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SMARCA4 gene encodes BRG1 , a member of the SWItch/sucrose non-fermentable protein family involved in epigenetic transcriptional regulation of important cellular processes. In the uterine corpus, SMARCA4 / BRG1 deficiency is associated with a novel class of undifferentiated uterine sarcomas, characterized by younger age onset, rhabdoid histology, focal phyllodiform architecture, high-risk pathologic findings, and dismal prognosis. Herein, we report a case of a 34-year-old Asian woman with a SMARCA4 / BRG1 -deficient uterine tumor fulfilling the clinicopathologic features of an undifferentiated uterine sarcoma. However, the tumor exhibited several unique features that have not been previously emphasized, including (1) conspicuous phyllodiform architecture recapitulating conventional adenosarcoma, (2) rhabdoid tumor cells forming cords and keratin-positive cohesive epithelial islands, and (3) cooccurrence with a spatially distinct and discrete endometrial complex atypical hyperplasia from the rest of the proliferation. By immunohistochemistry, the tumor cells were diffusely positive for synaptophysin, whereas BRG1 was lost. Pertinent molecular findings included frameshift mutations in the SMARCA4 gene, mutations in histone modification and chromatin remodeling genes, including KMT2C , ARID1B , KAT6A , and NCOR1 , and mutations in Wnt signaling involving APC and CTNNB1 . Copy number gain in MDM2 and CDK4 were also identified. The tumor mutation burden was intermediate (6.8/MB) and it was microsatellite stable. On balance, our case exhibited morphologic and molecular features that overlap with (1) an undifferentiated uterine sarcoma, (2) an adenosarcoma with sarcomatous overgrowth, and (3) a mixed adenosarcoma and undifferentiated endometrial carcinoma. These hybrid features further expand the molecular-morphologic spectrum of SMARCA4 / BRG1 -deficient uterine neoplasms., Competing Interests: C.H.W. is an NCI K12 clinician scientist fellowship awardee. The remaining authors declare conflict of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Mesonephric-like adenocarcinoma (MLA) of the ovary is a recently recognized, rare malignancy with aggressive clinical behavior, and is thought to originate from Mullerian epithelium with mesonephric transdifferentiation. Emerging evidence suggests that MLA may be classified as an endometriosis-associated neoplasm. The presence of a sarcomatous component within MLA is extremely rare, with common differential diagnoses including the spindle cell component of MLA, carcinosarcoma, as well as mixed Mullerian adenocarcinoma and adenosarcoma. Herein, we report a 58-year-old Chinese woman with bilateral ovarian solid-cystic masses. The left ovarian mass comprised a biphasic tumor with a predominantly high-grade sarcomatous component displaying heterologous mesenchymal differentiation, including liposarcoma, rhabdomyosarcoma and chondrosarcoma-like areas, with a null-type p53 expression. The epithelial component ranged from a bland appearance in areas diagnostic of adenosarcoma to a clearly invasive carcinoma, both with mesonephric-like phenotype, being negative for estrogen receptor, progesterone receptor, and Wilms' tumor 1, variably positive for paired box gene 8, GATA binding protein 3, and thyroid transcription factor 1, with a wild-type p53 expression. The differing p53 expression between the epithelial and sarcomatous elements mitigated against a diagnosis of carcinosarcoma. The right ovarian mass showed endometriosis with focal direct evidence of the development of malignancy within a benign endometriotic cyst, exhibiting the identical immunoprofile of MLA but originating as another malignancy. To the best of our knowledge, this case represents the first reported case of synchronous bilateral ovarian MLAs with separate origins, from high-grade Mullerian adenosarcoma and endometriosis respectively, which broadens the morphologic spectrum of MLA and provides further evidence supporting the Mullerian origin theory., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 by the International Society of Gynecological Pathologists.)

