Extraskeletal Mesenchymal Chondrosarcoma Research Articles

Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review.

Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection. A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, "primary intracranial chondrosarcoma", "extraskeletal mesenchymal chondrosarcoma", "mesenchymal chondrosarcoma" and "pediatric". Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described. The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2years (range 2months to 18years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired. IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis. SafeMaximal resection followed by radiotherapy is the preferred treatment strategy.

Primary Intracranial Extraskeletal Mesenchymal Chondrosarcoma of the Brain in a Pediatric Patient: A Case Report and Review of Literature

AbstractIntracranial extraskeletal mesenchymal chondrosarcoma, which is characterized by undifferentiated mesenchymal cells in the presence of occasional pockets of mature hyaline cartilage, is rare in our clinical practice and commonly seen in young adults. In the pediatric population, only a few cases have been reported. In this article, we describe a case of primary recurrent intracranial mesenchymal chondrosarcoma in an 11-year-old boy well treated by surgery, radiation, and chemotherapy. We also reviewed all previously published reports on pediatric patients on the basis of their manifestations and management.

Real-world experience of tyrosine kinase inhibitors in children, adolescents and adults with relapsed or refractory bone tumours: A Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) study.

We conducted a retrospective multi-centre study to assess the real-world outcome of regorafenib (REGO) and cabozantinib (CABO) in recurrent/refractory bone tumours (BTs) including osteosarcoma (OST), Ewing sarcoma (EWS) and chondrosarcoma (CS)/extra-skeletal mesenchymal CS (ESMC). After regulatory approval, data from patients with recurrent BT (11 institutions) were extracted from CanSaRCC (Canadian Sarcoma Research and Clinical Collaboration) database. Patient characteristics, treatment and outcomes were collected. Progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method. From July 2018 to May 2022, 66 patients received REGO or CABO; 39 OST, 18 EWS, 4 CS and 5 ESMC. Median age was 27.8 years (range 12-76); median starting dose was 60 mg for CABO (n = 37, range 40-60) and 120 mg for REGO (n = 29, range 40-160). Twenty-eight (42.4%) patients required dose reduction: hand-foot syndrome 7 (10.6%), nausea/vomiting 1 (1.5%), diarrhoea 1 (1.5%), 2 elevated LFTs (3%), elevated bilirubin 1 (1.5%) and mucositis 1 (1.5%). The median OS for patients with OST, EWS, CS and ESMC was 8.5 months (n = 39, 95% CI 7-13.1); 13.4 months (n = 18, 95% CI 3.4-27.2), 8.1 (n = 4, 95% CI 4.1-9.3) and 18.2 (n = 5, 95% CI (10.4-na), respectively. Median PFS for OST, EWS, CS and ECMS was 3.5 (n = 39, 95% CI 2.8-5), 3.9 (n = 18, 95% CI 2.1-5.9), 5.53 (n = 4. 95% CI 2.13-NA) and 11.4 (n = 5, 95% CI 1.83-14.7), respectively. Age, line of therapy, REGO versus CABO, or time from diagnosis to initiation of TKI were not associated with PFS on univariable analysis. Our real-world data show that TKIs have meaningful activity in recurrent BT with acceptable toxicities when started at modified dosing. Inclusion of TKIs in earlier lines of treatment and/or maintenance therapy could be questions for future research.

Extraskeletal mesenchymal chondrosarcoma of the abdomen: A rare clinical presentation

Extraskeletal mesenchymal chondrosarcoma (EMCS) is a rare malignant soft tissue tumor of chondroprogenitor cell origin. Originally, it was restricted to the bone only but that is no longer the case. Recent literature reports that 20–33% of these tumors occur at the extraskeletal sites. We report one such case, in which the tumor involved the anterior abdominal wall muscles and also had a large intra- abdominal mass that covered a large part of the peritoneal cavity. The clinical features and computed tomography findings suggested the diagnosis of a malignant desmoid tumor with intra-abdominal extension; however, the histopathological examination and the immunohistochemistry proved the tumor to be EMCS. The case is reported due to the dilemma in diagnosis, its rarity, large size, parietal, and intra-abdominal extension with multiple site involvement.

