Acute non-puerperal uterine inversion ascribed to malignant mixed Mullerian tumor of uterus: a rare presentation.

Non-puerperal uterine inversion is an extremely uncommon condition, and its occurrence due to malignant mixed Mullerian tumor (MMMT) of the uterus is quite exceptional. We report one such case of acute non-puerperal uterine inversion ascribed to MMMT in a 77-year-old postmenopausal woman. Such a case poses a diagnostic and management dilemma, and prior knowledge may result in a successful outcome.

Benign mixed Müllerian (duct) vaginal tumor in a 12-y-old goat.

In human and veterinary medicine, mixed Müllerian tumors (MMTs) are rarely diagnosed neoplasms of the tubular female genital tract. Although there are case reports of malignant MMTs in various species, benign MMTs have only been described once in a macaque. Here we present a case of benign MMT in a 12-y-old goat, and review the literature on uterine, cervical, and vaginal neoplasia in goats. The doe was presented with vaginal discharge and was euthanized because of the high suspicion of intraabdominal neoplasia. On gross examination, an ulcerated vaginal mass was identified. Histologically, 2 distinct cell populations were present: smooth muscle cells that were well differentiated and positive for alpha-smooth muscle actin, and ciliated columnar epithelial cells that lined ductal structures and had no signs of malignancy. These findings led to the diagnosis of neoplasia of Müllerian origin. Benign MMT should be considered as a differential diagnosis for uterine and vaginal neoplasms in goats.

Initial surgical management of bulky malignant type II tumors of the endometrium by a robotic approach.

Type II uterine tumors often present with very large, necrotic tumor burden in the uterus that leads to dilation and effacement of the cervix. In patients with this presentation, conventional hysterectomy poses a much greater challenge as the ureters are composed of the mass of the tumor and are at an increased risk for injury. Given this surgical challenge, many of these patients may begin with neoadjuvant chemo-radiation. However, these treatment modalities are associated with significant toxicity and negatively impact patient quality of life. Therefore, we describe a minimally invasive robotic surgical approach that aims to optimize quality of life without sacrificing prognosis. Outcomes of 4 patients are presented.

Adjuvant Treatment Modalities, Prognostic Factors, and Outcome of the Uterine Carcinosarcoma.

OBJECTIVE: To determine the impact of adjuvant therapy and other factors associated with the recurrence and survival of patients with uterine carcinosarcoma (UCS). METHODS: A total of 102 patients who underwhent surgery for UCS from 1998 to 2017 were included in the analysis. Data were analyzed using Kaplan-Meier methods and Cox proportional hazards regression. RESULTS: At 240 months, the actuarial recurrence rate was 34.3%. Distant recurrence was the most common recurrence pattern. Patients with higher CA 125 levels, sarcoma dominance, cervical involvement, advanced stage, no lymphadenectomy, and residual tumour had a significiantly higher risk of recurrence. Five-year disease-free survival (DFS) and overall survival (OS) were 67% and 77%, respectively. FIGO stage was found to be an independent prognostic factor for DFS and OS. Sarcoma dominance was independently associated with decreased OS. CONCLUSION: Sarcoma dominance is associated with poor survival in UCS. Adjuvant treatment was not found to affect recurrence or survival. Given this finding, more effective postoperative strategies are needed.

Staging MRI of uterine malignant mixed Müllerian tumors versus endometrial carcinomas with emphasis on dynamic enhancement characteristics.

PURPOSE: To determine whether staging pelvic magnetic resonance imaging (MRI) can distinguish malignant mixed Müllerian tumor (MMMT) from EC. METHODS: Thirty-seven treatment-naïve patients with histologically proven uterine MMMT and 42 treatment-naïve patients with EC, treated at our institution, were included in our retrospective study. Staging pelvic MRI scans were reviewed for tumor size, prolapse through cervical os, and other features. Time-intensity curves for tumor and surrounding myometrium regions of interest were generated, and positive enhancement integral (PEI), maximum slope of increase (MSI), and signal enhancement ratio (SER) were measured. The Fisher's exact test or Wilcoxon rank-sum test was used to compare characteristics between disease groups. Multivariate and univariate logistic regression models were used to distinguish MMMT from EC. Receiver operating characteristic analysis and the area under the curve (AUC) were used to evaluate prediction ability. RESULTS: MMMTs were larger than ECs with higher rate of tumor prolapse and more heterogeneous tumor enhancement compared to ECs. During the late phase of contrast enhancement, 100% of ECs, but only 84% of MMMTs, had lower signal intensity than the myometrium. Threshold PEI ratio ≥ 0.67 predict MMMT with 76% sensitivity, 84%, specificity and 0.83 AUC. Threshold SER ≤ 125 predict MMMT with 90% sensitivity, 50% specificity, and 0.72 AUC. CONCLUSION: MMMTs may show more frequent tumor prolapse, more heterogeneous enhancement, delayed iso- or hyper-enhancement, higher PEI ratios, and lower tumor SERs compared with EC. MRI can be used as a biomarker to distinguish MMMT from EC based on the enhancement pattern.

