Primary sarcoma of the cervix: an analysis of patient and tumor characteristics, treatment patterns, and outcomes

Ashley Albert,Anna Lee,Robert Allbright,Srinivasan Vijayakumar 대한부인종양학회 2020 Journal of Gynecologic Oncology Vol.31 No.3

Objective: Primary sarcoma of the cervix is rare and is associated with worse outcomes as compared to other histologies. The purpose of this study was to identify national treatment patterns and outcomes based on histological subtype using the National Cancer Database (NCDB). Methods: The NCDB was queried for patients with cervical cancer from 2004–2015. Clinico-demographic treatment details were obtained and compared between patients with squamous cell carcinoma (SCC), adenocarcinoma, and sarcoma of the cervix. Multivariable Cox regression and the Kaplan-Meier method was used to examine survival. Results: 107,177 patients met inclusion criteria including 81,245 (75.8%) women with SCC, 24,562 (22.9%) women with adenocarcinoma, and 1,370 (1.3%) women with sarcoma. Of the patients with cervical sarcoma, 680 (49.6%) patients had carcinosarcoma or malignant mixed Müllerian tumor, 255 (18.6%) patients had leiomyosarcoma, 197 (14.4%) patients had adenosarcoma, 28 (2.0%) patients had endometrial stromal sarcoma (ESS), 85 (6.2%) patients had rhabdomyosarcoma, and 125 (9.1%) patients had sarcoma not otherwise specified (NOS). Patients with sarcoma were older and more likely to be treated primarily with surgery. On multivariable Cox regression, sarcoma had decreased overall survival (OS) as compared to patients with SCC (hazard ratio=2.17; 95% CI=1.99–2.37; p<0.001). Among patients with sarcoma, 5-year OS was 89.2% for adenosarcoma, 66.2% for rhabdomyosarcoma, 55.6% for leiomyosarcoma, 45.8% for ESS, 31.6% for carcinosarcoma, and 29.2% for sarcoma NOS. Conclusions: Primary cervical sarcomas have inferior outcomes compared to SCC and adenocarcinoma. Sarcoma NOS and carcinosarcoma have the worst prognosis among sarcoma subtypes.

Vincristine, Irinotecan, and Temozolomide as a Salvage Regimen for Relapsed or Refractory Sarcoma in Children and Young Adults

주희영,박미림,이준아,박현진,박석연,김준혁,강현귀,양희철,박병규 대한암학회 2022 Cancer Research and Treatment Vol.54 No.2

