Primary retroperitoneal mucinous cystadenocarcinoma in pregnancy - case report.

INTRODUCTION: Primary retroperitoneal mucinous cystadenoma (PRMC) and its malignant variant - cystadenocarcinoma are extremely rare tumors known only from case studies and reviews. PRMC is a cystic tumor of the retroperitoneum, which remains asymptomatic for a long time and can reach significant proportions. It occurs mainly in women. CASE REPORT: We present the case of a 38-year-old healthy woman with diagnosed resistance in the right mesogastrium during a c-section. The delivery was terminated without a surgical intervention. The above mentioned resistance was examined further by ultrasound, computed tomography and magnetic resonance imaging. All imaging examinations showed a solid - cystic bilocular expansion, which looked like a mesenteric cyst in the right paracolic area. The patient was asymptomatic at all times, with only a palpable resistance of the abdominal wall. In November 2020, the patient underwent an elective surgery - extirpation of the cystic tumor and prophylactic appendectomy. The operation was performed classically, via midline laparotomy without perioperative perforation of the cystic tumor and without complications in the postoperative period. The histopathological examination showed a malignant variant of PRMC. So far, the patient remains free of any problems and is followed at the department of oncology. CONCLUSION: The goal of treatment is to achieve complete surgical removal of the tumor without its perioperative perforation. Due to the rare occurrence of the disease, there are no guidelines for the diagnosis and treatment of PRMC. Precisely because PRMC is a rare tumor, it should be part of the differential diagnosis of cystic tumors of the retroperitoneum, especially when young women are concerned.

Mucinous cystadenocarcinoma in the renal pelvis: primary or secondary? Case report and literature review.

INTRODUCTION: Cystic lesions in the renal pelvis may cause a diagnostic dilemma. These lesions may be benign or (pre)malignant and primary of secondary. CASE REPORT: A 65-year-old woman presents with a complex cystic mass in the lower pole of the left kidney measuring approximately 16 cm in size and classified as Bosniak type 2F with minimally thickened septa and thick calcifications on non-contrast-enhanced computed tomography. The histopathological findings are consistent with a mucinous cystadenocarcinoma in the renal pelvis. Given the absence of a primary focus and based on clinical and radiological examination, the diagnosis of a primary renal neoplasm is preferred. DISCUSSION: A review of the literature is performed to evaluate the histological differential diagnosis and confirm the diagnosis. Epidemiology with an overview of available similar cases since 2009, as well as etiological factors, treatment and prognosis was reviewed. CONCLUSION: Primary renal mucinous cystadenocarcinoma is an exceedingly rare tumor. Histopathology still remains the gold standard for the diagnosis of this tumor. Because of the rarity, secondary involvement from more common sites, such as ovary and gastrointestinal tract, should be thoroughly excluded.

Resection of biliary mucinous cystic neoplasms of the liver: a prospective cohort series of 13 consecutive patients.

BACKGROUND: Biliary mucinous cystic neoplasms (BMCNs) are uncommon neoplastic septated intrahepatic cysts which are often incorrectly diagnosed and have the potential for malignant transformation. OBJECTIVE: To assess the outcome of surgical resection of BMCNs. METHOD: A prospective liver surgery database was used to identify patients who underwent surgery at Groote Schuur Hospital Complex for BMCN from 1999 to 2015. Demographic variables including age and gender were documented as well as detailed preoperative imaging, location and size, operative treatment, extent of resection, histology, postoperative complications and outcome. RESULTS: Thirteen female patients (median age 45 years) had surgery. Eleven were diagnosed by imaging for symptoms. Two were jaundiced. One cyst was found during an elective cholecystectomy. Five cysts were located centrally in the liver. Before referral three cysts were treated with percutaneous drainage and two were treated with operative deroofing. Six patients had anatomical liver resections and seven patients had non anatomical liver resections of which two needed ablation of residual cyst wall. One patient needed a biliary-enteric reconstruction to treat a fistula. Median operative time was 183 minutes (range: 130-375). No invasive carcinoma was found. There was no operative mortality. One surgical site infection and one intra-abdominal collection were treated. Two patients developed recurrent BMCN after 24 months. CONCLUSION: BMCNs should be considered in middle aged women who have well encapsulated multilocular liver cysts. Treatment of large central BMCNs adjacent to vascular and biliary structures may require technically complex liver resections and are best managed in a specialised hepato-pancreatico-biliary unit.