Post-radiation Mullerian adenosarcoma with sarcomatous overgrowth: rare presentation of an uncommon malignancy.

Mullerian adenosarcoma is an uncommon biphasic malignant uterine tumor. It is composed of benign epithelial and malignant stromal elements. We present a case of a 45-year-old woman who presented with post-menopausal bleeding for three months. She had a significant past medical history of pelvic irradiation for squamous carcinoma of cervix 20 years ago. Pathology revealed adenosarcoma with sarcomatous overgrowth. The patient had a recurrence of pure sarcoma three months later and unfortunately succumbed to her disease. The role of radiation in the pathogenesis of adenosarcoma has been uncommonly described compared to its well established role in the development of carcinosarcoma. Our case fulfils the criteria for a radiation induced sarcoma. We review the salient clinical and pathological features of this uncommon lesion highlighting the importance of sarcomatous overgrowth in these lesions and the possible role of radiation in the development of these tumors.

Primary tumor types and origins in positive abdominopelvic washing cytology, a single institution experience.

INTRODUCTION: Abdominopelvic washing cytology is a common specimen evaluated for ovarian, fallopian tubal, and peritoneal cancer staging or other nongynecologic malignancies presented as metastases. We reviewed our experience in diagnosing abdominopelvic washing specimens and assessing the primary tumor types and origins of the positive abdominopelvic washings. MATERIALS AND METHODS: A pathology archive database search was performed for abdominopelvic washing specimens from 2007 to 2018. The corresponding cytologic diagnoses, results of ancillary studies, clinical histories, and surgical follow-up were reviewed. The primary sites were determined based on the synoptic reports, when available. RESULTS: A total of 5.8% (350 of 6023) of cases were positive for malignancy or neoplasm. Additionally, 1.3% (78 of 6023) were diagnosed as atypical cells. Of the 350 positive cases, 93.4% were müllerian tumors. The frequency of primary sites for müllerian tumors in descending order were: ovary, uterus, fallopian tube, peritoneum, and uncertain müllerian sites. The common ovarian tumors identified in pelvic washing in descending order were: high-grade serous carcinoma, serous borderline tumor, clear cell carcinoma, low-grade serous carcinoma, and endometrioid carcinoma. Gastrointestinal, breast, bladder, and lymphoma primaries were the 23 nongynecologic tumors identified in pelvic washings. CONCLUSIONS: Positive findings in abdominopelvic washing cytology is rare. The majority of the positive cases were from müllerian origins, with ovary and uterus as the most common sites. Endometrial adenocarcinoma, endometrioid type and ovarian high-grade serous carcinoma were the most common tumor types. Knowing prior history of malignancy, morphologic comparison with concurrent surgical cases, and performing ancillary studies are keys to improve diagnostic accuracy of abdominopelvic washings.

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.

Carcinosarcoma primario de vagina: neoplasia infrecuente. Reporte de caso y revisión de la literatura / Primary carcinosarcoma of the vagina: an uncommon neoplasm. Case report and literature review

Los tumores mixtos mullerianos del tracto genital femenino o carcinosarcomas son un grupo raro de neoplasiascomprendiendo menos del 2% de todos los canceres de origen ginecológico (1). El carcinosarcoma primario de vagina es un tumor muy raro, siendo muy pocos los casos reportados en la literatura

The Mullerian mixed tumors of the female genital tract or carcinosarcomas are a rare group of neoplasms com-pending less than 2% of all gynecological cancers origin (1). Primary carcinosarcoma of vagina is a very rare,being very few cases like this that have been reported in the literature

Mullerian adenosarcomas of the uterine cervix with sarcomatous overgrowth.

Mullerian adenosarcoma with sarcomatous overgrowth (MASO) of the uterine cervix is an extremely rare variant of adenosarcoma of the genital tract associated with aggressive clinical course. We searched the PubMed and Medline databases for MASO of the cervix and we identified and reviewed eleven cases published between years 2004 and 2017. The most common clinical picture includes abnormal vaginal bleeding, postcoital bleeding, pelvic pain and foul-smelling vaginal discharge. Therapeutic options for MASO are still undefined. Radical hysterectomy with sufficient tumour-free margins combined with adjuvant chemotherapy and radiotherapy should serve as an effective treatment tool with favourable outcome.

Unexpected PAX8 Immunoreactivity in Metastatic High-grade Breast Cancer.