The objective for the study was to analysis the epidemiology of adenosarcoma, and independent prognostic factors and impact of lymph node dissection (LND) of uterine adenosarcoma. Cases of patients with primary adenosarcoma were obtained from the Surveillance, Epidemiology, and End Results (SEER) database from 2000 to 2016. Overall survival was analyzed by the Kaplan-Meier method and log-rank test. The differences in baseline covariates between the 2 groups were adjusted by inverse probability of treatment weighting method. The prognostic factors were identified by univariate and multivariate Cox regression analysis and hazard ratio and 95% confidence interval (CI) of covariates were also estimated. 1129 patients with pathological primary adenosarcoma between 2000 and 2016 were identified from the surveillance, epidemiology, and end results database. The only 4 patients were male. 1027 patients with primary uterine adenosarcoma, and 53.1% underwent LND and only 3.5% patients were with positive lymph node. Age, marital status, largest tumor size, tumor grade, T stage and chemotherapy were significantly correlated with survival. Race, tumor number, LND, and radiotherapy did not affect overall survival in patients. Inverse probability of treatment weighting-adjusted K-M curve showed that LND did not improve survival and lymph node metastasis (LNM) did not affect survival. The majority of primary adenosarcoma patients are female with high incidence of uterus and rare incidence of distant metastasis. Age, marital status, tumor size, T stage, grade, and chemotherapy are independent prognostic factors of uterine adenosarcoma. LNM was not a significant prognostic risk factor, and LND did not benefit survival., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Uterine sarcomas represent a clinical challenge because of their difficult diagnosis and the poor prognosis of certain subtypes. The aim of this study was to evaluate the expression of the special AT-rich sequence-binding protein 2 (SATB2) in endometrial stromal sarcoma (ESS) and other types of uterine sarcoma by immunohistochemistry. We studied the expression of SATB2 on 71 full tissue sections of endometrial stromal nodule, low-grade ESS, uterine leiomyomas and leiomyosarcoma, undifferentiated uterine sarcoma, adenosarcoma, and carcinosarcoma samples. Nuclear SATB2 expression was then evaluated in an extended sample set using a tissue microarray, including 78 additional uterine tumor samples. Overall, with a cut-off of ≥10% of tumor cell staining as positive, the nuclear SATB2 score was negative in all endometrial stromal nodule samples (n=10) and positive in 83% of low-grade ESS samples (n=29/35), 40% of undifferentiated uterine sarcoma (n=4/10), 13% of leiomyosarcoma (n=2/16), 14% of adenosarcoma (n=3/22), and 8% carcinosarcoma (n=2/25) samples. Furthermore, in ESS patients, direct comparison of nuclear SATB2 scores with clinicopathologic parameters and other reported biomarkers such as progesterone receptor and estrogen receptor showed that nuclear SATB2 was associated with PR expression and a decreased risk of disease-specific death (odds ratio=0.06, 95% confidence interval=0.04-0.81, P=0.04). Our data suggest that SATB2 could be a marker with relative sensitivity (83%) for distinguishing between endometrial stromal nodule and ESS with potential prognostic value., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 by the International Society of Gynecological Pathologists.)

Müllerian adenosarcoma is an uncommon biphasic malignant tumor most often occurring in the uterine corpus and derived from native surface endometrium. We report a case of intramural uterine adenosarcoma arising in association with adenomyosis, in the absence of tumor involving the surface endometrium. This is an extremely rare phenomenon, with only 8 other published cases of uterine corpus adenosarcoma in the absence of surface endometrial involvement, 5 originating in adenomyosis and 3 in adenomyomas. We review these cases. The current FIGO staging system for uterine adenosarcoma assumes origin from the surface endometrium and does not address the rare occurrence of intramural tumors without a surface endometrial component. Such tumors are problematic to stage and could potentially be overtreated, particularly if there is deep myometrial involvement., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 by the International Society of Gynecological Pathologists.)

