Mesenchymal chondrosarcoma - A rare cause of pleural effusion.

Mesenchymal chondrosarcoma is a malignant neoplasm arising from cartilaginous bone or soft tissue. It is uncommon yet devastating. Our patient was a 21-year-old man who presented with pleuritic chest pain and weight loss. His chest radiograph showed left pleural effusion. His pleural effusion analysis was consistent with exudative pleural effusion. Tuberculosis workup was negative. Pleural fluid cytology did not yield malignant cells. Subsequently, his computed tomography of thorax showed left rib sclerotic lesion with soft tissue component. Biopsy of the soft tissue eventually confirmed the diagnosis of mesenchymal chondrosarcoma. He succumbed to his illness before the diagnosis was confirmed. We hope that through this case report, we are able to provide some insight into this rare condition.

Mesenchymal chondrosarcoma metastasising to the pancreas.

The mesenchymal chondrosarcoma (MC) is a rare malignant tumour and accounts for less than 3% of primary chondrosarcomas. Mostly MC arises from the craniofacial bones, the ribs, the ilium, the femur and the vertebrae. A 54-year-old man was treated due to an icterus of unknown origin. The medical history of the patient consists of a multimodal treated MC of the thoracic vertebrae. A CT imaging identified a 2×4 cm sized mass of the pancreatic head. Suspecting a pancreatic head carcinoma surgical removal was performed. Histopathological a metastasis of MC was diagnosed. Our patient left the hospital after 17 days and died 23 month after surgery. Metastases of MC to the pancreas are rare. When detecting a mass of the pancreas in patients with a medical history of an MC, a metastasis of these tumour should be taken in consideration.

Spontaneous Intraventricular Hemorrhage: A Rare Presentation of a Skull Base Mesenchymal Chondrosarcoma.

BACKGROUND: Chondrosarcomas are very rare malignant, slow-growing tumors that develop in or near the petroclival region of the brain. We report a very rare case in which the tumor originated from left petrous bone and induced intraventricular hemorrhage leading to an acute comatose presentation. CASE DESCRIPTION: A 28-year-old man initially presented to the outpatient department with a 1-month history of headache, vomiting, vertigo, and left facial numbness. A lesion at the cerebellopontine angle with extension into the middle cranial fossa was demonstrated on computed tomography and magnetic resonance imaging. The following night his condition worsened, and he presented to the emergency department with intraventricular hemorrhage with hydrocephalus. An external ventricular drain was placed in the emergency department to relieve hydrocephalus, and definitive surgical resection of the tumor was subsequently. Postoperatively, his Glasgow Coma Scale score improved, and he was transferred to the surgical intensive care unit where he remained for 3 days. He was subsequently stepped down to a special care unit and then to a ward room. The patient is currently awake and has grade II facial palsy (House-Brackmann), demonstrates spontaneous purposeful eye opening, inconsistently obeys single-step orders, demonstrates no meaningful phonation or vocalization, and has at least grade 4 power in all 4 extremities. He is currently fed through a nasogastric tube and is in rehabilitation. CONCLUSIONS: Our experience of petroclival junction chondrosarcoma causing intraventricular hemorrhage may be the first to be documented. Preferred treatment of this highly malignant lesion is radical removal with postoperative radiotherapy.

Primary intraspinal dumbbell-shaped mesenchymal chondrosarcoma with massive calcifications: a case report and review of the literature.

BACKGROUND: Mesenchymal chondrosarcoma is a rare malignant tumor arising from bone or soft tissues. Instraspinal dumbbell-shaped mesenchymal chondrosarcoma is even rarer; however, it should not be neglected by clinicians. CASE PRESENTATION: A 26-year-old female was referred to our hospital with a 1.5-month history of sciatic pain and numbness in the left leg. Computed tomography and magnetic resonance imaging scans revealed an intraspinal dumbbell-shaped mass which had distinguishing features of neurogenic tumors, surprisingly, with massive calcifications, and no tumor metastasis was found. Then the patient underwent a total resection of the tumor, and during the operation, we found that the right nerve root of the fifth lumbar almost disappeared. The tumor was diagnosed as mesenchymal chondrosarcoma by histopathological examination after operation. Adjuvant therapies were not performed. However, recurrence of the tumor occurred 5 months later, and she underwent a total resection again combined with radiotherapy after second surgery. CONCLUSIONS: To the best of our knowledge, this case study presents the first report in literature about primary instraspinal dumbbell-shaped mesenchymal chondrosarcoma with massive calcifications, which may provide some evidence for clinical practice. As the clinical symptoms and radiographic findings of mesenchymal chondrosarcoma are usually not specific, clinicians should consider it as a possible case and diagnose it through careful histopathological examination. Sometimes, calcification could be seen in tumors, which may influence or reflect the growth of tumor and disease prognosis. Although prognosis in mesenchymal chondrosarcoma varies from person to person, generally, complete resection, adjuvant therapy, and regular examinations are recommended to perform for patients with mesenchymal chondrosarcoma.

