Paediatric High-Grade Intracranial Mesenchymal Chondrosarcoma: A Case Report with 6 Years of Follow-Up without Recurrence.

INTRODUCTION: Intracranial mesenchymal chondrosarcoma (MSC) is an extremely rare tumour that constitutes only 0.015% of all central nervous system tumours. These tumours usually originate from skull base synchondrosis and are often observed in young adults during their second and third decades of life. Despite the absence of a consensus regarding adjuvant radiotherapy, radical excision remains crucial for the prognosis of MSC. CASE PRESENTATION: We herein present the case of a young male patient with intracranial MSC, a malignant tumour, for which no consensus regarding its treatment has yet been established. The patient underwent radical excision followed by adjuvant radiotherapy. Histological analysis revealed a poorly differentiated tumour containing necrotic areas. Notably, no signs of recurrence had been observed after 6 years. CONCLUSION: The absence of recurrence over a long follow-up duration suggests the importance of radical excision and adjuvant radiotherapy.

Mesenchymal chondrosarcoma: A Japanese Musculoskeletal Oncology Group (JMOG) study on 57 patients.

BACKGROUND: This study aimed to elucidate the clinical features and prognostic factors of mesenchymal chondrosarcoma (MCS) and investigate optimal treatment strategies. METHODS: Data from 57 patients with MCS were collected from a Japanese Musculoskeletal Oncology Group (JMOG) and retrospectively analyzed. RESULTS: Data from 29 males and 28 females were collected. Primary tumor sites were the head and neck (7 patients), trunk (35 patients), and extremities (15 patients). The tumors originating in the trunk were significantly associated with a worse OS compared with those originating at the other sites in all patients and those with localized disease (P = 0.020 and P = 0.019, respectively). In patients with localized disease, the tumors originating in the head and neck were significantly associated with better OS and MFS compared with those originating in the trunk (P = 0.024 and P = 0.014, respectively). Positive surgical margin was significantly correlated with the worse LRFS (P = 0.018). Adjuvant chemotherapy exhibited a clear trend toward improved OS when MCS was localized in the trunk or extremities (P = 0.057). CONCLUSIONS: Adequate surgery is considered to be the mainstay of treatment for localized MCS. Prognosis was different depending on the site of tumor origin.

Extraskeletal orbital mesenchymal chondrosarcoma: surgical approach and mini review.

Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and highly aggressive tumour. It has characteristic radiological features and pathognomic histological biphasic pattern. Radical resection with negative margins is the mainstay of treatment; role of adjuvant chemotherapy and radiotherapy is yet not well defined. We report a rare case of 18-year-old man who was diagnosed to have orbital MC. He presented with locally advanced disease with no vision in the affected eye. He underwent right orbital exenteration; a transcranial intradural approach was used to divide the optic nerve, and the temporalis muscle flap was utilised to fill the exenterated orbit. Though optic nerve involvement is rare in orbital MCs, a transcranial approach may be used effectively to avoid traction on optic chiasma and ensure margin-free resection in case of optic nerve involvement up to orbital apex. Unfortunately, prognosis remains dismal in MCs despite treatment.

Clinical outcomes for patients after surgery and radiation therapy for mesenchymal chondrosarcomas.

INTRODUCTION: We report the outcome of 23 patients with mesenchymal chondrosarcomas treated with surgery and radiation therapy +/- chemotherapy. The intent of the project was to review the impact of patient and treatment variables on treatment outcome, in particular with regard to extent of surgery and radiation dose. PATIENTS AND METHODS: Twenty-three patients with mesenchymal chondrosarcomas were treated with surgery and radiation therapy (min. dose 44 Gy; max. dose 78 Gy; median dose 60 Gy; mean dose 61 Gy). RESULTS: The median survival for the entire cohort of patients was 21.65 years (95% confidence interval ± 4.25). The 5- and 10-year OS was 78.6%. Median disease-free survival for the 23 patients was 7.2 years. Disease-free survival (DFS) at 3 and 5 years was 70.7% and 57.8%, respectively. The local control rate at 5 and 10 years was 89.5% (95%CI 64.1-97.3%). Only three patients experienced local failure, three patients had regional failure, and eight developed distant metastases. CONCLUSIONS: In this cohort of patients local tumor control was high when using a combination of surgery and radiation. There was not a clear relationship between radiation dose and local tumor control. J. Surg. Oncol. 2016;114:982-986. © 2016 Wiley Periodicals, Inc.

