A CARE-compliant article: A case report of retroperitoneal endometrial stromal sarcoma with multiple pulmonary metastases and literature review.

RATIONALE: Endometrial stromal sarcoma is an extremely rare mesenchymal neoplasm occurring in the extrauterine. Retroperitoneal endometrial stromal sarcoma with multiple pulmonary metastases, in particular, is extremely rare. PATIENT CONCERNS: Forty-seven-year-old woman (gravida 3, para 2), was referred to our institution with complaints of fever. DIAGNOSES: Ultrasound and computed tomographic imaging of the abdomen identified the presence of masses in the pelvic region. Additionally, computed tomographic scans and X-ray evaluations of the thorax detected dispersed masses in both the lungs and pelvic area. Histopathological analysis of the pelvic region indicated the presence of low-grade endometrial stromal sarcoma. A computed tomography-guided pneumocentesis was conducted to further characterize the pulmonary lesions, confirming the diagnosis of low-grade endometrial stromal sarcoma. INTERVENTIONS: The patient underwent tumor resection, subsequent treatment with Medroxyprogesterone acetate for 6 months, received microwave ablation for multiple lung metastases, PARP1 inhibitor for 4 courses, and has been undergoing chemotherapy (epirubicin/ifosfamide) up to the present time. OUTCOMES: Partial remission was obtained after the above treatment and this patient is now still alive maintaining for 3 years. LESSONS: The identification and management of this disease remain a significant challenge due to its low prevalence. Further research involving a larger number of cases is necessary to ensure consistency in diagnosis and to establish effective treatment guidelines.

Pancreatic resections for metastases: A twenty-year experience from a tertiary care center.

BACKGROUND: Literature data about pancreatic resections for metastases are limited to small series, so that the role of surgery in this setting remains unclear. We herein report our experience from a tertiary care center, analyzing the outcomes of patients who underwent pancreatic resections for metastases and discussing the role of surgical resection in their management. MATERIALS AND METHODS: From January 1999 to January 2019, 26 patients underwent pancreatic resections for metastases from renal cell carcinoma (RCC-group) or other primitive tumors (non-RCC-group). Details regarding pre-, intra-, post-operative course, and follow-up, prospectively collected in a database of pancreatic resection, were retrospectively analyzed and compared. RESULTS: RCC-group was composed of 21 patients, non-RCC-group of 5 patients. RCC-group presented a longer disease-free interval: 96.4 vs. 5.4 months (p < 0.001). In 9/21 patients (42.9%) of RCC-group the surgical resection of other organs or vascular structures was performed, while in non-RCC-group pancreatic resection alone was performed in all cases, p = 0.070. No local recurrence was reported in all cases. The systemic recurrence rate was 42.9% (9/21 patients) in RCC-group and 80% (4/5 patients) in non-RCC-group, p = 0.135. RCC-group presented a longer DFS and OS: 107.5 vs. 25.2 months (p = 0.002), and 109.1 vs. 36.2 months (p = 0.016), respectively. CONCLUSIONS: Radical pancreatic resection may confer a survival benefit for RCC metastases, while for other primitive tumors it should be applied more selectively. For RCC pancreatic metastases, an aggressive surgical approach, even in patient with locally advanced tumors, or associated extra-pancreatic localizations, or recurrent metastases should be taken in consideration.

Metastatic low-grade endometrial stromal sarcoma of uterus presenting as a primary pancreatic tumor: case presentation and literature review.

