Cistoadenocarcinoma biliar / Biliary Cystadenocarcinoma

Introducción: El cistoadenocarcinoma biliar es un tumor quístico poco frecuente, que se origina a partir del epitelio hepatobiliar, cuyo crecimiento es lento y sus síntomas son variables e inespecíficos. Objetivo: Presentar el caso de un paciente portador de un cistoadenocarcinoma biliar. Presentación del caso: Se presenta el caso de una paciente femenina de 56 años de edad que acude a la institución por presentar dolor en el hipocondrio derecho, fiebre y pérdida de peso. Al examen físico se constata una masa palpable en dicha zona abdominal, de bordes lisos, no dolorosa, dura, de escasa movilidad y relacionada con el hígado. Conclusiones: El cistoadenocarcinoma biliar es una neoplasia quística maligna poco frecuente del hígado. La mayoría de los pacientes presentan dolor abdominal, masa palpable y pérdida de peso. El tratamiento quirúrgico es la norma y la resección con márgenes libres se considera la variante de tratamiento más empleada(AU)

Introduction: Biliary cystadenocarcinoma is a rare cystic tumor, which originates from the hepatobiliary epithelium, whose growth is slow, and whose symptoms are variable and nonspecific. Objective: To present the case of a patient with a biliary cystadenocarcinoma. Case presentation: The case is presented of a 56-year-old female patient who came to our institution with pain in the right hypochondrium, fever and weight loss. Physical examination revealed a palpable mass in such abdominal area, with smooth edges, nonpainful, hard, with low mobility and related to the liver. Conclusions: Biliary cystadenocarcinoma is a rare malignant cystic neoplasm of the liver. Most patients present with abdominal pain, a palpable mass and weight loss. Surgery is the standard treatment and margin-free resection is considered the most commonly employed treatment variant(AU)

Management of Mucinous Cystic Neoplasms of the Liver.

BACKGROUND: Mucinous cystic neoplasms of the liver (MCN-L) including biliary cystadenomas (BCA) and biliary cystadenocarcinomas (BCAC) are rare cystic lesions that comprise less than 5% of all liver cysts and affect only a small subset of individuals. We herein review the current evidence regarding the clinical presentation, imaging characteristics, tumor markers, pathological findings, clinical management, and prognosis of MCN-L. METHODS: A comprehensive review of the literature was performed using MEDLINE/Pubmed and Web of Science databases. In PubMed, the terms "biliary cystadenoma," "biliary cystadenocarcinoma," and "non parasitic hepatic cysts" were queried to identify the most recent data on MCN-L. RESULTS: US imaging, CT, and MRI, as well as consideration of clinicopathological features, are required to appropriately characterize and diagnose hepatic cystic tumors. BCA are premalignant lesions and cannot be reliably differentiated from BCAC based on imaging alone. As such, both types of lesions should be treated with margin-negative surgical resection. Following surgical resection, recurrence is fairly low among patients with BCA and BCAC. Despite having worse long-term outcomes than BCA, the prognosis following surgical resection of BCAC still remains more favorable than other primary malignant liver tumors. CONCLUSION: MCN-L are rare cystic liver tumors that include BCA and BCAC, which can be difficult to differentiate based on imaging alone. Surgical resection remains the mainstay of management for MCN-L with recurrence being generally uncommon. Future multi-institutional studies are still required to better understand the biology behind BCA and BCAC to improve the care of patients with MCN-L.

Salivary gland cystadenocarcinoma: Rare neoplasm in buccal mucosa.

A 72-year-old female was referred for diagnosis of a lesion located in the right buccal mucosa, with duration unknown. At intraoral examination, the lesion appeared as a well-delimited, mobile, and submucosal nodule. A benign mesenchymal neoplasm was the main hypothesis of diagnosis. Histopathological analysis revealed salivary gland neoplasm formed by atypical cells often arranged in microcystic structures, with frequent intraluminal papillary projections. The tumor cells presented positivity for CK7 and negativity for CK20. Based on these features, the diagnosis of cystadenocarcinoma was established. The patient was submitted to wide surgical resection. No recurrence was observed after 48 months. Although rare, cystadenocarcinoma should be considered in the differential diagnosis of oral submucosal nodules.

