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

Diagnosis, Differential ; Humans ; Sarcoma, Myeloid/diagnosis*

Humans ; Dendritic Cell Sarcoma, Follicular/diagnosis* ; Granuloma, Plasma Cell/diagnosis* ; Biopsy, Needle ; Gastrointestinal Neoplasms ; Hepatitis

Female ; Humans ; Reference Standards ; Sarcoma/diagnosis* ; Soft Tissue Neoplasms ; Uterine Neoplasms/diagnosis*

OBJECTIVE: To observe the expression of HELQ and RAD51C in normal endometrial and endometrial stromal sarcoma (ESS) and analyze their correlation with the clinical features of the patients. METHODS: The expressions of HELQ and RAD51C proteins were detected by immunohistochemical staining in normal endometrial tissues (14 cases) and tumor tissues from patients with ESS (37 cases) treated in Hunan Provincial Cancer Hospital from January, 2013 to December, 2016. The correlations of the expressions of the two proteins with the patients'age, FIGO staging, tissue type, tumor size, and lymph node metastasis were analyzed. RESULTS: Immunohistochemical staining showed that the expressions of HELQ and RAD51C were both decreased in ESS patients compared with the normal group, and there was a positive correlation between HELQ and RAD51C expression ( < 0.05). HELQ expression in ESS was correlated with the tumor size and type. The expressions of HELQ and RAD51C were not correlated with the patients' age, FIGO stage and status of lymph node metastasis ( > 0.05). CONCLUSIONS Homologous recombination- directed DNA repair involving HELQ and RAD51C may participate in the occurrence and progression of ESS.
DNA Helicases ; genetics ; DNA-Binding Proteins ; genetics ; Endometrial Neoplasms ; diagnosis ; physiopathology ; Endometrium ; physiopathology ; Female ; Gene Expression Regulation, Neoplastic ; Humans ; Lymphatic Metastasis ; physiopathology ; Sarcoma, Endometrial Stromal ; physiopathology

Clear cell odontogenic carcinoma (CCOC), a very rare neoplasm located mostly in the mandible, has been regarded as a benign tumor. However, due to the accumulation of case reports, CCOC has been reclassified as a malignant entity by the World Health Organization. Patients with CCOC present with regional swelling and periodontal indications with variable pain, often remaining misdiagnosed for a long period. CCOC has slow growth but aggressive behavior, requiring radical resection. Histologic analysis revealed the monophasic, biphasic, and ameloblastic types of CCOC with clear cells and a mixed combination of polygonal and palisading cells. At the molecular level, CCOC shows the expression of cytokeratin and epithelial membrane antigen, along with markers that assign CCOC to the sarcoma family. At the genetic level, Ewing sarcoma breakpoint region 1-activating transcription factor 1 fusion is regarded as the key feature for identification. Nevertheless, the scarcity of cases and dependence on histological data delay the development of an efficient therapy. Regarding the high recurrence rate and the potential of distant metastasis, further characterization of CCOC is necessary for an early and accurate diagnosis.
Ameloblasts ; Diagnosis ; Humans ; Keratins ; Mandible ; Mucin-1 ; Neoplasm Metastasis ; Odontogenic Tumors ; Recurrence ; Sarcoma ; Sarcoma, Ewing ; Transcription Factors ; World Health Organization

STUDY DESIGN: A retrospective multi-center study.OBJECTIVES: To analyze oncological outcomes according to the resection type and surgical margin following surgical treatment for primary spinal sarcoma.SUMMARY OF LITERATURE REVIEW: Previous studies using registry databases have shown that surgery and negative margins were associated with improved survival for primary spinal sarcoma. However, few studies have comprehensively analyzed the clinical significance of the resection type and surgical margin for the oncological outcomes of this rare malignancy.MATERIALS AND METHODS: We retrospectively reviewed consecutive patients who underwent surgical resection for primary spinal sarcoma between 1997 and 2016 at two tertiary medical centers. Overall survival and the occurrence of local recurrence and distant metastasis were compared between the groups using Kaplan-Meier curve analysis and the log-rank test.RESULTS: Thirty-three patients (21 males,12 females) with a mean age of 45.1 years and a median follow-up of 36 months were included. There were 13 (39.4%) chondrosarcomas, 12 (36.4%) osteosarcomas, and eight different histological diagnoses. The cohort was categorized into four groups: 1) total en bloc resection with a negative margin (n=12; 36.4%), 2) total en bloc resection with a positive margin: (n=5; 15.2%), 3) total piecemeal resection (n=12; 36.4%), and 4) subtotal resection (n=4; 12.1%). Total en bloc resection with a negative margin was associated with improved overall survival (p=0.030) and less distant metastasis (p=0.025) and local recurrence (p=0.004).CONCLUSIONS: Achieving a negative margin through total en bloc resection, although technically demanding, improves oncological outcomes in primary spinal sarcoma.
Chondrosarcoma ; Cohort Studies ; Diagnosis ; Follow-Up Studies ; Humans ; Neoplasm Metastasis ; Osteosarcoma ; Recurrence ; Retrospective Studies ; Sarcoma ; Spine

