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[Uterine adenosarcoma. Apropos of a case of uterine and vaginal adenosarcoma with a corrected genital prolapse].

Uterine adenosarcoma is an apparently benign mixed tumor with a muller type epithelial component and a sarcomatous stromal component. We present a patient with remodeled genital prolapse who developed a bifocal localization in the endometrium and vagina. These tumors are uncommon, 8% of the sarcomatous tumors of the uterus. Their usual presentation may mimic cervical or endometrial polyps. Pathology is required for positive diagnosis which evidences the two components. The diagnosis may be missed at the first examination (two-thirds of the cases in certain series). Treatment is surgical with hysterectomy in peri- or post-menopaused women and tumoral excision or curettage in younger women. The prognosis is good but can worsen if invasion extends beyond one-third of the myometrial thickness or if pure sarcomatous transformation occurs.

Adenosarcoma↗

Adenosarcoma of the uterus: a clinicopathologic, DNA flow cytometric, p53 and mdm-2 analysis of 11 cases.

Eleven patients with uterine adenosarcoma diagnosed between 1970 and 1995 were evaluated according to DNA ploidy, S-phase fraction, p53 and mdm-2 expression, and traditional clinical and pathological prognostic factors, such as tumor stage, grade and mitotic index. DNA flow cytometric analysis and immunohistochemical staining for p53 and mdm-2 were performed on paraffin-embedded archival tissue from the uterine tumors. The patients ranged in age from 41 to 90 years (median, 76 years). Only one patient was premenopausal at the time of diagnosis and five (45%) were nulliparous. One patient had received previous pelvic irradiation for anal squamous carcinoma. Six of the tumors (55%) were pure adenosarcoma and five (45%) were adenosarcoma with sarcomatous overgrowth. Nine patients had a stage I tumor and two had a stage II tumor. Among the six adenosarcomas we found three DNA diploid tumors, two DNA aneuploid tumors, and one DNA multiploid tumor. All adenosarcomas had an S-phase fraction less than 10%, except one that was not assessable. None was p53 positive and only one overexpressed mdm-2. All five adenosarcomas with sarcomatous overgrowth were DNA aneuploid, three (60%) had an S-phase fraction > 10%, two (40%) were p53 positive, and one (20%) overexpressed mdm-2. Five of the eleven patients suffered recurrences, and three (60%) of these developed lung metastases. During the observation period four (36%) patients (2 adenosarcomas and 2 adenosarcoma with sarcomatous overgrowth) died of disease, three patients died of intercurrent disease without recurrence, and the remaining four are alive with no evidence of disease. The overall five-year survival rate for all stages was 69%; for patients with AS it was 80%, while for those with adenosarcoma with sarcomatous overgrowth it was 50%. There were no variables which correlated with survival. In conclusion, we found hat the typical adenosarcoma had a tendency to be of low stage, have a lower mitotic rate and an S-phase fraction <10%. On the other hand, adenosarcomas with sarcomatous overgrowth were of high grade, had a high mitotic rate, and were DNA aneuploid with an S-phase fraction >10%. None of the variables studied correlated with survival. Tumors that were p53-positive or overexpressed mdm-2 did not behave worse than their negative counterpart. All patients who recurred with distant metastases died of disease.

Journal Article↗

Cluster of uterine mullerian adenosarcoma in the Washington, DC metropolitan area with high incidence of sarcomatous overgrowth.

