Chemotherapy in Ewing��s sarcoma

Ewing’s sarcoma constitutes three per cent of all pediatric malignancies. Ewing’s sarcoma has generally been more responsive to chemotherapy than adult-type sarcomas, and chemotherapy is now recommended for all patients with this disease. It is essential to integrate local control measures in the form of surgery and/or radiotherapy at the appropriate time, along with chemotherapy to eradicate the disease. This approach has improved the survival substantially to the tune of 70% in localized disease, although outcome for metastatic disease remains dismal. Newer therapeutic approaches are required to improve outcome for metastatic and recurrent or refractory Ewing’s sarcoma in organized co-operative group trials.     

Carotid reconstruction in nonagenarians: is surgery a viable option?

Because of limited longevity and perceived increased perioperative risk, the optimal treatment of significant carotid stenosis in nonagenarians is controversial. This study was conducted to evaluate our results in this demographic group. A retrospective review was performed of carotid endarterectomies (CEAs) done in nonagenarians at Cedars-Sinai Medical Center between 1996 and 2006. During this period, a total of 2,038 CEAs were performed on patients of all ages. Data abstracted included demographics, patient risk factors, indications for surgery, perioperative complications, and survival. Fifty-three (2.8%) CEAs were performed as the primary procedure on 49 patients aged 90 or greater during the study period. Of these patients, 11 (22.4%) had diabetes, 38 (77.5%) had hypertension, and 31 (63.3%) had coronary artery disease. Eleven patients (22.4%) had a history of smoking, and there were no current smokers. Renal disease was present in three (6.1%) patients, one of whom was dialysis-dependent. The median length of stay was 2 days with a range of 1 to 24 days. Five patients (10.2%) required the intensive care unit following surgery. There were no postoperative strokes, and none of the patients had suffered ipsilateral stroke during follow-up. One patient (1.8%) had a perioperative myocardial infarction. One patient died in the perioperative period (1.8%). The 1-month stroke and mortality results did not differ significantly from those in patients under the age of 90, 0.3% and 0.4%, respectively (p = nonsignificant by Fisher's exact test). Using Kaplan-Meier life-table analysis, the 1- and 5-year survival rates were 84 +/- 5% and 33 +/- 9%, respectively. Our study demonstrates that in a group of well-selected nonagenarians, CEA is a safe procedure with acceptable perioperative morbidity.     

A spontaneously ruptured primitive neuroectodermal tumor/extraosseous Ewing’s sarcoma of the kidney with renal vein tumor thrombus

Primitive neuroectodermal tumor/extraosseous Ewing’s sarcoma (PNET/EES) is a childhood disease rarely seen in adults. It is a soft tissue tumor, which is often observed in the paraspinal region and lower extremity. We report the case of a 32-year-old man who presented with sudden abdominal pain on the right upper quadrant that was radiated to the right flank. During the operation, a spontaneously ruptured right kidney mass was found. The histopathologic and immunohistochemical characteristics of the excised mass were consistent with PNET/EES. This is the first known reported case of spontaneously ruptured PNET/EES of the kidney with renal vein tumor thrombus. The clinical details and the management of this case are described.     

Pathology of Ewing��s sarcoma/PNET: Current opinion and emerging concepts

Ewing’s sarcoma/PNET are small round cell tumors showing a varying degree of neuroectodermal differentiation. They are one of the commonest tumors of childhood and occur in bone and within soft tissues. Traditionally, light microscopy with the aid of immunohistochemical stains was suitable for diagnosis. But now translocation analyses are being used not only for the diagnosis and classification of small round cell tumors, but to ascertain their prognostic significance, detect micrometastasis, and monitor minimal residual disease, with potential for targeted therapy. This article analyzes the pathology, biology, and molecular aspects of Ewing’s sarcoma/PNET and discusses their clinical and therapeutic implications.     

