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Cancers Apr 2023The most common tumour of the pancreas is ductal adenocarcinoma (PDAC). It remains one of the most lethal non-neuroendocrine solid tumours despite the use of a... (Review)
Review
The most common tumour of the pancreas is ductal adenocarcinoma (PDAC). It remains one of the most lethal non-neuroendocrine solid tumours despite the use of a multi-approach strategy. Other, less-common neoplasms, which are responsible for 15% of pancreatic lesions, differ in treatment and prognosis. Due to the low incidence rate, there is a lack of information about the rarest pancreatic tumours. In this review, we described six rare pancreatic tumours: intraductal papillary mucinous neoplasm (IPMN), mucinous cystadenoma (MCN), serous cystic neoplasm (SCN), acinar cell carcinoma (ACC), solid pseudopapillary neoplasm (SPN) and pancreatoblastoma (PB). We distinguished their epidemiology, clinical and gross features, covered the newest reports about courses of treatment and systematised differential diagnoses. Although the most common pancreatic tumour, PDAC, has the highest malignant potential, it is still essential to properly classify and differentiate less-common lesions. It is vital to continue the search for new biomarkers, genetic mutations and the development of more specific biochemical tests for determining malignancy in rare pancreatic neoplasms.
PubMed: 37190144
DOI: 10.3390/cancers15082216 -
Journal of Gastrointestinal and Liver... Sep 2020
Topics: Biopsy; Cystadenoma, Mucinous; Duodenal Neoplasms; Duodenoscopy; Female; Humans; Middle Aged; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 32830822
DOI: 10.15403/jgld-2497 -
Archives of Pathology & Laboratory... Feb 2019Urachal neoplasms are uncommon and represent a minor portion of bladder tumors. According to the recently updated World Health Organization classification (2016), these... (Review)
Review
Urachal neoplasms are uncommon and represent a minor portion of bladder tumors. According to the recently updated World Health Organization classification (2016), these tumors are classified as adenomas, adenocarcinomas, nonglandular neoplasms, and mixed carcinomas. The mucinous cystic neoplasms represent a small percentage of urachal tumors with morphologic spectrum ranging from benign mucinous cystadenoma to borderline mucinous cystic tumor of low malignant potential and to malignant mucinous cystadenocarcinoma. Benign urachal mucinous cystic adenomas are exceedingly rare, and only a few cases have been reported in the literature to date. The goal of this review is to summarize the clinical features, histopathologic characteristics, treatment, and prognosis of urachal mucinous cystadenoma in light of differentiating them from mucinous cystic tumor of low malignant potential and mucinous cystadenocarcinoma.
Topics: Adenocarcinoma; Adolescent; Adult; Aged; Cystadenoma, Mucinous; Female; Humans; Male; Middle Aged; Urinary Bladder Neoplasms; Young Adult
PubMed: 30398914
DOI: 10.5858/arpa.2017-0319-RS -
Revista Espanola de Enfermedades... May 2017The real prevalence of pancreatic cystic lesions remains unknown. The malignant potential of some of these lesions remains a cause for significant concern. Thus, it is... (Review)
Review
The real prevalence of pancreatic cystic lesions remains unknown. The malignant potential of some of these lesions remains a cause for significant concern. Thus, it is mandatory to develop a strategy to clearly discriminate those cysts with a potential for malignant transformation from those that do not carry any significant risk. Intraductal papillary mucinous neoplasms and mucinous cystadenomas are mucinous cystic neoplasms with a known malignant potential that have gained greater recognition in recent years. However, despite the numerous studies that have been carried out, their differential diagnosis among other cysts subtypes and their therapeutic approach continue to be a challenge for clinicians. This review contains a critical approach of the current recommendations and management strategies regarding intraductal papillary mucinous neoplasms and mucinous cystadenomas, as well as highlighting the limitations exposed in current guidelines.
