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.

Synchronous Mucinous Borderline Tumor of the Ovary and Low-Grade Appendiceal Mucinous Neoplasm

We present a rare case of synchronous mucinous borderline tumor of the ovary and low-grade appendiceal mucinous neoplasm (LAMN). We performed a left adnexectomy to diagnose the left ovarian borderline malignancy and an ileostomy because of the swollen appendix during the operation. It was diagnosed as left ovarian mucinous borderline malignancy and LAMN. LAMN causes peritoneal dissemination, ovarian metastasis, and peritoneal pseudomyxoma. The appendix and ovary are close to each other anatomically and can metastasize if there is a tumor in either. For ovarian mucinous tumors, it is necessary to search the gastrointestinal tract, especially the appendix, as the primary lesion. For appendix tumors, it is necessary to search for the ovary. Since LAMN may be associated with borderline ovarian malignancies, as in this case, there is a possibility of the duplication of tumors when searching for ovarian mucinous tumors as the primary tumor and if ovarian tumors are found. Since LAMN and mucinous ovarian tumors have similar histological features, immunohistochemical staining is useful for their differentiation because they show different immunostaining patterns.

A Case of Low-Grade Appendiceal Mucinous Neoplasm of Its Difficultly to Distinguish from a Right Ovarian Tumor Due to Postmenopause

We here present a rare case of appendiceal tumor mimicking ovarian tumor in menopause woman. The patient was a 56-year-old woman, G1P1, who presented to our hospital with a right adnexal cyst diagnosed at another hospital. Transvaginal echocardiography showed a cyst in the right adnexal region, and pelvic contrast-enhanced MRI revealed a small cyst in the same region. The left ovary was atrophic and identifiable. It was unclear whether the cyst was contiguous with the gastrointestinal tract. Blood tests showed no elevation of tumor markers. We considered its possibility of a gastrointestinal origin, but since right normal ovary was not found, we thought the tumor was of ovarian origin and decided on a laparoscopic resection of the right adnexa. Intraoperatively, we observed atrophied bilateral normal ovaries, and the pelvic tumor was contiguous to the appendix. Surgeons performed a laparoscopic appendectomy after consultation with us. After resection we searched the abdominal and pelvic cavities, but found no obvious disseminated lesions. The histological diagnosis was low-grade appendiceal mucinous neoplasm (LAMN), a rare benign tumor of the appendix. Appendiceal tumors can be difficult to differentiate from right ovarian tumors due to their close anatomic location in the pelvis. It is possible to determine whether the tumor is of ovarian or appendiceal origin by identifying normal ovaries and the location of the feeding vessels into the tumors. In our case, there were no lesions other than the appendix, but LAMN can metastasize to the ovary, cause pseudomyoxoma peritonei, or be an overlapping tumor with an ovarian tumor. If an appendiceal tumor is diagnosed after surgery for ovarian tumor, the intra-abdominal cavity should be searched for metastasis or dissemination, and a thorough search for ovarian lesions should be performed with the possibility of an overlapping tumor in mind.

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.

Mucinous neoplasm of the appendix:A case report and review of literature

BACKGROUND Appendiceal mucinous neoplasms(AMNs),although not classified as rare,are relatively uncommon tumors most often discovered incidentally during colorectal surgery.Accurate identification of AMNs is difficult due to non-specific sym-ptoms,overlapping tumor markers with other conditions,and the potential for misdiagnosis.This underscores the urgent need for precision in diagnosis to pre-vent severe complications.CASE SUMMARY This case report describes the unexpected discovery and treatment of a low-grade AMN(LAMN)in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma(AC).Preoperatively,non-specific gastroin-testinal symptoms and elevated tumor markers masked the presence of AMN.The tumor,presumed to be an AMN peritoneal cyst intraoperatively,was con-firmed as LAMN through histopathological examination.The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile:Positive for Ho-meobox protein CDX-2,Cytokeratin 20,special AT-rich sequence-binding protein 2,and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8.This profile aids in distinguishing appendiceal and ovarian mucinous tumors.Postoperative recovery was uncomplicated,and the patient initiated adjuvant chemotherapy for the colon AC.CONCLUSION This case highlights the diagnostic complexity of AMNs,emphasizing the need for vigilant identification to avert potential complications,such as pseudomyxoma peritonei.