Extraskeletal Ewing sarcoma presenting as intra-abdominal or pelvic disease in adult female patients is very rare and may lead to diagnostic difficulty due to clinical and histologic overlap with Mullerian adenocarcinomas, which are far more common. We report a case of an intra-abdominal Ewing sarcoma in a postmenopausal female patient whose clinical and radiological presentation closely resembled that of peritoneal carcinomatosis. Biopsy of an omental nodule revealed numerous histologic features suggestive of a Mullerian carcinoma, including gland-like rosettes, strong, diffuse PAX8 immunoreactivity and cytokeratin expression. After excluding other differential diagnostic considerations, the possibility that this might represent an intra-abdominal Ewing sarcoma was entertained. Reverse transcriptase polymerase chain reaction testing demonstrated the presence of an EWSR1-ERG fusion transcript, confirming the diagnosis. The differential diagnostic considerations when dealing with this unusual clinical scenario and the uncommon yet important pitfall of PAX8 immunoreactivity in Ewing sarcoma are discussed., Competing Interests: The authors declare no conflict of interest., (Copyright © 2019 by the International Society of Gynecological Pathologists.)

Dedifferentiated carcinoma is defined as undifferentiated carcinoma coexisting with a second component of FIGO grade 1 or 2 endometrioid carcinoma. It is a rare entity with highly aggressive behavior. Dedifferentiated carcinoma combined with another primary uterine tumor is even rarer. We describe a case containing 3 different morphologies comprised of a dedifferentiated carcinoma associated with a low-grade endometrioid carcinoma coexisting with a low-grade Müllerian adenosarcoma. We also used targeted genomic analysis to show all 3 components arise from the same founding clone and identify novel mutations that drive tumor progression.

Rationale: Uterine adenosarcoma (UA) with sarcomatous overgrowth (ASSO) is a rare and aggressive disease. Herein, wereported the case of a young patient with advanced uterine ASSO., Patients Concerns: A 29-year-old woman with the diagnoses of endometrial polyp and adenomyosis underwent hysteroscopic endometrial polypectomy for the giant endometrial polyp. Postoperative regular ultrasound scan indicated thickened endometriumand an ill-defined mass with continuous enlargement in the myometrium of the posterior wall of the uterus, which was considered as an adenomyoma. Two years after hysteroscopy, she was re-admitted due to lower abdominal distension and large pelvic mass. At that time, she had taken oral short-acting contraceptives for 2.5 years., Diagnoses: Magnetic resonance imaging (MRI) of the pelvis revealed an irregular mass with the size of 12*56*107 mm in the right annex area, without distinct border with the rectum, moreover, an uneven intrauterine echo that has no obvious boundary with uterine wall. Right ovarian cancer and adenomyoma were initially considered., Interventions: The patient received transperitoneal retroperitoneal pelvic combined with total viscera resection, including uterus, bilateral appendages and rectum, omentectomy, appendectomy, lymphadenectomy, and ileostomy. Postoperative pathology confirmed ASSO in the uterine cavity and muscular layer, the whole cervical duct and the right adnexal. She underwent 2 systemic chemotherapy sessions after the surgery. The chemotherapy regimen was ifosfamide 2.5 g day 1 to 3, with liposomal doxorubicin 40 mg day 1., Outcomes: The final diagnosis was uterine ASSO, International Federation of Gynecology and Obstetrics stage IVa. The patient has been following-up so far, with no progression., Lessons: Review of the case indicated that history of long-term oral short-acting contraceptives and giant endometrial polyps may be the high-risk factors for UA. For patients with high-risk factors, the follow-up ultrasound scan should be more frequently conducted. Moreover, 3D-ultrasound, MRI and outpatient hysteroscopy are recommended for routine screening. Placement of levonorgestrel-releasing intra-uterine system after hysteroscopy may be an effective intervention for patients with a high risk of giant polyps. Cluster of Differentiation 10, Estrogen receptor, Progesterone receptor, and nuclear antigen may be predictors for prognosis and selection of individualized treatment program.

