A case of retroperitoneal mucinous cystadenoma treated successfully by laparoscopic excision

A case of retroperitoneal mucinous cystadenoma treated laparoscopically is reported. A 26-year-old woman, who complained of abdominal fullness, was referred to us after a large intra-abdominal mass was discovered at her local hospital. Ultrasound and computed tomography showed a fluid-filled mass, 9 cm in diameter, in the left retroperitoneal space. Laparoscopic surgery was performed. We found a smooth and thin-walled cystic tumor that displaced the descending colon to the right and arose from the retroperitoneum, loosely adhering to the psoas muscle. We successfully extirpated the tumor laparoscopically. The surgical specimen consisted of a multilocular, red to yellowish-gray, cystic mass with a smooth, glistening external surface, and inner lining. The mass was filled with thin, yellowish fluid. The histologic diagnosis was benign mucinous cystadenoma. The postoperative course was uneventful, and the patient remains free of recurrence 15 months after surgery. Retroperitoneal mucinous cystadenoma is a rare entity, and the treatment of choice is surgical excision. Laparoscopic extirpation should be considered for the treatment of selected retroperitoneal cystic lesions.     

Mixed epithelial and stromal tumor growing with polypoid pattern in the renal pelvis

A 68-year-old woman was found incidentally to have right hydronephrosis and a renal pelvic mass by abdominal ultrasonography. Radiographic examinations revealed a heterogeneous renal pelvic tumor, and right nephroureterectomy was performed. The tumor was well circumscribed yellow-whitish solid mass with scattered cysts. Histologically, the tumor was composed of both mesenchymal and epithelial components. The mesenchymal elements consisted of fibroblasts and smooth muscle cells, and the epithelial elements of cystic and tubular structures lined by cuboidal epithelium. Atypia and mitoses were not identified. The patient was free of recurrence 42 months postoperatively. Mixed epithelial and stromal tumor of the kidney is a recently recognized neoplasm that occurs almost exclusively in perimenopausal woman. Similar tumors have been reported previously under various names, including adult mesoblastic nephroma and cystic hamartoma of the renal pelvis. Histogenesis of the tumor is still controversial.     

肝胆管囊性肿瘤临床病理特点及治疗(附8例报告)

目的 分析肝胆管囊性肿瘤(囊腺瘤和囊腺癌)的临床、病理特点及治疗。探讨其中卵巢样间质的免疫表型及组织来源。方法 回顾性分析7例肝胆管囊腺瘤和1例囊腺癌的临床表现、病理特征及治疗结果。对4例卵巢样间质细胞行CD117、CD34、ER、PR、Vim、SMA、S100、Desmin等免疫组织化学检测。结果 8例病人中女性7例，男性1例。表现为上腹不适，伴黄疸、腹泻或闭经等。3例为手术后复诊病例。镜下见部分囊腺瘤内衬上皮明显异型增生。检测4例囊腺瘤中的卵巢样间质细胞，4例均表达CD117、Vim及SMA；分别有3例表达CD34及ER、PR；2例表达S100；Desmin无表达。肿瘤全部切除后无复发；部分切除或同时做内引流术均复发。结论 肝胆管囊性肿瘤好发于女性。上腹不适是其常见表现。术前诊断困难。手术完全切除是最佳治疗方案。在肝胆管囊腺瘤和囊腺癌间存在交界性病变。推测肿瘤来源于内、中两个胚层。卵巢样间质成分来源于原始间质细胞，具有平滑肌和神经双向分化，其生长可能依赖神经和雌、孕激素调节。     

Peritoneal hepatobiliary cystadenoma--case report

Hepatobiliary cystadenoma with mesenchymal stroma are infrequent form of cystic neoplasm that may be found in females only. It is difficult to reach correct diagnosis prior to surgery. We are presenting a case of 32 years-old female referred to our institution for revealing a cause of discomfort and pain in right subcostal region whereas peritoneal hepatobiliary cystadenoma has been determined. Abdominal ultrasonography and computerized tomography revealed cystic lesion adjacent to gall bladder, which was initially thought to be of echynococcal origin. At surgery, a mesenterial cystic neoplasm has been revealed, having a close contact with gall bladder, without signs of its infiltration. Pathophysiology discovered hepatobiliary cystadenoma with mesenchymal stroma. Pre-surgical differential diagnosis in hepatobiliary cystadenoma may be very difficult, especially if, like in the presented case, neoplasm has extra hepatic localization. Radical surgical excision is treatment of choice, concerning malignant potential of these neoplasms.     

