Background: Follicular dendritic cell sarcoma (FDCS) accounts for about 0.4% of soft tissue sarcomas. Approximately onethird of cases occur in extranodal sites and about 28% of extranodal FDCS may metastasize. Intra-abdominal occurrence is rare and there is limited published data to guide oncologists on how to best treat this malignancy. Case Presentation: This is a case of a 33-year-old female who came in due to incidental finding of a left supraclavicular mass with 2-year history of early satiety. Neck node biopsy revealed a poorly differentiated malignant tumor with positive staining for CD21, CD23, vimentin and S100 consistent with FDCS. PET-CT revealed an intensely FDG-avid large mass in the left upper abdomen with signs of necrosis and mass effect. The patient was given three different chemotherapy regimens that included (1) gemcitabine/docetaxel, (2) single agent doxorubicin and (3) ifosfamide/etoposide, but she progressed on all these. Off-label use of bendamustine was then offered and after just the first cycle, the patient reportedly regained strength and was able to get up from wheelchair with noted interval decrease in size of the cervical mass. Unfortunately, the patient deteriorated and succumbed to infection and multiple pulmonary embolisms. Conclusion Intra-abdominal FDCS is a rare malignancy with heterogenous outcomes with no uniform treatment strategy at present. Molecular tumor board discussion and multi-disciplinary approach in extranodal FDCS is important in the diagnosis and management. Patients with multiple poor prognostic factors are at risk for tumor recurrence, metastasis, and death.
Dendritic Cell Sarcoma, Follicular ; Abdominal Neoplasms ; Drug Therapy ; Bendamustine Hydrochloride ; Prognosis

Objective: To investigate the clinicopathological features and differential diagnosis of CIC-rearranged sarcoma (CRS). Methods: Five CRSs of 4 patients (2 biopsies of pelvic cavity and lung metastasis from case 4) diagnosed in the First Affiliated Hospital of Nanjing Medical University were enrolled from 2019 to 2021. All cases were evaluated by clinical presentation, H&E, immunohistochemical staining and molecular analysis and the related literature was reviewed. Results: There were one male and three females, the age at diagnosis ranged from 18 to 58 (mean 42.5) years. Three cases were from the deep soft tissues of the trunk and one case from the skin of foot. Grossly, the tumor size ranged from 1 to 16 cm. Microscopically, the tumor was arranged in nodules or solid sheets. The tumor cells were typically round or ovoid, with occasional spindled or epithelioid morphology. The nuclei were round to ovoid with vesicular chromatin and prominent nucleoli. Mitotic figures were brisk (>10/10 HPF). Rhabdoid cells were seen in four of five cases. Myxoid change and hemorrhage were observed in all samples and two cases showed geographic necrosis. Immunohistochemically, CD99 was variably positive in all samples, while WT1 and TLE-1 were positive in four of five samples. Molecular analysis showed CIC-rearrangements in all cases. Two patients succumbed within 3 months. One had mediastinal metastasis 9 months after surgery. One underwent adjuvant chemotherapy and remained tumor-free 10 months after diagnosis. Conclusions: CIC-rearranged sarcoma is uncommon and shows aggressive clinical course with dismal prognosis. The morphological and immunohistochemical characteristics can largely overlap with a variety of sarcomas; hence, knowledge of this entity is vital to avoid potential diagnostic pitfalls. Definitive diagnosis requires molecular confirmation of CIC-gene rearrangement.
Repressor Proteins/genetics* ; Sarcoma/therapy* ; Humans ; Male ; Female ; Adult