OBJECTIVES: Uterine sarcoma is a rare malignant tumor, which is usually diagnosed in postmenopausal women. These sarcomas are occasionally misdiagnosed as uterine fibroids, thereby leading to delayed diagnosis in the advanced stages. We analyzed the sonographic and clinical characteristics of unexpected uterine sarcomas detected after surgery in women in the late reproductive age.METHODS: The medical records of 61 patients preoperatively diagnosed with uterine leiomyomas through sonography but confirmed as uterine sarcomas after surgery from January 2005 to December 2018 at Asan Medical Center were retrospectively analyzed. We evaluated the clinical symptoms, sonographic findings, and Doppler indexes, and investigated whether there were any significant characteristics that could clearly differentiate uterine sarcoma from fibroids.RESULTS: The most common clinical finding was increased mass size (15 patients, 24.6%), while 9 patients (14.8%) showed no symptoms. Ultrasonography showed that the maximum diameter of most fibroids was > 5 cm (49 patients, 80.3%), and the average diameter was 75.6 ± 36.3 mm. All the patients showed heterogeneous echogenicity in sonographic imaging. Secondary degeneration of the myomas was reported in 36 patients (59%), and approximately 90% (32/36, 88.9%) showed cystic changes. Of the 40 patients who underwent the evaluation of vascularity, 35 showed increased vascularity of the mass.CONCLUSIONS: In this study, sarcomas misdiagnosed as leiomyomas were usually > 5 cm, and ultrasonography showed heterogeneous echogenicity and irregular cystic degeneration. No definite clinical symptoms were helpful; a thorough evaluation is necessary to rule out uterine sarcomas in women having uterine mass with these characteristics.
Chungcheongnam-do ; Delayed Diagnosis ; Diagnostic Errors ; Female ; Humans ; Leiomyoma ; Medical Records ; Myoma ; Retrospective Studies ; Sarcoma ; Ultrasonography

Sarcomatoid mesothelioma is not very common, mesothelioma is directly attributable to occupational asbestos exposure, with 90% of cases showing a history of exposure. A 66-year-old male was admitted with an abdominal pain that persisted for 3 weeks. He had no abdominal mass. Computed tomography showed soft tissue thickening in perihepatic space and nodularities in omentum and peritoneum with ascites. There was no absolute diagnosis evidence in ascites analysis. Although the pathology of ascites was free for malignancy, the patient underwent omentum biopsy for definitive diagnosis. In laproscopic exploration, there was omental cake, peritoneal nodular seeding. It was suspected cancer carcinomatosis. Immunohistochemical findings suggested that it was sarcomatoid masothelioma. This is the rare case of a peritoneal sarcomatoid mesothelioma, without any exposure to asbestos.
Abdominal Pain ; Aged ; Asbestos ; Ascites ; Biopsy ; Carcinoma ; Diagnosis ; Humans ; Male ; Mesothelioma ; Omentum ; Pathology ; Peritoneum ; Sarcoma

PURPOSE: A dedifferentiated chondrosarcoma is a rare lethal tumor characterized by a low grade chondrosarcoma juxtaposed with a high grade dedifferentiated sarcoma, such as osteosarcoma, fibrosarcoma. The aim of our study was to document the clinical manifestation and oncologic outcomes of a dedifferentiated chondrosarcoma. MATERIALS AND METHODS: This study identified 11 patients who were diagnosed and treated for dedifferentiated chondrosarcoma between January 2007 and December 2016. The identified cohort was then reviewed regarding age, sex, symptom onset, tumor location, magnetic resonance imagings (MRIs), surgical margin, and pathologic diagnosis. The time to local recurrence and/or metastasis, follow-up duration, and the patients' final status were analyzed. RESULTS: The patients were comprised of 7 males and 4 females with a mean age of 54 years (range, 33–80 years). The location of the tumor was in the femur in 6 cases, pelvis in 4 cases, and metatarsal in 1 case. The average tumor diameter was 12.7 cm (range, 6.0–26.1 cm). At the time of diagnosis, 2 patients showed pathologic fracture; 1 patient was Enecking stage IIA, 9 patients were stage IIB, and 1 patient was stage III. Eight patients were classified as a primary dedifferentiated chondrosarcoma and 3 patients were secondary. One of the primary lesions was misinterpreted initially as a low grade chondroid lesion by MRI and underwent curettage. Local recurrence occurred in 8 cases and distant metastasis occurred in 10 cases with a mean duration of 8 months (range, 2–23 months) and 7 months (range, 1–32 months), respectively. The three-year overall survival of patients with dedifferentiated chondrosarcoma was 18%, and 10 patients died due to disease progression. CONCLUSION: Dedifferentiated chondrosarcoma developed lung metastases in the early period of the clinical courses and the prognosis was dismal.
Chondrosarcoma ; Cohort Studies ; Curettage ; Diagnosis ; Disease Progression ; Female ; Femur ; Fibrosarcoma ; Follow-Up Studies ; Fractures, Spontaneous ; Humans ; Lung ; Magnetic Resonance Imaging ; Male ; Metatarsal Bones ; Neoplasm Metastasis ; Osteosarcoma ; Pathology ; Pelvis ; Prognosis ; Recurrence ; Sarcoma