Primary mucinous tumors of the renal pelvis: Clinical, histopathological, and molecular analysis of three cases.

Primary mucinous tumors of the renal pelvis are extremely rare and pose challenges in terms of diagnosis and treatment. This study reviewed the clinical and pathological characteristics of mucinous tumors of the renal pelvis, including mucinous cystadenocarcinomas and mucinous cystadenomas. Immunohistochemical analysis was conducted in three cases, along with KRAS gene detection using the Amplification Refractory Mutation System (ARMS) method. The results revealed mucinous epithelium with acellular mucinous pools in all cases, and acellular mucinous pools were observed in the renal parenchyma and perirenal fat capsules. All tumors expressed CK20 and CDX2, and one case showed KRAS gene mutation. The study suggests that mucinous cystadenomas of the renal pelvis may exhibit borderline biological behaviors. This study is the first to report a KRAS gene mutation in a mucinous cystadenoma of the renal pelvis, offering valuable insights into the diagnosis and treatment of this rare condition.

Mucinous cystadenoma and benign mesonephric-like proliferation in the ovary - Further evidence for clonal relationship.

Mesonephric-like adenocarcinomas rarely occur in the uterus and the ovary. Benign mesonephric-like (ML) proliferations and hyperplasia have been described solely within the ovary. Pathogenetic data are very limited. We report a case with microscopic focus of benign ML-proliferation in association with mucinous cystadenoma in the ovary. The immunophenotype was distinct (mucinous tumor: focal weak nuclear positivity for PAX-8, CK 7, patchy cytoplasmic positivity for p16 and negativity for estrogen receptor, CD 10, TTF-1, p53 wildtype; mesonephric component: diffusely positive for PAX-8, CK 7, luminal CD 10, TTF-1, focal staining for estrogen receptor, patchy cytoplasmic for p16, p53 wildtype). On NGS-analysis there was clonal mutation of KRAS p.G12C. The data provide additional evidence for the concept of transdifferentiation (Müllerian tissue representing Wolffian/mesonephric features on histology and immunostaining) within the pathogenesis of mesonephric proliferation of the female genital tract and demonstrate the clonal relationship between these distinct morphologic components.

Implications of pregnancy on MCN of the pancreas: A multicentric case-control study.

BACKGROUND: Mucinous cystic neoplasms (MCN) of the pancreas express estrogen and progesterone receptors. Several case reports describe MCN increasing in size during gestation. The aim of this study is to assess if pregnancy is a risk factor for malignant degeneration of MCN. METHODS: All female patients who underwent pancreatic resection of a MCN between 2011 and 2021 were included. MCN resected or diagnosed within 12 months of gestation were defined perigestational. MCN with high grade dysplasia or an invasive component were classified in the high grade (HG) group. The primary outcome was defined as the correlation between exposure to gestation and peri-gestational MCN to development of HG-MCN. RESULTS: The study includes 176 patients, 25 (14 %) forming the HG group, and 151 (86 %) forming the low grade (LG) group. LG and HG groups had a similar distribution of systemic contraceptives use (26 % vs. 16 %, p = 0.262), and perigestational MCN (7 % vs 16 %, p = 0.108). At univariate analysis cyst size ≥10 cm (OR 5.3, p < 0.001) was associated to HG degeneration. Peri gestational MCN positively correlated with cyst size (R = 0.18, p = 0.020). In the subgroup of 14 perigestational MCN patients 29 % had HG-MCN and 71 % experienced cyst growth during gestation with an average growth of 55.1 ± 18 mm. CONCLUSIONS: Perigestational MCN are associated to increased cyst diameter, and in the subset of patients affected by MCN during gestation a high rate of growth was observed. Patients with a MCN and pregnancy desire should undergo multidisciplinary counselling.

Mayer-Rokitansky-Kuster-Hauser syndrome complicated with giant mucinous cystadenoma and inguinal herniation: case report.