Topics: Cystadenoma, Mucinous; Diagnosis, Differential; Humans; Pancreatic Neoplasms; Practice Guidelines as Topic; Prognosis
PubMed: 28112959
DOI: 10.17235/reed.2017.4630/2016 -
International Journal of Surgery... Jun 2015Mucocoele of the appendix is rarely encountered but consultant general surgical and trainees must be aware of it as a differential diagnosis, due to the nuances... (Review)
Review
INTRODUCTION
Mucocoele of the appendix is rarely encountered but consultant general surgical and trainees must be aware of it as a differential diagnosis, due to the nuances associated with its management. We aimed to provide a comprehensive review of the current literature concerning this rare surgical pathology.
METHODS
Search terms "appendi*", "tumour", "malignancy", "mucino*" and "cystadenoma" were used in combination to identify papers from PubMed. Abstracts and full text were manually reviewed to identify suitable papers.
RESULTS
Full search results included 311 articles. Review of titles and abstracts led to further full text review of 46 articles. Of these 30 were selected for inclusion based on relevance, adequate sample size and recent publication date.
DISCUSSION
Mucocoele of the appendix describes dilatation with associated luminal mucin and can result from benign and malignant processes. It contributes 0.2-0.7% of all appendiceal pathologies. The most common presenting symptoms are abdominal pain and a palpable mass in the right iliac fossa. Computed tomography of the abdomen and pelvis is key in facilitating diagnosis, although CEA and CA19-9 also have a role. The major complication of malignant causes of mucocoele is progression to pseudomyxoma peritonei. Treatment is surgical with or without chemotherapy depending on the underlying cause. Prognosis depends on aetiology.
CONCLUSION
Mucocoele of the appendix is a rare diagnosis. However, given the possibility of neoplastic peritoneal dissemination, it should be considered as a diagnosis, especially in older females with non-specific symptoms similar to appendicitis.
Topics: Abdominal Pain; Appendiceal Neoplasms; Appendicitis; Cecal Diseases; Cystadenoma, Mucinous; Diagnosis, Differential; Female; Humans; Mucocele; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Tomography, X-Ray Computed
PubMed: 25917270
DOI: 10.1016/j.ijsu.2015.04.052 -
BMJ Case Reports Oct 2020A 60-year-old woman was investigated for abdominal pain and increasing asthenia. Abdominal CT revealed a 25 mm hypodense cystic lesion in the tail of the pancreas. MRI...
A 60-year-old woman was investigated for abdominal pain and increasing asthenia. Abdominal CT revealed a 25 mm hypodense cystic lesion in the tail of the pancreas. MRI showed a multiloculated cystic lesion, T1-hypointense and T2-hyperintense lesion, without wall enhancement. Endoscopic ultrasound detected a 25 mm multi-loculated cystic lesion, with regular margin and without pancreatic duct communication. Diagnosis of pancreatic mucinous cystadenoma was discussed and the patient was referred to surgery. She underwent distal pancreatectomy with spleen preservation. Pathological examination revealed the diagnosis of pancreatic mesothelial cyst. Histologically, the cyst was multiloculated, lined by cuboidal epithelium, ovoid nuclei and amphophilic cytoplasm, without mucin deposition or cytological atypia. Immunohistochemistry examination revealed positive staining for cytokeratin 5/6, vimentin and calretinin. At 1-year follow-up, she is in her usual health, without any symptoms.
Topics: Cystadenoma, Mucinous; Diagnosis, Differential; Endosonography; Epithelium; Female; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Middle Aged; Pancreas; Pancreatectomy; Pancreatic Cyst; Pancreatic Neoplasms; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 33028569
DOI: 10.1136/bcr-2020-236255 -
Journal of Clinical and Translational... Jun 2019Biliary mucinous cystadenomas are cystic neoplasms commonly mistaken for simple cysts. They are rare and generally benign tumors, often incidentally found on imaging and... (Review)
Review
Biliary mucinous cystadenomas are cystic neoplasms commonly mistaken for simple cysts. They are rare and generally benign tumors, often incidentally found on imaging and during unrelated surgical interventions. They tend to be slow growing though may reach symptomatic dimensions. Misdiagnosis of biliary mucinous cystadenomas may have serious consequences secondary to their potential for malignant transformation into biliary mucinous cystadenocarcinomas. Here, we review the epidemiology, etiology, pathology, diagnostic modalities, histology, and available treatment methods for mucinous cystadenomas reported in current literature.
