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 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

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

PURPOSE: Clear cell chondrosarcoma may have a benign appearance even on a magnetic resonance imaging (MRI). Hence, it can be confused with benign bone tumors, such as a giant cell tumor or chondroblastoma. The aim of our study was to document the doctorassociated diagnostic errors in patients with clear cell chondrosarcoma and oncologic outcomes of these lesions, which were misdiagnosed as benign bone tumors. MATERIALS AND METHODS: We identified 10 patients who were diagnosed with and treated for clear cell chondrosarcoma between January 1996 and December 2014. One patient was excluded due to insufficient clinical data. We then reviewed their data regarding age, gender, symptom onset, tumor location, initial imaging diagnosis, and associated previous treatment. We examined the errors of surgeons and pathologists with respect to patient and tumor characteristics. We also analyzed treatment delay, time to local recurrence, metastasis, follow-up duration, and the oncologic outcome. RESULTS: The initial presumptive diagnosis based on MRIs for all 9 patients was benign bone tumor. Among 8 patients who underwent inappropriate procedure, half of them were diagnosed as clear cell chondrosarcoma immediately after the curettage. As for the remaining 4 patients, the surgeon did not send any tissue samples to a pathologist for a definite diagnosis in three patients and a pathologist made an incorrect diagnosis in one patient. We performed an appropriate surgery on all patients with a wide surgical margin. The average treatment delay was 27 months (range, 0–127 months), and the average follow-up duration was 65 months (range, 13–164 months). One patient had local recurrence after 12 months. Metastatic disease developed in 2 patients with a median time to definitive treatment of 24 months (12–37 months). Ten-year overall survival of patients with clear cell chondrosarcoma was 78%, and two patients died due to disease progression. CONCLUSION: Misdiagnosis of clear cell chondrosacroma as a benign bone tumor is not uncommon, even for experienced orthopaedic oncologists, resulting in definite curative surgery without biopsy. An inappropriate primary treatment may increase the risk of local recurrence and metastasis. Therefore, a proper subsequent surgery is mandatory for patients with clear cell chondrosarcoma who received inadvertent curettage.
Biopsy ; Chondroblastoma ; Chondrosarcoma ; Curettage ; Diagnosis ; Diagnostic Errors ; Disease Progression ; Follow-Up Studies ; Giant Cell Tumors ; Humans ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Recurrence ; Surgeons

OBJECTIVETo study the clinicopathologic features, diagnosis and differential diagnosis of extraskeletal myxoid chondrosarcoma (EMC).

METHODSThe clinical and pathologic features of 7 cases of EMC encountered in Fujian Provincal Hospital and Fuzhou General Hospital of Nanjing Military Command during the period of 2005 to 2015 were analyzed. Immunohistochemical study and PAS staining were carried out. Relevant literature was reviewed.

RESULTSThe male-to-female ratio was 6 to 1. The age of patients ranged from 21 to 50 years (median = 36 years). The maximum tumor dimension ranged from 2.5 to 15.0 cm (mean = 8.4 cm). The sites of involvement included left neck, right shoulder, left thigh, right thigh, right upper arm and abdomen. Most patients presented with painless lumps. Histologically, all cases showed similar features. Low-power examination showed a nodular or lobulated architecture, with intervening fibrous septa and myxoid matrix in the background. The tumor cells were arranged in cords or tufted clusters. They were spindly to epithelioid / rhabdoid (plasmacytoid) in shape, with eosinophilic to sometimes vacuolated cytoplasm. Intracytoplasmic eosinophilic inclusion bodies and coagulative necrosis were focally seen. Mitotic figures were rare (less than 2 per 10 high-power fields). Immunohistochemical study showed that the tumor cells were positive for vimentin (7/7) and INI1 (7/7). They were focally positive for CKpan (2/7), p63 (3/7), CD99 (3/7), S-100 protein (1/7) and synaptophysin (2/7). Ki-67 proliferation index ranged from 10% to 40%. The tumor cells were negative for α-smooth muscle actin, desmin, myoD1, CD34 and CD117. The cytoplasm of the tumor cells was positive for PAS. EWSR1 gene signal was detected in 5 cases.

CONCLUSIONSEMC is a rare malignant mesenchymal tumor. Arrival at correct diagnosis relies on morphologic examination and immunohistochemistry. Molecular pathology is helpful when necessary. The primary treatment modality for EMC is complete surgical excision and the prognosis is satisfactory.

Adult ; Chondrosarcoma ; diagnosis ; pathology ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Neoplasms, Connective and Soft Tissue ; diagnosis ; pathology ; S100 Proteins ; Synaptophysin ; Vimentin ; Young Adult

Chondrosarcoma is a malignant tumor that originates from cartilaginous cells and is characterized by cartilage formation. Only 5% to 10% of chondrosarcoma occurs in the head and neck area, and it is uncommon in the temporomandibular joint area. This report describes an unusual case with a rare, large chondrosarcoma in a 47-year-old woman who presented with painless swelling and trismus. Computed tomography showed a large mass approximately 8.5×6.0 cm in size arising adjacent to the lateral pterygoid plate and condyle. There were features suggestive of bone resorption. The tumor was resected in a single block with perilesional tissues, and a great auricular nerve graft was performed because of facial nerve sacrifice. Microscopic examination of sections stained with H&E revealed chondrocytes with irregular nuclei and heterogeneous hyper chromatic tumor cells embedded in the chondrocyte lacuna. The diagnosis was a grade I chondrosarcoma. There was no evidence of recurrence at the 8-month follow-up, and a reconstruction surgery with fibular osteocutaneous free flap was performed. We report this unusual entity and a review of the literature.
Bone Resorption ; Cartilage ; Chondrocytes ; Chondrosarcoma* ; Diagnosis ; Facial Nerve ; Female ; Follow-Up Studies ; Free Tissue Flaps ; Head ; Humans ; Middle Aged ; Neck ; Osteosarcoma ; Recurrence ; Temporomandibular Joint* ; Transplants ; Trismus