Immunohistochemistry (IHC) is often critical for distinction between metastatic carcinomas of Mullerian organ and breast origin. Paired box family protein 8 (PAX8) has been described as a transcription factor highly specific to neoplasms derived from Mullerian organs, thyroid, and kidney. PAX8 IHC with polyclonal and monoclonal antibody reagents was performed on 27 primary and 22 metastatic breast carcinomas. Eight of 27 primary breast carcinomas (30%) were positive for PAX8 with the monoclonal antibody reagent only; 0 of 22 were polyclonal anti-PAX8 immunoreactive. Substantial numbers of metastases had positive immunoreactivity for polyclonal anti-PAX8 (23%). Each of these metastases and additional cases (45% total) also had positive immunoreactivity for monoclonal anti-PAX8, including 5 of 7 brain metastases. IHC with monoclonal anti-PAX8 was positive on 6 of 7 primary breast carcinomas corresponding to PAX8-positive metastases. Together, these results indicate a significant fraction of breast carcinoma metastases and corresponding primary neoplasms have immunoreactivity for PAX8, and positivity rates depend on the antibody used. Diagnoses of metastatic breast carcinoma were achieved with the aid of clinical history and additional IHC in cases of PAX8 immunoreactivity. Contextual interpretation is imperative for PAX8 IHC, particularly when the differential diagnosis includes metastatic breast carcinoma with limited diagnostic material available.

Rare primary malignant mixed Müllerian tumor of the mediastinum: A case report.

RATIONALE: Malignant mixed Müllerian tumor (MMMT) of extragenital organs is rare, especially in male. To our knowledge, this is the first reported case of primary MMMT at the mediastinum in male. PATIENT CONCERNS: A 54-year-old male was admitted to the hospital due to repeated stimulating dry cough for 2 years. His systemic examination was unremarkable. Laboratory workup revealed that all blood indicators were within normal limits. But subsequent computerized tomography (CT) scans of chest showed an abnormal soft tissue density in the area of its left, measuring approximately 4 cm in anterior-posterior dimension and 7 cm in maximum transverse dimension. DIAGNOSES AND INTERVENTIONS: The pathogenesis of these tumors remains controversial. The diagnosis is combined with biopsy and immunohistochemical staining. So, the patient underwent radical surgical resection and pathologic examination of the excised specimen was consistent with the diagnosis of MMMT. After surgery, he was treated by sequential chemoradiotherapy. OUTCOMES: The patient died from tumor recurrence 16 months later. LESSONS: MMMT is a rare, highly aggressive tumor associated with interesting embryological origin, a definite diagnosis of which is only confirmed on pathological assessment. Due to its high degree of malignancy and high rate of recurrence, complete macroscopic excision of the tumor is recommended as soon as possible.

Uterine carcinosarcoma (malignant mixed Müllerian tumor): case report in a goat and literature review.

Carcinosarcomas are biphasic malignant tumors composed of 2 distinct neoplastic cell populations, epithelial cells and mesenchymal cells. A 13-y-old, female, mixed-breed goat was presented with a 1-wk history of anuria and lethargy. Transabdominal ultrasonography showed an irregular and heterogeneous structure in the region of the bladder and uterus and changes in the echogenicity of both kidneys. Given the poor prognosis, euthanasia was elected. Autopsy revealed a large mass within the uterine cervix and confirmed the changes in the urinary tract. Histopathology and immunohistochemistry revealed a mixed, anti-cytokeratin AE1/AE3-positive epithelial, and vimentin-positive mesenchymal neoplasm consistent with a homologous carcinosarcoma, also called malignant mixed Müllerian tumor, with areas of double-labeling. We highlight the complexity of the diagnosis of uterine neoplasms in domestic animals and in goats in particular.

Uterine Carcinosarcomas - Diagnosis and Management.

Uterine carcinosarcomas are rare tumors that account for less than 5% of all uterine malignancies. These tumors (previously called malignant mixed Müllerian tumors) are dedifferentiated carcinomas that comprise carcinomatous and sarcomatous elements and arise from a single malignant clone. They are considered a high-risk variant of endometrial adenocarcinoma because carcinosarcomas share more similarities in epidemiology, risk factors, and clinical behavior with endometrial carcinoma than with uterine sarcomas. The clinical features, diagnosis, staging, and treatment of uterine carcinosarcoma will be discussed in this review.

Low grade Mullerian adenosarcoma of pouch of Douglas recurring as bilateral ovarian high grade Mullerian adenosarcoma with rhabdomyosarcomatous overgrowth after 11 years.