[ULTRASOUND CRITERIA OR THE OPERABILITY AND EFFICACY OF THE TREATMENT OF THE MALIGNANT EPITHELIAL OVARIAN TUMOURS].

In order to isolate the main sonographic criteria of ovarian cancer operability the dynamical U.S. examination was performed on 65 women with epithelial tumors of II-III stages before and during 5 years after treatment beginning, which included (in different combinations) cytoreductive surgery and neoadjuvant chemotherapy. Only total 14 (21.5%) relapses were revealed. The U.S. prognostic criteria of the ovarian cancer treatment efficacy with and without neoadjuvant chemotherapy were defined.

Differential diagnosis of pancreatic serous oligocystic adenoma and mucinous cystic neoplasm with spectral CT imaging: initial results.

AIM: To investigate the imaging characteristics of pancreatic serous oligocystic adenoma (SOA) and mucinous cystic neoplasms (MCNs) using spectral computed tomography (CT) and to evaluate whether quantitative information derived from spectral imaging can improve the differential diagnosis of these diseases. MATERIALS AND METHODS: From February 2010 to June 2013, 44 patients (24 SOAs and 20 MCNs) who underwent spectral CT imaging were included in the study. Conventional characteristics and quantitative parameters were compared between the two disease groups. Logistic regression was used for multiparametric analysis. The receiver-operating characteristic curve was used to evaluate the diagnostic performance of single parameter and multiparametric analysis. Two radiologists diagnosed the diseases blinded and independently, without and with the information of the statistical analysis. RESULTS: Tumour location, contour, size, and monochromatic CT values at 40 keV to 70 keV, iodine concentration, and effective atomic number (effective-Z) in the late arterial phase were the independent factors correlated with category. Multiparametric analysis with logistic regression showed that tumour size, location, and contour were the most effective variations, and obtained an area under the ROC curve (AUC) of 0.934. With the knowledge of statistical analysis, the accuracy of the first reader increased from 70.5% to 86.4%, and the accuracy of the second reader increased from 81.8% to 90.9%. CONCLUSIONS: Although CT spectral imaging provided additional information and multiparametric analysis obtained better performance than single-parameter analysis in differentiating MCNs from SOAs, multiparametric analysis with the combination of quantitative parameters derived from CT spectral imaging did not improve the diagnostic performance. Tumour size, location, and contour played an important role in differentiating MCNs from SOAs.

Pancreatic mucinous cystic neoplasm size using CT volumetry, spherical and ellipsoid formulas: validation study.

CONTEXT: The accuracy for determining pancreatic cyst volume with commonly used spherical and ellipsoid methods is unknown. The role of CT volumetry in volumetric assessment of pancreatic cysts needs to be explored. OBJECTIVES: To compare volumes of the pancreatic cysts by CT volumetry, spherical and ellipsoid methods and determine their accuracy by correlating with actual volume as determined by EUS-guided aspiration. Setting This is a retrospective analysis performed at a tertiary care center. Patients Seventy-eight pathologically proven pancreatic cysts evaluated with CT and endoscopic ultrasound (EUS) were included. Design The volume of fourteen cysts that had been fully aspirated by EUS was compared to CT volumetry and the routinely used methods (ellipsoid and spherical volume). Two independent observers measured all cysts using commercially available software to evaluate inter-observer reproducibility for CT volumetry. MAIN OUTCOME MEASURES: The volume of pancreatic cysts as determined by various methods was compared using repeated measures analysis of variance. Bland-Altman plot and intraclass correlation coefficient were used to determine mean difference and correlation between observers and methods. The error was calculated as the percentage of the difference between the CT estimated volumes and the aspirated volume divided by the aspirated one. RESULTS: CT volumetry was comparable to aspirated volume (P=0.396) with very high intraclass correlation (r=0.891, P<0.001) and small mean difference (0.22 mL) and error (8.1%). Mean difference with aspirated volume and error were larger for ellipsoid (0.89 mL, 30.4%; P=0.024) and spherical (1.73 mL, 55.5%; P=0.004) volumes than CT volumetry. There was excellent inter-observer correlation in volumetry of the entire cohort (r=0.997, P<0.001). CONCLUSIONS: CT volumetry is accurate and reproducible. Ellipsoid and spherical volume overestimate the true volume of pancreatic cysts.

Pancreatic pseudocyst or a cystic tumor of the pancreas?

