Frontiers of Ovarian Carcinosarcoma.

OPINION STATEMENT: Ovarian carcinosarcoma (OCS), also known as a malignant mixed Müllerian tumour (MMMT), is a rare and aggressive form of cancer that accounts for less than 5% of ovarian cancers. It is characterized by high morbidity and mortality rates, with a median overall survival (OS) of less than 2 years. Several factors, including advancing age, nulliparity, reduced lactation rates, decreased use of oral contraceptive pills, genetic mutations in BRCA (breast cancer) genes, and the use of assisted reproductive technology, may increase the risk of OCS. Poor prognostic factors include an advanced stage at diagnosis, older age, lymph node metastasis, suboptimal surgical cytoreduction, the presence of heterologous features on histopathology, and increased expression of vascular endothelial growth factor (VEGF), tumour protein p53, and p53 alongside Wilms tumour 1 (WT1). The main treatment approach for OCS is cytoreductive surgery followed by platinum-based chemotherapy, although immunotherapy is showing promise. Homologous recombination deficiency (HRD) testing may enhance outcomes by enabling personalized immunotherapy and targeted therapies for specific patient groups, thereby reducing unnecessary side effects and healthcare costs. However, there is currently a lack of standardised treatment regimens for OCS patients, with most studies consisting of case reports and a shortage of suitable comparator groups. This article aims to provide clinicians with information on the epidemiology, risk factors, prognostic factors, and latest therapeutic advancements in OCS.

Gallbladder carcinosarcoma: current perspectives and new development.

Introduction: Gallbladder carcinosarcoma (GBCS) is a rare neoplasm, and previous studies regarding to GBCS were case reports/case series, absence of large retrospective analyses, or systemic review. This review summarizes the current literature on accurate information of GBCS to assist clinicians to accurately diagnose and treat this malignancy.Areas covered: The authors retrieved relevant documents of GBCS from PubMed and Medline. This review elaborates on the knowledge of GBCS covering epidemiology, potential mechanism, clinical manifestation, diagnosis, treatment, and prognosis.Expert opinion: The majority of GBCS patients are easily misdiagnosed as GBC and usually treated as GBC. However, the biological behavior and outcome of GBCS is different from that of GBC. GBCS should be considered as a separate disease.

Carcinosarcoma Arising from Inverted Papilloma in a Patient with History of Radiotherapy for Sinonasal Squamous Cell Carcinoma.

BACKGROUND Carcinosarcoma of the sinonasal tract is an extremely rare malignant neoplasm; it is often designated as carcinoma with spindle cell or sarcomatoid features. We report a case of carcinosarcoma arising in a pre-existing inverted Schneiderian papilloma in the left maxillary antrum and nasal cavity of a 72-year old male patient. CASE REPORT The patient had a significant history of radiotherapy for squamous cell carcinoma in the sinonasal area, 3 decades ago. The patient presented with chief complaints of left nasal blockage, nasal discharge, anosmia, and occasional epistaxis. Computed tomography scan displayed a lobular soft tissue mass resulting in narrowing of the nasopharyngeal airway with massive destruction of palatal tissue. The lesion was resected via endoscopic surgery. Macroscopically, a white fleshy appearance with necrosis was noted in the submitted specimen. Microscopically, the tumor was composed of pleomorphic epithelial and spindle cells with numerous mitoses and remarkable tissue necrosis. Residual inverted papilloma (IP) with high-grade dysplasia, and minimal foci of moderately differentiated squamous cell carcinoma (SCC) component was present at the tumor margin. A distinct zone of transition of SCC to spindle cell carcinoma (SpSCC) was noted and confirmed by focal positivity of p63 in epithelial and sacromatoid components. The pleomorphic sarcomatoid tumor was positive for vimentin with Ki67 highlighting 70% of tumor cells. A final diagnosis of sinonasal spindle cell carcinoma associated with residual inverted papilloma was rendered. CONCLUSIONS Due to the rarity of such cases, the prognosis and response to treatment is unclear. No effective directed treatment has been developed. Unfortunately, the patient refused any further treatment and died of persistent disease. To the best of our knowledge, only one case of sinonasal carcinosarcoma arising from dysplastic inverted papilloma has been reported. The distinct possibility of previous radiotherapy contributing to development of sarcomatoid features in this neoplasm should also be considered.

