Aggressive angiomyxoma first diagnosed as levator hernia.
When a 32-year-old woman was first seen, physical findings suggested she had a large levator hernia, but at the time of surgical resection an aggressive angiomyxoma was found.
Adult↗
Biomedical subjects
L M Roth
Publications and source records attributed to L M Roth.
Gonadoblastoma. Immunohistochemical and ultrastructural observations.
Gonadoblastoma is an unusual tumor that typically arises in a streak gonad or an abnormal testis of an individual having a Y chromosome. It is a mixed tumor composed of primitive germ cells and sex cord cells arranged in characteristic nests containing hyaline material. Whether the supportive gonadal sex cord cells are granulosa or Sertoli cells has not been clearly established, but one prior ultrastructural study favored Sertoli cell differentiation. Our studies indicate that the intermediate filaments of these cells react to monoclonal antibodies raised against cytokeratin and vimentin. The hyaline material reacts strongly with anti-laminin antibodies, indicating basement membrane material. Additionally, rodlike condensations of the intermediate filaments (historically called Charcot-Böttcher "crystalloids") were similar to those seen in the Sertoli cells of a series of postpubescent testes examined ultrastructurally. These findings support the Sertoli-like differentiation of these cells.
Adolescent↗
Ten-year follow-up of patients receiving cisplatin, doxorubicin, and cyclophosphamide chemotherapy for advanced epithelial ovarian carcinoma.
Fifty-six patients were randomly assigned to receive either one-day cisplatin, doxorubicin, and cyclophosphamide (PAC) chemotherapy (PAC-I) or five-day PAC (PAC-V) for advanced epithelial ovarian carcinoma. Follow-up has been 120+ months or to death. Ninety-one percent had either suboptimal stage III or stage IV disease and 55% had grade 2 or 3 lesions. Two patients died of toxicity and were free of disease at autopsy. A third patient died of congestive heart failure with no disease at 103 months. Additionally, eight patients had a negative second-look laparotomy, and three (37.5%) are alive with no evidence of disease (NED) 133 to 144 months after diagnosis. Five patients (62.5%) died of disease 2 to 123 months after negative second-look. Patients with optimal stage III disease had a longer median progression-free interval (PFI) and survival (33.3 and 44.5 months, respectively) than those with suboptimal or stage IV disease (16.4 and 22.5 months, respectively), and the difference in median PFI is significant (P less than .02). Patients with ascites at diagnosis had a shorter median PFI and survival (14.7 and 18 months) than those without ascites (30.0 and 33.0 months). Both differences were significant (PFI, P less than .04; survival, P = .005). PAC produces response rates that are superior to those obtained historically with single-agent alkylating therapy. Late recurrences after negative second-look laparotomy suggest that 5-year survival data may be inadequate in ovarian carcinoma.
Adult↗
Osteosarcoma of the uterine cervix associated with hyperplastic and atypical mesonephric rests.
The authors describe a unique case of osteosarcoma associated with atypical mesonephric rests occurring in the right lateral wall of the uterine cervix. The tumor was examined histologically, cytologically, immunohistochemically, and ultrastructurally and was compared with another osteosarcoma that filled an entire uterine cavity. Comparison with other neoplasms of the uterine cervix indicates that the lesion should be classified separately. Previously reported neoplasms associated with mesonephric rests within the cervix are also reviewed.
Aged↗
Large luteinized follicular cyst of pregnancy and puerperium.
The case we have reported is that of the largest luteinized follicular cyst of pregnancy and puerperium described. Histologically, distinct layers of both luteinized granulosa and theca cells were observed in this case, in contrast to those described previously. Nuclear atypia was prominent and appeared to be degenerative.
Adult↗
Poorly differentiated (small cell) carcinoma of the ovary in young women: evidence supporting a germ cell origin.
The clinical and pathologic features, including immunohistochemistry and electron microscopy, of six cases of poorly differentiated carcinoma of the ovary (small cell carcinoma) are presented. These tumors occurred in six young patients ranging in age from 10 to 24 years. Two patients had hypercalcemia. All tumors were unilateral, and four patients had advanced stage disease at presentation. Histologic features included sheets, nests, and cords of cells in a fibrous stroma, focal microcysts, and a dimorphic population of small and large cells. Eosinophilic, hyaline globules occurred in five cases, intercellular basement membrane-like substance in two cases, and glycogen in all cases. Five of six cases stained strongly for cytokeratin and vimentin; intracytoplasmic laminin was identified in three cases; and three cases were believed to show faint positivity for alpha-1-antitrypsin. Stains for alpha-fetoprotein were negative. Ultrastructural examination of two cases showed granular material in dilated rough endoplasmic reticulum, intermediate filaments, intracytoplasmic dense globules, maculae adherens, and extracellular basement membrane-like material. All of the cases proved rapidly fatal despite various therapies, as did a histologically similar testicular tumor that was admixed with seminoma and teratoma. We interpret these findings to indicate that this ovarian cancer is most likely of germ cell origin, and it may be related to yolk sac tumor, although it is clearly distinct from the classical yolk sac tumor.