Cervical subtotal en-bloc spondylectomy of C6 mesenchymal chondrosarcoma.

INTRODUCTION: We present a case of C6 mesenchymal chondrosarcoma and discuss safe posterior to anterior approach subtotal en-bloc spondylectomy. MATERIALS AND METHODS: A 29-year-old male consulted for our department with severe posterior neck pain doing exercise. CT scan demonstrated a primary osteolytic lesion on C6 left transverse foramen and MRI demonstrated the tumor involved C6 vertebra from layers B, C and F sectors 4-6 encasing left vertebral artery. Preoperatively neurointerventional radiology service occluded the left vertebral artery and tumor feeding artery using coil embolization. Posterior approach consist of C5-C7 laminectomy, left sided C6 and C7 nerve root sacrifice, posterior disc removal and release of C5-6-7 and posterior reconstruction. Then, position was changed to supine, and the anterior approach was followed as C5-6, C6-7 discectomy, left vertebral artery ligation and cut, longus coli resection and C6 subtotal spondylectomy with en-bloc resection of mass, mesh cage insertion and C5-C7 anterior plate fixation. During operation, frozen biopsy was performed on 8 areas (longus coli, lateral margin, anteroinferior margin, posterior margin, posterosuperior margin, C5 transverse foramen, posteroinferior margin, inferior margin) after wide resection. Tumor free margin was confirmed. RESULTS: After operation, he complained of tingling sensation of left thumb and forearm medial side, and elbow extensor motor grade was checked to 4/5 postoperatively. In the followed-up radiograph, the tumor was completely removed, and the instability of joint was not seen. As a result of observing follow-up CT at a year after the surgery, recurrence findings have not been shown up to now, and the progression of neurologic symptoms has not been shown either. CONCLUSION: Based on the Grand Round case and relevant literature, we discuss the case of mesenchymal chondrosarcoma occurring from the C6 cervical spine treated with cervical subtotal en-bloc spondylectomy. Successful en-bloc resection of the tumor was achieved using posterior to anterior approach.

Pulsed radiofrequency treatment of piriformis syndrome in a pregnant patient with malignant mesenchymal tumor.

Cancer is frequently seen in women of reproductive age. Diagnosis, management of treatment, and safety of the therapeutic approach are particularly important for these patients. Presently described is pain management in a case of pregnancy with malignant mesenchymal tumor. A 23-year-old woman in 30th gestational week presented with severe pain in right hip and back of the right thigh. Piriformis block successfully decreased pain and was followed by pulsed radiofrequency (PRF) to the piriformis muscle. PRF, as a non-neurodestructive method, is a safe and effective method to treat cancer pain in a pregnant patient.

Tumor-induced hypophosphatemic rickets in an adolescent boy--clinical presentation, diagnosis, and histological findings in growth plate and muscle tissue.

CONTEXT: The mechanism behind disabling muscle weakness in tumor-induced hypophosphatemic rickets is obscure. Histological investigation of growth plate tissue of patients with tumor-induced osteomalacia has so far not been reported. PATIENT: A mesenchymal tumor was detected in the left distal fibula by (68)Ga-DOTATOC in a 17-yr-old boy with adolescent onset of severe hypophosphatemic rickets. Disabling muscle weakness improved within days after surgery, and normal mobility was restored within months. METHODS AND RESULTS: The resected tissue included part of the growth plate allowing immunohistochemical investigation. Positive staining of FGF23 was found in the tumor cells and in hypertrophic chondrocytes, osteoblasts, and osteoclasts of the adjacent growth plate. This distribution matched that found in growth plate tissue of a healthy control. We found positive staining for the somatostatin receptor not only in the tumor but also within the growth plate and adjacent bony tissue in the patient and the healthy control. Muscle tissue provided evidence for a partial defect in respiratory chain complexes I-IV. Biochemical markers were nearly or completely restored to normal 12 months after surgery. CONCLUSIONS: Hypertrophic growth plate chondrocytes are a target or source of FGF23 in tumor-induced osteomalacia. Low serum phosphate, FGF23, or other factors produced by the tumor may interfere with mitochondrial function.