Molecular genetic characterization reveals linear tumor evolution in a pulmonary sarcomatoid carcinomas patient with a novel PHF20-NTRK1 fusion: a case report.

BACKGROUND: Pulmonary sarcomatoid carcinoma (SC) consists of both carcinomatous and sarcomatous tumors with high degree of malignancy, rapid progression, and poor prognosis. However, little is known regarding how pulmonary SC develops and progresses. CASE PRESENTATION: A 66-year-old male was initially diagnosed with stage IIIa lung cancer containing both adenocarcinoma (ADC) and SC. Adjuvant chemotherapy was administrated post-surgery, however, recurrence with SC only soon followed. Mutation profiling of the patient's microdissected ADC and SC components of the primary lesion and recurrent tumor was performed by targeted next-generation sequencing (NGS) of 416 cancer-relevant genes. Our data showed that primary SC/ADC and the recurrent SC shared multiple gene mutations including EGFR, NF1, TP53, CDKN2B, and SMARCA4, while both primary and recurrent SCs had a unique TP53 exon 4 splicing mutation frequently observed in sarcoma. Interestingly, a novel PHF20-NTRK1 fusion was acquired in the recurrent SC, which may be a potential driver for SC recurrence. CONCLUSIONS: The molecular genetic characteristics of tumor tissues at different stages reveals a linear tumor evolution model in this case, and support that the primary SC derived from the original lung ADC during the evolution of the tumor. We also identified a novel PHF20-NTRK1 fusion, which may contribute to the disease recurrence, and that can be potentially targeted with NTRK1 inhibitors for treatment.

Radiation-Induced Uterine Carcinosarcoma after Concurrent Chemoradiotherapy for Cervical Squamous Cell Carcinoma

Abstract Objective To describe a case of radiation-induced uterine carcinosarcoma 6 years after a cervical squamous cell carcinoma treatment, which imposed some diagnostic and management challenges. Case Report A 57-year-old woman with a history of pelvic chemoradiotherapy ~ 6.5 years before the event described in this study, following an International Federation of Gynecology and Obstetrics (FIGO) stage IIB cervical cancer, presented with a cervical mass, involving the uterine cavity, the cervical canal and the upper two thirds of the vagina. The biopsy showed a poorly differentiated carcinoma, and a positron emission tomography (PET) scan excluded distant metastasis, although it was unable to define the origin of the tumor as either a new primary malignancy of the endometrium/cervix or as a cervical recurrence. Surgical staging procedure was performed, and the diagnosis was endometrial carcinosarcoma, FIGO stage IIB. The patient was not able to complete the adjuvant therapy, and the progression of the disease was remarkable. Conclusion The present case highlights one of the less common but more serious consequences of radiotherapy for cervical cancer, which has an increasing incidence in younger women, raising concerns about the long-termconsequences of its management.

Radiation-Induced Uterine Carcinosarcoma after Concurrent Chemoradiotherapy for Cervical Squamous Cell Carcinoma.

OBJECTIVE: To describe a case of radiation-induced uterine carcinosarcoma 6 years after a cervical squamous cell carcinoma treatment, which imposed some diagnostic and management challenges. CASE REPORT: A 57-year-old woman with a history of pelvic chemoradiotherapy ∼ 6.5 years before the event described in this study, following an International Federation of Gynecology and Obstetrics (FIGO) stage IIB cervical cancer, presented with a cervical mass, involving the uterine cavity, the cervical canal and the upper two thirds of the vagina. The biopsy showed a poorly differentiated carcinoma, and a positron emission tomography (PET) scan excluded distant metastasis, although it was unable to define the origin of the tumor as either a new primary malignancy of the endometrium/cervix or as a cervical recurrence. Surgical staging procedure was performed, and the diagnosis was endometrial carcinosarcoma, FIGO stage IIB. The patient was not able to complete the adjuvant therapy, and the progression of the disease was remarkable. CONCLUSION: The present case highlights one of the less common but more serious consequences of radiotherapy for cervical cancer, which has an increasing incidence in younger women, raising concerns about the long-term consequences of its management.

