A variant of Zinner syndrome with ectopic ureteral insertion into the seminal vesicle.

Zinner syndrome comprises a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction, which can be accompanied by additional abnormalities of the genitourinary tract in some cases. Patients may be asymptomatic or present with urinary, reproductive, and/or local pain symptoms. Diagnosis is most commonly achieved via MRI. Here, we present the case of an 18-year-old male previously diagnosed with unilateral renal agenesis, who presented with testicular and penile pain, along with urinary urgency and frequency. MRI of the abdomen and pelvis revealed all three components of Zinner syndrome as well as an ectopic ureter emptying into the seminal vesicle. Our case adds to the existing limited literature on this rare syndrome and broadens the understanding of how this syndrome can present both clinically and radiologically.

Challenges in the Diagnosis and Treatment of Aneurysmal Bone Cyst in Patients with Unusual Features.

OBJECTIVES: Aneurysmal bone cyst (ABC) is a benign but locally aggressive tumor. It has several challenging features. The aim of this study is to identify challenges in the diagnosis and treatment of ABC especially in patients with unusual features. METHODS: This retrospective study involved medical record review of primary ABC patients with one or more of the following features: unusual clinical presentation with a mass or a pathological fracture especially at an unusual age, rare locations, radiological findings suggesting other diagnoses especially sarcoma, and a nondiagnostic histopathology of biopsy samples. RESULTS: 25 patients (17 males and 8 females) were included. Most patients were either younger than 10 or older than 20 years. 10 patients presented with a mass or a pathological fracture. Unusual locations include the scapula, the olecranon, the hamate, the calcaneus, and the first metatarsal bone. Extension into the epiphysis occurred in 2 patients with proximal fibula and olecranon ABCs. Two separate synchronous cysts existed in the proximal epiphysis and middiaphysis of one humerus. Radiological imaging suggested other primary diagnoses in 8 patients. Core needle biopsy was diagnostic in only 2 of 7 patients. The main treatment was intralesional resection/curettage with bone grafting. Wide resection was performed in 4 patients. Recurrence rate was 28%. Recurrence risk factors included the following: age less than 10 years, male gender, and proximal femur location. Late recurrence occurred in 3/7 patients. One patient with asymptomatic radiological recurrence showed subsequent spontaneous resolution one year later. CONCLUSIONS: This study presented multiple unusual features of ABC including: unusual age, rare locations, and nondiagnostic radiological and histopathological findings. These features can complicate the diagnosis and management. Given these features, especially with pathological fractures, a well-planned incision, the use of frozen section examination, and the application of either external fixation or plate osteosynthesis for fracture fixation can be recommended.

Anterior versus posterior approach to treat cervical spondylotic myelopathy, clinical and radiological results with long period of follow-up.

BACKGROUND: Cervical spondylotic myelopathy increases with age, but not all cases are symptomatic. It is usually diagnosed clinically and radiologically (X-ray and magnetic resonance imaging). Surgical treatment is indicated in severe symptomatic cases, while treatment controversy exists in the presence of less severe cases. Anterior and posterior approaches are generally used for decompression with no significant differences in the results of both. METHODS: A total of 287 patients of cervical spondylotic myelopathy were treated at our hospital between January 2004 and December 2015. Only 140 patients were eligible for our study. They had at least 5 years of follow-up using full clinical scores and radiological evaluation. They were divided into two groups: group I with 73 patients (aged 23-79 years) underwent posterior decompression, lateral mass instrumentation, and fusion, while group II with 67 patients (aged 33-70 years) underwent anterior decompression, instrumentation, and fusion. Neck Disability Index, local score, and X-ray were used in the evaluation of the patients. RESULTS: Preoperative mean ± standard deviation of Neck Disability Index of both the groups was 32.06 ± 6.33 and 29.88 ± 5.48, which improved in the last visit (>5 years) to 5.81 ± 7.39 and 2.94 ± 5.48 for groups I and II, respectively (p value <0.05). The local score of groups I and II was (P = 1, F = 21, G = 31, E = 19) and (P = 1, F = 12, G = 36, E = 18), which on discharge day improved to (P = 1, F = 4, G = 12, E = 55) and (P = 0, F = 3, G = 6, E = 58) at last follow-up, respectively. Fusion rate was nearly equal for both the groups during all the follow-up intervals and it was 91.1% and 91.7% in the last follow-up. CONCLUSION: There were no significant differences in the clinical and radiological results between the anterior and posterior approaches used in the surgical treatment of spondylotic cervical myelopathy. However, statistically significant results of Neck Disability Index of anterior approach were not clinically important and may be due to changes in the size and shape of the neck in group II.