PURPOSE: It is generally accepted that bony reconstruction after type III (pubic) internal pelvectomy is not necessary. However, technical problems in type III resection, functional outcome according to the extent of resection, and the usefulness of synthetic material to decrease the risk of hernia has not been well addressed. MATERIALS AND METHODS: Fifteen patients who underwent type III internal pelvectomy were extracted and the pathologic diagnosis, Enneking's stage, location of tumor and size, operation time, amount of transfusion, surgical margin, local recurrence, distant metastasis, and functional outcomes were analyzed according to the extent of resection. RESULTS: Pathologic diagnosis was chondrosarcoma in 9, Ewing's sarcoma in 3, metastatic carcinoma in 2, and osteosarcoma in 1 patient. There were 4 patients with local recurrence and one with concomitant lung metastasis. Average Musculoskeletal Tumor Society functional score was 26.7. According to the extent of resection, functional score of 7 cases with unilateral both rami (6) or ischium (1) resection was 26, 4 cases with unilateral both rami and partial contralateral pubic ramus resection was 25, and 4 cases with unilateral both rami including ischium was 24. Two patients had tumor related complication. One patient with a huge intrapelvic tumor aroused at the symphysis pubis showed urethral invasion at presentation, therefore, urethral resection and permanent suprapubic cystostomy was inevitable. The other patient with bilateral pubic ramus involvement by tumor showed caudal displacement of the uterus after pregnancy (4 years after primary resection). She underwent Caesearian section for delivery. CONCLUSION: Regardless of the extent of pubic bone resection, functional outcome was similar. The risk of abdominal or pelvic organ hernia was minimal even without the use of artificial material for soft tissue reconstruction; however, when the extent of resection crosses the symphysis pubis, selective application of an additional procedure to reinforce the pelvic floor may be considered.
Chondrosarcoma ; Cystostomy ; Diagnosis ; Hemipelvectomy* ; Hernia ; Humans ; Ischium ; Lung ; Neoplasm Metastasis ; Osteosarcoma ; Pelvic Floor ; Pelvic Neoplasms ; Pelvis* ; Pregnancy ; Pubic Bone ; Recurrence ; Sarcoma, Ewing ; Treatment Outcome ; Uterus

Arrested pneumatization of the sphenoid sinus is a developmental variant that is not always well recognized and is often confused with other pathologies associated with the skull base. This report describes the case of a patient referred for cone-beam computed tomography (CBCT) imaging for dental implant therapy. CBCT demonstrated a well-defined incidental lesion in the left sphenoid sinus with soft tissue-like density and sclerotic borders with internal curvilinear opacifications. The differential diagnoses included intraosseous lipoma, arrested pneumatization of the sphenoid sinus, chondrosarcoma, chondroid chordoma, and ossifying fibroma. The radiographic diagnosis of arrested pneumatization was based on the location of the lesion, its well-defined nature, the presence of internal opacifications, and lack of expansion. Gray-scale CBCT imaging of the area demonstrated values similar to fatty tissue. This case highlighted the fact that benign developmental variants associated with the skull base share similar radiographic features with more serious pathological entities.
Adipose Tissue ; Chondrosarcoma ; Chordoma ; Cone-Beam Computed Tomography ; Dental Implants ; Diagnosis ; Diagnosis, Differential ; Fibroma, Ossifying ; Humans ; Lipoma ; Pathology ; Skull Base* ; Sphenoid Sinus*

Small round cell tumors (SRCTs) are a heterogeneous group of neoplasms composed of small, primitive, and undifferentiated cells sharing similar histology under light microscopy. SRCTs include Ewing sarcoma/peripheral neuroectodermal tumor family tumors, neuroblastoma, desmoplastic SRCT, rhabdomyosarcoma, poorly differentiated round cell synovial sarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, small cell malignant peripheral nerve sheath tumor, and small cell schwannoma. Non-Hodgkin\'s malignant lymphoma, myeloid sarcoma, malignant melanoma, and gastrointestinal stromal tumor may also present as SRCT. The current shift towards immunohistochemistry and cytogenetic molecular techniques for SRCT may be inappropriate because of antigenic overlapping or inconclusive molecular results due to the lack of differentiation of primitive cells and unavailable genetic service or limited moleculocytogenetic experience. Although usage has declined, electron microscopy (EM) remains very useful and shows salient features for the diagnosis of SRCTs. Although EM is not always required, it provides reliability and validity in the diagnosis of SRCT. Here, the ultrastructural characteristics of SRCTs are reviewed and we suggest that EM would be utilized as one of the reliable modalities for the diagnosis of undifferentiated and poorly differentiated SRCTs.
Chondrosarcoma, Mesenchymal ; Cytogenetics ; Diagnosis ; Gastrointestinal Stromal Tumors ; Genetic Services ; Humans ; Immunohistochemistry ; Lymphoma ; Melanoma ; Microscopy ; Microscopy, Electron ; Microscopy, Electron, Transmission* ; Neurilemmoma ; Neuroblastoma ; Neuroectodermal Tumors ; Osteosarcoma ; Pathology ; Peripheral Nerves ; Reproducibility of Results ; Rhabdomyosarcoma ; Sarcoma, Myeloid ; Sarcoma, Synovial