INTRODUCTION: Coexistence of Mayer-Rokitansky-Kuster-Hauster syndrome (MRKH) with other conditions is rare, especially when MRKH was found in a young woman presenting with ovarian malignancy. This case report wishes to highlight MRKH complicated with giant mucinous cystadenoma and bilateral inguinal hernia. CASE REPORT: A 22-year-old nulligravid woman was admitted with primary amenorrhea and abdominal mass. Abdominal examination revealed a cystic mass 25 × 25 × 20 cm in size and a vagina 1 cm in length. Pelvic magnetic resonance imaging (MRI) showed a giant multiloculated left ovarian mass amidst the absence of uterus. During the surgery, the giant multiloculated cystic mass was identified as mucinous cystadenoma on frozen section. Bilateral medial inguinal hernia was also identified. DISCUSSION: MRKH coexisting with other disease is rare but considering other structures arising from paramesonephric duct (PMD) may exist, allows the possibility of other structural anomalies. CONCLUSIONS: The present report illustrates a rare case of MRKH syndrome with giant ovarian cystadenoma and inguinal hernia.

Mucinous cystadenoma of the ovary with xanthogranulomatous oophoritis: the first case report in the literature.

This case report presents a unique and unprecedented occurrence of mucinous cystadenoma of the ovary accompanied by xanthogranulomatous oophoritis, a rare inflammatory condition. To the best of our knowledge, this is the first documented case of its kind in the medical literature. The patient, a 25-year-old woman, presented with abdominal pain, fever, and discomfort, prompting further investigation that led to the unexpected discovery of these coexisting pathologies.

Anaplastic Carcinoma Arising From a Mural Nodule in Benign Mucinous Cystadenoma.

Giant abdominopelvic tumors continue to present a diagnostic and therapeutic challenge for all surgeons despite all the advances in the world of imaging. Particularly, one of the most important challenges is to determine its probable origin for adequate surgical planning. Even though mostly all of these tumors are benign ovarian tumors, extraordinarily, malignant mural nodules may develop from the wall of these benign tumors, carrying an invariable unfavorable prognosis for the patient. This case highlights the importance of a correct diagnostic approach using ultrasound and abdominal computed tomography scans and confirming the diagnosis through a histopathologic examination. The treatment for these cases is surgical resection and posterior oncological treatment if needed. This case shows how timely treatment is one of the principal determinators of morbidity and mortality.

Giant ovarian mucinous cystadenoma in Derna, Libya

Giant mucinous cystadenomas of the ovary are seldom documented in literature within developed countries, primarily due to the widespread availability of diagnostic technologies. However, in developing nations, such situations pose a challenge owing to limited access to diagnostic tools and constrained resources. Herein, we present the case of 35-year-old women, who reported a three-year history of a progressively enlarging giant mucinous cystadenoma which was initially mistaken for obesity, accompanied by abdominal distension and pain, and was not diagnosed during the cesarean section performed three weeks prior to the significant increase in size. Cystectomy performed, the resected cyst measured 25x23x17 cm, with no evidence of ascites. Subsequent histopathological analysis confirmed the diagnosis of mucinous cystadenoma. The patient experienced a complete postoperative recovery without any complications. Notably, the presence of vague gastrointestinal symptoms unrelated to the actual diagnosis underscores the importance of considering giant ovarian cysts as a potential differential diagnosis for ambiguous abdominal symptoms, given the varied clinical presentations they may manifest. This case underscores the necessity for multidisciplinary collaboration among various specialists to facilitate early diagnosis. Furthermore, it emphasizes the indispensable role of histopathological examination in ensuring accurate diagnosis and appropriate management of ovarian cysts

An enormous benign primary retroperitoneal mucinous cystadenoma: a case report and literature review of a seldom seen abdominal pathology.

Introduction and importance: Retroperitoneal neoplasia comprise less than 2% of all tumours. benign primary retroperitoneal mucinous cystadenoma (PRMC) is an extremely rare tumour. Their clinical course is overall silent unless the patient presents with a vague abdominal or pelvic pain, abdominal distention, or a palpable mass. Their aetiology remains theorized and since 1989, only 46 cases (excluding ours) worldwide were documented in the literature. The majority of cases were discovered in females but the overall tumour incidence rate is still undetermined due to its rarity. Well-timed recognition of this pathology permits the necessary curative surgical intervention to take place. Case presentation: We hereby illustrate the rare case of a 23-year-old female who presented to the surgical clinic complaining solely of an unexplained gradual increase of the abdominal contour. Their presurgical radiological analysis yielded an intraabdominal large-sized well-demarcated retroperitoneal mass. Clinical discussion: Thorough resection of the mass was accomplished via open surgery. The subsequent microscopic analysis of excised tumour yielded the diagnosis of primary retroperitoneal mucinous cystadenoma of benign nature. Conclusion: Primary retroperitoneal mucinous cystadenoma is a seldom seen tumour. The scarcity of its occurrence is further highlighted by the published data. Based on their conclusive review of the available published English-based literature, ours is the 47th documented case of a benign PRMC and it is the first documented case from our country; Syria. The impact of these findings warrants raising awareness on the subject and considering PRMC as a differential diagnosis when presented with a similar case in the clinical practice.

