Salivary gland second cancer after bone sarcoma treatment.

PURPOSE: Second malignant neoplasms (SMN) or second cancers may occur in 2-5 % of childhood cancer survivors within 5-25 years after diagnosis and treatment of a bone sarcoma. The most common are leukemia and breast cancer; salivary gland SMN are exceptional. To enhance the literature, we reviewed our patients with bone sarcomas and evaluated the incidence and outcome of salivary gland SMN. MATERIALS AND METHODS: We retrospectively studied all patients with osteosarcoma and Ewing's sarcoma treated at the Istituto Ortopedico Rizzoli with chemotherapy from January 1983 to December 2012. There were 883 osteosarcoma and 543 Ewing's sarcoma patients. We evaluated the date of diagnosis and histology of bone sarcoma, chemotherapy administered, date of diagnosis and histology of SMN, and survival of patients. RESULTS: The 10-year incidence of SMN was 3.6 %; the most common were breast cancer, leukemia, sarcomas, and salivary gland neoplasms. The incidence of salivary gland SMN was 0.5 %; there were five male and two female patients with a mean age of 19 years (range 13-28 years) who experienced a salivary gland SMN within a mean interval of 79 months (range 51-97 months). The most common salivary gland involved was the parotid followed by the submandibular gland. One of the seven patients with salivary gland SMN died from his SMN. CONCLUSIONS: Treating physicians should be aware of the risk of salivary gland SMN after chemotherapy for bone sarcomas in children and adolescents. Close follow-up of childhood bone sarcoma survivors for SMN is important.

Resurfaced allograft-prosthetic composite for proximal tibial reconstruction in children: intermediate-term results of an original technique.

BACKGROUND: Reconstruction of the proximal part of the tibia in children with megaprostheses or standard composite devices removes the distal femoral physis, and osteoarticular allografts have a high incidence of mechanical failure. We evaluated the results of an alternative original technique for proximal tibial reconstruction after tumor resection. METHODS: Nineteen children, ranging in age from six to sixteen years, who underwent surgical resection and reconstruction with a resurfaced allograft-prosthetic composite were retrospectively reviewed at a mean follow-up time of seventy-eight months. Eighteen children had high-grade sarcoma (fourteen had osteosarcoma and four had Ewing sarcoma), and one child had an aggressive giant cell tumor of the proximal part of the tibia. Surgical resection and its complications, disease-free survival of the patients, implant survival, and functional results according to the Musculoskeletal Tumor Society scoring system were analyzed at the time of the latest follow-up. RESULTS: In all patients, wide surgical margins were obtained and no local recurrence was observed. At the time of the latest follow-up, nine of the eighteen patients with a high-grade malignant tumor were continually disease-free, another six had had metastases but had no evidence of disease, and three had died of the disease. The patient with a giant cell tumor was continually disease-free after 168 months. The mean implant survival was sixty-eight months (range, six to 188 months). The major complications were one deep infection; two nonunions, which healed after surgical revision; and six allograft fractures. Limb-length discrepancy was the most frequent complication but, following contralateral epiphysiodesis, resulted in a mean discrepancy of 1.9 cm at the time of the latest follow-up. At the time of the latest follow-up, eight patients had a Musculoskeletal Tumor Society score deemed as excellent or good. CONCLUSIONS: In children and young teenagers with bone tumors of the proximal part of the tibia requiring an intra-articular resection, knee reconstruction with a resurfaced allograft-prosthetic composite may represent a valid alternative to conventional megaprostheses or to osteoarticular allograft.

Lipofibromatosis: magnetic resonance imaging features and pathological correlation in three cases.

Lipofibromatosis is a rare, benign, but infiltrative, soft tissue tumor seen in children. We present three cases of lipofibromatosis, each with different magnetic resonance imaging features and correlate this with the histological findings. The patients comprised two males and one female who presented in infancy; at birth, 5 months, and 7 months of age. Clinically, the masses were painless and slow-growing. The masses ranged in size from 2 to 6 cm and involved the distal extremities in two cases (one foot, one wrist) and the trunk. Magnetic resonance imaging showed lipomatous lesions with varying amounts of adipose and solid components in each case. There were no capsules at the periphery of the lesions. One case showed a fat-predominant lesion, another an equal mixture of fat and solid tissue, and the third was predominantly solid. This was reflected in the histology, which showed corresponding features. Radiological and histopathological differential diagnoses are reviewed.

Difficulty distinguishing benign notochordal cell tumor from chordoma further suggests a link between them.

