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[Mixed müllerian tumours of the endometrium. About four cases developed on tamoxifen treatment].

We report four cases of mixed müllerian tumors of the endometrium arising in postmenopausal women taking tamoxifen for breast carcinoma. Various histopathologic features were encountered: one müllerian adenosarcoma and three malignant mixed müllerian tumors so called carcinosarcomas (in one case the sarcomatous component was homologous composed of tissues normally found in the uterus, while the others were heterologous because they were containing some elements normally not found in the uterus). Concern has been raised about prolonged tamoxifen treatment and subsequent occurrence of endometrial adenocarcinoma. Then, attention has been drawn through high-risk histologic subtypes including poorly differentiated patterns, uterine sarcomas and such mixed müllerian tumors. There is no consensus regarding internal surveillance of women receiving tamoxifen. The usefulness of pelvic sonography has not been demonstrated.

Aged↗

Rhabdomyosarcoma of the uterus: report of two cases, including one of the spindle cell variant.

Most uterine sarcomas fall into the category of leiomyosarcoma, endometrial stromal sarcoma, or undifferentiated sarcoma. Pure rhabdomyosarcomas are extremely rare, although a rhabdomyosarcomatous element may be present as a component of an adenosarcoma or carcinosarcoma (malignant mixed müllerian tumor). This report describes two uterine rhabdomyosarcomas in 28- and 67-year-old women. These were of spindle cell and pleomorphic types, respectively. At presentation the pleomorphic rhabdomyosaroma was stage IV, exhibiting massive pelvic and abdominal dissemination that mimicked an ovarian neoplasm. The spindle cell rhabdomyosarcoma was stage I, being confined to the uterus. Grossly, both uterine tumors had a polypoid appearance. Immunohistochemically, tumor cells were positive with the skeletal muscle markers sarcomeric actin, myoglobin, and myoD1. The patient with stage IV disease died within a short time of diagnosis and the other patient is alive and well at 2 years' follow-up. This report adds to the published literature on uterine rhabdomyosarcomas. This is the first reported uterine case of the spindle cell variant of embryonal rhabdomyosarcoma. Based on these cases and the published literature, rhabdomyosarcoma, especially the pleomorphic variant, appears to be a very aggressive neoplasm with an extremely poor prognosis. Immunohistochemical demonstration of skeletal muscle differentiation is necessary for a definitive diagnosis.

Actins↗

Pure cystic nephroblastoma of the ovary with a review of extrarenal Wilms' tumors.

A second case of pure ovarian extrarenal Wilms' tumor (EWT) is presented. A clinical stage Ic tumor occurred in the right ovary of a 21-year-old female and corresponded to a 19-cm multilocular mass which histologically was a cystic, partially differentiated Wilms' tumor, closely resembling the highly differentiated metanephric adenoma. This pattern is reported for the first time in an ectopic location. At the interface between epithelial nests and ovarian tissue, plaques of alpha-inhibin positive cells were detected that corresponded to foci of peripheral stromal luteinization. Differential diagnosis with entities such as retiform Sertoli-Leydig cell tumors and with adenosarcoma should be made. The literature on EWT is also reviewed.

Adult↗

Adenomyomatosis of endometrium and endocervix-a hamartoma.

A 44-year-old woman was found to have a sessile lesion replacing most of her endometrium and endocervical mucosa, consisting of an intimate admixture of endometrial glands, endometrial stroma, and smooth muscle. On the basis of a previous curettage, it was concluded that the lesion had been present for at least seven years. The lesion is considered to be related to the pedunculated adenomyomatous polyp. The possibilities that it may represent adenomyosis, neoplasm, or hamartoma are discussed, and the latter is considered to be the most likely possibility. The presence of smooth muscle distinguishes the present case from the recently described and probably closely related entities of papillary adenofibroma, benign müllerian tumor, and müllerian adenosarcoma.

Adult↗

Uterine angiosarcomas: a morphologic and immunohistochemical study of four cases.