The postmortem fate of Pat Gregory: a disinterred Native American.

In late June 1990, the Mono County Sheriffs Department in Bridgeport, CA contacted the Physical Anthropology Human Identification Laboratory (PAHIL) at California State University, Chico to seek assistance in the identification of a recently discovered skull. To assist with possible identification, the cranium received a classic physical anthropological/morphological analysis to suggest the decedent's sex, age at death, ancestral affiliation, and uniqueness. It was concluded the cranium was that of an older male, and someone with mixed ancestry, probably Native American/White. Suggested uniquenesses were an eroded and greasy texture, with adhering white sand, evidence of healed antemortem nasal fractures, and a bifid left occipital condyle. The cranium was confiscated from a man suspected of vandalizing a Native American cemetery just south of the community of Lee Vining. The cemetery was established in the mid-1800's by local Native American tribes. Although ownership of the land was disputed by the US Forest Service (the Inyo National Forest), and the Los Angeles Department of Water and Power (LADWP), county authorities claimed that because the incident involved the desecration of a cemetery and human remains, it was a legal issue, and therefore, the Sheriff's Department had jurisdiction over the case if not the land. The suspect pled guilty to the possession of Native American remains but claimed not to have desecrated a grave. Over the next year and a half, members of the Native American community representing various tribes sought the return of the cranium, while also seeking assurance that it belonged to the vandalized grave. While county, US Forest Service, and LADWP officials continued to argue over whom had jurisdiction of the remains the superior court judge ordered the county to pay for any analysis necessary to determine if the cranium belonged to the decedent in question. This report addresses the conclusions of that analysis and the disposition of the case. Furthermore, the report addresses the forensic value to Native Americans of the continued study of a wide variety of human skeletal remains.

Acquired megacolon is associated with alteration of vasoactive intestinal peptide levels and acetylcholinesterase activity.

Based upon previous morphologic studies, we hypothesized that the development of acquired megacolon was associated with abnormalities of enteric neurotransmitter concentrations and enzymatic activities. Specimens were obtained at surgery from patients with normal descending-sigmoid colon (n = 13) and patients with sigmoid megacolon (n = 6; defined by radiologic measurement). Radioimmunoassays were used to measure the non-adrenergic, non-cholinergic inhibitory neuropeptide, vasoactive intestinal peptide, and the non-adrenergic, non-cholinergic excitatory neuropeptide, substance P, while spectrophotometric assays were used to quantitate acetylcholinesterase activity and choline acetyltransferase activity. There were significantly decreased concentrations of vasoactive intestinal peptide and decreased acetylcholinesterase activity in muscularis externa from patients with acquired megacolon. In megacolon, vasoactive intestinal peptide-containing nerve fibers appeared to be diminished in circular and longitudinal smooth muscle, and immunostaining of nerve cell bodies in the plexus submucosus externus appeared diminished. These results suggest the hypothesis that production of vasoactive intestinal peptide is altered allowing secondary colonic hypertrophy to develop from prolonged cholinergic nerve-mediated contractions of circular smooth muscle. As a corollary to this hypothesis, colonic dilatation might result from prolonged contraction of longitudinal smooth muscle.

