Pseudomyxoma peritonei and malignant mucocele of the appendix. A case report

A case of pseudomyxoma peritonei originating in a malignant mucocele of the appendix, a very rare clinical entity, is presented. The cytologic findings in the gelatinous ascitic fluid of epithelial clusters with minimal nuclear atypia suggested a diagnosis of pseudomyxoma peritonei. Both routine stains and special stains for mucus assisted in making the cytologic diagnosis. Histologic examination of the laparotomy specimen confirmed the presence of a well-differentiated mucinous cystadenocarcinoma, with an apparent appendiceal origin. The origins and relationships of appendiceal mucocele and pseudomyxoma peritonei are discussed.     

Cytology of pseudomyxoma peritonei

Cytology of pseudomyxoma peritonei
The cytology of five cases of pseudomyxoma peritonei is described. It is characterized by large masses of mucinous material, some of which is wrapped up in a network of elongated fibroblast-like cells. Irregular clusters of flat branching vimentin-positive fibroblast-like cells may be found as the disease progresses. Goblet or tumour cells were not found in the cytology preparations, although they were seen in the histological sections of the one case which was biopsied. The appendiceal origin of pseudomyxoma peritonei could not be traced in three of four male patients, and the cause of the disease remained undetermined. Autopsy of the fourth case demonstrated a complete replacement of the appendix by mucus.     

Pseudomyxoma extraperitonei occurring 35 years after appendicectomy: a case report and review of literature

Background

Pseudomyxoma peritonei is a rare condition consisting of mucinous ascites, most commonly arising from mucinous tumors of the appendix and occasionally from the ovary. Very rarely mucinous implants arise in the retroperitoneum without any intra-peritoneal involvement. This has been termed as pseudomyxoma extraperitonei.

Case presentation

We report a case of a 57 year old man who developed pseudomyxoma extraperitonei, 35 years after undergoing an appendicectomy for a perforated appendix.

Conclusions

Pseudomyxoma extraperitonei has been previously reported, however we report the longest incubation period of 35 years for this condition.

     

Argyrophilia in ovarian serous tumors. A comparative study in 127 epithelial ovarian tumors.

The distribution of argyrophil cells in epithelial ovarian tumors was studied in 127 cases. The results showed that not only mucinous tumors and endometrioid tumors contained argyrophil cells, but also some serous tumors expressed argyrophilia. 31% of serous tumors including 40% of serous adenocarcinomas contained variable numbers of argyrophil cells. Argyrophilia has been demonstrated in mucinous tumors, endometrioid tumors and Brenner tumors before. However, this is the first time the presence of argyrophilia in serous tumors has been noticed. Moreover, the argyrophil cells in 5 serous carcinomas showed reactivity with Neuroendocrine (chromogranin A) antibody but not with serotonin. The expression pattern of argyrophilia in the serous tumors was different from that of the mucinous tumors; in the former, argyrophil granules appeared in apical portions or throughout the cytoplasm of single or clustered cells. In addition, the argyrophilia in some serous tumors and endometrioid tumors decreased after diastase digestion. Ultrastructurally, no typical neurosecretory granule was found in the argyrophilic serous tumors. The findings in this study suggest that argyrophilia could be quite frequently found in ovarian epithelial tumors and in itself is not a very specific differential characteristic of carcinoid tumors. The argyrophilia found in a variety of epithelial ovarian tumors might lend additional support to the histogenesis and close relationship between the common epithelial tumors of the ovary.     

Identification of glycoproteins associated with different histological subtypes of ovarian tumors using quantitative glycoproteomics

Ovarian cancer is the most lethal gynecologic malignancy in adult women. The origin of epithelial ovarian tumors is both morphologically and biologically heterogeneous, and different subtypes of ovarian tumors have different clinical outcomes. In spite of the heterogeneous nature of ovarian carcinoma, the current biomarkers and treatments for this disease are not subtype-specific. To discover the molecular basis of the ovarian tumor subtypes, we analyzed extracellular glycoproteins of seven common subtypes and normal ovary tissues using quantitative glycoproteomic analysis. Glycoproteins for different ovarian tumor subtypes were identified by liquid chromatography-tandem mass spectrometry and quantitated by spectral counting and then verified by iTRAQ labeling and Western blotting. Glycoproteins uniquely expressed in different subtypes of ovarian tumors or commonly expressed in most subtypes were identified. Using Western blots, we verified that mesothelin was overexpressed in serous carcinoma and transitional-cell carcinoma, CEA5 and CEA6 were overexpressed only in mucinous carcinoma, while versican and periostin were overexpressed in most subtypes of ovarian tumors. This study presents the first proteomic characterization of different ovarian tumor subtypes. The identified glycoproteins for histological subtypes of ovarian tumors will facilitate the understanding of the molecular basis, diagnosis of ovarian tumor subtypes, and predictions for treatment responses to therapeutic agents.     

