Primary retroperitoneal tumor: mucinous cystoadenocarcinoma.

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological behavior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence. During routine abdominal ultrasound a new tumor was found. An abdomen magnetic resonance imaging was done and confirmed the presence of a cystic lesion in the right iliac fossa. After discussion in multidisciplinary committee, surgical complete resection of the tumor, along with bilateral adnexectomy, was performed successfully. Histopathological examination revealed a mucinous adenocarcinoma adjacent to a mucinous cystadenoma. Immunohistochemical analysis supported the diagnosis of a primary retroperitoneal lesion. The patient had an uneventful recovery and has remained disease-free during the two-year postoperative follow-up. PRMCs are challenging to diagnose preoperatively due to nonspecific symptoms. Surgical excision is the mainstay of treatment. The long-term prognosis and optimal therapeutic strategies require further investigation.

Los cistoadenocarcinomas mucinosos primarios retroperitoneales (CMPR) son tumores extremadamente raros con una comprensión limitada de su patogénesis y comportamiento biológico. Describimos el caso de una mujer de 50 años sometida a tratamiento quirúrgico. La paciente tenía antecedentes de cirugías previas por cistodenoma mucinoso mesentérico, sin evidencia de recurrencia. Durante una ecografía abdominal de rutina se encontró un nuevo tumor. Se realizó una resonancia magnética abdomen que confirmó la presencia de una lesión quística en la fosa ilíaca derecha. Luego de discutir el caso en el comité multidisciplinario, se realizó con éxito la resección quirúrgica completa del tumor, junto con la anexectomía bilateral. El examen histopatológico reveló un adenocarcinoma mucinoso adyacente a un cistodenoma mucinoso. El análisis inmunohistoquímico apoyó el diagnóstico de lesión primaria retroperitoneal. La paciente tuvo una buena recuperación y permaneció libre de enfermedad durante dos años de seguimiento postoperatorio. Los CMPR son difíciles de diagnosticar debido a que presentan síntomas inespecíficos. La escisión quirúrgica es la base del tratamiento. El pronóstico a largo plazo y las estrategias terapéuticas óptimas requieren más investigación.

Mucinous cystadenoma and carcinoid tumor arising from an ovarian mature cystic teratoma in a 60 year-old patient: a case report.

BACKGROUND: Mature cystic teratomas (MCT) of the ovary are benign ovarian germ cell neoplasms. Malignant transformation is possible but rare and ovarian carcinoid tumors in MCT are among the most extremely rare subtypes. CASE PRESENTATION: We report a case of a 60-year-old Iranian woman suffering from postmenopausal bleeding and hypogastric pain for the last 40 days. An adnexal mass was detected during the physical examination. Ultrasound imaging showed a (55 × 58) mm mass in the left ovary. Total abdominal hysterectomy, bilateral salpingooophorectomy and comprehensive staging surgery were performed for the patient. Intraoperative frozen section of the left ovarian mass was indicative of a malignant tumor. She was diagnosed with a carcinoid tumor with benign mucinous cystadenoma arising on MCT of the ovary, confirmed in the histopathology and immunohistochemistry examination. The tumor was classified as low grade and no chemotherapy cycles were considered. The patient was followed up long-term and no recurrence was observed during 14 months of examinations. CONCLUSION: Ovarian carcinoids arising from MCT are rare neuroendocrine neoplasms, and proper diagnosis of these tumors requires careful histopathology evaluation and appropriate examination. Therefore, it is necessary to consider these tumors as a possible differential diagnosis and evaluate them in individuals (especially postmenopausal women) who have abdominal pain or abnormal bleeding and a palpable mass.

Ovarian tumour-cutaneous fistula as a primary presentation of pelvic malakoplakia coexisting with a benign ovarian tumour mimicking advanced ovarian cancer.

