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Cystadenocarcinoma (papillary cystadenocarcinoma) of the submandibular gland.

Cystadenocarcinoma (papillary cystadenocarcinoma) of the salivary gland is a rare malignant neoplasm. Major locations of this neoplasm are the parotid gland, the sublingual gland, and minor salivary glands, while occurrence in the submandibular gland is extremely rare. As far as we know, only one report, written in the French language, has been published concerning cystadenocarcinoma of the submandibular gland, but no report is available in the English language. In this report, we describe a case of cystadenocarcinoma arising from the submandibular gland of a 54-year-old male patient. The neoplasm is a low-grade carcinoma, and pre-operative examination may not show typical characteristics of malignant neoplasms. Therefore, as was true in this case, the differential diagnosis from benign lesions is sometimes difficult. This is the first report on cystadenocarcinoma of the submandibular gland in the English language and the first to show a computed tomography (CT) scan and magnetic resonance imaging (MRI) of this neoplasm in the submandibular gland.

Cystadenocarcinoma, Papillary↗

Pancreatic cystadenoma and cystadenocarcinoma.

Cystadenomas and cystadenocarcinomas are rare but not exceptional tumours of the pancreas. In this report six cases are added to the more than 300 cystadenomas and over 100 cystadenocarcinomas reported in the literature. We present here the case histories of four cystadenomas and two cystadenocarcinomas with complete follow-up. There is no evidence of recurrence after a maximum of 22 years in cystadenoma and 4 years in cystadenocarcinoma following resection. We have the impression that cystic lesions of the pancreas are nowadays more often detected than in former years. This might be due to abdominal investigation by computerized tomography and ultrasound on a large scale. Pathological features of proliferative cystic lesions of the pancreas permit distinction of three different tumours: serous cystadenoma, mucinous cystadenoma and cystadenocarcinoma. The serous cystadenoma is benign. The mucinous cystadenoma has to be considered as potentially malignant. The cystadenocarcinoma is malignant by definition. As benign and malignant elements can be found in the same cyst biopsy cannot exclude cystadenocarcinoma. Therefore all cystadenomas and cystadenocarcinomas should be totally excised, especially as prognosis after resection of cystadenocarcinoma is much better than after resection of adenocarcinoma of the pancreas.

Adult↗

[Biliary cystic neoplasm: biliary cystadenoma and biliary cystadenocarcinoma].

Biliary cystic tumors, such as cystadenoma and cystadenocarcinoma, are rare cystic tumors of liver accounting for fewer than 5% of all intrahepatic cysts of biliary origin. Most biliary cystic tumors arise from intrahepatic bile duct and 10-20% arise from extrahepatic bile duct like common hepatic duct, common bile duct, and gallbladder. The first case report of biliary cystic neoplasm in Korea dated back to 1975 by Bae et al, and over 40 cases of cystadenoma and 35 cases of cystadenocarcinoma were reported since then. These tumors usually present in middle-aged women with a mean age of 50 years. Biliary cystadenomas are lined by single layer of cuboidal or columnar epithelium and are very often multilocular with septal or papillary foldings. Over 80% of cystadenoma have dense mesenchymal stroma composed of dense spindle cells, like ovary. The epithelial lining of cystadenocarcinoma exhibits cellular atypia, mitotic activity, and infiltrative growth, but part of lining epithelium retain the feature of cystadenoma, which support the adenoma-carcinoma sequence. The size of tumors varies from 1.5 to 35 cm. Many patients are asymptomatic, except for the presence of palpable mass. When symptoms are present, they include epigastric or right upper quadrant pain or jaundice by enlarged mass. Biliary cystic tumor should be considered when a single or multilocular cystic lesion with papillary infoldings is detected in the liver by computed tomogram (CT) or ultrasound (US). Cystic wall and internal foldings can be seen enhanced by enhanced CT. US reveals a hypoechoic cystic mass with echogenic septation or papillary infoldings. Cystadenocarcinoma should be suspected when there is elevated mass or nodule in the wall or foldings, or thickened cystic wall on CT or US. But it is extremely difficult to differentiate between cystadenoma and cystadenocarcinoma by imaging alone. Increased tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, in serum or cystic fluid have been reported in biliary cystic tumor. But tumor markers cannot distinguish cystadenocarcinoma from cystadenoma or both from other cystic lesions of liver. Malignant cells are not usually recovered in patients with cystadenocarcinoma who underwent cystic fluid cytology before and during surgery. The treatment of choice is radical excision of the mass by means of lobectomy or wide tumor excision. Aspiration, marsupialization, and drainage must be avoided. Inadequate excision of both cystadenoma and cystadenocarcinoma may lead to recurrence. Prognosis after complete excision is excellent.