Objective: Patients with advanced sarcomas have a dismal prognosis with few effective therapies. The purpose of this study was to evaluate the efficacy and safety of anlotinib in the treatment of advanced sarcoma and to explore the relationship between adverse events (AEs) and efficacy. Methods: Data from 45 advanced sarcoma patients who received anlotinib monotherapy at Affiliated Cancer Hospital of Zhengzhou University between June 2018 and August 2021 were retrospectively analyzed. According to Response Evaluation Criteria In Solid Tumors (RECIST) Version 1.1, the objective remission rate (ORR) and disease control rate (DCR) were calculated, and the progression free survival (PFS) and treatment-related AEs were recorded and analyzed. Survival analysis was conducted using the Kaplan-Meier survival rates were compared using the Log rank test. Results: Forty patients were treated for more than 1.5 months and received efficacy evaluation. The ORR and DCR after 3 months were 7.5%(3/40) and 80.0%(32/40), respectively. The overall ORR was 2.5%(1/40), the total DCR was 27.5%(11/40), and the median progression-free survival (m-PFS) was 6.70 months; The m-PFS of alveolar soft tissue sarcoma (ASPS) was 10.27 months, which was significantly longer than that of other subtypes of sarcoma (P=0.048). In addition, the DCR of ASPS and synovial sarcoma (SS) was significantly better than that of osteosarcoma (P<0.05). The most common AEs were elevated thyroid stimulating hormone (17.8%, 8/45), anemia (15.6%, 7/45), fatigue (11.1%, 5/45). Five patients developed grade 3 AEs after treatment; The PFS of patients with hand-foot syndrome after treatment was significantly longer than that of patients without hand-foot syndrome (14.10 vs 6.00, P=0.024). Conclusions: The efficacy of anlotinib in the treatment of ASPS and SS is better than that of other subtypes. The PFS in the group with hand-foot syndrome was significantly longer than that of the group without hand-foot syndrome.
Humans ; Hand-Foot Syndrome ; Retrospective Studies ; Sarcoma/drug therapy* ; Sarcoma, Synovial/drug therapy* ; Soft Tissue Neoplasms ; Bone Neoplasms

Objective: Patients with advanced sarcomas have a dismal prognosis with few effective therapies. The purpose of this study was to evaluate the efficacy and safety of anlotinib in the treatment of advanced sarcoma and to explore the relationship between adverse events (AEs) and efficacy. Methods: Data from 45 advanced sarcoma patients who received anlotinib monotherapy at Affiliated Cancer Hospital of Zhengzhou University between June 2018 and August 2021 were retrospectively analyzed. According to Response Evaluation Criteria In Solid Tumors (RECIST) Version 1.1, the objective remission rate (ORR) and disease control rate (DCR) were calculated, and the progression free survival (PFS) and treatment-related AEs were recorded and analyzed. Survival analysis was conducted using the Kaplan-Meier survival rates were compared using the Log rank test. Results: Forty patients were treated for more than 1.5 months and received efficacy evaluation. The ORR and DCR after 3 months were 7.5%(3/40) and 80.0%(32/40), respectively. The overall ORR was 2.5%(1/40), the total DCR was 27.5%(11/40), and the median progression-free survival (m-PFS) was 6.70 months; The m-PFS of alveolar soft tissue sarcoma (ASPS) was 10.27 months, which was significantly longer than that of other subtypes of sarcoma (P=0.048). In addition, the DCR of ASPS and synovial sarcoma (SS) was significantly better than that of osteosarcoma (P<0.05). The most common AEs were elevated thyroid stimulating hormone (17.8%, 8/45), anemia (15.6%, 7/45), fatigue (11.1%, 5/45). Five patients developed grade 3 AEs after treatment; The PFS of patients with hand-foot syndrome after treatment was significantly longer than that of patients without hand-foot syndrome (14.10 vs 6.00, P=0.024). Conclusions: The efficacy of anlotinib in the treatment of ASPS and SS is better than that of other subtypes. The PFS in the group with hand-foot syndrome was significantly longer than that of the group without hand-foot syndrome.
Humans ; Hand-Foot Syndrome ; Retrospective Studies ; Sarcoma/drug therapy* ; Sarcoma, Synovial/drug therapy* ; Soft Tissue Neoplasms ; Bone Neoplasms

