Diagnostic Challenges and Treatment Options for Mucocle of the Appendix: A Comprehensive Review.

Mucocles of the appendix, encompassing mucinous cystadenomas and mucinous cystadenocarcinomas, represent rare but clinically significant appendiceal lesions characterized by the accumulation of mucin within the appendix lumen. This review explores the diagnostic complexities and treatment strategies associated with mucocles, emphasizing the importance of its accurate recognition and management. Diagnostic challenges arise due to overlapping symptoms with acute appendicitis and other appendiceal pathologies, necessitating a multidimensional approach that includes imaging, histopathological analysis, and clinical correlation. Treatment options range from appendectomy for benign lesions to more extensive surgical procedures, such as right hemicolectomy for malignant forms. Prognostic factors, including histological subtype and tumor size, influence treatment decisions and long-term outcomes. By synthesizing current evidence and clinical insights, this review aims to provide a comprehensive framework for clinicians to navigate the complexities of mucocles of the appendix, offering perspectives that can guide effective management and future research endeavors.

Mucinous Cystadenoma of the Urachal Remnant Successfully Resected by Laparoscopic Surgery: A Case Report.

A urachal remnant is a disorder resulting from a disturbance in the closure process of the urachus. A 55-year-old man was referred to our hospital for treatment of gallstones. The computed tomography scan revealed a cystic mass in the lower abdomen connecting to the urinary bladder. The preoperative diagnosis was a urachal cyst. Simultaneous laparoscopic cholecystectomy, mass resection, and urachectomy were performed. The mass on the cranial side of the urinary bladder was located on the median umbilical ligament. Both were resected and removed, along with the umbilicus. The postoperative course was uneventful. The histopathological diagnosis was urachal mucinous cystadenoma. There is no sign of a recurrence. A complete resection without damage is especially important for mucinous tumors of the urachal remnant because the injury to the tumor may lead to the development of pseudomyxoma peritonei. Only seven cases of mucinous cystadenoma of the urachal remnant were reported in English literature, and only one of these was treated with laparoscopic surgery. In our case, complete resection was possible by taking advantage of the magnifying effect of laparoscopic surgery. Furthermore, we are able to provide very clear intraoperative images and specimen photographs, which we believe will be useful for readers. Laparoscopic surgery will be beneficial when treating similar cases in the future. However, it should be kept in mind that a safe resection requires careful and meticulous technique.

Intraoral Salivary Gland Cystadenomas: A Case Series Study and Literature Review.

BACKGROUND: Salivary gland cystadenoma (SGCA) is a rare benign tumor that predominantly occurs in the parotid gland. SGCAs affecting the minor salivary glands are uncommon and often resemble, clinically and histopathologically, other salivary gland lesions. METHODS: This study aimed to describe a series of four cases of SGCA affecting intraoral sites and performed a literature review of well-reported SGCA published in the English-language literature. RESULTS: SGCA cases included in this series were diagnosed in the buccal mucosa, lip, and hard palate of female patients aged between 19 and 78 years. All cases underwent excisional biopsy and were histologically characterized by a multicystic growth with variable degrees of capsule formation and were lined by several types of epithelium, including some cell types that are infrequently reported in SGCA. In some cases, a small collection of lymphocytes was observed adjacent to cystic formations. All SGCA were positive for periodic acid-Schiff, and immunohistochemical reactions were positive for CK7 and p63. The follow-up time ranged widely from 3 to 53 months, and to date, no recurrence has been observed. CONCLUSION: The literature review revealed a total of 33 published studies accounting for 55 SGCA cases.

Discriminative diagnosis of ovarian endometriosis cysts and benign mucinous cystadenomas based on the ConvNeXt algorithm.

PURPOSE: The objective of this study was to develop a deep learning model, using the ConvNeXt algorithm, that can effectively differentiate between ovarian endometriosis cysts (OEC) and benign mucinous cystadenomas (MC) by analyzing ultrasound images. The performance of the model in the diagnostic differentiation of these two conditions was also evaluated. METHODS: A retrospective analysis was conducted on OEC and MC patients who had sought medical attention at the Fourth Affiliated Hospital of Harbin Medical University between August 2018 and May 2023. The diagnosis was established based on postoperative pathology or the characteristics of aspirated fluid guided by ultrasound, serving as the gold standard. Ultrasound images were collected and subjected to screening and preprocessing procedures. The data set was randomly divided into training, validation, and testing sets in a ratio of 5:3:2. Transfer learning was utilized to determine the initial weights of the ConvNeXt deep learning algorithm, which were further adjusted by retraining the algorithm using the training and validation ultrasound images to establish a new deep learning model. The weights that yielded the highest accuracy were selected to evaluate the diagnostic performance of the model using the validation set. Receiver operating characteristic (ROC) curves were generated, and the area under the curve (AUC) was calculated. Additionally, sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratio, negative likelihood ratio, and odds ratio were calculated. Decision curve analysis (DCA) curves were plotted. RESULTS: The study included 786 ultrasound images from 184 patients diagnosed with either OEC or MC. The deep learning model achieved an AUC of 0.90 (95 % CI: 0.85-0.95) in accurately distinguishing between the two conditions, with a sensitivity of 90 % (95 % CI: 84 %-95 %), specificity of 90 % (95 % CI: 77 %-97 %), a positive predictive value of 96 % (95 % CI: 91 %-99 %), a negative predictive value of 77 % (95 % CI: 63 %-88 %), a positive likelihood ratio of 9.27 (95 % CI: 3.65-23.56), and a negative likelihood ratio of 0.11 (95 % CI: 0.06-0.19). The DCA curve demonstrated the practical clinical utility of the model. CONCLUSIONS: The deep learning model developed using the ConvNeXt algorithm exhibits high accuracy (90 %) in distinguishing between OEC and MC. This model demonstrates excellent diagnostic performance and clinical utility, providing a novel approach for the clinical differentiation of these two conditions.

