A rare case of extragenital Müllerian adenosarcoma / Philippine Journal of Obstetrics and Gynecology

@#A 51-year-old gravida 5 para 5 (5005) presented with an increasing abdominal girth and a palpable abdominal mass. She was initially diagnosed with ovarian new growth and underwent exploratory laparotomy. Intraoperatively, the uterus, Fallopian tubes, and ovaries were grossly normal and a large mass was seen attached to the cecum where the appendix should be referral to surgery service was done. Right hemicolectomy and ileostomy were performed. The histopathology report was “suggestive of a Müllerian adenosarcoma (MAS) involving the appendix and cecum.” Microscopic examination showed evidence of endometriosis with no evidence of sarcomatous overgrowth, features that are favorable prognostic factors associated with higher disease-free survival. Postoperatively, the plan of management was hormonal therapy. Extragenital MAS is rare. This case is the fourth case to be reported in the literature to arise from the colon. Although there is still no standard of treatment, accurate diagnosis is imperative for appropriate management.

Mullerian adenosarcoma with sarcomatous overgrowth in a premenopausal patient / Philippine Journal of Obstetrics and Gynecology

@#We report a case of a 33-years old, nulligravid, diagnosed with mullerian adenosarcoma with sarcomatous overgrowth (MASO), who presented with vaginal bleeding and recurrent endometrial polyp. MASO is a rare type of uterine sarcomas, it is a variant of adenosarcomas with poor prognosis. The index patient underwent primary surgical management with lymphadenectomy with a final stage of IC. Histologic diagnosis was Mulllerian adenosarcoma with sarcomatous overgrowth. A panel of immunostaining for estrogen receptors, progesterone receptors and CD 10 showed diffused positivity for the hormones with loss of CD 10 which is consistent with MASO. The rarity of MASO has a distinctive histologic features which merits meticulous sectioning as the clinical course and management vary. It has a poor prognosis due to its short and fast course of the disease.

High grade adenosarcoma of endocervix and endometrial cavity with a heterologous component of rhabdomyosarcoma. Case report and review of the literature / Adenosarcoma de alto grado de endocérvix y cavidad endometrial con componente heterólogo de rabdomiosarcoma. reporte de caso y revisión de la literatura

RESUMEN Objetivo: presentar el caso de una paciente con diagnóstico de adenosarcoma de alto grado de endocérvix y de cavidad endometrial, con componente heterólogo, y hacer una revisión de la literatura con especial atención al diagnóstico y manejo terapéutico de esa patología. Materiales y métodos: Se presenta el caso de una paciente de 31 años, que consulta al Hospital Universitario Virgen Macarena de Sevilla, institución regional de tercer nivel, por presentar san grado genital originado por una masa polipoidea endocervical que se llevó a biopsia con resultado de un leiomiosarcoma poco diferenciado de alto grado de endocérvix. Posteriormente fue llevada a histerectomía total abdominal. En el estudio de la pieza quirúrgica el resultado fue: adenosarcoma de endocérvix y cavidad endometrial, con componente heterólogo de rabdomiosarcoma. Con los términos: "adenosarcoma", "endocervical", "cérvix", "uterus", "heterologous", en las bases de datos Medline vía PubMed se realizó una búsqueda de artículos de revisión bibliográfica, reportes y series de casos clínicos que describían aspectos del adenosarcoma cervicouterino y del componente heterólogo de rabdomiosarcoma, en inglés y español, publicados desde 1974. Resultados: se hallaron seis artículos correspondientes a revisiones de la literatura, reportes o series de casos, donde se describen los aspectos más importantes referentes al diagnóstico y tratamiento de esta patología. Conclusiones: esta patología se caracteriza, en ocasiones, por un crecimiento rápido y agresivo, donde es importante el diagnóstico precoz y el tratamiento óptimo, basado en una combinación de cirugía, radioterapia y quimioterapia, aunque dada su baja prevalencia se necesitan aún más estudios para poder confirmar estos datos.

