Denys W. Fortune

Adenocarcinoma in situ of the cervix

SummaryAdenocarcinoma in situ (ACIS) of the cervix is rare and is frequently overlooked. To characterize this disease more fully, 21 cases were studied. All except two patients presented with abnormal smears. The distribution of ACIS was focal in two cases, multicentric in three, and diffuse and continuous in 15 (in one case it was unknown). The depth of crypt involvement varied from 0.5 to 4 mm and the volume was estimated to range from 0.25 to 1,500 mm3. ACIS should and can be distinguished from early (“microinvasive”) adencarcinoma in most cases by its limitation to the glandular field, by the constant admixture of neoplastic and normal glands, and by the lack of stromal response. Invasive adenocarcinoma cannot be excluded by target biopsy, the diagnosis of ACIS requiring conization. If the surgical margins are disease free, conization alone may be adequate therapy.

Low malignant potential tumors of the ovary: a study of 76 cases.

&NA; This study analyzes the clinical and pathological features of 76 patients who were diagnosed as having ovarian low malignant potential tumor over a 20‐year period: 39 (51.3%) of the tumors were mucinous, 29 (38.2%) serous, three (3.9%) endometrioid, and five (6.6%) mixed. Patients with serous tumors were significantly younger (mean age 40 years) than those with mucinous tumors (mean age 50.5 years). Serous tumors were more frequently bilateral (48.3%) than mucinous tumors (12.8%). The extent of tumor (FIGO stage) at the primary laparotomy was related to the prognosis: the survival of 14 patients with stage III and six patients with stage II tumor was significantly inferior to that of the 56 patients with stage I tumors (P < .01). Patients with stage III mucinous low malignant potential tumors and pseudomyxoma peritoneii fared badly. Four patients with stage II and 13 patients with stage III tumors had residual tumor after primary surgery. Five of these patients received no subsequent treatment of whom four are alive and well from 5.5 to 19 years after diagnosis. Three patients received pelvic radiotherapy. Eleven patients were treated with chemotherapy for residual or recurrent tumor but only one (9.1%) had an unequivocal response. (Obstet Gynecol 68:338, 1986)

Intravenous leiomyomatosis of the uterus: a clinicopathologic study of 22 cases.

Twenty-two cases of intravenous leiomyomatosis (IVL) of the uterus were examined at the Royal Womens Hospital, Melbourne, within a 22-year period. The mean age of the patients was 46 years (range, 23-66). Nine patients presented with a pelvic mass, nine with abnormal uterine bleeding, and one with prolapse of the uterus. In three cases, the presenting features were unknown. Surgical exploration confirmed the presence of a uterine mass (mean size, 7.8 cm) which in seven cases extended into the broad ligament. In only 11 cases was involvement of the myometrial vessels obvious on gross examination. However, no extension into the iliac veins or inferior vena cava was found. In seven cases, the tumor involved to a minimal degree the immediately adjacent or contiguous myometrial veins, and the diagnosis of IVL was made incidentally on microscropic examination. Of the 16 patients with follow-up (mean, 7.5 years) all are now disease free. Five years after the diagnosis of IVL, one patient (case 10) had a pulmonary smooth-muscle tumor resected. All 22 patients were treated by surgery alone, and to date none have died from disease. Our findings support the benign biologic behavior of IVL even in the presence of metastases.

Fibroepithelial polyps with atypical stromal cells (pseudosarcoma botryoides) of vulva and vagina. A report of 13 cases.

SummaryThirteen cases of female genital tract fibroepithelial polyps containing atypical stromal cells are described, eight arising from the vulva, a site of origin not previously reported. These tumours must be differentiated from sarcoma botryoides, which they resemble both grossly and microscopically. Although fibroepithelial polyps are benign, two of our cases recurred after incomplete excision.

Angiomyxomas of the umbilical cord.

Rare benign angiomyxomatous tumors of the umbilical cord, associated with a macerated stillbirth, are described. Only 12 cases of vascular tumor of the cord have been reported and only 2 of these were associated with fetal death. The histopathology of this unusual tumor is presented together with a review of the literature.

Mortality and two year outcome of infants of birthweight 500-1500 g: relationship with neonatal cerebral ultrasound data.

