Clinical diagnosis and management of pancreatic mucinous cystadenoma and cystadenocarcinoma:Single-center experience with 82 patients

BACKGROUND Mucinous cystic neoplasm(MCN)of the pancreas is characterized by mucinproducing columnar epithelium and dense ovarian-type stroma and at risk for malignant transformation.Early diagnosis and treatment of MCN are particularly important.AIM To investigate the clinical characteristics of and management strategies for pancreatic mucinous cystadenoma(MCA)and mucinous cystadenocarcinoma(MCC).METHODS The clinical and pathological data of 82 patients with pancreatic MCA and MCC who underwent surgical resection at our department between April 2015 and March 2019 were retrospectively analyzed.RESULTS Of the 82 patients included in this study,70 had MCA and 12 had MCC.Tumor size of MCC was larger than that of MCA(P=0.049).Age and serum levels of tumor markers carcinoembryonic antigen(CEA),carbohydrate antigen(CA)19-9,and CA12-5 were significantly higher in MCC than in MCA patients(P=0.005,0.026,and 0.037,respectively).MCA tumor size was positively correlated with serum CA19-9 levels(r=0.389,P=0.001).Compared with MCC,MCA had a higher minimally invasive surgery rate(P=0.014).In the MCA group,the rate of major complications was 5.7%and that of clinically relevant pancreatic fistula was 8.6%;the corresponding rates in the MCC group were 16.7%and 16.7%,respectively.CONCLUSION Tumor size,age,and serum CEA,CA19-9,and CA12-5 levels may contribute to management of patients with MCN.Surgical resection is the primary treatment modality for MCC and MCA.

Primary retroperitoneal mucinous cystadenoma:A case report

Primary retroperitoneal mucinous cystic tumors are extremely rare.These tumors can be classified as a primary retroperitoneal mucinous cystadenoma with or without borderline malignancy or primary retroperitoneal mucinous cystadenocarcinoma.The most common of these is primary retroperitoneal mucinous cystadenoma,which almost always occurs in female patients;only ten cases have been reported in males.The most common clinical findings for this tumor type include nonspecific abdominal pain and a palpable abdominal mass.A definitive diagnosis is usually obtained from histopathology after surgical excision.Here,we report the case of a 60-year-old female patient who complained of abdominal pain that had been present for 3 mo and presented with a palpable abdominal mass.Multidetector computed tomography scanning revealed a large,unilocular cystic mass in the left retroperitoneal space.Surgical intervention was performed and the tumor was completely removed.Histopathologic examination confirmed that the tumor was a primary retroperitoneal mucinous cystadenoma.Two years after surgery,the patient remains disease free.

Giant mucinous biliary cystadenoma:a case report

BACKGROUND: Biliary cystadenoma is a very rare cystic neoplasm of the liver. Its clinical features, diagnosis, pathologic characteristics, and optimal surgical management have not been defined clearly. In this article we describe the details of this rare disease. METHODS: A 40-year-old woman with a mass of the liver was verified by ultrasonography and LT. Ultrasonography showed a mixed echo of 18.4 cmx14.72 cmx 15.54 cm in the left lobe of the liver. CT showed a vesicula of 19.9 cmx 13.5 cm in the right epigastrium, with a low density, clear edge, uneven density, and calcified shadow. The patient received successfully a left hepatectomy. Laboratory examination showed an elevation of CA125 to 62.62 U/ml and CA199>1000 U/ml. RESULTS: After the left hepatectomy, the patient was fully recovered. Her biliary cystadenoma was characterized by specific histological findings. During operation, a large cystic lesion was seen in the left hepatic lobe; its surface was dark red with abundant blood supply. Gross examination showed that the tumor almost occupied. The whole left lobe with a small amount of normal liver tissue close to the deltoid ligament. Pathologically, additional lobulated spaces were seen in the tumor with a lot of mucusa. The interior wall was lined with bile duct tissue, indicating a benign mucinous biliary cystadenoma. CONCLUSIONS: Ultrasonography and CT are the major methods for the diagnosis of mucinous biliary cystadenoma liver. Operation is the best way of treatment.

Combined goblet cell carcinoid and mucinous cystadenoma of the vermiform appendix

Goblet cell carcinoid is an uncommon primary tumor of the vermiform appendix, characterized by dual endocrine and glandular differentiation. Whether goblet cell carcinoid represents a morphological variant of appendiceal classical carcinoid or a mucin-producing adenocarcinoma is a matter of conjecture. Rare cases of goblet cell carcinoid with other concomitant appendiceal epithelial neoplasms have been documented. In this report, we describe a rare case of combined appendiceal goblet cell carcinoid and mucinous cystadenoma, and discuss the possible histopathogenesis of this combination.

