Primary Mesenchymal Chondrosarcoma Research Articles

Primary orbital mesenchymal chondrosarcoma: clinicopathological characteristics and prognostic prediction

PurposeThis study aimed to analyze the clinicopathological characteristics of a rare malignant tumour, primary orbital mesenchymal chondrosarcoma (MCS), and identify the risk factors influencing its prognosis. MethodsA total of 15 patients with histologically confirmed primary orbital MCS were enrolled between April 2013 and October 2022. The relationships between clinicopathological features and outcomes, including recurrence and survival were analyzed. ResultsDuring the follow-up period, 9 patients (60%) experienced disease relapse, and 7 patients (46.7%) succumbed to the disease. Immunohistochemical analysis showed positive rates for SMA, CD-99, S-100, vimentin, and Bcl-2 at 60%, 60%, 53.3%, 100%, and 93.3%, respectively. The Ki67 index was positively associated with the recurrence and mortality rates. ConclusionOur study suggested that the Ki67 index could be a vital prognostic factor for MCS. Further follow-up studies are required to assess the efficacy of chemotherapy and radiotherapy in improving patient outcomes.

Multimodal therapy in the management of primary orbital mesenchymal chondrosarcoma.

To evaluate the ophthalmic manifestations, radiographic features, and prognosis of Chinese patients with primary orbital mesenchymal chondrosarcoma (MCS). The study included 6 cases with primary orbital MCS treated at Tianjin Eye Hospital from January 2009 to December 2019. Patitent ophthalmic manifestations, radiographic features, diagnosis, pathology, therapeutic regimens, and prognosis were retrospectively reviewed. Six patitents with primary orbital MCS were identified. The mean age at the first visit was 33y (range, 25-42y). All six patients displayed manifestations of exophthalmos, diplopia, limitation of eye displacement, upper eyelid oedema, decreased visual acuity and ptosis. The mean disease history and range were 5 and 2-8mo, respectively. The tumors were located in the superonasal extraconal compartment (2/6, 33.3%), intraconal compartment (2/6, 33.3%), and bitemporal extraconal compartment (2/6, 33.3%), respectively. Radiographic features were a well-defined, orbital mass with calcification and ossification on computed tomography (CT), and marked heterogenous enhancement on dynamic magnetic resonance imaging (MRI). Five patients were treated with tumor resection and one patient received orbital exenteration. Five patients in the cohort received postoperative radiation therapy, two patients received chemotherapy, and one patient did not receive postoperative adjuvant therapy because he refused. The histopathologic classification revealed a tumour composed of a mixture of mature chondroid tissue surrounded by small, round, and undifferentiated mesenchymal cells. Immunohistochemistry revealed Bcl-2, vimetin, CD99, and S-100 were expressed were expressed. After surgeries, two patients have developed a local recurrence. The median recurrence time of 58mo (52-64mo). One patient had distant recurrence included the lungs occurred 52mo after the initial surgery. The possibilty of orbital MCS need to be considered when a painless, slowly growing orbital mass with calcification and ossification. From our experience, trimodality treatment of radiation therapy, chemotherapy and surgery maybe the best option. Orbital MCS has a high tendency for late recurrence, regular long-term follow-up after complete excision is mandatory.

Rare Primary Spinal Mesenchymal Chondrosarcoma: A Case Report

Primary extradural mesenchymal chondrosarcoma (MCS) is a very rare intraspinal tumor. Proper clinical evaluation with magnetic resonance imaging (MRI) followed by biopsy of the mass can confirm the diagnosis. Since MCS has a high tendency of recurrence and metastasis, the prognosis is guarded. We report a 52 years old male with primary extradural MCS in the lumbosacral region (L5-S3). Subtotal excision and biopsy were performed which confirmed the diagnosis. The patient received post excisional radiotherapy but expired after four months. Though rare, this tumor should be kept in differentials which helps in early diagnosis and thus can be treated with radical excision of the mass along with postoperative radiotherapy.