The clinical, gross, microscopic, and immunohistochemical features of four examples of exceedingly rare uterine angiosarcomas reviewed at the Armed Forces Institute of Pathology between 1970 and 1997 are presented. One of the cases described has been reported previously. Based on our findings and a review of the literature, uterine angiosarcomas are aggressive lesions that occur predominately in peri- and postmenopausal women with uterine bleeding and anemia. Grossly, the lesions are of large size at initial presentation with deep myometrial extension. Histologically, the lesions demonstrate characteristic features of angiosarcoma and, when associated with a benign smooth muscle proliferation within the uterus, tend to demonstrate an epithelioid morphology. The immunohistochemical reactivity with CD31, factor VIII, and CD34 and lack of reactivity with smooth muscle actin, keratin, and estrogen receptor confirm the vascular nature of these lesions and exclude the differential diagnostic considerations of carcinosarcoma (MMMT), leiomyosarcoma, adenosarcoma, and hemangiopericytoma. The overall survival of these lesions is poor; the majority of women die of disease within 1 year of diagnosis.

Aged↗

A clinicopathologic analysis of atypical proliferative (borderline) tumors and well-differentiated endometrioid adenocarcinomas of the ovary.

Atypical proliferative (borderline) endometrioid tumors (APTs) and well-differentiated endometrioid carcinomas of the ovary constitute a spectrum of morphologically diverse proliferative tumors. There is currently no agreement on the criteria for distinguishing them. We report the clinicopathologic features of 56 proliferative endometrioid tumors focusing on the criteria for invasion, the clinical significance of microinvasion and cytologic atypia, and prognosis. Endometriomas, adenofibromas, adenosarcomas and moderately to poorly differentiated carcinomas were excluded, as were patients with concurrent endometrioid carcinoma of the endometrium. The tumors were classified as atypical proliferative tumor (APT) (33 tumors), APT with intraepithelial carcinoma (high-grade cytology in a tumor lacking stromal invasion) (three tumors), APT with microinvasion (invasion <5 mm) (five tumors), and invasive carcinoma (invasion > or = 5 mm) ( 15 tumors). All tumors were confined to the ovary (stage I). In 50 patients, the tumor involved one ovary, and in three patients, the tumors were bilateral. The predominant growth pattern was adenofibromatous in 29 tumors and glandular or papillary in 27 tumors. In 8 (24%) of 41 APTs, areas of benign adenofibroma were identified, and in 13 (87%) of 15 carcinomas, areas of associated APT were identified. Stromal invasion was manifested by confluent glandular growth in all 15 invasive carcinomas and all tumors with microinvasion. Destructive infiltrative growth was also present in 2 (13%) of 15 carcinomas. Confluent glandular growth was the most common manifestation of stromal invasion and therefore served as the best criterion for the diagnosis of carcinoma. Squamous differentiation was observed in 24 tumors, and mucinous differentiation was seen in 20 tumors and was most often seen in APTs. Endometriosis was present in 14 patients with APTs and one patient with carcinoma. Four patients had hyperplasia or atypical hyperplasia of the endometrium. One patient with an APT had a concurrent peritoneal serous neoplasm. Twenty-one patients had available clinical follow-up. Twenty (95%) of 21 patients, including six with invasive carcinoma, two with microinvasion, one with intraepithelial carcinoma, and 11 with APT were alive with no evidence of disease with a mean follow-up of 47 months. One patient with carcinoma had recurrent tumor after 46 months and was alive 40 months after resection of the recurrent tumor. In this large series of proliferative endometrioid tumors, all were stage I and only one patient had a recurrence. Most carcinomas contained evidence of a precursor APT, and in some APTs, an associated benign adenofibroma was identified. Microinvasion or intraepithelial carcinoma occurred in 19% of APTs. This finding likely reflects the various stages of endometrioid carcinogenesis in the ovary. For clinical management, we suggest that these tumors be divided into two categories-APTs and well-differentiated carcinoma-because based on the available data, cytologic atypia and microinvasion appear not to affect the prognosis.

Adolescent↗

Uterine and vaginal effects of unopposed ultralow-dose transdermal estradiol.

