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

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

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

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

OBJECTIVE: To explore the efficacy of the resection of periacetabular malignant tumors and the reconstruction with modular endoprosthesis.
 METHODS: From August 2006 to December 2012, 22 patients with periacetabular malignant tumors, who received the resection and reconstruction with modular prosthesis, were retrospectively reviewed. There were 11 males and 11 females, and the average age was 44 (16-65) years old. Pathological results showed there were 13 cases of chondrosarcoma, 5 cases of osteosarcoma, 2 cases of Ewing's sarcoma, 1 case of maligant fibrous histiocytoma, and 1 case of giant cell tumor. According to the classification system by Enneking, there were 11 cases of Type II+III resection, 5 cases of Type I+II+III resection, 3 cases of Type I+II resection, and 3 cases of Type II resection.
 RESULTS: All patients were followed up. The average time for follow-up was 49 (11-103) months. At the last time of follow-up, 13 patients (59%) were still alive, 9 patients (41%) died due to their primary disease. Metastasis occurred in 8 patients (36%), and local recurrence occurred in 5 patients (23%). The mean Musculoskeletal Tumor Society (MSTS) score for 13 cases of alive patients at the latest time of follow-up was (18.5±5.7) months. The mean score for 11 patients, whose limb salvage were successful, was 20.7±2.0.
 CONCLUSION Reconstruction with modular prosthesis after wide resection for periacetabular malignant tumor can achieve satisfied outcome.
Acetabulum ; pathology ; surgery ; Adolescent ; Adult ; Aged ; Bone Neoplasms ; mortality ; surgery ; Chondrosarcoma ; mortality ; surgery ; Female ; Giant Cell Tumors ; mortality ; surgery ; Hip Prosthesis ; Histiocytoma, Malignant Fibrous ; mortality ; surgery ; Humans ; Limb Salvage ; Male ; Middle Aged ; Neoplasm Metastasis ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Osteosarcoma ; mortality ; surgery ; Prosthesis Implantation ; statistics & numerical data ; Retrospective Studies ; Sarcoma, Ewing ; mortality ; surgery ; Treatment Outcome

Primary cartilaginous tumors of the laryngeal lesion are rare and it accounts for only 1% of laryngeal tumor pathology. They are most commonly located on the endolaryngeal surface of cricoid cartilage, and it is extremely rare to find them in vocal muscle lesion. In most cases, treatment of choice is based on surgical excision, and postoperative radiotherapy is typically recommended. We experienced a case of a 22-year-old young woman without underlying disease, presenting extraskeletal myxoid chondrosarcoma of vocal cord, especially in the vocal muscle. The patient underwent mass excision through suspension laryngoscope and radiation therapy.
Chondrosarcoma* ; Cricoid Cartilage ; Female ; Humans ; Laryngeal Muscles ; Laryngoscopes ; Larynx* ; Pathology ; Radiotherapy ; Vocal Cords ; Young Adult

Bone Neoplasms ; pathology ; Chondrosarcoma, Mesenchymal ; pathology ; Dura Mater ; pathology ; Humans ; Meninges ; pathology

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