bromochloroacetic-acid and Pseudomyxoma-Peritonei

We report two rare cases of neoplastic pseudomyxoma peritonei associated with splenic mucinous epithelial cysts and review previously reported cases of splenic mucinous lesions in order to investigate the extent and implications of such an association.. The majority of mucinous lesions of the spleen appear to be associated with pseudomyxoma peritonei. The clinicopathological profile of these cases conforms to that of neoplastic pseudomyxoma peritonei, showing a similar age of onset, outcome and histological features. Most of the cases were associated with a confirmed or suspected appendiceal primary. The immunophenotype (cytokeratin 7 negative; cytokeratin 20 and CEA positive) of the lesions of both our cases, including those in the ovary, was suggestive of a gastrointestinal origin.. Splenomegaly due to cystic intrasplenic mucinous epithelial lesions may occasionally be the presenting feature of pseudomyxoma peritonei or herald tumour recurrence. Mucinous epithelial cysts of the spleen may also precede the development of pseudomyxoma peritonei. All cases of pseudomyxoma peritonei should be investigated for splenic involvement and, conversely, a primary mucinous neoplasm sought elsewhere in the abdomen in all cases of splenic mucinous cysts.

Topics: Cysts; Epithelial Cells; Fatal Outcome; Female; Humans; Immunoenzyme Techniques; Keratins; Male; Middle Aged; Mucins; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Splenic Diseases

Coordinate expression profiles for cytokeratins 7 and 20 (CK7 and CK20) are useful for distinguishing certain types of adenocarcinomas but use for distinction of primary and secondary mucinous tumors in the ovary is limited due to the existence of a number of tumor types exhibiting overlapping CK7/CK20 immunoprofiles; the use of staining distribution patterns in the distinction of tumors with shared profiles has not been evaluated in detail. We report analysis of both coordinate expression profiles and staining distribution in 179 rigorously classified mucinous tumors in the ovary, including 53 primary tumors [35 atypical proliferative (borderline) mucinous tumors of gastrointestinal type and 18 invasive mucinous carcinomas] and 126 secondary tumors [28 colorectal adenocarcinomas, 54 appendiceal tumors (23 adenocarcinomas, 31 low-grade adenomatous mucinous tumors associated with pseudomyxoma peritonei), 14 pancreatic adenocarcinomas, 8 endocervical adenocarcinomas, 5 gastric adenocarcinomas, 4 gallbladder/biliary tract adenocarcinomas, and 13 adenocarcinomas of unknown primary sites). A CK7+/CK20+ immunoprofile was the most common profile in primary ovarian tumors (74%), upper gastrointestinal tract tumors (78%), and endocervical tumors (88%) but was occasionally observed in lower intestinal tract tumors (colorectal: 11%; appendiceal: 13% of low-grade tumors, 35% of carcinomas). A CK7-/CK20+ immunoprofile was the most common profile in lower intestinal tract tumors (79%) and was uncommon in upper gastrointestinal tract tumors (9%), rarely seen in primary ovarian tumors (4%), and not seen in endocervical tumors. A CK7+/CK20- profile was observed in some primary ovarian (23%), upper gastrointestinal tract (13%), and endocervical tumors (13%) but not in lower intestinal tract tumors. For CK7+ tumors, staining distribution was very frequently diffuse (>50% of tumors cells positive) in primary ovarian, upper gastrointestinal tract, and endocervical tumors, whereas staining distribution was often focal (<50% of tumors cells positive) when present in colorectal and appendiceal carcinomas but not in low-grade appendiceal tumors. For CK20+ tumors, staining distribution was variable but often focal in primary ovarian tumors and nonlower intestinal tract tumors, whereas the pattern was almost always diffuse in lower intestinal tract tumors. Immunohistochemical staining distribution can supplement CK7/CK20 coordinate expression profiles to distinguish subsets of prim

