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

BACKGROUND: Mesenchymal 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 PRESENTATION: A 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. CONCLUSIONS: MCS 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.

Bone Pathology for Hematopathologists.

Bone pathology can be challenging because the skeleton is a living tissue prone to developing a diverse array of inflammatory, metabolic, genetic, reactive, circulatory, and neoplastic abnormalities. Several areas of bone pathology are particularly difficult or problematic for hematopathologists given the close resemblance of some hematologic entities to primary/metastatic bone lesions; examples include plasmacytic disorders versus osteoblastic tumors and lymphoma/leukemia versus round cell tumors of bone. This article provides a conceptual and practical overview of selective bone disorders commonly encountered in the differential diagnosis of hematologic diseases.

Intracranial Extraskeletal Mesenchymal Chondrosarcoma: Case Report and Review of the Literature of Reported Cases in Adults and Children.

BACKGROUND: Intracranial extraskeletal mesenchymal chondrosarcoma is a rare, malignant variant of chondrosarcoma that is characterized by undifferentiated mesenchymal cells interspersed with pockets of mature hyaline cartilage. CASE DESCRIPTION: In this study, we report a 23-year-old female patient who underwent multiple craniotomies for tumor resection, as well as adjuvant radiotherapy and chemotherapy. We review the literature for reported cases and discuss the histopathologic features, radiologic findings, therapeutic approaches, and outcomes associated with this rare tumor. CONCLUSIONS: Intracranial extra-skeletal mesenchymal chondrosarcomas are very aggressive tumors, and their management should emphasize attempting gross total resection followed by adjuvant treatment modalities, including radiation therapy and/or chemotherapy.

Intracranial Mesenchymal Chondrosarcoma: Report of 16 Cases.

OBJECTIVES: Limited data regarding intracranial mesenchymal chondrosarcoma (MCS) are available. The goal of this study was to report the clinical characteristics, challenges in management, and poor outcomes of intracranial MCS. METHODS: Clinical data for 16 patients with MCS were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. RESULTS: This study included 11 male and 5 female patients with a mean age of 22.9 ± 14.4 years. The most common presentations were headache (n = 10; 62.5%), followed by cranial deficits (n = 7; 43.6%). The radiologic spectrum for MCS was broad, and only 18.8% (3/16) of MCSs were correctly diagnosed preoperatively. Aggressive resection (including subtotal resection and gross total resection) and partial resection was performed in 62.5% (10/16) and 37.50% (6/16) of patients. With a median follow-up of 34 months (range, 10-78 months), 5 patients (31.3%) died and 8 patients (50%) developed tumor recurrence. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 86%, 53%, and 42% and 93%, 70%, and 56%, respectively. Although the differences were not significantly different, aggressive resection and the use of radiotherapy tended to improve the prognosis of the patients. CONCLUSIONS: Clinical characteristics of MCS are variable. The current management of intracranial MCS referring to conventional chondrosarcoma could not yield satisfactory outcomes. Further study is needed to identify the optimal treatments.

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.

Intraspinal mesenchymal chondrosarcoma: report of a pediatric case and literature review.

PURPOSE: Mesenchymal chondrosarcoma (MCS) is an aggressive variant of chondrosarcoma and is a rare tumor, particularly within the pediatric population. Commonly, MCS originates in the bone, but it can also arise in extraskeletal sites, such as the brain and the intraspinal area. Due to the rarity of this tumor, there are no guidelines for its optimal treatment. METHODS: We report a case of intradural extramedullary MCS, located at the T11-T12 level, in a 14-year-old male. The tumor was documented by magnetic resonance imaging and treated with gross total resection (GTR) without adjuvant treatment. We further reviewed the relevant pediatric literature and discussed the management and outcome of intracranial and intraspinal MCS. RESULTS: The patient's follow-up showed no evidence of disease 2 years from diagnosis. A total of 51 cases of intracranial and intraspinal MCS have been reported (24 intraspinal and 27 intracranial). Recurrence has been described in only 4 patients with intraspinal MSC, and among them 3 received adjuvant chemotherapy and radiotherapy. GTR seems to reduce the risk of recurrence and, due to a higher cancer-mortality rate for these patients, adjuvant chemotherapy and radiotherapy are recommended in case aggressive surgery is not possible. CONCLUSIONS: According to our single experience, we would suggest that adjuvant therapy might be unnecessary in cases where a localized MCS undergoes GTR. Chemotherapy and radiotherapy should be recommended when GTR cannot be obtained. Further studies are needed to investigate a standard treatment approach for this rare tumor.

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.

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.

Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study.