BACKGROUND: Much discussion about benign notochordal cell tissue in vertebrae has centered on the nature of its relationship, if any, to chordoma. Often referred to as benign notochordal cell tumors (BNCTs), these lesions have unique morphological features, however, differentiating between notochordal cells in discs, BNCT, and chordoma can be difficult. They are described as radiologically distinct from chordoma, with lysis, contrast enhancement, and a soft tissue mass indicating chordoma. METHODS: All chordomas diagnosed at our institution, the Istituto Ortopedico Rizzoli (Bologna, Italy), prior to 2008 were reviewed, yielding 174 cases. Five were limited to bone; one was a recurrent chordoma without original data available. The remaining four were re-evaluated in detail. RESULTS: There were three women and one man, aged 33-57 years (mean, 48 years). Two were BNCTs and two were mixed lesions containing BNCT and chordoma. On computed tomography, all were radiopaque with areas of lysis. One BNCT was heterogeneous on magnetic resonance imaging, enhancing after contrast. Microscopically, one BNCT had a well-defined cystic area with a sclerotic border. The other had a minute atypical area; it recurred as chordoma. The mixed lesions had areas of definitive BNCT, definitive chordoma, and atypical areas that did not meet the criteria for either. The atypical areas in all three cases 'blended' with areas of chordoma or BNCT. CONCLUSION: These cases illustrate the ongoing challenges in differentiating between BNCT and chordoma. All had unique imaging features; three had atypical microscopic areas blending with BNCT or chordoma, strengthening the argument for a relationship between the two entities and supporting the idea that some BNCTs may progress to chordoma. Our study dispels the notion that any single radiologic criterion used to distinguish between chordoma and BNCT is reliable, opening the discussion as to whether or how to monitor BNCTs.

How to read a pathology report of a bone tumor.

The interpretation of a biopsy specimen involving bone is one of the most challenging feats for a pathologist, as it is often difficult to distinguish between benign or reactive lesions and malignant tumors on microscopic analysis. Therefore, correlation with the clinical data and imaging is essential and sometimes it is only the evolution of certain characteristics over time or information garnered from molecular analysis that can provide an accurate diagnosis. The pathology report is critical in that it will define subsequent patient management; its wording must precisely reflect those elements that are known with certainty and those that are diagnostic hypotheses. It must be systematic, thorough, and complete and should not be limited to a simple conclusion. The pathologist must first ensure the completeness and correct transcription of the information provided with the specimen, then describe and analyze the histology as well as the quality and representative nature of the sample (as they relate to the radiographic findings and preliminary/final diagnoses), and finally, compare what is seen under the microscope with the assessment made by the radiologist and/or surgeon. This analysis helps to identify difficult cases requiring further consultation between the radiologist and pathologist. There are multiple reasons for misinterpretation of a pathology report. An important and largely underestimated reason is varied interpretations of terms used by the pathologist. Standardized pathology reports with concise phrases as well as multidisciplinary meetings may limit errors and should be encouraged for optimal diagnostic accuracy.

Enchondroma vs. chondrosarcoma: a simple, easy-to-use, new magnetic resonance sign.

INTRODUCTION: There is no clear radiologic or pathologic agreement on the differences between enchondroma and conventional chondrosarcoma, which has huge therapeutic consequences. Microscopically, an enchondroma is composed of "islands of intramedullary hyaline cartilage surrounded by marrow fat", and a chondrosarcoma a "diffuse cartilaginous replacement (invasion) of the marrow which leads to complete 'trapping' of host lamellar bone trabeculae." The marrow around islands of cartilage should be detectable on magnetic resonance imaging (MR). Enchondroma may be the precursor of chondrosarcoma; benign cartilaginous islands are often seen microscopically at the periphery of chondrosarcoma. We attempted to detect these islands at the periphery of chondrosarcomas on MR and correlate them microscopically. MATERIALS AND METHODS: We examined our database for all patients with a chondrosarcoma of the long and flat bones between 1990 and 2007. Only those with a preoperative MR who underwent an en bloc resection were included, yielding 32 patients. We looked for low-signal islands surrounded by high (fat) signal on T1-weighted images, and high-signal islands surrounded by low signal on T2-weighted fat saturated images at the periphery of the main tumour mass. Microscopic correlation was performed in all cases. RESULTS: On microscopy, there were 23 conventional chondrosarcomas, nine dedifferentiated. Peripheral islands surrounded by fat were detected on MR in 19 cases, corresponding to benign cartilage in 18 cases and to the benign scar of a needle biopsy tract in one. There were no peripheral islands detected radiographically or microscopically in 13 cases. CONCLUSION: Cartilaginous islands microscopically detected at the periphery of some chondrosarcomas are easily and reliably diagnosed on MR.

Molecular diagnosis in Ewing family tumors: the Rizzoli experience--222 consecutive cases in four years.

