ABSTRACT
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.
ABSTRACT
Renal mesenchymal chondrosarcoma is a rare malignant soft tissue tumor. This paper reports a patient who was admitted to hospital because of left renal tumor, and underwent robot-assisted laparoscopic partial nephrectomy. The patient was diagnosed as left renal mesenchymal chondrosarcoma by pathological and genetic examination after operation. There was no local recurrence and metastasis six months after operation.
ABSTRACT
Small round cell lesions of the bone encompass a heterogeneous group of tumors and tumor-like lesions, including Ewing sarcoma, small cell osteosarcoma, mesenchymal chondrosarcoma, neuroblastoma, non-Hodgkin's lymphoma (NHL), “Ewing-like” undifferentiated round cell sarcomas, metastasizing small cell carcinoma, along with plasma cell dyscrasia and Langerhan's cell histiocytosis. At the same time, there are tumor mimics, for example, chronic osteomyelitis, which has overlapping radiologic features with Ewing sarcoma and a primary intraosseous NHL. An exact diagnosis necessitates integration of clinical, radiologic, pathologic, and ancillary test results, including immunohistochemical and molecular results. Currently, there are several immunohistochemical markers and specific molecular signatures, driving most of these tumors, available, for an exact diagnosis. This review focuses on a pragmatic approach towards uncovering specific small round cell lesions of the bone, emphasizing upon integration of traditional morphology with ancillary techniques, including immunohistochemical markers and molecular techniques, the latter, especially in cases of Ewing sarcoma, Ewing-like undifferentiated round cell sarcoma, mesenchymal chondrosarcoma, and neuroblastoma. Subsequent to the diagnostic approach, including an impact on treatment, individual intraosseous round cell lesions have been described in detail. The references include updated articles from PUBMED.
ABSTRACT
Chondrosarcoma (CS) is a malignant tumor of long and flat bone characterized by the formation of cartilage. Mesenchymal chondrosarcoma is a rare variety of chondrosarcoma .It is a biphasic tumor with areas of spindle cell mesenchyme along with chondroid differentiation in the connective tissue stroma. A 17 year old male presented to us as a painless mass in maxilla. Contrast enhanced computed tomography (CECT) showed a lytic expansile lesion in the right maxillary bone with foci of calcification within soft tissue lesion. Fine needle aspiration cytology (FNAC) and incisional biopsy was performed which confirmed the diagnosis of maxillary Mesenchymal chondrosarcoma .The patient underwent right subtotal maxillectomy with 2 cm margins. The review of literature shows that very lesser number of maxillary Mesenchymal chondrosarcoma cases were reported so far. Therefore an attempt is made to add this rare case of MC of maxillary alveolus in the Existing literature.
ABSTRACT
Mesenchymal chondrosarcoma originated in the primitive mesenchymal tissue .It usually devel-ops in the short bones such as hand ,foot and body bone ,while extremeIy rare in the orbit .We report a case of mesenchymal chondrosarcoma of the orbit which is confirmed by pathology .
ABSTRACT
Se presenta una paciente de 15 años de edad con el diagnóstico de condrosarcoma mesenquimal de hueso temporal derecho con infiltración del músculo temporal y de la duramadre de la fosa media, desplazamiento del lóbulo temporal sin infiltración de parénquima cerebral, diagnosticada en enero del 2010, la cual recibió tratamiento quirúrgico con resección total de la lesión mediante craneotomía temporal, radioterapia de intensidad modulada y quimioterapia como tratamiento coadyuvante.
A 15 year-old patient is present with the diagnostic of a mesenchymal chondrosarcoma of the temporal bone with infiltration of the temporal muscle and with to scroll up of the temporal lobe on January 2010. She had got a temporal craniotomy a radical insensitive modulate radiotherapy and chemotherapy as adjuvant treatment.