BACKGROUND: Metastatic tumors to the pancreas are uncommon, accounting for approximately 2% of pancreatic malignancies. The most common primary tumors to give rise to pancreatic metastases are carcinomas. CASE PRESENTATION: A 50-year old female patient was investigated for a cause of abdominal discomfort. She had a 2-year history of menorrhagia and dysmenorrhea which was ascribed to a fibroid uterus. On imaging, she was found to have a large solid and cystic mass in the tail of the pancreas. Imaging also confirmed a fibroid uterus. A distal pancreatectomy and splenectomy showed a 9 cm circumscribed mass within, and grossly confined to, the parenchyma of the pancreatic tail. Microscopically, the pancreatic lesion was lobulated, and well-circumscribed, but focally infiltrative. It comprised sheets of uniform spindled to epithelioid cells with round to oval nuclei, coarse to vesicular chromatin, visible nucleoli, nuclear grooves and clear to eosinophilic cytoplasm. Prominent arterioles were identified. The stroma was collagenized in areas. Occasional hemosiderin-laden macrophages were seen, and focal cystic change was present. There was no evidence of nuclear pleomorphism, mitotic activity or necrosis, and there was no evidence of endometriosis despite multiple sections being taken. Immunohistochemistry showed that the tumor cells were positive for CD10, estrogen receptor (ER), progesterone receptor (PR), Wilms tumor-1 (WT-1) and smooth muscle actin (SMA). RNA sequencing detected a PHF1 rearrangement. The morphological, immunohistochemical and molecular features were of a low-grade endometrial stromal sarcoma (LG-ESS). Subsequent total hysterectomy and bilateral salpingo-oophorectomy 3 months later, showed uterine fibroids and a 5 cm low-grade endometrial stromal sarcoma confined to the uterus, with lymphatic invasion. CONCLUSIONS: To the best of our knowledge, this is the first documented case of metastatic endometrial stromal sarcoma of uterus presenting as a primary pancreatic neoplasm. An unexpected extra-uterine location and unusual presentation of ESS may make the diagnosis challenging, despite classic histological features. Morphological, immunohistochemical and molecular findings must be combined to render the correct diagnosis.

Treatment of uterine high-grade endometrial stromal sarcoma with apatinib combined with chemotherapy: A case report.

RATIONALE: The standard treatment for uterine high-grade endometrial stromal sarcoma (HGESS) is chemotherapy after surgery. However, the traditional combination chemotherapy has certain limitation, for example, the cancer cells will quickly become resistant to the chemotherapy drugs. Apatinib is a small-molecule antiangiogenic agent which has shown promising therapeutic effect against diverse tumor, but it still remains unknown whether apatinib has an antitumor effect in patients with endometrial stromal sarcoma (ESS). Here, we report a case of pulmonary metastasis from uterine HGESS successfully treated with apatinib combined with chemotherapy. We also review relevant literature discussing treatment of ESS. PATIENTS CONCERNS: A 54-years-old Chinese woman complained of intermittent pain in the waist and abdomen for 4 months. The patient was diagnosed as uterine fibroids before operation. The surgeon performed a total hysterectomy with bilateral salpingo-oophorectomy, resection of peritoneal disseminated lesions, and the pathological examination revealed a HGESS. DIAGNOSIS: Uterine HGESS stage IV with lung metastases. INTERVENTIONS: The patient underwent surgery, chemotherapy, chemotherapy combined with apatinib, apatinib maintenance therapy, and radioactive particle implantation for lung metastasis. OUTCOMES: The patient experienced the above interventions and achieved good results. And continue oral apatinib (500 mg daily) as maintenance therapy. It has been 16 months since the initial diagnosis, and the patient is still in follow-up. LESSONS: Apatinib combined with chemotherapy and apatinib monotherapy as maintenance therapy could be a new therapeutic strategy for ESS.

Extrapelvic Metastases in Endometrial Stromal Sarcomas: A Clinicopathological Review With Immunohistochemical and Molecular Characterization.

Endometrial stromal sarcoma is a rare uterine tumor associated with favorable outcomes despite its ability to recur and metastasize to distant sites. Most recurrences are local, being limited to the abdomen/pelvis, but distant metastases can occur. Metastatic endometrial stromal sarcoma can occur many months to years after the original diagnosis or may present prior to the primary, potentially creating a diagnostic challenge. We report a bi-institutional review of 10 cases of endometrial stromal sarcoma with extrapelvic metastases without a prior history of endometriosis. The histologic, immunophenotypic, and molecular characteristics of these tumors are analyzed in the context of a relevant literature review.

Removal of an endometrioid stromal sarcoma from the inferior vena cava and right atrium.

Entometrioid stromal sarcomas are seen in extra-uterine as well as extra-gonadal sites and have a strong association with endometriosis. Although having better prognosis than other sarcomas, yet these tumors may relapse (whether local or distant) in up to 56% of cases, even as late as 20 years after surgery. We report a case of a 30-year-old female patient with a mass in the inferior vena cava and right atrium which was surgically removed using cardiopulmonary bypass and deep hypothermic circulatory arrest and turned to be an entometrioid stromal sarcoma. The patient gave a history of endometriosis followed by the appearance of a low-grade ovarian endometrioid stromal sarcoma 4 years before the development of the mass in the IVC and right atrium.

Hepatic endometrial stromal sarcoma.