The Ewing's family of tumors (EFTs) are characterized by chimeric transcripts generated by specific chromosomal rearrangements. The most common fusions are between the EWSR1 gene on chromosome 22 and the ETS family of transcription factors; rarely, FUS (on chromosome 16) substitutes for EWSR1. The detection of specific translocations using molecular analysis is now a routine part of the pathological examination of EFT. Here, we report our experience with molecular diagnosis of EFT during the 4 years (2006-2009) at the Rizzoli Institute. We analyzed 222 consecutive tumors with a presumptive diagnosis of EFT using molecular techniques and IHC. We found five distinct types of EWSR1-FLI1 fusion transcripts resulting from translocation t(11;22), three types of EWSR1-ERG transcripts resulting from t(21;22), and one type of t(2;22) resulting in EWSR1-FEV fusion. Molecular investigation validated 92% of cases ultimately diagnosed as EFT; IHC validated 76% of the cases. Thus, despite the difficulties and limitations associated with both molecular and IHC analysis on fresh and formalin-fixed, paraffin-embedded tissue, a combination of these techniques is the best approach to enhancing the accuracy of EFT diagnosis. We also present our method for choosing which molecular techniques to apply. Finally, we collected the most prevalent breakpoints reported in the literature, indicating which exons are involved, the sequence breakpoints, and the NCBI reference sequences.

Vaginal metastasis of a Ewing Sarcoma five years after resection of the primary tumor.

A 35-year-old female presented with pain and swelling of the left wrist. A diagnosis of Ewing sarcoma was made and she underwent neoadjuvant chemotherapy and surgery. Macroscopic viable areas remained on the map of the surgical specimen; as such, she was classified as a poor responder and received high dose adjuvant chemotherapy. She remained disease-free for five years, until age 40. A vaginal polyp was then detected during a routine gynaecologic examination. It was removed and histopathology revealed metastatic Ewing sarcoma. To our knowledge, this is the first reported case of a vaginal metastasis of Ewing sarcoma.

Sciatic nerve release following fracture or reconstructive surgery of the acetabulum. Surgical technique.

BACKGROUND: Sciatic neuropathy associated with acetabular fractures can result in disabling long-term symptoms. The purpose of this retrospective study was to evaluate the effect of sciatic nerve release on sciatic neuropathy associated with acetabular fractures and reconstructive acetabular surgery. METHODS: Between 2000 and 2004, ten patients with sciatic neuropathy associated with an acetabular fracture were treated with release of the sciatic nerve from scar tissue and heterotopic bone. Additional surgical procedures included open reduction and internal fixation of the acetabulum (five patients), removal of hardware and total hip arthroplasty (three patients), and removal of hardware alone (one patient). The average age of the patients was forty-three years. All patients were followed with serial examinations and assessments for a minimum of one year (average, twenty-six months). RESULTS: All patients had partial to complete relief of radicular pain, of diminished sensation, and of paresthesias after the nerve release. Four of seven patients with motor loss and two of five patients with a footdrop demonstrated improvement in function after the nerve release. No patient had evidence of worsening on neurologic examination after the release. CONCLUSIONS: Sciatic nerve release during reconstructive acetabular surgery can decrease the sensory symptoms of preoperative sciatic neuropathy associated with a previous acetabular fracture. Motor symptoms, however, are less likely to resolve following nerve release.

Posterior stabilization at the cervicothoracic junction: a biomechanical study.

STUDY DESIGN: This study biomechanically evaluated three fixation devices for stability with posterior two- and three-column injuries. OBJECTIVES: To find an effective means of posteriorly stabilizing injuries at the cervicothoracic junction. SUMMARY OF BACKGROUND DATA: The cervicothoracic spine is complex anatomically and has been a difficult challenge in approach and stabilization of traumatic and degenerative disorders. METHODS: Twenty-one human cadaveric spines (C3-T3) were loaded in flexion, extension, lateral bending, and axial torsion. A posterior two-column injury was created at C7-T1. One of three posterior fixation systems was applied (two rod-screw systems, one plate-screw system, all with screws at C5, C6 and T1, T2). The spines were tested again. A three-column injury was created by transecting the remaining anterior structures; the spines were tested a final time. RESULTS: In flexion-extension, there were no significant differences in stiffness between intact and instrumented two-column injury specimens for all systems; the instrumented three-column injury was significantly (P < 0.05) less stiff than intact specimens in extension. Ranges of motion and neutral zones decreased from intact to instrumented two-column injuries and increased from intact to three-column constructs. In lateral bending and axial rotation, all systems were stiffer than intact spines for both injuries; ranges of motion and neutral zones were reduced for both injuries compared with intact specimens. CONCLUSION: All three systems stabilize the cervicothoracic junction with a posterior two-column injury in flexion-extension, lateral bending, and axial rotation; none was adequate for a three-column injury, particularly in extension. A three-column injury at this level would warrant supplemental anterior fixation.