Mullerian adenosarcoma is an uncommon variant of uterine sarcoma. Twelve uterine adenosarcomas were diagnosed during a 42-month period at the Washington Hospital Center in Washington, DC. Based on estimated incidence data derived from the US Department of Defense beneficiary population, an estimated relative risk of 15.4 (95% confidence interval, 7.7-31.0) was calculated, indicating a significantly increased incidence of adenosarcoma in the population studied (p<0.0000001). Among 10 patients who underwent hysterectomy, six (60%) of their tumors had sarcomatous overgrowth. In comparison with the previously reported proportion of adenosarcomas with sarcomatous overgrowth, approximately 16%, the proportion with sarcomatous overgrowth was significantly higher than expected (p<0.01). Mullerian adenosarcoma with sarcomatous overgrowth was first described in 1989 and suggests that the cluster of adenosarcomas reported herein may be due in part to the current classification of some uterine tumors as adenosarcoma with sarcomatous overgrowth that previously would have been classified as other types of uterine sarcoma. Nonetheless, even after reviewing and updating the classification of all sarcomas diagnosed at the Washington Hospital Center from 1985 to 1998, the ratio of adenosarcomas to uterine adenocarcinomas during the 1994-1998 period was 4.7 times (p<0.005) that of the 1985-1993 period, suggesting a more modest but real increase in the occurrence of this tumor. Correct classification of mullerian adenosarcomas with sarcomatous overgrowth is important because the limited available data suggest that the prognosis is notably worse than that for adenosarcomas without sarcomatous overgrowth.

Adenosarcoma↗

Adenofibroma and adenosarcoma of the uterus: a clinicopathologic study of 35 cases.

The clinical and histopathologic features of ten adenofibromas and 25 adenosarcomas of the uterus were studied. The most useful criterion for distinguishing adenofibroma from adenosarcoma was the frequency of mitotic figures found in the stroma. Adenofibromas had fewer than four mitotic figures per 100 HPF in the most active areas; adenosarcomas had four or more. Myometrial invasion, histologically malignant heterologous mesenchymal elements, and marked atypia of stromal cells were histologic features detected only in adenosarcoma. Of the women with adenosarcoma, ten (40%) had recurrences, with a median interval to recurrence of five years. The only morphologic feature that correlated with aggressive behavior of adenosarcoma was deep myometrial invasion. Adenofibroma and adenosarcoma are in the family of mixed mesodermal tumors, but are distinct clinical and pathologic entities.

Adenofibroma↗

[Heterologous mesodermal adenosarcoma of the endocervix. Description of a clinical case].

We describe a case of an endocervical heterologous mesodermal adenosarcoma, found in a 43 year old woman. Among mesodermal neoplasms, various histological types are distinguished: the carcinosarcoma, the embryonal rhabdomyosarcoma or botryoid sarcoma, and the adenosarcoma; the last is formed by a benign epithelial component and by a malignant stromal component, that may contain heterologous tissues, such as cartilage, skeletal muscle, etc. Adenosarcoma is a tumor of the uterine corpus and seems to be most common among menopausal women. A primitive adenosarcoma of the uterine cervix is very rare; in fact the overall percentage of the uterine cervical sarcoma is 0.2-0.4%. The patient, age 43 years, with regular menstrual bleeding came to an outpatient clinic referring a post coital metrorrhagia. After a control examination, a polyp from the uterine cervix was removed; the histopathologic diagnosis was: fibroangioadenomatous polyp of the isthmus with cartilaginous metaplastic areas. Two months later, the patient was referred to our clinic and another cervical polyp was removed. The histological diagnosis was adenosarcoma with chondrosarcomatous heterologous mesodermal component. Then the patient was operated and the postoperative histological examination confirmed the preoperative diagnosis. A literature review about the uterine adenosarcoma etiopathogenesis is reported, and a suitable diagnostic iter, is discussed.

Adenosarcoma↗

Extrauterine müllerian adenosarcoma of the peritoneal mesothelium: a clinicopathologic and electron microscopic study.

An unusual case of a müllerian adenosarcoma of the peritoneal mesothelium in a 32-year-old pregnant woman is described. On emergency laparotomy, a tumor was found in the pelvis connected with the right broad ligament only, and was removed. Light and electron microscopic examination showed a rare example of an extrauterine müllerian adenosarcoma composed of a stromal sarcoma, muscle tissue resembling primitive myoblasts, and sex-cord-like tubular lumens. The patient was followed, and after 22 months the primary surgery was reexplored. On laparotomy, no evidence of disease was found, and the ovaries were tumor-free. Histologic examination of the pelvic peritoneum demonstrated small areas of adenosarcoma resembling elements of the previous tumor. Müllerian adenosarcoma is a rare tumor of müllerian origin, and its less aggressive behavior as compared with mixed müllerian sarcoma is warranted. The better prognostic outlook of müllerian adenosarcoma is probably related to the histologic structure, but depends in addition on the size of the tumor and the site of origin. No case of peritoneal origin of müllerian adenosarcoma has been previously reported; we discuss this unusual location.