Nuclear morphometry and AgNOR quantification: computerized image analysis on ovarian mucinous tumor imprints

OBJECTIVE: To determine the morphometric characteristics of nuclei and silver-stained nucleolar organizer regions (AgNORs) on cytologic imprints and their value in differential cytodiagnosis of benign, atypical proliferative (borderline) and malignant ovarian mucinous tumors. STUDY DESIGN: Forty-six mucinous ovarian tumor imprints (16 benign, 15 borderline, 15 malignant), were analyzed. Nuclear area, outline, "shape factor" and "form factor" were measured on Papanicolaou-stained smears. AgNOR quantification included 7 variables related to the number and area of single, cluster, total and relative AgNOR content per nucleus and the size distribution of AgNORs. RESULTS: Nuclear area and shape factor allowed distinguishing borderline and malignant tumors. The nuclear area in benign tumors was larger than that in borderline tumors; malignant tumors had the highest values. Single and cluster AgNORs were statistically significantly different in borderline tumors compared with malignant tumors, except for the cluster AgNOR area. The total AgNOR area, number and relative area increased from benign through malignant tumors, with statistically significant differences among all groups. By AgNOR size distribution, small AgNORs discriminate malignant from borderline and benign tumors. CONCLUSION: Combining nuclear morphometry and AgNOR analysis on cytologic imprints could be a diagnostically useful method in the assessment of mucinous ovarian tumors.     

Hyperthermic intraperitoneal chemotherapy (HIPEC) and cytoreductive surgery (CS) as treatment of peritoneal carcinomatosis: preliminary results in Croatia

The purpose of our study was to evaluate initial results following introduction of Hyperthermic Intraperitoneal Chemotherapy (HIPEC) and Cytoreductive Surgery (CS). Twenty two patients with intraperitoneal malignancy undergone cytoreductive surgery (CS) and hyperthermic intraoperative chemotherapy (HIPEC) between January of 2007 and January 2010. Nine patients had adenocarcinoma of colorectal origin, 8 patients had ovarian cancer, and 5 had pseudomyxoma peritonei. Inclusion criteria were diagnosis of peritoneal carcinomatosis based on intraoperative assessment during first operative procedure for intraabdominal malignancy or follow-up diagnostic imaging proof Excluded were patients with known malignant proliferation outside abdomen, liver metastasis and ASA score 4 and higher. All patients with pseudomyxoma peritonei diagnosis are alive, with mean follow-up time 24.8 months (range 15-35). In group of patients with adenocarcinoma from colorectal origin, 3 died, resulting in mean survival time 7.6 months (range 1-16). In group of patients with ovarian cancer, 2 died, resulting in mean survival time 13.8 months (range 0-31). Two patients died in early postoperative period. Most of the patients had some sort of mental disorder. Although HIPEC with CS improves survival, during introduction period higher morbidity and mortality could be expected.     

Fine needle aspiration cytologic findings of breast mucinous neoplasms: differential diagnosis between mucocelelike tumor and mucinous carcinoma

OBJECTIVE: To report the cytologic findings of mucocelelike tumor and mucinous carcinoma. STUDY DESIGN: All mucinous neoplasms diagnosed by fine needle aspiration and confirmed by histologic examination were reviewed to detect the cytologic findings helpful for the differentiation. The cytologic findings were correlated with the histologic findings. RESULTS: Cytologically, mucinous carcinomas were highly cellular and showed many single epithelial cells and variably formed epithelial cell clusters in abundant extracellular mucin. Malignant cells exhibited round, atypical nuclei; granular chromatin; and small nucleoli. Mucocele like tumor showed low cellularity with scanty, monolayered, small sheets of epithelial cells and abundant, extracellular mucin. Myoepithelial cells were present within the epithelial cell sheets. Tumor cells were usually small, with uniform, round nuclei; fine chromatin; and absence of nucleoli. CONCLUSION: Cytologic findings of mucocelelike tumor and mucinous carcinoma were different in cellularity, shape of cell clusters and nuclear features, although mucocelelike tumors having a carcinoma component were similar to mucinous carcinoma. Awareness of the cytologic findings of breast mucinous neoplasms is important to make a specific diagnosis.     