Large mucinous biliary cystadenoma with "ovarian-like"stroma:A case report

Hepatobiliary cystadenomas are rare tumors that are difficult to diagnose preoperatively. They can reach large sizes that make them real intraoperative ＂surprises＂. A 63-year-old woman, presented with a symptomatic huge abdominal cystic mass, underwent complete resection of the mass with safety margins. Histopathological report revealed mucinous hepatic cystadenoma with ＂ovarian- like＂ stroma and areas of sclerohyalinization. The differential diagnosis of the large cystic tumors which occupy the right hemiabdomen must include the biliary cystadenoma; the complete resection of the tumor with safety margins avoids local recurrence, and therefore represents the optimal therapy because of the malignant potential of the disease. The postoperative follow-up includes abdominal ultrasound or CT scan and CA 19-9 measurement.

Primary retroperitoneal mucinous cystadenoma:Report of a case and review of the literature

Primary retroperitoneal mucinous cystadenomas (RMCs) are very rare and their biological behavior and histogenesis remain speculative. An accurate preoperative diagnosis of these tumors is difficult because no effective diagnostic measures have been established. We describe a 29-year-old woman with abdominal pain and a palpable mass. Computed tomography (CT) of the abdomen revealed a retroperi-toneal cystic mass which was resected successfully at laparotomy. Histopathological examination of the resected mass confirmed the diagnosis of RMC. There was no evidence of disease 2 years after surgery.

Laparoscopic fenestration of multiple giant biliary mucinous cystadenomas of the liver

Biliary cystadenomas of the liver are rare, cystic neoplasms of the biliary ductal system usually occur in middle aged women. We report a case of synchronous multiple huge biliary mucinous cystadenomas with unique features. This is, according to our knowledge, the first report in the literature about three synchronously occurring hepatobiliary cystadenomas. Cystadenomas have a strong tendency to recur, particularly following incomplete excision, and a potential of malignant transformation. A therapeutic re-evaluation may be necessary when the diagnosis of hepatobiliary cystadenoma is made after the operation and an open liver resection should be considered.

Elevation of Serum CA-125 in Mucinous Cystadenoma of the Ovary with Torsion: A Rare Occurrence and Review of the Literature

Aim: Benign neoplasms of the ovary originating from epithelial tissue are common tumors in adult women. They are, however, rarely seen in children or adolescent girls with elevation of serum carbohydrate antigen- 125 (CA-125). The present report describes a rare case of premenarchal women with a giant mucinous cystadenoma (MCA) of the ovary with torsion complicated with elevation of serum CA-125. Case: A 15-year-old, premenarchal, previously healthy girl was referred to our hospital with a 2-week history of left lower abdominal pain. Physical examinations showed a firm and mobile mass with tenderness in the left lower quadrant. Tumor markers showed CA-125 at 124.1 U/ml. An enhanced computed tomography scan showed a multiloculated tumor that was partly solid, compressing the small intestine, uterus, and urinary bladder, but no signs of organ invasion, lymph node swelling, or ascitis. Via a lower transverse incision, the right Fallopian tube was observed to have twisted 1620。 counterclockwise, and a tense cyst measuring 22.0 × 12.0 × 10.5 cm and weighing 1.78 kg was found in the right ovary. Release from torsion and unilateral salpingo-oophorectomy with tumor removal was performed because blood flow to the right Fallopian tube did not improve after torsion release. The histopathological findings showed an MCA of the ovary without cell dysplasia. The patient did not receive adjuvant chemotherapy following surgery. There was no evidence of recurrence at 2 years. Disucussion: We need to consider MCA of the ovary when there is elevation of the serum CA-125 and an ovarian mass.