Mesenchymal chondrosarcoma of the orbit: a unique radiologic-pathologic correlation.

PURPOSE: To report a unique radiologic-histopathologic correlation of mesenchymal chondrosarcoma of the orbit in a 24-year-old Asian woman. METHODS: Clinicopathologic case review. RESULTS: The patient was examined for left-sided proptosis of several months' duration. CT showed a left orbital mass depicting a central radiolucent, nonenhancing component, and a denser peripheral enhancing portion. Histopathologic examination of the orbital mass showed a biphasic pattern of a mesenchymal chondrosarcoma exhibiting features of a high-grade sarcoma with hemangiopericytoma pattern that corresponds to the radiopaque portion of the mass and areas of chondrosarcoma that correlated with the radiolucent component of the tumor. The patient underwent exenteration of the left orbit followed by radiotherapy and chemotherapy. The last follow-up (8 years, 1 month) disclosed no evidence of recurrence or metastatic disease. CONCLUSIONS: In this case, a unique CT-histopathologic correlation of the mass was established. The authors believe that recognizing the different radiologic features of the orbital tumor can help clinicians in establishing the correct preoperative diagnosis of this potentially lethal neoplasm. To the best of the authors' knowledge, this diagnostic correlation has not been previously reported.

Primary extraskeletal mesenchymal chondrosarcoma arising from the pancreas.

We report here on a case of primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas. A 41-year-old man was evaluated by CT to find the cause of his abdominal pain. The CT scans showed a heterogeneously enhancing necrotic mass with numerous areas of coarse calcification, and this was located in the left side of the retroperitoneal space and involved the body and tail of the pancreas. Portal venography via the celiac axis also showed invasion of the splenic vein. Following excision of the mass, it was pathologically confirmed to be primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas.

Oncogenic osteomalacia in a case with a maxillary sinus mesenchymal tumor.

We herein describe the rare case of a 41-year-old woman with oncogenic osteomalacia due to a tumor in the maxillary sinus who presented with chronic general pain that had been gradually deteriorating. The patient's laboratory findings revealed hypophosphatemia due to renal phosphate wasting, an inappropriately low serum 1 alpha,25-dihydroxyvitamin D3 level for hypophosphatemia and an unusually high serum level of fibroblast growth factor 23 (FGF23). The causative tumor was surgically removed, resulting in a rapid resolution of the patient's biochemical abnormalities. An improvement of the abnormal multiple deposits on (99)Technetium-methylene diphosphonate bone scintigraphy and an increase in the bone metabolism markers suggested the development of bone remodeling within 49 days after the operation. The pathologic diagnosis of the tumor was a "phosphaturic mesenchymal tumor, mixed with a connective tissue variant." The expression of FGF23 was demonstrated in the tumor by the immunohistochemical techniques and a Western analysis.

Femoral mesenchymal chondrosarcoma with secondary aneurysmal bone cysts mimicking a small-cell osteosarcoma.

Mesenchymal chondrosarcoma is a rare but aggressive, high-grade malignancy of primitive cartilage-forming mesenchyme that arises most commonly from skeletal sites. Although there are radiological findings suggestive of the diagnosis, imaging features often overlap with those of other skeletal sarcomas. The definitive diagnosis relies on the histological finding of a typical bimorphic appearance, consisting of nests of small, round, poorly differentiated cells and more mature cartilaginous tissue. To highlight this, we present the case of a 21-year-old man who was referred to our institution with a history of right knee pain. Initial imaging and histological evaluation of a core biopsy of the lesion suggested osteosarcoma of the distal right femur; after review, however, the correct diagnosis of mesenchymal chondrosarcoma was made. Adequate tissue sampling and thorough histological evaluation of biopsy specimens is vital for the accurate diagnosis of primary bone malignancies, especially those of chondroid origin.