ABSTRACT Objective: To present the case of a patient diagnosed with high grade adenosarcoma of the endocervix and the endometrial cavity, with a heterologous component, and to conduct a review of the literature focusing on the diagnosis and therapeutic management of this disease condition. Materials and methods: We present the case of a 31-year-old female patient who came to Virgen Macarena University Hospital of Seville, a Level III regional institution, complaining of genital bleeding arising from an endocervical polypoid mass. The biopsy of the mass revealed a high grade, poorly differentiated leiomyosarcoma of the endocervix. The patient was taken later to total abdominal hysterectomy. The study of the surgical specimen provided the following result: adenosarcoma of the endocervix and endometrial cavity with a heterologous rhabdomyosarcoma component. A search was conducted in the Medline database via Pubmed using the terms "adenosarcoma," "endocervical," "cervix," "uterus," "heterologous." The search included literature review articles, case reports and clinical case series describing aspects of cervical adenosarcoma and the heterologous rhabdomyosarcoma component, published in English and Spanish since 1974. Results: Six articles corresponding to literature reviews, case reports or case series in which the most relevant aspects of the diagnosis and treatment of this disease condition are described were retrieved. Conclusions: This condition is characterized, on occasions, by rapid and aggressive growth, hence the importance of early diagnosis and optimal treatment based on a combination of surgery, radiation therapy and chemotherapy. However, due to its low prevalence, further studies are needed in order to confirm these data.

Mullerian adenosarcoma (heterologous) of uterine cervix with sarcomatous overgrowth: A case report with review of the literature

Mullerian adenosarcoma is a rare biphasic malignant neoplasm of cervix characterized by an admixture of benign epithelial elements and a malignant sarcomatous stromal component, which may be either homologous or heterologous. Mullerian adenosarcoma with stromal overgrowth (MASO) in an aggressive variant of adenosarcoma, which is extremely rare with only two such cases reported till date. In this report, we present a case of MASO of cervix with heterologous elements in a 55/F presenting with postmenopausal bleeding. As it commonly simulates clinically and radiologically as benign cervical polyp, the gynecologists and pathologists should be aware of this extremely rare entity presenting with aggressive clinical course.

Imaging description of extragenital müllerian adenosarcoma: A case report / Descrição da imagem do adenosarcoma mülleriano extragenital: relato de caso

Abstract Müllerian adenosarcoma is a very rare gynecological disease, comprising 5% of uterine sarcomas. Extragenital localizations are even rarer.We report a very interesting case of a 27-year-old woman complaining of pelvic pain, with a subsequent diagnosis of extragenital Müllerian adenosarcoma. This is the first case reported in the literature with a complete and wide imaging description. Even if rare, Müllerian adenosarcoma should be hypothesized in case of young female patients presenting with suspicious pelvic mass.

Resumo O adenosarcoma Mülleriano é uma doença ginecológica muito rara, compreendendo 5% dos sarcomas uterinos. Localizações extragenitais são ainda mais raras. Relatamos um caso muito interessante de uma mulher de 27 anos queixando-se de dor pélvica com diagnóstico subsequente de adenosarcoma Mülleriano extragenital. Este é o primeiro caso relatado na literatura com uma descrição completa e ampla de imagem. Mesmo que raro, o adenosarcoma Mülleriano deve ser hipotetizado no caso de pacientes jovens do sexo feminino com massa pélvica suspeita.