Purpose No standard salvage regimen is available for relapsed or refractory sarcoma. We investigated the efficacy and toxicity of the vincristine, irinotecan, and temozolomide combination (VIT) for relapsed or refractory sarcomas of variable histology in children and young adults. Materials and Methods We retrospectively reviewed data from the relapsed or refractory sarcoma patients who were treated with VIT. The VIT protocol was given every 3 weeks as follows: vincristine, 1.5 mg/m2 intravenously on day 1, irinotecan, 50 mg/m2/day intravenously on days 1-5, and temozolomide, 100 mg/m2/day orally on days 1-5. Results A total of 26 patients (12 males) with various sarcoma histology were included in the study. Most common diagnosis was rhabdomyosarcoma (n=8) followed by osteosarcoma (n=7). Median age at the start of VIT was 18.5 years (range, 2.0 to 39.9). VIT was delivered as 2nd to 7th line of treatment, with 4th line most common (9/26, 34.6%). Median number of VIT courses given was 3 (range, 1 to 18). Of the 25 evaluable patients, there was two partial response (PR) and 11 stable disease (SD) with an overall control rate (complete remission+PR+SD) of 52%. PR was seen in one (50%) of the two evaluable patients with Ewing sarcoma and one (14.3%) of the seven patients with osteosarcoma. Overall survival and progression-free survival rates were 79.3% and 33.9% at 1 year, and 45.5% and 25.4% at 2 years, respectively. There was no treatment-related mortality. Conclusion The VIT regimen was effective and relatively safe in our cohort of sarcoma patients. Purpose No standard salvage regimen is available for relapsed or refractory sarcoma. We investigated the efficacy and toxicity of the vincristine, irinotecan, and temozolomide combination (VIT) for relapsed or refractory sarcomas of variable histology in children and young adults.Materials and Methods We retrospectively reviewed data from the relapsed or refractory sarcoma patients who were treated with VIT. The VIT protocol was given every 3 weeks as follows: vincristine, 1.5 mg/m2 intravenously on day 1, irinotecan, 50 mg/m2/day intravenously on days 1-5, and temozolomide, 100 mg/m2/day orally on days 1-5.Results A total of 26 patients (12 males) with various sarcoma histology were included in the study. Most common diagnosis was rhabdomyosarcoma (n=8) followed by osteosarcoma (n=7). Median age at the start of VIT was 18.5 years (range, 2.0 to 39.9). VIT was delivered as 2nd to 7th line of treatment, with 4th line most common (9/26, 34.6%). Median number of VIT courses given was 3 (range, 1 to 18). Of the 25 evaluable patients, there was two partial response (PR) and 11 stable disease (SD) with an overall control rate (complete remission+PR+SD) of 52%. PR was seen in one (50%) of the two evaluable patients with Ewing sarcoma and one (14.3%) of the seven patients with osteosarcoma. Overall survival and progression-free survival rates were 79.3% and 33.9% at 1 year, and 45.5% and 25.4% at 2 years, respectively. There was no treatment-related mortality.Conclusion The VIT regimen was effective and relatively safe in our cohort of sarcoma patients.

우측 협부에 발생한 윤활막육종 증례보고

허정,이근철,박정민,김석권,노미숙,이진화 대한성형외과학회 2005 Archives of Plastic Surgery Vol.32 No.6

Synovial Sarcoma is the fourth most common sarcoma, accounting for 8-10 % of all sarcomas. Synovial sarcoma is highly malignant tumor of mesenchymal origin but rarely occurres in head and neck area. Less than 100 cases of synovial sarcoma occurring in head and neck area have been reported all over the world.Pathologically, there is two type of synovial sarcoma: monophasic variant is composed of only one cell type and "classic" (biphasic) synovial sarcoma has two cellular component, a spindle cell(fibrosarcoma-like) component and a pseudoepithelioma component.Recommended treatment is wide resection with negative margins. The role of chemotherapy and radiation therapy is controversial.We experienced a 42-year-old male patient with slowly enlarging, deep seated mass on right cheek. In the first operation, we suggested that the mass maybe benign tumor. But, initial excisional biopsy specimen of the primary lesion was consistent with synovial sarcoma. The final diagnosis was monophasic synovial sarcoma which was composed of spindle cells. Radical resection was performed two months later because remnant tumor was found on follow up MRI. No further treatment was done. There were no recurrence or metastasis on follow up MRI, chest CT and whole body bone scan after 15 months.This is a report of a rare case of synovial sarcoma of the face with a literature review.

Delta-like Factor 1 as a Possible Therapeutic Target for Sarcomas

김한수,안선희,김순자,박종웅,한일규 대한정형외과학회 2020 Clinics in Orthopedic Surgery Vol.12 No.3

Background: Cancer stem cells (CSCs) are cells characterized by their self-renewal and tumorigenic potential. The purpose of this study was to discover the role of the delta-like factor 1 (DLK1) in sarcoma. Methods: mRNA expression of DLK1 from 13 sarcoma cell lines was examined. Isolated CSCs from the tumors were examined using fluorescence-activated cell sorting (FACS) with CD133, the CSC marker, or sphere-forming assay. The relationship between DLK1 and CSCs in sarcoma was examined using cell proliferation and cell invasion assays after they were treated with DLK1 short interfering RNA (siRNA). Results: A high expression of DLK1 mRNA was observed in all sarcoma cell lines. However, CSCs were isolated from over expressed sarcomas of the DLK1 gene, and they have shown to be expressed lower than the wild type. The anti-cancer effects of DLK1 siRNA inhibited cell proliferation and invasion in U2OS, A204, and sw872. In addition, treatment with DLK1 siRNA inhibited cell invasion in sw872 CSCs. DLK1 gene induces tumorigenesis in various sarcoma cells and regulates the invasiveness of liposarcoma. These results suggest that DLK1 could serve as a possible therapeutic target for sarcoma. Conclusions: Our study showed that the DLK1 gene induces tumorigenesis in various sarcomas and is associated with invasive mechanism in sarcoma. These results suggest DLK1 could serve as a possible therapeutic target in a variety of sarcomas.

