Chondrosarcoma secondary to hereditary multiple osteochondromas with spinal cord compression: A case report and systematic review.

Background: Hereditary multiple osteochondromas (HMOs) are a rare genetic disorder characterized by the formation of multiple benign osteochondromas that can undergo malignant transformation into chondrosarcoma. Case Description: A 24-year-old male with a history of HMO and osteochondroma surgery 4 years ago, presented with back pain and paresthesias. The magnetic resonance showed a right paravertebral infiltrating mass at the T12-L1 level causing spinal cord compression. Following en bloc resection of the tumor, the patient's symptoms/ signs resolved. The final pathological diagnosis was consistent with a chondrosarcoma. Conclusion: Chondrosarcomas secondary to HMO with spinal cord compression are rare. These patients often presenting with significant myelopathy/cord compression should undergo gross total resection where feasible to achieve the best outcomes.

Secondary Chondrosarcoma Arising in Synovial Chondromatosis of Wrist Joint.

Introduction: Chondrosarcoma of the synovium is a rare and malignant form of cartilaginous tumor that originates in synovial tissue. There have only been a limited number of reported cases of malignant transformation of synovial chondromatosis (SC) into secondary chondrosarcoma (SCH), primarily in the hip and knee, in patients with resistant illness. The occurrence of chondrosarcoma in SC of the wrist is highly uncommon, as evidenced by only a single previous case study that has been documented in the literature. Case Report: This study presents a case series of two patients with primary SC who developed SCH at the wrist joint. Conclusion: Clinicians treating localized swellings of the hand and wrist should be alert to the likelihood of a sarcoma diagnosis to minimize delays to definitive therapy.

Gigantic secondary pelvic chondrosarcomas treated with pelvic resection type I and III: A case report.

INTRODUCTION: Patients with osteochondromatosis have a higher risk of malignant transformation into secondary chondrosarcoma. Chondrosarcoma at the pelvic region tends to present late and therefore pose a significant challenge for orthopedic surgeons because of the large tumor size, local extension, and complex anatomy with proximity to major neurovascular structure, intestinal and urinary tract. PRESENTATION OF CASE: A 44-year-old male presented the chief complaint of 15 years growing lumps on his left buttock and right groin, presenting with pain. Plain radiography revealed popcorn calcification at the left iliac wing and right superior pubic rami. Multiple exostoses were also visible. MRI showed a larger tumor diameter at the left iliac wing by 33 cm and right pubic rami by 13 cm. The histopathological result from the biopsy suggested low-grade chondrosarcoma. RESULT: The patient underwent pelvic resection type I and III in two-stage surgery. About one month after the first surgery, there was a postoperative infection. Debridement and antibiotic therapy resulted in a desirable functional outcome with an MSTS score 27 and no local recurrence sign during a one-year follow-up. DISCUSSION: Low-grade chondrosarcomas are not sensitive to radiation and chemotherapy; wide surgical resection is the mainstay of treatment. Chondrosarcoma at the iliac wing can be treated by pelvic resection type I, and further reconstruction needed to prevent pelvic tilting. Chondrosarcoma at pubic rami can be treated by pelvic resection type III. CONCLUSION: Proper patient selections, preoperative planning, and wide surgical margins with reconstruction provide desirable local control and clinical outcomes following pelvic resection.

Chondrosarcomas in children and adolescents.

Chondrosarcomas in children and adolescents are uncommon and constitute < 5% of all chondrosarcomas. There are very few studies discussing extremity chondrosarcomas in young patients.The pelvis is the most common site, followed by the proximal femur.As cartilaginous tumours can be quite challenging to diagnose, it is best for these lesions to be discussed in a multidisciplinary meeting which includes a radiologist and a pathologist specializing in bone tumours.Treatment principles are similar to those in adults, with adequate surgical excision respecting oncologic principles being the mainstay of treatment. Select extremity Grade I chondrosarcomas may be managed with extended intralesional curettage without increasing the risk for local recurrence or metastatic disease, but case selection is critical and should be based on clinical, imaging and histological characteristics.Chondrosarcomas are resistant to chemotherapy and relatively radioresistant. For mesenchymal chondrosarcomas, there may be a role for chemotherapy, though data on this is limited.Prognosis and rate of recurrence correlate directly to the adequacy of the surgical resection.Chondrosarcomas in younger patients behave in a similar fashion to those in adults, and outcomes in the young are no different from those in adults. Cite this article: EFORT Open Rev 2020;5:90-95. DOI: 10.1302/2058-5241.5.190052.

The giant aggressive chondroma: A rare entity, a difficult approach.

INTRODUCTION: enchondromas rarely exceed 3-6 cm in long bones. Although the risk of developing secondary chondrosarcoma has been reported up to 4% in solitary lesions, it is not known if size represents a risk factor for transformation. OBJECTIVE: to describe three exceptional cases of enchondromas of the entire femur whereof one dedifferentiated in chondrosarcoma. RESULTS: two patients present stable disease at 5 and 6 years of follow-up; the third, already diagnosed with a dedifferentiated chondrosarcoma, died 14 months after the index surgery for systemic disease. CONCLUSION: based on these observations, our hypothesis is that lesion size is an important risk factor for malignant transformation.