Mesenchymal chondrosarcoma of bone and soft tissue: a systematic review of 107 patients in the past 20 years.

BACKGROUND: Mesenchymal chondrosarcoma(MCS) is a rare high-grade variant of chondrosarcoma. Consensus has not been reached on its optimal management. Resection with wide margins is usually recommended, but the effect of margins has been demonstrated by little positive evidence. Moreover, the effectiveness of adjuvant chemo- and/or radiotherapy remains controversial. OBJECTIVES: To describe the clinical characteristics and outcomes of MCS of bone and soft tissue, to assess the efficacies of surgery, chemotherapy and radiation, and finally to deliver a more appropriate therapy. MATERIALS AND METHODS: We reviewed EMBASE-, MEDLINE-, Cochrane-, Ovid- and PubMed-based to find out all cases of MCS of bone and soft tissue described between April 1994 and April 2014. Description of treatment and regular follow-up was required for each study. Language was restricted to English and Chinese. Issues of age, gender, location, metastasis, and treatment were all evaluated for each case. Kaplan-Meier Method and Cox Proportional Hazard Regression Model were used in the survival analysis. RESULTS: From the 630 identified publications, 18 meeting the inclusion criteria were selected, involving a total of 107 patients. Based on these data, the 5-, 10-and 20-year overall survival are 55.0%, 43.5% and 15.7% respectively. The 5-, 10-, 20- year event-free survival rates are 45.0%, 27.2% and 8.1%, respectively. Treatment without surgery is associated with poorer overall survival and event-free survival. Negative surgical margins could significantly bring down the local-recurrence rate and are associated with a higher event-free survival rate. Chemotherapy regime based on anthracyclines does not benefit the overall survival. The addition of radiation therapy is not significantly associated with the overall or event-free survival. However, we recommend radiation as the salvage therapy for patients with positive margin so as to achieve better local control. CONCLUSIONS: This review shows that surgery is essential in the management of MCS of bone and soft tissue. Appropriate adjuvant therapy may reduce local recurrence, but cannot benefit the overall survival.

Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma.

BACKGROUND: Mesenchymal chondrosarcoma (MSC) is a rare variant of chondrosarcoma. Because of the rarity of the disease, most studies only contain a small number of patients and thus the prognostic variables and role of adjuvant therapies remain controversial. QUESTIONS/PURPOSES: We therefore asked (1) what the overall and disease-free survival were for patients with this diagnosis at 5 and 10 years; (2) whether there were significant prognostic factors associated with survival; and (3) whether use of adjuvant chemotherapy or radiotherapy was associated with survival in patients with MSC. METHODS: We retrospectively reviewed the cases of MSC diagnosed from 1979 to 2010 at one referral center. Forty-three cases were identified. Thirty-seven cases were analyzed for demographics, treatments, and outcomes. Thirty patients with localized disease were analyzed for prognostic factors. The minimum followup was 1 month (mean, 6 years; range, 1 month to 17 years). There were 17 females and 20 males. The mean age at diagnosis was 33 years (range, 11-65 years). Nineteen cases were skeletal and 18 cases were extraskeletal. Seventy-six percent of the tumors were located in the trunk. RESULTS: Five- and 10-year overall survival was 51% and 37%, respectively. Five- and 10-year disease-free survival was 23% and 5%, respectively. Age (< 30 years) and male sex were associated with poorer overall and disease-free survival in patients presenting with a localized tumor, respectively. Patients who did not receive radiotherapy were more likely to have a local recurrence. Adjuvant chemotherapy failed to show a significant association with overall, disease-free, metastasis-free, or local recurrence-free survival. CONCLUSIONS: The present study reinforced the role of adjuvant radiotherapy for local tumor control. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Intracranial mesenchymal chondrosarcoma: case report and literature review.