Rare Primary Spinal Mesenchymal Chondrosarcoma: A Case Report

Primary extradural mesenchymal chondrosarcoma (MCS) is a very rare intraspinal tumor. Proper clinical evaluation with magnetic resonance imaging (MRI) followed by biopsy of the mass can confirm the diagnosis. Since MCS has a high tendency of recurrence and metastasis, the prognosis is guarded. We report a 52 years old male with primary extradural MCS in the lumbosacral region (L5-S3). Subtotal excision and biopsy were performed which confirmed the diagnosis. The patient received post excisional radiotherapy but expired after four months. Though rare, this tumor should be kept in differentials which helps in early diagnosis and thus can be treated with radical excision of the mass along with postoperative radiotherapy.

Mesenchymal chondrosarcoma: imaging features and clinical findings.

To describe imaging and clinical features of primary mesenchymal chondrosarcoma (MCS) and evaluate for presence of a distinct biphasic pattern on imaging. Patients with a pathologic diagnosis of MCS were identified along with imaging of their primary tumor. Size, location, appearance (lytic, sclerotic, or mixed), presence, extent and distribution of calcifications, cortical destruction, soft tissue extension, periosteal reaction, contrast enhancement, and radiotracer uptake were recorded. The presence of T2-hyperintense tumor lobules on MRI and a biphasic morphology (distinct calcified and non-calcified components) on CT were assessed. Presence and location of metastases were documented. Twenty-three patients (mean age 28.0 ± 13.8years) were reviewed (13 skeletal, 10 extraskeletal). Overall mean tumor size was 10.2 ± 7.2cm, 7.1 ± 7.3cm in non-metastatic and 13.2 ± 5.9cm (p = 0.004) in metastatic cases. Locations were extremities (n = 11), head/neck (n = 4), chest wall (n = 4), pelvis (n = 3), and retroperitoneum (n = 1). Skeletal MCS were aggressive mixed lytic and sclerotic (n = 8), purely lytic (n = 4), or juxtacortical (n = 1) lesions with cortical destruction and soft tissue extension. Chondroid calcifications were common (80%). On MRI, the presence of T2-hyperintense lobules was seen in 35%. A biphasic morphology on imaging was seen in 30%. Metastases were common (52%) with the most common site being the lungs (75%). All tumors were hypermetabolic with a mean SUVmax of 14.3 (5.6-34) on PET/CT. Skeletal MCS commonly present as aggressive lytic bone lesions with chondroid calcifications. A biphasic morphology was seen in one-third of cases. Metastases were common at initial presentation and more commonly seen with larger tumors.

Primary mesenchymal chondrosarcoma of the kidney without HEY1-NCOA2 and IRF2BP2-CDX1 fusion: A case report and review.

Mesenchymal chondrosarcoma (MC) of the kidney is rare. To the best of our knowledge, the current report is the first case of a giant extraskeletal MC that arose primarily from the right kidney and mimicked renal cell carcinoma at the locally advanced stage (cT3bN0) with vena cava thrombus and multiple pulmonary arterial tumor emboli. Additionally, the literature on renal EMC is reviewed and the possibilities of oncogenic heterogeneity are discussed. A 64-year-old woman was admitted to Yamagata University Hospital for sudden onset of asymptomatic gross hematuria. CT revealed a 90 mm renal mass without calcification in the right kidney and tumor thrombus extending to the inferior vena cava. Radical nephrectomy with thrombectomy was performed. Lung metastasis was detected 2 months later. The patient received systemic chemotherapy, which was only marginally effective. She died of the malignancy 8 months after surgery. Microscopic examination of the tumor revealed typical histology of MC and a lack of HEY1-NCOA2 and IRF2BP2-CDX1 gene fusions in the tumor tissues. Not all MC patients may exhibit chromosomal alterations in the tumor, suggesting the presence of genetically heterogeneous pathways of MC oncogenesis. Further studies are required to confirm the present findings and reinforce the molecular diagnosis of MC.