Malakoplakia is a rare granulomatous, chronic inflammatory disease generally affecting the urogenital organs, though it can arise in other organs. The clinical manifestations of malakoplakia vary depending on the affected organ. The final diagnosis is confirmed by the presence of Michaelis-Gutmann bodies on pathology. This report describes a case of pelvic malakoplakia accompanied by an ovarian tumour-cutaneous fistula, initially misdiagnosed as advanced ovarian cancer invading the anterior abdominal wall with left pleural effusion based on imaging studies and increased serum carbohydrate antigen 19-9. The patient underwent left thoracentesis and fluid collection from the fistula tract for cytology, which showed no malignancy. She underwent primary debulking surgery, including removal of the fistula tract from anterior abdominal wall. Histopathological examination revealed malakoplakia coexisting with mucinous cystadenoma of the left ovary. For postoperative management, she received prolonged oral antibiotics for 6 months. There was no evidence of disease recurrence at the 24-month follow-up.

[Pancreatic mucinous cystadenoma with atypical clinical presentation]. / Mutsinoznaya tsistadenoma podzheludochnoi zhelezy ­ netipichnyi variant techeniya zabolevaniya.

We present a 33-year-old patient with atypical clinical course of pancreatic mucinous cystadenoma. The tumor had connection with pancreatic ductal system and led to bleeding into cystic cavity. This contributed to incorrect preoperative diagnosis of post-necrotic cyst. The final diagnosis of mucinous cystadenoma was established after histological examination. Distal pancreatectomy excluded incorrect treatment.

A rare case of infection and spontaneous rupture in ovarian mucinous cystadenoma.

Ovarian cysts exhibit variable clinical presentations depending on their size, type and resulting sequelae. Rupture of ovarian cysts is infrequent, and cyst infections are even rarer. Here, we report an unusual case involving a young, non-pregnant woman who presented acutely with features of peritonitis and sepsis and was found to have a complex adnexal mass. Following a rigorous diagnostic evaluation, which included an urgent exploratory laparotomy and salpingo-oophorectomy, common diagnoses including tubo-ovarian abscess, endometriotic cyst and pelvic tuberculosis were ruled out. Instead, she was diagnosed with an ovarian mucinous cystadenoma that had become infected, possibly due to pelvic inflammatory disease, leading to spontaneous rupture. Such a presentation has rarely been reported, especially in a non-pregnant setting. Therefore, we emphasise the importance of considering this rare complication as a potential differential diagnosis in similar clinical presentations and discuss the management implications, including the importance of adequately treating pelvic inflammatory disease.

A case report of huge pancreas mucinous cystic neoplasm during pregnancy: How doctors think.

RATIONALE: Pancreas mucinous cystic neoplasm (PMCN) is uncommon, and its occurrence during pregnancy is rare. The management of PMCN during pregnancy, including diagnosis and surgical timing, is a great challenge. PATIENT CONCERNS: A nontender epigastric mass of the upper abdomen was detected by palpation in a 35-year-old woman, gravida 2, para 1, during the 36th week of gestation. She was referred to our institution for further evaluation. DIAGNOSES: Magnetic resonance imaging (MRI) showed a multilocular cystic mass in the body and tail of the pancreas (16.7/12.1/17.6 cm), well-circumscribed with a hyper signal on T2-weighted MRI images. The diagnosis of a pancreatic cyst, probable mucinous, was established. INTERVENTIONS: The patient was informed of the possibilities of malignancy, rapid growth, and rupture of the tumor. After a laparotomy and cesarean section, a large cystic tumor was discovered adherent to the pancreas, spleen, mesocolon, and retroperitoneum. The spleen was preserved since there was no evidence of invasion. According to macroscopic examinations, the tumor measured 18 cm was filled with a dark yellow-brownish mucinous fluid and did not appear to communicate with the pancreatic ducts. OUTCOMES: After six months of follow-up, there were no signs of recurrence in the patient. LESSONS: PMCN may need to be surgically resected in cases characterized by malignancy risk during pregnancy. As female sex hormones may influence the behavior of PMCN during pregnancy, surgical timing should be determined based on the stage of pregnancy, malignancy status, and condition of the mother and fetus.

