Primary retroperitoneal mucinous cystadenoma： Report of a case and review of the literature

Primary retroperitoneal mucinous cystadenomas （RMCs） are very rare and their biological behavior and histogenesis remain speculative. An accurate preoperative diagnosis of these tumors is difficult because no effective diagnostic measures have been established. We describe a 29-year-old woman with abdominal pain and a palpable mass. Computed tomography （CT） of the abdomen revealed a retroperitoneal cystic mass which was resected successfully at laparotomy. Histopathological examination of the resected mass confirmed the diagnosis of RMC. There was no evidence of disease 2 years after surgery.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature

Lymphangiomas are rare benign cystic tumors of the lymphatic system. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas, and approximately 186 cases have been reported. They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors, including those arising from the liver, kidney and pancreas. This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient. The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention, abdominal pain, anorexia, fever, nausea and diarrhea. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis. Surgical removal of the cyst was accomplished without incident. A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.     

Mucinous biliary cystadenoma with mesenchymal stroma: Expressions of CA 19-9 and carcinoembryonic antigen in serum and cystic fluid

a case of mucinous biliary cystadenoma with mesenchymal stroma (CMS tumor) in a 64-year-old woman is reported. The patient presented with acute abdominal pain and a palpable mass in the upper abdomen. Computed tomography and abdominal sonography showed characteristic multilocular cysts in the left lobe of the liver. Serum CA 19-9 was elevated to 108 U/ml with normal carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) levels. The levels of CA 19-9 and CEA in the cystic fluid were high at 7430 U/ml and 576ng/ml, respectively. The serum CA 19-9 returned to 35 U/ml 4 weeks after tumor resection. These corresponding findings of both tumor markers in the serum and cystic fluid imply that (1) CA 19-9 and CEA both exist in the epithelial component of CMS tumors as evidenced by immunohistochemical stain, (2) serum CA 19-9 is a valuable marker in the diagnosis and monitoring of CMS, and (3) in cystic fluid, there are more significantly high levels of CA 19-9 in CMS compared with levels in simple cyst and polycystic liver disease. Therefore, measurement of CA 19-9 in cystic fluid and serum may be helpful in the differential diagnosis of hepatic cystic lesions.     

Simultaneous mucinous cystadenoma of ovary and mucinous cystadenocarcinoma of pancreas

Mucinous cystic tumors were discovered synchronously in the tail of the pancreas and in the right ovary of an adult female. Both tumors were amenable to surgical resection. The pancreatic tumor was a noninvasive mucinous cystadenocarcinoma and the ovarian tumor was a mucinous cystadenoma. We feel these tumors represent two primaries, an uncommon occurrence, and not a single primary tumor with metastasis.     

Simultaneous mucinous cystadenoma of ovary and mucinous cystadenocarcinoma of pancreas.

Mucinous cystic tumors were discovered synchronously in the tail of the pancreas and in the right ovary of an adult female. Both tumors were amenable to surgical resection. The pancreatic tumor was a noninvasive mucinous cystadenocarcinoma and the ovarian tumor was a mucinous cystadenoma. We feel these tumors represent two primaries, an uncommon occurrence, and not a single primary tumor with metastasis.     

Asymptomatic lymphangioma involving the spleen and retroperitoneum in adults

Lymphangioma, a benign neoplasm of the lymphatic system, is common in children but rare in adults. Its clinical manifestations include abdominal pain, nausea,vomiting and a palpable mass. However, abdominal sonography or abdominal computed tomography (CT) scan can also incidentally reveal lymphangioma. A larger or symptomatic lymphangioma is treated with total resection to prevent recurrence, infection, torsionand enlargement. Although lymphangioma rarely becomes malignant, its prognosis is generally good.We report a cystic lymphangioma of the spleen and retroperitoneum, which was incidentally found in a 56-year-old man who was hospitalized due to a colon mass. Physical examination showed no specific findings.Abdominal CT revealed a 5.7 cm, non-enhanced multilobulated cystic mass with multiple septa in the spleen and a 10 cm lobulated cystic mass in the paraaorticarea. Splenectomy and retroperitoneal resectionof the cystic mass were conducted. The endothelium of splenic and retroperitoneal cyst was immunohistochemically stained with D2-40 antibody. The patient was finally diagnosed with splenic cystic and retroperitoneal cavernous lymphangioma.     