BACKGROUND: Mesenchymal chondrosarcoma is a very rare malignant cartilaginous forming tumour in central nervous system (CNS), which is rarely encountered in clinical practice and generally occurs in young adults. This article describes a case of primary intracranial mesenchymal chondrosarcoma in a 31-year-old woman and reviews the literature on its manifestations and management. CASE REPORT: This patient had suffered from severe headache, intermittent nausea and vomiting for 1 week. Systemic examination was unremarkable. Magnetic resonance imaging (MRI) demonstrated a giant, heterogeneous, intensely enhancing mass of 6 × 5 × 4 cm, occupying the bilateral frontal and based on the anterior falx cerebri, which was initially thought to be a simply meningioma. The patient underwent a bicoronal craniotomy and gross total resection of the tumour. Pathologic examination revealed the mesenchymal chondrosarcoma. CONCLUSION: Intracranial mesenchymal chondrosarcoma is an extreme rare neoplasm, which should be considered in the differential diagnosis of intracranial mass like a meningioma. We emphasize the importance of surgical intervention and combination of microsurgical resection and radiotherapy, it should be the therapeutical choice of the future.

A fatal case of mesenchymal chondrosarcoma of the mandible.

A 34-year-old man presented with an expansile, erosive tumor involving the left side of the mandible, with secondary invasion into the maxilla, measuring 13 Χ 7 cm. Microscopic analysis revealed a malignant small round cell neoplasm with focal cartilaginous differentiation. Immunohistochemical analysis revealed positivity for vimentin, NSE and CD99 with primitive small round cells, and S100 positivity with neoplastic chondrocytes. To the best of our knowledge, this is perhaps the largest reported case of mesenchymal chondrosarcoma of the maxillofacial region. Diagnosed as inoperable, he was treated with radiation and chemotherapy only to die within a few months.

An unusual case of primary mesenchymal chondrosarcoma in orbit with intracranial extension.

An unusual case of primary orbital mesenchymal chondrosarcoma with intracranial extension is reported, with special emphasis on the radiological findings.

Carcinoma adrenocortical no funcionante con mielolipoma. Una rara asociación / Non-functioning adrenocortical carcinoma associated with myelolipoma. A case report

El carcinoma adrenocortical es un tumor infrecuente con una incidencia de 1 por millón de habitantes por año. El 80% aproximadamente de estos carcinomas son funcionantes, representando los no funcionantes solo un 20% de los carcinomas comunicados en las mayores series de la literatura. Se trató de paciente masculino de 55 años de edad con tumor adrenal derecho de 75×62mm. La pieza quirúrgica fue procesada para histología e inmunohistoquímica convencional. El tumor estaba constituido por células redondeadas, eosinofílicas, dispuestas en sabanas y en trabéculas. Se observaron de 3–5 figuras de mitosis en 50 campos de gran aumento y se identificó invasión vascular y sinusoidal. Se evidenció la presencia de mielolipoma entremezclado con el tumor y en la periferia del mismo. Solo 5 casos de carcinoma adrenocortical y concomitante mielolipoma han sido descritos en la literatura médica. En nuestro conocimiento mielolipoma asociado con carcinoma adrenocortical no funcionante no ha sido descrito previamente(AU)

Adrenocortical carcinoma is a rare tumour, with an annual incidence of only one case per million. Functioning tumours account for about 80% of all adrenocortical carcinomas, whereas non-functioning tumours are even rarer, accounting for only 20% of adrenal carcinomas in the largest reported series. We present a case of a 75×62mm right adrenal tumour occurring in a 55 year old male. Routine histological and immunohistochemical examinations were carried out on the surgical specimen. The tumour cells were mostly round, eosinophilic cells arranged in a sheet-like or trabecular pattern separated by thick fibrous bands. 3 to 5 mitoses were seen in 50 high-power fields; vascular and sinusoidal invasion was observed. A myelolipoma was found intermixed with the tumour at its periphery. To date, only five cases of adrenocortical carcinoma with concomitant myelolipoma have been published and to the best of our knowledge, this is the first report of myelolipoma associated with non-functioning adrenocortical carcinoma(AU)

The clinical approach towards chondrosarcoma.

