Ovarian Carcinosarcoma Mimicking Symptoms of Recurrent Diverticulitis: A Case Report.

Ovarian carcinosarcoma, also known as malignant mixed müllerian tumor, is a rare and highly aggressive form of ovarian cancer. This report discusses a case where initial misdiagnosis underscored the complexity of diagnosing this condition. The findings highlight the critical nature of considering ovarian malignancies in the differential diagnosis for postmenopausal women presenting with abdominal pain and altered bowel habits. The significance of utilizing advanced imaging techniques and tumor markers in the early detection of ovarian carcinosarcoma is emphasized, demonstrating how such strategies can substantially affect patient management and outcomes. This case also illustrates the effectiveness of a multidisciplinary approach in treating this challenging malignancy, contributing to our understanding and management of ovarian carcinosarcoma.

Clinical Profile and Treatment Outcomes of Malignant Mixed Mullerian Tumors of the Uterus: A Single-Center Experience.

Background A malignant mixed Mullerian tumor (MMMT), otherwise known as carcinosarcoma, is a rare and aggressive malignancy involving the uterus. Despite aggressive treatment, they have poor outcomes. We performed this study to analyze the clinical profile, prognostic features, and treatment outcomes of patients treated for MMMT of the uterine corpus in our center. Methodology A study sample from a database of patients treated at a tertiary care center in South India between January 2015 and December 2019 was analyzed retrospectively. A total of 29 non-metastatic patients were included in the study. The diagnosis of MMMT was confirmed by a pre-operative biopsy specimen or endometrial curettage. Information regarding the patient, tumor characteristics, details of the surgery, adjuvant therapy, and follow-up details were collected retrospectively. Disease-free survival (DFS) and overall survival (OS) were plotted using the Kaplan-Meier method, and the log-rank test was used to identify any significant prognostic factor. Statistical analyses were performed using IBM SPSS software, version 21 (IBM Corp., Armonk, NY). Results The population's median age was 60 years (interquartile range (IQR): 52-65). The homologous type was the most common pathology seen in 21 patients. The median size of the tumor was 7 centimeters (cm) (IQR: 6-10). Around two-thirds of the patients received adjuvant radiation therapy. Paclitaxel and carboplatin-based adjuvant chemotherapy were used in 60% of the patients. Nearly 90% of the recurrences were distant recurrences. During the follow-up, 17 patients developed a recurrence. The median DFS was 12 months (95% CI: 7.7-16.2). The median OS was 26 months (95% CI: 10.6-41.3). The three-year OS rate was 42%. Patients with an age >60 years had a median overall survival of 11 months, compared to 37 months for patients <60 years (p = 0.026). The median overall survival of patients with tumor sizes >10 cm was 12.5 months, and it was 35 months when the tumor size was less than 10 cm (p = 0.03). Patients receiving radiotherapy (RT) had an improved survival (39 months) compared to those who did not receive RT (12.5 months) (p = 0.046). The median overall survival for patients after the recurrence was 12 months. As most patients were elderly with an Eastern Cooperative Oncology Group (ECOG) performance status of two at baseline, only a few patients were fit for second-line chemotherapy. Conclusion A malignant mixed Mullerian tumor is an aggressive tumor of the uterine corpus with poor survival rates. Surgical resection remains the mainstay of treatment. Adjuvant radiation therapy improves survival. The age of the patient, myometrium invasion, tumor size, and positive peritoneal cytology were found to be prognostic factors.

Racial and Ethnic Disparities in Gynecologic Carcinosarcoma: A Single-Institution Experience.

We aimed to determine the incidence, treatment regimen, and treatment outcomes (including progression-free survival and overall survival) of gynecologic carcinosarcoma, a rare, aggressive, and understudied gynecologic malignancy. This retrospective review included all patients with gynecologic cancers diagnosed and treated at a single tertiary care comprehensive cancer center between January 2012 and May 2021. A total of 2116 patients were eligible for review, of which 84 cases were identified as carcinosarcoma: 66 were uterine (5.2% of uterine cancers), 17 were ovarian (3.6% of ovarian cancers), 1 was cervical (0.28% of cervical cancers), and 1 was untyped. Of the patients, 76.2% presented advanced-stage disease (stage III/IV) at the time of diagnosis. Minority patients were more likely to present with stage III/IV (p < 0.0001). The majority of patients underwent surgical resection followed by systemic chemotherapy with carboplatin and paclitaxel. The median PFS was 7.5 months. Of the patients, 55% were alive 1 year after diagnosis, and 45% were alive at 5 years. In the studied population, minorities were more likely to present with more advanced disease. The rate of gynecologic carcinosarcomas was consistent with historical reports.