Subject(s)
Humans , Adolescent , Female , /surgery , /diagnosis , Bone Neoplasms/surgery , Bone Neoplasms/diagnosis , Temporal Bone , Combined Modality Therapy , Craniotomy , /drug therapy , /radiotherapy , Fatal Outcome , Bone Neoplasms/drug therapy , Bone Neoplasms/radiotherapy , Skull BaseABSTRACT
Objective:To assess the surgical outcome of patients with mesenchymal chondrosarcoma (MCS) treated in our insti-tute. This study was also designed to describe the clinical characteristics, treatment, and outcome of MCS to provide a better understand-ing of its clinical management. Methods:A total of 27 patients with MCS were treated in Peking University People's Hospital, Beijing, China from October 1997 to March 2011. Demographic information and follow-up data were obtained and statistically analyzed. Re-sults:Among the 27 patients, 9 were males and 18 were females with a mean age of 30.4 years (ranging from 14 years to 51 years). The median follow-up time was 42.6 months (ranging from 6 months to 104 months). Among the total number of tumor cases, 22 and 5 were detected in bone tissues and extra-skeletal sites, respectively. A total of 25 patients underwent surgery, but only 17 achieved the standard surgical margin of wide excision. Among these patients, 16 and 13 were subjected to chemotherapy and irradiation. The three-and five-year survival rates were 65%and 49.5%, respectively. Conclusion:MCS is a rare tumor resulting in morbidity with local recur-rences and long-term metastases. In this study, standard multimodal regimens were proposed to treat MCS. The results recommended wide resection with suitable surgical margins as the preferred treatment. However, further studies should be conducted because the infor-mation about the benefits of chemotherapy and radiotherapy for the control of local or systemic symptoms of MCS remains insufficient.
ABSTRACT
Mesenchymal chondrosarcoma is a rare disease with poor prognosis. Treatment including wide or radical excision is very important. Radiotherapy and chemotherapy are additional treatment options, but no conclusive results for their efficacy have been shown until date. Imaging modalities can give important clues for diagnosis and management planning. Angioembolization before surgery could be useful as prophylaxis to control intraoperative bleeding, increasing the likelihood of complete resection.
Subject(s)
Chondrosarcoma, Mesenchymal , Rare DiseasesABSTRACT
Extraskeletal mesenchymal chondrosarcoma (EMC) is a rare and a malignant chondrogenic neoplasm. As a particularity of this neoplasm, about one-third of the cases develop outside the bone, with intramuscular site being a very rare location for development of EMC. The diagnosis of mesenchymal chondrosarcoma can be very challenging, especially in cases without conspicuous cartilaginous differentiation. In such cases its distinction from other small cell mesenchymal neoplasms cannot be safely established. This, however, is of major clinical interest as it implicates different treatment protocols as well as a different prognosis. We hereby present a case of EMC at a unusual location in a 23-year-old female with the purpose to highlight its morphologic features and to discuss its differential diagnosis.
ABSTRACT
Mesenchymal chondrosarcoma is a rare aggressive variant of chondrosarcoma that frequently occurs in extraskeletal location. A 28-year-old female presented with a history of dyspnea and fever and succumbed to her illness before a conclusive diagnosis was established. An autopsy performed revealed the presence of an extraskeletal mesenchymal chondrosarcoma (ESMC) involving the pleura. Only one case of ESMC of the pleura has been reported previously. Herein, we report the second case of ESMC of the pleura.
ABSTRACT
Mesenchymal chondrosarcomas are rare malignant tumors of the bone and soft tissue. Spinal mesenchymal chondrosarcomas are even rarer and, to the best of our knowledge those that are concomitantly located in the intradural and extradural regions, have never been reported. We report a case of a 25-year-old man with back pain and bilateral progressive weakness of the lower extremities. Magnetic resonance imaging revealed a markedly enhanced dumbbell-shaped mass at the T7 level. The lesion was intradurally located at the left side of the spinal cord, and extended extradurally to the extraforminal space through the T7-8 intervertebral foramen. The tumor was completely excised through a posterior approach. Microscopic examination and immunohistochemical studies confirmed mesenchymal chondrosarcoma. Postoperative radiation therapy and chemotherapy were also performed to prevent local recurrence and metastasis. The patient has been symptom-free for two years after surgery. Herein, we reviewed and discussed the clinical characteristics, treatments, and outcomes of primary intraspinal mesenchymal chondrosarcomas in the literature.
Subject(s)
Adult , Humans , Back Pain , Chondrosarcoma, Mesenchymal , Lower Extremity , Magnetic Resonance Imaging , Neoplasm Metastasis , Recurrence , Spinal CordABSTRACT
Mesenchymal chondrosarcoma is very aggressive and represents approximately 1% of all chondrosarcomas. While it affects a very wide age range, the peak frequency is in the second decade of life. It may occur in the head and rib region with a predilection for the maxillofacial skeleton. The small cell undifferentiated component may assume a hemangiopericytoma-like vascular pattern and should be distinguished from hemangiopericytoma. Treatment is en bloc resection, the intended tissue margins of excision should be designed to extend well beyond the actual tumor margin, as mesenchymal chondrosarcomas. Aggressive behavior of mesenchymal chondrosarcoma of the jaw, with a tendency for delayed recurrence and metastasis even many years after treatment. The most frequent site of metastasis was the lung. Here we present 52 years old , female case of mesenchymal chondrosarcoma occurs on Rt. mandible.
