4.Clinical and imaging features of eight cases of Ewing sarcoma of the jaw.
Yinglian FENG ; Tiemei WANG ; Zitong LIN ; Lei ZHANG ; Xiaofeng HUANG ; Guowen SUN ; Shu XIA
West China Journal of Stomatology 2023;41(2):185-189
OBJECTIVES:
This study investigate the clinical and imaging features of Ewing sarcoma (ES) of the jaw.
METHODS:
Eight cases of pathologically diagnosed ES of the jaw from January 2010 to June 2022 were included in the study. Clinical and radiological features were retrospectively analyzed.
RESULTS:
Among the eight cases, the mean age at onset was 29.4 years, and the male to female ratio was 7∶1. The predilecting site was the posterior part of mandible, accounting for 75% of the cases. The lesions often exhibited early numbness of the lower lip and lymphadenopathy. The main radiographic manifestation of mandibular lesions was ill-defined radiolucency, mixed with fibrous or brush-like tumor matrix, and soft tissue mass. The maxillary ES lesions mainly presented as lytic bone destruction accompanied by adjacent soft tissue mass. Periosteal ossification was rarely seen.
CONCLUSIONS
The clinical and imaging characteristics of ES in the jaw are helpful for its diagnosis.
Male
;
Humans
;
Female
;
Sarcoma, Ewing/pathology*
;
Retrospective Studies
;
Radiography
;
Mandible/pathology*
;
Lip
;
Bone Neoplasms
8.Radiation-associated sarcomas of bone and soft tissue: a clinicopathological analysis of 46 cases.
L H GONG ; W F LIU ; L LI ; X Q SUN ; M ZHANG ; Y DING
Chinese Journal of Pathology 2023;52(10):995-1000
Objective: To investigate the clinical, imaging, histological, and molecular features and the differential diagnosis of radiation-associated sarcomas of bone and soft tissue. Methods: Forty-six cases of radiation-associated sarcomas of the bone and soft tissue in Beijing Jishuitan Hospital from January 2010 to January 2022 were retrospectively analyzed; and the imaging, histological features and immunophenotype were examined. Results: There were 33 females and 13 males, aged from 18 to 74 years, with a mean of 52 years. The most common site of radiation-associated sarcomas were the limbs and spine (15 cases), followed by the chest (9 cases). The primary diseases included epithelial tumors (15 breast cancer, 6 cervical cancer, and 5 bowel cancer), hematolymphoid tumors, bone and soft tissue tumors and infectious lesions. The latent period of radiation-associated sarcomas ranged from 2-22 years, with an average of 11.6 years. Histopathologically, the morphology was divergent from the primary tumor. The most common malignant tumor type was undifferentiated sarcoma (22 cases), followed by osteosarcoma (16 cases). The immunophenotype of radiation-related sarcoma was almost the same as the corresponding soft tissue sarcoma. Conclusions: Radiation-induced sarcoma has a wide range of primary tumor types and its imaging, morphology and immunohistochemical features are similar to those of the primary sarcoma of bone and soft tissue. Clinical correlation is often recommended for the differential diagnosis.
Male
;
Female
;
Humans
;
Retrospective Studies
;
Sarcoma/pathology*
;
Osteosarcoma/diagnostic imaging*
;
Soft Tissue Neoplasms/pathology*
;
Bone Neoplasms/pathology*
9.CIC-rearranged sarcoma: a clinicopathological analysis of six cases.
C L ZHAO ; L C SUN ; J B ZHANG ; Y Y SUN
Chinese Journal of Pathology 2023;52(10):1025-1027
10.Primary synovial sarcoma of lung: a clinicopathological analysis of 12 cases.
X W ZHANG ; J G WEI ; J P SUN ; Z G XU ; Q CHENG ; L ZHANG ; L F KONG
Chinese Journal of Pathology 2023;52(11):1120-1125
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

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