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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↗

Immunohistochemical determination of estrogen and progesterone receptor positivity in uterine adenosarcoma.

BACKGROUND: Given the paucity of data regarding hormone dependency, it was the purpose of this study to screen for the presence of estrogen and progesterone receptors in uterine adenosarcoma (UAS). METHODS: One hundred and five centers were asked to screen their files for uterine adenosarcomas. A immunohistochemical estrogen and progesterone receptor determination was performed. RESULTS: Twenty-eight primary UAS were stained, including one cervical adenosarcoma. Sarcomatous overgrowth could be observed in eight. Furthermore, two cases of recurrent UAS, one only consisting of endometrial stromal sarcoma, were stained. UAS lacking sarcomatous overgrowth showed estrogen receptor positivity in 17/20 (85%) and 16/20 (80%) in the epithelial and sarcomatous component, respectively. Progesterone positivity was observed in 13/20 (65%) and 12/20 (60%) in the epithelial and sarcomatous component, respectively. In 18/20 (90%) of the cases, either the estrogen or the progesterone receptor stained positive in the sarcomatous component. UAS with sarcomatous overgrowth showed estrogen receptor positivity in 4/8 (50%) and 0/8 (0%) in the epithelial and sarcomatous component, respectively. Progesterone positivity was observed in 2/8 (25%) and 1/8 (12%) in the epithelial and sarcomatous component, respectively. The stromal component of both recurrent cases stained moderately positive for estrogen receptor whereas progesterone receptor was considered negative. CONCLUSION: The observation that the sarcomatous component of UAS without sarcomatous overgrowth frequently expresses hormone receptors might be of significant clinical importance.

Adenosarcoma↗

Primary vaginal adenosarcoma with sarcomatous overgrowth.

BACKGROUND: Primary vaginal adenosarcomas are extremely rare, and typical adenosarcomas are of low malignancy. However, aggressive forms with sarcomatous overgrowth have been reported, those appear to have a poor prognosis. CASE: A 52-year-old woman who had undergone prior surgery for uterine leiomyoma and an ovarian cyst (total abdominal hysterectomy and left salpingo-oophorectomy) presented 10 years later with a rapidly enlarging tumor arising from the vaginal cuff. Repetitive biopsy samples revealed a mixture of benign epithelial gland and malignant stromal components with periglandular stromal hypercellularity and sarcomatous overgrowth. A histological diagnosis of mullerian adenocarcinoma was made. The patient died from recurrent disease 9 months after surgery. CONCLUSION: Regardless of primary focus, adenosarcoma with sarcomatous overgrowth is associated with postoperative recurrence and a fatal outcome.

Adenosarcoma↗

Adenosarcoma in a patient with vaginal endometriosis.

BACKGROUND: Adenosarcoma in a patient with extraovarian endometriosis is a rare event and can be easily overlooked. CASE: A woman with a history of endometriosis underwent multiple resections of a vaginal mass and medical treatment for presumed recurrent endometriosis. Eventually, a vaginal adenosarcoma was diagnosed. CONCLUSION: The possibility of adenosarcoma should be considered if an enlarging mass occurs at the site of extraovarian endometriosis.

Adenosarcoma↗

Müllerian adenosarcoma of vagina arising in persistent endometriosis: report of a case and review of the literature.

BACKGROUND: Primary adenosarcoma arising in vaginal endometriosis poses a diagnostic challenge, especially in superficial vaginal biopsies. CASE: A 56-year-old woman, with a prior diagnosis of ovarian endometriosis, presented with a rapidly enlarging mass of the vaginal vault. Two prior biopsies were benign and showed endometriosis. The third vaginal biopsy revealed benign endometriotic glands cuffed by a cellular stroma with moderate cytologic atypia, a histological appearance diagnostic of Müllerian adenosarcoma. A 16-cm vaginal mass that had infiltrated the pelvic structures was resected. CONCLUSIONS: Close clinical follow-up of extrauterine endometriosis and clinical-pathologic correlation is necessary. Histological features such as cellular stromal cuffing around benign endometriotic glands are critical in arriving at a timely diagnosis of adenosarcoma in patients with persistent extrauterine endometriosis, even in superficial vaginal biopsies.

Adenosarcoma↗

Molecular cytogenetic characterization of a case of Müllerian adenosarcoma.

Müllerian adenosarcoma is a distinctive type of mixed Müllerian tumor of the female genital tract. To our knowledge, no cytogenetic data have been documented on Müllerian adenosarcoma in the literature so far. We report here the chromosomal findings of a Müllerian adenosarcoma in a 15-year-old female. Cytogenetic and molecular cytogenetic analysis revealed a complex karyotype involving chromosomes 2, 8, 10, 13, 19, and 21. These numerical and structural abnormalities may be of etiologic significance. This report may highlight the potential value of molecular cytogenetic analysis in differential diagnosis of Müllerian tumors. More cases are warranted to further genetically characterize this type of neoplasm.