Clinical analysis of 19 mullerian adenosarcoma of uterus / 中国实用妇科与产科杂志

OBJECTIVE: To investigate the clinical characteristics and treatment experience of mullerian adenosarcoma ofuterine.METHODS:A retrospective analysis was conducted on the clinical data of 19 women with Mullerianadenosarcoma of uterus between January 2005 and December 2015.RESULTS:The patients were 28 to 70 years old,withan average of 51.9 years old.9 were premenopause and 10 were postmenopause.The main clinical features are abnormalvaginal bleeding and cervical neoplasms.Preoperative b-ultrasound examination showed abnormal findings in 11 patientsand MRI examination in 8 patients.Sixteen patients received preoperative pathological examination(diagnosticcurretage or cervical biopsy),and the diagnosis accuracy reached 87.5%.Seventeen patients received surgical treatmentand 7 patients received postoperative chemotherapy.The patients were followed up until June 2018,for 11 to 148 months,and 7 died,of which one died from complicated renal failure and 6 died in 5 years after surgery.4 were lost to follow-up,and 8 survived.CONCLUSION:Clinical symptoms of Mullerian adenosarcoma of the uterus are not typical,and abnormalvaginal bleeding is often the first symptom.Endometrial curettage or cervical biopsy can improve the clinical diagnosisrate,and surgery and chemotherapy are the main treatment.

Diagnosis and treatment progress of cervical adenosarcoma / 肿瘤研究与临床

Cervical adenosarcoma is a very rare type of Mullerian adenosarcoma and it is easily confused with benign polyps, therefore, differential diagnosis is necessary. The primary clinical symptoms are vaginal abnormal bleeding and abdominal pain, which can be prevented through the study of high-risk factors. Surgery excision has been considered as the main treatment at present. Due to the low incidence of cervical adenosarcoma, its etiology, pathogenesis, diagnosis and treatment need to be discussed further. This paper reviews the diagnosis and treatment progress of cervical adenosarcoma.

Metastasizing uterine mullerian adenosarcoma in a young female: Diagnostic enigma

Uterine adenosarcomas are uncommon tumors. It is a biphasic tumor with both epithelial and mesenchymal component. The epithelial component is benign in nature, and the mesenchymal component is malignant. Metastasis is rare in adenosarcoma. We report a case of adenosarcoma with lymph nodal metastasis. A 20-year-old female presented with history of per vaginal bleeding for 1 month. Per vaginal examination revealed a fungating mass protruding through the cervical os. Ultrasonography and magnetic resonance imaging showed a large intrauterine mass. Biopsy of the mass done at an outside hospital was reported as rhabdomyosarcoma. Hence, she was given one cycle of neoadjuvant chemotherapy. Following this, she had profuse bleeding. Emergency hysterectomy with pelvic lymph nodal dissection was performed. The final histopathology was reported as adenosarcoma. One pelvic lymph node showed metastatic deposit of rhabdomyosarcomatous element. In young females presenting with polypoidal mass, uterine adenosarcoma can be considered in the differential diagnosis.

Prognosis-related clinicopathologic characteristics of FIGO stage Ⅰ Müllerian adenosarcoma of uterus / 中华病理学杂志

Objective@#To investigate the clinicopathological features of FIGO stage Ⅰ uterine Müllerian adenosarcoma and clinical prognosis.@*Methods@#Fifteen cases of uterine Müllerian adenosarcoma at FIGO stage Ⅰ were collected at PLA General Hospital from 2005 to 2017. Twelve cases with complete follow-up data were divided into 2 groups: group A (7 patients with survival) and group B(5 patients of death or tumor progression). Clinicopathologic features were compared between the two groups.@*Results@#The median age of the patients was 43 years and 56 years, and the tumor size was 4.3 cm and 7.3 cm for group A and B, respectively. Cases in group A were FIGO ⅠA and ⅠB stage tumors and were mainly low grade in histology (5/7) with rare tumor hemorrhagec, necrosis (1/7) and sarcomatous overgrowth. In contrast, most cases in group B were high grade sarcomas(3/5) with frequent hemorrhage, necrosis(3/5) and sarcomatous overgrowth(4/5). Most cases of group A expressed ER, PR and CD10 (6/7) and low Ki-67 index of ≤20%(5/7). While most group B cases lost expression of ER and PR (3/5), significantly reduced expression of CD10 and higher Ki-67 index of ≥30%(4/5).@*Conclusions@#Most of uterine adenosarcomas are of low malignant potential. The main prognostic indicator is advanced tumor stage. For patients at stage Ⅰ, sarcomatous overgrowth, high-grade histology, deep myometrial invasion, decreased or absent expression of CD10, ER and PR, increased Ki-67 index(≥30%) and hemorrhagic necrosis may indicate poor prognosis. Müllerian adenosarcomas arising from endomeriosis may present unusual growth patterns.