Giant low-grade appendiceal mucinous neoplasm: A case report

BACKGROUND Appendiceal mucinous neoplasm (AMN) is extremely rare. Since the disease does not manifest a characteristic profile of clinical symptoms, it is easy to misdiagnose and still difficult to diagnose without operation. Here, we report a case of low-grade AMN (LAMN) and summarize its clinical features, diagnosis, and treatment. CASE SUMMARY A 63-year-old postmenopausal woman presented with a history of right lower abdominal mass. The patient underwent laparotomy, which showed an appendiceal mucocele originating from the apex of the appendix, and a simple appendectomy was performed. The subsequent histological assessment identified an LAMN with no lymph node involvement and negative surgical margin. The patient received six cycles of chemotherapy after surgery, and to date, more than a year after the surgery, the patient remains in good health. CONCLUSION A unified, standardized, detailed, and accurate pathological diagnosis is needed for LAMN, to facilitate selection of an appropriate surgical plan. In addition, the surgeon should record the details of the tumors in the surgical records in order to facilitate follow-up after surgery.

Reoperation for heterochronic intraductal papillary mucinous neoplasm of the pancreas after bile duct neoplasm resection:A case report

BACKGROUND Intraductal papillary neoplasm of the bile duct(IPNB)and intraductal papillary mucinous neoplasm(IPMN)of the pancreas have similar pathological manifestations.However,they often develop separately and it is rare for both to occur together.Patients presenting with heterochronic IPMN after IPNB are prone to be misdiagnosed with tumor recurrence.CASE SUMMARY A 67-year-old male patient was admitted 8.5 years after IPNB carcinoma and 4 years after the discovery of a pancreatic tumor.A left hepatic bile duct tumor with distal bile duct dilatation was found 8.5 years ago by the computed tomography;therefore,a left hepatectomy was performed.The postoperative pathological diagnosis was malignant IPNB with negative cutting edge and pathological stage T1N0M0.Magnetic resonance imaging 4 years ago showed cystic lesions in the pancreatic head with pancreatic duct dilatation,and carcinoembryonic antigen continued to increase.Positron emission tomography showed a maximum standard uptake value of 11.8 in the soft tissue mass in the pancreatic head,and a malignant tumor was considered.Radical pancreatoduodenectomy was performed.Postoperative pathological diagnosis was pancreatic head IPMN with negative cutting edge,pancreaticobiliary type,stage T3N0M0.He was discharged 15 d after the operation.Follow-up for 6 mo showed no tumor recurrence,and quality of life was good.CONCLUSION IPNB and IPMN are precancerous lesions with similar pathological characteristics and require active surgery and long-term follow-up.

Role of frozen section assessment for intraductal papillary and mucinous tumor of the pancreas

Intraductal papillary mucinous neoplasms(IPMN) of the pancreas include a spectrum of dysplasia ranging from minimal mucinous hyperplasia to invasive carcinoma and are extensive tumors that often spread along the ductal tree.Several studies have demonstrated that preoperative imaging is not accurate enough to adapt the extent of pancreatectomy and have suggested routinely using frozen sectioning(FS) to evaluate the completeness of resection and also to check if ductal dilatation is active or passive,in order to avoid an excessive pancreatic resection.Separate main duct and branch duct analysis is needed due to the difference in the natural history of the disease.FS accuracy averages 95%.Eroded epithelium on the main duct,severe ductal inflammation mimicking dysplasia and reactive epithelial changes secondary to obstruction can lead to inappropriate FS results.FS results change the planned extent of resection in up to 30% of cases.The optimal cut-off leading to extend pancreatectomy is not consensual and our standard option is to extend pancreatec-tomy if FS reveals:(1) at least IPMN adenoma on the main duct;or(2) at least borderline IPMN on branch ducts;or(3) invasive carcinoma.However,the decision to extend resection must be taken after a multidisciplinary discussion since it does not exclusively depend on the FS result but also on age,general condition and expected prognosis after resection.The main limitation of using FS is the existence of discontinuous("skip") lesions which account for approximately 10% of IPMN in surgical series and can lead to reoperation in up to 8% of cases.

