Autoimmune oophoritis: a clinicopathologic assessment of 12 cases.

Twelve cases of histologically confirmed autoimmune oophoritis are described. Eight presented with symptoms and laboratory evidence of premature ovarian failure (POF). Four were diagnosed unexpectedly after hysterectomy for endometrial pathology or for sequelae of cystic enlargement of the ovaries. Two of eight patients tested had serum anti-ovarian autoantibodies (Aab), while five of seven had anti-adrenal Aab. Two women had, or subsequently developed, Addison's disease, and two patients had Hashimoto's disease at presentation. All women with this disease risk the development of adrenal failure and hypothyroidism. Microscopically, 11 cases showed a lymphoplasmacytic infiltrate that spared primordial follicles but involved, with progressive intensity, early and late preovulatory follicles and corpora lutea. Sparse perivascular and perineural inflammatory infiltrates were also present. The twelfth case appeared to be a unique case of granulomatous oophoritis, considered autoimmune because of the folliculotropic nature of the inflammatory process. Three cases showed evidence of follicular dysplasia.

Composite mucinous and granulosa-cell tumor of ovary: case report of a unique neoplasm.

A 63-year-old woman presented with signs and symptoms of an estrogen-producing ovarian tumor. At laparotomy, this tumor proved to be a multilocular right ovarian mass 20 cm in greatest diameter. The cystic spaces were lined by typical benign mucinous epithelium of the endocervical type, while the greatly thickened cyst walls contained a diffuse proliferation of granulosa cells. These two disparate components were intimately mixed at a variably complex interface to form a composite tumor. While composite tumors showing sex cord-stromal and epithelial elements are well documented, they have all, to date, been of moderately to poorly differentiated androblastomatous or Sertoli-Leydig cell type, associated with heterologous mucinous elements of intestinal differentiation. The combination of granulosa-cell tumor with mucinous elements of endocervical or müllerian type has not hitherto been reported and is of uncertain histogenesis.

Hypercalcemia in association with mucinous adenocarcinoma of the ovary: a case report.

Hypercalcemia in association with mucinous adenocarcinoma of the ovary has not previously been reported. Such a case is presented.

Vulvar adenosquamous carcinoma arising in a hidradenoma papilliferum, with rapidly fatal outcome: case report.

A 65-year-old woman with adenosquamous carcinoma of the vulva arising in a hidradenoma papilliferum died from disseminated tumor 2 months after local excision. The light and electron microscopic features of the lesion are presented. The possible origins of vulvar adenosquamous carcinoma are discussed.

Extraovarian peritoneal serous papillary carcinoma. A clinicopathologic study of 31 cases.

The rate and clinical features of patients admitted to King George V Hospital with extraovarian peritoneal serous papillary carcinoma during a 9-year period were reviewed. In this time, 31 of 236 (13%) patients with an initial diagnosis of invasive serous ovarian carcinoma fulfilled the surgicopathologic criteria for this entity. All patients had disseminated tumor equivalent to ovarian Stage III and IV disease (International Federation of Gynecology and Obstetrics [FIGO]) and with predominantly high-grade neoplasms. They were managed by surgical exploration, tumor debulking where possible, and postoperative chemotherapy. A comparison with a contemporaneous series of 139 patients with primary epithelial ovarian carcinoma matched for stage and grade of disease and managed similarly showed no difference in actuarial survival. The median survival times were 11.3 months for patients with extraovarian serous papillary carcinomas and 13.5 months for patients with equivalent primary ovarian neoplasms. The features of the disease and the treatment regimens used are discussed.

Changing character of cervical cancer in young women.

To examine the hypothesis that the pattern of cervical cancer is changing data on women presenting with the disease over 34 years were studied retrospectively. During 1953-86, 2628 women with cervical cancer were referred to a large tertiary referral hospital in Sydney; 418 were aged 35 or less. During the period of review the proportion of young women with the disease increased from under 9% in the 1950s and 1960s to about 25% in the 1970s and 1980s; a similar but less pronounced trend was apparent for the whole of New South Wales in the 1970s and 1980s. The prevalence of less common morphological types of cervical cancer increased throughout the period, particularly in the young. Pelvic lymph node metastases were identified in younger patients with stage Ib and IIa tumours more commonly in the later years of the study, suggesting that the disease was becoming more severe. Overall rates of recurrence improved over time, but an apparent increase in early recurrences was observed in young patients with Ib and IIa tumours and without nodal disease. The results suggest that the clinical and pathological behaviour of cervical cancer changed over the period of review.