Cranial ultrasounds were performed on 218 (96%) of 227 liveborn infants of birthweight 500‐1500 g delivered in the Royal Womens Hospital, Melbourne, Australia, in an 18‐month period concluding in March 1982. Seventy‐two (31.7%) of the children died; 28 children (38.9%) had cerebroventricular haemorrhage, 35 (48.6%) showed no bleeding and there were nine (12.5%) with no data. Paired necropsy and ultrasound data were congruent in 22 (88%) of 25 children. One hundred and forty‐eight (95.5%) of 155 survivors were seen at 2 years of age. Forty‐one (28%) had cerebroventricular haemorrhage; nine children (6%) had both ventricular dilatation and haemorrhage and two had ventricular dilatation alone. Apart from a marginal advance in gestation and higher number of immigrant and less educated mothers in children without cerebroventricular haemorrhage, all other perinatal, biographical and social variables between those with haemorrhage and those without were similar. The major handicap rate overall was 14.2% (21 patients). The children with cerebroventricular haemorrhage had a trend for greater prevalence of handicap and lower mean Bayley psychological scores. This was even more evident with ventricular dilatation being present. Of children with major handicap 57.1% (12/21) had normal serial ultrasound findings during their primary hospitalization. Major handicap occurred in 15% (3/20) of children with grade 1 haemorrhage, 23.5% (4/17) with grade 2 or 3 bleeds and 25% (1/4) of those with grade 4 haemorrhage. Laterality of cerebral palsy did not correlate with ultrasound findings. Ultrasound findings did not improve statistical prediction of deaths or major handicap.

Fetoplacental Function and Antenatal Complications When the Fetus is Malformed

Summary: The patterns of maternal urinary oestriol excretion in 115 pregnancies in which the fetuses were malformed are presented. The incidences of antenatal complications, fetal distress in labour and Caesarean section are analysed and compared with those in a control series of 693 pregnancies in which the fetuses had similar malformations.

Pathology of the vulva.

Increasing agreement is being reached among the International Society for the Study of Vulvar Disease, the International Society of Gynecological Pathologists, the World Health Organization, and the International Federation of Gynecology and Obstetrics concerning the terminology used to describe the abnormalities of and surgical procedures for vulvar disease. This paper focuses on Pagets disease of the vulva, which is beset with recurrence and a higher incidence of invasive disease than previously recognized, although diagnostic methods have improved. Agreements on the definition for and measurement of superficially invasive squamous cell carcinoma of the vulva have also been reached. The prognostic features of tumors likely to be associated with nodal metastases are being delineated with a view to the use of conservative surgery. The risks in underestimating a tumors capacity to metastasize are emphasized.

Endometrial carcinoma in gonadal dysgenesis with and without estrogen therapy

2 cases of endometrial carcinoma arising in patients with gonadal dysgenesis are reported. A 29-year-old woman originally presenting with menorrhagia was found to have developed Grade 1 adenocarcinoma 5 years later. The patient showed some features of Turners syndrome, and analysis of blood lymphocytes revealed chromosomal mosaicism. Total hysterectomy and salpingo-oophorectomy were performed, and an extensive adenosquamous tumor was found. The patient has shown no evidence of tumor recurrence for 13 years. A 2nd patient, presenting with menorrhagia at 21 years of age, was found to have focal adenomatous hyperplasia Grade 2 within a year. She was placed on cyclic hormone therapy. 6 years after 1st examination Grade 1 adenocarcinoma with squamous metaplasia was diagnosed and removed by hysterectomy with wedge resection of the ovaries. This patient has also remained well for 7 years after the surgery. Perhaps the development of endometrial carcinoma in these cases, theoretically improbable because of the infantile uteri, is due to the high levels of unopposed endogenous estrogens. In light of this possibility, cyclic estrogen-progestogen therapy might be more appropriate than the traditional estrogen replacement therapy for treatment of cases of gonadal dysgenesis.

Obstetric complications when the fetus has congenital heart disease

Abstract Maternal urinary estriol excretion was subnormal in 9 of 20 pregnancies in which the fetal heart was malformed. In 16 of the 20 patients, estriol measurement was indicated by clinical conditions knows to be associated with low estriol excretion. Such serious fetal defects as complete transposition of the great vessels, Fallots tetrad, and aortic atresia were found to be compatible with normal estriol excretion.