Primary intradural extramedullary spinal mesenchymal chondrosarcoma: case report and literature review

BackgroundMesenchymal chondrosarcoma (MCS) is a rare malignant variant of chondrosarcoma with a high tendency of recurrence and metastasis. Intradural extramedullary spinal MCS is exceedingly rare and usually found in pediatric patients. Herein, we present an elderly patient with primary intradural extramedullary spinal MCS. Relevant literatures are reviewed to disclose characteristics of intradural extramedullary spinal MCS.Case presentationA 64-year-old female presented with urinary difficulty and tightness of upper back preceding progressive weakness of right lower extremity. Magnetic resonance imaging revealed an intradural extramedullary tumor at the level of 3rd thoracic vertebra. This patient underwent total tumor resection and then received adjuvant radiotherapy. Histopathological examination showed that the tumor composed of spindle and round cells with high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix. Along with immunohistochemical findings and the existence of HEY1-NCOA2 fusion transcript, the diagnosis of MCS was confirmed. Neurologic deficit recovered nearly completely after surgery. No evidence of local recurrence or distant metastasis was found 5 years after treatments. Including the current case, a total of 18 cases have been reported in the literature with only one case with local recurrence and one case of mortality. The current case was the eldest patient diagnosed with primary intraspinal MCS in the literature.ConclusionsMCS rarely appears in the intradural space of the spine. In contrast to classic MCS, treatment outcome of primary intradural extramedullary spinal MCS is usually excellent as total tumor resection is commonly achievable. Adjuvant radiotherapy may reduce local recurrence and chemotherapy may be associated with fewer recurrences especially for unresectable tumors.

The Differences Between Intracranial Mesenchymal Chondrosarcoma and Conventional Chondrosarcoma in Clinical Features and Outcomes

To report differences in clinical features and outcomes between intracranial mesenchymal chondrosarcoma (MCS) and conventional chondrosarcoma (CCS). Clinical data of patients with primary intracranial MCS and CCS were retrospectively extracted and analyzed to compare differences between MCS and CCS. Seventy-four patients with intracranial chondrosarcoma (61 cases with MCS and 13 cases with CCS) were included. Compared with patients with CCS, patients with MCS presented at a younger mean age (21.1 years vs. 34.5 years, P < 0.001) and had a poor mean preoperative Karnofsky performance scale score (64.6 vs. 77.8, P= 0.014). Compared with CCS, MCS was less often located in the skull base (38.5% vs. 96.7%, P < 0.001) and had a larger tumor volume (87.8 cm3 vs. 26.7 cm3, P < 0.001). Rates of gross total resection in MCS and CCS subgroups were 41.1% (n= 25) and 46.2% (n= 6), respectively; rates of adjuvant radiotherapy postoperatively were 44.2% (n= 27) and 46.2% (n= 6), respectively. After mean follow-up of 41.7 months, 1-year, 3-year, and 5-year overall survival and progression-free survival of MCS were significantly shorter than overall survival and progression-free survival of CCS. Multivariate analysis revealed that tumor pathology and extent of surgery were independent predictors for tumor recurrence. Clinical features of MCS are quite different from CCS. Treatment strategies used for CCS do not yield satisfactory outcomes for MCS. Treatment of MCS should be aggressive and individualized.

Imaging Findings of Retroperitoneal Primary Extraskeletal Mesenchymal Chondrosarcoma: A Case Report

: Imaging knowledge regarding retroperitoneal primary extraskeletal mesenchymal chondrosarcoma (ESMC) is limited. We report a new case of ESMC originated from the retroperitoneum. The tumor was indicated a well-defined mass with marker calcification, the parenchyma showed gradual enhancement pattern on the biphasic enhanced computed tomography and wash-in-wash-out enhancement pattern on the contrast-enhanced magnetic resonance imaging and hyperintensity on diffusion-weighted imaging which implying the dense cellularity of tumor cells. Sonography indicated an increased echogenicity nodular area in the mass. These imaging features further enriched the imaging knowledge of this rare tumor and presented valuable imaging characteristics to diagnose retroperitoneal primary ESMC.