Endometrial stromal sarcomas are rare tumors that may recur or metastasize many years after their initial presentation. Though most recurrences are within the pelvis, distant metastases can occur, and are most common to the lungs. Metastases to the liver are extremely rare. Herein we report two cases of endometrial stromal sarcoma with metastases to the liver without a prior history of endometriosis, accompanied by their histology, immunohistochemistry, and molecular analysis in the context of a relevant literature review.

Cardiac metastatic endometrial stromal sarcoma 17 years after hysterectomy.

We report a 60-year-old female who underwent resection of an endometrial stromal sarcoma of the right ventricle 17 years following a hysterectomy and radiation therapy for the same tumor.

[Pneumothorax Caused by Multiple Pulmonary Metastases of a Uterine Endometrial Stromal Sarcoma;Report of a Case].

A 53-year-old woman who had undergone hystero-oophorectomy for uterine endometrial stromal sarcoma in our hospital 9 months previously was referred to our hospital because of bilateral pneumothorax. Chest computed tomography scan on admission revealed multiple thin-walled cavity nodules in both lung and a bilateral pneumothorax, suggesting pulmonary metastases of the uterine endometrial stromal sarcoma. We surgically treated the pneumothorax and diagnosed the nodules as metastatic lesions. They were pathologically diagnosed as metastatic uterine endometrial stromal sarcoma.

Primary extragenital endometrial stromal sarcoma of the lung: first reported case and review of literature.

BACKGROUND: Endometrial stromal sarcomas arising in extrauterine and extraovarian sites, in the absence of a primary uterine lesion are quite rare, especially in the absence of endometriosis. They usually present as an abdominal or pelvic mass lesion. CASE PRESENTATION: In 2007, a 45-year-old woman underwent total hysterectomy for in situ squamous cell carcinoma of the cervix. In 2014, an upper left pulmonary lobectomy was performed for a mass, which was provisionally diagnosed as primary carcinosarcoma of the lung. A second histological revision of the lung surgical specimen was performed in the Pathology Unit of our Institute. After extensive immunohistochemical analyses, the preferred diagnosis was spindle-cell sarcoma, consistent with high-grade extragenital endometrial stromal sarcoma (EESS). A review of all slides of the hysterectomy specimen confirms the original diagnosis: no evidence of stromal tumor was found. Afterwards, the patient developed multiple and metachronous pulmonary lesions and a scapular soft tissue mass, which showed the same morphophenotypic features of the first lung mass. The patient was treated with antiblastic therapy, surgical resection and radioablation, when appropriate. To date, the patient has no signs or symptoms. CONCLUSIONS: The authors present the first case of primary EESS arising in the lung with no association with endometriosis published to date. Detailed clinical history and follow-up are also described. Moreover, extensive literature review is reported, along with differential diagnoses, immunohistochemical and molecular findings, pathogenetic hypotheses and treatment options. The knowledge of EESS potential extrauterine location and of its peculiar morphophenotypic aspects are required for a correct diagnosis, and for choosing the most suitable treatment.

Uterine leiomyosarcoma and endometrial stromal sarcoma have unique miRNA signatures.

OBJECTIVE: To compare the microRNA (miRNA) profiles of uterine endometrial stromal sarcoma (ESS) and leiomyosarcoma (LMS), and to compare the miRNA signatures of primary and metastatic uterine LMS. METHODS: Eight primary LMS, 9 primary ESS and 8 metastatic LMS were analyzed for miRNA profiles using TaqMan Human miRNA Array Cards. Findings for 20 differentially expressed miRNAs were validated in a series of 44 uterine sarcomas (9 primary uterine ESS, 17 primary uterine LMS, 18 metastatic LMS) using qPCR. Frizzled-6 protein expression was analyzed in 30 LMS (15 primary, 15 metastases). Frizzled-6 was silenced in SK-LMS-1 uterine LMS cells using siRNA and the effect on invasion, wound healing and matrix metalloproteinase-2 (MMP2) activity was assessed. RESULTS: Ninety-four miRNAs were significantly differentially expressed in ESS and LMS, of which 76 were overexpressed in ESS and 18 overexpressed in LMS. Forty-nine miRNAs were differentially expressed in primary and metastatic LMS, of which 45 were overexpressed in primary LMS and 4 in metastases. Differential expression was confirmed for 10/20 miRNA analyzed using qPCR. Frizzled-6 silencing in SK-LMS-1 cells significantly inhibited cellular invasion, wound healing and MMP-2 activity. CONCLUSIONS: Differential miRNA signatures of ESS and LMS provide novel data regarding transcriptional regulation in these cancers, based on which new potential diagnostic markers, prognostic biomarkers and therapeutic targets may be explored. Differences in miRNA profiles of primary and metastatic LMS may improve our understanding of disease progression in this aggressive malignancy.