Clinico-Pathological Characteristics, Management, and Prognostic Factors of Patients with Uterine Carcinosarcoma: a Retrospective Analysis.

Uterine carcinosarcoma is a rare, highly aggressive, rapidly progressing neoplasm associated with a poor prognosis. It comprises 1-5% of all uterine malignancies but accounts for 16.4% of all deaths caused by uterine malignancies. There is a definite paucity of data available from the Indian subcontinent. Hence, we retrospectively conducted this study to analyze the clinical and pathological characteristics and outcomes of women with uterine carcinosarcoma in the past 10 years managed at the tertiary care center. This is a retrospective study of women with histologically proven uterine carcinosarcoma treated at a tertiary cancer center in South India between August 2009 and April 2019. Inpatient and outpatient records were reviewed; clinicopathological data were collected; and follow-up and survival data were ascertained. Over a period of 10 years, 20 patients were diagnosed with uterine carcinosarcoma. The majority of patients were postmenopausal (80%). Post-menopausal bleeding was the main presenting complaint in about 80% of patients. More than two-thirds of patients presented in the early stage (stage I, 55%; stage II, 20%). All patients underwent staging laparotomy. Patients with good performance status (85%) received adjuvant concurrent chemoradiotherapy and chemotherapy. At a median follow-up of 40 months, 7 (35%) patients were alive, out of which 6 are disease-free and 1 had a recurrence. The event-free survival at a median follow-up of 40 months was 40% and the overall survival was 48.5%. The outcome did not significantly differ based on the age, tumor histology (heterologous versus homologous), stage, and depth of myometrial invasion. Uterine carcinosarcoma, though rare, needs to be recognized as a distinct entity, and treated aggressively. Surgery is the cornerstone of therapy. Adjuvant concurrent chemoradiation and chemotherapy improve local control and may delay recurrence, but have shown little survival advantage. The optimal adjuvant treatment for this uncommon disease is yet to be established, highlighting the need for larger multicentric studies on this tumor.

Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget's Disease Case Report.

The occurrence of more than one primary malignant tumor in a single patient is rare. Multiple primary malignancies can pose difficulties in differential diagnosis between primary tumors and metastasis. Here, we present a case report with multiple primary malignancies. The patient is a 45-year-old female who was diagnosed with cervical mixed squamous neuroendocrine adenocarcinoma, metastasized carcinosarcoma and extramammary vulvar Paget's disease. The patient was first diagnosed with a microinvasive squamous cervical carcinoma in situ. After a few months, the amputation of a small residual tumor and histological evaluation revealed an IA1-stage poorly differentiated (G3) mixed squamous and neuroendocrine cervical adenocarcinoma. After two years, the disease had progressed and biopsies from altered sites were taken. Histological diagnosis from an ulcerated vulvar region revealed extramammary vulvar Paget's disease. A biopsy from vagina polyp revealed an earlier diagnosed mixed squamous and neuroendocrine cervical adenocarcinoma. However, histological diagnosis from an inguinal lymph node biopsy was unexpected and revealed carcinosarcoma. It indicated either the development of another primary malignancy, or an unusual spread of metastasis. Clinical presentation as well as diagnostic and treatment challenges are discussed in this case report. This case report shows that multiple primary malignancy cases are difficult to manage both for clinicians and the patient because the therapeutic options can become limited. This complex case was managed by a multidisciplinary team.

Metastatic ovarian carcinosarcoma in a patient undergoing in-vitro fertilization: A case report.