OBJECTIVE: To investigate uterine effects of unopposed ultralow-dose transdermal estradiol administered to postmenopausal women for 2 years. METHODS: Postmenopausal women (n = 417), aged 60-80 years, with a uterus and with bone mineral density that was normal for age (z score >or=-2.0) were randomly assigned to receive unopposed transdermal estradiol (14 microg per day) or identical placebo patch. We evaluated effects on endometrial histology, vaginal bleeding, and vaginal epithelial cell maturation. RESULTS: At baseline, estradiol and placebo groups were similar in age (67 +/- 5 years) and in median baseline serum estradiol level (4.8 pg/mL, interquartile range 2.7, 8.0 pg/mL). In the estradiol group, median estradiol level increased to 8.6 pg/mL, (interquartile range 4.4, 13.9 pg/mL, P < .001). In the estradiol group, focal atypical endometrial hyperplasia developed in 1 woman, and adenosarcoma of the uterus developed in 1 woman. The placebo group had no endometrial hyperplasia. Endometrial proliferation occurred in 8.5% of the estradiol group and in 1.1% of the placebo group (P = .06). Incidence of vaginal bleeding was 12.4% in the estradiol group and 8.6% in the placebo group (P = .3). Vaginal epithelial cells showed greater maturation in the estradiol group than in the placebo group (P < .001) but less than typically observed with standard doses of estrogen. CONCLUSION: During 2 years of treatment with ultralow-dose unopposed estradiol, treatment and placebo groups had similar rates of endometrial hyperplasia, endometrial proliferation, and vaginal bleeding. This therapy apparently causes little or no endometrial stimulation. LEVEL OF EVIDENCE: I.

Administration, Cutaneous↗

Uterine adenomyoma: a clinicopathologic review of 26 cases and a review of the literature.

The aim of this study is to highlight the importance of diagnosing uterine adenomyoma and help in differentiating it from other sinister lesions. Adenomyoma of the uterus is a circumscribed nodular aggregate of benign endometrial glands surrounded by endometrial stroma with leiomyomatous smooth muscle bordering the endometrial stromal component. It may be located within the myometrium, or it may involve or originate in the endometrium and grow as a polyp. A retrospective analysis of 26 consecutive cases of uterine adenomyomas diagnosed in the Department of Pathology, Government Medical College, Chandigarh from January 1994 to December 2004 was done, and their clinical and histological features were analyzed. The criterion used for case identification was a circumscribed mass composed of benign endometrial glands with a stromal component consisting of endometrial type stroma surrounded by leiomyomatous smooth muscle. Mitotic figures were counted within 50 high-power fields (hpf) and recorded as the highest number per 10 hpf. The age of the patients ranged from 22 to 60 years (mean age, 41 years). The most common presenting symptom was abnormal vaginal bleeding (n = 15). Thirteen patients underwent panhysterectomy; 7, total hysterectomy; 1, subtotal hysterectomy; 4, polypectomy or tumor removal; and 1, curettage. Of the 26 cases of adenomyoma, 24 were in the corpus, 1 was in the cervix, and 1 was in the broad ligament. An associated leiomyoma was noted in 12 cases (46.9%). The adenomyomas were firm in consistency and, on cut section, showed a gray-white surface. Five tumors showed cystic spaces filled with dark brown material. On microscopic examination, the tumors were well demarcated from the surrounding structures. The endometrial glands were mostly tubular and showed relatively regular spacing from each other without any back-to-back arrangement. The glands were lined by benign proliferative pseudostratified columnar epithelium. An occasional typical mitotic figure was noted in these glands in a few cases. The glands were surrounded by endometrial stroma which was compact and spindly. This stroma was, in turn, bordered by leiomyomatous smooth muscle. Thick-walled blood vessels were commonly observed. One to two typical mitotic figures per 10 hpf were noted in the endometrial stroma in few cases; however, no mitosis was noted in the myometrial component. Associated adenomyosis was also noted in 8 cases (30.8%). Adenomyomas have to be distinguished from a number of other lesions, for example, adenomyosis, leiomyoma with entrapped glands, atypical polypoid adenomyoma, endometrial polyps, adenofibroma, and adenosarcoma. This study highlights the importance of correctly identifying this fairly common entity and helps to distinguish adenomyoma from other similar appearing benign and malignant lesions.

Adenomyoma↗

Ovarian serous cystadenofibroma with stromal sex cord elements: report of a unique case.

This report describes an ovarian serous cystadenofibroma, in a postmenopausal woman, which exhibited extensive sex cord differentiation, in the form of solid and hollow tubules resembling Sertoli cell elements, within the stroma. The sex cord elements, which were located just beneath the epithelium, were positive with both alpha inhibin and calretinin and negative with epithelial membrane antigen. They were also CD56 positive but negative with other neuroendocrine markers. True sex cord structures or sex cord-like elements have been described in ovarian adenosarcomas and pure stromal tumors, especially fibromas. However, as far as we are aware, these elements have not been reported in a serous cystadenofibroma. The endometrium exhibited simple hyperplasia, perhaps secondary to estrogenic activity of the sex cord elements. We discuss the significance of CD56 positivity of the sex cord elements.