Intra-abdominal mucinous adenocarcinoma of urachal origin: report of a case

Intra-abdominal mucinous cystic tumors can be difficult to diagnose preoperatively. We report a case of histologically diagnosed primary urachal adenocarcinoma: a rare type of bladder tumor. This case report is interesting for clinicians. The patient was an 86-year-old man who presented with acute abdominal pain. Computed tomography (CT) showed a large cystic mass with calcification, near the apex of the urinary bladder. Laparotomy revealed a large intra-abdominal cystic mass adherent to the anterior abdominal wall and superior to the urinary bladder. We performed laparoscopic-assisted resection and partial cystectomy. The cystic mass measured approximately 15 × 14 × 11 cm and contained mucinous material. Histological examination revealed that it extended to the muscle of the bladder wall and that its epithelium was composed of atypical cells with increased papillary morphology. The mucinous material was glycoprotein with degenerative fatty tissue, and calcification was recognized partly in the specimen. Thus, we comprehensively diagnosed a mucinous cystic adenocarcinoma of urachal origin.     

Lymphangiomyoma of the shoulder: an exceptional localisation. A case report

Lymphangioleiomyomatosis (LAM) is a rare condition usually occurring in young women of childbearing age. It is characterised by the presence of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes, and/or other organs, with a few reports of isolated extrapulmonary cases. We report the case of a 26-yr-old female who presented with a painless shoulder mass. Ultrasonography, computed tomography and magnetic resonance imaging revealed an intermuscular mass with cystic and solid tissue components, measuring 6 cm x 4 cm. The mass was removed surgically after the diagnosis of LAM was made following tru-cut biopsy. This case corresponds to an exceptional localisation of LAM.     

Esophagus Tissue Engineering: Hybrid Approach with Esophageal Epithelium and Unidirectional Smooth Muscle Tissue Component Generation In Vitro

Purpose  The aim of this study was to engineer the two main components of the esophagus in vitro: (a) esophageal epithelium and (b) smooth muscle tissue. Furthermore, (a) survivability of esophageal epithelial cells (EEC) on basement membrane matrix (BMM)-coated scaffolds and (b) oriented smooth muscle tissue formation on unidirectional BMM-coated collagen scaffolds was investigated. Methods  Both EEC and smooth muscle cells (SMC) were sourced from Sprague–Dawley rats. The EEC were maintained in vitro and seeded onto BMM-coated 2-D collagen scaffolds. Similarly, smooth muscle cells were obtained using an explants technique and seeded on unidirectional 3-D BMM-coated collagen scaffolds. Cell–polymer constructs for EEC and SMC were maintained in vitro for 8 weeks. Results  Protocols to obtain higher yield of EEC were established. EEC formed a layer of differentiated epithelium after 14 days. EEC survivability on polymers was observed up to 8 weeks. Unidirectional smooth muscle tissue strands were successfully engineered. Conclusion  Esophageal epithelium generation, survivability of EEC on BMM-coated scaffolds, and engineering of unidirectional smooth muscle strands were successful in vitro. The hybrid approach of assembling individual tissue components in vitro using BMM-coated scaffolds and later amalgamating them to form composite tissue holds promises in the tissue engineering of complex organ systems. This research is funded by the European Union within the 6th Framework Program (EuroSTEC; LSHC-CT-2006–037409). XXIst International Symposium on Pediatric Surgical Research, 2–4 October 2008, Leipzig, Germany     