Chondrosarcoma: an analysis of clinical characteristics, treatment outcomes, and prognostic factors in a decade-long single center experience

Aim: Management of chondrosarcoma patients is associated with histologic subtypes and clinical behaviors. This study describes our institution's management and outcome experience for 12 chondrosarcoma patients with higher grades in rarer locations. Materials and Methods: Pathologically diagnosed chondrosarcoma patients between January 2013 and January 2023 were retrospectively scanned. Patients under 18 years, duplicate records, second primary malignancy, index surgery at another hospital were excluded, and data of remaining 12 patients were statistically analyzed. Results: Twelve patients (6 female, 6 male) had median age of 62 (21-94) years. Locations of primary tumors were femur (n=4), larynx (n=1), left gluteus (n=1), lung (n=1), periscapular soft tissue (n=1), tarsal bone (n=1), distal metacarps (n=1), scapula (n=1), and sternum (n=1). There were local relapses in three cases (chest wall, ipsilateral femur, acetabulum, and ipsilateral gluteal region) and distant metastasis in three cases (contralateral lung, lung, bilateral lung, and pleura) during the follow-up. The median tumor size was 5.65cm (1.5-21.5cm). Histologic subtypes were Chondrosarcoma (Grade I) (n=4), Chondrosarcoma (Grade II) (n=1), Chondrosarcoma (Grade III) (n=2), Dedifferentiated Chondrosarcoma (Grade III) (n=2), Extraskeletal Mesenchymal Chondrosarcoma (n=3). Surgical treatment was eight wide resections, one lobectomy, one laryngectomy, one amputation, and one curettage. Five patients received chemotherapy (2 neoadjuvant chemotherapy, 3 adjuvant chemotherapy), two patients received low dose (50.4Gy) (3DCRT; IMRT), and two patients received high dose (>60Gy) (IMRT) adjuvant radiation therapy. The median follow-up was 26.5 months (2-121 months). The median survival was 26.5 months (2-121 months). Median relapse-free survival was three months (0-96 months). The overall and relapse-free survivals at two years were 81.5% and 50%; at five years, 71.3% and 25%. Conclusion: Our results demonstrate that surgery is the gold standard treatment, but well-managed adjuvant oncologic treatment provides undeniable advantage for overall survival in selected cases.

Long-Term Surgical Outcomes of Two Patients with Intracranial Extraskeletal Mesenchymal Chondrosarcoma and a Brief Literature Review.

Objective Intracranial extraskeletal mesenchymal chondrosarcomas (IEMCs) are malignant aggressive neoplasms. IEMCs originate from the meninges or parenchyma. In the current study, we aimed to figure out the importance of gross total resection (GTR) and adjuvant radiotherapy (RT) by evaluating all reported IEMCs through the literature that included our two patients. Methods and Methods We presented two IEMC patients who were treated at our institutions and followed up for a long duration. To understand the appropriate management for IEMC, we conducted a systematic literature review for previously reported series and cases of IEMCs. Results We surgically treated two young males with IEMC initially diagnosed at their age of 18 and 20 years. The patients were initially treated with GTR and GTR followed by RT, and followed-up for 218 and 73 months, respectively. Through both the patients, we obtained 83 reported IEMC patients from the literature. The mean age of the reported cases was 24.5 ± 16.0 years (2 months–71 years). Female predominance was 54.2%. The mean progression-free and overall survivals were 27.9 and 39.0 months, respectively. The progressiveness rate was 56%. The presence of progressiveness was a poor prognostic factor ( p = 0.0008). GTR was achieved in 53.0% of the patients. There was a significant difference between patients who received GTR compared with those who did not receive GTR ( p = 0.035). Conclusion Regarding their malignancy and progressiveness, we recommended the maximal surgical resection with wide margins followed by RT as appropriate management for IEMCs with close follow-up. The timely treatment provides high life quality and avoids life-threatening complications.