Pancreatic pseudocysts are the most common cystic lesions of the pancreas and may complicate acute pancreatitis, chronic pancreatitis, or pancreatic trauma. While the majority of acute pseudocysts resolve spontaneously, few may require drainage. On the other hand, pancreatic cystic tumors, which usually require extirpation, may disguise as pseudocysts. Hence, the distinction between the two entities is crucial for a successful outcome. We conducted this study to highlight the fundamental differences between pancreatic pseudocysts and cystic tumors so that relevant management plans can be devised. We reviewed the data of patients with pancreatic cystic lesions that underwent intervention between June 2007 and December 2010 in our hospital. We identified 9 patients (5 males and 4 females) with a median age of 40 years (range, 30-70 years). Five patients had pseudocysts, 2 had cystic tumors, and 2 had diseases of undetermined pathology. Pancreatic pseudocysts were treated by pseudocystogastrostomy in 2 cases and percutaneous drainage in 3 cases. One case recurred after percutaneous drainage and required pseudocystogastrostomy. The true pancreatic cysts were serous cystadenoma, which was treated by distal pancreatectomy, and mucinous cystadenocarcinoma, which was initially treated by drainage, like a pseudocyst, and then by distal pancreatectomy when its true nature was revealed. We conclude that every effort should be exerted to distinguish between pancreatic pseudocysts and cystic tumors of the pancreas to avoid the serious misjudgement of draining rather than extirpating a pancreatic cystic tumor. Additionally, percutaneous drainage of a pancreatic pseudocyst is a useful adjunct that may substitute for surgical drainage.

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.

Adult granulosa cell tumour camouflaged as mucinous neoplasm.

This case report delves into the diagnostic intricacies and clinical management of adult granulosa cell tumour (AGCT) in a woman in her 50s, presenting with pain abdomen. Initial imaging investigations like ultrasound suggested diagnosis of benign cystadenoma. Further MRI revealed a large well-defined multiloculated lesion so a diagnosis of neoplastic aetiology/likely mucinous cystadenocarcinoma was offered. However, the definitive diagnosis was established through meticulous histopathological examination, revealing characteristic features of AGCT, a rare ovarian neoplasm. The case underscores the diagnostic challenges posed by AGCT, the importance of integrating clinical, radiological and histopathological data, and the necessity for a multidisciplinary approach for accurate diagnosis and optimal patient management.

Mature cystic teratoma with co-existent mucinous cystadenocarcinoma: describing a diagnostic challenge-a case report.

BACKGROUND: Mature cystic teratoma co-existing with a mucinous cystadenocarcinoma is a rare tumor that few cases have been reported until now. In these cases, either a benign teratoma is malignantly transformed into adenocarcinoma or a collision tumor is formed between a mature cystic teratoma and a mucinous tumor, which is either primarily originated from epithelial-stromal surface of the ovary, or secondary to a primary gastrointestinal tract tumor. The significance of individualizing the two tumors has a remarkable effect on further therapeutic management. CASE PRESENTATION: In this case, a mature cystic teratoma is co-existed with a mucinous cystadenocarcinoma in the same ovary in a 33-year-old Iranian female. Computed Tomography (CT) Scan with additional contrast of the left ovarian mass suggested a teratoma, whereas examination of resected ovarian mass reported an adenocarcinoma with a cystic teratoma. A dermoid cyst with another multi-septate cystic lesion including mucoid material was revealed in the gross examination of the surgical specimen. Histopathological examination revealed a mature cystic teratoma in association with a well-differentiated mucinous cystadenocarcinoma. The latter showed a CK7-/CK20 + immune profile. Due to the lack of clinical, radiological, and biochemical discoveries attributed to a primary lower gastrointestinal tract tumor, the immune profile proposed the chance of adenocarcinomatous transformation of a benign teratoma. CONCLUSIONS: This case shows the significance of large sampling, precise recording of the gross aspects, histopathological examination, immunohistochemical analysis, and the help of radiological and clinical results to correctly diagnose uncommon tumors.

Primary Retroperitoneal Mucinous Cystadenocarcinoma during Long-term Administration of Infliximab for the Treatment of Crohn's Disease.

We herein report a rare case of primary retroperitoneal mucinous cystadenocarcinoma (PRMC) in a 60-year-old man. The patient, who had been treated with infliximab for Crohn's disease of the colon for 13 years, was referred to our hospital for lower back pain. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging revealed multiple cystic lesions in the right retroperitoneum, the calcification of the cyst, and bone lesions. Bone and CT-assisted biopsies of the retroperitoneal lesions revealed poorly differentiated adenocarcinoma. The patient was diagnosed with PRMC with bone metastases using immunohistochemical staining and positron emission tomography/CT.