Adult↗

Müllerian adenosarcoma presenting as cervical polyps: a report of seven cases and review of the literature.

OBJECTIVE: To emphasize the importance of early diagnosis in cases of müllerian adenosarcoma that appeared as benign-looking cervical polyps. METHODS: We examined seven cases of müllerian adenosarcoma of the uterus in patients 14-63 years of age (median 39 years). Tissue protruding from the external os and an initial diagnosis of a cervical polyp were common findings for all patients. On repeated examination, all lesions were interpreted as müllerian adenosarcomas. RESULTS: Histologic examination demonstrated benign glands with a sarcomatous stroma, which typically formed periglandular cuffs of increased cellularity. The sarcomatous stroma was homologous in four cases and contained heterologous elements such as striated muscle, lipoblast, and cartilage in three cases; one patient had a sarcomatous overgrowth of stromal elements. The question of a müllerian adenofibroma versus adenosarcoma was raised in three cases with the general appearance of slit-like glands surrounded by a stroma with fibrosis and a low mitotic rate. Using the criteria of stromal cellularity--marked stromal atypia and a mitotic index of two figures per ten high-power fields--the cases were classified as adenosarcomas. The sarcomatous overgrowth, the presence of heterologous elements, and a high mitotic rate seem to be important prognostically. CONCLUSION: Gynecologists and pathologists should be aware of the difficulties and delay in the diagnosis of müllerian adenosarcoma when the tumor presents as a benign-looking cervical polyp.

Adolescent↗

Primary peritoneal mesodermal adenosarcoma: report of a case and review of the literature.

BACKGROUND: Mesodermal (müllerian) adenosarcoma arising from the peritoneum is rare and is thought to arise from pluripotent mesothelial and mesenchymal cells of the pelvic cavity or from endometriotic deposits. CASE: A case of primary peritoneal mesodermal adenosarcoma arising from the omentum is described. A 50-year-old woman presented with sudden abdominal distension. Initial laparotomy revealed a 13-kg mass arising from the omentum, which was determined from frozen and paraffin sections to be serous cystadenofibroma. The tumor recurred within 10 months and weighed 18 kg at a second laparotomy. Histopathology and review of the original tumor established the correct diagnosis of mesodermal adenosarcoma. The patient died from disseminated disease 6 months later. CONCLUSION: Adenosarcomas are difficult to differentiate from adenofibromas or endometriosis histologically because of the presence of large areas of low cellularity and infrequent mitotic figures. In such cases, stromal nuclear atypia and periglandular stromal cuffing are features that are diagnostic of adenosarcoma.

Adenosarcoma↗

Müllerian adenosarcomas of the uterus with sex cord-like elements. A clinicopathologic analysis of eight cases.

Eight cases of uterine müllerian adenosarcoma with sex cord-like elements (SCEs) occurred in patients 22-85 years of age (median, 41) who presented with abnormal uterine bleeding. Seven patients had hysterectomy; one had curettage and radiation only. The polypoid endometrial tumors were composed of adenosarcoma admixed with SCEs; the latter accounted for 5-50% of the tumor. The SCEs were multifocal in six cases and formed a solitary mass at the base of the adenosarcoma in the remaining two cases. Benign-appearing epithelial-type cells, often containing abundant eosinophilic or foamy, lipid-rich cytoplasm, were arranged in solid nests, trabeculae, and solid or hollow tubules. Myometrial invasion was observed microscopically in six cases. Of seven patients with postoperative follow-up information, six were alive with no evidence of recurrent tumor at postoperative intervals of 3-11 years. The patient treated only by curettage and radiation had persistent intrauterine tumor at 7 months and died of an unrelated illness at 16 months. This study indicates that occasional uterine adenosarcomas contain SCEs similar to those described in endometrial stromal tumors and uterine tumors resembling ovarian sex cord tumors. The clinical and pathologic features of these tumors are otherwise similar to those of typical uterine adenosarcomas.