Complete surgical excision of a high‐grade extraskeletal mesenchymal chondrosarcoma, resulting in a longer survival than previously reported

AbstractMesenchymal chondrosarcomas are rare malignant tumours composed of bone and soft tissue. Extraskeletal mesenchymal chondrosarcomas (EMCS) that form in the absence of bone are still rare, accounting for only 1% of chondrosarcomas in dogs. The handful of cases reported have varied survival rates, making it difficult to predict survival accurately. We report on a case of an 11‐year‐old Schnauzer cross with a high‐grade (III) EMCS on the right flank. No metastases were detected at presentation, and complete surgical resection was performed with no adjunct therapy. Despite previous reports implying a poor prognosis, this patient showed no signs of either local recurrence or metastases, 17 months post‐surgical resection, suggesting that despite features of high histologic grade, complete surgical excision may lead to a longer disease‐free interval.

Extraskeletal Mesenchymal Chondrosarcoma of the Uterus.

Mesenchymal chondrosarcoma is an uncommon malignant mesenchymal tumor with an aggressive behavior. Diagnoses of mesenchymal chondrosarcoma are established based on histomorphological, immunohistochemical, and molecular findings. Only one case of extraskeletal mesenchymal chondrosarcoma (EMC) of the uterus has been reported. This article presents the second case of primary uterine EMC, occurring in a 33-year-old woman. We describe the histological and immunophenotypical features of EMC. Our observations will help pathologists and clinicians perform accurate histological diagnoses of uterine EMC and plan appropriate treatment strategies for this rare tumor.

Extraskeletal mesenchymal chondrosarcoma mimicking a thrombosed venous aneurysm with femoral vein involvement: A case report

Abstract Introduction/Objective Mesenchymal chondrosarcoma is a high-grade primitive mesenchymal tumor, which accounts for <5% of all chondrosarcomas and commonly affects young adults (peak incidence in second to third decade of life). The tumor has a widespread anatomic distribution, frequently involving the craniofacial bones as well as extraskeletal sites. The clinical presentation of an inguinal mass mimicking a thrombosed venous aneurysm is unusual and represents a potential diagnostic pitfall. Methods/Case Report A 42-year-old female with hypertension and obesity initially presented with a two-week history of left lower leg swelling. Venous doppler revealed presumed venous thrombosis, and she was prescribed apixaban while no history of coagulopathy, immobility, or recent surgery was noted. Two months later, she had residual swelling. Follow-up CT scan favored a large, peripherally thrombosed venous aneurysm arising from the left common femoral vein, while MRI showed a lobulated, inguinal soft tissue mass abutting the vein. Biopsy of the mass demonstrated a spindle cell mesenchymal neoplasm; subsequent resection revealed a pink-to-tan, well-circumscribed, and encapsulated mass (5.2 cm) with focal left common femoral vein invasion. Microscopically, the lesion demonstrated poorly-differentiated, oval-to-spindle cells with prominent staghorn vasculature interspersed were focal areas of well-differentiated hyaline cartilage. Immunohistochemical stains showed that the lesional cells were negative for cytokeratin cocktail, EMA, SMA, desmin, S100 protein, SOX10, STAT6, MUC4, MDM2, and ER. Next-generation sequencing (NGS) revealed a HEY1-NCOA2 gene fusion, confirming the diagnosis of extraskeletal mesenchymal chondrosarcoma. Results (if a Case Study enter NA) N/A Conclusion Extraskeletal mesenchymal chondrosarcoma can rarely present as an inguinal soft tissue mass with vascular invasion, mimicking a thrombosed venous aneurysm. Molecular confirmation of HEY1-NCOA2 gene fusion can help confirm the diagnosis in unusual clinical presentations.

Extraskeletal mesenchymal chondrosarcoma in central nerve system: a clinicopathological analysis

Objective: To investigate the clinicopathological features, immunophenotype, molecular genetics and prognosis of extraskeletal mesenchymal chondrosarcoma in central nerve system (CNS). Methods: The clinicopathological findings, immunohistochemistry and genetic analysis of four cases of extraskeletal mesenchymal chondrosarcoma in Xuanwu Hospital between 2014 and 2019 were reviewed and followed up. Results: The ages of patients ranged from 20-35 years. Three patients had intracranial lesions and one had intradural tumor. The characteristic histologic features were undifferentiated small cells together with scattered islands of hyaline cartilage. There was hemangiopericytoma-like pattern with calcification and ossification. The tumor cells were positive for VIM and SOX9; and the small cells were positive for CD99, NSE and NKX3.1. The cells in chondroid matrix were positive for S-100. All tumor cells were negative for markers including CKpan, EMA and desmin. At molecular analysis, HEY1-NCOA2 fusion transcripts were identified in three patients. The fusion points were between exon 4 of HEY1 and exon 13 of NCOA2. Follow-up information was obtained in two patients, and both were free from recurrence or metastasis at 8 and 20 months. Conclusions: Extraskeletal mesenchymaI chondrosarcoma is a rare CNS disease with poor prognosis. In addition to SOX9, NKX3.1 can be another useful antibody for the differential diagnosis. The combination of pathological characteristics, immunophenotype and genetic profile of tumor is essential for diagnosis.