Pancreatic cystadenoma: diagnostic value of ultrasonics and x-ray computed tomography

We report six cases of pancreatic cystadenoma (three mucinous cysts, three microcystic adenomas). All patients were women. The mean age at the time of diagnosis was 50 years (with a range from 24 to 70 years). The mean age of patients having mucinous cysts (36.6 years) was lower than that of patients having microcystic adenomas (63.3 years). Abdominal pain was the first symptom in five patients. A palpable abdominal mass was found in three cases. The type of the tumor was demonstrated by ultrasonography and computed tomography and was confirmed by laparotomy in five cases. The tumors involved the body and tail of the pancreas. A distal pancreatectomy was performed in five cases, with a splenectomy in four cases. The post-operative course was uneventful. One patient was not operated. The histological examination of the three mucinous cysts showed benign tumors. This study emphasizes the usefulness of ultrasonography and computed tomography for the diagnosis of pancreatic cystadenomas. Surgical therapy is mandatory for mucinous cysts because of their potential malignancy, but a careful follow-up might be proposed for poor-risk patients having a microcyst adenoma, since this tumor is benign.     

Large mucinous biliary cystadenoma with ＂ovarian-like＂ stroma： A case report

Hepatobiliary cystadenomas are rare tumors that are difficult to diagnose preoperatively. They can reach large sizes that make them real intraoperative "surprises". A 63-year-old woman, presented with a symptomatic huge abdominal cystic mass, underwent complete resection of the mass with safety margins. Histopathological report revealed mucinous hepatic cystadenoma with "ovarianlike" stroma and areas of sclerohyalinization. The differential diagnosis of the large cystic tumors which occupy the right hemiabdomen must include the biliary cystadenoma; the complete resection of the tumor with safety margins avoids local recurrence, and therefore represents the optimal therapy because of the malignant potential of the disease. The postoperative follow-up includes abdominal ultrasound or CT scan and CA 19-9 measurement.     

胰腺黏液性囊腺瘤的诊断和外科治疗

目的 探讨胰腺黏液性囊腺瘤(MCN)的诊断和外科治疗效果.方法 回顾性分析2003年1月至2008年6月问在青岛大学医学院附属医院手术治疗的20例MCN患者的临床表现、病理特征、治疗方法和生存率等临床和随访资料.结果 患者以腹痛、上腹饱胀不适、恶心、呕吐为主要临床表现,11例有上腹部压痛,6例可触及上腹肿块.术前均行B超及CT检查,诊断13例为良性MCN,4例为胰腺浆液性囊腺瘤,3例为黏液性囊腺癌.肿瘤主要位于胰腺体、尾部,直径4～14 cm.20例均行手术治疗,以胰十二指肠切除术、胰体尾切除术为主.术后病理证实,良性MCN 10例,交界性MCN 3例,黏液性囊腺癌7例.术后平均随访26个月,良性MCN和交界性MCN患者均健在,无复发;黏液性囊腺癌患者3年生存率50%.结论 MCN主要好发于女性,临床表现无特征性,术前B超和CT检查可进行诊断,手术切除是惟一有效的治疗方法,预后较好.     

Inflammatory myofibroblastic tumor successfully treated with chemotherapy and nonsteroidals: A case report

Inflammatory myofibroblastic tumor (IMT) occurring at retroperitoneal sites has rarely been reported. We report the case of a previously well 14-year-old girl with no history of abdominal disease whose past medical history and family tumor history were unremarkable. She complained of intermittent abdominal pain for one month. An abdominal mass was found on physical examination and abdominal contrast-enhanced computed tomography (CT) showed a hypodense soft mass, the size and location of which suggested a well delineated retroperitoneal tumor surrounding the superior mesenteric vessels measuring 3.3 cm × 4.5 cm × 4.5 cm with enlarged lymph nodes. The patient underwent an exploratory laparotomy followed by biopsy and was subsequently diagnosed with retroperitoneal IMT. She was successfully treated with postoperative chemotherapy and oral diclofenac sodium. Following completion of therapy the mass was no longer palpable and no longer visible on CT scanning. The use of methotrexate and cisplatin for aggressive myofibroblastic tumors is also reviewed.     

A case of macrocystic serous cystadenoma of the pancreas

Summary A case of macrocystic serous cystadenoma of the pancreas is presented, and literature is reviewed. A 35-yr-old woman presented with mild upper abdominal pain. Abdominal ultrasonography and an abdominal computed tomography revealed a multiloculated and calcified cyst in the body of the pancreas. A T1-weighted image, using magnetic resonance imaging, revealed a low-intensity mutiloculated, pancreatic mass. In contrast, T2-imaging of the tumor showed a high-intensity mass. Endoscopic retrograde cholangiopancreatography showed no contact between the main pancreatic duct and the tumor. The preoperative diagnosis was a mucinous cystic neoplasm. Tumor enucleation was performed. Subsequent microscopic examination of this tumor suggested the diagnosis of a macrocystic serous cystadenoma of the pancreas.     