Multilocular prostatic cystadenoma presenting as a large complex pelvic cystic mass.

Symptomatic prostatic cysts are not common. We report a case of a large complex cystic pelvic mass encountered in a Jordanian man that proved to be a huge midline prostatic cyst with seminal vesicle contained within the wall. This finding represents a variation of the newly proposed diagnosis, multilocular prostatic cystadenoma.

Flow cytometric evaluation of early invasive cervical cancer.

OBJECTIVE: To define the role of flow cytometry as a prognostic indicator in early cancers of the uterine cervix. METHODS: Flow cytometry was used to determine ploidy, DNA index, and S-phase fraction on 141 samples from the tumors of 53 women with stage IB cancers of the cervix treated by radical hysterectomy and pelvic lymphadenectomy as primary therapy. Multiple samples of the same tumor were analyzable for 47 (89%) of the subjects. One-way analysis of variance for the multiple samples was used to compare the heterogeneity of flow cytometry data, both within each tumor and between patients. Flow cytometry results, as well as previously described clinical and pathologic prognostic factors, were correlated to recurrence and survival using Cox regression hazard ratios and Kaplan-Meier estimates. RESULTS: We found DNA aneuploidy in 25 (47%) of the cancers, with a mean (+/- standard error) DNA index of 1.52 +/- 0.07. The mean S-phase fraction was 7.6 +/- 0.4% for diploid tumors and 9.2 +/- 0.4% for aneuploid tumors. The cancers from 24 women (45%) were homogeneously diploid, 18 (34%) were consistently aneuploid, and five (9%) had mixed diploid/aneuploid samples. Analysis of variance of the multiple samples for each woman revealed a greater standard deviation (SD) between patients than within any individual tumor for both DNA index (ratio of between SD to within SD 2.1; P < .0001) and S-phase fraction (ratio 1.6; P < .0001). Of the previously described clinical and pathologic prognostic factors, only depth of invasion, expressed as either percent of cervical wall thickness or as thirds, was correlated with recurrence or survival. Neither the DNA index nor S-phase fraction correlated significantly with recurrence or survival. CONCLUSIONS: These results suggest that alterations in the DNA content or proliferative activity of early invasive cancers of the uterine cervix do not reflect biologic behavior in terms of recurrence or survival, and that this behavior is not due to tumor heterogeneity.

Infiltrating syringomatous adenoma of the nipple.

Infiltrating syringomatous adenoma of the nipple is a distinct, benign clinical entity. It is similar histologically to a syringoma, a benign tumor originating in the ducts of the dermal sweat glands. When located in the nipple, this lesion has been mistaken for nipple duct adenoma or tubular carcinoma. Infiltrating syringomatous adenoma of the nipple is locally infiltrating but does not metastasize. Appropriate local management depends on an accurate diagnosis. Following is a case report, review of the literature, and therapeutic options for infiltrating syringomatous adenoma of the nipple.

Multilocular cystic renal cell carcinoma.

Multilocular cystic renal cell carcinoma (MCRCC) appears to be a distinct subtype of renal cell carcinoma with characteristic gross and microscopic features. The authors' ten-year experience (1977-1987) included six cases of MCRCC that were followed for a minimum of two years, with neither recurrence nor metastasis observed in any of the cases. During this period, there were 855 urologic procedures for the upper urinary tract, with 256 neoplasms or cysts identified. These included 32 simple cysts, 41 transitional cell carcinomas, 133 renal cell carcinomas, 17 papillary renal cell carcinomas, and 33 miscellaneous tumors. Histologically, the MCRCCs were well-demarcated multicystic lesions containing variably sized aggregates of neoplastic clear cells showing grade 1 nuclear features and little or no mitotic activity. The cyst walls were densely fibrotic, and the lining was often devoid of epithelium. Flow-cytometric analysis performed in five of the six cases with the use of paraffin-embedded tissue showed the tumors to be diploid in all instances, with low proliferative activity. The authors believe that this tumor is a low-grade variant of renal cell carcinoma and should be studied further to determine appropriate therapy.