Solitary hepatic lymphangioma: Report of a case

A 52-year-old woman presented with upper abdominal pain. Abdominal ultrasonography showed a 4-cm well-defined mass containing solid and cystic components in segment IV of the liver, and contrast-enhanced T1-weighted magnetic resonance imaging revealed heterogeneous enhancement within the tumor, indicating a solid or fibrous component. There were no cystic lesions in any other organs. A partial hepatectomy was performed, based on a preoperative diagnosis of sclerosing hemangioma and biliary cystadenoma or cystadenocarcinoma. Pathologically, the tumor appeared to be a multilocular and cystic lesion lined by attenuated endothelial- like cells with no atypia. Immunohistochemistry demonstrated the endothelial-like cells to be positive for the lymphatic-specific markers D2-40, LYVE-1, and Prox-1, which proved helpful for confirming the diagnosis as solitary hepatic lymphangioma. This case is presented with details of the pathologic and radiologic findings, because solitary hepatic lymphangioma is an extremely rare tumor and no previous reports have provided details of the immunohistochemical characteristics.     

Ciliated foregut cyst of the gallbladder: report of a case

We report the rare case of a gallbladder cyst arising from the foregut remnants. A 36-year-old woman was referred to our hospital after screening ultrasonography (US) detected a tumor in the gallbladder. On admission, she was well and her blood analyses were all normal. US showed a cystic mass with internal high-echoic lesions, and computed tomography (CT) demonstrated a protruding tumor with slight enhancement in the gallbladder. Angiography provided no additional information; however, sequential CT-arteriography (CTA) clearly demonstrated that this tumor was a cystic lesion. Surgical exploration was performed, first because of the difficulty in establishing a definite diagnosis, and also because the patient wanted the tumor removed. The resected specimen contained a unilocular cystic tumor that looked like a submucosal tumor. Histologically, the wall of the cyst was lined by ciliated stratified columnar epithelium with interspersed goblet cells and underlying smooth muscle fibers. The mass was finally diagnosed as a congenital ciliated foregut cyst of the gallbladder. Cysts of the gallbladder are uncommon and the majority are acquired. To our knowledge, this represents only the fourth report of a ciliated foregut cyst of the gallbladder in the literature. Although rare, an awareness of this entity could allow a preoperative diagnosis to be made, whereby surgical exploration may be avoided. CT-A is a very useful diagnostic tool, especially when the nature of the tumor presents a difficult differential diagnosis.     

Multilocular cystadenoma and cystadenocarcinoma of the prostate

BACKGROUND: Multicystic prostatic tumors are rare, with only a few reported cases of prostatic cystadenoma and cystadenocarcinoma in the scientific literature. METHODS: A retrospective review of our tumor registry over the last 25 years identified 2 rare cystic tumors of the prostate: 1 multilocular cystadenoma and 1 multilocular cystadenocarcinoma. RESULTS: The first case illustrates the clinical and pathologic features of prostatic multilocular cystadenoma. A 42-year-old man presented with a 16-cm suprapubic mass causing displacement of adjacent visceral organs. Pathologic examination after prostatectomy confirmed it to be a multilocular cystadenoma of the prostate. The patient's postoperative course was uneventful, and his serum prostate-specific antigen level remained at < or =0.04 ng/ml throughout the course of his disease. In the second case, we present an 80-year-old male presenting with a 12-cm cystic mass of the prostate. His serum prostate-specific antigen level remained at > or =9.0 ng/ml throughout the course of his disease. The tumor had an aggressive local growth pattern, with invasion into perirectal adipose tissue. This patient underwent a pelvic exenteration, followed by adjuvant systemic chemotherapy and complete androgen blockade. Despite aggressive treatment, he had 3 recurrences over 4 months but remains alive with disease at 23-month follow-up. CONCLUSIONS: Cystadenocarcinoma of the prostate is locally aggressive and should be included in the differential diagnosis of cystic lesions of the prostate.     

Lymphoepithelial Cyst of the Pancreas: Report of a Case

An extremely rare case of a lymphoepithelial cyst (LEC) of the pancreas is described herein. A pancreatic cystic tumor was initially detected in a 50-year-old man at a medical checkup. On admission, his serum carbohydrate antigen (CA) 19-9 level was 8 100 U/ml and a computed tomography scan revealed a well-circumscribed multilocular cystic tumor in the pancreatic head and body. Magnetic resonance cholangiopancreatography showed no communication between the pancreatic ducts and the tumor. A distal pancreatectomy with lymph node dissection was performed because the lesion was suspected to be a mucinous cystadenoma or cystadenocarcinoma of the pancreas. However, histological examination revealed that the cyst was lined by stratified squamous epithelium and surrounded by lymphoid tissue, thereby confirming the diagnosis of LEC of the pancreas. The superficial layer of squamous epithelium and the cystic contents were found to be immunohistologically positive for CA19-9. Establishing a preoperative diagnosis of LEC is quite difficult because it resembles other cystic neoplasms of the pancreas in radiographic features and is frequently associated with an elevation of serum tumor markers such as CA19-9. Received: June 21, 1999 / Accepted: March 24, 2000     