Obstructive Jaundice Caused by Mucinous Cystic Tumor of Gallbladder: A Case Report and Literature Review

Mucinous cystic tumor of the gallbladder is an extremely rare benign tumor, with potential for malignant degeneration. Mucinous cystic tumors of the cystic duct are divided into mucinous cystadenoma and mucinous cystadenocarcinoma. Currently, cystadenoma is generally considered to be a precancerous lesion of cystadenocarcinoma. At present, there are few cases reported worldwide, and there are no relevant guidelines for diagnosis and treatment of this disease. This article presents the collected clinical data of a patient with mucinous cystic tumor of the gallbladder who was admitted to the First Affiliated Hospital of Hunan Normal University, with the characteristics of the disease summarized in combination with a focused literature review.     

Mucinous cystic neoplasm of the pancreas during pregnancy: the importance of proper management

We describe a case of huge mucinous cystic tumor of the pancreas in a 26-year-old woman during pregnancy. Ultrasonography demonstrated a well-delimited cystic mass in the left upper abdominal quadrant, suggestive of benignity. Magnetic resonance imaging showed a large cystic mass resembling a mucinous cystic tumor of the pancreas. After this assessment the patient underwent surgical exploration and a huge cystic tumor of the pancreas was discovered. The tumor was enucleated and distal pancreatectomy was performed. The resected margin of the specimen was free of tumor. In this case report we discuss the management of mucinous pancreatic tumors during pregnancy and we briefly review the previously reported cases of mucinous pancreatic tumors in pregnant patients. We conclude that surgical resection of these tumors should be strongly considered in pregnancy. Removal of the tumor appears to be a safe procedure without harmful effects to the fetus.     

The simultaneous occurrence of mucinous cystadenomas in liver and pancreas

The occurrence of mucinous cystadenomas localized to the liver and pancreas simultaneously and treated with a single surgical procedure has been described for the first time in this report. A 47-year-old woman attended the outpatient clinic complaining of abdominal pain and the appearance of an abdominal mass. On clinical examination, hepatomegaly was found. An abdominal computed tomography scan showed a large cystic lesion of the left lobe of the liver, thus causing the hepatomegaly. Moreover, the computed tomography scan showed a cystic lesion of the pancreas. Both lesions had thick walls and septa. Magnetic resonance imaging of the liver and pancreas confirmed the presence of septa within the cysts. Surgery was performed owing to the suspected malignancy. It should be emphasized that the patient had preoperatively received prophylactic treatment for hydatosis. A hepatic pericystectomy and enucleation of the pancreatic lesion were performed during the surgery. Pathology showed a mucinous cystadenoma without sign of malignancy. At the 4-year follow-up, no recurrence was found. This case is of interest for several reasons: the unusual double presentation, the treatment, and the follow-up. This case report confirms the common origin of mucinous cystic tumours of liver and pancreas.     

Giant biliary mucinous cystadenoma of the liver

Biliary mucinous cystadenomas (BMC) of the liver are rare benign cystic tumors, however an estimated 20% undergo malignant transformation. They have recently been redefined as mucinous cystic neoplasms in the 2010 WHO classification. The preferred treatment is through radical resection, as there are high recurrence rates with other treatment modalities; however this is often not possible in patients with bilobar or giant cysts, and liver transplantation may be indicated. We present a patient with a giant biliary mucinous cystadenoma of the liver and discuss the management with reference to the literature. A 47 year-old woman presented with a 6-week history of moderate epigastric discomfort on a background of 12 months of symptom-free abdominal distension. A giant cystic bilobar tumor of the liver measuring 22 × 23 × 17 cm was diagnosed and characterised by ultrasound scan and magnetic resonance imaging. Serum bilirubin, alkaline phosphatase and gamma-glutamyl transpeptidase were elevated, though other laboratory data including tumor markers (CEA, aFP, CA19-9) were within normal limits. Total excision of the cyst was not possible due to its size and position, and the patient underwent cyst drainage, a sub-total cyst excision and omentoplasty. Histology confirmed a benign biliary mucinous cystadenoma with an ovarian stroma. Though the patient remained clinically well, routine post-operative computed tomography (CT) surveillance showed an 11 cm recurrent cyst at 6 months. A partial cyst resection with close follow-up, regular CA19-9 serology and ultrasound/CT imaging, may be a reasonable alternative for bilobar or giant cysts. However should any features pathognomonic of malignancy develop, then a liver transplantation is indicated.     