BACKGROUND: Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations. PATIENTS AND METHODS: Specialist centres collaborated to report prognostic factors and outcome for 113 patients. RESULTS: Median age was 30 years (range: 11-80), male/female ratio 1.1. Primary sites were extremities (40%), trunk (47%) and head and neck (13%), 41 arising primarily in soft tissue. Seventeen patients had metastases at diagnosis. Mean follow-up was 14.9 years (range: 1-34), median overall survival (OS) 17 years (95% confidence interval (CI): 10.3-28.6). Ninety-five of 96 patients with localised disease underwent surgery, 54 additionally received combination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 local recurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) and OS were 7 (95% CI: 3.03-10.96) and 20 (95% CI: 12.63-27.36) years respectively. Chemotherapy administration in patients with localised disease was associated with reduced risk of recurrence (P=0.046; hazard ratio (HR)=0.482 95% CI: 0.213-0.996) and death (P=0.004; HR=0.445 95% CI: 0.256-0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; P=0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (P<0.0001). Data on radiotherapy indications, dose and fractionation were insufficiently complete, to allow comment of its impact on outcomes. Median OS for patients with metastases at presentation was 3 years (95% CI: 0-4.25). CONCLUSIONS: Prognosis in MCS varies considerably. Metastatic disease at diagnosis has the strongest impact on survival. Complete resection and adjuvant chemotherapy should be considered as standard of care for localised disease.

Congenital sacral mesenchymal chondrosarcoma in a neonate: a case report and review of literature.

Mesenchymal chondrosarcomas are rare malignant tumours in children, especially, in neonates. The authors present a case of congenital mesenchymal chondrosarcoma in a 1-day neonate located in sacrum. According to the authors' literature searches, this case is the first congenital sacral mesenchymal chondrosarcoma. We also reviewed the papers published in English literatures.

[Chondrosarcomatous metaplastic carcinoma of the breast, a rare tumor]. / Carcinoma metaplásico condrosarcomatoide de mama, una entidad inusual.

Metaplastic carcinomas of the breast are uncommon, accounting for less than 0.2% of all breast cancers. Clinically and radiologically, metaplastic carcinomas are indistinguishable from typical ductal carcinomas, and the diagnosis is made histologically by the finding of a mesenchymal component. We present a case of chondrosarcomatous metaplastic breast carcinoma whose definitive diagnosis required immunohistochemical techniques to confirm the malignant epithelial component of the tumor. Accurate diagnosis is important because this tumor behaves differently: it usually spreads through the blood (whereas typical epithelial carcinomas spread through the lymph vessels), metastases present during follow-up rather than before diagnosis, and the five-year survival rate is 35%.

Radiological features and pathology of extraskeletal mesenchymal chondrosarcoma.

OBJECTIVE: The objective of this study was to elucidate the imaging and pathological features of extraskeletal mesenchymal chondrosarcomas (EMCs). METHODS: Imaging findings of eight EMC cases were retrospectively analyzed. RESULTS: Soft tissue masses with different patterns of mineralization were found in five cases on computed tomographic scans. On magnetic resonance images, peripherally located EMCs demonstrated mixed signal intensity on T2-weighted images and heterogeneous enhancement with both calcified and noncalcified areas. CONCLUSION: EMCs exhibited several characteristic imaging features, which when used in combination with the mineralization pattern, enhancement of the calcified area, and signal intensity feature might have diagnostic value for this rare tumor.

Intraspinal mesenchymal chondrosarcoma in a 14-year-old patient: diagnostic and therapeutic problems in relation to the review of literature.

Mesenchymal chondrosarcoma (MC) is an infrequent, highly malignant neoplasm of the soft tissues and bone. It is very rare in the pediatric age group, especially in the intraspinal location. Only 24 cases have been reported to date. The authors present a case of a 14-year-old boy with an intraspinal MC who died of the disease 50 months from the initial diagnosis and after the third local recurrence. The patient was treated with a combination of chemotherapy, radiotherapy, and surgery. The authors review the clinical presentation, diagnostics, and the efficacy of treatment of pediatric patients with MC reported in the literature from 1978 to 2010.

Quiz. Correct answer to the quiz. Check your diagnosis. Mesenchymal chondrosarcoma of the orbit: two cases and a brief review of the literature.

Mesenchymal chondrosarcoma (MChS) is a rare, high-grade malignant tumor which occurs both in the bone and soft tissue. The extraskeletal location comprises one third of all MChS and in review of the up-to-date literature, about 30 cases of the orbital involvement were found. The authors present clinical, radiological and pathological findings of two cases of MChS of the orbit occurring in young adult females: primary extraskeletal MChS of the orbit and skeletal MChS of the ethmomaxillary complex with secondary orbit involvement. The histopathological examination revealed a characteristic biphasic pattern composed of small round to spindle-shaped cells, mimicking Ewing sarcoma family of tumors, with areas of a haemangiopericytoma-like pattern and admixed cartilage foci. One of the patients had local recurrence 3 years after initial surgical removal. Subsequently, she underwent enucleation followed by chemotherapy. The other patient had a biopsy and debulking resection of the tumor and started chemotherapy. Ten months follow-up of this patient show no evidence of metastasis.

Mesenchymal chondrosarcoma: clinicopathologic study of 20 cases.