Primary mesenchymal chondrosarcoma of the orbit: Histopathological report of 3 pediatric cases

Mesenchymal chondrosarcoma (MCS) is an unusual tumor mainly found in the skeleton. Around third of the cases occur in extra-skeletal sites with the orbit being the third most common site in these cases. In previous reviews of the orbital cases, it has been concluded that orbital MCS tends to occur in women in the second or third decades of life. However, 8 cases of orbital MCS have been reported so far in the pediatric age group (age less than 18 years-old) one of which has been considered congenital MCS in a 5-days old newborn girl. We describe 3 additional pediatric cases with primary orbital MCS and they were all males. Our cases presented with proptosis and calcific orbital masses on imaging studies. Histopathological examination of the excised masses shared the typical presence of undifferentiated mesenchymal cells and immature areas of cartilage. The diagnosis of MCS was further confirmed by immunohistochemical staining. Brief review of the literature in relation to this diagnosis in the orbit is also presented.

Primary ovarian mesenchymal chondrosarcoma: report of a case

Primary ovarian mesenchymal chondrosarcoma: report of a case

Pancreatic involvement by mesenchymal chondrosarcoma harboring the HEY1-NCOA2 gene fusion

Mesenchymal chondrosarcoma (MC) is an aggressive small, round, blue cell tumor with chondrogenic differentiation that typically arises in bony sites. Approximately, a third of these tumors develop in extraskeletal sites such as the meninges, and somatic soft tissue. The MCs are well-circumscribed, lobulated masses, with focal calcification. Histologically, 2 distinct populations of neoplastic cells characterize MC: sheets of primitive small, round, blue cells surrounding islands of well-developed hyaline cartilage with mature chondrocytes in lacunae. Involvement of the gastrointestinal tract and pancreas by primary or metastatic MC is a relatively rare occurrence. We identified 8 patients with MC in our departmental archives from 1990 to 2015, two of which had pancreatic involvement. The patients were young women who developed masses in the distal pancreas. Molecular testing demonstrated that both tumors harbored the recently described HEY1-NCOA2 gene fusion. These cases illustrate that pancreatic involvement can occur in MC, and the demonstration of HEY1-NCOA2 fusion can be helpful to confirm the diagnosis.

Primary mesenchymal chondrosarcoma with bilateral kidney invasion and calcification in renal pelvis: A case report and review of the literature.

Mesenchymal chondrosarcoma (MC) is a rare malignant cartilaginous forming tumor. MC of the kidney is extremely rare, with only seven cases reported in the literature. The present study described the case of a 17-year-old male, who presented with sudden severe pain in the right flank and a high fever. Imaging studies demonstrated a large soft heterogeneous mass (7.8×9.5×15 cm) located between the liver and right kidney with no clear demarcation, and a well-demarcated mass (1.3×2.4 cm) with patchy dense calcification occupying the left renal pelvis. Following the diagnosis of a Wilms' tumor, the patient underwent a right radical nephrectomy and the pathological diagnosis was MC of the kidney. To the best of our knowledge, the current study presents the first case of MC with bilateral kidney invasion and calcification in the renal pelvis. In addition, the clinical, radiological and pathological features, and the management of this unusual neoplasm were discussed.

Intracranial Mesenchymal Chondrosarcoma: A Case Report

Primary intracranial mesenchymal chondrosarcoma is an extremely rare tumor. We present a case of primary intracranial mesenchymal chondrosarcoma in a young female who presented with headache, loss of vision, and one episode of convulsions associated with nausea and vomiting. MRI brain revealed a large, well-marginated, lobulated, partially calcified, intensely enhancing lesion with a broad dural base in bilateral frontal regions with marked mass effect suggestive of meningioma. Bilateral frontal craniotomy with gross tumor excision was performed. Histopathology revealed mesenchymal chondrosarcoma. Adjuvant radiotherapy was delivered and patient is currently on follow-up. We emphasize that intracranial mesenchymal chondrosarcoma is a rare neoplasm and should be considered in the differential diagnoses of intracranial mass along with meningioma. Treatment of choice is surgery followed by adjuvant radiotherapy. We review the relevant literature and discuss the characteristics of this rare tumor.

Primary Renal Mesenchymal Chondrosarcoma: A Case Report

A 16-year-old female patient presented to our emergency department with a left upper extremity radiculopathy prompting several imaging studies. Magnetic resonance imaging of the neck revealed multiple cervical vertebrae lesions and computed tomography imaging demonstrated a 15-cm calcified mass originating from the left kidney and extending into the surrounding structures. Pathologic assessment of the open left radical nephrectomy specimen revealed a primary renal mesenchymal chondrosarcoma, a tumor that has only been documented in 5 prior case reports in the literature.