Adenofibroma↗

Efficacy of adjuvant CYVADIC chemotherapy in early-stage uterine sarcomas: results of long-term follow-up.

Data on adjuvant chemotherapy in early-stage uterine sarcomas are conflicting and most often based on small patient groups with relatively short duration of follow-up. Approximately 60% of patients present with stage I disease with an overall 5-year survival of 30-50% when treated with surgery alone. This study examines the efficacy and results of long-term follow-up of a multiagent chemotherapy regimen of cyclophosphamide, vincristine, doxorubicin, and dacarbazine (CYVADIC) as adjuvant treatment for patients with stage I uterine sarcoma. Between 1982 and 1999, 24 evaluable patients with completely staged uterine sarcomas received adjuvant multiagent chemotherapy with vincristine sulfate (1mg /m(2)) on days 1 and 4, doxorubicin (40 mg /m(2)) and cyclophosphamide (400 mg /m(2)) on day 2, and dacarbazine (200 mg /m(2)) on days 1 through 4 for a total of nine monthly cycles or until recurrence of disease was documented. Survival distributions were calculated by the Kaplan-Meier method, and statistical significance was determined with the log-rank test. Factors significant on univariate analysis were analyzed in a multivariate fashion using Cox proportional hazards model. The histologic distribution of patients was 46% leiomyosarcoma, 33% mixed mullerian tumors, 13% stromal sarcomas, 4% adenosarcomas, and 4% hemangiosarcoma. The patients received 206 of a planned 216 cycles of chemotherapy. The median follow-up of the patient population was 93 months (range 11-213 months). Eight patients (33%) developed recurrent disease. The median time to recurrence was 19 months (range 7-184 months). The estimated survival for the entire group was 88, 75, and 69% at 2, 5, and 15 years, respectively. Factors that did not affect survival included age, histology, and tumor grade. Four patients required dose reductions secondary to grade 2-3 toxicities (hematologic). Grade 1 neurotoxicity was observed in six patients (25%) and grade 2 neurotoxicity in one patient (4%). Adjuvant CYVADIC chemotherapy appears to be safe and well tolerated in patients with stage I uterine sarcomas. Our data provide information on the longest duration of follow-up ever reported and suggests that CYVADIC may have a potential role in the adjuvant treatment of early-stage uterine sarcoma.

Adult↗

Extrauterine low-grade endometrial stromal sarcoma: report of three cases.

Three cases of rare low-grade endometrial stromal sarcoma of the extrauterine tissue are presented. Each one occurred in the ovary, pelvic and abdominal cavities. Two were associated with endometriosis. Histologically, the tumors were characterized by an infiltrative and diffuse proliferation of uniform round or oval cells, abundant small vessels, low mitotic activity, the presence of foam cells and vascular invasion. Immunohistochemically, all tumors expressed vimentin, muscle markers (desmin, muscle-specific actin and alpha-smooth muscle actin) and progesterone receptors. Two tumors were diploid and one was aneuploid by flow cytometry. All patients were well with no evidence of disease 16-39 months after surgery. It is suggested that this neoplasm may arise with or without endometriosis under hormonal influence. This rare variant of Müllerian tumors should not be confused with adenosarcoma and soft tissue tumors, such as smooth muscle tumors and solitary fibrous tumor.

Abdominal Neoplasms↗

"True" versus "apparent" stage I epithelial ovarian cancer: value of frozen section analysis.