Non-Neoplastic and Neoplastic Cysts of the Pancreas.

Inflammatory, developmental, and neoplastic lesions may all present as cystic masses on imaging. Pseudocyst is the most common of these and presents in association with a history of pancreatitis. Pancreatic cystic neoplasms are uncommon compared to solid neoplasms. They often present incidentally; therefore, an incidentally discovered cyst in the pancreas should be assessed with a high index of suspicion for neoplasm. The most common and frequently encountered cystic neoplasms include serous cystadenoma, mucinous cystic neoplasm, and intraductal papillary mucinous neoplasm. Less common epithelial cystic neoplasms include acinar cell cystadenoma and cystadenocarcinoma. Any solid neoplasm occurring in the pancreas or vicinity of the pancreas that has undergone cystic degeneration may present as a cystic mass. Non-epithelial lesions, such as lymphangioma, are also included in the differential diagnosis. The work-up needs to begin with a review of the clinical and imaging findings to establish a differential diagnosis. The primary focus of the pathologist will be first on differentiating mucinous from non-mucinous entities, since this will determine if the mass is an intraductal papillary mucinous neoplasm or a mucinous cystic neoplasm. If it is mucinous, the next step is to determine if the cystic neoplasm contains cells with high-grade cytological features. If it is non-mucinous, the pathologist needs to assess for neoplastic cells that would indicate a different neoplastic process. The cytological features need to be integrated with cyst fluid carcinoembryonic antigen and amylase measurements. Currently, molecular pathology is being integrated into the analysis of pancreatic cyst fluids. Here we will cover the cytological features and ancillary findings in cystic masses of the pancreas.

Six cases of central cystadenocarcinoma and review of relevant papers.

The present study was a review of cases of central cystadenocarcinoma over the past 29 years and provides clinical, radiological, and pathological information about these rare lesions. Six cases of central cystadenocarcinoma treated between 1991 and 2019 at Peking University Hospital of Stomatology in Beijing, China, were retrospectively analysed. A comprehensive review of clinical records was summarised and the histological diagnosis was revised using the 2017 World Health Organization criteria. The mean age of patients with central cystadenocarcinoma was 63 (range 51-75) years, and the male:female ratio 1:1. The clinical signs included localised swelling, pain, lower lip numbness, and trismus. There were more cases in the mandible than in the maxilla. All lesions were unilocular or multilocular in radiolucent regions with or without clear margins. The preferred treatment of central cystadenocarcinoma was surgical excision with wide margins, and no local recurrence was found during follow-up. Central cystadenocarcinoma often occurred in middle-aged or elderly patients. Because cystadenocarcinoma is somewhat rare, metastatic tumours of the jaw should be considered when diagnosing cystadenocarcinoma.

A Prospective Study on Contrast-Enhanced Endoscopic Ultrasound for Differential Diagnosis of Pancreatic Cystic Neoplasms.

BACKGROUND AND AIMS: To determine the value of contrast-enhanced endoscopic ultrasound (CE-EUS) for differentiation of pancreatic cystic neoplasms (PCNs). METHODS: From April 2015 to December 2017, 82 patients were enrolled in this study. All patients were confirmed to have PCNs by surgical pathology. Prior to surgery, all patients underwent fundamental B-mode EUS (FB-EUS) and CE-EUS, 65 of whom underwent computed tomography (CT) and 71 of whom underwent magnetic resonance imaging (MRI). The enhanced mode data of PCNs were recorded. The diagnostic accuracy of CE-EUS in classifying PCNs was compared with that of CT, MRI and FB-EUS. The ability of CE-EUS to identify PCNs was evaluated by comparing the enhanced mode of PCNs. RESULTS: There was a significant difference between benign and malignant lesions in enhanced mode (P = 0.017). The enhanced modes of benign lesions were mostly type II and type III, while those of malignant lesions were type 0, type I, and type IV. The sensitivity, specificity, and accuracy of type 0, type I, and type IV enhanced mode as the diagnostic criterion for malignant lesions were 80%, 65.3%, and 67.1%, respectively. CE-EUS demonstrated greater accuracy in identifying PCNs than did CT, MRI, and FB-EUS (CE-EUS vs. CT: 92.3% vs. 76.9%; CE-EUS vs. MRI: 93.0% vs. 78.9%; CE-EUS vs. FB-EUS: 92.7% vs. 84.2%). CONCLUSION: Compared with CT, MRI, and FB-EUS, CE-EUS is better at differentiating PCNs. CE-EUS is expected to be another important imaging technique for the diagnosis of PCNs.