Prognostic implications of proliferative activity and DNA aneuploidy in Astler-Coller Dukes stage C colonic adenocarcinomas.

Paraffin-embedded surgical specimens from 69 patients who underwent resections of otherwise untreated Dukes stage C adenocarcinoma of the colon were examined for proliferative activity, DNA aneuploidy, DNA index, and proportion of aneuploid cells by flow cytometry. Results were correlated to clinical characteristics of the patients and to overall survival times. DNA aneuploid tumors were identified in 60 cases (87%), diploid tumors in 9 cases (13%). The mean S-phase fraction for all cases was 17.6%, with a standard deviation (SD) of 7.8. In univariate statistical analysis, younger patient age, lower tumor proliferative activity, DNA index less than or equal to 1.2, and presence of only 1-4 lymph nodes with tumor involvement were found to be significant predictors of improved patient survival. In multivariate Cox regression analysis, low tumor proliferative activity, younger patient age, and location of the tumor in the right or transverse colon were found to be significant independent predictors of increased patient survival. When tumor proliferative activity was stratified into statistically defined subgroups, patients with tumors of low proliferative activity (S-phase less than mean - 0.5 SD) had significantly longer survival than patients with tumors of moderate proliferative activity (S-phase value greater than mean - 0.5 SD and less than mean +0.5 SD) or high proliferative activity (S-phase greater than mean +0.5 SD). These results suggest that tumor proliferative activity in Dukes C colon carcinoma may be an important biological factor in determining patient prognosis.

Dual-parameter flow cytometric analysis coupling the measurements of forward-angle light scatter and DNA content of archival ovarian carcinomas of low malignant potential.

Paraffin-embedded archival specimens from 45 cases of ovarian carcinoma of low malignant potential (OCLMP) were analyzed by flow cytometry (FCM) using propidium iodide (PI) staining. Since single-parameter FCM analysis is often deficient in the resolution of subtle near-diploid DNA-aneuploid populations, forward-angle light scatter (FALS) was measured as a second parameter. DNA aneuploidy was identified in 15 cases (33%). In 7 of those 15 cases, aneuploidy was resolved with single-parameter FCM; in the remaining 8 cases, DNA aneuploidy was resolved only following dual-parameter analysis coupling DNA content and FALS. In all 15 cases, a single near-diploid aneuploid population was observed (mean DNA index = 1.2); there were no tetraploid aneuploid cases. The proliferative activity for all 45 cases studied ranged from 1.0% to 8.9%, with a mean of 3.5%. No difference in mean proliferative activity was observed between the aneuploid and diploid tumors (P greater than .05). To exclude the possibility that PI staining artifacts caused the observed aneuploidy, five of the eight cases shown to be aneuploid by dual-parameter analysis were further studied using an alternate DNA-binding dye, DAPI, yielding similar results. To exclude the possibility that contaminating stromal and/or inflammatory cells caused the observed aneuploidy, samples from a subset of the dual-parameter cases were sorted, revealing the aneuploid populations to be composed primarily of tumor nuclei.(ABSTRACT TRUNCATED AT 250 WORDS)

Comparison of endometrial biopsy and urinary pregnanediol glucuronide concentration in the diagnosis of luteal phase defect.

To determine if pregnanediol glucuronide (PG) excretion is useful in luteal phase assessment, we compared daily first morning urinary PG concentrations during the luteal phase in nine normal and nine deficient cycles. Total luteal pregnanediol excretion (44.1 +/- 11.3 versus 64.0 +/- 11.6 area units +/- SEM) was not different. However, significantly less pregnanediol was excreted by the abnormal group during the 1st 5 days of the luteal phase (12.7 +/- 1.2 versus 18.0 +/- 1.7 area units +/- SEM, respectively). Thus, delayed PG excretion may be characteristic of luteal phase defect and measurement of urinary PG may be useful only if daily samples during the early luteal phase are obtained.