Composite tumor of mucinous cystadenoma and somatostatinoma of the kidney

Approximately 30 cases of carcinoid tumor of the kidney have been reported in the English literature, including three cases found as components of teratomas. Renal composite tumors associated with somatostatinoma have not been described. A 53-year-old female presented with an incidentally found right renal cystic lesion. Computed tomography demonstrated a cystic lesion associated with a solid nodule in the right kidney and postcontrast dynamic MRI revealed enhancement of the solid nodule. The patient underwent radical nephrectomy for the kidney lesion and is now well without recurrence 21 months after the operation. From the histopathological findings we diagnosed the cystic lesion as a composite tumor composed of mucinous cystadenoma and carcinoid tumor. Immunohistochemistry demonstrated the majority of cells of in carcinoid portion to be positive for antisomatostatin staining. The present case is the first documented composite tumor of mucinous cystadenoma and somatostatinoma of the kidney.     

Hepatobiliary cystadenoma combined with multiple liver cysts: report of a case

Hepatobiliary cystadenomas are rare benign tumors with malignant potential. They are almost always solitary lesions accompanied by multilocular cysts in the liver, and are difficult to differentiate from cystadenocarcinoma, despite the diagnostic modalities available. This report describes a case of hepatobiliary cystadenoma with multiple cysts in the left hepatic lobe, diagnosed by magnetic resonance imaging in a 48-year-old woman. Abdominal computed tomography revealed only multiple cystic lesions in the left lobe, but cholangiography via a nasogastric biliary drainage tube combined with percutaneous transhepatic cholangiography showed a stenotic region with fine irregularity in the left lateral posterior segmental bile duct and left lateral anterior segmental bile duct. Hepatobiliary cystadenocarcinoma with multiple liver cysts was suspected. We performed left hepatectomy, and microscopic examination confirmed a diagnosis of hepatic cystadenoma with multiple liver cysts. There was no nuclear atypia or mitosis in the epithelium of the locus, which was constructed of simple columnar-to-cuboidal epithelium with basal nuclei. The patient is well without recurrence more than 4 years after surgery. Received: July 5, 2000 / Accepted: January 9, 2001     

Cystadenoma of the seminal vesicle

Primary tumors of the seminal vesicle are extremely rare. Among them, there is a spectrum of tumors derived from both epithelium and stroma and so classified as epithelial-stromal tumors. Herein, we report a case of a cystadenoma in a 49-year-old asymptomatic man, detected in a routine ultrasonography for liver disease follow-up. The digital rectal examination detected a large mass anterior to rectum and posterior to bladder. Computed tomography scan and magnetic resonance imaging showed a normal prostate and a 9.0 cm cystic tumor, replacing the left seminal vesicle. The gross appearance and microscopic aspect was compatible with cystadenoma of seminal vesicle. Patient's postoperative recovery was uneventful. He is currently alive, 3 years after the diagnosis, with no signs of recurrence.     

Hepatobiliary cystadenoma with mesenchymal stroma.