Intracranial mesenchymal chondrosarcoma with osteoid formation: report of a pediatric case.

CASE REPORT: We present a case of a 14-year-old girl with a 3-week history of severe progressive headache and intermittent vomiting. Magnetic resonance imaging (MRI) revealed a large intensely enhancing mass, which seemed to arise from the right side of the cerebral fossa with implant base in the inferior face of the tentorium and significant mass effect on the nearby structures. A presumptive preoperative diagnosis of meningioma was made. Subtotal surgical resection was performed using the occipital approach. Histologically the neoplasms had the classic features of a mesenchymal chondrosarcoma associated with the focal presence of osteoid matrix. DISCUSSION: Clinical features, therapeutic approaches and prognosis of this rare tumour are discussed with regard to the known 30 cases in the literature.

Mesenchymal chondrosarcoma of the vagus nerve.

Mesenchymal chondrosarcoma is a rare, aggressive, malignant neoplasm, which arises from extraskeletal sites in 30-40 per cent of cases. It is extremely rare in children. We present a novel case of childhood mesenchymal chondsarcoma arising from the vagus nerve in the neck, resulting in paralysis of the right vocal fold. The clinicopathologic features and management of this case are described along with a brief discussion on the aetiology of vocal fold paralysis in this age group. Current literature on extraskeletal presentation of mesenchymal chondrosarcoma is reviewed.

Microvasculature and VEGF expression in cartilaginous tumors.

We examined the microvasculature and VEGF expression in 26 cartilaginous lesions (CL) including 5 enchondromas, 9 grade 1 chondrosarcoma (CS), 6 grade 2 CS, 4 grade 3 CS, 1 mesenchymal, and 1 myxoid chondrosarcoma. The degree of neovascularization was measured by counting microvessels on H&E and factor VIII related antigen immunostained slides. Vessels were divided into pericartilage vessels (PCV) and intracartilage vessels (ICV). PVC comprised vessels around the lobules or invading the lobules but themselves surrounded by noncartilaginous stroma (ie, fibrous stroma); ICV consisted of those vessels present inside the tumoral nodules and in direct apposition with malignant cells or tumoral stroma. A direct correlation was seen between histological type and grade of CS and pericartilage vessels. In contrast, ICV were found only in higher-grade CS. No enchondromas and only 1 of 9 grade 1 CS had ICV. This patient had Ollier's disease. All but 2 of the grade 2 CS showed ICV (average, 20.5). The exceptions were predominantly grade 1 CS with focal grade 2 areas and extensive areas of necrosis. All but 1 grade 3 CS had ICV, the exception being a case of metastatic CS to the lung. Malignant chondrocytes of high-grade lesions stained strongly for vascular endothelial growth factor (VEGF), a potent angiogenic factor. The only high-grade tumors that did not express VEGF did not show ICV either. Enchondromas and grade 1 CS, most without ICV, did not express VEGE In summary, PCV are present in all categories of tumoral cartilage and the number increases with histological grade; ICV are found in high-grade lesions, and the exceptions show extensive necrosis; VEGF expression by malignant chondrocytes is seen in high-grade lesions almost exclusively, and among these in those lesions that showed intracartilage vessels. It is possible that PCV are involved in supporting tumor growth, whereas ICV might be involved in the acquisition of metastatic potential by cartilage tumors. VEGF expression is strongly associated with the presence of ICV.

Tumor-induced osteomalacia and symptomatic looser zones secondary to mesenchymal chondrosarcoma.

Tumor-induced osteomalacia is a rare clinical entity that is associated with soft-tissue or skeletal tumors. We present a case report of a patient with a chest wall mesenchymal chondrosarcoma who presented with bone pain. The patient had skeletal changes in the femoral neck and fibula consistent with osteomalacia and laboratory values suggesting phosphate diabetes. The patient was treated with tumor resection and phosphate supplementation with reversal of the signs and symptoms of osteomalacia. Tumor-induced osteomalacia is vitamin-D-resistant and often reversed by complete removal of the tumor. Most commonly, the causative tumors are of vascular, mesenchymal, or fibrous origin. The osteomalacia is associated with bone pain, muscle weakness, and radiographic changes. Tumor-induced humoral factors have been implicated in causing the osteomalacia, but the definite etiology has yet to be determined. Current treatment includes complete tumor resection and electrolyte supplementation.