Bilateral Ovarian Torsion Due to a Giant Mucinous Cystadenoma, Contralateral Mature Teratoma, and Paratubal Cyst in a Young Adult.

Giant ovarian cysts (>10 cm) are rare due to the widespread use of routine imaging. However, in the absence of compressive symptoms, giant cysts remain a diagnostic challenge, especially in patients with larger body habitus. Complications of benign ovarian cysts are infrequent but can include torsion of the adnexa. Bilateral torsion is a rare emergency that can threaten a patient's future fertility. In this case, we report on a 24-year-old female with bilateral torsion due to a triad of adnexal masses including a 30 cm mucinous cystadenoma, a 10 cm mature teratoma, and an 8 cm paratubal cyst. Controlled fluid aspiration was performed prior to en bloc resection of the cystadenoma due to the emergent nature of the case and lack of malignant features.

Primary Retroperitoneal Serous Cyst Adenoma: A Case Report and Literature Review.

Primary retroperitoneal serous cyst adenomas (PRSCs) are extremely rare thin-walled cystic lesions whose pathogenesis is not well understood. Clinical presentation varies depending on the lesion's size and location, i.e., larger lesions compress adjacent organs, giving the impression of malignancy. Although advances in imaging techniques enable to identify various characteristics of retroperitoneal cystic lesions, there are no pathognomonic signs to confirm the diagnosis. The exact diagnosis is based on the histology after complete surgical excision. An open surgical approach is considered the traditional method of complete resection; however, laparoscopic techniques have increasingly been employed. Diagnostic aspiration is discouraged due to the potential risk of seeding if the lesion is malignant. We present the case of a 51-year-old woman who underwent complete excision of a large right retroperitoneal cyst, histologically confirmed as PRSC with a review of the background and management options of this phenomenon.

Application of Ultrasound Scores (Subjective Assessment, Simple Rules Risk Assessment, ADNEX Model, O-RADS) to Adnexal Masses of Difficult Classification.

BACKGROUND: Ultrasound features help to differentiate benign from malignant masses, and some of them are included in the ultrasound (US) scores. The main aim of this work is to describe the ultrasound features of certain adnexal masses of difficult classification and to analyse them according to the most frequently used US scores. METHODS: Retrospective studies of adnexal lesions are difficult to classify by US scores in women undergoing surgery. Ultrasound characteristics were analysed, and masses were classified according to the Subjective Assessment of the ultrasonographer (SA) and other US scores (IOTA Simple Rules Risk Assessment-SRRA, ADNEX model with and without CA125 and O-RADS). RESULTS: A total of 133 adnexal masses were studied (benign: 66.2%, n:88; malignant: 33.8%, n:45) in a sample of women with mean age 56.5 ± 7.8 years. Malignant lesions were identified by SA in all cases. Borderline ovarian tumors (n:13) were not always detected by some US scores (SRRA: 76.9%, ADNEX model without and with CA125: 76.9% and 84.6%) nor were serous carcinoma (n:19) (SRRA: 89.5%), clear cell carcinoma (n:9) (SRRA: 66.7%) or endometrioid carcinoma (n:4) (ADNEX model without CA125: 75.0%). While most teratomas and serous cystadenomas have been correctly differentiated, other benign lesions were misclassified because of the presence of solid areas or papillae. Fibromas (n:13) were better identified by SA (23.1% malignancy), but worse with the other US scores (SRRA: 69.2%, ADNEX model without and with CA125: 84.6% and 69.2%, O-RADS: 53.8%). Cystoadenofibromas (n:10) were difficult to distinguish from malignant masses via all scores except SRRA (SA: 70.0%, SRRA: 20.0%, ADNEX model without and with CA125: 60.0% and 50.0%, O-RADS: 90.0%). Mucinous cystadenomas (n:12) were misdiagnosed as malignant in more than 15% of the cases in all US scores (SA: 33.3%, SRRA: 16.7%, ADNEX model without and with CA125: 16.7% and 16.7%, O-RADS:41.7%). Brenner tumors are also difficult to classify using all scores. CONCLUSION: Some malignant masses (borderline ovarian tumors, serous carcinoma, clear cell carcinoma, endometrioid carcinomas) are not always detected by US scores. Fibromas, cystoadenofibromas, some mucinous cystadenomas and Brenner tumors may present solid components/papillae that may induce confusion with malignant lesions. Most teratomas and serous cystadenomas are usually correctly classified.