Adolescent↗
Ovarian carcinoma with extracellular mucin production: reassessment of "pseudomyxoma ovarii et peritonei".
Peritoneal implants of mucin and columnar epithelium with variable amounts of free peritoneal mucin complicated 11 mucinous ovarian neoplasms. Slides of the primary ovarian tumors were reviewed in eight cases. Although all contained areas of mucinous cystadenomatous tumor of low malignant potential, the neoplasms displayed additional features not well recognized as manifestations of invasive disease in the ovary. Irregular pools of mucin were present in the ovarian stroma and contained at least rare fragments of columnar epithelium in most cases. The epithelium was often more plentiful in the peritoneal mucin. Cytologic atypia was minimal in all except one case. Goblet cells were present in both the ovarian tumor epithelium and the peritoneal implants in most cases and may have a role in dissemination of the tumor. We propose the term "ovarian carcinoma with extracellular mucin production" for this form of mucinous carcinoma; this term is both descriptive of its histologic appearance and indicative of its malignant behavior. Eight of the 11 patients died of their disease from 8 months to 13.5 years after diagnosis. One additional patient died of chemotherapy-related leukemia. The mean survival was 4.2 years. The short-term prognosis is better than that for mucinous carcinoma in general, but most patients eventually die of their disease.
Adenocarcinoma, Mucinous↗
Recent developments in the pathology of germ cell tumors.
This article describes some of the recent developments in the pathology of germ cell tumors of the testis. Many germ cell tumors show different types of differentiation. Two different explanations for this phenomenon include the differentiation of other germ cell elements from totipotential embryonal carcinoma cells or the direct differentiation of neoplasms from a malignant intratubular germ cell. Although the concept that there is a subset of seminomas having a poorer prognosis still exists, the histologic identification of such "anaplastic seminoma" remains an unachieved goal, and we, therefore, do not recommend the use of the term anaplastic seminoma at present. A recent analysis of spermatocytic seminomas has failed to demonstrate that they are capable of meiotic division. They are composed of cells differentiating in the direction of spermatocytes, but they have not achieved that stage. The prognosis, in general, remains excellent, although recently sarcomas have been reported in association with spermatocytic seminomas with metastasis of the sarcomatous elements. The presence of human chorionic gonadotropin-producing syncytiotrophoblastic giant cells in otherwise pure seminomas does not appear to adversely affect the prognosis. Yolk sac tumors have a varied histology that many pathologists do not recognize. The presence of intercellular basement membrane (parietal differentiation) is useful in the recognition of yolk sac tumor. Sometimes solid foci of yolk sac tumor may be mistaken for seminoma, and alpha-fetoprotein and cytokeratin stains may be useful in this situation, although the presence of basement membrane, hyaline globules, and focal microcysts by light microscopy may obviate the need to use them. Hepatic and enteric (or endometrioid) differentiation may occur in yolk sac tumors and cause diagnostic confusion. The development most "non-germ" cell malignancies in patients with germ cell tumors appears to occur by transformation of aneuploid teratomatous elements at the primary or metastatic site. The identification of such malignancies depends on the recognition of invasion by the elements rather than on high-grade cytologic atypia. Unusual patterns of choriocarcinoma and yolk sac tumor may be encountered following chemotherapy, and there is circumstantial evidence that some sarcomas and carcinomas occurring in patients with testis cancer may develop directly from yolk sac tumor.
Drug Therapy↗
Evaluation of the reproducibility of the World Health Organization classification of common ovarian cancers. With emphasis on methodology.
Seven pathologists independently classified 50 slides of ovarian tumors using category I of the World Health Organization classification (WHO I), each case being seen twice under different random code numbers. Intraobserver reproducibility and interobserver reproducibility, based on consistent interpretations, were both suboptimal. However, scrutiny suggested that no pathologist was a source of excessive variability, nor was suboptimal interobserver reproducibility simply due to intraobserver variability. Neither could excessive variability be attributed to skewing of results by a subgroup of unclassifiable cases. However, clearcut sources of variability were identified among the categories of WHO I, namely, mixed epithelial, unclassified epithelial, and undifferentiated carcinoma. There was also considerable variability in distinguishing serous and endometrioid neoplasms, and in identifying tumors of low malignant potential. These findings should not be misconstrued as implying that pathologists in routine practice cannot diagnose common ovarian cancers reproducibly for patient care purposes. Availability of clinical and macroscopic data, extensive sampling, histochemistry, and consultation combine, in an uncontrolled and highly individualistic fashion, to render routine service work very different from this highly controlled formal exercise. Furthermore, at the current state of the therapeutic art, many of the taxonomic problems identified in this study may have little clinical significance. Nonetheless, this study has strengthened the evidence that there may be important problems in classifying common ovarian cancers reproducibly using WHO I, and that WHO I may require greater clarity to enhance reproducibility. Current emphasis on quality assurance dictates reconsideration of the literature on reproducibility of histopathologic taxonomy, which has tended to inculpate pathologists as sources of variability. Virtually all of this literature is subject to some degree of skepticism due to deficiencies in methodology. Consideration of the question of how to measure reproducibility in anatomic pathology leads us to suggest that the community of pathologists should address the need to decrease ambiguity in classification systems as an important step toward optimizing reproducibility.