Sarcoma Immunotherapy: Confronting Present Hurdles and Unveiling Upcoming Opportunities

정세한,Sharmin Afroz,Donghyun Kang,Jeonghwan Noh,서주연,June Hyuk Kim,유혜진,Hyun Guy Kang,김이준,김진홍 한국분자세포생물학회 2023 Molecules and cells Vol.46 No.10

Sarcomas are rare and heterogeneous mesenchymal neoplasms originating from the bone or soft tissues, which pose significant treatment challenges. The current standard treatment for sarcomas consists of surgical resection, often combined with chemo- and radiotherapy; however, local recurrence and metastasis remain significant concerns. Although immunotherapy has demonstrated promise in improving long-term survival rates for certain cancers, sarcomas are generally considered to be relatively less immunogenic than other tumors, presenting substantial challenges for effective immunotherapy. In this review, we examine the possible opportunities for sarcoma immunotherapy, noting cancer testis antigens expressed in sarcomas. We then cover the current status of immunotherapies in sarcomas, including progress in cancer vaccines, immune checkpoint inhibitors, and adoptive cellular therapy and their potential in combating these tumors. Furthermore, we discuss the limitations of immunotherapies in sarcomas, including a low tumor mutation burden and immunosuppressive tumor microenvironment, and explore potential strategies to tackle the immunosuppressive barriers in therapeutic interventions, shedding light on the development of effective and personalized treatments for sarcomas. Overall, this review provides a comprehensive overview of the current status and potential of immunotherapies in sarcoma treatment, highlighting the challenges and opportunities for developing effective therapies to improve the outcomes of patients with these rare malignancies.

금속단판 절단원에서의 유상피성 육종 1예

김민기,김규상,김태우,류향우,유재홍 大韓産業醫學會 2008 대한직업환경의학회지 Vol.20 No.2

배경: STS의 발생에 대해서 다양한 원인이 알려져 있으며,손상과 이물질도 그 원인으로 알려져 있으나 그 발생 증례는 매우 드물다. 저자들은 금속단판 절단원에서 지속적인 손상과 금속 이물질로 추정되는 물질과 관련성을 보이는 ES 증례를 경험하였기에 문헌고찰과 함께 보고하고자 한다. 증례: 환자는 17년간 금속단판 절단원으로 근무하였다. 작업 도중 지속적으로 작은 금속파편에 손바닥을 찔렸으며,작업 중에 금속파편이 손에 박혀 있어 퇴근 후에 매일 제거하였다. 2004년 3월에 손바닥에 종괴가 촉지되었으며, 2006년 11월에 수부 X-ray에서 이물질과 종괴가 발견되어 제거하였다. 종괴는 ES로 진단받았으며 환자가 사용했던 모든 금속봉에는 크롬과 니켈이 함유되어 있었다. 결론: 증례와 같은 이물질 및 손상과 관련을 보이는 STS 특히 ES은 매우 드물며,이물질에 의해 자주 노출되고 손상을 받는 근로자에서 육종이 발생했을 때,그 작업관련성을 의심하여 볼 필요성이 있다. Background: Soft tissue sarcoma is a rare condition with many causative factors. However a soft tissue sarcoma from a metallic foreign body and injury is rare. We report a case of a soft tissue sarcoma caused by a metallic foreign body and injury. Case: The patient had been worked as a metal cutter for 17 years. While working, his palms were constantly pricked by small metallic splinters, which were generally removed after work. On march 2004, he had palpitated mass on his right palm. On November 2006, a right hand x-ray showed a mass and metallic foreign body at the 1st volar space which was confirmed to be an epitheloid sarcoma. All metallic products of his workplace contained chromium and Nickel. which are known to cause soft tissue sarcomas. A review of the literature revealed foreign-body and injury induced oxidative and nitrative stress to be associated with a developing sarcoma. In addition, Cr and Ni are associated with the development of sarcoma. Therefore it was concluded this case ofepitheloid sarcoma was associated with his employment. Conclusion: A soft-tissue sarcoma associated with a metal foreign-body and injury is rare and their relationship is difficult understand. If a case of sarcoma is encountered on a metal associated worker, their job association should be considered.