Malignant solitary fibrous tumor of the kidney with liver metastasis: A case report and literature review.

Solitary fibrous tumor (SFT) is a rare spindle cell soft tissue tumor which is rarely encountered in the clinical setting and imaging findings are nonspecific, mainly occurring in the tissue structure of the serosa. However, there is very little report on SFT originating in the kidney in the medical literature. We report a case of SFT with liver metastasis in an adult female and discuss the pathological features as it appears in our case.

Cystadenocarcinoma of the mandible.

Papillary cystadenocarcinoma of the salivary gland is a rare malignant tumor and occurs in major and minor salivary glands. Papillary cystadenocarcinoma of the mandible is exceptionally rare. It is usually a low-grade destructive tumor with a papillary and cystic architecture. This case describes a unique presentation, location, and radiographic appearance of this lesion.

Low-grade intraductal carcinoma (low-grade cribriform cystadenocarcinoma) with tumor-associated lymphoid proliferation of parotid gland.

We report a rare case of low-grade intraductal carcinoma with tumor-associated lymphoid proliferation (TALP) in the parotid gland of a 75-year-old woman. Grossly, the tumor was solid and cystic. Histologically, the tumor consisted of a papillary-cystic, micropapillary, or focally cribriform proliferations of epithelial cells with low-grade cytological atypia. The interspaces between the epithelial components were filled with prominent lymphoid stroma and lymphoid follicles, superficially mimicking Warthin tumor. The neoplastic epithelial cells were positive for S100 protein by immunohistochemical staining. There was an attenuated layer of myoepithelial cells all around the epithelial components, indicating a non-invasive (in situ) nature. Although TALP is a rare finding in intraductal carcinoma, it should be considered as a histological variation of this kind of tumor. The relationship between intraductal carcinoma, low-grade cribriform cystadenocarcinoma, low-grade salivary duct carcinoma, and salivary duct carcinoma in situ is also discussed in this report.

Contrast enhanced ultrasound features of hepatic cystadenoma and hepatic cystadenocarcinoma.

OBJECTIVE: Hepatic (biliary) cystic tumor (HBCT) is a rare focal cystic liver lesion, which has been rarely described in the literature. In our current multicenter, retrospective study, we aimed to analyze contrast enhanced ultrasound (CEUS) features and its diagnostic performance in histologically proved HBCT. MATERIAL AND METHODS: Twenty-three patients with single HBCT were retrospectively analyzed. Histologically, 17 (73.9%) were benign hepatic (biliary) cystadenoma (HBCA), 6 (26.1%) were hepatic (biliary) cystadenocarcinoma (HBCAC). All CEUS examinations were assessed by two independent radiologists in consensus. Criteria of CEUS imaging evaluation included the contrast enhancement pattern of lesion (hypoenhancing, hyperenhancing, isoenhancing in comparison to the surrounding liver parenchyma) during the arterial, portal venous and late phases. RESULTS: After injection of ultrasound contrast agents, most of the HBCTs (78.3%, 18/23) had typical honeycomb enhancement pattern of the cystic wall, septa or mural nodules. Comparing between HBCA and HBCAC, hyperenhancement of the honeycomb septa during the arterial phase was more common in HBCA (p = .047). However, hypoenhancement during the portal venous and late phases was the characteristic of HBCAC (p = .041). CONCLUSIONS: The EFSUMB algorithm for CEUS for characterization of solid focal liver lesions is also applicable to HBCT. CEUS evaluation can avoid further diagnostic investigations or invasive biopsy procedure.