Adult↗

Detection of tumor-specific antigens in human mucinous cystadenocarcinoma of the ovary by immunodiffusion.

Rabbit antiserum to a tissue extract of human mucinous cystadenocarcinoma of the ovary reacted with tissue extracts of normal ovary and various ovarian malignancies, and ascitic or cystic fluids of ovarian origin by Ouchterlony double gel diffusion and precipitin inhibition techniques. The tumor-associated antigen(s) of mucinous cystadenocarcinoma, which were demonstrated by Ouchterlony double diffusion, were not present in tissue extract of pooled normal ovaries and cystic fluid of benigh tubo-ovarian cyst. An organ-associated tumor antigen as well as the type-specific tumor antigen may exist in mucinous cystadenocarcinoma of the ovary. The mucinous cystadenocarcinoma was not very immunologically different but was distinguishable from serous cystadenocarcinoma and other types of ovarian cancer by double gel diffusion. Precipitin-inhibition reactions demonstated that the adsorbed antiserum to human ovarian mucinous cystadenocarcinoma mixed with tissue extracts of dysgerminoma and serous cystadenocarcinoma, and ascitic fluid of papillary embryonal adenocarcinoma of the ovary could not eliminate the specific precipin line developed with tissue extract of mucinous cystadenocarcinoma.

Antigens, Neoplasm↗

Endocrine cells in hepatobiliary cystadenomas and cystadenocarcinomas.

We investigated the distribution of endocrine cells in hepatobiliary cystadenoma (n = 5, two associated with mesenchymal stroma) and cystadenocarcinoma (n = 3) immunohistochemically. In normal livers (n = 20) and livers affected by hepatolithiasis (n = 15) used as controls, endocrine cells revealed by chromogranin immunostaining were located exclusively in normal or proliferating intrahepatic peribiliary glands. In the eight cases of hepatobiliary cystadenoma and cystadenocarcinoma, endocrine cells were present in four cases (50%) (1 cystadenoma, 1 cystadenoma with mesenchymal stroma, and 2 cystadenocarcinomas). Endocrine cells tended to be located beneath and among the columnar epithelial cells. Intrahepatic peribiliary glands were located in the vicinity of cystadenoma or cystadenocarcinoma in six (75%) of the eight cases, and they frequently showed cystic dilatation and contained endocrine cells. Intrahepatic peribiliary glands were located in the vicinity of the endocrine cells in all cystadenomas and cystadenocarcinomas that were positive for endocrine cells. These data show that about 50% of hepatobiliary cystadenomas and cystadenocarcinomas contain endocrine cells and suggest that hepatobiliary cystadenoma and cystadenocarcinoma may originate from intrahepatic peribiliary glands.

Aged↗

Mucinous cystadenocarcinoma of the parotid gland: report of a case with fine needle aspiration findings and histologic correlation.