Huge mucinous borderline ovarian cystadenoma in a premenarchal girl

Epithelial ovarian tumors are common in adult women, but rare in children. Especially mucinous ovarian cystadenoma is extremely rare, with only 16 cases in premenarchal girls reported to date. We present a case of 12-year-old premenarchal girl with symptoms of lower abdominal distension. CT showed a large multilocular tumor spreading throughout the entire abdominal cavity from the right upper quadrant to the pelvic cavity. The tumor was brought out little by little with aspiration of the fluid contents with a small incision. The tumor was found to originate from the left ovary, and oophorectomy was performed. The tumor measured 26 × 18 cm and weighed 5860 g. Histological diagnosis was mucinous ovarian cystadenoma with borderline malignancy. No evidence of recurrence had been seen as of 2 years postoperatively. In the review of premenarchal mucinous borderline ovarian cystadenoma, all cases were stage I and underwent salpingo-oophorectomy or oophorectomy. Those patients were alive without recurrence. Al-though mucinous borderline ovarian cystadenoma was characterized as malignancy, premenarchal cases showed favorable outcomes and salpingo-oohprectomy or oophorectomy appears appropriate.

Association of endometrioma with ovarian teratoma and mucinous cystadenoma in a patient diagnosed with endometriosis: A case report

BACKGROUND Co-occurrence of different tumor types in a same patient’s ovaries diagnosed with endometriosis is a rare phenomenon.CASE SUMMARY In this article we present an uncommon association of three distinct ovarian pathologies in a same woman presenting with adnexal mass.A 31-year-old nulliparous woman with a large persistent adnexal mass underwent laparoscopic surgery.Imaging demonstrated a multi-cystic mass with internal echoes.Tumor markers were within normal range.Based on histopathologic assessment,benign mucinous cystadenoma and mature cystic teratoma of the right ovary together with endometrioma of left ovary were revealed.CONCLUSION In cases of large adnexal mass,the of existence of more than one tumor type and the involvement of the contralateral ovary is possible.Also,the possibility of concurrent underlying malignancy or diminished ovarian reserve should be kept in mind.

Giant benign mucinous cystadenoma: A case report

Giant benign tumours of the ovary are rare in the modern world due to the improved technologies and general awareness. They are known to cause pressure symptoms to the surrounding structures. Though they appear frighteningly large, they are amenable to surgical debulking with good results in survival and post operative recovery. Here we would like to present a case where the patient could not access medical care, which led to the presentation of a very large mucinous cystadenoma which responded remarkably to surgical excision. The patient could go back to her normal life following the procedure. Conclusions Giant benign mucinous tumours are a rarely seen in the modern world. They have excellent surgical results and survival rates.

Resection of the main trunk of the superior mesenteric vein without reconstruction during surgery for giant pancreatic mucinous cystadenoma:A case report

Pancreatic tumors, with peri-pancreatic main vascular invasion, especially the superior mesenteric vein(SMV) or the portal vein, are very common. In some cases, vascular resection and reconstruction are required for complete resection of pancreatic tumors. However, the optimum surgical method for venous management is controversial. Resection of the SMV without reconstruction during surgery for pancreatic tumors is rarely reported. Here we present the case of a 58-year-old woman with a giant pancreatic mucinous cystadenoma adhering to the SMV, who underwent an en bloc tumor resection, including the main trunk of the SMV and the spleen. No venous reconstruction was performed during surgery. No ischemic changes occurred in the bowel. The presence of several welldeveloped collateral vessels was shown by 3-dimensional computed tomography examination. The patient had an uneventful postoperative period and was discharged. This case indicated that the main trunk of the SMV can be resected without venous reconstruction if adequate collateralization has formed.

Laparoscopic central pancreatectomy with gastro-pancreatic anastomosis for symptomatic serous cystadenoma:A case report and literature review

Surgery for lesions of the proximal part of the pancreatic body or neck can be challenging,and when enucleation is not possible,central pancreatectomy is an option.Laparoscopic central pancreatic resection is rarely described worldwide;it is considered a difficult procedure mainly because of the risk of double pancreatic fistula developing at two sites of resection.However,it seems to be an excellent alternative to distal pancreatectomy or pancreaticoduodenectomy,with the advantages of preserving functioning parenchyma and reducing endocrine and exocrine failure.Nevertheless,patients with pancreatic lesions requiring central resection are often managed with the open approach in many hospitals due to the complexity of total laparoscopic central pancreatectomy,which requires advanced laparoscopic skills,expertise and experience.Here,we report a case of a 29-year-old female who underwent total laparoscopic central pancreatic resection with gastro-pancreatic anastomosis for symptomatic serous cystadenoma.We discuss the details of case management and review the relevant literature.