OK-432 injection therapy for cystadenocarcinoma of the parotid gland: A case report.

OK-432 is an immunomodulator that has been reported to be efficacious as an injection therapy for cervical lymphomas and ranulas. We performed OK-432 injection therapy to treat a cystadenocarcinoma of the parotid gland in a 72-year-old man. The 50 × 46-mm tumor was located in the deep lobe of the gland. The tumor had compressed the glossopharyngeal, vagus, and hypoglossal nerves, causing neurally mediated syncope, hoarseness, dysphagia, and dysarthria. A concentration of 5 KE/2 ml of OK-432 was injected. Within 2 months, the cyst had disappeared; no recurrence was apparent during 59 months of follow-up. To the best of our knowledge, no previous report has described injection of OK-432 for malignant cystic disease. We describe the injection method, injection dose, and postinjection course in the hope that this information will prove useful for future applications against malignant cystic disease.

Rare biliary cystic tumors: a case series of biliary cystadenomas and cystadenocarcinoma.

Cystic lesions of the liver are common and a major proportion is formed by parasitic cysts and simple cysts. Biliary cystic tumors (BCTs), namely biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC), are rare tumors which usually arise from the intrahepatic biliary tree. BCAs have malignant potential and are difficult to differentiate from BCAC pre-operatively on radiological imaging. Here we have presented 4 patients with BCTs and reviewed the literature pertaining to them.The data of four patients with BCA/BCAC diagnosed and treated at our institute were retrieved from our database and records were reviewed for age, sex, history, imaging, surgery, pathology and follow-up. Mean age of the patients was 53.5 years (range 30-71 years). Two male and two female patients presented with abdominal pain, of which one male patient had pancreatitis at diagnosis. Characteristic features were seen on pre-operative imaging (cystic lesions with internal septations) and biliary communication was identified in the patient with pancreatitis. Three patients were diagnosed with a BCA on final histology, while one patient had a BCAC. Following surgical resection, all the patients are asymptomatic and disease free with a mean follow-up of 24 months (range 10-40 months). In conclusion, BCTs should be suspected in the presence of a well-encapsulated, cystic hepatic lesion with internal septations. Although pre-operative distinction between BCA and BCAC is difficult, the lesion, whenever possible, should be completely resected as long-term outcomes are good, especially with BCA.

Cystadenocarcinoma of the salivary glands with potential lymph node metastasis.

Cystadenocarcinoma derived from the salivary gland in the head and neck region is an extremely rare malignancy. Therefore, the clinicopathological characteristics is not well understood. The purpose of this study was to clarify the clinicopathological characteristics, and present a total of four patients who were treated at the National Cancer Center Hospital East during the period between 1995 and 2012. The patients were all male, with ages ranging from 47 to 74 years old. The primary sites were the parotid glands, submandibular gland and minor salivary gland of the tongue. Within the follow-up period from 19 to 54 months, lymph node metastases were observed in three of the four patients. However, all the patients were salvaged by additional resection, including neck dissection, and remain alive to date without distant metastases. Cystadenocarcinoma is classified as a low-grade histological subtype of salivary gland tumors. Although the tumor has the potential to produce lymph node metastases, as shown in our patients, it is generally an indolent tumor with a good prognosis as compared with high-grade subtypes. This study suggests that a long-term follow up paying close attention to lymph node metastases is necessary for cystadenocarcinoma.

Contrast-enhanced ultrasound findings in a case of primary chest chondrosarcoma mimicking a porta hepatis mass.