Increased risk of second malignancy in pancreatic intraductal papillary mucinous tumors: Review of the literature

AIM: To analyze the available evidence about the risk of extrapancreatic malignancies and pancreatic ductal adenocarcinoma associated to pancreatic intraductal papillary mucinous tumors(IPMNs).METHODS: A systematic search of literature was undertaken using MEDLINE, EMBASE, Cochrane and Web-of-Science libraries. No limitations for year of publication were considered; preference was given to English papers. All references in selected articles were further screened for additional publications. Both clinical series and Literature reviews were selected. For all eligible studies, a standard data extraction form was filled in and the following data were extracted: study design, number of patients, prevalence of pancreatic cancer and extrapancreatic malignancies in IPMN patients and control groups, if available.RESULTS: A total of 805 abstracts were selected and read; 25 articles were considered pertinent and 17 were chosen for the present systematic review. Eleven monocentric series, 1 multicentric series, 1 casecontrol study, 1 population-based study and 3 case report were included. A total of 2881 patients were globally analyzed as study group, and the incidence of pancreatic cancer and/or extrapancreatic malignancies ranged from 5% to 52%, with a mean of 28.71%.When a control group was analyzed(6 papers), the same incidence was as low as 9.4%.CONCLUSION: The available Literature is unanimous in claiming IPMNs to be strongly associated with pancreatic and extrapancreatic malignancies. The consequences in IPMNs management are herein discussed.

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.

Malignant transformation of pulmonary bronchiolar adenoma into mucinous adenocarcinoma:A case report

BACKGROUND Bronchiolar adenoma(BA)and ciliated muconodular papillary tumor are rare tumors that have bilayered cell proliferation and continuous expression of p40 and CK5/6 in the basal cell layer.Diagnosis is difficult because of the limited knowledge of these tumors and their morphological similarities to malignant tumors,including invasive mucinous adenocarcinoma,especially based on the histopathology of intraoperative frozen sections.These tumors are now considered to be benign neoplasms,with malignant transformation reported in only a few cases.CASE SUMMARY A 57-year-old woman presented with a 17.0 mm×7.0 mm nodule in the lower lobe of the left lung.Hematoxylin-eosin staining and immunohistochemistry of a surgical specimen were performed.The tumor consisted of a BA area and a mucinous adenocarcinoma(MA)area.In the BA area,the tumor had a bilayered structure of luminal cells and basal cells.The basal cells were positive for CK5/6 and p40,but the MA area was negative for these biomarkers.The Ki-67 proliferation index was low(1%-2%).The patient was diagnosed with BA accompanied by MA,and had a favorable outcome.CONCLUSION The present study indicated that BA may be carcinogenic,and suggests that clinicians should be aware of its potential for malignant transformation.

Visualizing the landscape of appendiceal tumor research after 2010: A bibliometric study

BACKGROUND Despite the rarity of appendiceal tumors,research in this field has intensified,resulting in a growing number of studies and published papers.Surprisingly,no comprehensive bibliometric analysis has specifically addressed appendiceal tumors.AIM To offer a thorough analysis of the current landscape and future trends in appendiceal tumor research.METHODS In our bibliometric analysis studies,we explored the Web of Science Core Collection database.The bibliographic details of the chosen publications were automatically converted and analyzed using the bibliometric package in the R environment.Additionally,we employed VoSviewer to create cooperation network maps for countries,institutions,and authors,as well as clustering maps for keywords.Furthermore,CiteSpace,another software tool,was utilized to build dual-map overlays of journals and analyze references with citation bursts.RESULTS Our study included 780 English-language articles published after 2010.The number of related publications and citations has increased in the past decade.The United States leads in this area,but there is a need to improve cooperation and communication among countries and institutions.Co-occurrence analysis also revealed close collaboration among different authors.Annals of Surgical Oncology was the most influential journal in this field.Analysis of references with high cocitations and references with citation bursts,consistent with analysis of keywords and hotspots,indicated that current research primarily centers on the classification and management of appendiceal mucinous neoplasms and consequent pseudomyxoma peritonei.Despite the abundance of clinical studies,a greater number of in-depth basic research studies should be conducted.CONCLUSION Current research on appendiceal tumors focuses on classification and management of appendiceal mucinous neoplasms and pseudomyxoma peritonei.Enhanced collaboration and basic research are vital for further advancement.