BACKGROUND: Mucinous cystadenocarcinoma of the salivary gland is a rare entity. Review of the literature from 1991 to 1999 revealed no previous reports on its cytologic features. CASE: A 25-year-old man had a slowly growing, painless mass in the left parotid gland. Fine needle aspiration biopsy, performed prior to surgical excision, showed clusters of minimally atypical epithelial cells in which occasional vacuolated cells containing mucin could be seen. Pathologic evaluation of the resected parotid mass showed it to be a mucinous cystadenocarcinoma. CONCLUSION: The cytologic differential diagnosis of mucinous cystadenocarcinoma is with low grade mucoepidermoid carcinoma and with mucinous adenocarcinoma. Mucinous cystadenocarcinoma must be cystic; cysts may be present in low grade mucoepidermoid carcinoma, but their size and prominence varies. Mucinous adenocarcinoma is not cystic but gelatinous. Nuclei are bland in both mucinous cystadenocarcinoma and low grade mucoepidermoid carcinoma but are atypical in mucinous adenocarcinoma. There is no squamous differentiation in either mucinous cystadenocarcinoma or mucinous adenocarcinoma, but it is subtle in low grade mucoepidermoid carcinomas. Mucinous cystadenocarcinoma should be considered a potential candidate in the differential diagnosis of mucinous lesions that can occur in the salivary gland.

Adult↗

Glycosphingolipids of various human ovarian tumors: a significantly high expression of I3SO3GalCer and Lewis antigen in mucinous cystadenocarcinoma.

Among several human ovarian tumors, which include mucinous cystadenocarcinoma, serous cystadenocarcinoma, and clear cell adenocarcinoma, the mucinous cystadenocarcinoma showed a unique glycosphingolipid composition. In particular, more than 90% of the acidic glycosphingolipids in the mucinous cystadenocarcinoma is comprised of sulfolipids, which are hardly detected in normal ovary and are contained in concentrations of less than 40% in the other type of ovarian tumors. By means of negative ion fast atom bombardment mass spectrometry and gas liquid chromatography, the major sulfolipid in mucinous cystadenocarcinoma is confirmed to be I3SO3-GalCer with N-cerebronoyl phytosphingosine, that which contrasts with I3SO3-GalCer with N-nonhydroxy fatty acyl sphingosine as the major molecular species in the other ovarian cancers. In mucinous cystadenocarcinoma, galactosylceramide is found in the relatively high concentration and is also composed of N-cerebronoyl phytosphingosine. In addition, the concentrations of glycolipids with Le(a) and Le(b) antigenicities are significantly higher in mucinous cystadenocarcinoma than those in normal ovary and the other ovarian tumors.

Chromatography, Thin Layer↗

Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients.

Fifty-two hepatobiliary cystadenomas and 18 hepatobiliary cystadenocarcinomas were drawn from the files of the Armed Forces Institute of Pathology and Rhode Island Hospital and studied in an attempt to correlate light microscopic features of the tumors with immunohistochemical and follow-up data. The cystadenoma patients ranged in age from 2 to 87 years at the time of initial diagnosis (mean, 45 years). All the cystadenomas were multilocular with benign cuboidal to columnar epithelium, and 44 (85%) had densely cellular spindle cell ("ovarian-like") stromata; 96% were female. Fifty-one cystadenomas were macrocystic lesions, typically lined by mucinous epithelium; one of the benign lesions was a serous cystadenoma (microcystic adenoma) reminiscent of the more commonly encountered pancreatic lesion of the same name. The cystadenocarcinoma patients ranged in age from 24 to 90 years at the time of first diagnosis (mean, 59 years); eight patients (44%) were male. All but one of the lesions were multilocular with malignant in situ (one case) or invasive tubulopapillary (15 cases), solid (one case), or adenosquamous (one case) epithelial components. Areas of preexisting benign cystadenoma were found in six (33%), an observation suggesting that benign lesions may evolve into malignant ones in some patients. Most cystadenomas and cystadenocarcinomas arose in the liver, a few in the extrahepatic biliary system (including the gallbladder). On follow-up, the cystadenoma patients in general were successfully treated by surgical excision of the lesions in toto; patients treated by subtotal resection often had persistent symptomatic disease. Four cystadenocarcinoma patients died of their tumors; another two patients were alive with persistent disease at last follow-up. In both the benign and the malignant lesions, most tumor cells were positive on immunohistochemical staining with antibodies to cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen; scattered chromogranin-positive cells also appeared in a few tumors of both types. Immunohistochemistry did not yield a diagnostic immunoprofile to distinguish cystadenoma from cystadenocarcinoma or from other epithelial lesions arising within the abdominal cavity. At least two types of cystadenocarcinoma exist, one developing exclusively in female patients, usually accompanied by an "ovarian-like" stroma, which follows an indolent course; and the other, lacking the distinctive cellular stroma, seen in males, follows a more aggressive course and is more likely to result in the patient's death from tumor. It remains an open question whether the cystadenocarcinomas lacking a mesenchymal stroma, which arise in women, will follow the same aggressive course as similar lesions arising in men.