Female↗
Invasive and noninvasive implants in ovarian serous tumors of low malignant potential.
Ovarian serous tumors of low malignant potential ("borderline" serous tumors) are classified according to the histologic features of the primary ovarian tumor, without regard to any coexisting extraovarian disease. The peritoneal implants display a range of histologic appearances, ranging from benign glands (endosalpingiosis), to noninvasive papillary glandular proliferations resembling the ovarian neoplasms, to irregular glands associated with a desmoplastic stroma and having features of invasive disease. This review of 16 patients with histologically documented extraovarian tumor implants seen at Indiana University Medical Center, Indianapolis, and 13 patients whose tumor implants have been previously described in the literature indicates that the clinical stage of disease has much greater prognostic significance than does the implant histologic features. There is a tendency for patients with more advanced disease to have invasive implants. However, within a given clinical stage, disease progression or recurrence was not influenced by the presence or absence of invasive histologic characteristics in the tumor implants.
Adult↗
The differentiation of carcinomas of teratomatous origin from embryonal carcinoma. A light and electron microscopic study.
Carcinomas derived from teratomatous epithelium occur rarely in the metastases of patients with testicular cancer. These carcinomas of teratomatous origin (CTO) are easily confused with residual embryonal carcinoma (EC). For that reason, we compared the light and electron microscopic appearances of 6 CTOs with those of 12 ECs. As seen by light microscopy, the CTOs formed glands and more frequently had well-defined cytoplasmic borders, eosinophilic cytoplasm, and nonoverlapping, regular nuclei with small or absent nucleoli and little chromatin clumping and clearing, compared with the ECs. Mucin was present in the cells or glandular lumina of three CTOs but was absent in all the ECs. The demonstration of cytoplasmic glycogen was of no differential aid. The most useful differentiating ultrastructural features were long tight junctions and telolysosomes, which occurred in all of the EC but which were absent in the six cases of CTO. Desmosomes with inserting tonofilaments were present in three cases of CTO but were much less developed in EC. Two cases of CTO had microvilli with anchoring rootlets; such anchoring rootlets were not observed among EC. The distinction of CTO from residual EC is important, because CTO will likely need to be treated in a different manner.
Cell Transformation, Neoplastic↗
Ovarian adenomyoma associated with an endometriotic cyst.
An adenomyoma was found in an endometriotic cyst of the right ovary of a 36-year-old woman. We have not seen a previous report of this entity. The most likely source of the smooth muscle component of this tumor is from within endometrial tissue lining the cyst.
Adult↗
Yolk sac differentiation in germ cell tumors. A morphologic study of 50 cases with emphasis on hepatic, enteric, and parietal yolk sac features.
We assessed 50 germ cell tumors with areas of yolk sac tumor (YST) for a variety of features including histologic patterns; hyaline droplets; syncytiotrophoblastic elements; hepatic, enteric, and parietal yolk sac differentiation; and granulomatous reaction. Of prime interest was the fact that many YSTs formed hepatic-like foci (22%), enteric-like glands (34%), and parietal yolk sac structures (92%). Hepatoid areas were characterized by nests and cords of polygonal, acidophilic cells with prominent nucleoli and intense cytoplasmic staining for alpha-fetoprotein. Enteric differentiation occurred as well-defined glands with a sharp, striated border and relatively bland nuclear features. Ultrastructurally these glands had apical microvilli with associated glycocalyx and long anchoring rootlets. The apical cytoplasm and luminal contents stained for carcinoembryonic antigen. Parietal yolk sac differentiation was characterized by the intercellular accumulation of basement membrane substance as generally thick and longitudinally arranged bands of eosinophilic material. Such material, by electron microscopy, was both intra- and extracellular, and had irregular outlines and inhomogeneous electron density. It contrasted with the strictly intracellular, round, homogeneous, hyaline globules that, we believe, represent visceral yolk sac differentiation. This intercellular material stained positively for laminin, a basement membrane component. Assessment of 22 embryonal carcinomas and 24 germinomas failed to show hepatic, enteric, and parietal yolk sac features, with one possible exception. We believe these features, especially parietal yolk sac differentiation, are helpful in differentiating YSTs from embryonal carcinomas and germinomas.
