Solitary juvenile xanthogranuloma in the spine pretreated with neoadjuvant denosumab therapy followed by surgical resection in a 5-year-old child: case report and literature review.

PURPOSE: We present a case report that describes neoadjuvant denosumab therapy initiated in a child with a solitary giant cell-rich juvenile xanthogranuloma tumor involving the spine, and review the current literature. METHODS: A giant cell-rich histiocytic lesion involving the 11th thoracic vertebral body was identified in a healthy 5-year-old girl with persistent back and pelvic pain for several months. Imaging examinations and an open biopsy were performed to obtain a definite pathologic diagnosis. As the tumor appeared to be aggressive in nature, we administered adjuvant therapy with denosumab preoperatively and then performed a total spondylectomy. RESULTS: Histopathology confirmed that the tumor was juvenile xanthogranuloma. No tumor metastases or recurrence were detected at the 3-year follow-up, and the patient was asymptomatic. CONCLUSIONS: In giant cell-rich tumors, denosumab is occasionally used as neoadjuvant or adjuvant therapy, especially for tumors in difficult locations or with substantial soft tissue extensions. Rare adverse events in children include skin infections and disruption of calcium homeostasis. Surgical treatment is aimed at removing the tumor and relieving the symptomatic spinal cord compression. Use of denosumab as neoadjuvant therapy for juvenile xanthogranuloma involving the spine has not been reported previously.

Early Delivery of Sacrococcygeal Teratoma with Intraspinal Extension.

Sacrococcygeal teratoma (SCT) with intraspinal extension is rare. There is a risk of paraplegia associated with prolonged spinal cord compression. We present the case of an infant with a prenatal diagnosis of an SCT with a large intraspinal component that was causing compression of the lower spinal cord. Ultrasound at 33 weeks showed bilateral lower extremity and foot movement without hydrops or cardiac failure. Multidisciplinary decision was made to administer betamethasone and proceed with Cesarean delivery at 34 weeks. A vigorous live-born female was delivered and a multilevel laminectomy was performed at day of life 4. The pelvic resection was performed at 4 months. Pathology revealed mature teratoma. She had an uncomplicated postoperative course, is ambulatory, continent of stool, and has no evidence of recurrence. We conclude that intraspinal extension of SCT should be evaluated prenatally with ultrasound and fetal MRI. If there is concern for spinal cord compression, early delivery and urgent decompressive laminectomy may diminish the neurologic sequelae of prolonged spinal cord compression. Since these cases are rare, risks of prematurity need to be weighed against the neurologic risks. These infants should be treated with a multidisciplinary approach.

[Giant Ganglioneuroma of the Spine and Mediastinum]. / Objemný ganglioneurom pátere a mediastina.

UNLABELLED: The case of a 15-year-old girl with a large ganglioneuroma in the mediastinum and spinal canal is presented. The tumour initially manifested as scoliosis. Its diagnosis was made on the basis of CT scanning and magnetic resonance imaging, and confirmed by thoracoscopic mediastinal biopsy. Radical tumour excision was indicated. The first stage involved removal of the tumour from the spinal canal through a posterior approach, and transpedicular fixation of the spinal column with correction of the curve. At the second-stage procedure, the tumour was removed from the pleural cavity and mediastinum through thoracotomy. At two years after surgery, the spondylodesis was completed with autologous bone grafts that healed within 6 months. At 3-year follow-up the patient was with neither clinical findings, nor subjective complains and imaging methods showed no signs of tumour recurrence. KEY WORDS: ganglioneuroma, scoliosis, correction, thoracotomy, spinal canal, mediastinum.

Palliative embolization for metastases of the spine.