Cytologic findings in infiltrating micropapillary carcinoma and mucinous carcinomas with micropapillary pattern

OBJECTIVE: To examine the observation that some mucinous carcinomas have a micropapillary pattern and are mucinous variants of the highly angioinvasive infiltrating micropapillary carcinomas (IMPC). STUDY DESIGN: We evaluated cytologic findings of 13 IMPC and 55 mucinous carcinomas for comparative features. RESULTS: In mucinous carcinomas, 37 of 50 (74%) had a micropapillary pattern. This group included 27 cases with pure mucinous micropapillary morphology (MUMPC), 8 MUMPC associated with a ductal carcinoma of the IMPC type (MUIDC) and 2 cases of mixed mucinous carcinomas with an MUMPC and a solid variant ofpapillary carcinoma (SVPC) component. On cytology both IMPC and mucinous carcinomas with micropapillary pattern demonstrated the micropapillary pattern, that is, angulated clusters or abortive papillae and ball-like clusters. However, the IMPC smears revealed numerous singly scattered tumor cells and larger fragments with shrub-like branching and the MUMPC had psammoma bodies. The mixed MUMPC and SVPC showed the classic cytologic features of MUMPC admixed with abundant singly dispersed tumor cells in the background representing the SVPC component. CONCLUSION: Although IMPC and the MUMPC share the micropapillary pattern on histologic examination, mucin alters the appearances in aspirates. Recognition of this morphologic spectrum will help in understanding the behavior of these tumors.     

Peritoneal washings in ovarian tumors. Potential sources of error in cytologic diagnosis

Peritoneal washings were performed on 48 patients with suspected or known ovarian carcinoma. The procedure was part of the initial surgical staging in 27 patients with presumed stage I and II ovarian cancer and was performed during second-look operations in 21 other cases with proven ovarian malignancy. This paper presents the microscopic features of the washings, with particular emphasis on the cytologic differentiation between benign and malignant findings outside of the ovary. Thirty-four cases showed benign or reactive mesothelial cells and no evidence of peritoneal disease. The washings of six patient showed malignant cells, which were confirmed histologically. Notable atypia that mimicked ovarian carcinoma was found in eight patients who had benign or borderline lesions. These findings included papillary and glandlike epithelial structures, with varying degrees of cellular atypia and psammoma bodies. The histologic counterparts of these atypicalities were Müllerian inclusions, mesothelial proliferations and borderline serous tumors. The differential diagnosis between these entities is essential because false-positive cytologic diagnoses may alter postoperative treatment in some patients.     

Ascitic fluid cytology in a case of metastatic malignant mixed mesodermal tumor of the ovary

The cytologic features of the abdominal fluid from a patient with a malignant mixed mesodermal tumor (MMMT) of the ovary are presented. Both malignant epithelial and stromal elements were cytologically appreciated and confirmed by histologic examination. Other ovarian neoplasms that can present with malignant sarcomatous elements or mixed epithelial and sarcomatous elements are discussed; this case documents the importance of recognizing these features when staging patients with unusual ovarian neoplasms. To our knowledge, this is the first complete report of the ascitic fluid cytology of an MMMT of either ovarian or uterine origin.     

Fine needle aspiration cytology of a recurrent juvenile granulosa cell tumor

Fine needle aspiration biopsy was performed on a recurrent juvenile granulosa cell tumor of the ovary in a 24-year-old woman. The cytologic appearances and the results of histochemical, immunocytochemical and ultrastructural studies are described, and the differential diagnosis with other ovarian tumors is discussed.     

Fine needle aspiration cytology of primary soft tissue tumors. Morphologic analysis of the most frequent types.

