ABSTRACT
RATIONALE: Malignant mixed Mullerian tumor (MMMT) is also known as carcinosarcoma, mostly occurring in the uterus, and occurred in ovary is very rare. The disease is highly aggressive. Two cases of MMMT of ovary and their imaging characteristics were collected in our study. PATIENT CONCERNS: A 77-year-old and an 80-year-old woman were admitted to the obstetrics and gynecology department of our hospital on June 22, 2019, and December 10, 2019, respectively. The first patient presented with abdominal distension with poor appetite without obvious triggers. Another patient had been menopausal for 18 years and presented with vaginal bleeding with dull pain in the left lower abdomen without obvious cause. DIAGNOSES: Both patients underwent pelvic magnetic resonance imaging plain and enhanced scan after admission, which indicated pelvic mass. Postoperative pathology confirmed MMMT in the adnexal region. INTERVENTIONS: Both patients underwent total hysterectomy and bilateral adnexectomy. OUTCOMES: Postoperatively, the first patient developed complications such as renal failure and gastrointestinal bleeding and was sometimes unconscious. Symptomatic treatment was not effective, and the patient died about 1 month after discharge. The other patient recovered well after surgery, and imaging examinations confirmed no evidence of regrowth of the tumor during an average 36-month follow-up. LESSONS: The disease is highly malignant and progresses rapidly. The elevation of CA125 should be taken seriously. The imaging findings of MMMT has certain characteristics. Multi-sequence magnetic resonance imaging may help to distinguish this disease from other pelvic tumors. Once found, surgical treatment is needed as soon as possible, followed by postoperative adjuvant radiotherapy and chemotherapy.
Subject(s)
Mixed Tumor, Mullerian , Ovarian Neoplasms , Ovary , Aged, 80 and over , Female , Humans , Hysterectomy , Magnetic Resonance Imaging , Mixed Tumor, Mullerian/diagnostic imaging , Ovary/pathology , Uterus/pathology , Aged , Ovarian Neoplasms/diagnostic imagingABSTRACT
This was a 57-year-old woman who presented with mild discomfort in the right groin. Physical examination revealed a mass in the right groin, and by ultrasound, the mass was hypoechoic and solid with some internal vascularity. The clinical differential diagnosis included lymphoma and others. The mass was excised for pathologic evaluation. Gross examination of the specimen revealed a 3 × 2.4 × 2 cm, solid and cystic mass. Microscopically, it was a biphasic tumor consisting of carcinomatous and sarcomatous components. The tumor was seen contiguous with endometriosis and atypical endometrioid hyperplasia. The histologic findings were consistent with malignant mixed Mullerian tumor (MMMT) arising from endometriosis in the right groin. The tumor involved the resection margin. Subsequent chest/abdominal/pelvic computed tomography did not reveal evidence of tumors, and diagnostic peritoneal/pelvic laparoscopy did not show diseases. Postoperatively, the patient received 6 cycles of chemotherapy consisting of carboplatin and paclitaxel, followed by radiation in the right groin. Malignant transformation from endometriosis occurs in less than 1% of endometriosis cases, and about 80% of the transformed tumors occur in the ovaries. The most commonly transformed malignant tumors are endometrioid and clear cell carcinomas, with rare adenosarcoma and endometrial stromal sarcoma reported. To our knowledge, we are reporting the first case of MMMT arising from endometriosis in the groin.
Subject(s)
Adenosarcoma , Endometriosis , Mixed Tumor, Mullerian , Female , Humans , Middle Aged , Groin/pathology , Endometriosis/pathology , Adenosarcoma/diagnosis , Adenosarcoma/pathology , Adenosarcoma/surgery , Pelvis/pathologyABSTRACT
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.
Subject(s)
Mixed Tumor, Mullerian , Uterine Inversion , Uterine Neoplasms , Aged , Female , Humans , Mixed Tumor, Mullerian/diagnosis , Mixed Tumor, Mullerian/pathology , Mixed Tumor, Mullerian/surgery , Uterine Inversion/diagnosis , Uterine Inversion/etiology , Uterine Inversion/surgery , Uterine Neoplasms/complications , Uterine Neoplasms/diagnosis , Uterine Neoplasms/surgery , Uterus/pathology , Uterus/surgeryABSTRACT
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.
