURPOSE: Common bile duct (CBD) stone is a relatively common disease in elderly patients. There have been many reports about the efficacy and safety of LCBDE. But for elderly patients, only a few studies about its efficacy and safety exist. The aim of this study is to evaluate the efficacy, safety and the surgical outcome of LCBDE in patients who are 70 years or older and compare the results with those of the younger group. METHODS: From January 2000 to November 2009, 132 patients underwent LCBDE. We divided these patients into two groups according to age and conducted a retrospective analysis. The elderly group included patients who were 70 years old or older (n = 64), and the younger group included those who were younger than 70 (n = 68). The elderly group was compared to the younger group with respect to their clinical characteristics, operation time, postoperative hospital stay, open conversion rate, first meal time, postoperative complication, recurrence rate and mortality. RESULTS: The elderly group showed high American Society of Anesthesiologists score (2.2 vs. 1.9) (P = 0.003), preoperative morbidity (47 vs. 29) (P 0.05). CONCLUSION: LCDBE is a safe and effective treatment modality for CBD stones not only for younger patients but also for elderly patients.

Combined Operation of Laparoscopic Cholecystectomy during Laparoscopic Appendectomy in Patient with Chronic Gallbladder Disease

PURPOSE: Laparoscopic cholecystectomy (LC) for asymptomatic gallbladder disease has been increasing since 1990 due to the development of LC technique. Moreover, it might be considered when someone with asymptomatic gallbladder disease needs emergency operation due to acute abdomen such as acute appendicitis. The aim of this study is to identify the efficacy of concurrent laparoscopic appendectomy with cholecystectomy. METHODS: From January 1997 to June 2009, 40 patients underwent laparoscopic appendectomy with cholecystectomy at Ewha Womans University Mokdong Hospital. 40 patients were enrolled in this study. We analyzed, retrospectively, clinical and diagnostic information, time and open conversion rate in operation, length of postoperation hospital stay, and complication rate. RESULTS: The mean age was 44.53+/-15.34 and male to female ratio was 1 to 1.86. 5% of all patients underwent abdominal surgery and 20% of all patients had associated diseases. Most common associated symptoms and final diagnosis were right lower abdominal pain (72.5%) and simple gallbladder stone (62.5%). Mean operation time was 98.75+/-33.14 min. and there was no open conversion. Mean length of postoperation hospital stay was 5+/-2 days. There was one (2.5%) postoperative complication. CONCLUSION: Combined operation of laparoscopic cholecystectomy during laparoscopic appendectomy can be a good modality for patients with chronic gallbladder disease.

A Case of Gastric Rupture after Cardiopulmonary Resuscitation by a Bystander

The presence of a bystander who can implement cardiopulmonary resuscitation would appear to increase chances of survival. However, there have been many reported complications associated with bystander CPR. Gastric rupture is a rare complication following cardiopulmonary resuscitation. An incidence of 0.1% has been reported in the literature. The majority of reported cases have been associated with inappropriate airway management or esophageal intubation. Gastric rupture can occur during chest compressions when the stomach is overinflated due to difficult airway management or esophageal intubation. Here we present the case of a patient with sudden cardiac arrest who experienced gastric rupture and pneumoperitoneum after bystander cardiopulmonary resuscitation.

Comparison of Long-term Follow-up Results of Open Common Bile Duct Exploration and Laparoscopic Common Bile Duct Exploration in Common Bile Duct Stone Disease

PURPOSE: Currently, in treatment of symptomatic common bile duct (CBD) stone, the endoscopic retrograde cholagiopancreatography (ERCP) is usually carried out as an initial evaluation and removal of CBD stones. But, many cases necessitate surgical exploration of CBD and stone removal. Recently, laparoscopic CBD exploration (LCBDE) procedure is increasing due to the high success rate of stone clearance with the easy use of a choledochoscope and the advantage of less aggressive laparoscopic procedure properties. We reviewed the long-term results and efficacy of LCBDE in relation to traditional open CBDE results to demonstrate the superiority of LCBDE. METHODS: From July 1997 until July 2007, 189 consecutive patients with CBD stones were enrolled in a retrospective study. Those patients were divided into two-groups: Laparoscopic CBDE (group L) and Open CBDE (group O), and compared the patients' clinical characteristics, postoperative outcomes and follow up data. RESULTS: Of 189 patients who underwent successful CBD exploration, 66 (34.9%) were open group and 123 (65.1%) were laparoscopic group. Stone clearance rate was 100% in both groups. The mean operation time, incidence of postoperative complications and hospital days had no significant difference between the two groups (P>0.05). The cases of T-tube insertion and recurrence of CBD stone were significantly more in open group (P<0.05). CONCLUSION: On investigation of long-term follow up data of the two groups, CBD stone recurrence cases were significantly fewer in laparoscopic group. The lower incidence of complication and stone recurrence is evidence of the superiority of laparoscopic procedure in CBD exploration and removal of CBD stones.

