Pulmonary Carcinosarcoma: A Surveillance,Epidemiology, and End Results (SEER) Analysis

IntroductionPulmonary carcinosarcoma (PC) is a rare malignant neoplasm composed of epithelial and mesenchymal components. It accounts for < 1% of thoracic cancers and is not fully understood. This study examined Surveillance, Epidemiology, and End Results (SEER) data to describe demographic and clinical characteristics of patients with PC and assessed survival outcomes by treatment modality and stage.Patients and MethodsSEER data were reviewed to identify patients diagnosed with primary PC (1973-2012). Overall survival (OS) and disease-specific survival (DSS) were analyzed by univariate/multivariable Cox proportional hazards models and Kaplan-Meier methods.ResultsA total of 411 patients were included. Median age was 67 (range, 24-96) years. Disease stage at the time of initial diagnosis was known for 74.7% of the identified patients (307/411). Of these patients, 23.1% had localized disease. Survival was significantly better for patients with localized disease (OS: 31 vs. 6 months, P < .001; DSS: 54 vs. 8 months, P < .001). Additionally, patients who received surgery alone had significantly improved OS (20 months; P < .001) and DSS (32 months; P < .001) compared to patients who received combined surgery and radiotherapy (OS: 7 months; DSS: 8 months) or radiotherapy alone (OS: 4 months; DSS: 4 months).ConclusionTreatment with surgery alone resulted in superior survival outcomes compared to other treatment modality combinations, regardless of patient age and disease stage. Within the limitations of this study, providers may wish to consider these findings when devising patient treatment plans.     

Syringocystadenocarcinoma papilliferum associated with atypical stroma: A hitherto undocumented variant of sarcomatoid carcinoma

Carcinosarcomas are biphasic tumors composed of admixed malignant epithelial and mesenchymal components. Numerous terms have been used to name such neoplasms; therefore, terminological confusion is frequent. Most examples of carcinosarcomas are encountered in non‐cutaneous sites, with approximately 100 cases of cutaneous carcinosarcomas reported so far in the English literature. Although different theories have been suggested to explain the occurrence of these peculiar neoplasms, histogenetic mechanisms should be better hypothesized depending on each individual case. Even though prognosis tends to be related to the specific components of the lesion, especially the epithelial one, it seems that cases of cutaneous localization usually have a better outcome. We report an exceedingly rare case of syringocystadenocarcinoma papilliferum which showed an atypical stroma with sarcomatoid appearance, and highlight that the terminology used for this spectrum of lesions is disorganized and confusing.     

Cáncer sincrónico de carcinosarcoma uterino y carcinoma seroso tubárico. A propósito de un caso y revisión de la bibliografía

We report a rare case of uterine carcinosarcoma involving a 74-year-old woman. The patient complained of posmenopausal bleeding. The disease was eventually diagnosed as simultaneous uterine carcinosarcoma and high-grade serous tubal carcinoma.Clinical examination found a cervical tumour and transvaginal ultrasound showed a heterogeneous intrauterine image. The histological result of both findings was carcinosarcoma.The computed tomography scan and magnetic resonance imaging reported similar findings for intrauterine and cervical tumour, with the same features as a possible myoma or mass with sarcomatous degeneration.Given the suspicion of a high-risk variant of endometrial adenocarcinoma, the primary management of carcinosarcoma is surgery. Complete surgical staging included total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic and para-aortic lymph node dissection.The pathological findings revealed a uterine carcinosarcoma tumour, invading less than half the myometrium and the stromal connective tissue of the cervix but not extending beyond the uterus. The histopathological studies of bilateral adnexectomy and the peritoneal biopsy demonstrated the presence of high-grade serous tubal carcinoma. The lymph study was negative for malignancy.We concluded a synchronous diagnosis of uterine carcinosarcoma stage II and high-grade serous tubal carcinoma stage IIIB.A detailed literature search and management of this entity are discussed.     

喉癌肉瘤1例

癌肉瘤是一种由上皮和间质成分组成的恶性肿瘤，喉部癌肉瘤罕见，恶性程度高，侵袭性强，预后差。     

Role of TGF-β signaling in uterine carcinosarcoma

Uterine carcinosarcomas (UCS) are rare (3-4%) but highly aggressive, accounting for a disproportionately high (16.4%) mortality among uterine malignancies. Transforming growth factor beta (TGFβ) is a multifunctional cytokine that regulates important cellular processes including epithelial-mesenchymal transition (EMT). Existence of biphasic elements and a report demonstrating amplification of TGFβ at 19q13.1 prompted us to investigate the role of TGFβ signaling in UCS.Here we demonstrated the components of TGFβ pathway are expressed and functional in UCS. TGFβ-I induced significant Smad2/3 phosphorylation, migration and EMT responses in UCS cell lines which could be attenuated by the TGFβ receptor I (TGFβR-I) or TGFβ receptor I/II (TGFβR-I/II) inhibitor developed by Eli Lilly and company. Importantly, TGFβ-I induced proliferation was c-Myc dependent, likely through activation of cell cycle. c-Myc was induced by nuclear translocation of nuclear factor of activated T cells (NFAT-1) in response to TGFβ-I. Inhibition of NFAT-1 or TGFβR-I blocked c-Myc induction, cell cycle progression and proliferation in UCS. In corroboration, mRNA levels of c-Myc were elevated in recurrent versus the non-recurrent UCS patient samples. Interestingly, in the absence of exogenous TGFβ the TGFβR-I/II inhibitor enhanced proliferation likely through non-Smad pathways. Thus, inhibition of TGFβR-I could be efficacious in treatment of UCS.     

肝肉瘤样癌1例报告

正肝肉瘤样癌(sarcomatiod hepatocellular carcinoma,SHC)是一种原发于肝脏的罕见恶性上皮性肿瘤,具有恶性程度高、预后极差的特点,甚至有报道1年存活率几乎为零。吉林大学第一医院收治肝肉瘤样癌患者1例,现报道如下。1病例资料患者男性,61岁,半年前无明显诱因出现肝区钝痛,未经系统检查治疗,入院前4 d出现肝区疼痛加重,门诊行腹部CT