Metastatic Extraskeletal Mesenchymal Chondrosarcoma of the Pancreas: Report of an Unusual Case with Review of Literature

Extraskeletal mesenchymal chondrosarcoma (ESMC) metastasizing to the pancreas in isolation is a rare occurrence. We report a 49-year-old gentleman who had undergone excision of an ESMC of the thigh in 2009 and presented with sudden onset abdominal pain and icterus in 2019. Radiological imaging revealed calcified mass of the pancreas with multiple nodules with extension into the adipose tissue. Distal pancreatectomy was performed and the pathology revealed a bimorphic tumor composed of undifferentiated round blue cells with abrupt transition to hyaline cartilage, typical of mesenchymal chondrosarcoma. To the best of our knowledge, there are only seven prior cases of metastatic ESMC of the pancreas in the English literature. Surgical intervention appears to be the preferred modality of treatment for metastatic pancreatic tumors. These patients may have long latency period before metastasizing and seem to have a good survival period post excision.

Extraskeletal Mesenchymal Chondrosarcoma in Nasal Cavity: A Rare Case Report.

Extraskeletal mesenchymal chondrosarcoma (ESMC) originate from the nasal cavity have rarely been reported, especially its imaging features, which makes the preoperative diagnosis difficult. Here, we report the clinical, computed tomography, and magnetic resonance imaging features of a 60-year-old female patient with pathologically confirmed ESMC in the nasal cavity to help provide more reference for diagnosis before operation. Extraskeletal mesenchymal chondrosarcoma in the nasal cavity demonstrates typical imaging features, such as mesh-like enhancement, calcification, hemorrhage, necrosis, cystic degeneration, and so on.

Extraskeletal Mesenchymal Chondrosarcoma

Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. It usually presents during the second to third decades of life, and has a slight predominance in females. Histologically, it has a typical biphasic pattern comprising small cells and islands of hyaline cartilage. It can pose a diagnostic challenge in small biopsy specimens where 1 of the 2 components can be absent. The prognosis is extremely variable; survival varies depending on the location of the tumor.

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 Intracranial Mesenchymal Chondrosarcoma: A Case Report

Mesenchymal chondrosarcoma is a rare malignancy characterized by a biphasic pattern of small undifferentiated round cells intermixed with island of well-differentiated cartilaginous matrix. Although this type of tumor exhibits widespread distribution in the body, intracranial mesenchymal chondrosarcoma is rare. Radical excision of intracranial mesenchymal chondrosarcoma is important for obtaining a positive prognosis but the effectiveness of chemotherapy and radiotherapy remains uncertain. An 18-year-old patient presented with generalized seizure with magnetic resonance imaging showing a large dura-attached solid mass in the frontal convexity, which was presumptively diagnosed as a meningioma. Gross total resection of tumor was achieved without postoperative deficit. The final histological diagnosis was extra-skeletal mesenchymal chondrosarcoma, grade I. There was no adjuvant treatment after surgery. Five years after surgery, the prognosis was good after gross total resection only; however, long-term follow-up is necessary in the future. J Med Cases. 2018;9(5):135-138 doi: https://doi.org/10.14740/jmc3040w

Clinical benefit of antiangiogenic therapy in advanced and metastatic chondrosarcoma