Mucinous Cystadenoma Arising in a Uterine Isthmocele: A Case Report.

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.

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.

Primary retroperitoneal mucinous cystic neoplasm of borderline malignancy with KRAS and GNAS co-mutation: a case report.

Primary retroperitoneal mucinous cystic neoplasms are rare retroperitoneal tumors, which are histologically similar to mucinous cystic neoplasms of the ovaries. Only 31 cases of primary retroperitoneal mucinous cystic neoplasm with borderline malignancy (PRMCN-BM) have been reported (26 in women and five in men). We describe an additional male patient with PRMCN-BM. A 39-year-old man presented to our hospital with back pain. Twelve years earlier, he had undergone an orchiectomy for a germ cell tumor. Computed tomography showed a 6.9- × 4.4-cm cystic mass in the left pararenal space. Laparoscopic mass excision was performed, and a unilocular cystic mass was found in the pararenal space near the lower pole of the left kidney. A histopathological examination showed a cyst lined by atypical mucinous intestinal epithelium without stromal invasion. Targeted next-generation sequencing identified two hotspot mutations, with one each in the KRAS and GNAS genes. Outpatient follow-up 10 months after surgery showed no evidence of tumor recurrence. PRMCNs are extremely rare retroperitoneal neoplasms, especially in men. These neoplasms are rarely considered in the differential diagnosis of retroperitoneal masses, and their preoperative diagnosis is difficult. Evaluation of additional patients is required to better determine the prognosis of PRMCNs and the optimal postoperative follow-up.

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.

Postmenopausal woman with 24 kilograms ovarian mucinous cystadenoma: a rare case report.

A case of a 53-year-old postmenopausal woman presenting a giant ovarian cystic mucinous tumor weighing 24 kg is reported here. When she was seen first at our outpatient clinic, she had gross abdominal distension since 2 years, and she complained of unbearable aggressive pain. Her computed tomography (CT) scan was done which came suggestive of ovarian serous cystadenoma of large massive size 35 x 40 x 32 cm with moderate ascites. On exploratory laparotomy, a giant, totally cystic, vascularized and smooth mass attached to the right ovary was encountered. On the postoperative tenth day, she was discharged without any problem. Histopathology report of the right ovarian cystic mass came suggestive of multiloculated cyst with capsule intact with Borderline Mucinous tumor of right ovary weighing 24 kg. This is both one of the largest known examples in the literature and the largest ovarian cyst ever seen at our institution.

Brenner Tumor of the Ovary: A 10-Year Single Institution Experience and Comprehensive Review of the Literature.

Brenner tumors (BTs) are surface-epithelial stromal cell tumors that are categorized by the World Health Organization as benign, borderline, and malignant. Due to the rarity of BTs, the published literature on these tumors is comprised primarily of case reports and small retrospective studies. We performed a pathology database review spanning the last ten years at our institution revealing nine reported benign BTs. We collected the clinical and pathological data of patients associated with those BTs, describing the clinical presentation and imaging results, and assessing the possible risk factors associated with them. The average age at diagnosis was 58 years. BTs were discovered incidentally in 7/9 cases. The tumor was multifocal and bilateral in 1/9 cases and ranged in size from 0.2 cm to 7.5 cm. Associated Walthard rests were found in 6/9 cases and transitional metaplasia of surface ovarian and/or tubal epithelium was found in 4/9 cases. One patient had an associated mucinous cystadenoma in the ipsilateral ovary. Another patient had an associated mucinous cystadenoma in the contralateral ovary. In conclusion, we found that Walthard rests and transitional metaplasia are common findings in association with BTs. Additionally, pathologists and surgeons need to be aware of the association between mucinous cystadenomas and BTs.

Recurrence Rates for Pediatric Benign Ovarian Neoplasms.

