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.

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.

Primary peritoneal carcinosarcoma: A report of two cases.

OBJECTIVE: Carcinosarcomas also known as malignant mixed mullerian tumors (MMMTs) contain both carcinomatous and sarcomatous elements. Most MMMTs are arising from female genital tract, including ovaries, uterus and fallopian tubes. Extragenital carcinosarcomas are extremely rare, with an estimation less than 40 cases so far. CASE REPORT: We report two cases of primary peritoneal carcinosarcomas. An 81-year-old woman with pelvic peritoneal carcinosarcoma, heterologous type, was treated with incomplete surgery without further chemotherapy, and died of disease soon. The other one was a 76 year-old woman with abdominal peritoneal carcinosarcoma, homologous type. After optimal debulking surgery and subsequent 6 cycles of combination of paclitaxel and carboplatin chemotherapy, the patient is free of tumor half of year. CONCLUSION: Active therapy, including complete cytoreduction surgery and carboplatin-paclitaxel chemotherapy might offer a chance of disease control for these unusual primary peritoneal carcinosarcomas.

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.

[Multimodal treatment of malignant mixed Müllerian tumor]. / Malignus kevert Müller-cso-eredetu tumor komplex kezelése.

INTRODUCTION AND AIM: The aim of our study was to evaluate the prognostic factors and treatment options of a very rare and highly aggressive type of uterine neoplasms, the malignant mixed Müllerian tumor, known as carcinosarcoma. METHOD: Between 2009 and 2017, 29 patients were treated with malignant mixed Müllerian tumor. At stage I, surgery and postoperative radiotherapy were performed. At stages II-IV, trimodal treatment (surgery, chemotherapy and radiotherapy) was administered. RESULTS: The average age of patients was 68.51 (49-90) years, mean body mass index was 30.22 (20.90-37.22). We have experienced recurrence of disease after complete resection in 6 cases (4 of 6 patients did not accept radiation therapy). Local recurrence has occurred after an average 15.52 (6-36) months, distant metastasis with an average 19.2 (8-32) months. Overall survival was 11.92 (1-75) months. Six patients are free of tumours at the moment. CONCLUSIONS: As overall survival has not increased in recent decades by using combined chemotherapy, there is no congruent consensus associated with the optimal treatment. The standard surgical treatment is total abdominal hysterectomy with bilateral oophorectomy, although due to high rates of recurrence and metastases, the necessity of lymphadenectomy and postoperative treatment is in the focus of recent studies. Though postoperative irradiation improves local control, the beneficial effect on overall survival is still not proven. Adjuvant chemotherapy decreases the rate of both pelvic and extrapelvic recurrence at the same time, although there is no recommendation for the optimal chemoterapeutic agent. Multimodal therapy should lead to better outcomes. Recently there are many ongoing studies with biologic and target therapies to improve efficiency, however, the relevant results will be disclosed in many years only, due to the small number of patients. Orv Hetil. 2018; 159(19): 741-7747.

Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review.

BACKGROUND: Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours' histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes. CASE PRESENTATION: A 79-year-old woman was referred to our Department with an abdominal mass resembling a fibroid with a haemorrhage. Her gynaecological history was negative. A transvaginal and transabdominal ultrasound examination revealed a multicystic mass resembling an ovarian tumour arising from the pelvis and extending up to the abdomen. At laparotomy a peritoneal mass arising from Douglas peritoneum was resected. The uterus and adnexa appeared normal, and a supra-cervical hysterectomy with bilateral salpingo-oophorectomy was performed. No macroscopic residual disease was present. Final pathology diagnosed a malignant peripheral nerve sheath tumors with divergent differentiation. Four weeks later a new, multicystic mass was found. Due to the progressive poor condition, the patient died four months after diagnosis. Histological slides were reviewed by external expert pathologists and the final diagnosis was of extra-genital adenosarcoma with sarcomatous overgrowth. Furthermore, we also collected and analysed articles written in English regarding extra-uterine and extra-ovarian adenosarcomas published between January 1974 and October 2016. PubMed was used as a database for this search. Clinical and pathological characteristics, treatments and outcomes were assessed. CONCLUSIONS: Only 41 cases has been reported in literature. Previous endometriosis and sarcomatous overgrowth showed an inverse effect on prognosis. Endometriosis was confirmed to have a positive effect on disease free survival Complete surgical resection is the mainstay of treatment. A worldwide registry is urgently required to collect data to standardize treatment and to obtain reliable data on prognosis.

A unique case of malignant mixed müllerian tumor with metastasis to the thyroid: a case report.

We present a case of a 61-year-old postmenopausal woman with a known diagnosis of malignant mixed müllerian tumor (MMMT) originally treated with systemic chemotherapy and total abdominal hysterectomy plus bilateral salpingo-oophorectomy, who developed a left cervical mass noted on a follow-up visit. Imaging and nuclear testing were both performed confirming the presence of a cervical mass arising from the thyroid. Our patient underwent surgical resection of the mass, and biopsy of the specimen was consistent with metastatic uterine carcinosarcoma mixed müllerian tumor. Chemotherapy was initiated with a positive outcome.

