Andrew G. Östör

Microinvasive adenocarcinoma of the cervix: A clinicopathologic study of 77 women

Objective To prove that microinvasive adenocarcinoma of the cervix exists and, like its squamous counterpart, carries an excellent prognosis. Methods Seventy-seven women with microinvasive adenocarcinoma of the cervix were seen from 1971 to 1995. Microinvasion was defined as depth of invasion or tumor thickness of at most 5 mm. Microscopic assessment was made on punch biopsies, serially sectioned conization specimens, and extensively sampled hysterectomy specimens. Results Most of the women had abnormal Papanicolaou smears. We made definitive diagnoses on conization specimens in 49 women, hysterectomy specimens in 22, and colposcopically directed punch biopsies in six (there being no residual disease in the subsequent conizationhysterectomy specimens). The length of microinvasive adenocarcinomas ranged from 0.8 to 21 mm, and the volume was between 3 and 1000 mm.3 The tumors were multicentric in 21 cases, but no true “skip” lesions were found. Overall, 58 cold-knife conizations were performed: the margins were free in 39 cases, involved in 18, and inconclusive in one. The one loop conization had involved margins. Definitive therapy included cold-knife conization in 16 women, combined with pelvic-node dissection in four. In the remainder of the women, we performed some type of hysterectomy. None of the 26 women who had radical hysterectomy had parametrial spread, and none of the 48 who had pelvic-node dissection or the 23 in whom one or both adnexa were removed had metastases. There have been two “recurrences” to date; one was an adenocarcinoma and the other a squamous cell carcinoma, both at the vault. Conclusion Microinvasive adenocarcinoma of the cervix is a clinicopathologic entity that appears to have the same prognosis, and should be treated in the same way, as its squamous counterpart.

Survey of congenital tumors in perinatal necropsies

Summary In an audit of 17,417 necropsies from 1939 to 1989 at the Royal Womens Hospital, Melbourne, Victoria, 46 congenital tumors comprising 24 teratomas, 8 vascular tumors, 6 neuroblastomas, three rhabdomyomas, two mesoblastic nephromas, one thyroid adenoma, one hepatic adenoma and one cerebellar medullo‐blastoma were found. The teratomas were generally large tumors and, because of their size and location, incompatible with extrauterine life. Ten teratomas were associated with polyhydramnios, three with obstructed labour, five of the fetuses were hydropic and four had malformations at sites distant to the tumor. Twenty teratomas occurred in stillbirths, half of whom were macerated. The vascular tumors were associated with polyhydramnios in three cases and hydrops fetalis in two. The neuroblastomas were all incidental findings. Four were intra‐adrenal, one was an adrenal tumor which had metastasized to the liver and the remaining case was a small paravertebral lesion. Two of the three rhabdomyomas were multiple and one of these occurred in a case of tuberous sclerosis. One of the mesoblastic nephromas occurred in a hydropic fetus who had the Arnold‐Chiari malformation. The thyroid and hepatic adenomas were small incidental lesions. The cerebellar medulloblastoma had led to marked hydrocephalus. Congenital tumors have different incidence, presentation and behaviour than those in childhood or adult life. The association of congenital tumors with congenital malformations and hydrops fetalis should always be remembered.

Endometrial Carcinoma in Young Women

Summary: Endometrial carcinoma in young women is uncommon. The majority of cases occur in women who have been taking oestrogen‐containing oral contraceptives, in those with the Stein‐Leventhal syndrome, or in those with gonadal dysgenesis treated by long‐term oestrogen replacement therapy. We have observed 4 women under 40 years of age with adenocarcinoma of the uterus in whom chromosomal abnormalities associated with Turners syndrome were confirmed only after the diagnosis of carcinoma had been made. None of these had received replacement oestrogen therapy. It is postulated that chromosomal abnormalities in young women with endometrial carcinoma are more common than previously thought. As the disease in this group appears to behave in a benign fashion, a conservative approach to therapy is advocated.

Alpha-fetoprotein-producing adenocarcinoma of the sigmoid colon with possible hepatoid differentiation

&NA; A patient with adenocarcinoma of the sigmoid colon producing alpha‐fetoprotein (AFP) is described. Hepatoid differentiation is suggested by the morphological appearance and production of hepatic‐type AFP. The possible histogenesis of such a tumor is discussed.