STUDY OBJECTIVE: To examine the recurrence rates of pediatric benign ovarian neoplasms METHODS: A retrospective review of females up to 21 years of age who underwent surgery for a benign ovarian neoplasm at 8 pediatric hospitals from January 2010 through December 2016 was conducted. Data include primary operation details, follow-up imaging, and reoperation details. RESULTS: Four hundred and twenty-six females were included in our cohort, with a median age of 15 years at the time of the primary operation. Of the patients, 69% had a mature teratoma, 18% had a serous cystadenoma, and 8% had a mucinous cystadenoma. Two-thirds of patients underwent ovarian-sparing surgery. There were 11 pathologically confirmed recurrences (2.6%) at a median follow-up of 12.8 months. The pathologically confirmed recurrence was 10.5 per 100 person-months at 12 months (SE = 5.7) for mucinous cystadenomas and 0.4 months (SE = 0.4) for mature teratomas (P = .001). For half of the patients, the pathologically confirmed recurrences occurred by 12.8 months, and for 75%, they occurred by 23.3 months. There were no differences in reoperation or recurrence on the basis of initial procedure (ovary-sparing surgery vs oophorectomy). CONCLUSION: We measured the pathologically confirmed recurrence rate for pediatric benign ovarian neoplasms in a large cohort. Oophorectomy was not protective against recurrence. Mucinous cystadenomas were at a greater risk of pathologically confirmed recurrence.

Laparoscopic pancreatic enucleation: cystic lesions and proximity to the Wirsung duct increase postoperative pancreatic fistula.

BACKGROUND: Risk factors for postoperative pancreatic fistula (POPF) following pancreatic enucleation by the open approach (OpenEN) are well known. However, ENs are more frequently performed laparoscopically (LapEN). The aim of this study was to analyze the risk factors of POPF following LapEN. METHODS AND PATIENTS: All patients in our prospective database who underwent LapEN were evaluated. We report the demographics, surgical, early and long-term outcomes. Numerous variables were analyzed to identify the risk factors of POPF. RESULTS: From 2008 to 2020, 650 laparoscopic pancreatic resections were performed including 64 EN (10%). The median age was 51 years old (17-79), median BMI was 24 (19-48), and 44 patients were women (69%). The main presentation was an incidental diagnosis (n = 40; 62%), pain (n= 10;16%), and hypoglycemia (n = 8;12%). The main indications were neuroendocrine tumors (40; 63%), mucinous cystadenomas (15; 23%), intraductal papillary mucinous neoplasie (3; 5%), and other benign cysts (6; 9%). Lesions were located on the distal pancreas (43; 67%), head (n = 17; 27%), and neck (4; 6%). The median size was 20 mm (9-110); 30 mm (20-110) for mucinous cystadenoma and 18 mm (8-33) for NET. The median operative time was 90 mn (30-330), median blood loss was 20 ml (0-800) ml, and there were no transfusions and one conversion. There were no mortalities and overall morbidity (n = 22; 34%) included grades B and C POPF (10;16%) and post-pancreatectomy hemorrhage (4; 6%). The median hospital stay was 7 days (3-42). There were no invaded lymph nodes and all cystic lesions were nonmalignant. After a mean follow-up of 24 months, there was no recurrence. The risk factors for grades B/C POPF were mucinous cystadenoma and proximity to the Wirsung duct < 3 mm. CONCLUSION: In this series, the outcome of LapEN was excellent with no mortality and a low rate of morbidity. However, the risk of POPF is increased with cystic lesions and those close to the Wirsung duct.

Total Laparoscopic Resection of an Extrahepatic Mucinous Biliary Cystadenoma with Liver Involvement (with Video).