Mixed malignant mullerian tumor with neuroendocrine features in an irradiated uterus for cervical carcinoma. A unique association? A morphological, immunohistochemisty and ultrastructural study.

Chemo-radiation represents an effective therapy for carcinoma of the uterine cervix. The endometrium may however receive a consistent dose of mutagenic radiations and patients may have an increased risk of secondary malignancies. Endometrial mixed malignant mullerian tumor (MMMT) is a rare, highly aggressive disease, and neuroendocrine features are even rarer. A 68 years old woman underwent radio-chemotherapy for a squamous cell carcinoma of the cervix. Follow up was uneventful until, eight years after radio-chemotherapy, imaging exams detected a diffuse enlargement of the uterine body. Radical hysterectomy revealed a multiphasic lesion with both sarcomatous and mixed carcinomatous components. The carcinomatous, component presented neuroendocrine histologic and ultrastuctural features and an intense expression of neuroendocrine immunohistochemistry markers. No residual cervical carcinoma was documented (pR0). The patient died of disease after 9 months. Reported cases further demonstrate how the irradiation of the uterus for cervical cancer carries a not negligible risk of developing a second endometrial cancer. The second cancer may develop years after initial therapy and may have aggressive histologic and clinical features. This case underlines the importance for a long follow-up in women having received radio-chemotherapy alone.

Mullerianosis vesical / Mullerianosis of the urinary bladder

No disponible

Primary malignant mixed Müllerian tumour (MMMT) of the vagina and review of the literature.

Primary malignant mixed Müllerian tumour (MMMT) of the vagina is a rare entity. We report a case of a 62-year-old woman who presented with a fixed and hard anterior vaginal wall mass with contact bleeding. She proceeded to have an anterior infralevator pelvic exenteration with urethrectomy and anterior vaginectomy, creation of an ileal conduit and bilateral lymph node dissection. Histopathological examination and immunohistochemistry confirmed the diagnosis of primary MMMT of the vagina. The patient was stage IVA at diagnosis. Despite chemotherapy and radiotherapy, she had progressive disease and eventually passed away at the age of 65 years.

Serous carcinomatous component championed by heparin-binding EGF-like growth factor (HB-EGF) predisposing to metastasis and recurrence in stage I uterine malignant mixed mullerian tumor.

The stage I uterine malignant mixed mullerian tumor (MMMT) shows different potential for progression. We reason that MMMTs with high-grade carcinomatous component and positivity for HB-EGF are prone to recurrence/metastasis in the early stage. A retrospective clinical and histopathologic review with immunohistochemical staining for HB-EGF, EGFR, and integrin-α5 was performed for 62 surgically staged MMMT cases. Recurrence/metastasis (RM) is 6/18 (33%) in stage I disease. Of all the clinicopathologic variables and biomarkers analyzed for stage I MMMT, serous carcinomatous component (83% [5/6] versus 17% [1/12], P = .0015) and HB-EGF expression (100% [6/6] versus 50% [6/12], P=.0339) were significantly different between groups with RM and without RM. The presence of serous carcinoma in all stages was 83% (5/6) in stage I with RM, 8% (1/12) in stage I without RM, 20% (1/5) in stage II, 36.4% (8/22) in stage III and 64.7% (11/17) in stage IV; this was paralleled by HB-EGF expression of 100% (6/6), 50% (6/12), 40% (2/5), 50% (11/22) and 71% (12/17) with a correlation coefficient r=0.9131 (P=.027). HB-EGF and integrin-α5 were highly expressed in MMMTs bearing serous carcinoma component, compared to endometrioid and unclassifiable/miscellaneous subtypes (84.6%/47.6%/33.3%, P=.025 for HB-EGF; and 61.5%/42.9%/20.0%, P=.021 for integrin-α5). The EGFR positivity was comparable among the three subtypes (48.1%, 47.6% and 26.7%, P=.326). This study indicates that serous carcinomatous component championed by expression of HB-EGF predisposes to recurrence/metastasis in stage I MMMT. This process might involve integrin-α5 and does not seem to require overexpression of EGFR. Further study is required.

Extragenital malignant mixed Mullerian tumor in the incisional hernia - primary carcinosarcoma in the abdominal wall: case report.

