An unusual presentation of subcutaneous granuloma annulare in association with juvenile-onset diabetes: case report and literature review.

Subcutaneous granuloma annulare (SGA) is a rarely reported subtype of granuloma annulare that occurs almost exclusively in children. The etiology of these lesions is unknown, although a possible relationship to insulin-dependent diabetes mellitus (IDDM) has been proposed in the literature. Here we present an unusual case of SGA on the right forearm in a 3-year-old girl. Unlike the typical lesions noted on the extremities, on magnetic resonance imaging her lesions involved the subcutaneous tissue and adjacent muscles in multiple locations. Histopathologically, the case was consistent with SGA but was unusual in its distribution involving multiple muscles, a finding that has not been previously reported. Weeks after incisional biopsy, she was readmitted with diabetic ketoacidosis (DKA) secondary to IDDM. After treatment of her DKA and control of her glycemia, the forearm SGA vanished, which supported the pathologic diagnosis and alleviated our concerns secondary to the unusual distribution.

Thoracoscopic assisted en bloc resection of a spine tumor.

Spine tumors are fairly common and the management is through a multimodality approach. Lesions of the thoracic and lumbar vertebrae have been treated with such extensive anterior and/or posterior approaches. The authors present a case of a 56-year-old lady with solitary T11 metastases from colonic carcinoma and a case of a 43-year-old lady with T5-T6 high-grade osteogenic sarcoma. The treatment consists of a wide vertebrectomy by posterior approach, after anterior release and sub-pleural dissection using a thoracoscopic approach. A thoracoscopic assisted anterior approach could reduce the duration and the morbidity of a vertebrectomy without affecting oncological management.

Morbidity of en bloc resections in the spine.

The morbidity of surgical procedures for spine tumors can be expected to be worse than for other conditions. This is particularly true of en bloc resections, the most technically demanding procedures. A retrospective review of prospective data from a large series of en bloc resections may help to identify risk factors, and therefore to reduce the rate of complications and to improve outcome. A retrospective study of 1,035 patients affected by spine tumors-treated from 1990 to 2007 by the same team-identified 134 patients (53.0% males, age 44 +/- 18 years) who had undergone en bloc resection for primary tumors (90) and bone metastases (44). All clinical, histological and radiological data were recorded from the beginning of the period in a specifically built database. The study was set up to correlate diagnosis, staging and treatment with the outcome. Oncological and functional results were recorded for all patients at periodic, diagnosis-related controls, until death or the latest follow-up examination (from 0 to 211 months, median 47 months, 25th-75th percentile 22-85 months). Forty-seven on the 134 patients (34.3%) suffered a total of 70 complications (0.86 events per 100 patient-years); 32 patients (68.1%) had one complication, while the rest had 2 or more. There were 41 major and 29 minor complications. Three patients (2.2%) died from complications. Of the 35 patients with a recurrent or contaminated tumor, 16 (45.7%) suffered at least one complication; by contrast, complications arose in 31 (31.3%) of the 99 patients who had had no previous treatment and who underwent the whole of their treatment in the same center (P = 0.125). The risk of major complications was seen to be more than twice as high in contaminated patients than in non-contaminated ones (OR = 2.52, 95%CI 1.01-6.30, P = 0.048). Factors significantly affecting the morbidity are multisegmental resections and operations including double contemporary approaches. A local recurrence was recorded in 21 cases (15.7%). The rate of deep infection was higher in patients who had previously undergone radiation therapy (RT), but the global incidence of complications was lower. Re-operations were mostly due to tumor recurrences, but also to hardware failures, wound dehiscence, hematomas and aortic dissection. En bloc resection is able to improve the prognosis of aggressive benign and low-grade malignant tumors in the spine; however, complications are not rare and possibly fatal. The rate of complication is higher in multisegmental resections and when double combined approach is performed, as it can be expected in more complex procedures. Re-operations display greater morbidity owing to dissection through scar/fibrosis from previous operations and possibly from RT. The treatment of recurrent cases and planned transgression to reduce surgical aggressiveness are associated with a higher rate of local recurrence, which can be considered the most severe complication. In terms of survival and quality of life, late results are worse in recurrent cases than in complicated cases. Careful treatment planning and, in the event of uncertainty, referral to a specialty center must be stressed.