Hydatidiform mole: clinicopathologic associations with the development of postevacuation trophoblastic disease.

Clinical information and histopathologic material for 165 patients with hydatidiform mole referred to the John I. Brewer Trophoblastic Disease Center of Northwestern University Medical School during one year were reviewed in order to identify characteristics more likely to be associated with the development of gestational trophoblastic tumors. Twenty-nine patients (18%) required chemotherapy for invasive mole or choriocarcinoma. Patients with uterine enlargement beyond that expected for dates and patients with ovarian theca-lutein cysts were much more likely to require treatment after molar evacuation (47% vs. 18% and 40% vs. 16%, respectively). There was no correlation between the initial human chorionic gonadotropin level, gestational age, uterine size per se, maternal age or gravidity and the subsequent clinical course. Histologically, the following factors were associated with an increased incidence of postmolar gestational trophoblastic tumor: (1) progressive nuclear atypia (26.7% if atypia present vs. 40% if absent); (2) necrosis and hemorrhage (39.1% if extensive vs. 12.8% if limited); (3) decreased trophoblast maturation (48% if less than 20% mature vs. 8.7% if greater than or equal to 20% mature); (4) trophoblast proliferation (50% if marked vs. 13.9% if limited); (5) increased ratio of cytotrophoblast to syncytium (33.3% if greater than 1 vs. 6.4% if less than 1); and (6) absence of Nitabuch's layer (21.4% if absent vs. 11.6% if present). Hydatidiform moles which demonstrate clinical or histopathologic evidence of excessively abnormal proliferative activity, as indicated by these features, are more likely to develop invasive mole or choriocarcinoma and should be considered for prophylactic chemotherapy.

Placental site trophoblastic tumor associated with immature ovarian teratoma.

This paper describes a woman who developed three malignancies within a very short time. Two of these, immature teratoma and placental site trophoblastic tumor, were rare gynecological neoplasms. The third was a breast carcinoma. This is the first report of an association of placental site trophoblastic tumor with a primary germ cell tumor of the ovary.

Flow cytometric evaluation of epithelial ovarian cancer.

We investigated the prognostic significance of deoxyribonucleic acid content and proliferative activity of tumor cell populations as measured by flow cytometry of the tumor specimens from 115 women with epithelial ovarian cancer. Deoxyribonucleic acid aneuploidy was found in 87 of 115 (76%) of these cancers with a mean deoxyribonucleic acid index of 1.6 and S-phase fraction of 14.7%. The S-phase fraction of the 28 (24%) diploid tumors was 7.0%. Deoxyribonucleic acid ploidy was significantly correlated with survival. S-phase fraction was significantly correlated with ploidy, residual tumor, histology, grade, ascites, time to recurrence, and survival. Diploidy versus aneuploidy were the best discriminating values for deoxyribonucleic acid index and an S-phase fraction of greater or less than 18% for that parameter. Multivariate analysis revealed stage, S-phase fraction, residual tumor, and grade to be independently associated with time to recurrence, and stage, age, S-phase fraction, and largest metastases were factors associated with survival. Deoxyribonucleic acid ploidy did not significantly improve either model. These results suggest that abnormalities of deoxyribonucleic acid content and the proliferative activity of tumor cell populations are reflective of their biologic activity.

Histopathologic and flow cytometric analysis of adenomatous colonic polyps.