Subject(s)
Goat Diseases , Mixed Tumor, Mullerian , Vaginal Neoplasms , Animals , Diagnosis, Differential , Female , Goat Diseases/diagnosis , Goat Diseases/pathology , Goats , Humans , Mixed Tumor, Mullerian/diagnosis , Mixed Tumor, Mullerian/pathology , Mixed Tumor, Mullerian/veterinary , Vagina , Vaginal Neoplasms/diagnosis , Vaginal Neoplasms/pathology , Vaginal Neoplasms/veterinaryABSTRACT
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.
Subject(s)
Mixed Tumor, Mullerian , Robotic Surgical Procedures , Uterine Neoplasms , Endometrium/pathology , Female , Humans , Hysterectomy , Mixed Tumor, Mullerian/pathology , Mixed Tumor, Mullerian/surgery , Quality of Life , Robotic Surgical Procedures/methods , Uterine Neoplasms/surgeryABSTRACT
Uterine adenosarcoma is a rare variant of mixed Mullerian tumors comprised of neoplastic glands with the benign appearance and sarcomatous stroma. The epithelium most often consists of endometrium- like cells, while the sarcomatous component usually shows low-grade homologous uterine sarcoma. These tumors present as a pelvic mass or an enlarged uterus with abnormal vaginal bleeding. Here, we present a case of 61 years old postmenopausal female patient with chief complaints of excessive vaginal bleeding and urine retention.
Subject(s)
Adenosarcoma , Mixed Tumor, Mullerian , Uterine Neoplasms , Adenosarcoma/diagnosis , Adenosarcoma/surgery , Female , Humans , Middle Aged , Uterine Hemorrhage/etiology , Uterine Neoplasms/diagnosis , Uterine Neoplasms/surgeryABSTRACT
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.
Subject(s)
Carcinosarcoma/therapy , Chemotherapy, Adjuvant/methods , Mixed Tumor, Mullerian/therapy , Radiotherapy, Adjuvant/methods , Uterine Neoplasms/therapy , Adult , Aged , Aged, 80 and over , Carcinosarcoma/pathology , Female , Humans , Middle Aged , Mixed Tumor, Mullerian/pathology , Neoplasm Recurrence, Local/epidemiology , Neoplasm Recurrence, Local/pathology , Neoplasm Staging , Prognosis , Retrospective Studies , Treatment Outcome , Uterine Neoplasms/pathologyABSTRACT
BACKGROUND: Malignant mixed Mullerian tumors of endometrial (MMMT-E) and ovarian (MMMT-O) origin are associated with poor prognosis. Suggestively epithelial-driven tumors, their treatment has shifted from anthracycline or ifosfamide-based towards taxane-based chemotherapy. It remains unclear whether this change associates with better outcomes. PATIENTS AND METHODS: A conjoined Australian and Swiss patient cohort of MMMT-E (N = 103) and MMMT-O (N = 17) was compared to patients with adenocarcinoma of the endometrium (EC, N = 172) and ovary (OC, N = 189). Clinicopathological characteristics, FIGO stage, first-line treatment, and patient outcomes were analyzed. The generated hypothesis was verified in an US-American cohort with high-grade serous ovarian cancer (HGSOC, N = 1290) and MMMT-O (N = 450) using immunohistochemistry and next-generation sequencing. RESULTS: Early stage I/II MMMT-E showed a survival plateau after 2.5 years, with no recurrence or death observed afterwards. Relapse-free survival was significantly worse in MMMT-E treated with platinum/taxanes (P = 0.024) compared to non-taxane regimen. Hypothesizing that also MMMT-O might benefit from an adjuvant non-paclitaxel regimen, a second independent cohort of MMMT-O and HGSOC patients was examined. p53 mutations dominated in both cancers with comparable frequency. PI3KCA and KRAS mutations were less frequent: they were more frequent in MMMT-O than in HGSOC (P = 0.015 and P = 0.018, respectively). MMMT-O responded better to a combination of carboplatin with anthracyclines than with taxanes (73.9% vs. 39.4%). CONCLUSION: Early stage I/II MMMT-E patients have excellent prognosis if no recurrence has appeared within the first 2.5 years. In MMMT-E, platinum/anthracycline or ifosfamide regimen associated with better outcomes than platinum/taxanes regimens. This might also apply to MMMT-O.