Adolescent↗

A case of biliary cystadenocarcinoma of the liver.

We describe a case of biliary cystadenocarcinoma of the liver, a very rare malignant tumor, in a 40-year-old man referred to our hospital because of sporadic abdominal pain. A left lobectomy of the liver was performed, and macroscopic examination of the resected specimen revealed prominent papillary projections in a multiloculated cystic tumor containing mucinous material. Histologic examination of the specimen showed a biliary cystadenocarcinoma without mesenchymal stroma, associated with a cystadenoma. Cystadenoma with mesenchymal stroma (CMS), which occurs exclusively in women, is generally regarded as a precancerous lesion, and patients with biliary cystadenocarcinoma with mesenchymal stroma have a good prognosis. Cystadenoma without mesenchymal stroma arises in both men and women. However, the origin and precancerous lesions of cystadenocarcinoma are unknown. Moreover, the prognosis of patients with biliary cystadenocarcinoma without mesenchymal stroma is poor, especially in men. The cystadenoma without mesenchymal stroma in our patient was considered a precancerous lesion. The present patient has shown no evidence of recurrence in the 8 years after hepatic resection. Previously reported cases of cystadenocarcinoma without mesenchymal stroma, especially those in men, have had a poor outcome. A good outcome in men is very rare.

Adult↗

[A quantitative study on nuclear DNA content and morphological parameters in ovarian cystadenocarcinoma].

OBJECTIVE: To study nuclear DNA content and morphological parameters in ovarian cystadenocarcinoma. METHODS: Nuclear DNA content and morphological parameters were quantitatively determined by image analysis technology in 56 cases of ovarian cystadenocarcinoma, 12 benign ovarian cystadenoma and 10 normal ovarian epithelial tissue. RESULTS: DNA content of ovarian cystadenocarcinoma was the highest among the three groups; and there were significant differences between the groups (P < 0.01). In the aspect of morphometry, nuclear circle was greatly different among the three groups (P < 0.01). There was no significant variances in nuclear parameter and area (P > 0.05). Nuclear DNA content of ovarian cystadenocarcinoma was related to its histopathological classification, histological grade and clinical stages (P < 0.01, P < 0.05). There was a reverse relationship between the DNA ploidy level and clinical prognosis. The 2-year survival rate of ovarian cystadenocarcinoma with diploidy/near diploidy pattern was higher than that with aneuploid pattern (P < 0.01). CONCLUSIONS: Nuclear DNA content of ovarian cystadenocarcinoma is a useful marker in reflecting the biological behavior and is of practical value in diagnosis, the choice of treatment methods and in the evaluation of results and prognosis.

Adolescent↗

Biliary cystadenoma and cystadenocarcinoma: report of 14 cases and review of the literature.

The clinical and pathologic features and long-term follow-up of eight patients with biliary cystadenoma and six patients with biliary cystadenocarcinoma are reported and the previous literature is reviewed. All the cystadenomas were in middle-aged women, but the six cystadenocarcinomas occurred in both male (4) and female (2) patients. The majority of the patients with cystadenoma and half of those with cystadenocarcinoma presented with an abdominal mass. Four of the patients whose cystadenoma was excised are alive and well for periods of time ranging from 2 1/2 to 13 years. Two of the patients with cystadenocarcinoma have survived for three years and for three years and eight months, respectively, after subtotal hepatic lobectomy. Morphologically the cystadenocarcinomas differ from the cystadenomas in that the former have cellular pleomorphism and anaplasia and infiltration of the underlying fribrous stroma; they can invade adjacent viscera and may occasionally metastasize to distant sites. The presence of benign epithelium in most cystadenocarcinomas supports their origin from cystadenoma.