INTRODUCTION AND IMPORTANCE: Ovarian carcinosarcomas (OCS) are highly aggressive tumors containing both carcinomatous and sarcomatous elements. Patients are typically older postmenopausal women who present with advanced disease, however rarely young women can be affected. CASE PRESENTATION: A 41-year-old woman undergoing fertility treatment was found to have a new 9-10 cm pelvic mass on routine transvaginal ultrasound (TVUS) 16 days after embryo transfer. Diagnostic laparoscopy revealed a mass in the posterior cul-de-sac that was surgically excised and sent to pathology for evaluation. Pathology was consistent with carcinosarcoma of gynecologic origin. Further work-up revealed advanced disease with apparent rapid progression. Patient underwent interval debulking surgery after four cycles of neoadjuvant chemotherapy with carboplatin and paclitaxel with final pathology consistent with primary ovarian carcinosarcoma and complete gross resection of disease. CLINICAL DISCUSSION: In the setting of advanced disease neoadjuvant chemotherapy with a platinum-based chemotherapy regimen followed by cytoreductive surgery is a standard approach to treatment of OCS. Given the rarity of disease, most data regarding treatment has been extrapolated from other forms of epithelial ovarian cancer. Specific risk factors for disease development of OCS including the long-term effects of assisted reproductive technology remain understudied. CONCLUSION: While OCS are rare highly aggressive biphasic tumors that primarily affect older postmenopausal woman, we present a unique case of OCS incidentally found in a young woman undergoing fertility treatment via in-vitro fertilization.

Malignant Mixed Mullerian Tumor Arising from Endometriosis in the Groin: A Case Report and Literature Review.

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.

Cutaneous metastasis of carcinomatous component of ovarian carcinosarcoma: A case report and review of the literature.

Skin metastasis of ovarian cancer is extremely rare. We report an unusual case of ovarian carcinosarcoma with cutaneous metastasis of carcinomatous component that displayed distinct clinical manifestation. A 48-year-old woman presented to the dermatologist complaining of a new onset of erythematous, plaque-like skin rash with multiple small nodules on the left inner thigh, the area measuring 8 × 5cm. While the patient had no history of dermatologic conditions, she underwent a total hysterectomy and bilateral salpingo-oophorectomy, omentectomy, and lymph node dissection 16 months ago with a pathology confirmed stage IIIC ovarian carcinosarcoma. Of note, the carcinomatous component, mainly adenocarcinoma with hybrid features of seromucinous, endometrioid and minor high-grade serous carcinoma, involved bilateral fallopian tubes, omentum, and parametrium with extensive lymph node metastases. A skin biopsy specimen revealed an adenocarcinoma involving epidermis, dermis, and subcutaneous tissue with nodular contours, consistent with metastatic carcinomatous component of carcinosarcoma. Both carcinomatous component of primary ovarian carcinosarcoma and metastatic adenocarcinoma in the skin demonstrated Pax8, WT-1, and ER positivity and a mutation pattern of p53. The patient passed away 15 months after identification of skin metastasis. This case represents a unique example of cutaneous metastasis of ovarian carcinosarcoma with distinct clinical manifestation and detailed histopathological description. Alertness to the possibility of cutaneous metastasis, in combination with clinical history, morphological and immunohistochemical findings, is critical for a definitive classification.

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.

Carcinosarcoma of Uterus: a Case Report.

Carcinosarcoma is a malignant mixed Müllerian tumor with a highly malignant, biphasic tumor consisting of both epithelial and mesenchymal components. A 59-year-old nulligravida came with postmenopausal bleeding. Hysteroscopy revealed highly vascular polypoidal mass with prominent vasculature. Gross examination of specimen showed a polypoid mass, occupying whole uterine cavity and invading more than half of myometrium. Immunohistochemical analysis showed epithelial component AE1/AE3 and stromal component desi and p16. Tumor cells were negative for ER. As carcinosarcoma is a highly aggressive less common variant of endometrial cancer, early diagnosis and aggressive treatment are important to minimize morbidity and overall survival.

Carcinosarcoma arising from high-grade serous carcinoma of the ovary without estrogen receptor, WT-1, and PAX8 immunoreactivity.