INTRODUCTION: This report presents a primary Mullerian carcinosarcoma localized in the incisional hernia i.e. anterior abdominal wall.There is no data in the literature about this localization of extragenital Mullerian carcinosarcoma. CASE OUTLINE: The patient had previous medical history of right-sided ovarian cystadenocarcinoma managed by hysterectomy, bilateral ovariectomy and chemotherapy. An incisional hernia occurred 1 year after the operation and Mullerian carcinosarcoma at the right border of the incisional hernia 16 years later. There was no tumor spreading into the abdominal cavity and pelvis. Full thickness of the abdominal wall resection and coexisting incisional hernia resulted in a large 25x20 cm abdominal wall defect managed by the modified components separation technique and implanting meshes. CONCLUSION: Major abdominal wall resection and abdominal wall reconstruction using the modified components separation technique reinforced with meshes could be one of possible solutions in the surgical treatment of primary malignant mixed Mullerian tumor localized in the abdominal wall.

Heterologous type of malignant mixed Müllerian tumor of the uterus presenting as a vulvar mass.

Carcinosarcoma is a rare, extremely aggressive tumor of the uterus with a poor prognosis. The authors describe a case of a 78-year-old woman who presented with a giant mass protruding through the cervix, vagina, and vulva. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. The histopathological examination of the surgical specimen revealed a malignant mixed Müllerian tumor. The clinical and pathological features, molecular data, and prognosis of this aggressive neoplasm are discussed. Although uterine carcinosarcomas are extremely rare, when a postmenopausal woman with a vulvar mass is admitted to the gynecology clinic, the physician should consider that the mass may be a carcinosarcoma.

Non-puerperal uterine inversion caused by malignant mixed mullerian sarcoma.

Non puerperal uterine inversions resulting from mixed mullerian uterine sarcoma are rare. We present a case of a postmenopausal woman with a large mixed mullerian tumour presenting as a huge abdominopelvic mass. It required a challenging surgical procedure to remove the tumour which is also described along with the review of literature.

Bilateral ovarian malignant mixed Mullerian tumour: a rare entity.

A 52-year-old postmenopausal woman presented with abdominal distension for 2 months. She had no vaginal bleeding and no relevant medical history. A physical examination showed a large palpable pelvic mass. A CT scan showed an ill-defined, heterogeneously enhancing, soft tissue attenuation lesion in the lower abdomen and pelvis involving the right adnexal region with massive ascites, and the left ovary did not reveal any evidence of disease. The omentum was thickened and heterogeneous. The patient underwent a total hysterectomy with infracolic omentectomy. The diagnosis was bilateral ovarian malignant mixed Mullerian tumour (MMMT) with omental deposits. Aggressive treatment includes surgery and chemotherapy. Women with this tumour have a significant increased risk of death compared to women with epithelial ovarian cancer and very poor prognosis. We report a case of MMMT of the ovary with clinicopathological correlation and diagnostic difficulties as malignant epithelial tumour resemble ovarian stromal sarcoma in postmenopausal female.

Transabdominal cardiophrenic lymph node dissection (CPLND) via incised diaphragm replace conventional video-assisted thoracic surgery for cytoreductive surgery in advanced ovarian cancer.

OBJECTIVE: The objective of this study is to describe the feasibility of the new approach, transabdominal CPLND, via incised diaphragm in patients with ovarian cancer by gynecologic oncologists instead of the conventional video-assisted thoracic surgery. METHODS: From November 2008 to December 2011, 11 women (10 primary and 1 recurrent ovarian cancers) underwent CPLND for the extensive cytoreductive surgeries via incised muscle of the right diaphragm by gynecologic oncologists. All ≥5 mm tumors in CPLN, which were the criterion for suspicious malignancy on preoperative axial computed tomogram, were completely resected by gynecologic oncologists. RESULTS: The median tumor size of the CPLN was 10mm (range, 7-17 mm) and metastasis was identified in 45% (5/11) of ≥5 mm CPLN on preoperative computed tomogram. The median number of harvested CPLND was 3 (range 1-12) and metastatic node was 1 (range, 0-10). There was no significant morbidity related to CPLND and mortality associated with surgery. Ten patients achieved the no gross residual disease and one patient accomplished gross residual-1, indicating residual disease measuring ≤1 cm in maximal diameter. CONCLUSION: Transabdominal CPLND via incised diaphragm is feasible as a part of the cytoreductive surgery without significant morbidities by gynecologic oncologist. This procedure could substitute the conventional video-assisted thoracic surgery.

Resection of an intramedullary high cervical metastasis from a malignant mixed Muellerian tumour.

We present a 66-year-old female patient with a high cervical intramedullary metastasis from a malignant mixed Muellerian tumour (MMMT; carcinosarcoma) with concomitant syringomyelia. She was admitted to our clinic with symptoms of cervical myelopathy. MRI revealed an intramedullary tumour of 2.6cm×1.2cm at the cervical vertebral body C2. We performed a laminectomy on C2 followed by a dorsal median myelotomy from C1 to C3 to resect the tumour. The surgical intervention removed the tumour completely and resolved the syringomyelia. During the 36months of follow-up, the patient presented in a stable condition with no evidence of tumour recurrence. To our knowledge, this is the first report of an intramedullary metastasis of a MMMT.