Placental site trophoblastic tumor. Report of three cases and review of the literature.

Three cases of placental site trophoblast tumor (PSTT) are added to 74 cases in the English language literature. One case presented with an anorexic syndrome, the other two with amenorrhea and abnormal vaginal bleeding. The three patients, whose lesions were confined to the uterus, were treated by total hysterectomy and their follow-up has been uneventful. Review of the literature showed that 62 patients with PSTT were alive and 15 had died, two as a complication of treatment. The diagnosis of PSTT may be difficult on a dilatation and currettage specimen. Metastases are a powerful indicator of adverse outcome. Prognosis, as assessed on pathologic criteria, is unreliable; however, a mitotic count of more than five mitoses per 10 high power fields is significant. Surgery is the mainstay of treatment. Hysterectomy is generally indicated, but young patients who wish to remain fertile may be treated by conservative surgery. In a few cases of progressive disease, chemotherapy has achieved remission, but generally chemotherapy and radiotherapy are ineffective. Long-term follow-up is essential as PSTT may progress after years of remission. Serum human chorionic gonadotrophin (hCG) levels are the best available marker of disease, but the disease may still progress even if hCG levels are not raised.

BILATERAL PULMONARY AGENESIS

Summary Two cases of bilateral pulmonary agenesis in infants of 31 and 41 weeks gestation are reported. In addition to absence of both lungs, major abnormalities not previously reported were also found in other systems. It is concluded that these abnormalities are not due to chromosomal abnormality but no alternative can be offered.

Microinvasive adenocarcinoma of the cervix: a cytohistopathologic study of 40 cases.

Cervical smears obtained from 40 women with a histologic diagnosis of microinvasive adenocarcinoma (MIA) were reexamined for features of invasion. In our study MIA was defined as stromal invasion by adenocarcinoma cells to 5 mm or less beyond a surface epithelium and without lymphovascular involvement. In 24 cervices, squamous carcinoma in situ was a coincidental histologic finding.

Collision Tumor: Serous Adenocarcinoma and Steroid Cell Tumor of the Ovary

Summary A “collision” tumor between a serous papillary adenocarcinoma and a steroid cell tumor of the ovary is described. No similar combination has been reported in the literature. The steroid cell component secreted testosterone, resulted in considerable virilization of the patient, and appears to have preceded the carcinoma by several years. It remains problematical whether the androgenic milieu may have predisposed to the development of the second, malignant, tumor.

Angiosarcoma of Breast Metastatic to the Ovary and Placenta

Summary: Angiosarcoma of the breast with spread to the ovary and placenta during a pregnancy 6 years after initial diagnosis is described. The ovarian lesion was discovered during Caesarean section. It is postulated that the pregnancy altered the biological behaviour of the tumor.

The accuracy and interobserver reproducibility of endometrial dating

Summary Although controversial, diagnosis of luteal phase defect (LPD) includes the morphological assessment of endometrial development. This study was conducted to determine if refresher training in the histological criteria could improve the accuracy and interobserver reproducibility of endometrial dating. Seventy‐eight endometrial biopsies were dated by a reference panel of two pathologists and then reviewed twice by a study panel of four pathologists. In the first review, usual practice was applied. Prior to the second review, they studied a standard document of histological criteria. Samples were dated as proliferative, secretory (post‐ovulatory day, POD), menstrual, and undatable. Accuracy levels based on the reference dating and agreement levels using s values were calculated per review and compared. The s for overall dating was 0.683 in the first review and 0.696 in the second. The respective first and second review s values were 0.736 and 0.771 for proliferative, and 0.794 and 0.764 for secretory. Amongst those dated as secretory in the first and second reviews, respectively, 31 and 28% were assigned the same POD by any two panellists, 68 and 63% were dated to within 1 day, and 77 and 71% were dated to within 2 days. Accuracy levels per panellist for overall dating were very high in both reviews but were low for individual PODs. Accuracy and interobserver reproducibility were unaffected by refresher training, suggesting the limits of histological dating have been reached.