Comparison of Primary Closure and T-tube Drainage following Laparoscopic CBD Exploration

PURPOSE: Laparoscopic common bile duct exploration (LCBDE) has traditionally been accompanied by T-tube drainage. However, patients must carry it for several weeks and often suffer problems related to the T-tube. So, primary closure of CBD has been proposed as a safe and effective alternative to T-tube placement after laparoscopic choledochotomy. The aim of this study was to compare primary closure versus T-tube drainage after LCBDE. METHODS: Between January 2000 and December 2005, 63 patients suffering from choledocholithiasis underwent LCBDE successfully through choledochotomy. Those patients were devided into two groups; primary closure group (group P) and T-tube placement group (group T). Patients' clinical characeristics, postoperative outcome and follow up data were compared between the two groups. RESULTS: Of 63 patients, 30 (48.6%) had primary closure of the choledochotomy and 33 (52.4%) had T-tube drainage. Stone clearance rate was 100% in both groups. The mean operation time and the incidence of postoperative complications had no significant difference between the two groups. The mean postoperative hospital stay (8.8 vs. 16.4 days, P<0.001) was significantly shorter in the P group compared to the T group. Each group had one recurrent CBD stone. None of both groups showed symptoms or signs associated with CBD stricture during the follow up period. CONCLUSION: Primary closure of choledochotomy after LCBDE can prevent the disadvantages associated with T-tube and lead to a shorter hospital stay. Therefore, primary closure should be considered as a safe alternative method after LCBDE.

A Case of Cystic Lymphangioma of Small Bowel Mesentery Presented with Small Bowel Volvulus

A cystic lymphangioma is a rare benign tumor that arises in an organ with a large number of lymphatics. It arises in patients of all ages and has variable presentations. An abdominal cystic lymphangioma most commonly occurs in the intestinal mesentery, with the retroperitoneum being the second location of choice, and it causes abdominal pain, hematochezia, intussusceptions and protein-losing enteropathy. If the cystic lymphangioma presents with symptoms, surgical excision is required for treatment and diagnosis. We report here a case of cystic lymphangioma of the small bowel mesentery that presented with a small bowel volvulus, along with a brief review of the literature.

The Outcome of Laparoscopic Common Bile Duct Exploration (LCBDE) for the Choledocholithiasis that ERCP Failed to Remove

PURPOSE: With the advent of endoscopic technology, ERCP (endoscopic retrograde cholangiopancreatography)/ES (endoscopic sphincterotomy) has become the main treatment for CBD stones. However, when ERCP fails to remove CBD stones, it remains unclear whether laparoscopic treatment is an alternative or not. The aim of this study was to investigate the outcome of LCBDE for the management of difficult choledocholithiasis. METHODS: This study was a retrospective analysis of 68 LCBDE cases that were performed at the Ewha Womans University School of Medicine, Mokdong Hospital from January, 2000 to March, 2006. Group A was defined that primary LCBDE was performed without ERCP/ES. Group B was defined that secondary LCBDE was performed after ERCP/ES had failed. The operative outcomes and postoperative complications were compared between the two groups. RESULTS: Primary LCBDE was performed for 33 patients (Group A) and secondary LCBDE after failure of ERCP/ES was performed for 35 patients (Group B). No significant differences in gender, mean age and associated diseases were noted between the two groups. The stone clearance rate was 100% for both groups. There were no significant differences between the two groups for the mean operative time (201.5 min for Group A vs 188.7 min for Group B: P=0.415), the open conversion rate (9.1% vs 0%, respectively: P=0.068), the complication rate (12.1% vs 8.6%, respectively: P=0.630) and the duration of the hospital stay (14.3 days vs 11.9 days, respectively: P=0.169). The recurrence rate along with a mean follow-up of 24 months showed no significant difference between the two groups (12.1% vs 2.9%, respectively). CONCLUSION: When ERCP is impossible or stone retrieval is incomplete, LCBDE is an alternative treatment for difficult CBD stones.

Retroperitoneal Lymphangioleiomyomatosis without Pulmonary Feature

Lymphangioleiomyomatosis (LAM) is discovered mainly in the pulmonary area, and the typical presenting symptoms include progressive dyspnea, pneumothorax, and chylous pleural effusion. An initial presentation of LAM with extrapulmonary symptoms is extremely rare. Most reports and reviews have concentrated on the pulmonary symptoms and radiological image of LAM. Some of reports focused on the abdominal abnormalities or retroperitoneal abnormalities in addition to pulmonary features. However, LAM without pulmonary features is extremely rare. We encountered a 36-year-old woman with a retroperitoneal mass measuring 7 cm in the anterior aspect of the right adrenal gland. The patient did not have any pulmonary symptoms or radiological abnormalities, and underwent a laparoscopic excision. The tumor was diagnosed histologically as a lymphangioleiomyomatosis. LAM without pulmonary feature is unusual case. We report this case with a review of the relevant literature.