We reviewed 98 cases of fine needle aspiration of soft tissue tumors with histologic confirmation performed during an eight-year period and propose a working morphologic classification based on the most prominent cytologic features. Six main tumor groups are recognized: myxoid, round cells, spindle cells, pleomorphic cells, polygonal cells and well differentiated. We believe that this classification allows recognition of the most common soft tissue tumors while helping with the differential diagnosis of other neoplasia, primary or secondary, with similar morphology and a similar presentation.     

Aspiration cytology of ovarian neoplasms. A review

Unlike other gynecologic tumors, which are accessible to inspection and biopsy, ovarian neoplasms are located within the pelvis or abdomen and are reachable only by laparoscopy or exploratory laparotomy. Because of its simplicity, fine needle aspiration cytology may be used as an alternative to surgical procedures. However, because of the great variety of tumor types in the ovary, cytologic interpretation of aspirates from that organ represents one of the most challenging fields in diagnostic cytology. Nevertheless, as clinicians become more experienced in using this technique and pathologists gain confidence in their ability to evaluate samples correctly, aspiration cytology may prove to be one of the most valuable and acceptable tools in the diagnosis of tumors of the female pelvis.     

Cytology of peritoneal washings in gynecologic patients. Diagnostic criteria and pitfalls

A total of 226 peritoneal washing specimens obtained from gynecologic patients over a three-year period was reviewed. The diagnostic problems encountered were: differentiating reactive mesothelium from low-grade malignancies; distinguishing between benign ovarian tumors, ovarian tumors of borderline malignancy and low-grade ovarian malignancies; and potential false-positive diagnoses in endometriosis. Low-grade malignancies could be distinguished from reactive mesothelium by evaluating subtle cytologic criteria and by comparing the cells to those of the tumor on histologic section. The differentiation of low-grade epithelial malignancies from benign epithelial lesions caused difficulties that could only be resolved by evaluating the histologic material. Positive peritoneal washings increased the stage in 8 of 110 patients undergoing initial surgery for gynecologic malignancy. Two of 76 patients undergoing a second-look laparotomy had positive washings without histologic evidence of tumor. These ten patients did less well than did those with similar histology but negative washing cytology, despite receiving additional therapy because of the cytologic findings.     

Morphologic and immunocytochemical studies of bronchioloalveolar carcinoma at Duke University Medical Center, 1968-1986

Between 1968 and 1986, the tumor registries at Duke University Medical Center and Durham VA Medical Center accumulated a total of 193 patients with a diagnosis of bronchioloalveolar carcinoma (BAC). All available histologic sections of primary lung tumors and all available respiratory and pleural cytologic material were reviewed for 135 cases having an initial histologic diagnosis of BAC and no history of a primary nonpulmonary adenocarcinoma. Thirty-nine cases showed a pure BAC pattern in histologic sections; 76 showed a dominant BAC pattern with focal areas of fibrosis and acinar differentiation; 16 were carcinomas with a focal BAC pattern; and 4 were adenocarcinomas lacking a BAC pattern. Of the 115 cases with at least a dominant BAC pattern, 51 showed predominantly mucinous differentiation while 64 showed predominantly nonmucinous differentiation. Adequate cytologic material was available for review from 111 patients. For cases having at least a dominant BAC pattern, tumor cells were present in 77 of 172 adequate sputums (44.8%), 36 of 133 bronchoscopy specimens (27.1%), 15 of 18 needle aspirates (83.3%) and 14 of 15 pleural fluids (93.3%). Of all patients in this group, 60.2% had at least one specimen positive for malignancy. No cytologic features clearly distinguished adenocarcinomas having only focal bronchioloalveolar differentiation from those with a pure or dominant BAC pattern. A significant degree of overlap was observed in the cytologic features of mucinous and nonmucinous tumors. Histologic sections from 19 mucinous and 16 nonmucinous tumors were stained with monoclonal antibody B72.3: all showed some staining, with no significant difference in degree of staining between the two groups. This suggests that expression of the tumor-associated glycoprotein TAG-72 is independent of mucinous differentiation.     