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.

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.

The frequency and prognostic role of P53 and P16 immunoexpression in primary ovarian mucinous tumors.

BACKGROUND: Primary ovarian mucinous tumors are uncommon. Factors leading to invasive progression and metastatic disease have not been fully delineated yet. The aim of this study is to determine the rates of p53 and p16 immunoexpressions in primary ovarian mucinous tumors, to investigate their relationship with clinicopathologic factors and their impact on prognosis and survival. METHODS: Seventy-eight primary ovarian mucinous tumors (30 mucinous cystadenomas, 30 mucinous borderline tumors (MBOT), 18 mucinous carcinomas (MOC)) were evaluated immunohistochemically with p53 and p16 staining. The demographic, clinicopathological data, and postoperative follow-up findings of the patients were analyzed. RESULTS: Mutation-type p53 staining was present in 1/30 (3.3 %) cystadenoma, 10/30 (33.3 %) MBOT and 9/18 (50 %) MOC (p = 0.001). p16 overexpression was detected in 3/30 (10.0 %) MBOT and 5/18 (27.8 %) MOC, but not in any cystadenoma (p = 0.04). The frequency of mutation-type p53 staining in MBOTs with microinvasion was higher (71.4 %) than in those without (28.6 %, p = 0.026). The frequencies of p16 or p53 mutations were similar in MBOTs with and without intraepithelial carcinoma, or mural nodule (p > 0.05). In MOCs with ovarian surface involvement, mutation-type p53 staining was detected in 66.7 % (6/9) and p16 overexpression in 55.6 % (5/9) of the cases. A significant difference was found between MOCs with or without ovarian surface involvement regarding the frequency of p16 overexpression (p = 0.029). Any relationship was not detected between survival and p53 and p16 expression in MOCs (p > 0.05). CONCLUSION: p53 and p16 mutation rates were higher in MOCs compared to mucinous cystadenomas and MBOTs and suggest a relevant role in the development of primary ovarian mucinous carcinoma, however further studies are needed in this regard.

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.

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.

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.

Cistoadenoma mucinoso gigante de ovario / Giant ovarian mucinous cystadenoma

Aunque el cistoadenoma mucinoso de ovario tiene un crecimiento benigno, puede crecer hasta tener grandes dimensiones. Se presenta el caso de una paciente de 70 años que acudió a consulta por presentar dolor abdominal recurrente y difuso. A la exploración física se palpó una gran masa que ocupaba toda la cavidad abdominal, hasta la apófisis xifoides, redondeada, renitente, de bordes definidos, no dolorosa, no movible. Presencia de edema en miembros inferiores. Durante el acto operatorio se identificó útero miomatoso y quiste gigante de ovario izquierdo, aproximadamente de 40 x 30 cm y 13,6 kg de peso. Se realizó histerectomía total abdominal y salpingooforectomía bilateral. La biopsia confirmó el diagnóstico de cistoadenoma mucinoso benigno de ovario y leiomiomatosis uterina. El posoperatorio transcurrió favorablemente. La incidencia de cistoadenoma gigante de ovario es desconocida, debido a la falta de un concepto estandarizado y las modalidades de imagen avanzadas disponibles en la actualidad(AU)

Although ovarian mucinous cystadenoma has a benign growth, it can grow to be very large. We present the case of a 70-year-old patient who came to the clinic for recurrent and diffuse abdominal pain. On physical examination, a large mass was palpated that occupied the entire abdominal cavity, up to the xiphoid appendix, rounded, retentive, with defined edges, non- painful, non-movable. Presence of edema in lower limbs. During the surgical act, a myomatous uterus and a giant left ovarian cyst measuring approximately 40 x3 0 cm and weighing 13.6 kg were identified. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. The biopsy confirmed the diagnosis of benign ovarian mucinous cystadenoma and uterine leiomyomatosis. The postoperative period progressed favorably. The incidence of giant ovarian cystadenoma is relatively unknown due to the lack of a standardized concept and currently available advanced imaging modalities(AU)

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.

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.

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.

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.

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.

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.