Management of Mucinous Cystic Neoplasms of the Liver.

BACKGROUND: Mucinous cystic neoplasms of the liver (MCN-L) including biliary cystadenomas (BCA) and biliary cystadenocarcinomas (BCAC) are rare cystic lesions that comprise less than 5% of all liver cysts and affect only a small subset of individuals. We herein review the current evidence regarding the clinical presentation, imaging characteristics, tumor markers, pathological findings, clinical management, and prognosis of MCN-L. METHODS: A comprehensive review of the literature was performed using MEDLINE/Pubmed and Web of Science databases. In PubMed, the terms "biliary cystadenoma," "biliary cystadenocarcinoma," and "non parasitic hepatic cysts" were queried to identify the most recent data on MCN-L. RESULTS: US imaging, CT, and MRI, as well as consideration of clinicopathological features, are required to appropriately characterize and diagnose hepatic cystic tumors. BCA are premalignant lesions and cannot be reliably differentiated from BCAC based on imaging alone. As such, both types of lesions should be treated with margin-negative surgical resection. Following surgical resection, recurrence is fairly low among patients with BCA and BCAC. Despite having worse long-term outcomes than BCA, the prognosis following surgical resection of BCAC still remains more favorable than other primary malignant liver tumors. CONCLUSION: MCN-L are rare cystic liver tumors that include BCA and BCAC, which can be difficult to differentiate based on imaging alone. Surgical resection remains the mainstay of management for MCN-L with recurrence being generally uncommon. Future multi-institutional studies are still required to better understand the biology behind BCA and BCAC to improve the care of patients with MCN-L.

[Two Operations for Intrahepatic Recurrence of Biliary Cystadenocarcinoma-A Long-Term Survivor].

We report a case of biliary cystadenocarcinoma in which long-term survival was achieved after 2 operations for intrahepatic recurrence. A 72-year-old man with biliary cystadenocarcinoma located mainly in segment 3 of the liver underwent left hepatectomy, extrahepatic bile duct resection, and lymph node dissection. Seven years and 9 months after the initial resection, he underwent partial liver resection(segment 5)for intrahepatic recurrence detected by computed tomography. Fifteen years and 7 months after the initial resection, he underwent repeat partial resection of the liver(segment 5)for intrahepatic recurrence. Histologically, these tumors were confirmed to be recurrence of biliary cystadenocarcinoma. He remains alive and well with no further recurrence 21 years and 6 months after the initial resection. This case and a literature review suggest that hepatic resection is a useful treatment option for intrahepatic recurrence of biliary cystadenocarcinoma.

Cystadenocarcinoma of the intrahepatic bile duct in a dog.

A 14-year-old spayed female Shih-Tzu was referred to the Veterinary Medical Teaching Hospital of Konkuk University for evaluation of an abdominal mass. In diagnostic imaging, two large cystic masses were identified. The affected liver lobes were surgically resected, and the specimens were submitted for histopathological evaluation and immunohistochemical staining. The two cystic lesions were diagnosed as biliary cystadenocarcinoma (BCAC). Recurrence and regional invasion were identified on ultrasonography 36 days postoperatively. The patient died on postoperative day 271. To the best of our knowledge, previously reported case studies of BCAC in dogs presented limited clinical information. In this report, we present a detailed picture comprising a range of clinical information and histopathological examination of BCAC in a dog.

Sequential thermal ablation in combination with sclerotherapy using lauromacrogol as a successful translative therapy for an unresectable huge biliary cystadenocarcinoma: The first experience assisted by contrast-enhanced ultrasound.

Biliary cystadenocarcinoma (BCAC) is an extremely rare intrahepatic cystic tumor. Patients usually present with nonspecific symptoms such as abdominal pain, abdominal distention, and abdominal mass. This tumor occurs most commonly in the left hemiliver and is thought to mainly develop from a benign biliary cystadenoma (BCA). At present, the disease is mainly diagnosed by ultrasound, CT, MR, and other imaging methods, and the main treatment is radical surgical resection. We reported a 75-year-old female with an unresectable huge BCAC (i.e., 161×145×122 mm in three orthogonal directions) and poor general condition (40 in Karnofsky Performance Status, KPS) who received sequential thermal ablation (i.e., cryoablation and microwave ablation) in combination with sclerotherapy using lauromacrogol. The diagnosis of intrahepatic BCAC was confirmed pathologically. Preablation grayscale US showed the BCAC with a clear boundary, regular shape, and cystic-solid mixed echogenicity, which appeared as a huge multilocular cystic lesions with thick internal sepatations. Preablation contrast-enhanced ultrasound (CEUS) showed honeycomb-like hyper-enhancement of the thick internal sepatations and cystic wall in the arterial and portal phase, and sustained enhancement of the thick internal sepatations and cystic wall in the late phase. 6-month postablation CEUS showed non-enhancement in most parts of the lesion in the arterial phase and 6-month postablation MRI showed the volume reduction ratio (VRR) was about 70%. The abdominal pain and abdominal distension were relieved remarkably, and her quality of life was greatly improved (70 in KPS). In conclusion, sequential thermal ablation in combination with sclerotherapy provides a successful translative therapy for this unresectable huge BCAC with a poor general condition, which makes subsequent curative surgery or ablation possible.