Adult↗

Müllerian adenosarcomas of the uterus with sarcomatous overgrowth. A clinicopathological analysis of 10 cases.

Ten cases of müllerian adenosarcoma of the uterus with sarcomatous overgrowth are described. The tumors were found in women 32 to 82 years of age (median, 59), most of whom presented with abnormal vaginal bleeding and an enlarged uterus. At the time of abdominal hysterectomy, performed in all the cases, bilateral ovarian and peritoneal metastases were found in one patient. Gross examination of the uterus revealed bulky polypoid masses filling the endometrial cavity. In six cases, these masses invaded the myometrium. In three of them, they reached the serosa. Histological examination in each case revealed typical adenosarcoma overgrown by a pure sarcoma that accounted for 25% to 80% of the tumor. In seven tumors, the pure sarcoma was of higher grade and exhibited a higher mitotic rate than the sarcomatous component of the associated adenosarcoma, whereas in three tumors, the two sarcomatous components were similar in appearance. On follow-up, three patients were alive with no evidence of recurrent tumor at postoperative intervals of 4 to 7 years. Recurrent tumor developed in the other seven patients, including hematogenous metastases in four of them, at intervals of 9 months to 6 years. Six patients died from tumor progression at intervals of 9 months to 10 years. This study indicates that, in contrast to typical müllerian adenosarcomas of the uterus, adenosarcomas with sarcomatous overgrowth are aggressive tumors frequently associated with postoperative recurrence or metastases and a fatal outcome.

Adult↗

Ascitic fluid cytology of adenosarcoma of the ovary: a case report.

Extrauterine adenosarcoma is very rare and originates in the ovary, adnexa, or myometrium. Cytologic study of ascites is very important to determine clinical staging of malignant ovarian tumors and provide adequate therapy for recurrence. The cytomorphologic features of adenosarcoma have been only rarely described. A 77-yr-old woman visited a hospital with a complaint of lower abdominal pain for 1 mo. A tumor originating from the right adnexa in the pelvis, and involving the rectum, was found in surgery. In the ascitic fluid cytology, a few dispersed tumor cells with large cytoplasm and nuclei were oval-shaped, with nuclear invagination. The chromatin was finely granular; one or two nucleoli were conspicuous. To our knowledge, this is the fifteenth reported case of adenosarcoma of the ovary, and there have been no prior reports describing the cytological features of ascitic fluid cells in adenosarcoma of the ovary.

Adenosarcoma↗

Ovarian conservation in a woman of reproductive age with müllerian adenosarcoma.

BACKGROUND: Total abdominal hysterectomy with bilateral salpingo-oophorectomy is generally considered optimal therapy for patients with uterine sarcomas. Local resection of the tumor or hysterectomy with ovarian conservation has been used in only a small number of patients. Recurrence risk in women undergoing ovarian-sparing surgery for müllerian adenosarcomas can be difficult to evaluate due to the paucity of literature in this area. We present a reproductive-age woman with a müllerian adenosarcoma and review the literature on conservative surgical management of this class of tumors. CASE: A 25-year-old nulligravida was diagnosed with a uterine adenosarcoma and the question of conservative surgical therapy arose. Following a literature review, discussion with the patient led to the decision for ovarian preservation at the time of hysterectomy. The pelvis and abdomen were grossly free of metastatic disease at laparotomy and all tumor was confined to the uterus on pathologic examination. She is free of disease 36 months postoperatively and is now considering in vitro fertilization using a surrogate. CONCLUSION: Ovarian conservation can probably be offered safely in carefully selected women of reproductive age with müllerian adenosarcomas.