Subject(s)
Female , Humans , Chondrosarcoma , Chondrosarcoma, Mesenchymal , Head , Hemangiopericytoma , Jaw , Lung , Mandible , Neoplasm Metastasis , Recurrence , Ribs , SkeletonABSTRACT
· AIM: To report a rare case of mesenchymalchondrosarcoma in the orbit and to explore its clinicmanifestations, pathologic characters, management andprognosis. · METHODS: We report a case of mesenchymalchondrosarcoma of the orbit. The clinical materials,including ophthalmological examination, computed tomo-graphy scan of the orbit, histopathology and immunohis-tochemistry of the biopsy specimen was reported, and itspertinent literatures were reviewed.· RESULTS: A 36-year-old female was seen with proptosisand decreased vision. Histopathology demonstrated anadmixture of undifferentiated mesenchymal cells andislands of mature hyaline cartilage. Immunohistochemicalstudies revealed positivity for vimentin and S-100, whichwas consistent with the diagnosis of mesenchymalchondrosarcoma.· CONCLUSION: Mesenchymal chondrosarcoma in theorbit is extremely rare malignant tumor. Multi-modalitytreatments (surgery, chemotherapy and radiotherapy)may lead to long-term survival.
ABSTRACT
Mesenchymal chondrosarcoma is a rare malignant tumor of bone and soft tissue. This aggressive form of chondrosarcoma represents only 3% to 9% of all chondrosarcomas. This neoplasm is characterized by sheets or clusters of undifferentiated spindle or round cells surrounding discrete nodules of well-differentiated cartilage. We experienced a case of mesenchymal chondrosarcoma on mandibular body. Two years ago, the patient had been treated the intrabony cystic lesion on mandiblular left body. At that time, cartilage portion was not detected in the cystic specimen. Two years after cyst enucleation, the recurred large neoplasm in the mandibular left body was noted, and it was diagnosed as 4.5 cm sized mesenchymal chondrosarcoma. The mandibular tumor was widely resected and rigid-plate and cervical musculocutaneous flap were used for reconstruction of resected bone and soft tissues. No complications and recurrence were noted for 6 months postoperatively.
Subject(s)
Humans , Cartilage , Chondrosarcoma , Chondrosarcoma, Mesenchymal , RecurrenceABSTRACT
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.
Subject(s)
Adult , Humans , Male , Abdominal Pain/etiology , Chondrosarcoma, Mesenchymal/complications , Contrast Media/administration & dosage , Diagnosis, Differential , Iohexol/analogs & derivatives , Necrosis , Pancreas/pathology , Pancreatic Neoplasms/complications , Portal Vein/diagnostic imaging , Radiographic Image Enhancement/methods , Rare Diseases , Retroperitoneal Space/diagnostic imaging , Splenic Vein/diagnostic imaging , Tomography, X-Ray Computed/methodsABSTRACT
Mesenchymal chondrosarcoma is a rare cartilaginous neoplasm of an extraskeletal origin, and this predominately occurs in the head and neck, and also in the lower extremities. Fewer than twenty cases of cardiac mesenchymal chondrosarcoma have so far been reported on. For the most part, the results of treatment for patients with this condition have been dismal. In this study, we describe a case of cardiac mesenchymal chondrosarcoma that responded to chemotherapy following surgical biopsy. A 46-year-old man was referred for evaluation of his pleural effusions in both lungs. Chest computed tomography revealed an ovoid-shaped mass in the posterior wall of the patient's left atrium. The echocardiogram revealed a large ovoid-shaped immobile mass (11 x 6 cm2) in the pericardiac space, which was attached to the posterior wall of the left atrium. Emergency pericardiostomy with closure thoracostomy was performed. Seven days later, a thoracotomy was performed for reduction and diagnosis of the cardiac mass. The pathological diagnosis was extraskeletal mesenchymal chondrosarcoma of the heart.. Postoperative chemotherapy was performed for the huge remaining mass with a combined regimen of etoposide, ifosfamide and cisplatin. After 6 cycles, the patient showed a partial response without symptoms. Although cardiac mesenchymal chondrosarcoma has been reported to be chemotherapy- resistant with a short survival duration, chemotherapy may prove to be an effective treatment modality.