Clinicopathologic study of adenosarcoma with sarcomatous overgrowth of the uterine / 临床与实验病理学杂志

Purpose To investigate the clinicopathologic characteristtics, immunophenotype and differential diagnosis of uterine adenosarcoma with sarcomatous overgrowth. Metheds The clinicopathological data of 4 cases of uterine adenosarcoma with sarcomatous overgrowth were collected, the histopathologic and immunohistochemical features were investigated, and the rele-vant literatures were also reviewed. Results All of tumors were arised from the endometrium with complains of postmenopausal vaginal bleeding or prolonged menstrual period. There is a poly-poid nodular in the uterine cavity with a pedicle or no pedicel, or rough endometrium. On the cut surface, the tumor was fish-like without distinct from the surrounding tissue. Light microsco-py show the tumors were composed of benign glands and malignant mesenchymal components, the sarcomatouscomponents ac-counted for over 25％. In 4 cases, 2 cases had heterologous com-ponent of rhabdomyosarcoma. The component of sarcomatous were positive for vimentin and CD10. The heterologous component of rhabdomyosarcoma were positive for desmin, MyoDl, and Myogenin.3 cases were died at in 5, 10, and 19 months after operation, 1 patient was disease free survival for 3 months. Conclusion Uterine adenosarcoma with sarcomatous overgrowth has a bad prognosis.

A Rare Case of Intramural Müllerian Adenosarcoma Arising from Adenomyosis of the Uterus

Müllerian adenosarcomas usually arise as polypoid masses in the endometrium of post-menopausal women. Occasionally, these tumors arise in the cervix, vagina, broad and round ligaments, ovaries and rarely in extragenital sites; these cases are generally associated with endometriosis. We experienced a rare case of extraendometrial, intramural adenosarcoma arising in a patient with adenomyosis. A 40-year-old woman presented with sudden-onset suprapubic pain. The imaging findings suggested leiomyoma with cystic degeneration in the uterine fundus. An ill-defined ovoid tumor with hemorrhagic degeneration, measuring 7.5 cm in diameter, was detected. The microscopic findings showed glandular cells without atypia and a sarcomatous component with pleomorphism and high mitotic rates. There was no evidence of endometrial origin. To recognize that adenosarcoma can, although rarely, arise from adenomyosis is important to avoid overstaging and inappropriate treatment.

Safety of ovarian preservation in premenopausal women with stage I uterine sarcoma / 부인종양

OBJECTIVE: To evaluate the oncologic safety of ovarian preservation (OP) in premenopausal women diagnosed with the International Federation of Gynecology and Obstetrics (FIGO) stage I uterine sarcoma. METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results database was accessed and a cohort of women aged ≤50 diagnosed between 1988–2013 with a sarcoma limited to the uterus was drawn. Based on site-specific surgery codes, women who underwent hysterectomy with or without oophorectomy and did not receive radiation therapy were selected for further analysis. Overall (OS) and cancer-specific (CSS) survival were determined following generation of Kaplan-Meier curves; comparisons were made with the log-rank test. A Cox-proportional hazard model was constructed to control for possible confounders. RESULTS: A total of 1,482 women were included in the analysis; 800 (54.0%) were diagnosed with leiomyosarcoma (LMS), 520 (35.1%) with low-grade endometrial stromal sarcoma (LG-ESS), and 162 (10.9%) with adenosarcoma (AS). The OP group included 418 women (28.2%). Differences in the rate of OP were noted based on histology (p=0.014), year of diagnosis (p=0.001), patient age (p<0.001) and race (p=0.012). There was no difference in OS (p=0.220) or CSS (p=0.210) between women who had OP and those who did not. Multivariate analysis confirmed that OP was not associated with a worse mortality. CONCLUSION: In this population-based cohort of women with sarcoma limited to the uterus, OP was not associated with worse oncologic outcomes. OP could be considered for women with LMS, sparing them from the morbidity associated with iatrogenic menopause. No conclusions could be made for those with LG-ESS or AS.