Diagnosis and planning in the management of musculoskeletal tumors: surgical perspective.

The evaluation of musculoskeletal tumors requires a close interaction between the orthopedic oncologist, radiologist, and the pathologist. Successful outcome can be achieved in a considerable number of patients by following the appropriate diagnostic strategies and staging studies. The aim of this article is to outline the presentation, imaging, and staging of the primary and metastatic bone and soft tissue tumors. Some of the image-guided interventions for these tumors are also presented.

Diagnosis and staging of spine tumors.

Persistent axial pain with or without neurologic changes should prompt workup for a possible tumor of the spine. Metastatic disease is more predominant than primary tumors, but still needs adequate evaluation before any management. The various steps of evaluation, diagnosis, and staging are reviewed.

Spine oncology: Daedalus, Theseus, and the Minotaur.

Over the past three decades, progress has been dramatic in the management of spine tumors. For example, advanced imaging technologies made available at manageable costs have lowered the threshold for scanning. CT, MRI, and PET imaging modalities have greatly enhanced the ability of the surgeon to accurately delineate the extension of the lesion within the bone, the soft tissue, and the spinal canal. Such enhancements have led to great leaps forward in preoperative planning and postoperative evaluation, including improved reconstruction options are resulting in improved outcomes. This article introduces the theme of this volume.

Management of benign tumors of the mobile spine.

In the treatment of primary tumors, complete local eradication is the main goal, as an oncologically appropriate surgical treatment can substantially improve the prognosis and even be considered a life-saving procedure. In deciding the best treatment for primary bone tumors of the spine, the choice of surgery, radiation therapy, chemotherapy, selective arterial embolization, or other medical treatments alone or in combination is based on diagnosis, staging, and a deep understanding of the biology and the behavior of each tumor. This article is a guide to diagnosing and treating such rare tumors.

Complications of en bloc resections in the spine.

Morbidity of surgical procedures for spine tumors is expected to be worse than for other conditions. This is particularly true for en bloc resections, a technically demanding procedure. En bloc resections can help improve the prognosis of aggressive benign and malignant tumors in the spine, but the related morbidity is high and sometimes fatal. Reoperations have higher risks because of dissection through scar/fibrosis from previous surgeries and possibly from radiation. Careful planning for treatment is mandatory, and if the surgeon is unsure, referral to a specialty center is necessary.

Metastatic behaviour of giant cell tumour of the spine.

Lung metastases from giant cell tumours (GCT) of the spine have not been specifically addressed in the literature. We reviewed our cases and compared the incidence, treatment, and outcomes with those from the extremities. Between 1970 and 2006, we identified seven cases (three females and four males) of lung metastases from a total of 51 cases of GCT of the spine (13.7%). Four of the seven patients had presented to our institution with a spine recurrence after previous treatments and the rest developed recurrences later. The treatments for the lung nodules consisted of metastectomy in two and chemotherapy in six patients. At the latest follow-up (ranging from 18 to 126 months), two had died of the disease, two had no evidence of the disease, and three were alive with disease. Our series shows a higher metastatic rate from spine GCT as compared to those from the extremities, but the overall behaviour and treatment outcomes of the lung metastases are similar. When there is a recurrence of GCT, with or without metastases, the local and possibly the metastases should be biopsied to confirm the original diagnosis. Progression of benign GCT into an aggressive sarcoma has been documented, and the method of management should be altered.

Surgical management of metastatic spine tumors.