Subject(s)
Antineoplastic Agents/therapeutic use , Chemotherapy, Adjuvant/methods , Endometrial Neoplasms/drug therapy , Mixed Tumor, Mullerian/drug therapy , Ovarian Neoplasms/drug therapy , Taxoids/therapeutic use , Adult , Aged , Female , Humans , Middle AgedABSTRACT
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.
Subject(s)
Adenosarcoma , Radiotherapy/adverse effects , Adenosarcoma/etiology , Adenosarcoma/pathology , Cervix Uteri/pathology , Female , Humans , Middle Aged , Mixed Tumor, Mullerian/pathology , Neoplasms, Radiation-Induced/pathology , Uterine Cervical Neoplasms/pathology , Uterine Neoplasms/pathologyABSTRACT
Malignant mixed Müllerian tumor remains an important contributor to morbidity and mortality in women with uterine cancer. Surgery is the primary treatment modality, followed by chemotherapy and/or radiation for advanced disease or high-risk patients. Clinico-epidemiologic characteristics and outcomes in older versus younger women with Malignant mixed Müllerian tumor may differ. We analyzed and now report on 15 consecutive patients with uterine Malignant mixed Müllerian tumor treated at our institution from 2000 to 2018. The mean age at diagnosis was 65 years; 60% (9/15) patients were overweight/obese. Forty-six percent (7/15) had hypercholesterolemia, an association not previously linked with Malignant mixed Müllerian tumor in the literature. All patients but one had surgical excision of the tumor. A third of patients received adjuvant radiation therapy. A majority of patients received chemotherapy; the preferred regimen was carboplatin-paclitaxel. The patients older than 70 had a tendency towards a more advanced disease stage at diagnosis and a significantly shorter cancer-specific survival than their younger counterparts (6 months vs. 102 months (hazard ratio 1.32, p = 0.02)). Our study's conclusions are restricted due to its relatively small size, retrospective design, and some variation in the chemotherapy doses administered in individual patients. Larger studies are needed to confirm the significance of our findings.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Mixed Tumor, Mullerian/therapy , Uterine Neoplasms/therapy , Aged , Carboplatin/administration & dosage , Female , Humans , Middle Aged , Neoplasm Staging , Paclitaxel/administration & dosage , Retrospective StudiesABSTRACT
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.
Subject(s)
Endometrial Neoplasms/diagnostic imaging , Magnetic Resonance Imaging/methods , Mixed Tumor, Mullerian/diagnostic imaging , Uterine Neoplasms/diagnostic imaging , Adult , Aged , Aged, 80 and over , Endometrial Neoplasms/pathology , Female , Humans , Middle Aged , Mixed Tumor, Mullerian/pathology , Neoplasm Staging , Retrospective Studies , Sensitivity and Specificity , Tumor Burden , Uterine Neoplasms/pathologyABSTRACT
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.