A Case of Solitary Brain Metastasis from Uterine Mullerian Adenosarcoma with Sarcomatous Overgrowth

Uterine adenosarcoma (AS) are rare tumors and have more favorable outcomes than the aggressive uterine carcinosarcomas. Uterine adenosarcoma with sarcomatous overgrowth (ASSO) is a variant form of AS and exhibits aggressive growth of tumor and the prognosis is relatively poor compared with typical AS. Usually patterns of metastasis have been known to behave like endometrial carcinoma and spread through the lymphatics. Brain metastasis from uterine AS is extremely rare. Herein, we report a case of successfully surgically removed solitary brain metastasis without any extracranial recurrence from uterine ASSO after 4 years of primary treatment.

MR Imaging Findings of Tamoxifen-associated Uterine Adenosarcoma: Report of Two Cases

Adenosarcoma of the uterus is a rare biphasic tumor containing benign glandular epithelial and malignant mesenchymal components. The tumor has been reported to be associated with antiestrogen therapy, particularly tamoxifen, but there have been a few case reports with MRI. We present two cases of MRI findings of uterine adenosarcoma after antiestrogen therapy, tamoxifen and toremifene in breast cancer patients. The tumor presents as a large polypoid mass occupying the endometrial cavity, and may protrude into the vagina. On MRI, the tumor typically shows solid components with scattered small cysts and heterogeneous enhancement. These findings are not significantly different from conventional adenosarcoma.

Mullerian Adenosarcoma Arising From Rectal Endometriosis

A Mullerian adenosarcoma is an extremely rare tumor characterized by a stromal component of usually low-grade malignancy and by a benign glandular epithelial component. A Mullerian adenosarcoma occurs mainly in the uterus, but also in extrauterine locations. Extrauterine Mullerian adenosarcomas are thought to arise from endometriotic deposits. A 36-year-old female presented to Daegu Catholic University Medical Center with a symptom of loose stool for several months. The imaging studies revealed a rectal mass, so she underwent a laparoscopic low anterior resection. Although extemporary pathology revealed an inflammatory myofibroblastic tumor, the final histologic diagnosis was a Mullerian adenosarcoma arising from rectal endometriosis. To our knowledge, except a concomitant rectal villotubular adenoma, cases of Mullerian adenosarcomas arising the rectal wall are rare. An adenosarcoma arising from endometriosis should be considered in the differential diagnosis of a pelvic mass, even one appearing in rectal wall, because ectopic endometrial tissue exists everywhere.

Clinicopathological spectrum of 19 adenosarcomas of female genital tract, including uncommon clinical associations and immunohistochemical profile, reviewed at a single institution.