Abdominal Neoplasms↗

Extrauterine Müllerian adenosarcoma of the peritoneum with an extensive rhabdomyosarcomatous element and a marked myxoid change.

A case of extrauterine Müllerian adenosarcoma of the peritoneum in a 20-year-old woman is reported. The tumor was widely based on the abdominopelvic wall and there were no unusual features in the genital organs. The cut surface of the tumor showed a marked gelatinous appearance. The tumor was composed of an admixture of benign Müllerian-type epithelium and sarcomatous stroma. The predominant element of the sarcomatous area was rhabdomyosarcoma, which showed a close resemblance to well-differentiated embryonal rhabdomyosarcoma. In another sarcomatous area, fibroblastic cells without myoblastic properties diffusely proliferated in a marked myxoid background with some collagen bundles. Both the mitotic count and Ki-67 proliferative index of these cells were lower than those of rhabdomyoblastic cells. On follow up, the patient was disease free for 1 year postoperatively, without any subsequent treatment. The present case indicates that extrauterine adenosarcoma can also show histological heterogeneity as do uterine adenosarcomas. The remarkable myxoid change of this tumor seemed to be more largely due to a fibromyxoid element than a rhabdomyosarcomatous element, and the coexistence of the former may be related to the less aggressive behavior of this tumor.

Adenosarcoma↗

Uterine adenosarcoma with sarcomatous overgrowth: a case report with cytology of overgrown poorly differentiated sarcoma and immunohistochemical identification of epithelial microinvasion.

In this report, we describe a case of adenosarcoma of the uterine corpus with overgrown and poorly differentiated sarcoma. The patient was an 80-year-old Japanese woman with abnormal genital bleeding. The endocervical biopsy showed undifferentiated carcinoma, and a cytologic examination at that time revealed the presence of cell types ranging from dispersed spindle to round atypical cells, suggestive of mesenchymal origin. The resected uterus had a polypoid mass in the corpus of the uterus and a solid white mass with the appearance of frank malignancy in the endocervix. The phyllode pattern was evident in the polypoid lesion and a diagnosis of adenosarcoma with sarcomatous overgrowth was made. The epithelial component was atypical with occasional microinvasion, which mimicked rhabdomyoblasts, but was easily identified as microinvasion by positive staining for epithelial, but not myogenic, markers. This case stresses the importance of the cytologic smear in the identification of frank sarcoma in an adenosarcoma, and it furthermore highlights the usefulness of immunohistochemistry in the distinction of epithelial microinvasion from rhabdomyogenic differentiation.

Adenosarcoma↗

Adenosarcoma of the uterine cervix: a clinicopathological study of 12 cases.

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.

Adenosarcoma↗

Adenosarcoma of the uterine cervix presenting as a cervical polyp.

A case of adenosarcoma arising from the uterine cervix of a 55-year-old female who complained of vaginal discharge is reported. A polyp, 6 x 2 x 1.5 cm in size, identified in the cervical canal was clinically diagnosed as benign cervical polyp and resected. Histologically, the polyp was composed of benign epithelial components and sarcomatous stroma wherein periglandular hypercellularity and some mitoses including atypical ones were noted. Immunohistochemically, stromal cells were positive for muscle-type actins, desmin and estrogen receptor. Adenosarcoma is a rare, biphasic tumor of the uterus and usually presents as a polypoid mass in the endometrial cavity. When they arise from the cervix, adenosarcomas may be confused with benign cervical polyps clinically and pathologically. As the former often recurs, microscopic differentiation is crucial for further treatment.

Actins↗

Müllerian adenosarcoma of the uterus: report of a case with imprint cytology.

BACKGROUND: Müllerian adenosarcoma is a rare morphologic variant of uterine sarcoma that, although well described histologically, is scarcely mentioned in the cytologic literature. CASE: A 75-year-old female was suspected of having atypical endometrial hyperplasia on an endometrial smear. However, subsequent imaging techniques revealed the presence of a bulky, polypoid mass filling the uterine cavity. On pathologic examination of the hysterectomy specimen, the polypoid tumor was diagnosed as mullerian adenosarcoma, homologous, with sarcomatous overgrowth, in which the sarcomatous component was compatible with high grade endometrial stromal sarcoma. Imprint smears of the tumor consisted of two morphologic patterns, sarcomatous and glandular. The sarcomatous tumor cells, with coarse chromatin and relatively scant cytoplasm, formed small aggregates or appeared alone. These cells were semiround or oval and had conspicuous nucleoli. In addition to these observations, small and large clusters of glandular cells with mild atypism were interspersed with the sarcomatous cells. CONCLUSION: Cytologic examination of müllerian adenosarcoma well reflects its pathologic features.