Sarcomatous Transformation of Osteochondroma of the Coronoid Process Forming Pseudoarthrosis With Zygomatic Arch Mistaken for Jacob Disease

Osteochondromas are the most common benign osseous neoplasms, with a propensity to involve long bones. The involvement of the coronoid process is extremely rare, and osteochondromas of the coronoid process may form pseudoarticulations with the zygomatic arch when large, an entity that is extremely rare and termed as Jacob disease. Sarcomatous degeneration of osteochondroma of the coronoid process is an extremely rare entity and has not been described to date. Only 1 case of primary mesenchymal chondrosarcoma has been described. We present a case of a 24-year-old man with a chondrosarcoma of the coronoid process, who was wrongly diagnosed with Jacob disease on the basis of radiologic findings.

Primary Extraskeletal Mesenchymal Chondrosarcoma of the Anterior Mediastinum

Primary extraskeletal mesenchymal chondrosarcoma (EMC) of the mediastinum is an extremely rare disease. To date, only seven cases have been reported in the literature 1-4 since it was first reported by Chetty in 1990.1 We experienced a case of primary mediastinal EMC presenting as an anterior mediastinal mass, a previously unreported site. Here, we describe the characteristic features of the lesion with a review of the literature to emphasize that EMC should be considered in the differential diagnosis of mediastinal tumors.

Giant Primary Mesenchymal Chondrosarcoma of the Lung: Case Report and Review of Literature

Mesenchymal chondrosarcoma, a rare malignant tumor, was predominantly occurring in the bone and may involve somatic soft tissue but it is extremely rare in the lung.We report the case of a 20-year-old female who presented with a 2-month history of irritant nonproductive cough and chest pain. The histopathologic examination revealed the tumor composed of atypical undifferentiated small cells and islands of matured chondroid matrix typically presented as bimorphic appearances. Immunohistochemical examination revealed that the tumor cells were positive for vimentin and CD99 for all components, and to S-100 limited to the areas of cartilage. In addition, previously reported cases of primary lung mesenchymal chondrosarcoma were reviewed, and the relevant clinical knowledge regarding its clinical manifestations, diagnosis, and treatment were discussed.

Primary mesenchymal chondrosarcoma of the kidney with synchronous implant and infiltrating urothelial carcinoma of the ureter

Primary mesenchymal chondrosarcoma of the kidney is rare, and it shows distinct undifferentiated tumor cells and well differentiated cartilagenous components. Also assident infiltrating urothelial carcinoma of the ureter is an extremely rare cancer. We report a case of primary mesenchymal chondrosarcoma occurring in the left kidney with an ipsilateral and distinct distal ureteric implant, and a coexisting infiltrating urothelial carcinoma of the ureter in a 64-year-old man. Histopathological examination and immunohistochemical studuies showed the classic features of mesenchymal chondrosarcoma in kidney, as well as a few infiltrating urothelial in ureter. Multitarget fluorescence in situ hybridization (FISH) suggested that the development of the urothelial carcinoma in the ureter may be triggered or induced by the chondrosarcoma component. The patient died 2 month after left nephro-ureterectomy. This is the first reported case of a primary mesenchymal chondrosarcoma of the kidney with coexisting infiltrating urothelial carcinoma of the ureter.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1522835667751019

Primary Orbital Mesenchymal Chondrosarcoma: Case Report and Review of the Literature

Orbital mesenchymal chondrosarcoma is a very uncommon lesion of the bone and extraskeletal tissue. To our knowledge, approximately 30 cases have been described. We present the case of a 52-year-old male who presented with a history of progressive proptosis and chemosis of the right eye caused by an orbital tumor. He underwent exenteration of the right orbit, and the histological examination revealed a mesenchymal orbital chondrosarcoma. This paper attempts to describe a rare entity that should be considered in the differential diagnosis of calcified orbital lesions, especially in young adults. Complete removal of the tumor is the mainstay of treatment, but adjuvant radiation therapy and chemotherapy should be considered.