Mucinous cystadenoma of appendix concomitant with perityphlic granuloma

We describe a case of mucinous cystadenoma of the appendix with perityphlic granuloma. The patient developed a hard palpable mass in the right lower quadrant and barium enema inducted irregular bowel wall at the cecum. Based on a preoperative diagnosis of carcinoma of the appendix, we performed a laparotomy. On operation, we found a tumor mass in the region of the appendix; the mass adhered strongly to the retroperitoneum and cecum. Right hemicolectomy was carried out on a diagnosis of carcinoma of the appendix. Microscopic examination revealed mucinous cystadenoma of the appendix with perityphlic granuloma. Mucinous cystadenoma in the appendix is a rare tumor. In this patient, the tumor was accompanied by granuloma formation in adjacent tissues because of mucin expelled from the appendix. This case emphasizes that granuloma formation can make of difficult to differentiate mucinous cystadenoma in the appendix from cancer.     

Primary Biliary Cystic Tumors of the Liver

Five cases of primary cystic tumors of the intrahepatic bile duct are documented over a 5-yr period. Clinically, two cases had recurrent episodes and one had a first attack of jaundice, fever, and pain in the upper abdomen; one complained of abdominal fullness and one was asymptomatic. The appearance of the tumors were single, large, multilocular, and cystic. Important radiological findings included irregular thickness of the cystic wall, internal septation, and papillary projection. Marked biliary dilation was demonstrated in four of the patients. On the cholangiogram, amorphous filling defects were seen in the dilated extrahepatic bile ducts, and mucinous material could be removed from the ducts as well. Obstruction of the bile ducts by this mucinous bile was assumed to be the cause of cholangitis in three patients. Histological examination revealed three cases of cystadenoma and two cases of cystadenocarcinoma.     

Effects of histologic type on levels of macrophage colony-stimulating factor in liquid contents of benign ovarian tumors

BACKGROUND: Normal ovarian tissue is rich in cytokines. Cytokines are important in the physiology of ovarian function. Most of the same cytokines that are found in normal ovarian tissue are also found in association with benign and malignant tumors in contrast to their functions in normal tissues. Thus, we measured macrophage colony-stimulating factor (M-CSF) levels in the liquid contents of benign ovarian tumors--serous cystadenoma, mucinous cystadenoma, and mature cystic teratoma--and investigated whether M-CSF levels were associated with the histologic type of the ovarian tumors. METHODS: We enrolled 65 patients, 52 with benign ovarian tumor and 13 in the early postmenopausal period with symptoms of a menopausal disorder. Among the 52 patients with benign ovarian tumor, 16 had serous cystadenoma, 21 had mucinous cystadenoma, and 15 had mature cystic teratoma. Immediately after surgery, the liquid content was drawn from the ovarian tumor, then centrifuged, and the separated supernatant was stored at -30 degrees C. The M-CSF level was determined by the sandwich enzyme-linked immunosorbent assay method with use of three antibodies. RESULTS: The level of M-CSF was 12,513 U/mL (median) (range, 0-169,000 U/mL) in serous cystadenoma, 915 U/mL (0-82,500 U/mL) in mucinous cystadenoma, and 149 U/mL (0-6,230 U/mL) in mature cystic teratoma. The M-CSF levels increased significantly from mature cystic teratoma to mucinous cystadenoma to serous cystadenoma. The serum M-CSF levels were 308 to 499 U/mL in patients with benign ovarian tumor. The M-CSF levels did not differ significantly among the three groups. The serum M-CSF levels were 162 U/mL (0-473 U/mL) in menopausal patients. CONCLUSIONS: Elevation of levels of M-CSF varies according to histologic type in benign ovarian tumors. This implies that the antitumor activities of M-CSF for serous cystadenoma, mucinous cystadenoma, and mature cystic teratoma differ by histologic type.     

Mucinous cystadenoma of the ascending colon: a novel presentation

Mucinous cystadenomas usually occur in the appendix, ovaries and pancreas. This is the first report to describe mucinous cystadenoma of the ascending colon. A 32-year-old female presented with abdominal pain. Radiographic studies demonstrated a space-occupying lesion in the ascending colon associated with intussusception. Right colectomy revealed a cystic mass within the ascending colon that contained thick mucin and was lined by mucin-producing columnar epithelium. Clinical and pathologic features with a brief review of the literature are presented.