Eileen A. Crawford

Early outcomes for malignant peripheral nerve sheath tumor treated with chemotherapy

Objectives:Malignant peripheral nerve sheath tumors (MPNST) are rare soft-tissue sarcomas with a tendency for recurrence and metastasis. Treatment using chemotherapy is controversial, but benefit with some agents has been described. This study aimed to analyze early survival outcomes using doxorubicin and ifosfamide chemotherapy for MPNST. Methods:Pathology records at our musculoskeletal tumor center were searched for patients with a new diagnosis of MPNST between 2003 and 2008. Treatment involved surgical resection, radiation, and chemotherapy with doxorubicin and ifosfamide. Ten patients met inclusion criteria, with mean age 40 years (range, 20–70). Four patients had metastatic disease on presentation. Four patients had neurofibromatosis type I (NF1). Results:Of 6 patients with nonmetastatic disease on presentation, 5 had no evidence of disease post-treatment. The sixth had positive margins after surgery and initially received no further treatment due to noncompliance. Three from this subgroup developed local recurrence, but none developed distant metastases and 1 died of disease at last follow-up. One- and 2-year disease-free survival (DFS) for this subgroup was 80% and 60%, respectively. One- and 2-year overall survival (OS) for the subgroup was 100%. Of 4 patients with metastatic disease on presentation, 2 had no evidence of disease post-treatment. One of these 2 developed local recurrence, but none from the subgroup developed new metastatic disease. Two of these 4 died of disease at last follow-up. One- and 2-year DFS for this subgroup was 100% and 50%, respectively. One- and 2-year OS was 75% and 50%, respectively. Two of the 4 patients presenting with metastatic disease had NF1. All 3 local recurrences and 2 of the 3 deaths in this study occurred in NF1 patients. Conclusions:For all patients, when combined with surgery and radiation, chemotherapy using doxorubicin and ifosfamide yielded 57% DFS and 80% OS at 2 years. NF1 patients appeared to have worse outcomes, with a statistically significantly lower DFS than non-NF1 patients. Limitations of this study include a small sample size, retrospective design, and use of different chemotherapy regimens.

Treatment of adult rhabdomyosarcoma.

Objectives:Rhabdomyosarcoma is an exceedingly rare tumor in adults, and standard chemotherapy used for children is much less effective in adults. This study examines short-term outcomes using doxorubicin, ifosfamide, and vincristine for adult rhabdomyosarcoma. Methods:Pathology records were searched for adults (age, >18) with rhabdomyosarcoma treated at our musculoskeletal tumor center. Treatment involved surgical resection, radiation therapy, and chemotherapy with doxorubicin, ifosfamide, and vincristine. Eleven met inclusion criteria. Mean age was 49 (range: 19–72). Tumors sites included upper extremity (4 patients), lower extremity (6), and cervix (1). Subtypes were pleomorphic (7), alveolar (1), embryonal (1), and mixed alveolar/embryonal (2). Results:Of the 7 patients with nonmetastatic disease, 6 had no evidence of disease posttreatment, but 1 died of myelodysplastic syndrome after 51 months. Three patients who received neoadjuvant chemotherapy had 100% tumor necrosis. One patient with positive margins scheduled for adjuvant chemotherapy had local recurrence and metastasis within 2 weeks and died 5 months later. Of the 4 patients with metastatic disease on presentation, 1 had complete response, 2 had partial response with later progression and death at 8 and 24 months, and 1 had immediate progression and died at 12 months. Mean overall survival was 24 months with 6 of 11 (55%) alive at last follow-up. Mean disease-free survival was 17 months for all patients and 23 months for the 7 patients who had remission of all disease. Conclusions:When combined with surgery and radiation therapy, chemotherapy using doxorubicin, ifosfamide, and vincristine yielded 55% overall and 64% disease-free survival at 2 years.

Osteosarcoma of the Proximal Part of the Radius in Mazabraud Syndrome: A Case Report

Malignant transformation of fibrous dysplasia into sarcoma is a rare event, occurring in less than 1% of cases1,2. Malignant transformation of fibrous dysplasia can also occur as part of Mazabraud syndrome, a syndrome characterized by fibrous dysplasia and intramuscular myxomas1. However, Mazabraud syndrome is so rare that only four cases of sarcomatous degeneration have been reported in the literature1,3-5. Here we report a fifth case, which involved the development of osteosarcoma in a fibrous dysplastic lesion of the proximal part of the radius in a patient with Mazabraud syndrome. The patient was informed that data concerning the case would be submitted for publication, and he consented. A sixty-three-year-old man with a history of melanoma presented to our orthopaedic oncology clinic with pain in the right forearm. Fifteen years previously, the melanoma had been treated with excision, lymph-node dissection, and chemotherapy. The patient had been doing well until two months before the current presentation, when the forearm began to be painful. He described the pain as dull, aching, and moderate in severity. It bothered him mostly when he was performing heavy lifting, and he felt some relief after taking ibuprofen. He had no history of recent trauma to the area or fever, chills, night sweats, or weight loss. He had a thirty-pack-year smoking history, and his sister had died of ovarian cancer at sixteen years of age. His physical examination did not reveal any palpable masses, erythema, skin changes, or sensorimotor abnormalities, although the right forearm was slightly larger than the left. The initial radiographs (Figs. 1-A and 1-B) showed a mixed lytic-sclerotic lesion spanning the length of the radius, with expansion of the radial head, neck, and entire diaphysis. The lesion was benign in appearance and had no …

Thorough debridement under endoscopic visualization with bone grafting and stabilization for femoral head osteonecrosis in children.