PubMed: 31293915
DOI: 10.14218/JCTH.2019.00017 -
Journal of Pediatric and Adolescent... Aug 2017Mucinous cystadenomas (MCAs) are benign epithelial ovarian tumors that occur rarely in children and adolescents. Because children and adolescents typically have their...
STUDY OBJECTIVE
Mucinous cystadenomas (MCAs) are benign epithelial ovarian tumors that occur rarely in children and adolescents. Because children and adolescents typically have their childbearing years ahead of them, conservative therapy is indicated. However, there is concern that ovarian cystectomy might be associated with significant recurrence risk in patients with MCA. Furthermore, guanine nucleotide binding protein, alpha stimulating (GNAS) gene mutations are associated with McCune-Albright syndrome, which is associated with cystic ovaries. We sought to evaluate the outcomes of children and adolescents with MCA treated conservatively. A subset of patients underwent GNAS gene testing.
DESIGN, SETTING, PARTICIPANTS, AND INTERVENTIONS
After institutional board review approval, the pathology database of a large urban children's hospital was queried to identify adolescents with MCA between the years 2008 and 2014. Fourteen patients, aged 8-18 years (median, 14), were identified. A buccal swab for genetic testing was obtained from a subset of consenting patients.
MAIN OUTCOME MEASURES
MCA recurrence; ovarian return to normal size; GNAS gene variants.
RESULTS
Two patients underwent oophorectomies, and the remaining 12 underwent cystectomies. Follow-up ultrasound examination revealed slow return of ovary to normal size. Of the 10 patients with available follow-up data, there were no recurrences at a median of 225 days from surgery. Four patients consented to a buccal swab for genetic testing, and the GNAS gene was noted to have rare variants in 2 patients.
CONCLUSION
This series supports the use of ovary-sparing surgery in the treatment of MCA. Further research exploring possible genetic variants such as the GNAS gene in children and adolescents diagnosed with MCA is warranted.
Topics: Adolescent; Child; Chromogranins; Cystadenoma, Mucinous; Databases, Factual; Female; GTP-Binding Protein alpha Subunits, Gs; Humans; Mutation; Neoplasm Recurrence, Local; Ovarian Neoplasms; Ovariectomy
PubMed: 28216128
DOI: 10.1016/j.jpag.2017.02.001 -
International Journal of Surgery Case... May 2022Mucinous cystadenoma occurs in 10-15% of all ovarian tumors. Diagnosis and treatment should be decided precisely as it has a chance to develop into pseudomyxoma...
INTRODUCTION AND IMPORTANCE
Mucinous cystadenoma occurs in 10-15% of all ovarian tumors. Diagnosis and treatment should be decided precisely as it has a chance to develop into pseudomyxoma peritonei (PMP). Management of PMP might be challenging especially when repeated surgery is needed.
CASE PRESENTATION
The first case, a 22-year-old lady with recurrent stomach enlargement for seven months. She had history of laparotomy surgery due to an ovarian tumor. Whole abdomen contrast CT scan showed a large cyst with mucinous fluid. We decided to do re-laparotomy and found a left ovarian cyst. Histological examination results confirm ovarian mucinous cystadenoma. The second case was, 55-year-old woman, with abdominal enlargement for six months. She had a history of laparotomy and chemotherapy due to pseudomyxoma peritonei. Post chemotherapy MRI showed persistent pseudomyxoma and two multilocular cysts from both adnexa. Debulking laparotomy was then conducted. We obtained 8 L of mucinous pseudomyxoma along with mucinous cyst from both ovaries. The final diagnosis concluded as a pseudomyxoma and we decide to close the follow-up of the patient.
CLINICAL DISCUSSION
Pseudomyxoma is caused by the production of mucin originating from intra-abdominal organs. Open surgery should be prioritized when the mucinous cystadenoma is detected to do a complete peritoneum evaluation and avoid perioperatively ruptured mucinous neoplasm. Pseudomyxoma often needed repeated surgical treatment and may exhibit different surgical findings and different pathologies.
CONCLUSION
Repeated surgery is logical and still no need for adjuvant chemotherapy in both cases. Accurate and precise diagnosis should be prioritized in order to prevent repeated surgery.
PubMed: 35658307
DOI: 10.1016/j.ijscr.2022.107141