저등급 자궁내막간질육종

박충학(Choong Hak Park),주선하(Seon Ha Joo),박호범(Ho Beom Park),서영훈(Young Hoon Suh),김동희(Dong Hee Kim) 대한산부인과학회 2000 Obstetrics & Gynecology Science Vol.43 No.1

Uterine sarcomas are relatively rare tumors of mesodermal origin that constitute 2-6% of uterine malignancies. Uterine sarcomas include endometrial stromal sarcoma (ESS), leiomyosarcoma (LMS), malignant mixed m llerian tumor (MMMT) of both homologous and heterologous type, pure heterologous sarcomas, blood vessel sarcomas, and lymphoma. Endometrial stromal tumors are divided into three types: 1) endometrial stromal nodule, 2) low-grade stromal sarcoma or endolymphatic stromal myosis, and 3) endometrial stromal sarcoma. The low grade endometrial stromal sarcoma is very rare. It shows occasional local recurrence, which might arise from endometrial stroma, from adenomyosis, and rarely from endometriosis. We have experienced a case of low grade endometrial stromal sarcoma of the uterus in a 54-year-old woman, which is presented with a review of literatures.

Cytology of Follicular Dendritic Cell Sarcoma on Intraoperative Touch Imprint Smears - A Case Report -

송주영,한지영,김루시아,박인서,김준미,Xian Ji Jin,최석진 대한병리학회 2009 Journal of Pathology and Translational Medicine Vol.43 No.6

Follicular dendritic cell (FDC) sarcoma is a neoplastic proliferation of FDCs. Because its cytologic findings can vary widely, both the cytomorphology and histopathology of FDC sarcoma can impose a significant diagnostic dilemma. We present cytologic features of FDC sarcoma assessed by intraoperative touch imprint. Intra-abdominal lymphadenopathies were noted in 54-year-old male with hepatitis B-virus associated liver cirrhosis. In contrast to cytologic features of classical FDC sarcoma, the tumor cells featured a large epithelioid or Reed-Sternberg cell-like shape scattered in a background with abundant inflammatory cells, which led to a mistaken diagnosis of malignant lymphoma. However, in accordance with cytologic features previously described in the literature, the tumor cells were characterized by a fragile cytoplasm with cytoplasmic processes in dendritic or reticulated patterns reminiscent of the ultrastructural features of FDC. Cytoplasmic features rendering nuclei with a tendency to form clusters or syncytial aggregates associated with reactive lymphocytes appear to be the most valuable finding in diagnosis of FDC sarcoma. Follicular dendritic cell (FDC) sarcoma is a neoplastic proliferation of FDCs. Because its cytologic findings can vary widely, both the cytomorphology and histopathology of FDC sarcoma can impose a significant diagnostic dilemma. We present cytologic features of FDC sarcoma assessed by intraoperative touch imprint. Intra-abdominal lymphadenopathies were noted in 54-year-old male with hepatitis B-virus associated liver cirrhosis. In contrast to cytologic features of classical FDC sarcoma, the tumor cells featured a large epithelioid or Reed-Sternberg cell-like shape scattered in a background with abundant inflammatory cells, which led to a mistaken diagnosis of malignant lymphoma. However, in accordance with cytologic features previously described in the literature, the tumor cells were characterized by a fragile cytoplasm with cytoplasmic processes in dendritic or reticulated patterns reminiscent of the ultrastructural features of FDC. Cytoplasmic features rendering nuclei with a tendency to form clusters or syncytial aggregates associated with reactive lymphocytes appear to be the most valuable finding in diagnosis of FDC sarcoma.