Topics: Adenocarcinoma; Adenocarcinoma, Mucinous; Coloring Agents; Female; Gastrointestinal Neoplasms; Humans; Immunohistochemistry; Keratin-20; Keratin-7; Keratins; Ovarian Neoplasms; Pseudomyxoma Peritonei; Uterine Cervical Neoplasms

CDX-2 is a highly sensitive and specific marker of intestinal epithelial cells and their neoplastic counterparts. CDX-2 status in pseudomyxoma peritonei (PMP) has been barely reported. The aim of this study was to investigate the clinicopathological features of 42 cases of PMP with a special emphasis on CDX-2.. All patients were treated by cytoreduction. Immunohistochemistry was performed for CDX-2, MUC-2, MUC-5AC, cytokeratin (CK) 7 and CK20. Statistical correlation was evaluated for age, sex, completeness of cytoreduction and histological subtype with overall and progression-free survival (OS and PFS). PMP consisted of 32 cases of disseminated peritoneal adenomucinosis and 10 cases of peritoneal mucinous carcinomatosis. The appendix evaluated in 25 cases showed two mucinous adenocarcinomas and 21 low-grade appendiceal mucinous neoplasms. CDX-2 was diffusely positive in 40 cases, with the remaining two cases being focally positive. All cases demonstrated diffuse reactions to CK20 and MUC-2, and variable reactions to MUC-5AC, while CK7 was variably positive in 38 cases. Five-year OS was 97%. Histological type was significantly correlated with PFS (P=0.02).. CDX-2 is diffusely and strongly positive in PMP. This is a useful marker to confirm an appendiceal origin of PMP, particularly when used in conjunction with CK7, CK20, MUC-2 and MUC-5AC.

Topics: Adult; Aged; Appendiceal Neoplasms; Biomarkers, Tumor; Carcinoid Tumor; CDX2 Transcription Factor; Disease-Free Survival; Female; Homeodomain Proteins; Humans; Immunohistochemistry; Keratin-20; Keratin-7; Keratins; Male; Middle Aged; Mucin 5AC; Mucin-2; Mucins; Neoplasms, Multiple Primary; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei; Retrospective Studies; Survival Analysis; Trans-Activators

It has generally been accepted that pseudomyxoma peritonei/disseminated peritoneal adenomucinosis originates from appendiceal low-grade adenomatous mucinous tumors. A woman who underwent an appendectomy 42 years ago, presented with a unilateral ovarian tumor whose immunohistochemical phenotype and its association with a teratoma, strongly suggest that pseudomyxoma peritonei originated from a ruptured mucinous tumour arising from a mature cystic teratoma.

Topics: Adenoma; Aged; Appendectomy; Douglas' Pouch; Fallopian Tubes; Female; Humans; Hysterectomy; Immunohistochemistry; Keratin-20; Keratins; Omentum; Ovarian Neoplasms; Ovariectomy; Pseudomyxoma Peritonei; Rupture, Spontaneous; Splenectomy; Teratoma

Pseudomyxoma peritonei (PMP) is a rare condition characterized by gelatinous ascites. Although the histologic attributes of PMP have been well studied, the cytologic features remain ill defined.. We reviewed the peritoneal washings (PW) in 67 patients with PMP to identify cytomorphologic features useful in classifying cases as either disseminated peritoneal adenomucinosis (DPAM) or peritoneal mucinous carcinomatosis (PMCA). Histologic specimens were correlated with the cytologic diagnoses. Correlation between cytologic diagnosis and patient outcome was investigated.. Neoplastic epithelial cells were identified in 63 of 67 PW (94%). Concordance with the histologic diagnosis was obtained in 61 of 63 cases. Of these 36.5% were cytologically classified as DPAM with primary appendiceal neoplasms in 19 cases. Thirty-four of 63 cases (53.9%) were cytologically diagnosed as PMCA based on PW cytology. Most were of appendiceal or colonic origin. Four cases displayed cytologic features of both DPAM and PMCA. Two discordant cases each with a cytologic diagnosis of PMCA had an appendiceal adenoma. Acellular mucin alone was identified in the PW in four cases. Analysis of follow-up data revealed that cases diagnosed as DPAM had a better prognosis than those diagnosed as PMCA.. Cytomorphologic features of epithelial cells in PW material can accurately categorize cases of PMP as either DPAM or PMCA. Furthermore, this categorization appears to have important prognostic implications.