This review provides an overview of the histopathology, classification, diagnostic procedures, and therapy of skeletal chondrosarcoma. Chondrosarcomas that arise de novo are primary chondrosarcomas, whereas chondrosarcomas developing superimposed on pre-existing benign cartilage neoplasms such as enchondromas or osteochondromas are referred to as secondary chondrosarcomas. Conventional chondrosarcomas can be categorized according to their location in bone into central, peripheral, and juxtacortical chondrosarcomas. Histological grading is related to prognosis; however, it is also subject to interobserver variability. Rare subtypes of chondrosarcoma, including dedifferentiated, mesenchymal, and clear cell chondrosarcoma, are discussed as well. Magnetic resonance imaging is necessary to delineate the extent of the intraosseous and soft tissue involvement preoperatively. Computed tomography is especially recommended in the pelvis and other flat bones where it may be difficult to discern the pattern of bone destruction and the presence of matrix mineralization. Wide, en-bloc excision is the preferred surgical treatment in intermediate- and high-grade chondrosarcoma. In low-grade chondrosarcoma confined to the bone, extensive intralesional curettage followed by local adjuvant treatment and filling the cavity with bone graft has promising long-term clinical results and satisfactory local control. Chondrosarcomas are relatively radiotherapy resistant; therefore, doses >60 Gy are needed in attempts to achieve local control after incomplete resection. Irradiation with protons or other charged particles seems beneficial in this curative situation. Chemotherapy is only possibly effective in mesenchymal chondrosarcoma, and is of uncertain value in dedifferentiated chondrosarcoma. Potential new systemic treatment targets are being discussed.

Mesenchymal chondrosarcoma. An analysis of patients treated at a single institution.

BACKGROUND: We analyzed clinical and treatment-related factors influencing the outcome of patients with mesenchymal chondrosarcoma (MC). Twenty-six patients (median age, 31 years) were identified using the Tumor Center and Chemotherapy Department database of the study institute. METHODS: Patients received surgery (24 patients) and/or radiotherapy (5 patients), and chemotherapy (12 patients). RESULTS: After a median follow-up of 48 months (7-237 months) 10 patients were alive. The 10-year overall survival (OS) was 27% in those who achieved complete surgical remission and 0% in those who did not (P = 0.0007). A worse 10-year probability of OS was observed in patients who were metastatic at presentation (metastatic 0%, localized 31%, P = 0.02), in patients with soft tissue MC (soft tissue MC 0%, bone MC 29%, P = 0.06) and in hemangiopericytoma-like MC (hemangiopericytoma-like MC 0%, Ewing's-like MC 33.5%, P = 0.9). In those patients who achieved complete surgical remission, the 10-year DFS was 76% for those who received chemotherapy and 17% for those who did not (P = 0.008). CONCLUSIONS: Our experience confirmed the importance of complete surgical remission in MC treatment and suggests that the addition of chemotherapy should offer a benefit in terms of DFS. Due to the rarity of MC, multicentrer studies are needed to identify the most active chemotherapy regimen.

Extraskeletal mesenchymal chondrosarcoma of the forearm: a case report.

Mesenchymal chondrosarcomas represent less than 10% of all chondrosarcomas. The majority of these tumors arise from the skeleton; only 22% have an extraosseous origin. Of the extraskeletal locations, the meninges, brain, and thigh are the most common. Involvement of the forearm is a rare occurrence. This case report describes an extraskeletal mesenchymal chondrosarcoma involving the forearm.

BNCT for recurrent intracranial meningeal tumours - case reports.