Enucleation of Biliary Cystadenomas: a Review.

BACKGROUND: Biliary cystadenomas are very rare benign tumors which can transform into cystadenocarcinomas. The largest case series reported on 221 cases over 30 years from 10 HPB centers, i.e., about 7 cases per center per decade. The recommended treatment is liver resection. Enucleation of biliary cystadenomas has been done rarely. The purpose of the study was to determine the outcome of enucleation of these cysts, particularly the mortality rate and the recurrence rate. METHODS: A keyword search was done using OVID followed by a search of the bibliography of papers describing the enucleation of biliary cystadenomas. Of 45 articles obtained, 25 were retained. The main reasons for exclusion were non-English language and review articles. RESULTS: One hundred three patients in the 25 studies were treated with enucleation. Thirteen studies described prior treatments that had failed with resulting recurrence requiring re-treatment. The main indication for enucleation was large central cysts for which liver resection would be high risk. There were no postoperative deaths in patients treated by enucleation. Thirteen studies provided long-term follow-up in 40 patients, a substantial number given the rarity of the tumor. There were no recurrences or transformations to malignancy. CONCLUSIONS: Enucleation seems to represent a reasonable treatment technique for BCA, especially when a large cystic lesion is located centrally and/or would require a large liver resection with significant loss of parenchyma.

Salivary gland cystadenocarcinoma: Rare neoplasm in buccal mucosa.

A 72-year-old female was referred for diagnosis of a lesion located in the right buccal mucosa, with duration unknown. At intraoral examination, the lesion appeared as a well-delimited, mobile, and submucosal nodule. A benign mesenchymal neoplasm was the main hypothesis of diagnosis. Histopathological analysis revealed salivary gland neoplasm formed by atypical cells often arranged in microcystic structures, with frequent intraluminal papillary projections. The tumor cells presented positivity for CK7 and negativity for CK20. Based on these features, the diagnosis of cystadenocarcinoma was established. The patient was submitted to wide surgical resection. No recurrence was observed after 48 months. Although rare, cystadenocarcinoma should be considered in the differential diagnosis of oral submucosal nodules.

Ovarian masses in children and adolescents: Analysis on 146 patients.

PURPOSE: Ovarian masses in pediatric population are the most common genital neoplasms, and these masses are often benign. The purpose of this study is to evaluate the pediatric ovarian masses operated in our hospital. METHOD: The records of patients, under the age of 18 who were operated in our hospital due to ovarian mass between 2012 and 2018 were reviewed retrospectively. Clinical findings, operational procedures, histopathologies, tumor markers and radiological images were evaluated. FINDINGS: During the study, 146 patients (5 patients were bilateral) were evaluated. The average age of the study patients was 14.01 ± 4.02 years. 107 of the study patients were benign, 37 were malignant and 2 were borderline. The most common symptom in benign masses was tenderness in lower abdominal (75.7 %). 124 of the patients (86.1 %) were in post-menarche period. 34 of the patients had ovarian torsion. Open surgery was conducted on 79.5 % (116/146) of the patients, and laparoscopic surgery was conducted on 20.5 % (30/146). The rate of oophorectomy was 24.6 % (36/146) throughout the operations. The most frequently conducted surgical procedure was cyst excision in benign masses and oophorectomy in malignant masses. In neoplastic masses, the ratio of pelvic mass palpation; and in non-neoplastic masses, lower abdominal tenderness was more apparent. The rate of ovarian torsion was 23.6 % (25/107) in benign masses and 24.3 % (9/37) in malignant masses. OUTCOMES: Pediatric and adolescent ovarian masses are mostly benign and majority of these occur at post-menarche period. The most common symptom was pelvic tenderness in benign masses, and palpable pelvic masses in malignant masses. For future fertility and low incidence of malignancy in these patients, ovarian preserving surgery should be considered for the first operation.

