Objective: To investigate the clinicopathological features, immunophenotype, molecular features and differential diagnosis of primary synovial sarcoma of the lung (PSSL). Methods: Twelve cases of PSSL were collected at Henan Provincial People's Hospital, during May 2010 and April 2021, and their clinicopathological parameters were summarized. SS18-SSX, H3K27Me3, and SOX2 were added to the original immunomarkers to evaluate their diagnostic value for PSSL. Results: The age of 12 patients when diagnosed ranged from 32 to 75 years (mean of 50 years). There were 7 males and 5 females, 2 left lung cases and 10 right lung cases. Of the 6 patients who underwent surgical resection, five cases were confined to lung tissue (T1), one case had mediastinal invasion (T3), two cases had regional lymph node metastasis (N1), and none had distal metastasis. Microscopically, 11 cases showed monophasic spindle cell type and one case showed biphasic type composed of mainly epithelial cells consisting of cuboidal to columnar cells with glandular and cribriform structures. It was difficult to make the diagnosis by using the biopsy specimens. Immunohistochemistry (IHC) showed CKpan expression in 8 of 12 cases; EMA expression in 11 of 12 case; TLE1 expression in 8 of 12 cases; S-100 protein expression in two of 12 cases; various expression of bcl-2 and vimentin in 12 cases, but no expression of SOX10 and CD34 in all the cases. The Ki-67 index was 15%-30%. The expression of SS18-SSX fusion antibody was diffusely and strongly positive in all 12 cases. SOX2 was partially or diffusely expressed in 8 of 12 cases, with strong expression in the epithelial component. H3K27Me3 was absent in 3 of 12 cases. SS18 gene translocation was confirmed by fluorescence in situ hybridization (FISH) test in all 12 samples. Six cases underwent surgery and postoperative chemotherapy, while the other six cases had chemotherapy alone. Ten patients were followed up after 9-114 months, with an average of 41 months and a median of 26 months. Five patients survived and five died of the disease within two years. Conclusions: PSSL is rare and has a broad morphological spectrum. IHC and molecular tests are needed for definitive diagnosis. Compared with current commonly used IHC markers, SS18-SSX fusion antibody has better sensitivity to PSSL, which could be used as an alternative for FISH, reverse transcription-polymerase chain reaction or next generation sequencing in the diagnosis of PSSL.
Male ; Female ; Humans ; Adult ; Middle Aged ; Aged ; Biomarkers, Tumor/analysis* ; Sarcoma, Synovial/diagnosis* ; In Situ Hybridization, Fluorescence ; Histones/genetics* ; Proto-Oncogene Proteins/metabolism* ; Oncogene Proteins, Fusion/genetics* ; Repressor Proteins/metabolism* ; Lung/pathology* ; Lung Neoplasms

YWHAE gene is located on chromosome 17p13.3, and its product 14-3-3epsilon protein belongs to 14-3-3 protein family. As a molecular scaffold, YWHAE participates in biological processes such as cell adhesion, cell cycle regulation, signal transduction and malignant transformation, and is closely related to many diseases. Overexpression of YWHAE in breast cancer can increase the ability of proliferation, migration and invasion of breast cancer cells. In gastric cancer, YWHAE acts as a negative regulator of MYC and CDC25B, which reduces their expression and inhibits the proliferation, migration, and invasion of gastric cancer cells, and enhances YWHAE-mediated transactivation of NF-κB through CagA. In colorectal cancer, YWHAE lncRNA, as a sponge molecule of miR-323a-3p and miR-532-5p, can compete for endogenous RNA through direct interaction with miR-323a-3p and miR-532-5p, thus up-regulating K-RAS/ERK/1/2 and PI3K-AKT signaling pathways and promoting the cell cycle progression of the colorectal cancer. YWHAE not only mediates tumorigenesis as a competitive endogenous RNA, but also affects gene expression through chromosome variation. For example, the FAM22B-YWHAE fusion gene caused by t(10; 17) (q22; p13) may be associated with the development of endometrial stromal sarcoma. At the same time, the fusion transcript of YWHAE and NUTM2B/E may also lead to the occurrence of endometrial stromal sarcoma. To understand the relationship between YWHAE, NUTM2A, and NUTM2B gene rearrangement/fusion and malignant tumor, YWHAE-FAM22 fusion gene/translocation and tumor, YWHAE gene polymorphism and mental illness, as well as the relationship between 17p13.3 region change and disease occurrence. It provides new idea and basis for understanding the effect of YWHAE gene molecular mechanism and genetic variation on the disease progression, and for the targeted for the diseases.
14-3-3 Proteins/metabolism* ; Breast Neoplasms/genetics* ; Cell Line, Tumor ; Cell Proliferation/genetics* ; Cell Transformation, Neoplastic/genetics* ; Colorectal Neoplasms/genetics* ; Endometrial Neoplasms ; Female ; Gene Expression Regulation, Neoplastic ; Humans ; MicroRNAs/genetics* ; Phosphatidylinositol 3-Kinases/metabolism* ; Sarcoma, Endometrial Stromal/pathology* ; Stomach Neoplasms/genetics* ; Transcription Factors/genetics* ; Translocation, Genetic