BACKGROUND: To present palliative selective and superselective arterial embolization with N-butyl-cyanoacrylate for cancer patients with spinal metastases. MATERIALS AND METHODS: We studied the files of 164 cancer patients (94 men and 70 women; mean age 57.6 years; range 35-81 years) treated from March 2003 to March 2013 with 178 selective arterial embolization procedures for metastases of the spine from variable primary cancers. We evaluated the technical success of the embolization procedure with post-procedural angiography, the clinical effect in pain relief, need for analgesics and tumor size reduction, and the embolization-related complications. RESULTS: Post-embolization angiography showed complete occlusion of the pathological feeding vessels in all procedures. Pain score and need for analgesics reduced by 50 % in 159 patients (97 %); no response was achieved in five patients with metastases of the sacrum. The mean duration of pain relief was 9.2 months (range 1-12 months). Metastatic tumor size reduced from a mean of 5.5 cm (range 3.5-7.5 cm) pre-embolization to a mean of 4.5 cm (range 3-5 cm) at the 6-month follow-up; the difference was not statistically significant. Ninety-three patients (56.7 %) experienced embolization-related complications the most common being post-embolization syndrome (80 patients, 48.8 %) followed by leg paresthesias (ten patients, 6 %), and rupture of a lumbar artery (one patient, 0.6 %). CONCLUSION: Selective arterial embolization with N-butyl-cyanoacrylate should be considered for pain palliation of patients with metastases of the spine. However, pain relief is temporary, and complications, although minor may occur.

Hypervascular cervical spine metastases: embolization by direct injection of Onyx-18.

PURPOSE: Spinal metastases are common in patients with cancer. Following lung and liver, spine is the most common site for cancers to metastasize. Many of them are hypervascularized. These cases are a particular challenge for the surgeon and represent a significant danger of massive blood loss during surgery. Hypervascularized metastases of the cervical spine also include the risk of postoperative bleeding with severe neurological impairment. We report a case of a 67-year-old women with breast cancer (BC) metastasis within the vertebral bodies of C3 and C4 with nearly complete bony destruction of the ventral column and intraspinal tumor masses compressing the spinal cord at level C3 and C4. The hypervascularized tumor was supplied by multiple minor vessels from both vertebral arteries, too small to be coiled individually. Due to an allergy to aspirin, intravascular stenting of the vertebral arteries was not an option. We decided to perform a preoperative direct injection of onyx-18 for embolization of the tumor. CONCLUSION: Presurgical direct injection of Onyx-18 for treating hypervascular spinal metastases of breast cancer seems to be an effective and safe technique and reduces intraoperative bleeding to a minimum.

[Kyphoplasty combined with intraoperative radiotherapy (Kypho-IORT). Alternative therapy for patients with oligometastatic spinal metastases]. / Kyphoplastie kombiniert mit intraoperativer Radiotherapie (Kypho-IORT). Therapiealternative für den oligometastasierten Patienten mit Wirbelsäulenmetastasen.

BACKGROUND: Due to a more effective systemic therapy the survival of patients suffering from malignant tumors has been significantly improved but a longer life span is often associated with a higher incidence of osseous metastases. The majority of these metastases are localized in the spine causing pain, instability and neurological impairments. The interdisciplinary management of spinal metastases previously consisted of stabilization followed by fractionated external body radiation therapy. A reduction in procedural severity and morbidity as well as consideration of self-sufficiency and hospitalization time are important target parameters for these palliative patients. METHOD AND RESULTS: Kyphoplasty combined with intraoperative radiotherapy (Kypho-IORT) is one of several modern treatment options, which involves a minimally invasive procedure with local high-dose transpedicular irradiation of the spine with low-energy (50 kV) X-rays. Immediately following irradiation, stabilization of the spine is carried out using kyphoplasty via the same access route so that a single stage procedure with excellent pain reduction and good local tumor control can be achieved. This article presents clinical data for this procedure and the different fields of indications are critically reviewed and compared to other therapy options. Methodological improvements and options for further individualization of therapy are demonstrated. CONCLUSION: The Kypho-IORT procedure is a safe, feasible and beneficial modern treatment option for instant stabilization and local tumor control in patients with spinal metastases. More than 100 operations have been successfully performed so that the method can be deemed suitable for inclusion in the clinical routine. A phase II dose escalation study has now been completed and submitted for publication and a 2-arm non-inferiority trial (phase III study) for comparison with conventional irradiation is in progress.