Adenosarcoma↗

Uterine adenosarcoma with rhabdomyosarcomatous overgrowth. Brief communication.

A case of a rhabdomyosarcomatous uterine adenosarcoma with sarcomatous overgrowth is presented. The rhabdomyosarcoma component constituted about 90% of the tumor. The patient died 5 years and 5 months after the operation. Rhabdomyosarcomatous uterine adenosarcoma even with a sarcomatous overgrowth does not seem to be more malignant than other adenosarcomas.

Adenosarcoma↗

Adenosarcoma of the uterine body in a 19-year-old woman--three year survival: case report.

BACKGROUND: Uterine adenosarcoma is a rarely by occurring tumor. It is composed of a benign adenoid structure and a sarcomatous stromal component. The average age of patients with a diagnosis of uterine adenosarcoma is about 70 years. CASE: We present a case of a 19-year-old woman with a rarely occurring uterine adenosarcoma manifesting itself by irregular bleeding and producing fragile polypous matter which was spreading into the vagina. The final diagnosis was made only by repeated biopsies. Abdominal hysterectomy with bilateral salpingo-oophorectomy, appendectomy and revision of iliac lymph nodes were performed. Teleradiotherapy was applied from 4 fields in 25 fractions to a total exposure of 50 Gy. It was followed by six cycles of chemotherapy containing 50 mg/m2 doxorubicin and 5 g/m2 ifosfamid administered in 21-day dose intervals. CONCLUSION: This case should demonstrate the difficulty of making the right diagnosis. Since the end of therapy the patient has been regularly seen in our onco-gynecologic department. Now, 40 months after the end of chemotherapy and 46 months after making the diagnosis, there are no signs of relapse.

Adenosarcoma↗

Adenosarcoma of the ovary. A case report.

Adenosarcoma of the ovary is a rare condition. We report a case of a 32-year-old patient that has been treated in our Department. The diagnosis of ovarian adenosarcoma was carried out after laparoscopy with removal of an ovarian endometriotic cyst. Laparoscopic homolateral ovariectomy was then performed and conservative treatment was decided on considering the young age, low stage and low grade of the disease. Second-look laparoscopy, clinical evaluation and ultrasound were performed for four years of follow-up. No recurrence has been detected. Conservative treatment should be proposed in fertile age with low-grade ovarian adenosarcoma, but a strict follow-up is always necessary.

Adenosarcoma↗

Mullerian adenosarcoma of the uterus: a clinicopathologic analysis of 100 cases with a review of the literature.

One hundred cases of mullerian adenosarcoma of the uterus were encountered in patients 14 to 89 years of age (median, 58 years), who usually had the symptom of abnormal vaginal bleeding. An enlarged uterus and tissue protruding from the external os were the most common findings on pelvic examination. Five patients presented on multiple occasions with "recurrent polyps" that were interpreted retrospectively as adenosarcomas. Primary treatment, known in 97 cases, included some form of hysterectomy in 93 of them, and conservative resection in four cases. Gross examination of the excised uteri disclosed polypoid masses, some of which had spongy cut surfaces, usually filling the endometrial cavity; less commonly, the tumors were confined to the endocervix or the myometrium or involved more than one site. Histologic examination revealed benign or atypical neoplastic glands within a sarcomatous stroma, which typically formed periglandular cuffs of increased cellularity, intraglandular polypoid projections, or both. The sarcomatous stroma was homologous in 78% of the cases and contained heterologous elements in the remainder. The stromal mitotic rate varied from 1 to 40 mitotic figures (MFs) (mean 9) per 10 high-power fields (HPFs). Extensive areas of stromal fibrosis that focally imparted a deceptively benign appearance to the tumor were common. Myometrial invasion was present in 15 cases, but was deep in only four. Recurrent tumor developed in 23 cases at postoperative intervals of 0.5 to 9.5 years (mean 3.4); in one third of such cases, the interval was 5 years or longer. Recurrent tumor was almost always confined to the vagina, pelvis, or abdomen; hematogenous spread occurred in only two cases. The only feature associated with an increased risk for recurrence was the presence of myometrial invasion. Criteria found useful in separating mullerian adenosarcomas from mullerian adenofibromas included, alone or in combination: two or more stromal MFs per 10 HPFs, marked stromal cellularity, and significant stromal cell atypia.

Adolescent↗