Somatic endometrial carcinomas with trophoblastic components have only rarely been described. To better characterize this distinctive combination of histotypes, we report herein 4 new cases, representing the largest cohort reported thus far, and review previously reported cases. The 4 new patients ranged in age from 61 to 77 yr (mean, 68 yr). The first patient had a grade 2 endometrioid carcinoma, surgical International Federation of Gynecology and Obstetrics stage IA, that recurred 5 months later at the vaginal apex with purely choriocarcinoma elements, suggestive of unsampled trophoblastic areas in the uterus. The 3 other patients were all International Federation of Gynecology and Obstetrics stage III, and included 2 cases of dedifferentiated endometrial carcinoma with 40% and 20% choriocarcinoma components, and 1 case of grade 1 endometrioid carcinoma with a 40% choriocarcinoma component. Postoperative serum β-human chorionic gonadotropin was elevated in all patients. All received adjuvant combination chemotherapy, but all were dead of disease with distant metastases at an average of 11.75 mo (range, 7-16 mo) after primary staging. Data from our cases were combined with those from 24 cases that had previously been reported in the literature between 1972 and 2016. Analysis of this combined data indicates that endometrial carcinoma with trophoblastic component is a rare neoplasm that occurs primarily in postmenopausal patients. The trophoblastic component is most commonly a choriocarcinoma and the somatic component is most commonly an endometrioid carcinoma or an adenocarcinoma/carcinoma reported without further specification; the somatic component may be a diverse array of histotypes or histotype admixtures. Serum and/or urine β-human chorionic gonadotropin is elevated in almost all patients, and fluctuations of β-human chorionic gonadotropin generally correlated with tumor relapses or recurrences. The stage distribution and patient outcomes in the current and previously reported patients suggests that trophoblastic differentiation usually, but not invariably denotes clinical aggressiveness.

Müllerian adenosarcoma (MA) is an uncommon biphasic neoplasm of the female genital tract, composed of malignant stroma and benign epithelium. Little is known about the molecular and cytogenetic aberrations in MA pathogenesis, including those with progression to sarcomatous overgrowth (SO). Herein, we report all cases of MA in which karyotyping was attempted at our institution. Twenty-one samples from 20 subjects consisted of 15 primary (7 without SO, 8 with SO) and 6 metastatic MA, were cytogenetically investigated in our institution. Karyotypes were successfully obtained in 14/21 (67%) cases and 9 (45%) had cytogenetic aberrations. Two (1 MA with SO and 1 metastatic MA) were markedly complex, displaying extreme aneuploidy with numerous rearrangements. Seven (2 MA without SO, 3 MA with SO, and 2 metastatic MA) demonstrated noncomplex clonal aberrations, of which 5 (71%) included an abnormality involving chromosome 8. Two tumors had rearrangements at 8q13 and another 3 tumors had extra copies of chromosome 8. In 5 cases, a normal karyotype (46,XX) was obtained (2 MA without SO, 2 MA with SO, and 1 metastatic MA). Further study is warranted to explore the genetic mechanism by which chromosome abnormalities, particularly those at 8q13, contribute to MA tumorigenesis.

Sex cord-like differentiation has been well documented in endometrial stromal tumors. On the basis of the extent of sex cord differentiation, uterine stromal tumors with this kind of differentiation have been classified into 2 subgroups of endometrial stromal tumor with sex cord-like elements and uterine tumors resembling ovarian sex cord tumor. When extensive, this differentiation has been accompanied with indolent clinical behavior and rather good prognosis in most cases. Sex cord differentiation has been rarely observed in uterine mullerian adenosarcoma. Only 3 cases of such occurrence have been reported in the English literature. Herein, we report a case of uterine mullerian adenosarcoma extensively overgrown by uterine tumor resembling ovarian sex cord tumor in a young woman. The presence of an ovarian sex cord tumor component has been confirmed by immunohistochemistry. To the best of our knowledge, this is the second report of such a case in the English literature.