Primary spine tumors: diagnosis and treatment.

BACKGROUND: Primary tumors are rare and those localized to a single location offer the potential for cure. To achieve this, early recognition of the primary tumor and proper workup and treatment are essential. METHODS: The authors reviewed the literature and best practices to provide recommendations on primary spine tumor treatment. Appropriate workup of primary spine tumors and treatment algorithms are also discussed. RESULTS: Patients suspected of a primary spine tumor should undergo fine-needle aspirate biopsy following consultation with the surgical team to ensure the biopsy tract is surgically resectable should the need arise. Once pathology is confirmed, metastatic workup should be performed to guide the level of treatment. If a localized lesion with poor radiation and chemotherapeutic response is diagnosed, then en bloc resection may be required for cure. If en bloc resection is not feasible or metastatic lesions are present, then radiation and medical oncology specialists must work in conjunction with the surgical team to determine the best treatment options. CONCLUSIONS: Patients with suspected primary tumors of the spine should be treated in a multidisciplinary fashion from the outset. With thoughtful management, these lesions offer the opportunity for surgical cure.

Controversial issues in kyphoplasty and vertebroplasty in malignant vertebral fractures.

BACKGROUND: Kyphoplasty (KP) and vertebroplasty (VP) have been successfully employed in the treatment of pathological vertebral fractures. METHODS: A critical review of the medical literature was performed and controversial issues were analyzed. RESULTS: Evidence supports KP as the treatment of choice to control fracture pain and the possible restoration of sagittal balance, provided that no overt instability or myelopathy is present, the fracture is painful and other pain generators have been excluded, and positive radiological findings are present. Unilateral procedures yield similar results to bilateral ones and should be pursued whenever feasible. Biopsy should be routinely performed and 3 to 4 levels may be augmented in a single operation. Higher cement filling appears to yield better results. Radiotherapy is complementary with KP and VP but must be individualized. CONCLUSIONS: In cases of painful cancer fractures, if overt instability or myelopathy is not present, unilateral KP should be pursued, whenever feasible, followed by radiotherapy. The technological advances in hardware and biomaterials, as well as combining KP with other modalities, will help ensure a safe and more effective procedure. Address.

Safety and efficacy of percutaneous vertebroplasty and interventional tumor removal for metastatic spinal tumors and malignant vertebral compression fractures.

OBJECTIVE: The purpose of this study was to determine the safety and efficacy of percutaneous vertebroplasty and interventional tumor removal in the management of metastatic spinal tumors and malignant vertebral compression fractures. SUBJECTS AND METHODS: Thirty-one patients with metastatic spinal tumors and malignant vertebral compression fractures were treated with percutaneous vertebroplasty and interventional tumor removal. Insertion of a 14-gauge needle and guidewire into the vertebral body was followed by sequential dilation of the track with working cannulae until the last cannula reached the anterior portions of the pedicle. Interventional tumor removal was performed with marrow nucleus rongeurs, and 5-10 mL of cement was injected into the treated vertebra. Outcome data (visual analog scale score, Oswestry disability index score, and Karnofsky performance scale score) were collected preoperatively; 1 week and 1, 3, and 6 months after the procedure; and every 6 months thereafter until death. RESULTS: The overall clinical assessment at the last follow-up evaluation showed that pain was completely resolved in 23 patients, decreased in six patients, and unimproved in two patients, yielding a pain relief rate of 94%. The average preoperative visual analog scale score was 7.2, which decreased to 2.4 at 1 month, 1.9 at 6 months, and 1.6 at 1 year and was maintained at 1.3 at the follow-up evaluations performed after more than 1 year. Statistically significant improvement in Oswestry disability index and Karnofsky performance scale scores was also seen between the preoperative evaluation and every follow-up assessment postoperatively (p<0.001). CONCLUSION: Percutaneous vertebroplasty and interventional tumor removal are safe, effective, and minimally invasive palliative therapies for reducing pain and improving function in patients with metastatic spinal tumors and malignant vertebral compression fractures.