Adenosarcoma↗

Adenosarcoma of the uterine corpus associated with ovarian thecoma.

We describe a case of adenosarcoma of the uterine corpus associated with ovarian thecoma in a 67-year-old woman. The patient underwent surgery under a diagnosis of ovarian carcinoma. The 110 x 70 mm-sized ovarian tumor was diagnosed as thecoma. The polypoid tumor of the uterine corpus which measured 30 x 15 mm was diagnosed as adenosarcoma. Cells of both epithelial and stromal elements of the adenosarcoma expressed estrogen receptors (determined by immunohistochemistry). These findings support the view that estrogen stimulation, including that by a pre-existing ovarian thecoma, may play a role in the development of mesenchymal and mixed epithelial / mesenchymal uterine tumors, including adenosarcoma.

Adenosarcoma↗

Uterine adenosarcoma: a clinicopathologic study of 11 cases with a reevaluation of histologic criteria.

Eleven biphasic uterine tumors with epithelial components and homologous stroma were reevaluated. Originally these were diagnosed as adenofibroma, adenosarcoma, carcinosarcoma, or mixtures thereof, but were now reclassified as adenosarcomas of which seven were "pure" and four mixed with foci of carcinosarcoma. Nine of the tumors arose in the endometrium and two in the endocervix. The mean patient's age was 55 years. The most common complaint was vaginal bleeding. Macroscopically these tumors presented as polypoid masses. The epithelial component consisted mainly of endometrial, endocervical, ciliated, and clear cells. Squamous metaplasia and focal hyperplasia were occasionally observed. Malignant epithelial change was only present in foci of carcinosarcoma. The stroma showed prominent cellular periglandular cuffs, occasionally round solid or perivascular nodules, and areas of focal or diffuse stromal hypercellularity. In all these areas stromal cells were atypical and/or pleomorphic. Stromal foam cells were seen in three cases. Mitotic activity was low ranging from one to three mitoses per 10 high power fields (HPF). Follow-up was negative exept in two cases with recurrence and abdominal metastases. It was concluded that stromal hypercellularity with atypism and pleomorphism in periglandular, perivascular location as well as of focal or diffuse nature, is characteristic of uterine adenosarcoma. Adenofibromas present a fibro-collagenous stroma lacking the crowded cellular areas. Mitotic activity is too variable to serve as a reliable diagnostic criterion. Uterine adenosarcomas are usually tumors of low grade malignancy but the lack of correlation between histologic appearance and biologic behaviour precludes prognostication in the individual patient.

Adult↗

Endometrial adenosarcoma with pelvic involvement following uterine perforation.

An 85-year-old woman had a 7-year history of recurrent uterine adenosarcoma. One year after curettage-related uterine perforation, she developed a pelvic mass that was attached to the uterine serosa and was histologically identical to her endometrial lesions. The pelvic neoplasm probably resulted from implantation of tumor through the myometrial tear and is the first reported example of serosal adenosarcoma following myometrial perforation. The definition of sarcomatous stroma in Müllerian adenosarcoma, and thus its separation from adenofibroma, has not been delineated. A review of the literature indicates, however, that lesions recurring after hysterectomy have greater than three mitotic figures per 10 high-power fields. Incompletely excised neoplasms, treated by dilation and curettage only, often regrow, regardless of mitotic rate. Adenosarcoma may have a deceptively bland low-power pattern and must be differentiated from adenofibroma and benign polyps.

Aged↗

Uterine adenosarcoma with sarcomatous overgrowth versus uterine carcinosarcoma: comparison of treatment and survival.