Six cases of central cystadenocarcinoma and review of relevant papers.

The present study was a review of cases of central cystadenocarcinoma over the past 29 years and provides clinical, radiological, and pathological information about these rare lesions. Six cases of central cystadenocarcinoma treated between 1991 and 2019 at Peking University Hospital of Stomatology in Beijing, China, were retrospectively analysed. A comprehensive review of clinical records was summarised and the histological diagnosis was revised using the 2017 World Health Organization criteria. The mean age of patients with central cystadenocarcinoma was 63 (range 51-75) years, and the male:female ratio 1:1. The clinical signs included localised swelling, pain, lower lip numbness, and trismus. There were more cases in the mandible than in the maxilla. All lesions were unilocular or multilocular in radiolucent regions with or without clear margins. The preferred treatment of central cystadenocarcinoma was surgical excision with wide margins, and no local recurrence was found during follow-up. Central cystadenocarcinoma often occurred in middle-aged or elderly patients. Because cystadenocarcinoma is somewhat rare, metastatic tumours of the jaw should be considered when diagnosing cystadenocarcinoma.

The impact of imaging on the surgical management of biliary cystadenomas and cystadenocarcinomas; a systematic review.

BACKGROUND: Biliary Cystadenomas (BCA) are considered to be benign but may transform to Biliary Cystadenocarcinomas (BCAC). The aim of this systematic review was to assess the diagnostic work-up and necessity of complete surgical resection. METHOD: A systematic literature search was performed in Embase.com, Medline (Ovid), Cochrane Central, Web-of-Science and Google Scholar. Articles reporting on diagnostic work-up or outcome of various treatment strategies were included. RESULTS: Fifty-one articles with 1218 patients were included: 971 with BCA and 247 with BCAC. Patients with BCA were more often female (91% vs 63.8%, p < 0.001). On radiologic imaging BCAC more often had calcifications (p = 0.008), mural nodules (p < 0.001) and wall enhancement (p < 0.001). Reported treatment strategies were resection, enucleation, or fenestration/marsupialization. Recurrence was reported in 5.4% after resection for BCA and 4.8% after resection for BCAC. Recurrence after fenestration/marsupialization varied from 81.6% to 100% for both BCA as BCAC. Mortality rate was 0 in patients with BCA and 24% in BCAC. CONCLUSION: Due to the difficulty in accurately diagnosing these biliary cystic lesions and the availability of different surgical approaches, patients with suspected BCA or BCAC should be treated in a center specialized in liver surgery with state-of-the-art imaging and all surgical techniques to prevent mismanagement of this rare disease.

[Laparoscopic appendectomy for mucinous cystadenoma of the appendix]. / Laparoskopicheskaia appendéktomiia pri mutsinoznoi tsistadenome cherveobraznogo otrostka.

Treatment of acute and chronic appendicitis is still an actual problem. There are some rare courses of the disease besides well-known complications of appendicitis. Mucocele is one of the rarest forms of chronic appendicitis. Mucocele is not accompanied by clinical symptoms and diagnosed accidentally in more than 25% of cases. The most serious complication of mucocele of the appendix is malignization observed in up to 36% of cases. Ultrasound, CT and colonoscopy are the most effective methods of perioperative diagnosis. Unclear symptoms, delayed diagnosis and surgical treatment increase the risk of complications (i.e. cystadenocarcinoma).

Laparoscopic Left Lateral Sectionectomy Using the Extrahepatic Glissonean Approach: A Secure Option for Achieving a Negative Margin for Lesions with Ductal Extension.