Percutaneous ethanol embolization and cement augmentation of aggressive vertebral hemangiomas at two adjacent vertebral levels.

This report describes a case of successful percutaneous direct-puncture ethanol embolization, followed by vertebroplasty, of an aggressive vertebral hemangioma (VH) involving two adjacent thoracic vertebral levels. In this case, the 78-year-old male patient presented with a 6-month history of progressive paraparesis due to spinal cord compression by a T8-T9 VH with an extensive epidural component. Follow-up demonstrated epidural component shrinkage with complete regression of symptoms at 3 months. This case suggests that exclusive percutaneous treatment may be considered for symptomatic VH even when two adjacent vertebral levels are affected.

Failure and success in the treatment of breast carcinoma in men: a case report.

BACKGROUND: Male breast cancer (MBC) represents a rare cause of vertebral body compression fractures along with severe pain restricting the patient's daily functioning. Limited number of cases, lack of awareness among patients and physicians leading to delayed management, further increase the average age and disease progression at presentation, resulting in a poor prognosis. Additionally, studies on MBC treatment protocols and the use of female algorithms are unavailable. The implementation of vertebroplasty or kyphoplasty often results in unsatisfactory outcome due to recurrent pain and loss of vertebral height. Stentoplasty could become an alternative procedure, as described in the following case study. CASE REPORT: 54-yr-old male patient with history of breast carcinoma presented with pain in the vertebral column. Baseline X-ray and CT scan revealed multiple osteosclerotic and osteolytic metastatic lesions in the thoracic vertebrae along with a compression fracture at T9. Stentoplasty was performed to limit fracture progression. Intraoperative scan revealed restoration of the vertebral body shape. Following surgery, direct reduction in pain was obtained. Postoperative 1-year follow-up did not show any loss in height of the operated vertebra. Results of adjuvant chemotherapy administration and a new method of treatment of compression fractures caused by metastatic lesions were compared with previously published studies. CONCLUSIONS: Stentoplasty with Vertebral Body Stenting-System is an innovative method that can be applied in kyphoplasty for compression fractures caused by metastatic lesions. Nevertheless, further research on the systemic treatment of MBC is needed.

[Kyphoplasty in combination with intraoperative radiotherapy. Technical and regulatory characteristics of a concept for treatment of vertebral metastases]. / Kyphoplastie in Kombination mit intraoperativer Radiotherapie. Technische und regulatorische Besonderheiten eines Konzepts zur Behandlung von Wirbelkörpermetastasen.

BACKGROUND: Operative and radiotherapeutic procedures are available for the treatment of symptomatic vertebral metastases. The method for treatment of vertebral metastases presented in this article involves a combination of intraoperative radiotherapy (IORT) and kyphoplasty. METHODS AND RESULTS: Kyphoplasty-IORT allows treatment of symptomatic vertebral metastases between vertebrae T3 and L5. With the patient under intubation narcosis an extrapedicular or bipedicular access to the vertebra is selected as for conventional kyphoplasty. This is followed by insertion of special sheaths of the radiation applicator and radiation therapy is intraoperatively administered via a radiation generator (Intrabeam®, Carl Zeiss Surgical, Oberkochen, Germany). The radiation dose is 8 Gy at a depth of 5-10 mm depending on the study protocol (50 kV X-radiation). Following radiation a conventional kyphoplasty procedure (Medtronic, USA) is carried out and the vertebra stabilized with cement. CONCLUSIONS: The procedure presented demonstrates a new approach to treatment of vertebral metastases and represents a valuable alternative to previously established methods.