Chondrosarcoma is the most common bone sarcoma in adults. Conventional chondrosarcoma, the commonest histological subtype, is largely resistant to anthracycline-based chemotherapy. There have been anecdotal reports of durable clinical benefit with antiangiogenic agents in this disease. A retrospective search of patients treated at three sarcoma referral centers was performed to identify patients with advanced chondrosarcoma treated with antiangiogenic agents. The aim of this study was to evaluate the efficacy and safety of antiangiogenic agents in advanced chondrosarcoma. Ten patients were identified; seven with conventional, one each with clear cell, extraskeletal mesenchymal chondrosarcoma and extraskeletal myxoid chondrosarcoma. The median progression-free survival for patients with conventional and clear cell sarcoma was 22.6 months. Median overall survival has not been met. Antiangiogenic therapy was well tolerated in this series of patients. Our retrospective data suggest that antiangiogenic therapy can provide prolonged clinical benefit in advanced chondrosarcoma patients. Further prospective trials are required to precisely define the role of this class of agent in advanced chondrosarcoma.

A child with extraskeletal mesenchymal chondrosarcoma in nasal cavity and sinus: a case report

A child with extraskeletal mesenchymal chondrosarcoma in nasal cavity and sinus: a case report

Posterior Mediastinal Chondrosarcoma- A rare entity

Introduction:Chondrosarcoma is a well defined tumor of soft tissue with calcification. Extraskeletal myxoid chondrosarcoma is an unusual sarcoma of soft tissue adding only 3% to all soft tissue tumors. Majority of mediastinal foci originates from variety of tissues, the reason being their diverse embryological and anatomical approximations. Chondrosarcomas are more common in males with 2:1 male to female ratio. The exact pathology of these tumors is unclear; however recent data ensures that these tumors have multidirectional delineation. Classical histopathological features of chondrosarcoma include S-100 positivity, EMA positivity. These features of Immunohistochemical favor extra skeletal myxoid chondrosarcoma.Case Report:Recent data signifies that the tumor is known for its rare occurrence, here we have reported a unique case of 40 years old male visited Abbasi Shaheed Hospital for pre employment checkup without any symptoms. All the tests were negative except Chest X-ray PA view which revealed dense mass on lower lobe of lung. For further evaluation, CT scan of chest with contrast was ordered and eventually the mass was resected surgically. On the basis of macro and microscopic findings, histopathological tests and immunohistochemical stains, the mass was found to be chondrosarcoma with myxoid origin. This tumor has to be distinguishing among the list of different diseases like hamartoma, hydatid cyst, and neuroendocrine tumors for the differential diagnosis of the case.Conclusion: Our paper reports an extraskeletal mesenchymal chondrosarcoma originating in the posterior mediastinum with a rare presentation.Â

Mesenchymal chondrosarcoma of the right buccal region: A case report and review of the literature.

Extraskeletal mesenchymal chondrosarcoma (EMCS) is a rare malignant cartilaginous tumor arising from the soft tissues. The most common areas of extraskeletal origin are the lower extremities, the orbits and the central nervous system, among others. In this study, the case of primary EMCS arising from the right buccal region in a 26-year old female is presented. Histological and immunohistochemical analysis confirmed the diagnosis of EMCS. Subseqently, the patient was treated with radical surgery, but declined chemotherapy or radiotherapy, which was recommended. One year after surgery, no recurrence had been identified in the patient. To the best of our knowledge, only one case of primary EMCS of the buccal region has been reported previously. In the current study, a case of primary EMCS of the buccal region is presented.

Condrossarcoma mesenquimal extraesquelético em um gato: relato de caso

Condrossarcoma mesenquimal extraesquelético (CME) é um neoplasma maligno e raro em animais domésticos. Descreve-se um caso de CME em uma gata que apresentava uma massa firme, branco-amarelada, medindo 18cm de diâmetro, aderida à musculatura do membro pélvico esquerdo. O exame citológico revelou presença de células fusiformes individualizadas pleomórficas e agregados de pequenas células ovais, sem bordas definidas em meio à matriz intercelular amorfa. Devido à impossibilidade de tratamento e ao prognóstico desfavorável, foi realizada eutanásia. Microscopicamente foram observadas células fusiformes indiferenciadas e agregados de células condroides pleomórficas. O diagnóstico de CME foi confirmado pelas técnicas de azul alciano, tricrômico de Masson e pela prova imunoistoquímica, utilizando-se anticorpos antivimentina.