OBJECTIVE: We encountered a case of high-grade serous carcinoma (HGSC) of the ovary which recurred as carcinosarcoma of the sigmoid colon. Tumor cells of both the primary carcinoma and the secondary carcinosarcoma were negative for estrogen receptor (ER), WT-1, and PAX8. It is well known that most ovarian carcinomas arising from the Müllerian duct are immunoreactive for these biologic parameters. To our knowledge, this is the first case report that provides the results of immunohistochemical analysis of WT-1 and PAX8 for a primary carcinoma and recurrent carcinosarcoma. CASE REPORT: A 61-year-old woman had an advanced right ovarian HGSC. After a primary debulking surgery (hysterectomy, bilateral salpingo-oophorectomy and omentectomy) and adjuvant chemotherapy, complete remission was achieved. However, four and a half years later, a tumor arising beside the sigmoid colon was detected. A tumorectomy was performed through combined partial resection of the ileum and sigmoid colon. Microscopically, the tumor was diagnosed as carcinosarcoma of the sigmoid colon, which had originated from HGSC of the ovary. Interestingly, the malignant cells of the primary carcinoma and epithelial components of the recurrent carcinosarcoma were negative for ER, WT-1, and PAX8. These immunohistochemical features were unusual. Three cycles of chemotherapy with the previously used regimen and three additional cycles of doxorubicin and ifosfamide combination chemotherapy were administered. Currently, 3 years after the final chemotherapy was administered, the patient remains healthy. CONCLUSION: HGSC of the ovary can recur as carcinosarcoma. Tumor cells of the primary HGSC without ER, WT-1, and PAX8 expression may have dedifferentiated and recurred as carcinosarcoma.

Case Report: Stage IIIc primary malignant mixed Müllerian tumor of the fallopian tube: A case of 5-year disease-free survival after cytoreductive surgery combined with peritoneal resection and adjuvant chemotherapy with paclitaxel plus carboplatin.

Malignant mixed Müllerian tumor (MMMT) of the fallopian tube is rare and has a poor prognosis. For the patient with fallopian tube MMMT, complete resection of the tumor invading the viscera and the peritoneum is a prerequisite for long-term survival. We report a case of stage IIIc MMMT of the fallopian tube treated by cytoreductive surgery (CRS), peritoneal resection, and adjuvant chemotherapy (paclitaxel plus carboplatin), with 5-year tumor-free survival. Postoperative chemotherapy combining platinum and paclitaxel is the most potent adjuvant therapy.

Paratubal mass carcinosarcoma: A case report of a rare malignancy in a rare location / Philippine Journal of Obstetrics and Gynecology

@#Carcinosarcoma, formerly known as malignant mixed Mullerian tumors (MMMTs) are highly aggressive tumors that include both malignant epithelial and mesenchymal or stromal elements. The most common site of carcinosarcomas in a female reproductive organ is the endometrium with an incidence of 2/100,000 females, whereas carcinosarcomas arising from the paratubal mass are extremely rare malignancies accounting for fewer than 0.1% of MMMTs. Carcinosarcomas of the Fallopian tube usually occur in the fifth to sixth decades in postmenopausal women with the most common presenting symptom of abdominal pain, followed by vaginal bleeding and abdominal distention. There have been limited published cases worldwide, that is, it has been a thing of interest to be analyzed in today’s era. A rare case of paratubal carcinosarcoma is highlighted in this paper as it discourses its clinicopathological characteristics and assesses the prognostic factors associated with treatment outcome and survival.

Uterine carcinosarcoma: An overview.

Uterine carcinosarcoma (UCS), also known as malignant mixed Müllerian tumor, is a rare gynecological malignancy characterized by poor prognosis. This "biphasic" neoplasm presents an admixture of epithelial and mesenchymal/sarcomatoid tumor cells which partially share their molecular signature and exhibit a typical epithelial-to-mesenchymal transition gene expression profile. Due to the rarity of this cancer, at present there is a scarcity of specific treatment guidelines. Surgical resection remains the best curative option for localized disease, whereas the addition of peri-operative radiotherapy, chemotherapy and chemoradiation has been shown to further improve disease outcomes. In the metastatic setting, palliative chemotherapy is currently the treatment of choice, although no consensus exists about the best regimen to be delivered. Besides standard treatment options for the advanced disease, mechanistic insights into UCS pathogenesis and identification of its histopathological and molecular features boosted the development of novel, and potentially more effective, therapeutic agents, that will be here discussed.

Intradural extramedullary spinal metastases from uterine carcinosarcoma: A case report.