Mullerian adenosarcomas are uncommon tumors of the female genital tract characterized by a synchronous proliferation of benign glands and sarcomatous stroma. In general, uterine Mullerian adenosarcomas are associated with a low risk of recurrence. The presence of "stromal overgrowth" (SO), historically defined by an estimate of the volume of sarcoma growing independently of epithelium, is associated with deep myometrial invasion, presence of heterologous elements, and poor outcomes. Very rarely, the stromal component can harbor foci resembling ovarian sex cord tumors (FROSCT). The aim of this study was to determine whether the presence of an extensive FROSCT component in Mullerian adenosarcomas has an impact on survival, akin to more typical types of SO. Six patients were included in this study. Age ranged from 39 to 71 yr. Five patients presented with uterine lesions (4 intracavitary, 1 isthmic), and 1 was located in the ovary. Tumors ranging in size from 2.5 to 19 cm were all diagnosed as Stage I. Morphologically, all had prominent FROSCT-like components that comprised 60% to 90% of tumor volume. Immunohistochemically, the FROSCT component was positive for CAM 5.2, vimentin, WT1, CD56, α-inhibin, calretinin, androgen and progesterone receptors, α-actin, and desmin. All patients are alive without disease at 26 to 102 mo. Compared with adenosarcomas with typical forms of SO, FROSCT overgrowth is low grade and not associated with recurrence or metastasis in this small series. Therefore, Mullerian adenosarcoma with extensive FROSCT should not be equated with SO.

Adenosarcoma (AS) is a rare biphasic neoplasm of the female genital tract characterized by an admixture of benign glandular and low-grade mesenchymal components. The classic low-power growth pattern is periglandular stromal proliferation accompanied by a variable degree of cytologic atypia and mitotic activity. However, as cytologic atypia is an objective criterion, and the number of mitotic figures required for diagnosis is inversely proportional to stromal cytologic atypia, there is a relatively wide variation in the decisive factors used among pathologists to diagnose an AS. Furthermore, the exact number of microscopic fields sufficient for diagnosis and/or the size of the fields adjusted to a specific microscope are not well established. These uncertainties are still an occasional source of misclassification of AS. Our study was conducted to explore the role of immunohistochemistry in the diagnosis of AS. Eight ASs were retrieved and compared with 14 endometrial polyps and 14 atypical polypoid adenomyomas. Immunohistochemical stains for Ki-67, caldesmon, smooth muscle actin, desmin, and CD10 were performed on all cases. All AS had a polypoid growth pattern with a variable increase in periglandular stromal cellularity and stromal nuclear atypia. The mitotic activity ranged from 1 to 15/10 high-power fields, and all AS demonstrated a distinct increase in Ki-67-positive nuclei in the periglandular zone compared with the adjacent stroma, regardless of the mitotic count. The Ki-67-labeling index in periglandular zones was ∼20% compared with <5% in the adjacent stroma. This zonation was not observed in any case of atypical polypoid adenomyomas or endometrial polyps. None of the other stains (CD10, smooth muscle actin, desmin, and caldesmon) helped to differentiate between these entities. Thus, characteristic zonation by Ki-67-staining pattern is a helpful adjunct to the routine morphology in the diagnosis of AS, particularly in curettage specimens, which may lack some of the classic morphologic diagnostic features.

Uterine adenosarcomas are uncommon mixed Müllerian neoplasms, most commonly arising in the uterine corpus. A new Federation of International Gynecologic Organization staging system for these tumors has recently been implemented. This staging system is an improvement on the earlier generic application of the 1988 Federation of International Gynecologic Organization staging system for endometrial cancer to adenosarcoma. Herein, we report 3 uterine adenosarcomas with unusual features. For 2 of these, no specific staging guidelines are provided by either the earlier or, more importantly, the new staging system. The first case is of an adenosarcoma arising in the eutopic endometrium with involvement of underlying adenomyosis without myometrial invasion; the second originated in a mural adenomyoma in the absence of eutopic endometrial involvement; and the third case encompassed synchronous endometrial and extrauterine (peritoneal) neoplasms. Such cases are rare, and there is insufficient evidence to be definitive about staging. Thus, we suggest a descriptive reporting strategy for adenosarcomas with these unusual features. We also propose a reporting nomenclature for such cases to ensure standardization such that they can be adequately recorded in synoptic reporting protocols. This will facilitate reliable data collection such that an evidence-based staging system for these scenarios may be derived.