OBJECTIVE: To study the clinical features, treatment, and prognosis of neonates with Kasabach-Merritt syndrome (KMS), and to provide a reference for optimizing the diagnosis and treatment of this disease. METHODS: A retrospective analysis was performed for the clinical and follow-up data of 16 neonates with KMS who were admitted to the Anhui Children's Hospital, Anhui Medical University, from January 2016 to December 2020. RESULTS: Of the 16 neonates, there were 13 boys (81%) and 3 girls (19%), with an age of 1 hour to 10 days on admission. Among these neonates, 13 (81%) had cutaneous hemangioma (2 in the head and face, 5 in the trunk, and 6 in the extremities) and 3 (19%) had liver hemangioma. The main clinical manifestations of bleeding tendency and scattered petechiae and ecchymosis were observed in 10 neonates (62%). All the 16 neonates had varying degrees of thrombocytopenia and coagulation disorders. They all received glucocorticoid treatment after admission and 7 (44%) of them had response, among whom 4 experienced recurrence. Among the neonates with no response to glucocorticoid treatment, 3 received sirolimus treatment, among whom 1 had the tumor volume reduced by 58.8% after 4 weeks of treatment, with platelet count and coagulation function returning to normal, while 2 had no significant reduction in tumor volume or significant increase in platelet count and achieved a tumor volume reduced by (43.7±0.4)% after 4 weeks of combined treatment with bleomycin arterial embolization, with platelet count and coagulation function returning to normal. After 4 weeks of bleomycin arterial embolization alone for 4 neonates, tumor volume was reduced by (52.0±3.4)%, and platelet count and coagulation function returned to normal. Blunt and sharp dissection was performed for 2 neonates. The tumor was removed completely during surgery in the 2 neonates, with no infection or recurrence after surgery, and platelet count and coagulation function returned to normal. The postoperative pathological examination showed Kaposiform hemangioendothelioma in 1 out of the 2 neonates. CONCLUSIONS KMS has characteristic clinical manifestations, histopathological features, and laboratory examination results. The KMS neonates who are not sensitive to glucocorticoids can achieve a good curative effect through arterial embolization and sirolimus treatment.
Child ; Female ; Hemangioendothelioma ; Humans ; Infant, Newborn ; Kasabach-Merritt Syndrome/therapy* ; Male ; Neoplasm Recurrence, Local ; Retrospective Studies ; Sarcoma, Kaposi

Extraskeletal Ewing sarcoma is a rare event, and extraskeletal Ewing sarcoma of the thyroid gland is even rarer. It has non-specific clinical manifestation and difficulty in early diagnosis. The diagnosis mainly depends on histology and immunohistochemistry. It possesses the features of high malignancy, high rate of local recurrence, and distant metastasis. Currently, the aggressive multimodal treatment contains surgery, chemotherapy, and radiotherapy. This study presented a case of extraskeletal Ewing sarcoma arising in the thyroid gland of a 30-year-old woman, who presented with supraclavicular mass and sense of dysphagia obstruction in Department of Otolaryngology, Head and Neck Surgery, Second Xiangya Hospital, Central South University in 2018. Imaging studies demonstrated a cystic-solid mass in inferior of the left leaf of thyroid gland and in the posterior of the trachea and esophagus. The patient underwent localized tumor resection. The pathological diagnosis revealed that it was a small round cell tumor, and the immunohistochemistry results were considered to be extraskeletal Ewing sarcoma. Subsequently, the patient was given chemotherapy and local radiation therapy. There was no evidence of tumor recurrence or metastasis.
Adult ; Combined Modality Therapy ; Female ; Humans ; Immunohistochemistry ; Neoplasm Recurrence, Local ; Sarcoma, Ewing/therapy* ; Thyroid Gland

Bone Neoplasms/therapy* ; Child ; Humans ; Sarcoma, Ewing/therapy*

OBJECTIVE: To explore the antitumor effects of ethanol extract from Ventilago leiocarpa Benth (EEVLB) on sarcoma 180 (S180) tumor-bearing mice and the potential mechanism. METHODS: Sixty mice were randomly assigned to 6 groups according to a random number table: normal group, model group, 5-fluorouracil (5-FU) group (0.02 g·kg RESULTS: EEVLB with different concentrations achieved inhibition of tumor growth in vivo, wherein the high-dose group showed the most significant reduction in tumor weight and increased apoptosis of tumor cells (P<0.05). In addition, both net weight gain and spleen index of mice showed uptrend in EEVLB treatment groups (P<0.05). Besides, serum levels of IL-2 and IL-6, percentages of CD3 CONCLUSIONS EEVLB exhibits promising antitumor activity in vivo. This effect might be due to activation of apoptotic signaling pathway, increase of cytokine levels and enhancement of immune function in tumor-bearing mice.
Animals ; Cell Line, Tumor ; Ethanol ; Mice ; Plant Extracts/therapeutic use* ; Rhamnaceae ; Sarcoma 180/drug therapy*