OBJECTIVE: Uterine adenosarcoma with sarcomatous overgrowth (ASSO) is a rare variant of uterine sarcoma first described in 1989. This clinicopathologic study was undertaken to compare the treatment and survival of uterine adenosarcoma with sarcomatous overgrowth to that of uterine carcinosarcomas. METHODS: A review of uterine sarcomas diagnosed at Washington Hospital Center from January 1988 to December 1998 was performed. Records were reviewed for demographic data, surgical staging, primary and adjuvant therapy, metastatic site, disease recurrence, and survival. All pathology was reviewed and diagnosis confirmed. Statistical analysis included chi(2) test and Student's t test. Kaplan-Meier survival curves were plotted to estimate the median and 5-year survival times. The log-rank test was used to compare survival times. A P value <0.05 was considered significant. RESULTS: Sixty patients were diagnosed with uterine sarcoma at Washington Hospital Center. Of these, 33 (55%) were uterine carcinosarcomas, 11 (18%) ASSOs, 6 (10%) adenosarcomas, and 10 (17%) leiomyosarcomas. Of the patients diagnosed with uterine ASSO, 3 (27%) were stage I, 3 (27%) stage II, 1 (9%) stage III, and 4 (36%) stage IV. All 11 patients with uterine ASSO underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and tumor debulking. Postoperative adjuvant therapy included chemotherapy (n = 4), radiation (n = 4), combination radiation and chemotherapy (n = 1), and no adjuvant therapy (n = 2). The overall median survival time of patients with uterine ASSO was 13 months. Nine of eleven patients are dead of disease, and two patients (both with stage I) are alive without evidence of disease at 18 and 19 months. Thirty-three patients with carcinosarcoma were identified, with follow-up available on 29 patients. Of these, 10 (34%) were stage I, 6 (22%) stage II, 3 (10%) stage III, and 10 (34%) stage IV. Twenty-seven of the twenty-nine patients diagnosed with carcinosarcoma underwent surgical therapy to include total abdominal hysterectomy, bilateral salpingo-oophorectomy, staging and tumor debulking. Two patients died prior to treatment. Postoperative adjuvant therapy included chemotherapy (n = 9), radiation (n = 13), combination (n = 1), and no further therapy (n = 4). Twenty of the twenty-nine patients are dead of disease; there were nine surviving patients at the time of this report (stage I-5, stage II-3, stage III-1). The median survival of these patients was 31 months, with an overall 5-year survival of 22%. Comparison of the Kaplan-Meier survival curves using the log-rank test suggests a worse prognosis for uterine ASSO. However, this did not reach statistical significance (P = 0.0522). CONCLUSIONS: Patients diagnosed with uterine ASSO have a poor prognosis similar to that of carcinosarcoma. Management should include complete surgical staging. Additional therapy in the form of radiation, chemotherapy, or both has been reported; however, the superiority of one modality could not be determined from our data.

Adenosarcoma↗

Use of medroxyprogesterone acetate in the treatment of Müllerian adenosarcoma: a case report.

BACKGROUND: Müllerianadenosarcoma is a rare pelvic malignancy that most commonly arises from the endometrium. These tumors are relatively insensitive to chemotherapy and radiation and are primarily treated by surgical resection. We report a case of müllerian adenosarcoma arising outside of the uterus from a background of endometriosis treated with a combination of surgical resection and medroxyprogesterone acetate. CASE: A 43-year-old woman with a history of endometriosis was diagnosed with advanced extrauterine müllerian adenosarcoma. After suboptimal tumor dubulking surgery she was treated with medroxyprogesterone acetate. Ten months postoperatively she remains without evidence of disease. CONCLUSION: Medroxyprogesterone acetate may be a useful drug in the treatment of advanced müllerian adenosarcoma.

Adenosarcoma↗

Mullerian adenosarcoma of the uterus associated with tamoxifen therapy.

Mullerian adenosarcoma of the uterus is a biphasic tumor exhibiting benign epithelial and malignant stromal component. This tumor may occasionally be associated with tamoxifen therapy which is used as an adjuvant drug for breast carcinoma. Reviewing the literature, we found only 12 adenosarcoma cases associated with tamoxifen therapy Thus, the clinical and pathological findings in a 58 years old postmenopausal woman who developed uterine adenosarcoma, following low dose tamoxifen therapy after 8 years, was discussed in this report.

Adenosarcoma↗