Report of a case: Retroperitoneal mucinous cystadenocarcinoma with rapid progression

IntroductionRetroperitoneal mucinous cystic neoplasms are uncommon, and little is known about the etiology of the disease. Malignant forms of these are extremely rare. Here, we report a case of primary retroperitoneal mucinous cystadenocarcinoma (PRMC), which demonstrated unexpectedly aggressive progression despite finding only a limited area of adenocarcinoma.Presentation of caseA 62-year-old woman with a complaint of abdominal discomfort was admitted to the hospital. Abdominal CT and MRI showed multiple large retroperitoneal cysts dislocating the right kidney nearly to the center of the abdomen. Transabdominal resection of the cysts was performed. Those cysts contained 1100 ml of mucinous fluids in total. Cytological examination of those fluids revealed no malignant cells. The cyst wall was lined with mucinous epithelial cells, and contained some ovarian-type stroma. Also, there was a focal area of adenocarcinoma in the cyst wall, and the lesion was diagnosed as primary retroperitoneal mucinous cystadenocarcinoma. Eight months later, the patient developed lumbar bone metastasis. Chemotherapy with S-1, an oral fluoropyrimidine, and docetaxel had been begun immediately; however, the disease had rapidly spread in the retroperitoneum. Eventually, the patient died of the disease 15 months after surgery.DiscussionRetroperitoneal mucinous cystic neoplasms are considered to be metaplasia of embryonal coelomic epithelium. Complete excision without rupture is essential. However, variance of biological aggressiveness might exist in PRMCs.ConclusionRetroperitoneal mucinous cystadenocarcinoma is a rare tumor, and it is urgently necessary to elucidate the etiology of an effective therapy for the disease.     

Sclerosing polycystic adenosis of the oral cavity

Minor salivary glands are ubiquitous in the oral cavity, and related diseases are often indolent and asymptomatic. We describe the unusual features of sclerosing polycystic adenosis, and its similarities to more sinister conditions of the minor salivary glands. Its importance is currently uncertain and research points to a pathogenesis that is neoplastic, which can create ambiguity. Sclerosing polycystic adenosis is a newly-discovered condition that merits further discussion and research to evaluate its full impact.     

Serum and cystic fluid CA 19–9 determinations as a diagnostic help in liver cysts of uncertain nature

Abstract: The distinction between hepatobiliary cystadenoma or cystadenocarcinoma and simple hepatic cyst complicated by intracystic hemorrhage may prove difficult to determine on the sole basis of clinical and radiological features because of the presence of intracystic structures and septations well-demonstrated by ultrasound examination in both situations. We investigated four patients with various types of hepatic cysts, in whom diagnostic difficulties led to further investigations. In this small group, CA 19–9 serum levels were abnormal only in the two patients with cystadenoma or cystadenocarcinoma. Cystic fluid CA 19–9 values were also five times higher in cystadenoma and cystadenocarcinoma than in other benign lesions. Our data thus suggest that the determination of serum and cyst fluid CA 19–9 may be of help in distinguishing between hemorrhagic simple cyst and cystadenoma or cystadenocarcinoma.     

Left trisegmentectomy and combined resection of the inferior vena cava, without reconstruction, for giant cystadenocarcinoma of the liver

A 54-year-old woman with giant liver cystadenocarcinoma underwent left trisegmentectomy with combined resection of the inferior vena cava (IVC) and the right hepatic vein. As a result, only the right inferior hepatic vein was preserved as a drainage vein. Because the perivertebral plexus and the azygos vein were both well developed, neither veno-venous bypass nor IVC reconstruction was performed. The developed collateral veins acted as the venous drainage pathway to maintain a stable systemic circulation. On the seventh postoperative day, portal vein flow dramatically decreased and the patient tended to liver failure. Prostaglandin E₁ (PGE₁) was administrated via the superior mesenteric artery. The portal flow then gradually increased and liver failure was avoided. Six months after the operation, she was re-admitted due to obstructive jaundice and presented with complete stenosis of the common bile duct (CBD). The jaundice persisted and liver dysfunction progressed. The patient died seven months after the operation. The confluence of the right inferior vein and the IVC could have been deformed, causing outflow blockade. The intrinsic shunt was not good enough to act as the drainage pathway, and IVC reconstruction may have been needed.     

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.     

Overview Article: Ultrastructural Features of the Common Epithelial Tumors of the Ovary: Part II

The ultrastructural features of benign and malignant serous, mucinous, and endometrial variants of ovarian carcinoma are presented. Clear cell tumors and Brenner tumors are also discussed.

Where possible, specific electron microscopic features are stressed.     