Adult ; Aged ; Algorithms ; Endometrial Neoplasms ; metabolism ; mortality ; surgery ; Female ; Humans ; Middle Aged ; Receptors, Estrogen ; metabolism ; Sarcoma, Endometrial Stromal ; metabolism ; mortality ; surgery ; Treatment Outcome

BackgroundDevelopment of innovative immunotherapy is imperative to improve the poor survival of the nasopharyngeal carcinoma (NPC) patients. In this study, we evaluated the T cell response to melanoma-associated antigen (MAGE)-A1, MAGE-A3, or synovial sarcoma X-2 (SSX-2) in the peripheral blood of treatment-naive NPC patients. The relationship of responses among the three proteins and the human leukocyte antigen (HLA)-A types were analyzed to provide evidence of designing novel therapy.

MethodsSixty-one NPC patients admitted into the Tumor Hospital affiliated to the Xinjiang Medical University between March 2015 and July 2016 were enrolled. Mononuclear cells were isolated from the peripheral blood before any treatment. HLA-A alleles were typed with Sanger sequence-based typing technique. The T cell response to the MAGE-A1, MAGE-A3, or SSX-2 was evaluated with the Enzyme-Linked ImmunoSpot assay. Mann-Whitney U-test was used to compare the T cell responses from different groups. Spearman's rank correlation was used to analyze the relationship of T cell responses.

ResultsHLA-A*02:01, A*02:07, and A*24:02 were the three most frequent alleles (18.9%, 12.3%, and 11.5%, respectively) among the 22 detected alleles. 31.1%, 19.7%, and 16.4% of the patients displayed MAGE-A1, MAGE-A3, or SSX-2-specific T cell response, respectively. The magnitudes of response to the three proteins were 32.5, 38.0, and 28.7 SFC/10 peripheral blood mononuclear cells, respectively. The T cell response against the three proteins correlated with each other to different extent. The percentage of A*02:01 and A*24:02 carriers were significantly higher in patients responding to any of the three proteins compared to the nonresponders.

ConclusionMAGE-A1, MAGE-A3, or SSX-2-specific T cell responses were detectable in a subgroup of NPC patients, the frequency and magnitude of which were correlated.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Alleles ; Antigens, Neoplasm ; immunology ; metabolism ; Carcinoma ; immunology ; metabolism ; Female ; HLA-A Antigens ; metabolism ; Humans ; Leukocytes, Mononuclear ; metabolism ; Male ; Middle Aged ; Nasopharyngeal Carcinoma ; Nasopharyngeal Neoplasms ; immunology ; metabolism ; Neoplasm Proteins ; metabolism ; Sarcoma, Synovial ; immunology ; metabolism ; Young Adult