Advances in the Diagnosis and Treatment of Appendiceal Mucinous Neoplasms

Primary appendiceal neoplasms represent a relatively low percentage of all gastrointestinal cancers. A subset of these neoplasms, those of epithelial origin, are characterised by the production of a considerable amount of mucus, which is referred to as appendiceal mucinous neoplasms (AMN). Appendiceal mucinous neoplasms (AMN) have a low incidence, are easily misdiagnosed, depend on postoperative examination for confirmation of the diagnosis, are prone to form a “diagnosis”, and have a high incidence of the disease. Furthermore, they are prone to form peritoneal pseudomyxoma peritonei (PMP), are controversial in surgical decision-making, are prone to recurring after surgery alone, and are tricky to manage clinically. In this paper, we review the pathological characteristics, diagnosis and treatment of appendiceal mucinous tumours in the light of recent literature reports, with a view to providing certain references for the clinical diagnosis and treatment of this disease. .

Asymptomatic low-grade appendiceal mucinous neoplasm:A case report

BACKGROUND Low-grade appendiceal neoplasms(LAMN)are characterized by low incidence and atypical clinical presentations,often leading to misdiagnosis as acute or chronic appendicitis before surgery.The primary diagnostic tool for LAMN is abdominal computed tomography(CT)imaging.Surgical resection remains the cornerstone of LAMN management,necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture.Laparoscopy,known for its minimal invasiveness,reduced postoperative discomfort,and expedited recovery,is a safe and reliable approach for LAMN treatment.Despite the possibility of pseudomyxoma peritonei development,appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes,which can be attributed to the disease’s slow growth and lower malignancy.CASE SUMMARY A 71-year-old male patient was admitted to our hospital with a pelvic spaceoccupying lesion detected 1 mo prior.Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses.No shifting dullness was noted,and digital rectal examination revealed no palpable mass.Enteroscopy revealed a raised,smooth-surfaced mass measuring 3.0 cm in the cecum.Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows.Laparoscopic surgery was performed and revealed a significantly dilated appendix,leading to laparoscopic resection of the appendix and part of the cecum.Post-surgical pathologic analysis confirmed LAMN.The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding,intestinal obstruction,or incision infection.No tumor recurrence was observed during a 7-mo follow-up period.CONCLUSION LAMN is a rare disease that lacks specific clinical manifestations.Abdominal CT plays a crucial role in diagnosing LAMN,and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.

Diagnosis and treatment of biliary mucinous cystic neoplasms: A single-center experience

Background: Biliary mucinous cystic neoplasms(BMCNs) are rare hepatobiliary cystic tumors, which can be divided into noninvasive and invasive types. This study aimed to investigate the diagnosis, treatment, and prognosis of BMCNs in a large single center. Methods: We analyzed 49 patients with BMCNs confirmed by postoperative pathology at the First Afflliated Hospital, Zhejiang University School of Medicine between January 2007 and December 2021. Results: Among the 49 patients, 37 were female(75.5%), and the average age was 57.04 years. Common symptoms included abdominal discomfort, jaundice and fever, while 22 patients(44.9%) had no symptoms. Serum carbohydrate antigen(CA) 19-9 and CA125 concentrations were elevated in 34.8% and 19.6% of patients, respectively. Forty-eight patients had tumors in the intrahepatic bile ducts and only one had a tumor in the extrahepatic bile duct. Forty-eight patients with noninvasive intrahepatic BMCNs were further analyzed in terms of pathological features: 34(70.8%) had low-grade intraepithelial neoplasms(LGINs), and 14(29.2%) had high-grade intraepithelial neoplasms(HGINs). The potential immunohistochemical markers of BMCNs were cytokeratin(CK) 19, CK7, estrogen receptor and progesterone receptor. Follow-up data for 37 patients with intrahepatic BMCNs were obtained. The median overall survival(OS) of BMCNs was not reached. The longest survival time was 137 months.The 5-and 10-year OS rates were 100% and 85.4%, respectively. The 5-and 10-year recurrence-free survival(RFS) rates were 93.9% and 80.2%, respectively. Conclusions: BMCNs are rare cystic neoplasms that commonly occur in middle-aged females. BMCNs can only be diagnosed and classified by postoperative pathology, as there are no specific clinical presentations, serological indicators or imaging modalities for preoperative diagnosis. Complete surgical resection is necessary for BMCNs, and the postoperative prognosis is favorable.