OBJECTIVES: The investigation was designed to explore the efficacy of boron neutron capture therapy (BNCT) as treatment for recurrent intracranial meningeal tumours. MATERIALS AND METHODS: Three patients with meningeal tumours, recurring after initial surgery, radiation therapy and several reoperations, were evaluated for treatment with BNCT by determination of the accumulation of boronophenylalanine fructose (BPA-F) in tumour and in surrounding tissue. Two of these patients were subsequently treated by BNCT. RESULTS: The present results indicate that BNCT could be effective in prolonging time to recurrence, and thus in extending survival time, for patients with recurrent intracranial meningeal tumours. CONCLUSIONS: BNCT is potentially an effective radiation treatment modality for malignant intracranial meningeal tumours, which could increase progression-free survival, thus reducing the need for additional surgical interventions. Indications for BNCT would be even larger if recurrent grade II meningiomas could be treated, as indicated by the results of the boron uptake study.

Mesenchymal chondrosarcoma of the orbit.

PURPOSE: To describe a patient with mesenchymal chondrosarcoma of the orbit, review clinical and imaging findings, and refine the differential diagnosis from other tumors of the orbit. METHODS: Interventional case report and literature review. RESULTS: A 21-year-old woman with a 6-month history of progressive proptosis presented with left-sided visual loss of recent onset. CT revealed a clearly outlined heterogeneous mass with calcified foci, whereas MRI showed an isointense signal to gray matter on T1- and T2-weighted images. The patient was diagnosed with mesenchymal chondrosarcoma of the orbit only after orbitotomy and subsequent histopathologic study. After surgery, she received adjuvant therapy with irradiation of the orbit. CONCLUSIONS: Mesenchymal chondrosarcoma is a rare malignant tumor of the orbit capable of mimicking several other lesions. Early diagnosis requires a high level of suspicion, particularly with regard to internally calcified lesions. The treatment of choice is complete surgical resection, or, when the tumor is not resectable or residual mass is present after surgery, chemotherapy and radiotherapy. Prognosis for patients with orbital mesenchymal chondrosarcoma is tentative at best, in part because of the rarity of the lesion.

Mesenchymal chondrosarcoma of the temporal bone.

A 64-year-old patient with mesenchymal chondrosarcoma of the temporal bone is described. CT and MRI showed an extensive mass with calcification involving the temporal bone and extending into the middle cranial fossa and nasopharynx. The tumor was ill-defined from surrounding normal bone, and a subtotal petrosectomy was carried out. The nasopharyngeal extension was removed secondarily using an endoscope. The clinical and diagnostic aspects and management of this rare lesion are discussed.

Computer-aided design and fabrication of an electron bolus for treatment of the paraspinal muscles.

PURPOSE: Demonstrate the technology for the design, fabrication, and verification of an electron bolus used in the preoperative irradiation of a mesenchymal chondrosarcoma in the paraspinal muscle region (T8-T12), in which the target volume overlay a portion of the spinal cord, both lungs, and the right kidney. METHODS AND MATERIALS: An electron-bolus design algorithm implemented on a three dimensional (3D) radiotherapy treatment planning system designed the bolus to yield a dose distribution that met physician-specified clinical criteria. Electron doses were calculated using a 3D electron pencil-beam dose algorithm. A computer-driven milling machine fabricated the bolus from modeling wax, machining both the patient surface and the beam surface of the bolus. Verification of the bolus fabrication was achieved by repeating the patient's computed tomography (CT) scan with the fabricated bolus in place (directly on the posterior surface of the prone patient) and then recalculating the patient's dose distribution using the 3D radiotherapy treatment planning system. RESULTS: A treatment plan using a 17-MeV posterior electron field with a bolus delivered a superior dose distribution to the patient than did the same plan without a bolus. The bolus plan delivered a slightly increased dose to the target volume as a result of a slightly broader range of doses. There were significant reductions in dose to critical structures (cord, lungs, and kidney) in the bolus plan, as evidenced by dose-volume histograms (DVHs). The patient dose distribution, calculated using CT scan data with the fabricated bolus, showed no significant differences from the planned dose distribution. CONCLUSIONS: A bolus can provide considerable sparing of normal tissues when using a posterior electron beam to irradiate the paraspinal muscles. Bolus design and fabrication using the tools described in this paper are adequate for patient treatment. CT imaging of the patient with the bolus in place followed by calculation of the patient's dose distribution demonstrated a useful method for verification of the bolus design and fabrication process.