Appendiceal mucocele as an exceptional cause of ileocecocolic intussusception in adults: a case report.

BACKGROUND: Intussusception is a rare condition in adults, accounting for 5% of intestinal intussusception and being responsible for approximately 1% of all adult bowel obstructions. Neoplastic origin is the most common etiology of intestinal intussusception in adults, unlike pediatric intussusception, which is usually idiopathic. Intussusception due to the appendiceal mucocele is exceptional, and only a few cases have been reported in the medical literature. CASE PRESENTATION: We report the case of a 25-year-old black African male patient with no medical history. He presented to the emergency department for abdominal pain, nausea, and bilious vomiting. The abdominal examination revealed typical signs of acute bowel obstruction. Enhanced abdominopelvic computed tomography showed an invagination of the last ileal loop, cecum, and ascending colon into the lumen of the transverse colon, with a rounded image with hypodense content and some calcifications compatible with an appendiceal mucocele. An emergency exploratory laparoscopy was performed and confirmed the ileocecocolic intussusception. Right hemicolectomy and ileocolic anastomosis were performed. The patient recovery postoperatively was uneventful, and he was discharged 4 days later. Histological examination of the surgical specimen confirmed the diagnosis of mucinous cystadenoma. CONCLUSION: The symptoms of bowel intussusception with the appendiceal mucocele as the lead point in adults are similar to any other bowel intussusception. Differential diagnosis is often carried out thanks to the injected abdominal computed tomography scan.

Mucocele of the Appendix: A Case Report and Review of Literature.

An appendiceal mucocele is a rare disease characterized by the dilation of the appendix lumen with mucus accumulation. Although this disease is often found incidentally during appendectomy, it is crucial to differentiate it from acute appendicitis preoperatively to select adequate surgical management. We present a case of a 31-year-old male, medically free, with right-sided abdominal pain associated with nausea and vomiting. He was diagnosed with appendiceal mucocele and underwent laparoscopic appendectomy. The absence of a distinct clinical presentation and biochemical parameters necessitates a comprehensive and collaborative diagnostic approach for mucocele of the appendix. Achieving an accurate diagnosis prior to surgery is imperative to ensure the appropriate surgical technique is chosen, thereby minimizing the risk of serious intraoperative and postoperative complications such as pseudomyxoma peritonei.

Primary borderline mucinous tumor of the testis with postoperative metastasis: A rare case report.

Primary borderline mucinous tumor of the testis is extremely rare and belongs to the ovarian epithelial type. Testicular borderline mucinous tumor is intermediate in malignancy between benign mucinous cystadenoma and mucinous carcinoma, and their clinical treatment is mainly based on radical testicular resection. The reported cases with PBMTs have a good prognosis with no cases of recurrence or metastasis after surgery reported. However, we herein report a 21-year-old male who may be the first reported case of PBMT of the testis with postoperative tumor metastasis.

Mucinous Cystadenoma Arising in a Uterine Isthmocele: A Case Report / Cistadenoma mucinoso surgindo em istmocele uterina: relato de caso

Abstract Isthmocele is a discontinuation of the myometrium at the uterine scar site in a patient with a previous cesarian section (CS). The cause of isthmocele appears to be multifactorial. Poor surgical technique, low incision location, uterine retroflection, obesity, smoking, inadequate healing of scars, and maternal age are possible related factors. Most patients with this condition are asymptomatic. However, women can present with postmenstrual bleeding, pelvic pain, subfertility, dysmenorrhea, infertility, and scar abscess. Brazil has one of the world s highest cesarean section rates. One of the consequences of the rising rate of CS is the isthmocele, an emerging female health problem. Here we report a case of mucinous cystadenoma arising in a uterine isthmocele, a complication, as far as we could investigate, not yet described in the literature.