Recent advances in diagnostic tests and radiologic imaging, and the development of novel chemotherapeutic agents and radiation methods have greatly altered the treatment options in patients who have spinal tumors. Improvements in fundamental understanding of the mechanisms of bone metastases, developments in spinal instrumentation, and recent introduction of recombinant bone morphogenetic proteins for spinal reconstruction offer promising strategies in selected patients. Clear applications of the fundamental surgical oncology still apply to spinal tumors. This article considers recent advances in management of the metastatic tumors to the spine.

Giant cell tumor of the mobile spine: a review of 49 cases.

STUDY DESIGN: This is a retrospective review of 49 cases of giant cell tumor (GCT) of the mobile spine treated surgically. OBJECTIVE: Our goal was to determine which factors influenced local recurrence. SUMMARY OF BACKGROUND DATA: GCT is a benign, locally aggressive tumor that rarely occurs in the spine. The management of local recurrence can be challenging. METHODS: We performed a retrospective analysis of GCTs of the mobile spine managed between 1970 and 2005. Median follow-up was 145 months with a minimum of 2 years or until death. We used the Kaplan-Meier method to test whether Enneking stage, surgery type, and surgical margin had statistically significant impact on local recurrence. The log rank test was used for comparison, and a P value of less than 0.05 was deemed significant. RESULTS: Of the 49 patients, 11 (22%) local recurrences occurred. The latest recurrence occurred at 60 months. Age less than 25 years was associated with a worse relapse-free survival (P = 0.03). En bloc resection was associated with better local control with Enneking stage III tumors (P = 0.01); however, intralesional resection provided adequate control of Enneking stage II tumors. There were 6 (12%) cases of metastasis, and 2 patients died from the progression of their disease. One patient died from the complications of the surgery. CONCLUSION: En bloc resection should be considered for Enneking stage III GCTs of the mobile spine. The choice of en bloc resection must be balanced with the inherent risks of the procedure. Intralesional resection of Enneking stage II tumors provides adequate local control. Patients should be followed for at least 5 years because local relapse can occur late.

Solitary enchondromas of long bones: pattern of referral and outcome.

OBJECTIVES: Enchondromas are benign cartilaginous tumors, often found incidentally and diagnosed by the radiographic appearance. Active growing enchondromas/low grade chondrosarcomas are diagnosed by clinical symptoms and possibly an aggressive appearance on the radiographs. This study aimed to answer the following questions: Who requests a referral? The radiologist reporting a possibility of sarcoma or the referring physician? What is the outcome of these patients? METHODS: We retrospectively reviewed the medical records of 115 patients with final diagnosis of enchondroma over three consecutive years and recorded the radiological diagnosis on report, patients' symptoms, our initial diagnosis, follow-up, and any decision for a biopsy/surgical management, as well as the histological final diagnosis. RESULTS: Nearly 80% of patients were referred from an orthopedic surgeon. About half of the imaging reports mentioned a malignancy in the differential diagnosis of enchondroma. Very few had the classic signs of an aggressive/growing cartilage tumor. In radiological evaluation, we found scalloping/cortical erosion, lytic areas, cortical breaks, soft tissue extension in only 12 cases of which 8 underwent a biopsy. Of the study patients, 65% were diagnosed with adjacent joint problems. CONCLUSION: Enchondromas are mostly diagnosed incidentally. They are frequently associated with adjacent joint or soft tissue pathologies, which are main source of the symptoms. Even small, well-defined lesions are often confused with a sarcoma or other malignancies, which may be due to the lack of education on bone tumors for both the radiologists and general orthopedists.

Iatrogenic seeding of a giant cell tumor of the patella to the proximal tibia.

Iatrogenic transplantation of tumor is a rare but avoidable complication, often resulting in additional morbidity or even mortality. We present a case of a 22-year-old woman with a giant cell tumor of the patella initially treated by curettage and bone grafting from the ipsilateral proximal tibia. Local recurrence and tumor growth in the proximal tibia required extensive surgeries. We will discuss the risk of iatrogenic metastasis by direct implantation, often attributable to inadequate surgical planning or poor surgical techniques. The cause and prevention of implantation metastasis are discussed and the basic principles of musculoskeletal tumor management emphasized.