Subject(s)
Humans , Middle Aged , Biopsy , Chondrosarcoma, Mesenchymal , Cisplatin , Diagnosis , Drug Therapy , Emergencies , Etoposide , Head , Heart Atria , Heart , Ifosfamide , Lower Extremity , Lung , Neck , Pericardial Window Techniques , Pleural Effusion , Thoracostomy , Thoracotomy , ThoraxABSTRACT
A mesenchymal chondrosarcoma is a rare malignant tumor occurring in both the bone and soft tissues, and has the characteristics of a malignant nature. Since its original description in 1959, several reports of this tumor occurring in the maxilla, mandible, rib and spine have been published. We report a case of a mesenchymal chondrosarcoma that occurred in the posterior part of C7, T1 and T2 and adjacent muscles in a 31-year-old man who was treated with a wide marginal excision.
Subject(s)
Adult , Humans , Chondrosarcoma, Mesenchymal , Mandible , Maxilla , Muscles , Ribs , SpineABSTRACT
Mesenchymal chondrosarcomas are very rare tumors, and they are more aggressive in comparison to the conventional type tumors. They can occur in any location containing mesenchymal cells, but most of them arise in the lower extremities, the leptomeninges and the eye orbits. Other sites are very uncommon and they represent approximately 1% of all chondrosarcomas; these rare tumor locations carry a poor prognosis. In this report, we present a case of extraskeletal mesenchymal chondrosarcoma of the pancreas. We could find only one case of extraskeletal mesenchymal chondrosarcoma with pancreatic metastasis from thigh in our PubMed-line search. We describe here a case of primary originating mesenchymal chondrosarcoma of the pancreas in a 40-year old man who presented with abdominal pain, and this is the first report of the pancreas as the primary origin of mesenchymal chondrosarcoma.
Subject(s)
Adult , Humans , Abdominal Pain , Chondrosarcoma , Chondrosarcoma, Mesenchymal , Lower Extremity , Neoplasm Metastasis , Orbit , Pancreas , Prognosis , ThighABSTRACT
Mesenchymal chondrosarcoma is a rare tumor occurring in both bone and soft tissues and exhibits characteristic of a malignant nature. The authors experienced a case of mesenchymal chondrosarcoma occurring in a 23-year-old woman which had invaded the cervical spine. The patient presented with severe both shoulder pain, left upper extremity weakness(Grade IV) and paresthesia at admission. Radiologic studies of the cervial spine showed an aggressive osteolysis of C4 vertebral body, pedicle and lamina with compression of the spinal cord posteriorly on C3, C4, C5 levels. The tumor was totally removed by a combined anterior and posterior approach. The removed vertebral body was replaced with autogenous bone and stabilized by Codman locking plate symtem. The pathological examination showed characteristic of mesenchymal chondrosarcoma.previous symptoms well improved postoperatively. The authors present a case of mesenchymal chondrosarcoma with review of literature.
Subject(s)
Female , Humans , Young Adult , Chondrosarcoma, Mesenchymal , Osteolysis , Paresthesia , Shoulder Pain , Spinal Cord , Spine , Upper ExtremityABSTRACT
Mesenchymal chondrosarcoma is a rare malignant tumor of skeletal and extraskeletal origin, and which shows aggressive local behavior as well as a high metastatic potential. We report 3 cases of mesenchymal chondrosarcoma. Two cases were male and one was female. The ages ranged from 25 to 32 years(mean: 28 years). Tissue was obtained by wide excision in two patients, and incisional biopsy in one. The mass locaterd in the rib(case 1), orbital floor(case 2), and abdominal wall(case 3). Roentgenographically, the tumor resembles ordinary chondrosarcoma, showing osteolytic and obstructive appearance with stippled calcification. Grossly, the tumor was lobulating, solid fish-fleshy like mass with calcification and ossification. Histologically, the tumor shows characteristic bimorphic pattern composed of islands of well differentiated hyaline cartilage admixed with a cellular area of undifferentiated small cells. The small cells usually displayed a hemangiopericytoid or an alveolar pattern.