The aim of this prospective study was to determine the clinical benefits of introducing peroperative frozen section analysis into the surgical management policy of women referred with an adnexal mass suspicious of ovarian cancer. All women surgically managed at the Northern Gynaecological Oncology Centre, Gateshead, UK, between July 1, 2002, and June 30, 2003, where frozen section analysis had been utilized were included for analysis. Correlation was determined between cases surgically staged following the frozen section result and the clinical need for staging based on the pathologic diagnosis from the paraffin section. During the 12-month period, 130 women underwent frozen section analysis. Paraffin section diagnoses included 74 benign tumors, 11 borderline tumors, 34 primary epithelial cancers, 5 nonepithelial cancers, and 6 metastatic tumors. All primary epithelial ovarian cancers were correctly identified as requiring a staging procedure based on the frozen section result. Four of seventy-four cases reported as benign on frozen section analysis were underdiagnosed; two were later diagnosed on paraffin section as borderline tumors and a further two as malignant (one low-grade adenosarcoma and one primary peritoneal cancer). Of the 130 cases, 55 (42.3%) underwent a staging procedure based on the frozen section result. The value of frozen section analysis in determining the need for the performance of a staging procedure had the following statistical test results: sensitivity = 92%, specificity = 88%, positive predictive value = 82%, and negative predictive value = 95%. Excluding the borderline tumors, metastatic tumors, and primary peritoneal tumor where staging did not impact subsequent clinical management, the statistical test results for frozen section analysis in determining the need for a staging procedure were sensitivity = 97%, specificity = 95%, positive predictive value = 90%, and negative predictive value = 99%. The clinical benefits of introducing frozen section analysis in the surgical staging policy of women with an adnexal mass suspicious of ovarian malignancy included avoidance of a surgical staging procedure in 95% of cases identified on paraffin section analysis to be benign. This benefit was without compromising the avoidance of chemotherapy in true stage I epithelial ovarian cancer cases. Additional benefits included the confirmation of malignancy where extraovarian lesions were suggestive but not indicative of malignant disease, and the intraoperative identification of metastatic disease of nonovarian origin.

Female↗

Sarcoma of breast, with particular reference to its origin from fibroadenoma.

Thirty-nine sarcomas of breast are described. The patients' ages ranged from 26 to 78 years but most patients were middle-aged or elderly. Evidence is adduced that 16 of the tumours arose from pre-existing fibroadenomas and others very probably did so. Many tumours were adenosarcomas and one was a carcinosarcoma which preserved its structure in a lymph-node metastasis. Recurrences were mostly local, but a small group had a much graver prognosis. A pleomorphic and giant-cell structure usually meant a high degree of malignancy but the significance of cartilaginous or bony metaplasia, present in seven tumours, was less certain. It is wise to regard all ;fibroadenomas' of the breast occurring in patients over 40 years of age as at least potentially malignant, and these should be examined histologically with care, particularly since sarcomatous change may be present in one part only and therefore easily overlooked.

Adenofibroma↗

My approach to the interpretation of endometrial biopsies and curettings.

A major proportion of the workload in many histopathology laboratories is accounted for by endometrial biopsies, either curettage specimens or outpatient biopsy specimens. The increasing use of pipelle and other methods of biopsy not necessitating general anaesthesia has resulted in greater numbers of specimens with scant tissue, resulting in problems in assessing adequacy and in interpreting artefactual changes, some of which appear more common with outpatient biopsies. In this review, the criteria for adequacy and common artefacts in endometrial biopsies, as well as the interpretation of endometrial biopsies in general, are discussed, concentrating on areas that cause problems for pathologists. An adequate clinical history, including knowledge of the age, menstrual history and menopausal status, and information on the use of exogenous hormones and tamoxifen, is necessary for the pathologist to critically evaluate endometrial biopsies. Topics such as endometritis, endometrial polyps, changes that are induced by hormones and tamoxifen within the endometrium, endometrial metaplasias and hyperplasias, atypical polypoid adenomyoma, adenofibroma, adenosarcoma, histological types of endometrial carcinoma and grading of endometrial carcinomas are discussed with regard to endometrial biopsy specimens rather than hysterectomy specimens. The value of ancillary techniques, especially immunohistochemistry, is discussed where appropriate.

Algorithms↗

Atypical polypoid adenomyoma of the uterus. An immunohistochemical study of a case.

We studied one case of atypical polypoid adenomyoma of the uerus immunohistochemically using antisera against keratins, vinentin, S-100 protein, desmin and actin. The stromal cells were reactive with anti-actin and antidesmin antibodies suggesting a muscular phenotype and confirming previous ultrastructural data. Immunohistochemical investigations have proved to be useful in differential diagnosis of APA with invasive adenocarcinoma, adenosarcoma and adenofibroma of the endometrium.

Actins↗

Polypoid adenomyomas: sonohysterographic and color Doppler findings with histopathologic correlation.