Adult↗

Cystadenoma and cystadenocarcinoma of the liver: a single center experience.

BACKGROUND: Biliary cystadenomas and cystadenocarcinomas comprise 5% of cystic lesions in the liver. Cystadenomas are often incorrectly diagnosed as simple cysts, which results in inadequate therapy. Recurrence and possible malignant transformation are consequences of incomplete excision. Cystadenocarcinomas are very rare tumors that are felt to be biologically indolent. STUDY DESIGN: A retrospective review of 18 cystadenomas and 4 cystadenocarcinomas treated at the Cleveland Clinic from July 1985 to November 2002. RESULTS: All 18 patients with cystadenomas were women; mean age was 48 years. The majority (16 of 18) were symptomatic. Preoperative CT scans demonstrated cyst(s) with septations in all patients. Fifty-five percent had undergone prior intervention(s) to treat the cyst. Thirteen patients had complete excision of the cystadenoma, either by enucleation or liver resection. None of the patients developed recurrent cystadenomas (mean followup 37 months). Of 4 patients with cystadenocarcinoma, 3 were women; mean age was 60 years. All were symptomatic. Preoperative CT scans demonstrated masses with both cystic and solid components. No patient had undergone prior intervention. All had a liver resection. Two patients died of metastatic disease at 6 and 12 months, respectively. One patient is alive and disease-free at 16 years; 1 is alive with metastatic disease 10 years after the liver resection. CONCLUSIONS: Cystadenomas are uncommon tumors that are often incorrectly diagnosed as simple cysts. Preoperative imaging that demonstrates the presence of internal septations highly suggests the diagnosis of cystadenoma. Intraoperative biopsy and frozen section(s) are essential, although they are not 100% accurate. Cystadenomas require complete excision to prevent recurrence and the possibility of malignant transformation. Cystadenocarcinomas are very rare. Despite complete resection, cystadenocarcinomas can recur in a short period of time. The biologic behavior of these tumors can vary widely.

Adult↗

Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings.

Biliary cystadenomas and cystadenocarcinomas are rare cystic neoplasms, usually intrahepatic in location, that are characterized pathologically by a multilocular appearance. We report the CT and sonographic findings in eight cases of biliary cystadenoma and three cases of biliary cystadenocarcinoma and correlate them with the surgical and pathologic findings. CT showed internal septa in eight of the 10 multiloculated lesions, whereas sonography showed septa in all five cases in which it was used. CT in two of the three cystadenocarcinomas showed thick and coarse mural and septal calcifications as well as large solid soft-tissue masses in one. CT showed mural soft-tissue nodules in the single case of a unilocular cystadenocarcinoma. Sonography and CT usually show the multilocular nature of biliary cystadenomas and cystadenocarcinomas. The presence of mural or septal nodules, discrete soft-tissue masses, and possibly thick and coarse calcifications increase the likelihood of a cystadenocarcinoma.

Adult↗

Clinical features and imaging diagnosis of biliary cystadenocarcinoma of the liver.

Biliary cystadenocarcinoma of the liver is a relatively rare disease. Herein, we reported a case of biliary cystadenocarcinoma with a review of the literature. A 71-year-old female was admitted with the chief complaint of epigastralgia. The imaging studies revealed a biliary cystadenocarcinoma in the left hepatic lobe with suspicion of direct invasion to the left and middle hepatic veins and inferior vena cava. However, there was no direct invasion of the tumor to these veins in operation findings, and an extended left hepatic resection was performed without resection of inferior vena cava. The tumor was histologically diagnosed as biliary cystadenocarcinoma of the liver. Diagnosis of biliary cystadenocarcinoma is usually difficult preoperatively, however, a diagnosis was possible with the use of imaging studies. It was suggested that this tumor originated from a benign cystadenoma because of the existence of a transitional zone between normal cells and atypical cells in the cystic wall. Systematic hepatectomy was recommended as the initial treatment in consideration of the features of cystadenocarcinoma.