We report a case of a 32-year-old woman who presented with upper abdominal pain. The physical examination revealed a palpable hard mass with a well-demarcated lower margin. Laboratory tests indicated hepatitis B viral infection. Computed tomography scan with intravenous contrast depicted a huge well-demarcated heterogeneous mass at the porta hepatis with irregular peripheral rim enhancement. Contrast-enhanced ultrasound showed peripheral irregular hyper-enhancement in the artery phase and hypo-enhancement in the portal and late phases. The postoperative histopathologic examination confirmed the diagnosis of low-grade chondrosarcoma. This is the first report of contrast-enhanced ultrasound findings of chondrosarcoma. There is some differential diagnosis to be discussed.

CT imaging of ovarian yolk sac tumor with emphasis on differential diagnosis.

Ovarian yolk sac tumors (YSTs) are rare neoplasms. No radiological study has been done to compare the imaging findings between this type of tumor and other ovarian tumors. Here we analyzed the CT findings of 11 pathologically proven ovarian YSTs and compared their imaging findings with 18 other types of ovarian tumors in the same age range. Patient age, tumor size, tumor shape, ascites and metastasis of two groups did not differ significantly (P > 0.05). A mixed solid-cystic nature, intratumoral hemorrhage, marked enhancement and dilated intratumoral vessel of two groups differed significantly (P < 0.05). The area under the ROC curve of four significant CT features was 0.679, 0.707, 0.705, and 1.000, respectively. Multivariate logistic regression analysis identified two independent signs of YST: intratumoral hemorrhage and marked enhancement. Our results show that certain suggestive CT signs that may be valuable for improving the accuracy of imaging diagnosis of YST and may be helpful in distinguishing YST from other ovarian tumors.

Intrahepatic biliary mucinous cystic neoplasms: clinicoradiological characteristics and surgical results.

BACKGROUND: Intrahepatic biliary mucinous cystic neoplasms are rare hepatic tumors and account for less than 5% of intrahepatic cystic lesions. Accurate preoperative diagnosis is difficult and the outcome differs among various treatment modalities.The aim of this study is to investigate the clinico-radiological characteristics of intrahepatic biliary mucinous cystic neoplasms and to establish eligible diagnostic and treatment suggestions. METHODS: Nineteen patients with intrahepatic biliary cystadenomas and two patients with biliary cystadenocarcinomas were retrospectively reviewed. Their clinico-radiological variables and survival outcome were analyzed. RESULTS: Of the 19 patients with biliary cystadenoma, 16 (84.2 %) were female. 11 (57.9 %) patients had symptoms before operation with the most common presenting symptom being abdominal pain. Among the patients with available data, serum and cystic fluid CA 19-9 levels were invariably elevated and the CA 19-9 level in the cystic fluid was significantly higher than that in the serum. Loculations (84.2 %) and septations (63.2 %) were the most common radiologic findings. For treatment, 11 (57.9 %) patients received radical resection by either enucleation or hepatic resection, while the remaining 8 (42.1 %) patients underwent only fenestration of liver cysts. Radical resection provided a significantly better clinical outcome than fenestration in terms of tumor recurrence (p = 0.018). The only two male patients with biliary cystadenocarcinoma received radical hepatic resection and achieved a disease-free survival of 16.5 months and 33 months, respectively. CONCLUSION: Intrahepatic biliary mucinous cystic neoplasms are rare and preoperative diagnosis is difficult. Internal septations and loculations on radiologic examinations should raise some suspicion of this diagnosis. Complete tumor excision is the standard treatment that may provide patients with better long term results after the operation.

Multiple primary hepatic malignant mesotheliomas mimicking cystadenocarcinomas on enhanced CT and FDG PET/CT.

A 50-year-old woman presented with upper abdominal pain for 2 months. Abdominal enhanced CT showed multiple hypodense cystic tumors in the liver with heterogeneous enhanced septations. FDG PET/CT showed hypermetabolic peripheral regions and internal septations of the tumors with SUVmax of 6.3. Multiple hepatic cystadenocarcinomas were suspected. The patient underwent resection of the tumors. Epithelial mesothelioma was confirmed by pathology. This case indicates primary hepatic mesothelioma should be added to the differential diagnosis of FDG-avid malignant hepatic tumors.