Introduction: Acquired immunodeficiency syndrome-Kaposi sarcoma (AIDS-KS) has unique clinical characteristics, often dis- seminated on presentation, a rapidly progressive course, and often fatal outcome. Describing the epidemiology and clinical characteristics of AIDS-KS in the Philippines may lead to early recognition, diagnosis, and management of this condition, which are the keys to preventing significant complications. Case Series: AIDS-KS in 11 Filipino MSM patients with a mean age of 36.55 years (SD 11.54) was described. Violaceous plaques and nodules were present for an average of 5.1 months prior to diagnosis confirmed by biopsy. Histopathologic findings from all pa- tients were consistent with KS. The median CD4+ count of patients was 44 cells/microliter (range, 4 to 181). Six patients presented with opportunistic infections (OI)/AIDS-related conditions (ARC). The most common OIs observed were pulmonary tuberculosis, oropharyngeal candidiasis, and Pneumocystis jiroveci pneumonia. Nine patients improved with highly active antiretroviral therapy (HAART). One patient required modification on his HAART regimen, which was shifted to 2 NRTI and ritonavir-boosted protease inhibitor, and one patient died due to AIDS-related complications. Conclusion This series of 11 cases of AIDS-KS showed similar demographic, clinical and histopathologic characteristics to pre- viously published studies. Findings suggest the need for earlier recognition and diagnosis. While HAART afforded clinical improve- ment in a majority of patients, other treatment options such as chemotherapy should be considered for appropriate patients.
Sarcoma, Kaposi ; Antiretroviral Therapy, Highly Active

Kaposi's sarcoma (KS) is an unusual vascular tumor associated with human herpesvirus-8 (HHV-8) infection, which is common in immunosuppressors. Although extremely rare, iatrogenic (drug-related) KS can occur in human immunodeficiency virus (HIV)-negative patients under immunosuppressive therapy. We report a 64-year-old male diagnosed with ulcerative colitis for 1 year. He was treated with methylprednisolone because of an acute severe disease flare. He presented with several popular violet lesions on the body 4 months after steroid therapy. Histological examination of skin biopsies showed Kaposi's sarcoma associated with HHV-8. The skin lesions regressed after steroid withdrawal and chemotherapy. Two key words "Kaposi's sarcoma" and "inflammatory bowel disease" were searched in Wanfang data and CNKI, but no relevant articles were found. Thirty-eight articles in English were retrieved on PubMed with the key words of ("ulcerative colitis" OR "Crohn's disease" OR "inflammatory bowel disease") AND (Kaposi sarcoma). Twenty-five cases of Kaposi's sarcoma related to inflammatory bowel disease (IBD) were reported. Including this case, the majority of 26 Kaposi's sarcoma related IBD patients were male (80.8%, 21/26). The average age was (51.1 ± 16.4) years. Twenty cases were ulcerative colitis and 6 were Crohn's disease. All the patients received immunomodulatory therapy, including glucocorticoid, azathioprine/mercaptopurine, methotrexate, cyclosporin and anti tumor necrosis factor α antibody. Thirteen cases were positive for HHV-8. There were 18 cases involving the distal ileum and colorectum only, 3 cases involving skin only, and 5 cases involving both skin and colorectum at the same time. Overall, the prognosis was good. Three patients only stopped immunosuppressive therapy, 1 received radiotherapy, 1 received chemotherapy, and 20 received surgery. Kaposi's sarcoma could be seen in IBD patients with immunomodulatory therapy. It is very important to distinguish from the skin lesions related to IBD or drug treatment. The adverse reactions of immunomodulatory therapy should not be ignored. In addition, attention should be paid to the cooperation of multi-disciplinary team, which can diagnose and treat rare cases earlier and more accurately.
Adult ; Aged ; Colitis, Ulcerative ; Crohn Disease ; Herpesvirus 8, Human ; Humans ; Immunosuppression Therapy ; Male ; Middle Aged ; Sarcoma, Kaposi