Infiltrating giant cell tumor in a case of Paget’s disease of bone

Giant cell tumor (GCT) of the bone, also called osteoclastoma, is a rare complication of Paget’s bone disease. We report a patient from Southern Italy who developed a GCT infiltrating the neighboring tissues. The natural history and the therapeutic outcomes of this unique complication of Paget’s bone disease are presented.     

Aneurysmal bone cyst of fourth metacarpal bone in a child: excision and reconstruction with fibular strut graft��a case report and review of literature

Aneurysmal bone cysts of small bones of hands are rare and pose diagnostic and therapeutic challenge. We report a case of aneurysmal bone cyst of fourth metacarpal bone in a 14-year-old boy who presented with history of pain and swelling for 6 months duration. Radiograph and magnetic resonance imaging revealed a fusiform swelling involving the diaphysis and the distal metaphysis of the fourth metacarpal bone without any cortical breach. The physeal plate was not involved. Histopathological finding confirmed the diagnosis to be aneurysmal bone cyst. The patient was treated with marginal resection of the tumour preserving the articular surface by retaining the epiphysis and the bony defect was reconstructed with autologous fibular strut graft. One-year follow-up revealed an excellent incorporation of the graft with no evidence of local recurrence and also excellent range of motion of the hand. Nonvascularized fibular strut graft is easy to harvest and provide an excellent functional outcome when used as intercalary autograft after excision of aneurysmal bone cyst of hand.     

Telling ��Everything�� but not ��Too Much��: The Surgeon��s Dilemma in Consultations about Breast Cancer

Background  

Although clinicians are often criticized for giving inadequate information to patients with cancer, current recommendations to “provide full information” or “all the information patients want” are impractical. We therefore examined how surgeons manage information-giving to patients with breast cancer in practice, and how their approach compared with what patients wanted.     

Zoledronic acid-induced transient hepatotoxicity in a patient effectively treated for Paget��s disease of bone

Bisphosphonate (BP)-induced hepatotoxicity is very rare. There are only a few reports of liver injury after BP treatment, including aledronate and risedronate in postmenopausal osteoporosis patients. We describe hereby the case of a patient with Paget’s disease of bone accompanied by nonalcoholic fatty liver disease (NAFLD) who developed transient hepatotoxicity after zoledronic acid (ZOL) treatment. NAFLD had been diagnosed 1 year before presentation, based on liver ultrasonography (US). One day after infusion, serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), and gamma-glutamyltransferase (GGT) were increased by 8.1, 6.7, and 6.7 times, respectively, compared with pretreatment values. Serum bilirubin remained normal. US revealed hepatic mild homogenous brightness without focal lesion of the liver or biliary ducts. Subsequent biochemical and serologic investigation did not reveal a specific liver or systematic disease. The patient remained asymptomatic, and ALT, AST, and GGT were normalized 7 days post-treatment. Although the mechanism by which ZOL may cause liver damage is elusive, physicians should be aware of this possible adverse effect and ZOL cautiously administered in NAFLD patients.     

Fibular grafting in diaphyseal Gorham’s disease: report of two cases and review of literature

Gorham’s disease of bone is a rare entity where the bone undergoes progressive osteolysis. Management of diaphyseal Gorham’s disease is a real challenge to manage as a large bony defect is created because of osteolysis. We report two such cases where we reconstructed the defect with fibular graft after a radiation dose of 30 Gy. The graft was well incorporated and radiological sign of union was noticed in proximal and distal metaphysis after a follow-up of 4 and 2 years. Fibular grafting can be a good method of reconstruction in diaphyseal Gorham’s disease of long bone, provided the diseased tissue has been excised adequately after local radiation therapy.     

Caudal Antia–Buch reconstruction for helical defect reconstruction: Burow’s triangle always in the lobule

Different reconstructive techniques regarding helical ear defect reconstruction exist. The best cosmetic results are achieved using the modified Antia–Buch chondrocutaneous advancement flaps. In addition to this flap when it is caudally based, we always place Burow’s triangle in the earlobe. This technique provides the most normal cosmetic appearance of the ear without the need for a cranial-based flap in most cases.