An unusual case of ovarian adenosarcoma arising from a smooth-walled serous cystadenoma is described. The ovary was replaced by a multiloculated, fluid-filled cyst without any solid or papillary areas. The malignant component was underdiagnosed during frozen section examination as benign cystadenoma because of the deceptively benign gross appearance of the tumor. On the permanent sections, a phyllodes-like pattern of stromal proliferation and periglandular condensation of atypical stromal cells with a mitotic count of 3 per 10 high-power fields was more apparent and led to the diagnosis of adenosarcoma. The malignant component could not be distinguished from the benign component using immunohistochemical analysis. To our knowledge, this is the first reported case of an adenosarcoma arising from a grossly benign cystadenoma and the third case in the literature of an adenosarcoma associated with a cystadeno(fibro)ma. This case also shows the challenges in differentiating adenosarcoma from a benign counterpart on both frozen and permanent sections.

Mullerian adenosarcoma is an uncommon, but not rare, mixed tumor containing a neoplastic but benign or mildly atypical epithelial element and a sarcomatous, usually low-grade, stromal component. The most common site is the uterine corpus but adenosarcoma also occurs in the cervix and ovary and more rarely in the vagina, fallopian tube, arising from peritoneal surfaces, or outside the female genital tract, for example in the intestine. Most uterine cases have a polypoid gross appearance, sometimes resulting in the formation of multiple polyps. Characteristic histologic features include a low power "phyllodes-like" architecture with leaf-like projections lined by a variety of benign Mullerian type epithelia, sometimes with squamous metaplasia. Intraglandular stromal protrusions are a characteristic feature. The stroma may be uniformly cellular but there is typically increased cellularity around the epithelial elements, resulting in the formation of a cambium layer. Using the World Health Organization definition, stromal mitotic activity of 2 or more per 10 high-power fields is required for a diagnosis of adenosarcoma but in practice the diagnosis is made with stromal mitotic activity less than this if the characteristic architecture and cambium layer is present. The stromal component is usually morphologically "low-grade" and of endometrial stromal or fibroblastic type (hormone receptor and CD10 positive). Sometimes it is high grade, resembling undifferentiated sarcoma. Additional features sometimes present include heterologous stromal elements or sex cord-like differentiation. Uterine adenosarcomas are, in general, low-grade neoplasms capable of local recurrence after polypectomy or hysterectomy and much less commonly distant metastasis. The 2 most important adverse prognostic factors, which sometimes coexist, are deep myometrial invasion and sarcomatous overgrowth; the latter is usually associated with morphologically "high-grade" stromal elements with loss of expression of hormone receptors and CD10. Adenosarcoma may be confused with a variety of lesions and one of the main differential diagnoses is adenofibroma in which the stromal component is, by definition, morphologically benign. However, occasional adenofibromas recur or even metastasize. As such, it has been suggested that all adenofibromas should be classified as adenosarcomas, albeit with low-malignant potential. Ovarian adenosarcomas are much more likely to exhibit malignant behavior than their uterine counterparts, probably due to the lack of an anatomic barrier to peritoneal dissemination.

Summary: A rare case of a clear cell adenocarcinoma and an adenosarcoma coexisting with a heterologous rhabdomyosarcoma in an endometriotic cyst of the ovary is reported. The tumor was composed of a cystic area and a solid area arising from the cyst wall. In the cystic lesion, a detached polypoid mass was also identified. The cyst wall was lined with a single layer of endometrial-type cells, whereas the solid area was composed of a clear cell adenocarcinoma. In the detached polypoid mass, an exophytic leaf-like pattern containing benign endometrial-type cells and squamous epithelial and rhabdomyosarcoma components, which were positive for desmin and myoglobin, was observed. Using X-chromosomal clonality assay, these clear cell adenocarcinoma and adenosarcoma components showed patterns of polyclonality. To the authors' knowledge, this is the first reported case of a clear cell adenocarcinoma and an adenosarcoma coexisting with heterologous rhabdomyosarcoma in an endometriotic cyst of the ovary.