Minimally invasive management of a floating prosthesis injury with locking plates.

Periprosthetic fractures involving a total knee arthroplasty pose a challenging treatment problem with a prevalence of up to 2.5% in the literature (Instr Course Lect 2001;50:379-389). The supracondylar region of the femur is commonly involved, often with minimal available bone in contact with the components. The clinical challenges are particularly more complex in the case of a combined distal femoral and proximal tibial periprosthetic fracture. This injury is considered a "floating prosthesis" injury because of the complete separation of the prosthesis from the remaining skeleton. In this report, a floating prosthesis injury, in combination with a femoral shaft fracture, is treated with 2 locking plates using a minimally invasive technique, with limited blood loss, immediate pain relief after surgery, and successful healing of all fractures with minimal deformity.

Superior dislocation of the patella with early onset patellofemoral arthritis: a case report and literature review.

Among the traumatic patellar dislocations, superior dislocation of patella without patellar ligament injury is very rare. We present in this article, a case of superior dislocation of the patella trapped by interlocked osteophytes in a 38-year-old female who had concurrent bilateral knee arthrosis. Successful reduction was achieved by closed manipulation without anesthesia. Neither redislocation nor symptoms of instability was seen after 36 months of follow-up, although some progression of arthritis was observed. A new classification including all traumatic patellar dislocation was also proposed.

Gamma probe assisted biopsy of suspected metastatic rib lesions.

Retrieving diagnostic tissue from a rib lesion can be challenging. Using a hand-held intraoperative gamma probe to target and biopsy the areas of increased radioisotope uptake has been limited largely to use by thoracic surgeons and interventional radiologists. Such techniques also have been used by orthopaedic oncologists in localizing osteoid osteomas. We pursued a similar technique in localizing the rib lesion. During the 10 months, two patients with a history of cancer and recent bone scans indicative of possible rib metastasis required biopsies for definitive tissue diagnosis. Both patients had gamma-probe localization of their rib lesions intraoperatively using minimally invasive techniques. The operation of the probe was simple with a short learning curve. Both patients had biopsies that yielded diagnoses verifying the abnormality on the staging bone scan. Localization was sensitive and accurate with histologic confirmation in both patients. The length and extent of surgery were markedly reduced with no complications. These results match those reported in the literature by thoracic surgeons and radiologists. The hand-held gamma probe assisted biopsy of suspicious rib abnormalities can be an effective surgical technique that the orthopaedic surgeon should consider. Additional experience with the technique will allow an assessment of the sensitivity and specificity.

Chemotherapy for bone sarcoma does not affect fertility rates or childbirth.

There have been unprecedented improvements in the survival of patients with bone sarcomas because of advances in chemotherapy during the past two decades. However, along with improved survival, there have been concerns of gonadal toxicity and fertility. These problems with chemotherapy are well documented for conditions like lymphomas and testicular cancers. There are few reports on fertility outcomes with chemotherapy for bone sarcomas. The purpose of this study was to review the rate of successful conceptions, pregnancy outcomes, incidence of birth defects, and fertility rates in young adults who had chemotherapy for high-grade bone sarcomas. A retrospective chart review of all eligible patients was done. No laboratory assessment of fertility was done. Fifteen of the 36 patients attempted conceptions, and all were successful [corrected] One miscarriage occurred and one medical termination of pregnancy was done because of a spinal metastasis. There were 13 successful full-term pregnancies with no birth defects. The conception rate was 1.6. Despite the common misconception of probable infertility, these patients can have high expectations to conceive, with uneventful childbirth and no birth defects in the newborns. Although counseling should include the possibility of infertility, patients also should be reminded of the high rate of success of having a normal conception and childbirth.