Topics: Adenocarcinoma, Mucinous; Adult; Aged; Aged, 80 and over; Ascites; Calbindin 2; Carcinoembryonic Antigen; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratin-20; Keratin-7; Keratins; Male; Middle Aged; Peritoneal Neoplasms; Pseudomyxoma Peritonei; S100 Calcium Binding Protein G

Pseudomyxoma peritonei syndrome is a rare disease that originates from an adenomatous lesion of the appendix that, from pressure, perforates to gain access to the free peritoneal cavity. The relative sparing of the small bowel surfaces allows for complete cytoreduction even though many kilograms of mucinous tumour exist at other sites within the abdomen and pelvis. The purpose of this study was to examine the mechanism whereby the small bowel remains free of gross tumour and peritoneal surface polyps form.. Peritoneal surface polyps were harvested and examined grossly and histologically. A hypothesis for their formation on small bowel and small bowel mesentery was proposed. Polyps are known to be associated with repeated motion of enteric contents moving past adenomatous tissue so that, over time, an elongated stalk is created. We have repeatedly observed pedunculated polyps on the peritoneal surface of the small bowel in patients with pseudomyxoma peritonei syndrome. No other site within the peritoneal cavity has had a pseudomyxoma polyp located upon its surface.. The peristaltic motion of the small bowel causes adherent adenomatous tissue to develop a stalk on the peritoneal surface. Motion not only creates polypoid lesions but also repeatedly clears mucinous tumour cells from the small bowel surface. With pseudomyxoma peritonei and with other types of cancerous dissemination, prevention of adherence by motion may interfere with the implantation of malignant cells.

Topics: Biomarkers; Humans; Immunohistochemistry; Keratins; Peritoneal Neoplasms; Peritoneum; Polyps; Pseudomyxoma Peritonei; Syndrome

To evaluate the origin of pseudomyxoma peritonei (PMP) in Chinese women.. The clinicopathologic features of 15 cases of PMP were reviewed. Immunostaining using antibodies for CK7 and CK20 was performed in the ovarian, appendiceal and peritoneal lesions of these cases.. Appendiceal pathology was documented in five cases, including four mucinous cystadenoma and one simple mucocele. Eight ovarian tumors were found, including seven mucinous cystadenocarcinomas of low malignant potential and one mucinous cystadenoma. Synchronous ovarian and appendiceal lesions were discovered in three cases. One patient had adenocarcinoma of the pancreas. The origin of mucin production was not known in four cases with metastatic adenocarcinoma found in two of them. Immunoreactivity for CK20 was demonstrated in the tissues derived from the peritoneum, ovary, appendix and pancreas while only 23% (3 out of 13 women) of the peritoneal lesions and 33% (2 out of 6 women) of the ovarian tumors were immunoreactive for CK7.. PMP is a heterogeneous lesion, which may develop from mucinous metaplasia of the peritoneum or from appendiceal, or ovarian lesions. Careful examination of the ovary and appendix with performance of appendectomy is advised in every case of PMP. Immunohistochemical examination of the peritoneal, ovarian or appendiceal lesions using antibodies, in particular that for CK7 would help in defining the origin of mucin production.

Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratin-20; Keratin-7; Keratins; Middle Aged; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies

Cytokeratin 7 (CK-7) has been shown to be uncommonly expressed in colonic epithelial tumors, as opposed to ovarian epithelial tumors, which are always CK-7 positive. The authors investigated the expression of CK-7 in 17 appendiceal cystadenomas and carcinomas, 20 mucinous borderline tumors of the ovary, 10 cases of simultaneous mucinous tumors of the appendix and ovary, three so-called high-stage mucinous borderline tumors of the ovary, and three cases of pseudomyxoma peritonei (PP) of unknown origin. Nine appendiceal cystadenomas were CK-7 negative; two of these were associated with PP, and the peritoneal lesions were negative as well. Three cystadenomas were CK-7 positive. Three appendiceal carcinomas were CK-7 negative, and in one case the metastases were also negative. Two carcinomas were CK-7 positive. All 20 ovarian borderline tumors were CK-7 positive. Six cases of simultaneous mucinous tumors of the ovary and appendix were CK-7 negative, as were their peritoneal mucinous deposits. Four cases showed a positive reaction in both appendiceal and ovarian sites. Two of three so-called high-stage ovarian borderline tumors were CK-7 negative. All three cases of PP of unknown origin were CK-7 negative. In conclusion, appendiceal cystadenomas are often CK-7 negative, whereas ovarian mucinous borderline tumors are always CK-7 positive. The concordant staining pattern for CK-7 of simultaneous mucinous tumors involving the appendix and ovary (60% of which were CK-7 negative) supports an appendiceal origin for these tumors. Our results also support an appendiceal (or colonic) source for any CK-7-negative mucinous tumor involving the ovary or the peritoneum. Furthermore, our findings are in agreement with the assumption that mucinous borderline-like tumors in the ovary associated with PP are not ovarian in origin but are often, if not always, metastatic from an appendiceal (or other) mucinous tumor.

Topics: Adult; Aged; Aged, 80 and over; Appendiceal Neoplasms; Carcinoma; Cystadenocarcinoma; Female; Humans; Keratins; Male; Middle Aged; Ovarian Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei

Cytokeratin 7 (CK-7) is a simple epithelial keratin that may be used to investigate the site of origin of adenocarcinomas. In fact, CK-7 is present in ovarian epithelial neoplasms but is generally absent in colonic carcinomas. This pattern of CK-7 expression may aid in elucidating the genesis of mucinous tumors occurring simultaneously in the ovary and appendix, accompanied by psuedomyxoma peritonei. Five such cases were immunostained with anti-CK-7, and all showed a concordant staining pattern of the appendiceal, ovarian, and peritoneal lesions. Two cases showed a negative reaction for CK-7 and thus would appear to represent ovarian and peritoneal metastases from an appendiceal primary tumor. Three cases were CK-7 positive, and the nature of these mucinous lesions remains open to debate; they may either represent independent primary tumors or originate from the appendix. For comparison, five Stage I mucinous borderline tumors of the ovary and their normal appendices were also stained with anti-CK-7. These ovarian tumors were all CK-7 positive, whereas the appendices were negative. It is concluded that CK-7 is capable of distinguishing a group of tumors that can reliably be classified as primary appendiceal neoplasms metastatic to the ovaries and peritoneum.

Topics: Adult; Aged; Aged, 80 and over; Appendiceal Neoplasms; Cystadenocarcinoma, Mucinous; Female; Humans; Immunohistochemistry; Keratins; Middle Aged; Ovarian Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei

Mucinous adenocarcinomas of the ovary were studied immunohistochemically for cytokeratins 7 and 18, either to determine whether the ovarian tumor was primary or a metastasis or to establish the histogenetic origin of the tumor. Primary ovarian tumors were strongly positive for both cytokeratins, while ovarian metastases from colonic cancers were negative for cytokeratin 7, as were the colonic cancers. Three of four ovarian tumors complicated by pseudomyxoma peritonei were negative for cytokeratin 7, indicating appendiceal origin. Two of seven mucinous tumors associated with dermoid cysts were negative for cytokeratin 7, suggesting gastrointestinal origin.

Topics: Adenocarcinoma, Mucinous; Adult; Aged; Colonic Neoplasms; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Keratins; Middle Aged; Ovarian Neoplasms; Pseudomyxoma Peritonei