A case of müllerian adenosarcoma arising in ovarian endometriosis is reported in which the whole of the mesenchymal component exhibited striking deciduoid morphology, a phenomenon that has not been previously described. The patient was not taking hormonal preparations. We discuss the differential diagnosis and the possible pathogenesis of the deciduoid stromal alteration.

A serous carcinoma and endometrial intraepithelial carcinoma were encountered in an endometrial adenofibroma in a 61-year-old woman. The carcinoma involved the myometrium and cervix (stage IIa). To our knowledge, this is the third documented case of an adenocarcinoma and the first serous carcinoma involving a uterine adenofibroma.

The terminology of uterine sarcomas is confusing and has been inconsistently applied. The relationships among the various types of uterine sarcomas are unknown. To elucidate the relationship between carcinosarcoma (malignant mullerian mixed tumor) and adenosarcoma, a series of 26 consecutive endometrial carcinosarcomas was evaluated for the presence of an adenosarcoma-like component. Four of 26 carcinosarcomas (15%) had an adenosarcoma-like component. The clinical and pathologic features of these tumors were otherwise similar to ordinary endometrial carcinosarcomas. A histogenetic schema for uterine sarcomas is proposed, interrelating endometrial stromal sarcomas, adenosarcomas with and without sarcomatous overgrowth, and poorly differentiated sarcoma not otherwise specified. Although most carcinosarcomas are accepted as being metaplastic or sarcomatoid carcinomas, it is suggested that the carcinomatous component of 8% to 16% of carcinosarcomas arise within, or in the endometrium adjacent to, an adenosarcoma. This latter group is then integrated into the histogenetic schema. Observations based on our data and the limited data in the literature that support this hypothesis include: 13% of endometrial carcinosarcomas have gross and microscopic features of collision tumors, 13% have an adenosarcoma-like component, 16% have a low-grade stroma, and approximately 8% are biclonal indicative of a collision tumor. The most parsimonious interpretation of these data indicates that approximately 8% to 16% of endometrial carcinosarcomas arise because of malignant transformation of the epithelial component within, or in the endometrium adjacent to an adenosarcoma.

We report a primary peritoneal mullerian adenosarcoma with sarcomatous overgrowth associated with endometriosis in a 50-year-old female. The tumor formed multiple peritoneal masses occupying the pelvis and subdiaphragmatic space. Histologically, the tumor was composed of benign-appearing mullerian glands surrounded by a sarcomatous stroma. In addition, one perisplenic mass was largely devoid of the epithelial component and was of higher grade than the remaining lesions. Multiple foci of endometriosis were associated with the pelvic masses. To our knowledge, primary peritoneal mullerian adenosarcoma with sarcomatous overgrowth associated with endometriosis has not been previously reported.

The clinical and pathologic features of 12 cervical adenosarcomas from the files of the Armed Forces Institute of Pathology are described. The patients ranged in age from 13 to 67 years (mean 37). The majority (58%) presented with abnormal bleeding. All tumors were located in the cervix and consisted of soft, tan, polypoid or papillary masses ranging in size from 1.5 to 4.5 cm. Microscopically, they showed a biphasic pattern with mesenchymal and epithelial components. There was a characteristic stromal condensation below the epithelial surface and around glandular structures. The cytologic atypia of stromal cells was 1+ in three, 2+ in five, and 3+ in four. The mitotic activity ranged from four to 28 (mean 7.0) mitotic figures per 10 high-power fields. One neoplasm contained cartilage and one striated muscle. Myometrial invasion was present in three. Treatment consisted of hysterectomy in nine patients and excisional biopsy in three. Two patients received radiotherapy; one before surgery and the other after hysterectomy. Two were treated with chemotherapy. Follow-up ranged from 9 months to 18.8 years. Nine patients were alive and well with no evidence of recurrent tumor at postoperative intervals of 0.8-18.8 years. One patient died 1 year after diagnosis with intraabdominal metastasis. One developed a recurrent tumor. This study demonstrates a favorable prognosis for patients with cervical adenosarcoma. Similar to patients with uterine adenosarcoma, prognosis is mostly affected by the presence of deep myometrial invasion.