Validation of serum tumor biomarkers in predicting advanced cystic mucinous neoplasm of the pancreas

BACKGROUND Early detection of advanced cystic mucinous neoplasms[(A-cMNs),defined as high-grade dysplasia or malignancy]of the pancreas is of great significance.As a simple and feasible detection method,serum tumor markers(STMs)may be used to predict advanced intraductal papillary mucinous neoplasms(IPMNs)and mucinous cystic neoplasms(MCNs).However,there are few studies on the usefulness of STMs other than carbohydrate antigen(CA)19-9 for early detection of A-cMNs.AIM To study the ability of five STMs-CA19-9,carcinoembryonic antigen(CEA),CA125,CA724,and CA242 to predict A-cMNs and distinguish IPMNs and MCNs.METHODS We mainly measured the levels of each STM in patients pathologically diagnosed with cMNs.The mean levels of STMs and the number of A-cMN subjects with a higher STM level than the cutoff were compared respectively to identify the ability of STMs to predict A-cMNs and distinguish MCNs from IPMNs.A receiver operating characteristic curve with the area under curve(AUC)was also created to identify the performance of the five STMs.RESULTS A total of 187 patients with cMNs were identified and 72 of them showed AcMNs.We found that CA19-9 exhibited the highest sensitivity(SE)(54.2%)and accuracy(76.5%)and a moderate ability(AUC=0.766)to predict A-cMNs.In predicting high-grade dysplasia IPMNs,the SE of CA19-9 decreased to 38.5%.The ability of CEA,CA125,and CA724 to predict A-cMNs was low(AUC=0.651,0.583,and 0.618,respectively).The predictive ability of CA242 was not identified.The combination of STMs improved the SE to 62.5%.CA125 may be specific to the diagnosis of advanced MCNs.CONCLUSION CA19-9 has a moderate ability,and CEA,CA125,and CA724 have a low ability to predict A-cMNs.The combination of STM testing could improve SE in predicting A-cMNs.

Ovarian mucinous tumor with mural nodules of anaplastic carcinoma:Three case reports

BACKGROUND Anaplastic carcinoma mural nodules in ovarian mucinous tumors are very rare.This study aimed to report the morphological characteristics,molecular detection results,clinical treatment and prognosis of three ovarian mucinous tumors with mural nodules of anaplastic carcinoma.CASE SUMMARY The pathomorphological features,molecular detection results,clinical treatment and prognosis of anaplastic carcinoma mural nodules were described in three cases.In case 1,sarcoma-like mural nodules(SLMNs)coexisted with anaplastic carcinoma mural nodules.No mutation was found in mucinous tumors.KRAS mutation was found in anaplastic carcinoma nodules and heterotypic cells were found in SLMNs.In case 2,KRAS mutation occurred in the mucinous epithelium and BRAF mutation occurred in mural nodules.In case 3,both mural nodules and mucinous tumors had the same KRAS mutation and a morphological transition between them was observed.All three patients died within 2 years,whether receiving chemotherapy or not.CONCLUSION Anaplastic carcinoma mural nodules may develop from dedifferentiation of mucinous tumors or are unrelated to mucinous tumors.

MSCT and MRCP features of intraductal papillary mucinous tumor of the pancreas

Objective:To study the multi-slice CT(MSCT) and magnetic resonance cholangio-pancreatography(MRCP) features of intraductal papillary mucinous tumor(IPMT) of the pancreas.Methods:The clinical information,laboratory val-ues,imaging features and pathological findings of 8 cases were reviewed.Results:There were 6 males and 2 females with average 71.3 years old in this series.These lesions involved pancreatic branch ducts or main duct.Among them,2 cases were involved in the pancreatic uncinate process,1 case in the pancreatic head,1 in the pancreatic body,2 in the pancreatic tail and 2 cases involved multiple pancreatic duct.The common complaint was chronic upper abdominal pain.The imaging features included cystic lesion and it's association with the dilated pancreatic main duct,and bulging of the duodenal papilla.Mucin plug,septa or mural nodule were found in these cystic lesions.5 cases were confirmed with endoscopic retrograde cholangio-pancreatography(ERCP).3 cases had undergone surgery.Pathological findings of the 3 cases were adenoma,adenocarcinoma and borderline tumor,respectively.Conclusion:The MSCT and MRCP features of IPMT are somewhat characteristic,and the correct diagnosis can be made with the combination of clinical features.Diameter of branch duct ≥ 3 cm with multiple or large mural nodules,or severe dilated main duct indicates the probability of malignant IPMT.