[Learning and teaching abilities of a newly inaugurated operation technique. Analysis of learning curves and transferability exemplified by Kypho-IORT]. / Lehrfähigkeit und Erlernbarkeit einer neu inaugurierten Operationsmethode. Analyse der eigenen Erfahrungskurve und des Weitergabeerfolgs am Beispiel der Kypho-IORT.

The Kypho-IORT procedure is a recently developed surgical technique to combine intraoperative radiotherapy with cement augmentation of the vertebra for spinal metastases. The technical feasibility and the operation principle of this new method have been described. In the following article the refinement of the standard operation procedure and the technical development of the method are described. Not only the procedural improvements but also the learning curves of the inaugurators are pointed out. Moreover, the article presents the measures which were necessary to educate trainees during surgical master classes in this new method and to transfer the method. The learning success was quantified by recording the accuracy reached by the trainees in the key procedure during hands-on cadaver exercises. Improvements of the standard operation procedure could be successfully transferred in a second master class. The method of Kypho-IORT and the demonstrated way of postgraduate education is feasible to instruct trainees. The Kypho-IORT procedure can be learnt and performed safely by running through the surgical master class.

Staging and treatment of osteoblastoma in the mobile spine: a review of 51 cases.

PURPOSE: To define the role of Enneking staging system and of the consequent different treatment options on the outcome of osteoblastoma (OBL) of the spine. METHODS: A retrospective review of 51 patients with OBL of the mobile spine conducted to compare the outcomes among the different types of treatments at long term follow-up (25-229 months, av.90). These 51 patients were previously staged according to Enneking staging system and treatment selected accordingly. 10 stage two (st.2) OBLs were treated with intralesional excision and 41 stage three (st.3) OBLs were treated either by intralesional excision or en bloc resection. The intralesional excision group was divided considering the use or not of radiation therapy after surgery. The recurrence rate was compared among these groups and also considering previous open surgery ("non intact" vs. "intact"). The statistical significance was defined using the Fisher Exact test. RESULTS: No local recurrence occurred in the st.2 patients treated by intralesional excision. Considering the st.3 patients, 2 local recurrences out of 13 patients occurred in the en bloc resection (15.4 %) group. All occurred in "non intact" cases (67 %). In the intralesional group, 5 local recurrences out of 27 patients occurred (18 %) being none in the group that received radiation therapy after surgery. Two occurred in the "intact" (7 %) and three in the "non intact" group (75 %). Considering all patients, the difference between the recurrence rate between "intact" and "non intact" groups was statistically significant (p < 0.002). CONCLUSIONS: Intralesional excision proved to be effective in st.2 lesions and en bloc resection in st.3. Radiotherapy seems to be an effective adjuvant treatment when en bloc resection is not feasible or requires unacceptable functional sacrifices. The first treatment significantly affects the prognosis as previously treated patients have worse prognosis.

[Role and limitations of vertebroplasty and kyphoplasty in the management of spinal metastases]. / Rolle und Grenzen der Vertebro-/Kyphoplastie im Metastasenmanagement der Wirbelsäule.

The spine is the most common site for skeletal metastases. Tumor-induced osteolysis may lead to pain, dysfunction and ultimately vertebral fracture. In some patients conventional surgery is not suitable because of the palliative therapy approach. Just for this patient population it was shown that cement augmentation of the vertebra is an effective therapy option and plays an important role. Nevertheless, cement augmentation of the vertebra has its limitations and should only be applied by appropriate indications.

Lower-limb progressive paraparesis management and diagnosis overview in a pregnant woman with vertebral haemangioma.