Endoscopic ultrasound-guided fine needle aspiration cytology of intraductal papillary mucinous tumor of the pancreas. A case report

BACKGROUND: Intraductal papillary mucinous tumor (IPMT) of the pancreas is a recently proposed pancreatic tumor entity with a peculiar clinical and pathologic profile. It presents with symptoms that are mostly attributed to the mass effect of the tumor. However, a long history of diabetes mellitus and/or chronic pancreatitis is usually noted. It is thought that IPMT has better prognosis than the usual pancreatic ductal adenocarcinoma and its early identification is important. On imaging studies these neoplasms appear usually as multiloculated, cystic tumors that cannot be easily differentiated from other cystic pancreatic neoplasms. CASE: A 73-year-old, white woman with recent symptoms related to bile duct obstruction and diabetes mellitus and a long history of "chronic pancreatitis" was found to have a pancreatic mass on computed tomography. Endoscopic ultrasound-guided fine needle aspiration of the pancreatic mass and dilated pancreatic duct was performed. Cytopathologic examination of the aspirated material revealed large cells with abundant, eosinophilic cytoplasm arranged singly or occasionally in large sheets with complex papillary fragments. Occasional goblet cells or cells with cytoplasmic mucin-containing vacuoles were also noted within the tumor tissue fragments. The nuclei were large, with nuclear size variability and prominent nucleoli. Copious amounts of mucin and numerous muciphages were noted in the background. A diagnosis of "cystic mucinous tumor, cannot rule out invasion," was entertained. The patient underwent partial pancreatectomy. The histologic features of the resected specimen were consistent with IPMT, with focal areas suggestive of early stromal invasion. CONCLUSION: The cytologic features encountered in the aspirate of this tumor are highly suggestive of IPMT and can help differentiate it from other pancreatic tumors with mucin production, such as the classic mucinous cystic neoplasm and the more common ductal carcinoma.     

Cytologic features of functioning stromal cells in a yolk sac tumor of the ovary. A case report

BACKGROUND: Functioning stromal cells are sometimes seen in primary and metastatic ovarian neoplasms. However, the cytologic features of functioning stromal cells have been described only rarely. CASE: A 19-year-old woman had an alpha-fetoprotein-producing ovarian yolk sac tumor with functioning stroma. Her preoperative serum testosterone level was elevated. Imprint cytology showed that the functioning stromal cells had centrally located nuclei with low nuclear/cytoplasmic ratios. Occasionally these cells had vacuolated cytoplasm, suggesting the presence of lipids. In sharp contrast, the yolk sac tumor cells had more pleomorphic and hyperchromatic nuclei. We were able to distinguish between neoplastic and functioning stromal cells on the basis of these findings. In addition, immunostaining for inhibin on imprint cytologic slides was of great help in identifying functioning stromal cells. CONCLUSION: Because functioning stromal cells may unexpectedly induce hormonal effects in a variety of ovarian tumors, it is important to identify such cells in cytologic specimens.     

Comparative proteomics of ovarian epithelial tumors

We analyzed 12 ovarian epithelial tumors using 2D PAGE-based comparative proteomics to construct intra- and inter-tumoral distance map trees and to discover surrogate biomarkers indicative of an ovarian tumor. The analysis was performed after laser microdissection of 12 fresh-frozen tissue samples, including 4 serous, 5 mucinous, and 3 endometrioid tumors, with correlation with their histopathological characteristics. Ovarian epithelial tumors and normal tissues showed an apparent separation on the distance map tree. Mucinous carcinomas were closest to the normal group, whereas serous carcinomas were located furthest from the normal group. All mucinous tumors with aggressive histology were separated from the low malignant potential (LMP) group. The benign-looking cysts adjacent to the intraepithelial carcinoma (IEC) showed an expression pattern identical to that of the IEC area. The extent of change on the lineages leading to the mucinous and serous carcinoma was 1.98-fold different. The overall gene expression profiles of serous or endometrioid carcinomas appeared to be less affected by grade or stage than by histologic type. The potential candidate biomarkers screened in ovarian tumors and found to be significantly up-regulated in comparison to normal tissues were as follows: NM23, annexin-1, protein phosphatase-1, ferritin light chain, proteasome alpha-6, and NAGK (N-acetyl glucosamine kinase). In conclusion, ovarian mucinous tumors are distinct from other ovarian epithelial tumors. LMP mucinous tumors showing histologically aggressive features belong to mucinous carcinoma on the proteomic basis.