Background: Adenosarcomas of the female genital tract have been rarely documented as case series from our continent. Materials and Methods: Over a seven-year period, 19 adenosarcomas were critically reviewed. Results: Nineteen tumors occurred in the age range of 21-65 years (mean: 43), in the endometrium (8), endometrium and cervix (4), cervix (4), and ovary (3). Four cases displayed coexisting leiomyomas; two, adenomyosis; two on background endometriosis; and one in post-treated cervix carcinoma. Histopathologically, the tumors were low grade (10; 52.6%) and high grade (9; 47.3%), the latter with sarcomatous overgrowth (SO) (7/9 cases). Dedifferentiation (8, 42.1%) and conspicuous decidualization (2) were noted. Immunohistochemically, the tumors focally expressed CD10 (4/6), smooth muscle actin (SMA) (3/8), desmin (8/11); diffuse vimentin (7/7), and estrogen receptor/progesterone receptor (ER/PR) (2/4). Ki-67 (6 cases) varied 5-20%. Seventeen patients underwent surgery and four received adjuvant treatment (3/4 high-grade tumors). Five tumors recurred (4 high-grade tumors with SO) and one metastasized. Among 11 patients, five were alive with disease (AWD) (mean: 29.4 months) and six, free of disease (FOD) (mean: 15 months), the latter mostly with low-grade type tumors (83.3% cases). Conclusions: Diverse clinicopathological spectrum was noted within adenosarcomas. Low-grade tumors were less aggressive than high-grade ones, with SO. Immunohistochemically, lower CD10 and ER/PR positivity was noted in high-grade tumors. Surgery formed the mainstay of treatment. Adjuvant treatment was offered in high-grade subtypes, including in tumors with SO.

Uterine mesenchymal tumors.

Uterine mesenchymal tumors are a heterogeneous group of neoplasms that can frequently be diagnostically challenging. Differentiation between the benign and malignant counterparts of mesenchymal tumors is significant due to differences in clinical outcome, and the role of the surgical pathologist in making this distinction (especially in the difficult cases) cannot be underestimated. Although immunohistochemical stains are supportive toward establishing a final diagnosis, the morphologic features trump all the other ancillary techniques for this group of neoplasms. This review therefore emphasizes the key morphologic features required to diagnose and distinguish uterine mesenchymal tumors from their mimics, with a brief description of the relevant immunohistochemical features.

Recurrent mullerian adenosarcoma like tumor of seminal vesicle.

Adenosarcoma like tumor of the seminal vesicle is reported herein. A 35-year-old male presented with mass in the pelvis between bladder and rectum, involving the seminal vesicle and prostate. Mass recurred after enucleation in four years. Histologically, the tumor was multicystic with bland ciliated lining epithelium and sarcomatous stroma. A wide excision was performed followed with chemotherapy and radiotherapy. Adenosarcomas have a low grade recurrent malignant potential and should be recognized.

Endometrial mullerian adenosarcoma after toremifene treatment in breast cancer patients: a case report / 부인종양

Toremifene is an anti-estrogen which has been shown to be effective in the treatment of breast cancer, and is thought to be a less uterotrophic agent than tamoxifen. The risk assessment concerning endometrial cancer has been inconclusive because of its rare use up to the mid-1990s. We report a case of an adenosarcoma, which is a very rare type of uterine malignancy, after toremifene treatment for 5 years in a breast cancer patient. After 1 year of toremifene use, the patient had a benign Mullerian adenofibroma. After an additional 4 years of toremifene treatment, the endometrial polypoid lesion was transformed into a Mullerian adenosarcoma. Although toremifene is a promising anti-estrogenic agent in the treatment of breast cancer patients, clinicians should not neglect the possibility of a uterine malignancy.

Mullerian adenosarcoma (heterologous) of the cervix with sarcomatous overgrowth: a case report with review of literature / 부인종양

Mullerian adenosarcoma is a rare biphasic malignant neoplasm of the cervix characterized by an admixture of benign epithelial elements and a malignant sarcomatous stromal component, which may be either homologous or heterologous. An aggressive variant of adenosarcoma, mullerian adenosarcoma with sarcomatous overgrowth (MASO) is extremely rare, with only two such cases being reported in the English literature to date. In this report we present a case of MASO of uterine cervix with heterologous elements in a 15-year-old unmarried girl presenting with foul smelling menstrual bleeding and passage of fleshy masses. Because MASO with heterologous elements seems to appear at the earliest stages of reproductive lifespan in women, and have an uncertain malignant potential, gynecologists and pathologists should be aware and think about the possibility of this tumor.