Endometrial Stromal Sarcoma Metastatic from the Uterus to the Inferior Vena Cava and Right Atrium.

Endometrial stromal sarcoma metastases usually occur within the pelvis and rarely involve the great vessels or the heart. We present the case of a 55-year-old woman who was referred for endovascular therapy to treat presumed thrombosis of the inferior vena cava. The suspected thrombus was recalcitrant to endovascular removal with use of an AngioVac venous drainage device. Results of an intraprocedural transvenous biopsy revealed the mass to be the intravascular extension of an endometrial stromal sarcoma. The patient underwent surgical excision of the tumor, and, shortly thereafter, a hysterectomy and salpingo-oophorectomy. This complex case highlights both the rarity of malignancy masquerading as caval thrombus and the importance of multispecialty collaboration.

Two-stage resection of a disseminated mixed endometrial stromal sarcoma and smooth muscle tumor with intravascular and intracardiac extension.

OBJECTIVE: Mixed endometrial stromal and smooth muscle tumor (MESSMT)-a rare mesenchymal uterine tumor of the uterus with atypical clinical symptoms-is susceptible to misdiagnosis and missed diagnosis. We report a case of a disseminated MESSMT with intravenous and intracardiac extensions treated with staging surgery and review previously documented cases of such tumors with intracardiac extension. CASE REPORT: The case involves a 45-year-old woman with disseminated MESSMT that originated in the uterus and progressed through the iliac vein, inferior vena cava, right atrium, and into the right ventricle, which closely resembled intravenous leiomyomatosis (IVL) grossly and microscopically. She presented with a 1-year history of dyspnea on exertion. IVL was highly suspected preoperatively based on computed tomography and magnetic resonance imaging findings. Two-stage surgeries were performed successfully. The postoperative pathology indicated a disseminated MESSMT. CONCLUSION: This case illustrates the important role of pathology and immunohistochemistry in the differential diagnosis of a rare tumor that mimics the characteristics of IVL with intracardiac involvement and demonstrates the therapeutic strategy for this rare entity.

Cystic, nodular and cavitary metastases to the lungs in a patient with endometrial stromal sarcoma of the uterus.

A 57-year-old woman, who had undergone hysterectomy for uterine myoma 11 years earlier presented with cystic, nodular and cavitary lesions simultaneously visible on computed tomography images of the chest. Histological examinations of both the resected lung and past "myoma" specimens demonstrated that the original uterine tumor was a low-grade endometrial stromal sarcoma (ESS) that had metastasized to the lungs. No previous reports have described the coexistence of cystic, nodular and cavitary lesions with pulmonary metastasis of ESS; however, we successfully correlated the radiologic appearance with the corresponding pathologic findings. Medroxyprogesterone acetate therapy has effectively kept the patient asymptomatic for approximately five years.

Endometrial stromal sarcomas: immunoprofile with emphasis on HMB45 reactivity.

OBJECTIVES: We describe the morphologic and immunohistochemical features of 17 endometrial stromal neoplasms, 16 sarcomas, and one stromal nodule. METHODS: We reviewed 35 cases interpreted as endometrial stromal neoplasms, but 17 high-grade endometrial stromal sarcomas (ESS) and one case of mixed endometrial sarcoma and leiomyosarcoma were excluded from the study. Data from the Surveillance Epidemiology and End Results program on low- and high-grade ESS for 1973 through 2003 were obtained. RESULTS: One uterine primary ESS had collections of clear cells (20%), while a metastatic ESS contained predominantly clear cells (90%). CD10 (88.2%) and smooth muscle actin (70.5%) were the most common positive immunohistochemical markers. The latter marker was located in the cytoplasm in 47% of the ESS and in the nucleus in 23.5%, a previously unreported feature. HMB45 was detected in 23.5% of the ESS, which contrasts with the 2% reported by other authors. CONCLUSIONS: The presence of clear cells and HMB45 reactivity does not justify the term perivascular epithelioid cell tumors for these neoplasms. Two of 17 patients with ESS died of metastatic disease. However, among 274 cases of ESS (all stages included) collected by the Surveillance Epidemiology and End Results Program of the National Cancer Institute during a 30-year period, the 10-year survival rate was 94%.