BACKGROUND: Intrahepatic mucinous biliary cystadenoma is rare, and extrahepatic MBC is even rarer. To our knowledge, total laparoscopic resection of an extrahepatic MBC that had extended intrahepatically has never been reported. PATIENTS AND METHODS: A 28-year-old female presented to our hospital with upper abdomen pain. Radiological investigations demonstrated a 7-cm multiloculated cystic lesion arising from the left hepatic bile duct extending to involve the extrahepatic biliary system down to and posterior to the back of the head of pancreas. The entire extrahepatic bile duct was involved, except for the gallbladder. Laparoscopic surgery was carried out using a five-port approach. A gourd-shaped well-defined multiloculated cyst was found extending from the extrahepatic biliary system proximally to involve the left hepatic duct intrahepatically. After cholecystectomy, the gourd-shaped cyst was opened at its narrowest part at the hepatic hilus to facilitate subsequent resectional surgery. The distal sac was dissected to the distal bile duct end at the duodenal wall and transected. The proximal sac was dissected and resected en bloc with the bifurcation of the right/left hepatic ducts, combined with left hepatectomy plus caudate lobectomy. The reconstruction was done by anastomosing the right anterior and posterior sectional bile ducts to a Roux-en-Y jejunal loop. Multiple intraoperative frozen sections demonstrated the lesion to be a benign MBC. RESULTS: The patient was discharged home 12 days after surgery. She was well on follow-up 24 months after surgery. CONCLUSION: Total laparoscopic resection is technically feasible to treat an extrahepatic MBC with intrahepatic extension.

Appendiceal tumors in patients undergoing primary surgery for mucinous ovarian tumors in a tertiary hospital, in Southern Thailand.

OBJECTIVE: To evaluate the prevalence of appendiceal tumors in patients diagnosed with mucinous ovarian tumors and to determine factors associated with coexisting appendiceal tumors. MATERIALS AND METHODS: Retrospective review of all patients who were diagnosed with mucinous ovarian tumors and underwent an appendectomy during surgery between January 2002 and June 2017 was performed. Univariate and multivariate logistic regression analyses were used to identify risk factors for coexisting appendiceal tumors. RESULTS: A total of 303 patients with mucinous ovarian tumors who underwent appendectomy were identified, including 77 (25.4%) mucinous cystadenoma and 226 (74.6%) mucinous borderline tumor or carcinoma. Twenty-one (6.9%) had coexisting appendiceal tumors including 8 that were primary appendiceal mucinous adenocarcinomas, 6 low-grade appendiceal mucinous neoplasms, 6 secondary appendiceal metastasis from the ovary, and one hyperplastic polyp. None of mucinous cystadenoma had coexisting appendiceal tumors. Multivariate analysis revealed advanced age ≥50 years, previous rupture of ovarian tumors, abdominal extension of tumors, and grossly abnormal appendix were independent factors for coexisting appendiceal tumors. CONCLUSION: Prevalence of coexisting appendiceal tumors in mucinous ovarian tumors was not uncommon. The risk factors were grossly abnormal appendix, abdominal extension of tumor, previous rupture of ovarian tumors, and advanced age.

Case 300: Ruptured Mucinous Cystadenoma of the Pancreas.

History Part one of this case appeared 4 months previously and may contain larger images. A 40-year-old woman presented to the outpatient department of our hospital with a 2-year history of abdominal pain, which had worsened in the past few days. There were no other constitutional symptoms; in particular, there was no weight loss or loss of appetite. The serum amylase and serum lipase levels were always within normal limits, even during the current episode. There was no history of pancreatitis, alcohol abuse, drug use, or trauma. The patient was evaluated at an outside clinic 2 years ago and underwent imaging, including US and dedicated pancreatic CT, which revealed a complex multiseptated noncalcified pancreatic cystic lesion. After CT, the patient underwent endoscopic US, which showed the lesion was suspected to be an infected parasitic cyst; fine-needle aspiration and cytology were not attempted due to the risk of iatrogenic rupture. The patient was advised to undergo surgical resection but declined the procedure and was lost to follow-up for 2 years before her current visit to our facility. On clinical examination, there were no clinically important findings, aside from mild to moderate pain on deep palpation and some shifting dullness that was suggestive of ascites. The patient always remained hemodynamically stable. US-guided ascitic fluid analysis was performed, which revealed amylase levels of 869 IU/L (normal range, 4-234 IU/L). Considering prior CT and endoscopic US reports, the patient was further evaluated with a pancreatic MRI protocol for lesion characterization and to explain the new-onset abdominal pain.