Secondary chondrosarcoma arising from osteochondroma: outcomes and prognostic factors.

AIMS: The aim of this study was to report the outcomes of patients who underwent definitive surgery for secondary chondrosarcomas arising from osteochondromas. PATIENTS AND METHODS: A total of 51 patients with secondary chondrosarcomas occurring from osteochondromas were reviewed. Median age was 36 years (interquartile range (IQR) 15 to 82). Median follow-up was 6.9 years (IQR 2.8 to 10.6). The pelvis was the most commonly affected site (59%). Histological grades were grade I in 35 (69%), grade II in 13 (25%), and grade III in three patients (6%). RESULTS: Preoperative biopsy histology correctly predicted the final histological grade in 27% of patients. The ten-year disease-specific survival (DSS) for all patients was 89.4%. Local recurrence occurred in 15 patients (29%), more commonly in pelvic tumours (37%) compared with limb tumours (19%). Four patients with pelvic tumours died from progression of local recurrence. No patient with limb tumours died of disease. Wide/radical margin was associated with improved local recurrence-free survival (p = 0.032) and local recurrence was associated with worse DSS (p = 0.005). CONCLUSION: We recommend that a secondary chondrosarcoma arising from osteochondroma of the pelvis is resected with wide/radical resection margins. The balance between the morbidity of surgery and risk of local recurrence needs to be considered in patients with limb secondary chondrosarcomas. Cite this article: Bone Joint J 2019;101-B:1313-1320.

Rare case of malignant transformation of a solitary spinal osteochondroma into recurrent metastatic chondrosarcoma.

Osteochondroma or osteocartilaginous exostosis is a commonly occurring primary tumor of the bone. Solitary spinal osteochondromas are, however, very rare, seen in only in 1-4% of all known cases and only few symptomatic cases have been reported in the literature so far. Further, while recurrence and malignant transformation are known to occur in osteochondroma, this is rare in the spine. We would like to report one such a case of an unusual presentation of recurrent, metastatic spinal chondrosarcoma in a patient with previous history of solitary cervical osteochondroma.

Chondrosarcoma of the temporomandibular joint: a case report and review of the literature.

OBJECTIVE: Chondrosarcoma is the second most common sarcoma arising in the bone, but it rarely involves the temporomandibular joint (TMJ). To date, 30 cases of TMJ chondrosarcoma have been reported in the English literature, and the authors report an additional case arising from a cystic lesion in a 60-year-old female patient. CLINICAL PRESENTATION: The clinical and radiological diagnosis of the lesion was initially synovial cyst, and periodic check-ups were done after aspiration of the lesion. After three years, the patient perceived swelling of the lesion, and surgical excision was performed. The final diagnosis was grade I chondrosarcoma. CONCLUSION: When clinicians detect a cystic lesion in the radiographic imaging of the TMJ, chondrosarcoma should be included in the differential diagnosis. In addition, computed tomography (CT) as well as magnetic resonance imaging (MRI) is recommended for the accurate diagnosis and proper preoperative planning in TMJ chondrosarcoma.

Chondrosarcoma of the proximal phalanx of the fourth digit: a rare location.

INTRODUCTION: Cartilaginous tumors involving the small bones of the hands and feet are usually benign such as enchondroma, chondromyxoid fibroma and chondroblastoma. The small bones of the hands and feet are rarely involved by primary chondrosarcoma. Proximal phalanges are the most common sites in the hands, but the fourth digit is the least common site. CASE PRESENTATION: We report a case of a 76-year-old Greek female who presented to our hospital with a painful swollen mass measuring 4.5 × 2.6 cm on the fourth digit of the left hand. The radiograph showed a destructive, permeative lytic tumor of the proximal phalanx with extension into soft tissue. The patient underwent curettage, and the microscopic examination of the specimen revealed grade 2 chondrosarcoma. CONCLUSION: Cartilaginous tumors involving the small bones of the hands and feet are usually benign such as enchondroma, chondromyxoid fibroma and chondroblastoma. Primary chondrosarcoma is the third most common malignancy of bone after myeloma and osteosarcoma, but the small bones of the hands and feet are very rarely involved by chondrosarcoma (1% of all chondrosarcoma). However, in these cases differentiation between a benign lesion and chondrosarcoma may be difficult. Occasionally chondrosarcoma of the hands and feet is associated with multiple recurrences or distal metastasis.

Secondary Chondrosarcoma Arising from Osteochondroma(tosis) / 대한골관절종양학회지

PURPOSE: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). MATERIALS AND METHODS: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and five were female. The mean age was 34 years. The mean follow-up period was 54 months. RESULTS: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. CONCLUSION: Comprehensive understanding of clinical, radiological and pathological features of secondary chondrosarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.