Cyst growth rate predicts malignancy in patients with branch duct intraductal papillary mucinous neoplasms.

BACKGROUND & AIMS: Little information is available about the clinico-pathologic characteristics of pancreatic branch duct intraductal papillary mucinous neoplasm (Br-intraductal papillary mucinous neoplasm [IPMN]) because of difficulties in diagnosis based on radiologic and tissue information. We investigated the natural history of Br-IPMN using imaging and surgical pathology data from patients. METHODS: Data were collected from patients admitted to a single tertiary referral institution from January 2000 to March 2009 (median follow up of 27.9 months); 201 patients were diagnosed with Br-IPMN with an initial cyst less than 30 mm without main pancreatic duct dilatation or mural nodules. The patients were followed for more than 3 months and examined by computed tomography (CT) at least twice. RESULTS: The mean size of the patients' initial cysts was 14.7 mm; the mean cyst growth rate was 1.1 mm/year. Thirty-five patients received surgery during follow up and 8 were confirmed to have malignant cysts. The malignant cysts were greater in final size than nonmalignant cysts (24.3 mm vs 16.9 mm; P = .003); they also grew by a greater percentage (69.8% vs 19.4%; P = .046) and at a greater rate (4.1 mm vs 1.0 mm/year; P = .001). The actuarial 5-year risk of malignancy was 41.6% in the group that received surgery and 10.9% for all patients. Cysts that grew more than 2 mm/year had a higher risk of malignancy (5-year risk = 45.5% vs 1.8%; P < .001). CONCLUSIONS: In combination with cyst size and the presence of mural nodules, cyst growth rate could be used to predict malignancy in patients with Br-IPMN.

A 64-year-old woman with a left upper-lung field lobulated mass.

Pulmonary mucinous cystic neoplasia (PMCN) is an uncommon primary lung malignancy. Approximately 80 cases have been reported in the literature. We describe a case and review the literature on this malignancy. Our patient was asymptomatic and presented with a left upper-lung field lobulated mass. Primary PMCN is a spectrum of mucinous cystic lesions that should be considered as a separate lung tumor entity due to its unique morphologic features and its distinct clinical behavior. Prognosis is generally better than other more common lung malignancies.

Total pancreaticoduodenectomy and segmental resection of superior mesenteric vein-portal vein confluence with autologous splenic vein graft in mucinous cystadenocarcinoma of the pancreas.

CONTEXT: Mucinous cystic tumors occur almost exclusively in middle-aged women and in the body or tail of the pancreas. Mucinous cystadenocarcinoma, a malignant sub-type of mucinous cystic tumors, in the head of the pancreas and in a middle-aged man is extraordinary, and the prognosis and proper management of mucinous cystadenocarcinoma has not been well documented. CASE REPORT: A 52-year-old male patient with a mucinous cystadenocarcinoma approximately 5.5 cm in size in the head of the pancreas underwent a total pancreaticoduodenectomy and segmental resection of the superior mesenteric vein-portal vein confluence with an autologous splenic vein graft due to tumor invasion. His postoperative course was uneventful and he received adjuvant chemotherapy. He has been followed-up periodically for more than 30 months after surgery without evidence of recurrence. CONCLUSION: Mucinous cystadenocarcinoma in the head of the pancreas in a middle-aged man is an extremely rare case. Because mucinous cystadenocarcinomas have a relatively good prognosis, an aggressive approach with major vascular resection is warranted in well-selected patients. Splenic vein interposition grafting could be used in cases which undergo total pancreaticoduodenectomy with segmental resection of the superior mesenteric vein-portal vein confluence if the splenic vein is healthy.

[Mucocele of the appendix - a heterogenous surgical pathology]. / Die Mukozele der Appendix - eine inhomogene chirurgische Krankheitsentität.