Distribution and survival of primary sarcoma in Korea

Sung Jun Jo,Kyeong Sik Kim,Kyo Won Lee,Jae Berm Park,Yoon-La Choi,Jeong Il Yu,Su Jin Lee,Dong Il Choi,Sung Joo Kim 대한종양외과학회 2018 Korean Journal of Clinical Oncology Vol.14 No.1

Purpose: Distribution and survival of sarcoma in Korea are not well described, after the changing of sarcoma classification on 2013. The researchers investigated the distribution and survival in single center 2017 cases of sarcoma. Methods: Patients with primary sarcoma, who underwent surgery, were investigated. All cases were collected during a 20 year period (1995–2015) from Samsung Medical Center in Korea. Histopathologic types were classified by World Health Organization (WHO) classification (2013). And overall survival rates were analyzed. Results: Between 1995 and 2015, 2017 patients were collected. The most frequent type of sarcoma was gastrointestinal tumor (15%), followed by liposarcoma (12%), leiomyosarcoma (9%), dermatofibrosarcoma (6%), giant cell sarcoma (6%). The most common primary site of sarcoma was the intra-abdominal area (45%, including visceral area). Extremities accounted for 26% of all cases. Sixteen percent of sarcoma were located in retroperitoneal area. The overall survival rate was 70.4% (median follow-up time, 36.8 months; range, 0.1–261.3 months). The best prognosis was dermatofibrosarcoma (100%, 5-year survival rate). The worst prognosis was angiosarcoma (39.3%). Survival analysis by the primary site demonstrated favor prognosis in extremities than head & neck, chest lesion. Conclusion: The researchers reported Korean sarcoma characteristics with using the new WHO classification.

Surgical Treatment Outcome of Primary Hepatic Sarcoma

( Sang Jin Kim ),( Jinsoo Rhu ),( Gyu-seong Choi ),( Jong Man Kim ),( Jae-won Joh ) 대한간학회 2020 춘·추계 학술대회 (KASL) Vol.2020 No.1

Aims: Primary hepatic sarcoma is rare disease with higher recurrence after surgical treatment. This study analyzed outcomes after surgical resection of patients with primary hepatic sarcoma. Methods: From 2001 august to 2016 september, 11 patients were pathologically diagnosed after surgical resection as hepatic sarcoma. Patient characteristics and tumor characteristics, treatment, tumor recurrence and patient survival were analyzed. Results: All patient except 1 was surgically treated with R0 resection. Mean follow up duration was 711 days after surgical resection. Among 11 patients, 5 patients were diagnosed as angiosarcoma, 3 were undifferentiated sarcoma, 1 was biliary cystadenocarcinoma, 1 was embryonal sarcoma and 1 was carcinosarcoma. Mean tumor size was 12.4cm and median tumor number was 1. All patients had recurrence and mean time to recurrence was 282 days. 9 patients get tumor recurrence within 1 years after surgery. These 9 patients are all expired due to cancer recurrence. Only 2 patients (18.2%) are still alive. 1 patient with angiosarcoma received central haptatectomy and got recurrence on liver segment 4 at 5 years later. RFA was done on S4 and he got no recurrence for 6 years. 1 patient with embryonal sarcoma received Rt. hemihepatectomy and got recurrence at abdominal wall at 1 year and 4 months later. After surgical resection and survive for 1 year without recurrence. Conclusions: Primary hepatic sarcoma has very poor outcome even after proper surgical resection. As known before, only embryonal sarcoma has better outcome, relatively.