Papillomavirus and cervical cancer: a clinical and laboratory study.

It is now widely accepted that HPV types 16, 18, 31, and 33 are associated with the development of high grade intraepithelial neoplasia and malignant lesions in the cervix. On this basis, the identification of HPV types in cervical scrape samples has been advocated as a supplement to cytological screening tests. However, little is known of the distribution of the virus at different sites in the lower female genital tract or of how this distribution may change during the natural course of HPV infection. In this survey, HPV DNA dot hybridizations and, in some instances, Southern blot hybridizations with mixed HPV 6/11 and 16/18 probes were undertaken to detect HPV DNA in cervical scrapes and biopsies of the cervix, vagina, and vulva. A total of 92 women attending a Sydney hospital were screened: 59 of these patients had cervical disease, either invasive cervical carcinoma (CaCx) or cervical intraepithelial neoplasia (CIN), grades I-III. A group of 33 women who lacked evidence of cervical abnormalities served as controls. HPV DNA, predominantly type 16/18, was detected in the cervical biopsies of 96% of the CaCx patients, 80% of the CIN III patients, and 65% of the CIN I-II patients. In contrast only 9% of the cervical biopsies from the control group contained detectable HPV 6, 11, 16, or 18 DNA. A high proportion of the women with cervical abnormalities had evidence of concurrent vaginal and/or vulval papillomavirus involvement. The significance of these findings for routine screening and subsequent management of patients with HPV-associated cervical disease is discussed.

Combined renal and pancreatic dysplasia in the newborn.

A term female infant with early onset respiratory distress and pneumothorax died on day 20 with renal failure. Autopsy revealed dysplastic kidneys with occasional cortical cysts. The pancreas showed focal dysplasia with cysts and dilated ducts lying in primitive mesenchyme. A mild chronic inflammatory infiltrate was present in the dysplastic areas of kidneys and pancreas. The abnormalities in the affected organs were similar to those in two siblings described by Ivemark et al. in 1959 and designated "familial dysplasia of kidneys, liver and pancreas". Relationships to subsequently reported cases, including some with major congenital malformations, and the validity of this dysplastic triad as a disease entity are examined.

Multifocal tumorigenesis in the upper female genital tract--implications for staging and management.

A review of 128 cases of "primary" ovarian müllerian carcinoma treated at the King George V Memorial Hospital was undertaken to determine the relative frequency with which such tumors were associated with evidence of multifocal primary neoplasia. Of the 128 cases studied, 115 were invasive carcinomas and 13 were noninvasive or borderline ovarian tumors ("tumors of low malignant potential"). Eight of 10 borderline serous ovarian tumors (80%) and 37 of 75 invasive serous carcinomas (49%) exhibited evidence of independent primary neoplasia at more than one anatomical site in the biopsy material available for review. Many of these cases represented bilateral primary ovarian tumors, but autochthonous extraovarian neoplasia was also commonly encountered. A single borderline endometrioid ovarian tumor and six of 15 endometrioid carcinomas (40%) were associated with biopsy-proven multifocal primary tumorigenesis. These were predominantly neoplasms in one or both ovaries plus adenocarcinoma in the uterine corpus. Other histological types of malignant common epithelial tumors of the ovaries did not demonstrate any such tendency, highlighting major differences in pathogenesis between members of this loosely associated group of ovarian cancers. Our study suggests that gynecological endometrioid and serous malignancies are commonly multifocal and we feel this has significant implications for the way these neoplasms are staged and therefore treated.

Kimura's disease: a clinico-pathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia.

Kimura's disease is a chronic inflammatory condition producing subcutaneous tumour-like nodules chiefly in the head and neck region. It is characterized histologically by lymphoid follicles, intense aggregations of eosinophils, vascular proliferation and fibrosis. Superficial lymph nodes and parotid glands are sometimes involved. The lesions may persist unchanged for years and new ones are apt to occur. Recurrences are also common. There are no systemic manifestations apart from peripheral blood eosinophilia. The lesion has been recognised as a distinct clinicopathological entity in the Far East for over 40 years. We describe 21 cases of Kimura's disease and discuss its relationship to angiolymphoid hyperplasia with eosinophilia. Although the pathogenesis and etiology of both these lesions are unknown we believe that there are sufficient significant clinical and pathological differences to justify their separation.

Adenocarcinoma of the fallopian tubes: a clinicopathological study of eight cases.