BACKGROUND: Mucoceles of the appendix are rare. After appendectomy, mucoceles are detected with a frequency of 0.2 to 0.3 %. Both stenosing / obliterating processes and alterations of the epithelium (hyperplasia, mucinous cystadenoma, cystadenoma with uncertain malignant potential (UMP), mucinous cystadenocarcinoma lead to the occurrence of mucoceles. The perforation of a mucocele with possible spread of mucus and cells into the abdominal cavity constitutes a severe complication (pseudomyxoma peritonei). Surgical resection is the curative approach for mucoceles of the appendix. MATERIALS AND METHODS: Data of patients who were treated for an appendiceal mucocele between 1995 and 2009 were analysed retrospectively with regard to clinical presentation, diagnostic measures, surgical procedure and histopathological result. Follow-up was evaluated in telephone interviews. RESULTS: We extracted 5 cases from our database. Clinical symptoms varied greatly among the individual patients, ranging from peracute abdominal pain in the right lower quadrant to chronic obstipation. Results from abdominal ultrasound and / or abdominal CT scans contributed to the indication for surgical intervention in all cases. In 2 patients surgery was stated as urgent whereas in 3 the operation was scheduled electively. In one patient the diagnosis of an appendiceal mucocele was stated preoperatively and in another intraoperatively. In 3 patients only the histopathological result revealed the underlying mucocele. We performed 1 open and 1 laparoscopic appendectomy, 1 open appendectomy with a partial resection of the coecum and 2 laparoscopic ileocoecal resections. One of the patients had a pseudomyxoma peritonei. The histopathological diagnoses ranged from mere epithelial hyperplasia to an adenoma with uncertain malignant potential and a mucinous cystadenocarcinoma. One patient's long-term follow-up could not be evaluated. All other patients had neither recurrence nor any complications after discharge. CONCLUSIONS: Mucoceles of the appendix present with a wide spectrum of clinical symptoms and histopathological alterations. Only an accurate histological analysis reveals the underlying pathological lesion correctly. This study emphasises that a mucocele of the appendix constitutes an important differential diagnosis in patients presenting with pathologies in their right lower abdominal quadrant.

18F-FDG PET/CT Findings in a Patient With Primary Mucinous Cystadenocarcinoma of the Breast.

A 50-year-old woman presented with a mass in the upper outer quadrant of her left breast, which proved to be a mucinous cystadenocarcinoma by biopsy. An F-FDG PET/CT was performed for staging, which showed that the breast tumor was the only lesion with abnormal F-FDG avidity. Breast-conserving surgery was performed following a negative sentinel lymph node biopsy of the left axilla, and primary mucinous cystadenocarcinoma of the breast was finally diagnosed.

A case of advanced mucinous cystadenocarcinoma of the pancreas with peritoneal dissemination responding to gemcitabine.

Mucinous cystic neoplasm(MCN)of the pancreas is a rare disease. A 34-year-old female was referred to our hospital for a giant cystic tumor in the left epigastrium, suspected of being a pancreatic MCN. The surgical findings revealed that the tumor originated in the pancreatic tail with the presence of peritoneal dissemination. A distal pancreatectomy and a splenectomy were performed, and the resected specimen histologically revealed an invasive mucinous cystadenocarcinoma of the pancreas. The postoperative computed tomography(CT)scan showed metastatic tumors of the Douglas pouch and the left ovary. Gemcitabine(GEM)was thereafter systemically administered for palliative chemotherapy with a regimen of 1,000 mg/m / 2week for 3 weeks, followed by a week of rest. When assessed by a CT scan after 4 courses of chemotherapy, marked shrinkage of the tumors was identified, and we could not detect the tumors clearly. Moreover, the serum CA19-9 level fell from 341 U/mL to almost normal and there were no severe adverse events. Therefore, systemic chemotherapy with GEM is considered to possibly be an effective treatment against MCN. We describe herein the first case of advanced mucinous cystadenocarcinoma of the pancreas with peritoneal dissemination responding to GEM and a brief review of the literature.

Urachal Mucinous Cystadenocarcinoma.

A 57-year-old man presented with a 6-month history of pelvic fullness. He had no lower urinary tract symptoms or altered bowel habits. On examination, there was a non-tender pelvic mass which extended from the pubic symphysis to the level of the umbilicus. CT scan of the abdomen demonstrated a 22×11×11 cm cystic mass arising from the pelvis extending into the midline and superiorly to the umbilicus. Other than raised carcinoembryonic antigen of 7.6 ng/mL (<5.0), the remainder of his blood test were unremarkable. Flexible cystoscopy demonstrated a convex deformity of the bladder wall in keeping with the compression and displacement as seen on the CT. The patient underwent an open excision of the cystic structure (urachal remnant), partial cystectomy, partial excision of anterior abdominal wall and pelvic lymphadenectomy. A check cystogram performed 12 days following the initial operation was unremarkable.