BACKGROUND: In recent years, improvements in oncological care have led to an increased incidence of intradural extramedullary spinal metastases (IESMs) attributed to uterine carcinosarcoma (UCS). When such lesions occur, they typically carry a poor prognosis. Here, we have evaluated newer treatments, management strategies, and outcomes for IESM due to UCS. CASE DESCRIPTION: A 59-year-old female with a history of recurrent UCS presented with the new onset of the left lower extremity pain, numbness, and episodic urinary incontinence. When the MR revealed an enhancing intradural extramedullary mass posterior to the L1 vertebral body, she underwent a focal decompressive laminectomy. Although she improved neurologically postoperatively, she succumbed to the leptomeningeal spread of her disease within 2 postoperative months. CONCLUSION: Management of IESM due to UCS requires multifaceted, individualized treatment modalities, including neurosurgery, radiation therapy, and medical oncologic management to maximize outcomes.

An immunohistochemical analysis of CD3, PD-L1, and CTLA-4 expression in carcinosarcomas of the gynecological tract and their metastases.

BACKGROUND: Carcinosarcoma of the gynecological tract is a rare tumor with a dismal prognosis. Its immune micro-environment has not been sufficiently studied. AIM OF THE STUDY: To study the immune micro-environment of gynecological carcinosarcomas. MATERIAL AND METHODS: Sixty-nine surgical specimens from 37 different patients, including 34 primary tumors and 35 metastases, were immunohistochemically studied for the expression of CD3, PD-L1, and CTLA-4. Clinical and histological features were recorded and correlated with immunohistochemical factors. RESULTS: Twenty-two cases involved the uterine corpus, 1 the uterine cervix, and 14 the adnexa. The overall survival ranged from 2 to 156 months, with a median survival of 16 months. CD3 expression was more frequent at the sarcomatous than the carcinomatous component. CTLA-4 expression was higher at the carcinomatous than the sarcomatous component. PD-L1 was negative in all cases studied. Tumor relapse, metastasis presence, metastasis localization, and overall survival did not correlate with CD3 or CTLA-4 expression. CONCLUSION: PD-L1 expression is not a feature of gynecological carcinosarcomas, despite a relatively frequent lymphocytic reaction. CTLA-4 expression is sometimes found in these tumors.

A report of two cases of dedifferentiated endometrioid carcinoma: A newly described underrecognized tumor of poor prognosis.

Dedifferentiated endometrioid carcinoma or dedifferentiated endometrioid adenocarcinoma (DEAC) is defined by the presence of undifferentiated carcinoma with endometrioid carcinoma. Undifferentiated component can be misinterpreted as solid component of high-grade endometrioid carcinoma or sarcomatous component of malignant mixed mullerian tumor. We present two cases of DEAC. Two postmenopausal women underwent hysterectomy for vaginal bleeding. Microscopically, sections from the endometrial tumors showed a biphasic growth consisting of an undifferentiated component and a glandular component with sharp transition between the two components. The undifferentiated component showed focal positivity for cytokeratin and vimentin, while glandular component was diffusely positive for cytokeratin and negative for vimentin expression.

Management and clinical outcomes in uterine malignant mixed Müllerian tumor: Lessons from a single-institution series.

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.

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.

Uterine inversion associated with malignancy – A challenge in surgical management: A case report / Philippine Journal of Obstetrics and Gynecology

@#Uterine inversion is a rare clinical problem. Most cases of uterine inversions are puerperal inversions wherein it is encountered as an obstetric emergency, and sometimes a diagnostic challenge in gynecology. Uterine inversions associated with malignancies such as endometrial carcinoma and sarcoma are even rare. We report 2 cases of this rare condition. A 55 year old diagnosed with endometrial carcinoma and a 60-year-old woman diagnosed with sarcoma (malignant mixed mullerian tumor) presented with mass protruding from the vaginal introitus. The diagnosis of complete uterine inversion was confirmed in both cases during laparotomy. Total abdominal and vaginal hysterectomy and bilateral salpingo-oophorectomy, bilateral pelvic lymph node dissection, paraaortic lymph node sampling was done. It required a challenging surgical procedure to remove the tumor along with the review of literature especially of its association with malignancies.