Resumo Istmocele é a descontinuidade do miométrio no local da cicatriz uterina em paciente com cesariana anterior. A causa da istmocele parece ser multifatorial. Má técnica cirúrgica, baixa localização da incisão, retroflexão uterina, obesidade, tabagismo, cicatrização inadequada de cicatrizes e idade materna são possíveis fatores relacionados. A maioria dos pacientes com esta condição é assintomática. No entanto, as mulheres podem apresentar sangramento pós-menstrual, dor pélvica, subfertilidade, dismenorreia, infertilidade e abscesso cicatricial. O Brasil tem uma das maiores taxas de cesariana do mundo. Uma das consequências da taxa crescente de cesarianas é a istmocele, um problema emergente de saúde feminina. Aqui relatamos um caso de cistoadenoma mucinoso originado em uma istmocele uterina, uma complicação ainda não descrita, até onde pudemos investigar.

A case report of mucinous cystadenoma with contralateral serous cystadenofibroma in identical twin.

Introduction: There are limited reports on ovarian neoplasm occurring among identical twins. Most previous reports showed ovarian teratoma found in both twins. Herein, we report for the first time a case of ovarian mucinous cystadenoma with contralateral serous cystadenofibroma found in twin siblings. Case report: One patient suffered from abdominal distension and the following computed tomography found ovarian mucinous cystadenoma. During the laparoscopy, another ovarian mass was found in the contralateral ovary. The histopathology revealed ovarian mucinous cystadenoma with contralateral serous cystadenofibroma. The twin sister had no symptoms but underwent gynecological screening. She also showed a similar finding, mucinous cystadenoma with serous cystadenofibroma on the contralateral ovary. Both patients underwent laparoscopic bilateral ovarian cystectomy. Conclusion: This is the first clinical report on left ovarian mucinous cystadenoma with right serous cystadenofibroma in twin siblings. Our cases support awareness of ovarian tumors in twin sisters.

Rare Non-Neuroendocrine Pancreatic Tumours.

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.

Coexistence of a huge Brenner's tumour and mucinous cystadenoma in a 62-year-old female: a case report from Syria.

The coexistence of a benign Brenner tumour and a mucinous cystadenoma is rare, and their relationship and origin are still enigmatic and challenging. Case presentation: In this manuscript, the authors report a case of a 62-year-old nulliparous Syrian woman who presented with severe abdominal distension, which was followed by laparotomy and the excision of a 25×20 cm-cyst; its pathological examination confirmed a benign Brenner's tumour and mucinous cystadenoma. Clinical discussion: Ovarian Brenner and mucinous are usually benign and can rarely grow asymptomatically to very big sizes. Herein, the authors aim to emphasize the importance of excluding malignancy by pathology examination. Conclusion: Walthard cell nests undergo metaplasia to give rise to different kinds of Brenner and mucinous neoplasm formations according to their genetic alterations. This paper adds to the available literature, which is still poor, by providing evidence of the first case of this rare combination from Syria with a review of different theories of origin and differential diagnoses. More studies directed towards exploring this combination's genetic origin are needed to boost our understanding of ovarian tumours in general.

Rare association of epidermoid cyst of ovary with mucinous cystadenoma- A Case Report.

Epidermoid cyst is a rare benign neoplasm of the ovary, lined by stratified squamous epithelium, lacking skin, adnexal structures, and other teratomatous elements. On the other hand, mucinous cystadenoma is one of the most common benign ovarian neoplasm that microscopically shows cystic areas lined by tall columnar mucinous epithelium. Although its coexistence with other tumours, such as mature cystic teratoma, squamous cell carcinoma, clear cell adenocarcinoma, Brenner's tumour, serous cystadenoma, etc., have been reported, a combination of benign epidermoid cyst and mucinous cystadenoma has rarely been documented in literature. We report a case of coexistence of epidermoid cyst and mucinous cystadenoma in an ovarian cyst.