OBJECTIVE: This study was undertaken to describe the sonographic features of polypoid adenomyomas of the uterus and to determine the diagnostic role of sonohysterography and color Doppler sonography in the evaluation of these lesions. METHODS: The sonographic findings for 46 histologically proved cases of polypoid adenomyomas of the uterus, accumulated over 10 years, were reviewed retrospectively. The pathologic diagnoses included typical polypoid adenomyoma (n = 36), atypical polypoid adenomyoma (n = 7), and low-grade adenosarcoma arising in polypoid adenomyoma (n = 3). RESULTS: Of 46 total uterine tumors, 31 were in the corpus, 12 were in the fundus, and 3 were in the isthmus. The mean tumor size was 3.5 cm (range, 0.5-9 cm). The tumors were polypoid in 30 cases, pedunculated in 11 cases, and sessile in the remaining 5 cases. Of the pedunculated tumors, 5 protruded into the endocervical canal and 2 had prolapsed into the vagina. Three distinct sonographic patterns were identified with respect to the presence of cystic areas: a solid mass (pattern 1) in 12 cases, a solid mass with cystic areas (pattern 2) in 32 cases, and a predominantly cystic mass (pattern 3) in 2 cases. The characteristic sonographic features of polypoid adenomyomas included heterogeneous or homogeneous isoechogenicity relative to the myometrium, a smooth surface, a poorly defined margin with the underlying myometrium, hemorrhagic foci, posterior shadowing, a single vascular pedicle entering the mass, and associated adenomyosis in the myometrium. CONCLUSIONS: Knowledge of the sonographic appearance of polypoid adenomyomas may facilitate diagnosis and may help distinguish these tumors from other polypoid uterine tumors.

Adenomyoma↗

Tamoxifen and the endometrium: review of 102 cases and comparison with HRT-related and non-HRT-related endometrial pathology.

Tamoxifen, a synthetic anti-estrogen that paradoxically acts as a partial estrogen agonist on the endometrium, is associated with an increased frequency of proliferative endometrial lesions, including hyperplasias, neoplasms, and polyps. Tamoxifen-related polyps are characteristically multiple and fibrotic. A variety of metaplasias and periglandular stromal condensation may be seen. Relatively few articles have focused on the descriptive morphology of the full range of tamoxifen-associated lesions. The present study further defines the histologic features in both endometrial polyps and nonpolyp endometrium. One hundred and two specimens (including 50 polyps) were reviewed using hormone replacement therapy-related endometrial specimens and conventional polyps as the control groups. The most characteristic findings of tamoxifen-associated lesions included polarized glands along the long axis of polyps (40%), a cambium layer (72%), frequent and diverse metaplasias, staghorn glands (36%), myxoid degeneration (12%), and small glands (36%). Similar morphologic features were identified in the hormone replacement therapy and control groups but to a variable, lesser extent. Overall, the tamoxifen group consisted of 18 cases of hyperplasia (11 complex, 7 simple) and one case each of adenofibroma, adenosarcoma, endometrial stromal sarcoma, and leiomyosarcoma. Although none of the features is diagnostic, the presence of diverse metaplasias, polarized glands, staghorn glands, and a cambium layer strongly suggest tamoxifen exposure especially if a number of these features are present concurrently within the same material.

Adult↗

Endometrial stromal sarcomas of the uterus with extensive endometrioid glandular differentiation: a report of three cases that caused problems in differential diagnosis.

Three cases of endometrial stromal sarcoma (ESS) with prominent glandular differentiation within the primary or recurrent tumors are described. Each case posed a problem in diagnosis and classification. The patients, who ranged in age from 41 to 47 years, presented with abnormal uterine bleeding, a pelvic mass, or a combination thereof. All the patients underwent hysterectomy with or without bilateral salpingo-oophorectomy. There was no evidence of extrauterine spread of tumor in any case. Polypoid tumors involved the endometrium in two cases, and in one of them, tumor deeply invaded the myometrium. The tumor in the third case was an infiltrative mass that was confined to the myometrium and its vessels. On microscopic examination, the tumors were low-grade ESSs that contained large numbers of endometrioid glands, which were benign appearing in two cases, and in the third varied from atypical to carcinomatous. In one of the cases in the first group, the glandular component was present only in recurrent tumor excised 10 years after hysterectomy; prominent foci of sex-cord-like differentiation were also present in the recurrent tumor. This patient was clinically free of tumor 27 months later; follow-up in the other two patients was uneventful. ESSs with prominent numbers of benign-appearing glands should be distinguished from adenomyosis, endometriosis, ESSs arising in adenomyosis or endometriosis, ESSs with sex-cord-like differentiation, and müllerian adenosarcomas. ESSs with carcinomatous glands should be distinguished from endometrial adenocarcinomas and malignant müllerian mixed tumors (carcinosarcomas). Extrauterine lesions that have been designated "aggressive endometriosis" may be examples of extrauterine ESS with prominent glandular differentiation.

Adult↗