In a detailed review of cystic hepatobiliary neoplasms, we identified a subset of 50 cases in which tumors were characterized by the presence of a mesenchymal cell layer interposed between an inner epithelial lining and an outer connective tissue layer. We have recently seen three such patients, making a total of 53 patients reported in the English literature. All of the patients were female, 44 of whom, with an average age of 41 years, had benign tumors. The average age of the remaining nine patients was 57 years and these patients had malignant tumors. In seven patients, the malignancy arose from the epithelial layer, but in two patients sarcomatous changes were identified in the mesenchymal tissues. The older age of the patients with malignant tumors with adequate serial biopsies in two cases supported the thesis that malignant transformation may occur in the benign tumors. Moreover the location of the tumor in one of our patients in whom the resected tumor was associated with anomalous right hepatic ducts and portal veins supported the theory that these tumors develop embryologically from nests of primitive hepatobiliary endodermal and mesodermal cells. Although surgical treatment was performed in all patients, 25% of the patients with benign hepatobiliary cystadenoma with mesenchymal stroma (CMS), and 33% of the patients with malignant CMS had tumor recurrence after primary resection. Ninety per cent of these patients had an incomplete resection at the time of their initial operations. Forty-four per cent of the patients with malignant CMS died after a mean follow-up of 17 months. We conclude that CMS (Edmonson's tumor) occurs uniquely in young female patients, develops from nests of primitive embryonal cells, has the potential for malignant transformation, and should be completely resected at primary operation to avoid recurrence.     

Epstein-Barr Virus-Associated Smooth Muscle Tumor of the Larynx: Report of a Rare Case Mimicking Leiomyosarcoma

We present a patient (male 54 years) with a history of renal transplant who 14 years post transplantation developed a symptomatic (stridor) laryngeal Epstein Barr virus (EBV)—associated smooth muscle tumor (EBV-SMT) in the absence of concomitant disease elsewhere. Nine years post transplantation the patient developed a subcutaneous EBV-SMT tumor located on the calf. The laryngeal tumor displayed low-grade nuclear atypia and was infiltrating into the surrounding soft tissue, focally ulcerating through the overlying epithelium. Histologic features included: neoplastic cells with myoid differentiation, a component of primitive appearing small mesenchymal cells with hyperchromatic nuclei, mitotic activity, intralesional small to medium sized blood vessels and T-lymphocytes. Both the myoid and small cell mesenchymal components strongly expressed smooth muscle actin and h-caldesmon, but not desmin, cytokeratins, CD34 or S-100 protein. There was strong positive nuclear reaction for EBV-RNA on in situ hybridization (EBER). No other tumor was detected on clinical and radiological examinations and no evidence of tumor in other sites, over 8 months of follow-up, till death was detected. This case emphasizes the importance of considering this pathologic entity when solitary smooth muscle actin-expressing tumors are encountered in the larynx of immunocompromised patients.     

A case of cystadenocarcinoma of the liver

A case of cystadenocarcinoma of the liver is reported. The patient was a 73-year-old woman in whom a tumor was detected in the lateral segment of the liver during a health examination. Ultrasonograms and computed tomograms showed a multilocular cystic mass. Magnetic resonance imaging (MRI) showed a multilocular lowintensity mass, including a high-intensity portion and a portal branch compressed by the tumor. MRI with gadolinium showed an enhanced cyst wall. The cystic part of the tumor became smaller and the solid part became larger over a 1-month period, indicating that the tumor was malignant. Subsegmentectomy (S₃) was performed and cystadenocarcinoma with cystadenoma was diagnosed by histopathological examination. Identification of changes in the appearance of a tumor should be helpful for the differential diagnosis of cystadenoma and cystadenocarcinoma.     

Seminal Vesicle Cystadenoma: A Rare Clinical Perspective

A 52-yr-old man presented with severe obstructive urinary symptoms. Ten years earlier, a digital rectal examination disclosed a small mass above the prostate, and a computed tomography (CT) scan showed a 3.5-cm cystic tumor of the right seminal vesicle. He had been followed conservatively elsewhere. Reevaluation of the mass with a CT scan and magnetic resonance imaging showed that the mass had grown to a maximal diameter of 14 cm. A transabdominal needle biopsy revealed benign fibromuscular tissue. The tumor was then resected by an open transvesical approach. Pathology was consistent with a benign seminal vesicle cystadenoma. The natural history, pathology, and surgical approach are described.     

Giant congenital urethral polyp

A case of giant congenital urethral polyp on a female newborn is reported. The lesion appeared as a large fleshy, exuberant tumor at the introitus between the labia minora. The mass was constituted by orderly arranged layers of transitional and squamous epithelium outside and loose connective tissue and smooth muscle in its core. The patient was treated by simple excision of the lesion.