Mullerian adenosarcoma (MA) of ovary with sarcomatous (rhabdomyoblastic) overgrowth is an extremely rare malignant type of female genital tract neoplasm. These tumours are highly aggressive and presence of heterologous elements is associated with worse prognosis. A 44 year old female presented with lower abdominal pain and distension. She had history of removal of tumour from pouch of Douglas in 2006 for which she did not receive any additional treatment nor did she keep continuous follow up. Current preoperative radiological examination revealed bilateral ovarian masses. She underwent abdominal hysterectomy with bilateral oophorectomy. Microscopic examination revealed biphasic tumours exhibiting sarcomatous overgrowth with rhabdomyoblastic differentiation. Review of the previous biopsy revealed low grade Mullerian adenosarcoma without sarcomatous overgrowth. Hence the current tumour was considered recurrent. This report highlights the aggressive nature of MA even with low grade morphological features and emphasizes the importance of continuous follow up and additional treatment.

[Retroperitoneal mixed malignant mullerian tumor : exceptional localisation and prognosis]. / Une tumeur mullérienne mixte maligne rétropéritonéale de localisation et pronostic exceptionnels.

Primary retroperitoneal carcinosarcoma or mixed malignant mullerian tumor (MMMT) is an extremely rare clinical entity. These aggressive tumors arise most commonly from genital tract. The retroperitoneal location is exceptional. Here we report the case of a 63-years old female diagnosed with heterologous, extra-genital, retroperitoneal carcinosarcoma, with malignant cells in the ascitic fluid and extra-ovarian metastatic implants. She was treated with complete radical surgical treatment consisting of resection of the retroperitoneal tumor, with omentectomy, hysterectomy, bilateral salpingooophorectomy and lumbo-aortic and pelvic lymphadenectomy. She received adjuvant chemotherapy with 6 cycles of Carboplatin and Paclitaxel. She is in complete clinical and radiological remission since the end of chemotherapy, for a total of 113 months. To our knowledge, this is the longest reported disease free survival of the extra-genital retroperitoneal MMMT. This case and the review of the literature illustrate the importance of surgical treatment. However, there are no evidence-based guidelines for the systemic management of these tumors.

Le carcinosarcome rétropéritonéal ou tumeur mullérienne mixte maligne (TMMM) est une entité clinique extrêmement rare. Ce sont des tumeurs agressives du tractus génital provenant des tissus mullériens. Les localisations rétropéritonéale et extra-génitale sont exceptionnelles. Ce cas clinique concerne une patiente de 63 ans atteinte d'un carcinosarcome extra-génital, retropéritonéal, hétérologue associé à des cellules malignes dans le liquide péritonéal et des implants métastatiques extra-ovariens. Elle a bénéficié d'une laparotomie de résection de la masse rétropéritonéale avec hystérectomie, annexectomie bilatérale, omentectomie et lymphadénectomie pelvienne et lombo-aortique, et a reçu une chimiothérapie adjuvante par 6 cycles de Carboplatine et Paclitaxel. Elle est en rémission clinique et radiologique complète depuis la fin de sa chimiothérapie, soit une durée de 113 mois. A notre connaissance, il s'agit de la durée de suivi sans récidive la plus longue rapportée dans la littérature. Ce cas, ainsi que la revue de littérature, mettent en évidence l'importance du traitement chirurgical. Par contre, il n'existe pas de standard thérapeutique pour la prise en charge adjuvante ou systémique de ces tumeurs.

Primary malignant mixed Müllerian tumors of the fallopian tube with cervix metastasis: A rare case report and literature review.

RATIONALE: Primary malignant mixed mullerian tumors of the fallopian tube is very rare and has only 1 case in the current literature with cervix metastasis. PATIENT CONCERNS: We reported a 49-year-old woman sufferring from primary malignant mixed mullerian tumors of the fallopian tube with cervix metastasis, and the imaging examination found a strip of solid mass in the right fallopian tube and a nodular mass in cervical canal, which were both hyperintense on T2 weighted image (T2WI) and diffusion weighted image (DWI) and continuous moderate enhancement on dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). DIAGNOSES: The diagnosis was confirmed according to the specific anatomical location and pathological examination which was proved as primary malignant mixed mullerian tumors of the fallopian tube with cervix metastasis. INTERVENTIONS: The patient underwent radical hysterctomy, bilateral adnexectomy, pelvic lymph node dissection, omentum majus excision and intravenous chemotherapy. OUTCOMES: Her posttreatment condition was good. LESSONS: Primary malignant mixed mullerian tumors of the fallopian tube can be located by magnetic resonance image examination, which may also offer several diagnostic tips according to changes in signal and enhancement. When combined with pathological findings, qualitative diagnosis can be determined. Surgery and adjuvant chemotherapy are considered as effective methods. Our paper discussed its epidemiology, clinical symptoms, pathologic characters, therapeutic method as well as magnetic resonance imaging findings suggesting the diagnosis and differential diagnosis, including precontrast scan, contrast scan and diffusion weighted image and provided magnetic resonance imaging characteristics of primary malignant mixed mullerian tumors of the fallopian tube described in other literatures.

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.