Vertebral haemangioma is a benign vascular tumour mostly seen in the thoracic region of the spine. Spinal haemangiomas are usually asymptomatic and are discovered incidentally. In a few patients, however, aggressive vertebral haemangiomas can cause local pain, radicular pain or neurologic deficits, which result from neural compression. The aetiology of the origin is unclear and is probably multifactorial. Hormonal and biological changes in pregnant women can lead to accelerated vascular growth of haemangioma. In our report, we present the case of a pregnant patient who was diagnosed with an aggressive vertebral haemangioma that further led to progressive paraparesis. We had to take the fact that she was pregnant into account in the diagnostic procedure, in the choice of examination method and also in the method of therapy. The goal of this case report is threefold: (1) provide an overview of the possible methods of management, specifically imaging, which will aid in diagnosis and based on that, (2) determining the appropriate therapy and (3) review the risks and benefits of each will be presented when choosing individual approaches.

A 68-Year-Old Woman with a Remote History of Breast Cancer Presenting with Low Back Pain to a Chiropractic Clinic in Hong Kong with Imaging Findings Consistent with a Vertebral Hemangioma and Vertebral Metastatic Lesions.

BACKGROUND Patients commonly visit chiropractic clinics for treatment for low back pain, which is often due to injury or degenerative spinal conditions. Rarely, serious underlying pathology may be identified. This report describes a 68-year-old woman with a remote history of breast cancer presenting with low back pain to a chiropractic clinic in Hong Kong with imaging findings consistent with vertebral hemangioma and vertebral metastatic lesions. CASE REPORT A 68-year-old woman with a history of breast cancer status after chemotherapy and mastectomy 20 years prior presented to a chiropractor with an acute exacerbation of chronic low back pain with lower extremity paresthesia. She previously visited her general practitioner and underwent radiography, which supported diagnoses of degenerative lumbar spondylosis and hemangioma of the fifth lumbar vertebra. Given the patient's worsening status and previous cancer, the chiropractor ordered lumbar magnetic resonance imaging at the initial visit, consistent with multilevel spinal metastasis. The chiropractor referred the patient to an oncologist who performed positron emission tomography/computed tomography, which suggested breast cancer recurrence and metastasis. The greatest hypermetabolic activity was evident within the level of the suspected vertebral hemangioma, suggesting this finding which initially appeared innocuous on plain radiography contained underlying metastasis. CONCLUSIONS This case illustrates that when patients fail to respond to treatment for low back pain, clinical referral should be undertaken for investigations to identify possible serious underlying pathology.

Percutaneous vertebroplasty as a treatment for painful osteoblastic metastatic spinal lesions.

Percutaneous vertebroplasty (PVP) has been used widely to treat pain caused by osteolytic spinal lesions, whereas vertebroplasty for osteoblastic spinal lesions is less known. The purpose of this study is to describe PVP as a highly effective miniinvasive procedure to treat painful osteoblastic metastatic spinal lesions. Four patients with painful osteoblastic metastatic spinal lesions were treated by PVP in the authors' department, and immediately relief of pain was achieved in all of them. The findings from this study may encourage more studies of PVP in palliative treatment of patients with osteoblastic lesions.

Intratumoural hydrogen peroxide injection during resection of metastatic vertebral melanoma. Technical note.

It is well recognized that the surgical removal of vertebral metastases of several malignant tumours often causes extensive bleeding, increasing the difficulties for surgeons and the risks for patients. Among the different subtypes of malignancies involved, melanoma has an extensive vascularity, and its resection is frequently challenging because of a continuous and annoying bleeding. A 55-year-old patient with a history of treated melanoma was operated on urgently for a severe T10-11 spinal cord compression by a huge metastasis involving the peridural space and the vertebral bone. During the resection, we injected, in successive stages, a total of 5 ml of 3% hydrogen peroxide (H(2)O(2)) solution, with good control of tumour bleeding. The patient had no problems related to the procedure, and 6 months after the surgery he is able to perform sustained walking. The aim of this manuscript is to report a safe, simple, effective, inexpensive and readily available technique for intraoperative haemostasis that could allow the control of tumour bleeding and facilitate its excision.