Subject(s)
Adenocarcinoma, Clear Cell/diagnosis , Carcinoma, Endometrioid/diagnosis , Cytodiagnosis/methods , Fallopian Tube Neoplasms/diagnosis , Mixed Tumor, Mullerian/diagnosis , Ovarian Neoplasms/diagnosis , Peritoneal Neoplasms/diagnosis , Adenocarcinoma, Clear Cell/pathology , Adult , Aged , Aged, 80 and over , Carcinoma, Endometrioid/pathology , Fallopian Tube Neoplasms/pathology , Fallopian Tubes/pathology , Female , Follow-Up Studies , Humans , Middle Aged , Mixed Tumor, Mullerian/pathology , Neoplasm Staging , Ovarian Neoplasms/pathology , Ovary/pathology , Pelvis/pathology , Peritoneal Neoplasms/pathology , Peritoneum/pathology , Young AdultSubject(s)
Complement System Proteins/metabolism , Mixed Tumor, Mullerian/pathology , Paraneoplastic Syndromes, Ocular/metabolism , Retina/metabolism , Retinal Degeneration/metabolism , Uterine Neoplasms/pathology , Autoantibodies/blood , Complement System Proteins/immunology , Enzyme-Linked Immunosorbent Assay , Female , Humans , Paraneoplastic Syndromes, Ocular/pathologyABSTRACT
Background. Ovarian carcinosarcomas are rare aggressive biphasic tumors. Evidence suggests that these tumors are monoclonal and that the sarcoma component is derived from a stem cell undergoing divergent differentiation. Currently, there remains a paucity of data regarding its origin, with few reports suggesting an association with serous tubal intraepithelial carcinoma (STIC) by immunohistochemistry and genetics. Objective. We sought to determine the relationship of carcinosarcoma to high-grade serous carcinoma and STIC by investigating for similar mutation signatures through next-generation sequencing. Methodology. A case of carcinosarcoma with associated high-grade serous carcinoma and STIC was macrodissected, and next-generation sequencing was performed on each component separately. Results. The STIC, high-grade serous carcinoma component, and chondrosarcoma component were all diffusely positive for p53 and p16 by immunohistochemistry. Next-generation sequencing demonstrated an identical TP53 gene c.376-1G>A 5' splice site pathogenic mutation in all 3 components. Conclusions. Our findings suggest that carcinosarcomas may also originate from the fallopian tube.
Subject(s)
Carcinosarcoma/genetics , Cystadenocarcinoma, Serous/genetics , Fallopian Tube Neoplasms/pathology , Mixed Tumor, Mullerian/genetics , Ovarian Neoplasms/genetics , Aged , Carcinosarcoma/diagnosis , Carcinosarcoma/secondary , Cyclin-Dependent Kinase Inhibitor p16/genetics , Cystadenocarcinoma, Serous/diagnosis , Cystadenocarcinoma, Serous/secondary , DNA Mutational Analysis , Fallopian Tube Neoplasms/diagnosis , Fallopian Tube Neoplasms/genetics , Female , High-Throughput Nucleotide Sequencing , Humans , Mixed Tumor, Mullerian/diagnosis , Mixed Tumor, Mullerian/secondary , Mutation , Ovarian Neoplasms/diagnosis , Ovarian Neoplasms/secondary , Tumor Suppressor Protein p53/geneticsABSTRACT
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.
Subject(s)
Abdominal Neoplasms/surgery , Carcinosarcoma/surgery , Mixed Tumor, Mullerian/surgery , Peritoneal Neoplasms/surgery , Abdominal Neoplasms/drug therapy , Aged , Aged, 80 and over , Antineoplastic Agents, Phytogenic/therapeutic use , Antineoplastic Combined Chemotherapy Protocols , Carboplatin/therapeutic use , Carcinosarcoma/drug therapy , Carcinosarcoma/pathology , Fatal Outcome , Female , Humans , Mixed Tumor, Mullerian/drug therapy , Paclitaxel/therapeutic use , Peritoneal Neoplasms/pathologyABSTRACT
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.
Subject(s)
Adenosarcoma/diagnosis , Cervix Uteri/pathology , Uterine Neoplasms/diagnosis , Adenosarcoma/classification , Adenosarcoma/pathology , Female , Humans , Middle Aged , Mixed Tumor, Mullerian/pathology , Soft Tissue Neoplasms/diagnosis , Soft Tissue Neoplasms/pathology , Tomography, X-Ray Computed , Ultrasonography , Uterine Neoplasms/classification , Uterine Neoplasms/pathologyABSTRACT
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