BACKGROUND: Advances in surgical techniques, implant technology, radiotherapy, and chemotherapy have increased the recovery chances of patients with bone sarcomas. Accordingly, patients' expectations on life quality have also increased, highlighting the importance of objective evaluation of the functional results of reconstruction. METHODS: Thirteen patients with distal femoral endoprosthesis, who had been followed for an average of 2.9 years were evaluated. Postural stability, daily energy expenditure, muscle power, and range of motion were the four parameters analyzed in this study. The Musculoskeletal Tumor Society (MSTS) score and Toronto Extremity Salvage Score (TESS) were used to assess postoperative function and examine correlations with other parameters. RESULTS: Patients had sedentary activities in 84% of their daily lives. They exhibited a slower speed in the walk across test and a higher sway velocity in the sit-to-stand test (p = 0.005). MSTS scores were significantly correlated with the daily energy expenditure and walking speed. CONCLUSIONS: Objective functional results acquired from various clinics will provide significant data to compare reconstruction techniques, rehabilitation protocols, and surgical techniques. In this way, it will be possible to satisfy the expectations of patients that increase in relation to enhanced recovery.
Drug Therapy ; Energy Metabolism* ; Extremities ; Femur ; Humans ; Quality of Life ; Radiotherapy ; Range of Motion, Articular ; Rehabilitation ; Sarcoma ; Walking

OBJECTIVETo study the clinicopathological features and prognosis of renal cell carcinoma with sarcomatoid differentiation (RCCS).

METHODSThe clinical data and pathological materials of 18 RCCS cases were retrospectively reviewed.The follow up data were available in 13 RCCS cases, and were compared with the follow up data of 20 cases of clear cell renal cell carcinoma (RCC).

RESULTSThe 18 RCCS patients included 14 males and 4 females, and were 49-79 years old (mean age: 62 years old). On gross examination, the tumor size was 3-19 cm in diameter (mean diameter: 9.8 cm). Histologically, all tumors were composed of a mixture of typical RCC with sarcomatoid component, including 9 clear cell RCC, 3 chromophobe RCC and one papillary RCC. The sarcomatoid components included 9 cases of fibrosarcoma, 3 cases of leiomyosarcoma, 5 cases of malignant fibrous histocytoma and one case of undifferentiated sarcoma. Immunohistochemistry showed that the sarcomatoid components were strongly vimentin-positive in 18 cases, and one or more epithelial markers (EMA, AE1/AE3, CK7, CK18) were expressed to varying degrees in 14 cases, but the high-molecular weight keratin 34βE12 was scarcely expressed. The sarcomatoid components presented positive expressions of CAIX in 88.9% (16/18) and CD10 in 72.2% (13/18) cases. Among the 18 RCCS patients, 13 patients were followed-up: 9 patients died in 1-25 months after the surgery, of which 5 cases died of lung or bone metastasis, and 4 patients died of systemic failure. The twenty RCC cases without sarcomatoid differentiation were followed up for 3-65 months after the surgery, and the majority of them was alive uneventfully except for 2 cases who died of lung or bone metastasis of the tumor. The Kaplan-Meier survival analysis showed that the median survival time of the 18 RCCS patients was 8 months, while that of the 20 RCC cases without sarcomatoid differentiation was 62 months (P<0.001).

CONCLUSIONSThe presence of sarcomatoid differentiation in renal cell carcinoma indicates highly aggressive behavior and poor prognosis. The positive expressions of the immune markers CAIX and CD10 may play important roles in the transformation from renal cell carcinoma to sarcomatoid component.

Aged ; Biomarkers, Tumor ; metabolism ; Carcinoma, Renal Cell ; metabolism ; mortality ; pathology ; Female ; Humans ; Immunohistochemistry ; Kaplan-Meier Estimate ; Kidney Neoplasms ; metabolism ; mortality ; pathology ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Sarcoma ; metabolism ; mortality ; pathology ; Tumor Burden

Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm that shows benign histologic features but may have clinical course of malignant disease. It has been reported to occur in the thigh, inguinal area, axilla, shoulder, neck, perineum or buttock. However, there have been few cases of abdominal organ involvement. A 21-year-old woman presented with a large palpable abdominal mass. A 7x4 cm sized round soft tissue tumor at right upper quadrant area was identified by abdominopelvic CT scan. Percutaneous ultrasound-guided biopsy revealed features of spindle cell tumor. On exploration, the tumor originated from transvers colon and was attached to gastrocolic ligament, transverse mesocolon and stomach. The tumor could be dissected with transverse colectomy and partial gastrectomy. The excised tumor, measuring 7x5x5 cm, was well demarcated and appeared as an ovoid mass with firm and myxoid cut surface. She was diagnosed with low-grade fibromyxoid sarcoma arising from transverse colon, and is currently being followed-up without recurrence or metastasis.
Colon, Transverse ; Female ; Humans ; S100 Proteins/metabolism ; Sarcoma/*diagnosis/pathology/surgery ; Soft Tissue Neoplasms/*diagnosis/pathology/surgery ; Stomach Neoplasms/pathology/secondary ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVETo study the regulation of luteolin on spleen cells and sarcoma S180 cells in normal ICR mice.

METHODSSpleen cells and S180 cells were incubated with different concentrations of luteolin (50, 100, 200, and 400 μmol/L). The effect of luteolin on spleen cells and sarcoma S180 cells was determined by MTT assay. The apoptosis was detected using propidium iodide staining flow cytometry. Intracellular reactive oxygen species (ROS) was determined by flow cytometric analysis. Activities of free radicals scavenging were determined by hydroxyl radical and DPPH tests.

RESULTSCompared with the solvent control group, 200 and 400 μmol/L luteolin increased the spleen cells viability (P < 0.05). Luteolin at 100, 200, and 400 μmol/L decreased activities of S180 cells (P < 0.01). The proportion of sub-G1 phase spleen cells was reduced after treated with 200 and 400 μmol/L luteolin (P < 0.05). The proportion of sub-G1 phase S180 cells was elevated after treated with 200 and 400 μmol/L luteolin (P < 0.05). Compared with the solvent control group, levels of intracellular ROS in spleen cells of ICR mice all increased; levels of intracellular ROS in S180 cells all decreased after treated with 50, 100, 200, and 400 μmol/L luteolin (P < 0.05). Luteolin scavenged hydroxyl radical and DPPH in a dose dependent manner.

CONCLUSIONLuteolin had bilateral regulation on viability and apoptosis of spleen cells and S180 cells (promoting the viability of spleen cells, inhibiting apoptosis of spleen cells, inhibiting the viability of S180 cells, and promoting apoptosis of S180 cells), which was worth further study and exploration.

Animals ; Apoptosis ; Apoptosis Regulatory Proteins ; metabolism ; Cell Survival ; Luteolin ; metabolism ; Mice ; Mice, Inbred ICR ; Reactive Oxygen Species ; Sarcoma ; Spleen ; metabolism

OBJECTIVETo study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T).

METHODSThe clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature.

RESULTSThe age of patients ranged from 20 to 68 years. The sites of involvement included lymph node, tonsil and soft tissue. Histologically, the tumor cells were arranged in sheets, fascicles or whorls and intimately admixed with abundant lymphocytes and plasma cells. They were oval to spindly in shape and contained pale eosinophilic cytoplasm, oval nuclei and distinct nucleoli.Immunohistochemical study showed that the tumor cells were positive for S-100 protein and CD68.

CONCLUSIONSIDCS/T is a rare malignant tumor with poor prognosis. It carries distinctive histologic pattern and immunophenotype. The entity needs to be distinguished from follicular dendritic cell sarcoma/tumor, anaplastic large cell lymphoma and other spindle cell sarcomas in occurring soft tissue.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Lymph Nodes ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Middle Aged ; Neck ; S100 Proteins ; metabolism ; Sarcoma ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; Thigh ; Tonsillar Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

Adult ; Angiomyolipoma ; pathology ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangiopericytoma ; pathology ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Nephrectomy ; Sarcoma, Synovial ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology ; surgery ; Vimentin ; metabolism