CONTEXT: Mesenchymal chondrosarcoma is a rare, high-grade malignancy of bone or soft tissue with a unique, biphasic histology and poor prognosis. Because of its rarity and variable length of disease-free survival, the natural history of the disease remains poorly understood. OBJECTIVE: To present clinical, radiographic, and histopathologic features of mesenchymal chondrosarcoma from one of the largest case series collected by a single, senior-level bone pathologist. DESIGN: Twenty cases were reviewed in consultations spanning 45 years. RESULTS: Eighteen tumors (90%) originated in bone, and 2 tumors (10%) were of extraskeletal origin. Of the skeletal tumors, locations included craniofacial bones (n  =  9; 50%), ribs and chest wall (n  =  4; 22%), sacrum and spinal elements (n  =  3; 17%), and lower extremities (n  =  2; 11%), whereas soft tissue tumors were located about the scapula (n  =  1; 50%) and lower extremity (n  =  1; 50%). Plain radiographs demonstrated calcified, osteolytic lesions with extraosseous extension. Typical histologic features were identified consisting of small, round or spindled cells, interspersed with hyaline cartilage islands. Seventeen patients (85%) were treated surgically, and 8 patients (40%) received adjuvant treatment. Seven patients (35%) were living at last follow-up, 1.8 to 12.5 years after diagnosis, and 8 patients (40%) died between 1.2 and 21.8 years after diagnosis. CONCLUSIONS: Mesenchymal chondrosarcoma presents multiple challenges. Diagnostic pitfalls include inadequate biopsy samples, which may result in sample error. Sox9 has been proposed as a unique marker for mesenchymal chondrosarcoma which may improve diagnostic specificity. Treatment and prognosis vary considerably. Patients who receive surgery and chemotherapy seem to fare better. Multicenter studies with higher sample numbers may improve our understanding of this malignancy.

Sacral mesenchymal chondrosarcoma in childhood: a case report and review of the literature.

Mesenchymal chondrosarcomas are rare malignant tumors in pediatric age group. The authors present a case of mesenchymal chondrosarcoma located in the sacrum in a 10-year-old-girl that was successfully treated with chemotherapy and radiotherapy after surgical excision. According to the authors' literature search, the patient is the first reported case of pediatric sacral primary mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma cases in pediatric age group published in English literature was reviewed.

Case report: Mesenchymal chondrosarcoma of the lumbar spine in a child.

BACKGROUND: Chondrosarcomas of the spine constitute 4% to 10% of all primary spinal bone tumors and approximately 70% of the cases occur during the second or third decade of life. Mesenchymal chondrosarcoma is a rare aggressive variant of chondrosarcoma. The prognosis of mesenchymal chondrosarcoma is usually poor with a tendency for late local recurrence and metastasis. CASE DESCRIPTION: We describe a case of primary mesenchymal chondrosarcoma affecting the L5 vertebra of a 9-year-old girl. The patient underwent a staged circumferential resection of the tumor after three rounds of neoadjuvant chemotherapy. The patient had additional chemotherapy and radiation therapy as an intralesional margin was achieved during the procedure. At 9 years followup, the patient was asymptomatic, neurologically intact, and remained in remission. LITERATURE REVIEW: We identified only four previously published cases of spinal mesenchymal chondrosarcoma in childhood, two of which had relatively early recurrence and poor survival, and two survived but with only short followup. PURPOSES AND CLINICAL RELEVANCE: As the clinical and radiographic findings of mesenchymal chondrosarcoma are nonspecific, the diagnosis of this rare tumor requires careful histopathologic review of the specimens. We suggest the differential diagnosis of every primary intraspinal tumor include tumors of mesenchymal origin. The prognosis is apparently not uniformly poor.

Mesenchymal chondrosarcoma of the orbit: an unusual site for a rare tumour.

Mesenchymal chondrosarcoma is a rare tumour with orbital involvement being an exceptional occurrence. We present a case of a 22-year old man with such disease, together with details of his management. A brief literature review of this uncommon tumour was also enclosed.

Primary orbital mesenchymal chondrosarcoma: a case report and literature review.

BACKGROUND: Mesenchymal chondrosarcoma (MC) is a subtype of chondrosarcoma, with an incidence varying from 1 to 8% of all chondrosarcomas. It is an aggressive neoplasm with a high tendency for late recurrence and occasional delayed distant metastasis. Orbital MC is very rare, and only approximately 30 cases have been described in the literature. We describe here one case of primary orbital MC. CASE REPORT: A 14-year-old boy without a past medical history presented with a 1-month history of progressive proptosis on the right eye. Computed tomography (CT) scans of the orbit revealed a right intraconic lesion, with areas of calcification. The lesion was excised. Histopathological analysis revealed that the tumor had a biphasic pattern, showing a combination of small cell malignancy and well-differentiated cartilage. Immunohistochemistry examination revealed a diffuse membrane expression of CD99 on the small cell malignancy; S-100 was positive only within the cartilage component. The patient received chemotherapy, and no metastatic disease was found at the 2-month follow-up. CONCLUSION: Although rare, MC should be considered in the differential diagnosis of a well-circumscribed orbital lesion in young adults, especially when CT scans reveal areas of calcification within the tumor.