Study on the mechanism of Chaihu DaYuan Yin in improving the tumor microenvironment of colorectal mucinous adenocarcinoma

Colorectal mucinous adenocarcinoma has the characteristics of high malignant degree,high mortality and low survival rate,easy to recurrence and metastasis after operation,and the prognosis of survival patients is also very poor.The research on immune-related therapy and tumor microenvironment has become a research hotspot.From the point of view of traditional Chinese medicine,Chaihu Dayuan Yin can dispel dampness,resolve phlegm and regulate Sanjiao membrane.It is suitable for tumors with phlegm-dampness and qi stagnation,especially those with abdominal and pelvic dampness heavier than heat,such as colorectal mucinous adenocarcinoma and so on.The concept of resolving phlegm and dredging Sanjiao based on the addition and subtraction of Chaihu Dayuan Yin is of great value to the clinical treatment and basic scientific research of tumors.

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.

molecular pathology of intraductal papillary mucinous neoplasms of the pancreas

Since the first description of intraductal papillary mucinous neoplasms(IPMNs)of the pancreas in the eighties,their identification has dramatically increased in the last decades,hand to hand with the improvements in diagnostic imaging and sampling techniques for the study of pancreatic diseases.However,the heterogeneity of IPMNs and their malignant potential make difficult the management of these lesions.The objective of this review is to identify the molecular characteristics of IPMNs in order to recognize potential markers for the discrimination of more aggressive IPMNs requiring surgical resection from benign IPMNs that could be observed.We briefly summarize recent research findings on the genetics and epigenetics of intraductal papillary mucinous neoplasms,identifying some genes,molecular mechanisms and cellular signaling pathways correlated to the pathogenesis of IPMNs and their progression to malignancy.The knowledge of molecular biology of IPMNs has impressively developed over the last few years.A great amount of genes functioning as oncogenes or tumor suppressor genes have been identified,in pancreatic juice or in blood or in the samples from the pancreatic resections,but further researches are required to use these informations for clinical intent,in order to better define the natural history of these diseases and to improve their management.

Mucinous nonneoplastic cyst of the pancreas: A truly novel pathological entity?

Recently, a novel and distinct pancreatic cystic tumor termed 'mudnous nonneoplastic' cyst was described in the literature. We report our experience with a 71-year-old female with a cystic tumor in the body of the pancreas demonstrating features suggestive of this diagnosis. We also review the literature regarding this 'novel' pathological entity and discuss critically its existence and its differential diagnoses.

Anaplastic carcinoma associated with a mucinous cystic neoplasm of the pancreas during pregnancy:Report of a case and a review of the literature

Oncogenesis of anaplastic carcinoma of the pancreas is a subject of controversy, because it shows sarcomatous nature with extremely poor prognosis. We herein report an unusual case of anaplastic carcinoma occurring with a recurrent mucinous cystic neoplasm in a 38-year-old female. A 10-cm retroperitoneal cystic mass was pointed out in the first pregnancy and a probable diagnosis of mucinous cystic neoplasm was made in October 2000. She refused surgery first and delivered her baby uneventfully. During her second pregnancy in 2002, however, she presented hematemesis and underwent urgent distal pancreatectomy, splenectomy and partial resection of the gastric wall where the tumor perforated. A diagnosis of borderline-type mucinous cystic neoplasm with ovarian-like stroma was made. Nine months later, CT visualized a recurrent cystic tumor near the pancreatic stump, which was subsequently resected. Pathology revealed that the tumor was composed of two different components of borderline-type mucinous cystic neoplasm and anaplastic carcinoma. The latter was intensely positive for vimentin, CD68, p53 and focally for cytokeratin, suggesting both sarcomatous and carcinomatous differentiation. She survived four years after the second surgery without tumor recurrence. Although the origin of anaplastic carcinoma has not been determined yet, it should be remembered that anaplastic carcinoma can occur in association with mucinous cystic neoplasm of more benign histology.