Aged↗

Mucinous cystadenocarcinoma of the appendix. The controversy persists: a review.

Mucinous cystic neoplasms of the appendix continue to engender considerable debate in their diagnosis, management, and role in pseudomyxoma peritonei. This review addresses ongoing controversies with these unique neoplasms. Case reports and institutional series of mucinous cystadenocarcinoma were retrieved from the literature using Medline Search (1985-1996) using the following key words: appendix; mucinous cystadenocarcinoma; pseudomyxoma peritonei. 284 patients with mucinous cystadenocarcinoma were identified. Abdominal pain was the most common presenting symptom and objective preoperative diagnosis was difficult. There were 22 patients with concomitant appendiceal and ovarian mucinous neoplasms. Sixty-seven patients presented with mucinous cystadenocarcinoma and pseudomyxoma peritonei. However, a unified definition of pseudomyxoma peritonei was lacking. The recurrence rate was higher when an appendiceal neoplasm was associated with pseudomyxoma peritonei. In the reported literature, about an equal number of patients were subjected to appendectomy alone or right hemicolectomy. However, there was a survival advantage for right hemicolectomy versus appendectomy alone. Management of disseminated pseudomyxoma peritonei remains controversial. Mucinous cystadenocarcinoma appears to be a spectrum of low-grade malignant tumors. For correct diagnosis, a high index of suspicion must be maintained. The appropriate treatment for a patient with mucinous cystadenocarcinoma should be a right hemicolectomy. Aggressive debulking may improve survival when associated with pseudomyxoma peritonei. A better definition of pseudomyxoma peritonei is needed. In evaluation of different treatment modalities and prognosis, patients should be grouped according to underlying primary pathology.

Adult↗

Cystadenocarcinoma of the liver: a case report.

BACKGROUND: Biliary cystadenocarcinoma as a very rare hepatic neoplasm may be difficult to distinguish from simple hepatic cysts, especially in the unilocular form. Although diagnosis of cystadenocarcinoma during open hepatic surgery demands a complete surgical resection, few reports described the correct approach to such lesions revealed by laparoscopic approach. Five cases of incidental cystadenocarcinoma after laparoscopic surgery for hepatic cystic lesions were presented with discussion of diagnostic and management implications. METHODS: Clinical records of 5 patients who had undergone operation and histological examination at our hospital from 1993 to 2002 were analyzed retrospectively. RESULTS: In all patients who received liver lobectomy, primary hepatic cystadenocarcinoma was diagnosed. Primary biliary cystic neoplasm was difficult to diagnose and the management of both benign and malignant tumors was similar in the 5 patients. CONCLUSIONS: Cystadenocarcinoma is often difficult to diagnose because its clinical manifestations are similar to those of hepatic cysts and other cystic lesions. Imagining examination is helpful in evaluating the disease. Complete excision of cystadenocarcinoma with a wide margin is the best treatment.

Cystadenocarcinoma↗

Cystadenoma and cystadenocarcinoma of the liver: localization of carcinoembryonic antigen.

Cystadenomas and cystadenocarcinomas of the liver are rare tumors. The distribution of carcinoembryonic antigen (CEA) in two cystadenomas and a cystadenocarcinoma was examined immunohistochemically by the peroxidase-labeled antibody method, at the light microscopic level. In the cystadenomas and areas consisting of benign-appearing cells in the cystadenocarcinoma, CEA was localized to the luminal surfaces of the glandular cells appearing as thin linear stains. In malignant epithelial cells forming nests and daughter cysts of the cystadenocarcinoma, CEA was demonstrated throughout the cytoplasm, in a diffuse pattern. Thus, the immunohistochemical localization of CEA may be helpful in the diagnosis of borderline lesions and in determining the distribution of benign and malignant epithelium in cystadenocarcinomas. The etiology, clinical features, pathology and treatment of these rare tumors are discussed.

Adult↗