Primary, papillary peritoneal neoplasia.
ABSTRACT Subsequent to the recognition of the intraperitoneal tumors of low malignant potential, clinicians have repeatedly faced the ambiguities inherent in a disease that seems aggressive on the basis of its wide distribution in the peritoneal cavity but benign on the basis of its histopathology and clinical course. Whereas the occasional case has been associated with extensive local reaction and ascites, except for a rare exception these tumors result in prolonged survival and in an absence of extraabdominal extension. The current review of 154 cases followed from 2 to 40 years, performed in an attempt to understand this perplexing disease, leads to the following conclusions: 1) Whereas frequently beginning on the ovary and showing a predilection for the pelvis, there are examples of widely disseminated peritoneal disease with minimal, if any, ovarian involvement; 2) the outcome without adjunctive therapy is excellent and thus such therapy is contraindicated in view of the death of only 2 of the 154 patients with disease, 1 of whom had had adjunctive intraperitoneal isotope therapy; and 3) this disease is best understood as a diffuse primary peritoneal tumor probably developing on the basis of irritating agents' reaching the abdominal cavity from the lower genital canal, a process similar to that proposed for the genesis of endometriosis. Such a low-grade primary in situ tumor that may involve the entire peritoneal cavity is compatible with prolonged survival.
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ABSTRACT: It is currently hoped that deaths from extra-uterine high-grade serous cancer (HGSC) will be reduced via opportunistic salpingectomy in healthy women. Accumulated data implicate the fimbria as a site of origin and descriptive molecular pathology and experimental evidence strongly support a serous carcinogenic sequence in the fallopian tube. Both direct and indirect ("surrogate") precursors suggest the benign tube undergoes important biologic changes after menopause, acquiring abnormalities in gene expression that are often shared with malignancy, including PAX2, ALDH1, LEF1, RCN1, RUNX2, beta catenin, EZH2 and others. However, the tube can be linked to only some HGSCs, recharging arguments that nearby peritoneum/ovarian surface epithelium (POSE) also hosts progenitors to this malignancy. A major sticking point is the difference in immunophenotype between POSE and Müllerian epithelium, essentially requiring mesothelial to Müllerian differentiation prior to or during malignant transformation to HGSC. However, emerging evidence implicates an embryonic or progenitor phenotype in the adult female genital tract with the capacity to differentiate, normally or during neoplastic transformation. Recently, a putative cell of origin to cervical cancer has been identified in the squamo-columnar (SC) junction, projecting a model whereby Krt7+ embryonic progenitors give rise to immuno-phenotypically distinct progeny under stromal influences via "top down" differentiation. Similarly, biphasic cell differentiation can be seen in the endometrium with a parallel in the juxtaposition of mesothelial and mullerian differentiation in the ovary. An abrupt mesothelial-Mullerian transition remains to be proven, but would explain the rapid evolution, short asymptomatic interval, and absence of a defined epithelial starting point in many HGSCs. Resolving this question will require accurately distinguishing progenitor from progeny tumor cells in HGSC and pinpointing where initial transformation and trans-differentiation occurs if the POSE is an origin. Both will be critical to expectations from prophylactic salpingectomy and future approaches to pelvic serous cancer prevention.The Journal of Pathology 12/2013; 231(4). DOI:10.1002/path.4263 · 7.33 Impact Factor
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ABSTRACT: Risk-reducing bilateral salpingo-oophorectomy (RRBSO) and risk-reducing mastectomy are widely used for BRCA1 and BRCA2 mutation carriers to reduce the risk of ovarian and breast cancer. To our knowledge, no risk-reduction therapy has addressed the BCRA1/2 carrier lifetime risk of intra-abdominal peritoneal carcinoma from an appendix source. We identified a BRCA1 carrier in a hereditary breast and ovarian cancer kindred who developed a low-grade malignant appendiceal mucocele 2 years after risk-reducing salpingo-oophorectomy. Our retrospective meta-analysis assessed the risk of intraperitoneal appendiceal cancer in BRCA1/2 carriers after RRBSO to determine whether elective risk-reduction appendectomy could reduce the incidence of intraperitoneal cancer. Data sources included the case report and 12 reports of BRCA1 and BRCA2 carriers after RRBSO with ovarian, fallopian tube, breast, and peritoneal cancer published from January 1, 1985, through April 30, 2012. Main outcome measures were nonovarian, non-fallopian tube, nonbreast, positive intra-abdominal peritoneal carcinoma in previously cancer-free BRCA1/2 carriers after RRBSO. The source of intraperitoneal cancer in BRCA1/2 carriers after risk-reducing salpingo-oophorectomy is highly likely the appendix. Use of risk-reduction appendectomy with RRBSO in younger BRCA1/2 carriers may reduce lifetime risk of malignant tumor and eliminate intraperitoneal cancer.JAMA SURGERY 03/2013; 148(3):285-91. DOI:10.1001/jamasurg.2013.1006 · 4.30 Impact Factor
Conference Paper: Sampling hot springs for radioactive and trace elements[Show abstract] [Hide abstract]
ABSTRACT: A brief description is given of techniques that have proved successful in obtaining samples of hot and cold spring waters for x-ray fluorescence, neutron activation, and radiometric analysis. The sampling methods require only lightweight, portable field apparatus. Various types of springs sampled are illustrated. Chemical geothermometer temperatures from these samples have compared well with reported measured subsurface temperatures. (LBS)09/1975