Humans ; Chondrosarcoma, Mesenchymal/pathology* ; Chondrosarcoma/surgery* ; Muscle, Skeletal/pathology* ; Bone Neoplasms/pathology*

Objective: To explore the diagnosis, surgical management and outcome of jugular foramen chondrosarcoma (CSA). Methods: Fifteen patients with jugular foramen CSA hospitalized in the Department of Otorhinolaryngology Head and Neck Surgery of Chinese PLA General Hospital from December 2002 to February 2020 were retrospectively collected,of whom 2 were male and 13 were female, aging from 22 to 61 years old. The clinical symptoms and signs, imaging features, differential diagnosis, surgical approaches, function of facial nerve and cranial nerves IX to XII, and surgical outcomes were analyzed. Results: Patients with jugular foramen CSA mainly presented with facial paralysis, hearing loss, hoarseness, cough, tinnitus and local mass. Computed tomography (CT) and magnetic resonance (MR) could provide important information for diagnosis. CT showed irregular destruction on bone margin of the jugular foramen. MR demonstrated iso or hypointense on T1WI, hyperintense on T2WI and heterogeneous contrast-enhancement. Surgical approaches were chosen upon the sizes and scopes of the tumors. Inferior temporal fossa A approach was adopted in 12 cases, inferior temporal fossa B approach in 2 cases and mastoid combined parotid approach in 1 case. Five patients with facial nerve involved received great auricular nerve graft. The House Brackmann (H-B) grading scale was used to evaluate the facial nerve function. Preoperative facial nerve function ranked grade Ⅴ in 4 cases and grade Ⅵ in 1 case. Postoperative facial nerve function improved to grade Ⅲ in 2 cases and grade Ⅵ in 3 cases. Five patients presented with cranial nerves Ⅸ and Ⅹ palsies. Hoarseness and cough of 2 cases improved after operation, while the other 3 cases did not. All the patients were diagnosed CSA by histopathology and immunohistochemistry, with immunohistochemical staining showing vimentin and S-100 positive, but cytokeratin negative in tumor cells. All patients survived during 28 to 234 months' follow-up. Two patients suffered from tumor recurrence 7 years after surgery and received revision surgery. No complications such as cerebrospinal fluid leakage and intracranial infection occurred after operation. Conclusions: Jugular foramen CSA lacks characteristic symptoms or signs. Imaging is helpful to differential diagnosis. Surgery is the primary treatment of jugular foramen CSA. Patients with facial paralysis should receive surgery in time as to restore the facial nerve. Long-term follow-up is necessary after surgery in case of recurrence.
Humans ; Male ; Female ; Young Adult ; Adult ; Middle Aged ; Facial Paralysis/etiology* ; Diagnosis, Differential ; Jugular Foramina ; Retrospective Studies ; Cough ; Hoarseness ; Neoplasm Recurrence, Local ; Chondrosarcoma/surgery*

Objective:To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone. Methods:Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary. Results:One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months. Conclusion:The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.
Adult ; Female ; Humans ; Male ; Middle Aged ; Chondrosarcoma/surgery* ; Fibrosarcoma ; Neoplasm Recurrence, Local ; Retrospective Studies ; Skull Base/surgery* ; Skull Base Neoplasms/surgery* ; Temporal Bone/pathology* ; Treatment Outcome

Humans ; Chondromatosis, Synovial/pathology* ; Chondrosarcoma/pathology*

Chondrosarcoma/surgery* ; Humans ; Neoplasms, Connective and Soft Tissue ; Soft Tissue Neoplasms