Eight cases of clinically evident adenocarcinoma of fallopian tubes were encountered in King George V Memorial Hospital between Jan. 1950 and Dec. 1981. The tumours occurred predominantly in postmenopausal women and usually presented with vaginal bleeding and/or abdominal pain. A pelvic mass was invariably present. The tubal carcinomas were associated with peritoneal endosalpingiosis, ovarian serous neoplasms and epithelial lesions of the breast. Tumour spread was mainly by invasion of the tubal wall with direct involvement of adjacent organs and subsequent transcelomic dissemination. Prognostic factors are discussed. One patient presented with cerebellar disturbance which was considered to be a paraneoplastic syndrome. We believe this to be the first case of such a syndrome in association with tubal carcinoma and the case history is presented in detail.

Argyrophilia and endometrial carcinoma.

The incidence and patterns of argyrophilia in 25 endometrial carcinomas were analysed and correlated with other pathological features. In addition, the frequency with which argyrophilia was identified within nonneoplastic endometrium and cervix from this carcinoma group was compared with that of a control group of 25 patients without malignant disease. Using the Grimelius stain, argyrophilia was observed in 68% of endometrial carcinomas, although in 28%, only rare positive cells were identified. Seven tumours (six argyrophilic carcinomas and one small-cell undifferentiated carcinoma) were examined by electron microscopy, which revealed neurosecretory granules. Argyrophilia was also found at the ultrastructural level. ACTH and calcitonin were revealed by the immunoperoxidase technique in four of the nine argyrophilic tumours examined. Argyrophilic cells in nonneoplastic endometrium were found in 10% of the carcinoma cases and 8% of the control cases; this has not been described previously. No correlation could be established between the presence or degree of argyrophilia and histological type or grade of carcinoma, extent of tumour infiltration, or argyrophilia in nonneoplastic endometrium or cervix. We conclude that argyrophilia in endometrial carcinomas has no clinicopathological significance. The histogenesis of endometrial and cervical argyrophilic cells is discussed.

Premature hypergonadotropic ovarian failure: clinicopathological study of 19 cases.

During the 5-year period 1977-1982, 57 patients below 35 years of age with secondary amenorrhea were assessed for hypergonadotropic (primary) ovarian failure. The histological findings within the ovaries as well as pertinent clinical and laboratory correlates are described. Nineteen had diagnostic ovarian biopsies performed. The importance of this technique is stressed. The ovaries of 14 patients showed absence of primordial follicles (true premature menopause); three others showed "resistant ovary syndrome" characterized by the presence of primordial follicles but little or no follicular development (including a case of galactosemia, in which the associated ovarian failure has been ascribed to follicular atresia). The remaining two revealed florid chronic perifollicular inflammatory reactions in the presence of both primordial and also developing follicles--one lymphoplasmacytic and the other granulomatous. The former has been previously suggested as evidence of an autoimmune process, but the latter has not hitherto been reported.

Early adenocarcinoma of the fallopian tubes. A case for multifocal tumorigenesis.

Seven cases of carcinoma in situ and/or early invasive carcinoma of the fallopian tube found in association with serous carcinoma of the ovary are described. These cases are considered to represent examples of multicentric tumorigenesis in Müllerian epithelia which have a common origin and have responded to a common carcinogenic influence. Early neoplastic lesions of the tube may be overlooked unless the tube is serially blocked. If this is done, such lesions may be expected in 5-10% of patients with serous carcinoma of the ovary. Criteria for diagnosis of carcinoma in situ are discussed, viz., a noninvasive proliferative epithelial lesion with disorganization of cellular relationships, significant mitotic activity and nuclear atypia (especially prominence of nucleoli). Criteria for diagnosis of invasive carcinoma of fallopian tube are also referred to particularly with respect to establishing a primary site of tumor origin in cases where masses are present in both ovaries and tubes.

Mucinous ovarian tumors with giant cell mural nodules. A report of two cases.

Two cases are reported in young women of a variant of ovarian mucinous tumor with giant cell mural nodules. The first tumor was a predominantly proliferating mucinous tumor with a giant cell mural nodule adjacent to foci of haemorrhage and reactive vascular proliferation. No invasive carcinoma could be identified. In the second tumor, which was examined by electron microscopy, a sharp demarcation was observed between typical invasive mucinous carcinoma and areas which, on low-power examination, were indistinguishable from malignant giant cell tumor of soft parts. Closer examination revealed, in these latter areas, an intimate mixture of anaplastic, predominantly mononuclear tumor cells and multinucleate osteoclast-like giant cells. The histogenesis of these lesions is suggested as being reactive to the tumor cells or their products and is discussed with reference to similar tumors reported from the ovary and other sites in the body.