INTRODUCTION: In patients with liver lesions with ductal extension, the corresponding Glissonean pedicle should be divided at its origin to achieve a negative ductal margin; however, during laparoscopic hepatectomy, it is difficult to precisely transect the liver and divide the Glissonean pedicle as planned. METHODS: We present a video of a laparoscopic left lateral sectionectomy using the extrahepatic Glissonean approach for a lesion with ductal extension. RESULTS: A 76-year-old woman presented with a cystic neoplasm in the liver segment 3 bile duct (B3). The preoperative workup suggested biliary extension of the lesion towards the origin of B3. A decision was made to perform laparoscopic left lateral sectionectomy with division of the segment 3 Glissonean pedicle (G3) at its origin, and, additionally, left hepatectomy if the B3 ductal margin turned out to be positive. During the procedure, prior to parenchymal transection, the Arantius' ligament was dissected, and G2 and G3 were extrahepatically taped. The ischemic border was visualized by clamping the isolated pedicle, and was also clearly demonstrated by indocyanine green fluorescence. After transecting the liver towards the tape, G3 was divided at its origin, and the frozen section of the ductal margin was negative for tumors. CONCLUSION: The extrahepatic Glissonean approach can help to obtain a maximal ductal margin for liver lesions with possible biliary extension, although the technique potentially poses the risk of bleeding and/or biliary injury, and requires expertise in hepatobiliary surgery. Further studies with larger sample sizes are warranted to validate the feasibility and efficacy of this strategy.

Clinicopathological analysis of borderline ovarian tumours and risk factors related to recurrence: experience of single institution.

This study was conducted to determine the demographic and clinicopathologic characteristics and evaluate the prognostic value of various factors, such as the extensiveness of surgery, related to the tumour itself and the clinical features in the recurrence of borderline ovarian tumours (BOT). We retrospectively evaluated the data of 103 patients with a borderline ovarian tumours treated at our institution between the years 2000 and 2012. The median age was 37 (16-79) years and the majority of the patients were premenopausal (76.7%). During the follow-up, 16 recurrences were observed (15.5%). The multivariate analysis showed that the micropapillary architecture and fertility sparing surgery were the only significant independent predictors for the development of a recurrence amongst all of the demographic and clinicopathological features. In our study group, we identified that the micropapillary architecture itself and the fertility sparing surgery had a significant impact on the development of a BOT recurrence. The patients who possess these features should be followed up more closely for a long time period. Impact statement What is already known on this subject? A borderline ovarian tumour is known as a recurrent disease. The recurrence rate varies between 5 and 20%. It is well known in the literature that patients treated by an oophorectomy have a relatively lower risk of development of a recurrence compared to the patients treated by cystectomy. What do the results of this study add? Although some of the clinicopathological features are shown to be risk factors for the development of a recurrence in many studies, some of the pathological-clinical and the demographic features have not been described as yet, or have been considered to be equivocal regarding the development of a recurrence. In this study, we investigate all possible demographic, pathological, and clinical factors associated with a recurrence. Not only the well-known pathological characteristics but also the new pathological parameters and clinical approaches have been investigated. For instance, microinvasion architecture and lymphadenectomy speculated in the literature as the risk factors for the development of a recurrence, have not been identified as risk factors in our study. On the other hand, our statistical analyses have revealed that micropapillary architecture should be described as a risk factor for the development of a recurrence. What are the implications of these findings for clinical practice and/or further research? We hope our study becomes influential in the literature on the field of a micropapillary architecture and the development of a recurrence. The patients carrying this feature have to be followed up very closely and carefully. Furthermore, our findings have indicated no significant relation between the performing of a lymphadenectomy and the rate of a recurrence. This result might be encouraging for the gynaecological surgeons to refrain from a lymphadenectomy for the borderline ovarian tumours.

Clinicopathological Features and Management of Appendiceal Mucoceles: A Systematic Review.

Appendiceal mucoceles (AMs) are rare mucin-containing neoplasms with malignant potential. Lack of evidence-based data exists defining clinicopathological features for management. MEDLINE search between 1995 and 2015 was performed using search criteria "Appendix mucocele." Systematic review of patient-, pathologic-, and treatment-related characteristics was performed and data analyzed. Among 276 cases of non-perforated AMs, 163 (59%) patients were female, with variable and nonspecific presentation. Patients were treated with appendectomy (52.1%), right hemicolectomy (17.6%), partial cecectomy (17.2%), and ileocecetomy (13.1%). Pathologic evaluation revealed the following: cystadenoma/low-grade appendiceal mucinous neoplasm (54%), unspecified/benign (25%), retention cyst (14.1%), cystadenocarcinoma (4.2%), and mucosal hyperplasia (2.9%). All 11 (4.2%) patients with cystadenocarcinoma were female (P = 0.004), odds ratio for malignancy 1.07 times higher for women. Synchronous colonic malignancy was reported in three patients (27%) with cystadenocarcinoma (P = 0.007), odds ratio of 12.1. AMs have low risk for malignancy. Treatment should begin with appendectomy-only and subsequently guided by pathologic diagnosis.