Mature Teratoma Associated with Bilateral Ovarian Carcinosarcoma - Accidental Association or Etiopathogenetic Determinism? - Case Report.

Carcinosarcoma is a rare form of ovarian cancer with mixed origin, and its association with mature teratoma is extremely rare. We present the case of patient T. M. aged 67, admitted into our clinic on the 15/05/2016, F.O. 4877 for the increase of the abdominal volume. On admission, the patient was afebrile, conscious, cooperative, cardio-respiratory balanced, having the abdomen distended in volume, sharp dullness in the flanks, positive wave sign bioumoral within normal limits except: uric acid = 6.64 mg / dL, serum glucose = 113.7 mg / dl, serum total proteins = 8.65 g / dl, the albumin / globulin subunit, CRP 33.63 mg / l, sideremia 51 ug / dl, CA 125 = 588.4 IU. Abdominal ultrasound: high volume fluid and multiple perihepatic formations and multiple formations with cystic transformation in the abdomen and pelvis. CT exam describes multiple tissular masses localized intraperitoneal in the abdominal-pelvic region, sheath fluid effusion, infiltrative, with mass effect on the digestive lumens, without visible CT obstruction. Surgical treatment consisted in evacuation of the ascites fluid, excision of the tumoral lumps situated in the great omentum, omentectomy, excision of the lumps of the gastrocolic ligament, bilateral ovariectomy and hysterectomy. Postoperative simple evolution. Histopathology confirmed the diagnosis of bilateral ovarian carcinosarcoma associated with tridermic mature teratoma (presence of brain tissue areas associated with cartilage, transitional type epithelium, tubal type epithelium, endometrial stroma type and fatty tissue). IHC confirms the compatibility with the diagnosis of ovarian carcinosarcoma (mixed malignant Mullerian tumor). The patient followed adjuvant polichemotherapy. The association of teratoma with carcinosarcomatoase elements confers a poor prognosis case.

Carcinosarcoma arising from uterine adenomyosis: A case report.

Carcinosarcoma arising from uterine adenomyosis is extremely rare. We encountered such a patient and herein provide a review of the literature. A 56-year-old woman was referred for a huge pelvic tumor, suspected to be an advanced uterine leiomyosarcoma. Intraoperative inspection revealed a mass, mainly located in the uterine myometrium, invading the uterine serosa. The tumor had previously spontaneously ruptured and disseminated to the pelvic cavity. Pathological and immunohistochemical examination revealed an infiltrative pattern of biphasic tumor cells composed of endometrioid adenocarcinoma and a nonepithelial component with rhabdomyosarcomatous differentiation. Benign endometrial glands with stromal cells were found adjacent to the area of the carcinosarcoma. The endometrium and both ovaries and fallopian tubes were microscopically free of tumor cells. The final diagnosis was heterologous type carcinosarcoma with rhabdomyosarcomatous differentiation, originating from uterine adenomyosis.

Endometriosis and carcinosarcoma--a hypothetical correlation or a proven pathogenetic pathway? Colon carcinosarcoma with origin in endometriotic foci--a case report.

We present the first case of a patient with a synchronic occurrence of three neoplasms: non-small cell lung cancer serous cancer of the ovary and carcinosarcoma of the colon. Moreover, the possible origin of the carcinosarcoma is an endometriotic focus, which is an extremely rare occurrence, especially in women with no history of endometriotic treatment. Immunohistochemical staining of the carcinosarcoma was positive for CD10, estrogen receptors and desmin--typical markers for endometriotic foci. The growth of endometriosis depends on estrogen, which is produced at reduced levels after menopause. However, in some cases endometriosis could be diagnosed de novo in postmenopausal women. On the basis of the reported patient we discuss possible correlations between endometriosis and carcinosarcoma, as well as treatment methods of carcinosarcoma.