Background: Osteonecrosis of the femoral head has become increasingly common after steroid therapy and as a consequence of improved survival in children with sickle cell anemia and leukemia. Multiple operative and nonoperative treatments have been explored in the pediatric patient population, and core decompression is a relatively safe and possibly effective option. To optimize core decompression further, we have tested a new technique involving thorough decompression of the osteonecrotic zone under endoscopic visualization (TDEV) combined with cancellous bone grafting and stabilization with a nail plate device. Methods: We retrospectively reviewed 16 hips in 13 patients (≤20 years old) with femoral head osteonecrosis related to steroid treatment, sickle cell anemia, or leukemia. The Steinberg classification system was used to classify all cases. Each patient underwent TDEV, bone grafting, and stabilization of the grafting site. Patients were followed up postoperatively for changes in pain level, functional ability, and Steinberg radiologic stage. Results: The mean follow-up was 28 months (range, 18-49 months). All patients in whom the procedure was successful had improvement in pain symptoms at latest follow-up, except for 1. Radiologically, all Steinberg stage II cases (B and C), except for 1, demonstrated good incorporation of graft without further progression of disease. Seven of the 8 patients that showed radiologic progression and deterioration of function or progressive symptoms had grade IIIB disease or higher at the time of procedure. Conclusions: Our initial results demonstrate that TDEV combined with cancellous bone grafting and stabilization produces encouraging early results for pediatric patients with lesions graded lower than Steinberg stage IIIB. Our findings were less optimistic for patients with higher-grade lesions. Our recommendation, therefore, is to use TDEV by trained surgeons for treatment of early-stage lesions, preferably less than Steinberg stage IIIB. Level of Evidence: Level IV, Therapeutic Study.

Symptomatic fat necrosis and lipoatrophy of the posterior pelvis following trauma

Posttraumatic fat necrosis and lipoatrophy can occur in the subcutaneous fat following falls, blunt injury, surgery, and minor procedures or injections. While these processes have no inherent serious medical consequences, they occasionally require treatment due to severe or concerning symptoms. Three patients (all women; average age, 47 years) who sustained blunt trauma to the pelvis and were diagnosed with posttraumatic fat necrosis or lipoatrophy were retrospectively identified from our orthopedic oncology records. All patients recalled blunt trauma to the posterior pelvis just prior to symptom onset; 2 patients fell down stairs and 1 fell from a bed. Chief symptoms were a painful mass, a painless mass, and chronic pain in the injured area. Magnetic resonance imaging (MRI) revealed atrophy of the subcutaneous fat in all cases and a small mass in 1 patient. A bright linear signal was seen on T2-weighted, fat-saturated images in 2 cases, likely representing scar tissue. One patient with chronic pain underwent surgery to provide better soft tissue coverage in the area of atrophic fat. The other 2 patients did not undergo surgical treatment: 1 was treated at a pain center for reflex sympathetic dystropy-type pain, and 1 remained pain free. Blunt trauma with subsequent fat atrophy and necrosis manifests as a mass, a subcutaneous fat defect, and even as chronic pain. Characteristic MRI findings are often sufficient for diagnosis, but any indeterminate masses should be further evaluated to rule out aggressive or malignant neoplasms. Chronic unrelenting pain despite treatment may be related to posttraumatic reflex sympathetic dystropy-like symptoms.

Concurrent osteochondroma and osteoblastoma of the proximal humeral shaft

We present what we believe is the first reported case of synchronous osteochondroma and osteoblastoma, occurring in the proximal humerus of a young man. A 15-year-old boy presented with a painful left arm mass for 3 months. A firm mass was palpable in the proximal medial arm, and he had mild triceps weakness secondary to pain. Imaging showed an eccentric lesion involving the proximal one-third of the humerus, with central lysis, surrounding sclerosis and edema, endosteal scalloping, and cortical thinning and expansion. The humeral diaphysis contained a bony exostosis with corticomedullary continuity, consistent with an osteochondroma with a cartilage cap <5 mm thick. Within the proximal portion of the osteochondroma was a 2-cm, edematous, rim-enhancing cystic lesion, concerning for a secondary process such as malignant transformation. On open biopsy, the mass grossly appeared to be a sessile osteochondroma, and was removed with a rongeur. The cystic lesion was curetted out of the bone. Pathology confirmed that the raised lesion was an osteochondroma. The cystic lesion contained osteoblasts in a matrix of osteoid and immature bone, characteristic of an osteoblastoma. The multidisciplinary team agreed that this was an osteoblastoma within an exostosis. At follow-up, the patients pain had fully resolved and radiographs showed good early healing. We wished to document the extraordinary, simultaneous existence of an osteochondroma and adjacent osteoblastoma in the proximal humerus of a young patient. Although similar in presentation, the tumors consist of cells of different origins, making the pathogenesis unclear.