We evaluated the histopathology, DNA content, and proliferative activity of colonic polyps independently. Paraffin-embedded specimens were used as source material. In each case, additional sections were cut at 3 microns and stained with hematoxylin-eosin and trichrome for histopathologic analysis. For DNA analysis and measurement of proliferative activity, the polyp parts were dissected and the nonpolypoid tissue was discarded. The study was limited to those specimens that were received in our department in the years 1972 and 1977. Of the 104 polyps that were submitted for flow cytometric analysis, 36 could not be analyzed owing to excessive debris or insufficient nuclei. DNA aneuploidy was identified in 32% of the cases, with a higher value noted in larger polyps and in severely dysplastic polyps, but these values were not statistically significant. Multiple adenomas from the same patient often showed different DNA histograms. When analyzed according to the percentage of cells in S phase, no significant difference was found in proliferative activity of polyps according to DNA content or size of the polyps. These results suggest that the diagnostic significance of aneuploidy and proliferative activity in polyps must be interpreted with caution.

Bilateral breast cancer.

The magnitude of the clinical problem of bilateral occurrence of breast cancer is controversial. In order to evaluate clinical and pathologic factors associated with increased risk for metachronous, contralateral breast cancer, we reviewed the records of 187 patients undergoing operative treatment for primary breast cancer by the same surgeon at a single institution. Variables analyzed included age, race, history of tuberculosis or cancer at other sites, family history of breast cancer or cancer at other sites, prior use of estrogens, tobacco, and alcohol, marital status, parity, age at first pregnancy, tumor size and location, histologic diagnosis, degree of cellular differentiation and involvement of axillary lymph nodes. The incidence of metachronous, contralateral breast cancer was 11.8 per cent. The only factor with a statistically significant association with bilateral cancer was histologic diagnosis characterized by multicentricity. Life table survival analysis revealed that the five-year survival following treatment for metachronous, contralateral cancer was 59 per cent. We conclude that bilateral breast cancer is a significant clinical problem, that histology characterized by multicentricity is associated with a higher incidence of bilateral breast cancer, and that favorable survival justifies an aggressive approach.

Neoplasms of endometrial stroma: histopathologic and flow cytometric analysis with clinical correlation.

Ten neoplasms of endometrial stroma (one stromal nodule, four endolymphatic stromal myoses, and five stromal sarcomas) were compared using clinical data as well as histopathologic and flow cytometric parameters. None of the patients with stromal nodules or endolymphatic stromal myosis had extrauterine disease at presentation or tumors displaying a mitotic rate greater than 10/10 hpf (high-power fields), nuclear pleomorphism, atypical mitotic figures, DNA aneuploidy, or a high proliferative index (greater than 10% S phase cells). The stromal nodules were circumscribed and behaved in a benign fashion. The patients with endolymphatic stromal myosis had infiltrative tumors that behaved as low-grade cancers with good responses to therapy. Four of the five patients with stromal sarcoma had extrauterine disease at presentation as well as tumors characterized by a mitotic rate much greater than 10 mitoses/10 hpf, nuclear pleomorphism, atypical mitotic figures, DNA aneuploidy, and a high proliferative index. These four patients had aggressive disease with poor response to therapy. The fifth of the stromal sarcomas had a high mitotic rate, but lacked the other features linked with aggressive behavior; this patient has responded well to treatment. The mitotic count may not be the most useful criterion for predicting biologic behavior in endometrial stromal tumors since it does not always reflect an increased rate of cell turnover as demonstrated by the percentage of cells in the S phase. DNA analysis by flow cytometry yields a more accurate picture of tumor behavior.

Histopathological and clinical correlations of cystosarcoma phyllodes.

We performed a detailed retrospective analysis of 25 patients treated primarily at Northwestern Memorial Hospital, Chicago, for cystosarcoma phyllodes. Histopathological evaluation correlated well with malignancy, but clinical suspicion did not. Pathological studies indicated that high-grade tumors, necrosis, infiltrating margin, and the presence of more than one mesenchymal element were often associated with aggressive behavior. Flow-cytometric analyses of DNA aneuploidy and proliferative index supported the grading system we used, since all four malignant cases examined showed high proliferative index, and three of the four cases showed aneuploidy. None of the low-grade cases, including the recurrent ones, showed increased proliferative index or aneuploidy. We believe that flow-cytometric analysis ought to be performed on cases in which there is doubt regarding the potential malignancy.