Postradiation carcinosarcoma of the corpus uteri--a case report.

INTRODUCTION: Radiation therapy is a very effective treatment modality, commonly used for numerous gynecological malignancies, e.g. cervical cancer. Unfortunately, ionizing radiation is associated with numerous side effects, including secondary cancer formation. A case of carcinosarcoma of the corpus uteri in a woman with a history of pelvic irradiation for cervical carcinoma is reported. The literature has been reviewed to present the incidence, optimal management, and prognosis in cases of postradiation uterine carcinosarcoma. CASE: A 55-year-old woman with a history of pelvic radiotherapy for cervical cancer five years earlier was diagnosed with a pelvic mass. Endovaginal ultrasound examination revealed a solid and cystic tumor, 12.5 cm in diameter. The patient was scheduled for surgery. Gross examination revealed an enlarged, plain corpus uteri, 12 cm in diameter. Both adnexa were normal. Pelvic and abdominal peritoneum were macroscopically normal and normal on palpation. Iliac and obturatory lymph nodes were enlarged on both sides. Radical hysterectomy, omentectomy, and ilio-obturator lymph node dissection were performed. The pathology report revealed carcinosarcoma of the corpus uteri with lymph nodes metastases--FIGO IIIC1. No adjuvant treatment was given. The patient is still alive and disease-free one year after surgery. CONCLUSIONS: Clinicians should.remain conscious of the potential carcinogenic effect of radiation therapy. Uterine carcinosarcoma may occur years after radiotherapy applied for cervical cancer. Therefore, long-term control following pelvic irradiation is always necessary.

Carcinosarcoma of native renal pelvis in recipient after a renal transplant: a case report.

BACKGROUND: Carcinosarcoma is a malignancy that rarely occurs in the renal pelvis. MATERIALS AND METHODS: We present a case of histologically proven, native renal pelvis carcinosarcoma in a 65-year-old woman who had accepted a renal transplant. We performed a laparoscopic ureterectomy, combined with lymph node dissection and immunosuppression with sirolimus (SRL), which was alternated with the conventional immunosuppressant--cyclosporine. RESULTS: This patient was still alive 34 months after renal transplantation. CONCLUSIONS: Operation is still the best choice, and the SRL may be beneficial for preventing the progression of a tumor.

Etiologic heterogeneity in endometrial cancer: evidence from a Gynecologic Oncology Group trial.

OBJECTIVE: Although the epidemiology of typical endometrial carcinomas (grades 1-2 endometrioid or Type I) is well established, less is known regarding higher grade endometrioid or non-endometrioid carcinomas (Type II). Within a large Gynecologic Oncology Group trial (GOG-210), which included central pathology review, we investigated the etiologic heterogeneity of endometrial cancers by comparing risk factors for different histologic categories. METHODS: Based on epidemiologic questionnaire data, risk factor associations, expressed as odds ratios (OR) with 95% confidence intervals (CI), were estimated comparing grade 3 endometrioid and Type II cancers (including histologic subtypes) to grades 1-2 endometrioid cancers. RESULTS: Compared with 2244 grades 1-2 endometrioid cancers, women with Type II cancers (321 serous, 141 carcinosarcomas, 77 clear cell, 42 mixed epithelial with serous or clear cell components) were older; more often non-white, multiparous, current smokers; and less often obese. Risk factors for grade 3 endometrioid carcinomas (n=354) were generally similar to those identified for Type II cancers, although patients with grade 3 endometrioid tumors more often had histories of breast cancer without tamoxifen exposure while those with Type II tumors were more frequently treated with tamoxifen. Patients with serous cancers and carcinosarcomas more frequently had breast cancer histories with tamoxifen treatment compared to patients with other tumors. CONCLUSIONS: Risk factors for aggressive endometrial cancers, including grade 3 endometrioid and non-endometrioid tumors, appear to differ from lower grade endometrioid carcinomas. Our findings support etiologic differences between Type I and II endometrial cancers as well as additional heterogeneity within Type II cancers.