Carcinoembryonic Antigen↗
Villous adenoma of the uterine cervix associated with invasive adenocarcinoma: a histologic, ultrastructural, and immunohistochemical study.
An unusual neoplasm of the uterine cervix had the features of a villous adenoma with an adjacent adenocarcinoma. Histologic and electron microscopic features of the lesion are described, as well as immunohistochemical staining of the lesion for carcinoembryonic antigen. Electron microscopic evidence of intestinal differentiation in the villous adenoma suggests a rarely recognized potential for intestinal metaplasia in the uterine cervix. The association of villous adenoma with cervical adenocarcinoma underscores the malignant potential and the need for complete excision of this rare cervical lesion.
Adenocarcinoma↗
Ovarian Brenner tumors. I. Metaplastic, proliferating, and of low malignant potential.
In several studies, attempts were made to establish criteria for distinguishing malignant Brenner tumors from proliferating and low malignant potential ones. Although these criteria can be applied to the majority of cases, there still exist tumors that present problems in classification. In applying the World Health Organization (WHO) criteria for Brenner tumors, the most important feature for distinguishing the intermediate forms from the malignant ones is the presence of stromal invasion in the latter. This feature has generally been considered difficult to employ because of the fundamental fibroepithelial nature of Brenner tumors, the stroma being derived from the ovarian stroma. A logical and relatively easily applicable classification of Brenner tumors is presented in this report. Although more complex than the WHO classification, it includes newly recognized variants of the Brenner tumor and avoids using the same terminology to describe different types and degrees of epithelial abnormalities. Fourteen unusual Brenner tumors were studied, intermediate between typical benign and frankly malignant ones, and were classified into 3 categories representing progressive epithelial abnormalities. These include metaplastic, proliferating, and tumors of low malignant potential. In none of these does stromal invasion occur. Each of these categories corresponds to a particular urothelial abnormality or neoplasm. Through this classification, a better understanding of the morphology and biologic behavior of unusual types of Brenner tumors can be expected.
Aged↗
Ovarian Brenner tumors. II. Malignant.
In this study, nine malignant Brenner tumors were reviewed and divided into well and poorly differentiated types. To meet the criteria for malignancy, stromal invasion must be observed. A component of typical benign, metaplastic, and/or proliferating Brenner tumor should be identified. The presence of these latter elements is necessary because the malignant component is often too poorly differentiated to be identified as a Brenner tumor, and a metastatic lesion cannot otherwise be ruled out on pathologic grounds. Well-differentiated tumors often occurred in close relationship to proliferating and occasionally to metaplastic areas, and poorly differentiated ones in relationship to low malignant potential areas. The malignant component may consist of transitional cell, squamous, or undifferentiated carcinoma or an admixture of these. A component of adenocarcinoma may be associated with other malignant elements, but pure mucinous or serous adenocarcinomas would be regarded as separate neoplasms. Although the number of cases is small, the well-differentiated tumors appear to have a better prognosis than the poorly differentiated ones.
Adenocarcinoma↗
Retiform differentiation in ovarian Sertoli-Leydig cell tumors. A clinicopathologic study of six cases from a Gynecologic Oncology Group study.
This report analyzes six ovarian Sertoli-Leydig cell tumors that showed retiform differentiation. The patients were young (6-29 years; average age, 17). The tumors were all limited to one ovary, and the patients have remained disease-free, with one exception, a patient who died of recurrent neoplasm 3.5 years after operation. On microscopic examination, the retiform areas were predominant in three cases and focal in the other three. The retiform areas consisted of an irregular anastomosing network of spaces lined by cuboidal cells, often with papillary formations and sometimes with tubules compressed to form slit-like spaces. In three cases the retiform areas appeared mature, and in three they were less differentiated. All tumors also had areas of typical Sertoli-Leydig cell tumor of either poor or intermediate differentiation. In the patient with metastatic disease, the metastases had a pure sarcomatoid pattern without any retiform areas.
Adolescent↗
Lymphangiosarcoma of the edematous thigh after radiation therapy for carcinoma of the vulva.
A 66-year-old patient was treated with external radiation therapy for an advanced carcinoma of the vulva. Seven years later, a lymphangiosarcoma developed in her edematous lower extremity. Lymphangiosarcomas have been reported to occur in postmastectomy patients; however, this is only the third case in a patient with a gynecologic primary malignancy. In anticipation of possible increased use of radiation therapy in vulvar carcinoma, gynecologists should be aware of this rare, highly aggressive neoplasm.
Aged↗