Objective: To investigate the immunohistochemical expression of NKX3.1 and NKX2.2 in mesenchymal chondrosarcoma (MC), and to explore the differential diagnostic value of NKX3.1 and NKX2.2 in MC and other types of small round cell malignant tumors. Methods: A total of 12 cases of MC and 97 other small round cell malignant tumors diagnosed in Jinling Hospital, Nanjing University School of Medicine from 2001 to 2020 were collected for NKX3.1 and NKX2.2 immunohistochemical detection. Among them, two kinds of NKX3.1 antibodies [rabbit polyclonal antibody and rabbit monoclonal antibody (EP356)] were used for detection in 12 cases of MC, and one NKX3.1 antibody (rabbit polyclonal antibody) was detected in 97 cases of other small round cell malignant tumors, and the relevant literature was reviewed. Results: The 12 MC patients included 7 females and 5 males, with a mean age of 33 years (14-54 years). Nine cases were from bone and three from soft tissue. Among the 12 MC patients, 8 patients had postoperative recurrence or metastasis, and 3 of them died of tumor recurrence or metastasis. Histologically, 12 cases of MC showed typical bidirectional differentiation.The positive rate of both NKX3.1 antibodies in MC was 12/12, NKX3.1 was focal weakly positive in only one of 12 chondrosarcomas (grade 3), 5 alveolar rhabdomyosarcomas, 5 embryonal rhabdomyosarcomas, and 5 solitary fibrous tumors, respectively. The remaining 70 cases of other small round cell malignant tumors were negative. The positive rates of NKX2.2 in MC, Ewing sarcoma and olfactory neuroblastoma were 12/12, 15/15 and 4/5, respectively. In 12 cases of chondrosarcoma (grade 3), 5 cases of poorly differentiated synovial sarcoma, 5 cases of alveolar rhabdomyosarcoma, and 5 cases of solitary fibrous tumor, NKX2.2 was focally and weakly positive in only one case, respectively, and all the remaining 50 cases of other small round cell malignant tumors were negative. Conclusions: The expression of NKX3.1 and NKX2.2 proteins are significant indicators in the diagnosis of MC, and the combined detection of NKX3.1 and NKX2.2 can help distinguish MC from most other small round cell malignant tumors.
Biomarkers, Tumor ; Chondrosarcoma, Mesenchymal/diagnosis* ; Diagnosis, Differential ; Female ; Homeodomain Proteins ; Humans ; Immunohistochemistry ; Male ; Nuclear Proteins

OBJECTIVE: To develop and validate radiomics models based on non-enhanced magnetic resonance (MR) imaging for differentiating chondrosarcoma from enchondroma. METHODS: We retrospectively evaluated a total of 68 patients (including 27 with chondrosarcoma and 41 with enchondroma), who were randomly divided into training group (=46) and validation group (=22). Radiomics features were extracted from TWI and TWI-FS sequences of the whole tumor by two radiologists independently and selected by Low Variance, Univariate feature selection, and least absolute shrinkage and selection operator (LASSO). Radiomics models were constructed by multivariate logistic regression analysis based on the features from TWI and TWI-FS sequences. The receiver-operating characteristics (ROC) curve and intraclass correlation coefficient (ICC) analyses of the radiomics models and conventional MR imaging were performed to determine their diagnostic accuracy. RESULTS: The ICC value for interreader agreement of the radiomics features ranged from 0.779 to 0.923, which indicated good agreement. Ten and 11 features were selected from the TWI and TWI-FS sequences to construct radiomics models, respectively. The areas under the curve (AUCs) of TWI and TWI-FS models were 0.990 and 0.925 in training group and 0.915 and 0.855 in the validation group, respectively, showing no significant differences between the two sequence-based models (>0.05). In all the cases, the AUCs of the two radiomics models based on TWI and TWI-FS sequences and conventional MR imaging were 0.955, 0.901 and 0.569, respectively, demonstrating a significantly higher diagnostic accuracy of the two sequence-based radiomics models than conventional MR imaging (<0.01). CONCLUSIONS The radiomics models based on TWI and TWI-FS non-enhanced MR imaging can be used for the differentiation of chondrosarcoma from enchondroma.
Chondroma ; Chondrosarcoma ; Humans ; Magnetic Resonance Imaging ; ROC Curve ; Retrospective Studies

Chondrosarcoma original from the zygomatic arch is a very rare disease with high malignancy. Surgery is the main means of treatment at present for duo to its poor sensitivity to radiochemotherapy. We reported a young patient who was recovery well in a 4-years follow-up without radiochemotherapy after a total resection of the tumor.
Bone Neoplasms ; Chemoradiotherapy ; Chondrosarcoma ; Humans ; Self Concept ; Zygoma

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

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