Waist subcutaneous soft tissue metastasis of rectal mucinous adenocarcinoma: A case report

BACKGROUND Rectal mucinous adenocarcinoma(MAC)is a rare pathological type of rectal can-cer with unique pathological features and a poor prognosis.It is difficult to diag-nose and treat early because of the lack of specific manifestations in some aspects of the disease.The common metastatic organs of rectal cancer are the liver and lung;however,rectal carcinoma with metastasis to subcutaneous soft tissue is a rare finding.CASE SUMMARY In this report,the clinical data,diagnosis and treatment process,and postope-rative pathological features of a patient with left waist subcutaneous soft tissue masses were retrospectively analyzed.The patient underwent surgical treatment after admission and recovered well after surgery.The final pathological diagnosis was rectal MAC with left waist subcutaneous soft tissue metastasis.CONCLUSION Subcutaneous soft tissue metastasis of rectal MAC is rare,and it can suggest that the tumor is disseminated,and it can appear even earlier than the primary ma-lignant tumor,which is occult and leads to a missed diagnosis and misdiagnosis clinically.When a subcutaneous soft tissue mass of unknown origin appears in a patient with rectal cancer,a ma-lignant tumor should be considered.

Mucinous cystadenoma of the appendix associated with adenocarcinoma of the sigmoid colon and hepatocellular carcinoma of the liver:Report of a case

Mucinous cystadenoma of the appendix is a rare condition and represents one of the three entities with the common name mucocele of the appendix. It is characterized by a cystic dilatation of the lumen with stasis of mucus inside it. Histopathologically mucocele is divided into three groups： focal or diffuse mucosal hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma. This condition is often associated with other neoplasia, especially adenocarcinoma of the colon and ovaries. We here describe a 57 year old male patient who presented with abdominal discomfort, constipation, fresh blood in stool and frequent urination. He had a big cystadenoma of the appendix associated with adenocarcinoma of the colon and hepatocellular carcinoma of the liver. The patient underwent right haemicolectomy, sigmoid colon resection and segmental resection of the liven Now 3 years later he has no evidence of disease relapse. According to this, we stress the need of accurate preoperative diagnosis and intraoperative exploration of the whole abdomen in these patients.

Evaluation of appendiceal mucinous neoplasms by curved lineararray echoendoscope:A preliminary study

BACKGROUND Preoperative diagnosis of appendiceal mucinous neoplasms is challenging,and there are few reports regarding the endosonographic characteristics of these neoplasms.AIM To provide a retrospective assessment of the imaging features of appendiceal mucinous neoplasms using endoscopic ultrasound(EUS)by curved linear-array echoendoscope.METHODS A database of all patients with appendiceal mucinous neoplasms who had received EUS examination at our hospital between January 2018 and July 2023 was retrospectively analyzed.The EUS characteristics and patients’clinical data were reviewed.RESULTS Twenty-two patients were included in the study.The linear-array echoendoscope successfully reached the ileocecal region in every patient.In the endoscopic view,we could observe the protrusion in the appendiceal orifice in all patients.A volcano sign was observed in two patients,and an atypical volcano sign was seen in two patients.EUS showed that all 22 lesions were submucosal cystic hypoechoic lesions with clear boundaries.No wall nodules were observed,but an onion-peeling sign was observed in 17 cases.CONCLUSION Linear-array echoendoscope is safe to reach the ileocecal region under the guidance of EUS.Image features on endoscopic and echoendosonograhic views could be used to diagnose appendiceal mucinous neoplasms.

Intraductal papillary mucinous neoplasm originating from a jejunal heterotopic pancreas:A case report

BACKGROUND Intraductal papillary mucinous neoplasm(IPMN)is a rare pancreatic tumor and has the potential to become malignant.Surgery is the most effective treatment at present,but there is no consensus on the site of resection.Heterotopic pancreas occurs in the gastrointestinal tract,especially the stomach and duodenum but is asymptomatic and rare.We report a case of ectopic pancreas with IPMN located in the jejunum.CASE SUMMARY A 56-year-old male patient suffered from severe pain,nausea and vomiting due to a traffic accident and sought emergency treatment at our hospital.Contrast-enhanced computed tomography of the whole abdomen suggested splenic congestion,which was considered to be splenic rupture.Emergency laparotomy was performed,and the ruptured spleen was removed during the operation.Unexpectedly,a cauliflower-like mass of about 2.5 cm×2.5 cm in size was incidentally found about 80 cm from the ligament of Treitz during the operation.A partial small bowel resection was performed,and postoperative pathology confirmed the small bowel mass as heterotopic pancreas with low-grade IPMN.CONCLUSION Ectopic pancreas occurs in the jejunum and is pathologically confirmed as IPMN after surgical resection.