[Uterine Carcinosarcoma induced by a vaginal foreign body: a case report and review]. / Carcinosarcome utérin secondaire à un textilome vaginal: à propos d'un cas et revue de la littérature.

Sarcomas are rare aggressive malignant tumors of the stromal tissue having a poor prognosis. The relationship between carcinogenesis of sarcomas around foreign bodies has been described in medical literature. The chronic inflammation induced by the foreign body produces oxidative and nitrative stress. It also liberates genotoxic mediators and enhances the carcinogenesis. The tumors grow up many years after surgery. We report a case of uterine carcinosarcoma induced by a vaginal foreign body, forgotten nine years before.

Sarcomatoid carcinoma of the remnant stomach: report of a case.

We herein report a case of sarcomatoid carcinoma that developed in a remnant stomach. A 76-year-old male with a history of distal gastrectomy for a duodenal ulcer 28 years earlier underwent investigation for a tumor in the remnant stomach. An endoscopic survey showed a round elevated tumor measuring 6 cm in diameter, and a biopsy specimen suggested carcinosarcoma. A total gastrectomy of the remnant stomach was performed, and the excised tumor was identified to be a malignant neoplasm consisting of both carcinomatous and sarcomatous components. A diagnosis of sarcomatoid carcinoma was made since the epithelial markers were positive even in the mesenchymal elements of the tumor. To our knowledge, only 4 cases of sarcomatoid carcinoma of the stomach have been reported in the English literature so far.

Uterine carcinosarcoma associated with pelvic radiotherapy for sacral chordoma: a case report.

OBJECTIVE: Postirradiation sarcoma of the female genital tract is rare, but a recognized event. Most reported cases have been associated with history of radiotherapy for various gynecologic conditions, particularly cancer of the uterine cervix and abnormal uterine bleeding. The occurrence of uterine sarcoma secondary to radiotherapy for a non-gynecologic tumor and, furthermore, this condition being simultaneous with the recurrence of primary tumor is unique. CASE REPORT: A 67-year-old woman presented with a uterine mass which was diagnosed as a sarcoma by endometrial curettage and history of pelvic radiotherapy 23 years previously for sacral chordoma. Surgical staging procedure for uterine malignancy was performed. The final pathologic diagnosis was carcinosarcoma of the uterus. CONCLUSION: In uterine masses seen in patients with history of irradiation to the pelvic field, the probability of uterine sarcomas should always be kept in mind. These tumors may occur simultaneously with recurrence of primary tumor previously treated by adjuvant radiation therapy.

Carcinosarcoma of the ovary: a review of the literature.

OBJECTIVE: Carcinosarcoma of the ovary is a rare tumor with a grim prognosis. This article critically reviews the literature pertinent to the pathology, pathogenesis, diagnosis, management, and outcome of patients with ovarian carcinosarcoma (OCS). METHODS: MEDLINE was searched for all research articles published in English between January 1, 1981 and August 30, 2011 which reported on patients diagnosed with carcinosarcoma of the ovary. Given the rarity of this tumor, studies were not limited by design or number of reported patients. RESULTS: Patients with OCS generally present with advanced stage disease, and symptoms similar to those of patients with epithelial ovarian cancer (EOC). Retrospective studies have shown that cytoreductive surgery improves outcomes in patients with OCS. Similarly, platinum-based chemotherapy appears to be active in the treatment of OCS. CONCLUSIONS: Ovarian carcinosarcomas are rare and aggressive tumors, associated with a poor prognosis. The mainstay of treatment remains cytoreductive surgical effort for metastatic disease followed by platinum-based chemotherapy. The role of targeted therapies may be promising in the treatment of OCS.