Spontaneous rupture of a hepatic cystadenoma and cystadenocarcinoma: report of two cases

Hepatobiliary cystadenomas and cystadenocarcinomas are rare tumors. Differentiating between these tumors and benign hepatic cysts may be difficult. Because of their rarity, diagnosis is often delayed and may result in inaccurate treatment, resulting in unnecessary morbidity and mortality. The purpose of this report is to draw attention to these entities and their complications. We report on two cases with spontaneous rupture of hepatobiliary cystadenoma and cystadenocarcinoma cysts, initially treated as simple hepatic cysts by aspiration, or by aspiration combined with sclerotherapy. The spontaneous rupture of the cysts appeared years after the initial treatment of the cysts, leading in one case to a prolonged stay in an intensive care unit. In both cases, a formal liver resection was carried out and microscopic investigations revealed a mucinous cystadenocarcinoma and cystadenoma. In conclusion, although hepatobiliary cystadenomas and cystadenocarcinomas are rare findings, they should not be forgotten in the diagnostic workshop when examining patients with hepatic cysts. If hepatobiliary cystadenomas and cystadenocarcinomas cannot be excluded following radiological imaging, surgery is recommended.     

A case of Krukenberg carcinoma metastasized from colon cancer resembling mucinous cystadenocarcinoma of the ovary

We report a case of a 44-year-old woman with bilateral ovarian carcinoma that had metastasized from the colon and mimicked primary mucinous cystadenocarcinoma. Macroscopically, both ovarian tumors were large, multiloculated cystic masses with abundant mucinous content. Histologically, they were lined with mucinous epithelium with mild to moderate nuclear atypia and showed stromal invasion and surface involvement. At first, the tumors were diagnosed as bilateral primary ovarian mucinous cystadenocarcinomas. However, three months after surgery, a large villous tumor was discovered in the ascending colon by colonoscopic examination and was surgically resected. Histologically, the colonic tumor was a villous adenomatous tumor with invasive components of mucinous adenocarcinoma composed of well-differentiated adenocarcinoma and exhibited abundant extracellular mucin production. As a villous adenomatous component was present in the mucosal area, the colonic tumor was considered a primary tumor. Therefore, the original diagnosis of bilateral ovarian tumors was revised for consistent with metastasis from the colon carcinoma, in line with the findings of immunohistochemistry and loss of heterozygosity analysis. This case highlights the importance of considering the possibility of metastatic tumors from the gastrointestinal tract in the diagnosis of mucinous ovarian tumors.     

Central papillary cystadenocarcinoma of the mandible: A case report and review of the literature

INTRODUCTION

Central papillary cystadenocarcinoma of the jaw is an extremely rare tumor with only three previously reported cases in the English literature. This tumor is a histologically low-grade cancer, affecting more commonly in the mandible than in the maxilla.

PRESENTATION OF CASE

A 65-year-old woman presented with a two months history of a rapidly growing, painless mass of the right ascending ramus of the mandible. The pathologic report from incisional biopsy was a papillary cystic tumor with a differential diagnosis of cystadenoma versus cystadenocarcinoma. Segmental mandibulectomy, parotidectomy and submandibular gland resection were performed. The final pathology was intraosseous papillary cystadenocarcinoma.

DISCUSSION

Clinical features of central papillary cystadenocarcinoma of the mandible mimic an odontogenic lesion and metastatic bone disease, careful review of radiograph and pathology should be done. Surgical excision with wide margins is the appropriate treatment. Postoperative radiation therapy should be considered in histologically aggressive or high-stage tumor.

CONCLUSION

This is the fourth case of central papillary cystadenocarcinoma of the mandible in the English literature. Although it is usually a low-grade cancer, en bloc resection with adjuvant postoperative radiotherapy in a high-stage disease, and long-term follow-up allow the patient to have a favorable prognosis.     

nm23H1、E-Cadherin和CD44基因产物在卵巢浆液性囊腺癌中的表达

目的：探讨肿瘤转移抑制基因nm23H1和黏附分子E－Cadherin以及CD44在卵巢浆液性囊腺癌中的表达，以及这种表达与临床指标、生物学行为之间的关系。方法：用免疫组化S－P法检测nm23H1、E－Cadherin和CD44基因蛋白在肿瘤组织中的表达。结果：肿瘤原发灶和相应淋巴结中，nm23H1、E－Cadherin和CD44蛋白表达与淋巴结转移、临床分期、组织学分期有一定关系。结论：nm23H1、E-Cadherin和CD44基因在卵巢浆液性囊腺癌的发展转移中起重要作用，对这三种基因的共同检测具有一定临床价值。