From Sacrum to Spine: A Complex Case of Sacrococcygeal Teratoma.

Neonatal tumors occur infrequently; sacrococcygeal teratoma (SCT) is a rare and abnormal mass often diagnosed on antenatal ultrasound. An SCT may cause serious antenatal complications, requires surgery in the neonatal period, and can lead to various long-term sequelae including fecal incontinence or constipation, urinary incontinence, and lower extremity mobility impairment. Even rarer are SCTs that include intraspinal extension necessitating complex neurosurgical intervention to relieve possible spinal cord compression or tumor tissue resection. A comprehensive understanding of the natural history of SCT provides frontline neonatal nurses and nurse practitioners with the expertise and language to support families during an infant's NICU admission. A glossary of key terms accompanied by a case review of a premature infant born with a large external SCT with intrapelvic and intraspinal components aids in enhancing knowledge related to the potential impact of an SCT on the central nervous system.

Neoadjuvant denosumab for the treatment of a sacral osteoblastoma.

PURPOSE: To present a case of aggressive sacral osteoblastoma (OB) treated with neoadjuvant denosumab therapy and en bloc resection. METHODS: Case report of a 14-year-old male with an aggressive OB affecting the superior articular process of the left first sacral segment. The lesion was lytic and metabolically active and involved the left-sided posterior elements of S1-S3 with extension into the spinal canal, affecting the left S1, S2, S3, S4 and S5 nerve roots. He was treated for 1 month with neoadjuvant denosumab followed by en bloc resection. RESULTS: Denosumab therapy caused regression of the tumour and converted the diffuse infiltrative mass into a well-defined solid (osteoma-like) structure, aiding surgical resection and preserving the S1, S4 and S5 nerve roots. Histologically, the treated lesion showed abundant sclerotic woven bone and osteoblasts with absence of osteoclasts. CONCLUSIONS: A short course of denosumab caused tumour regression, ossification and conversion of an aggressive OB into a sclerotic, well-defined lesion thus aiding surgical resection and preservation of neural structures. Neoadjuvant therapy reduced osteoclast numbers but PET showed that the lesion remained FDG avid post-therapy.

Volumetric Modulated Arc Therapy as an Example of the Advanced Capability of Modern Radiotherapy.

BACKGROUND: Family physicians and internal medicine specialists play an essential role in treating cancer patients. Modern technological advances in radiotherapy are not widely appreciated by primary care physicians. Bone metastases are a frequent complication of cancer. Palliative radiation therapy, as a component of modern advances in radiation treatments, should not subject normal bodily structures to excessive doses of irradiation. The sacrum is a common destination site for bone metastases, yet its concave shape along with its proximity to the rectum, intestines, and femoral heads creates treatment-planning challenges. OBJECTIVES: To investigated whether the volumetric modulated arc therapy (VMAT) technique is preferable to more conventional radiation strategies. METHODS: The study comprised 22 patients with sacral metastases who were consecutively treated between 2013 and 2014. Two plans were generated for the comparison: three-dimensional (3D) and VMAT. RESULTS: The planning target volume (PTV) coverage of the sacrum was identical in VMAT and 3D planning. The median values for the rectal dose for 3D and VMAT were 11.34 ± 5.14 Gy and 7.7 ± 2.76 Gy, respectively. Distal sacral involvement (S4 and S5) was observed in only 2 of 22 cases, while the upper pole of the rectum ended at the level above S3 in just 3 cases. CONCLUSIONS: Radiation therapy continues to be an integral component of the palliative armamentarium against painful metastases. Radiation oncologist, in conjunction with referral physicians, can tailor treatment plans to reflect the needs of a given patient.

[Pulmonary Metastasis of Sacral Chordoma Fifteen Years after Resection of Primary Site;Report of a Case].

A 71-years-old man, who had undergone resection for sacral chordoma 15 years before, was admitted to our hospital to treat a nodule in the right middle lobe detected by computed tomography. The nodule was resected and was histologically diagnosed as lung-metastasis of chordoma.

Alveolar soft-part sarcoma in the sacrum: a case report and review of the literature.

Alveolar soft part sarcoma (ASPS) is a rare disease of the soft tissue. Although the disease is rare, it is refractory to chemotherapy and radiation. En bloc surgical resection offers the best chance of cure. In this article we report the case of a 28-year-old woman who presented with buttock and leg pain, bowel, bladder and gait impairment and a large mass in the sacrum. Following surgical excision, the lesion was proven to be ASPS. On pathology, the mass was TFE3 (transcription factor E3) positive, indicating the presence of the ASPL-TFE3 (novel gene-transcription factor) translocation. Following surgery, the patient had improvement in her pain and ambulation; however, she refused adjuvant therapy to pursue hospice care and succumbed to her disease 2 years after surgery. On a review of the literature, it was found that ASPS of the bone constitutes a rare and formidable subset of this disease. Further, metastases related to ASPS are common in the lungs, liver, brain, and lymph nodes. The degree of dissemination is a predictor of outcome, with 5-year survival of 81-88% in patients with local disease and only 20-46% in patients with metastatic disease at the time of presentation. Brain metastases at the time of presentation portend the worst prognosis.

Solitary spinal epidural cavernous angioma: report of nine surgically treated cases and review of the literature.

PURPOSE: We intend to report the largest series of spinal epidural cavernomas (SEC), discuss their clinical features, imaging characteristics, surgical findings, outcome analysis and compare them with similar reports in the literature. METHODS: Among the cases of spinal tumors treated surgically by the authors, there have been nine cases of SEC's. All the data were collected prospectively and the cases have been followed after surgery up to the time of this analysis. RESULTS: There were six female and three male patients with the ages ranging between 13 and 74 years. The lesions were located in the thoracic spine (4 cases), lumbar spine (4 cases) and one at the sacral level. Clinical presentations included acute spinal pain and paraparesis in two, low back pain and radiculopathy in five, and slowly progressive myelopathy in the other two cases. The lesion was iso-intense with the spinal cord in T1W images and hyperintense in T2W images and showed strong homogeneous enhancement after contrast medium injection in most of our cases. In the presence of hemorrhage inside the lesion, it was hyperintense in both T1W and T2W MR sequences as in our case 6. In the single case presenting with acute hemorrhage, epidural hematoma was the only finding, our case 1. Complete surgical removal was achieved in all our cases, and confirmed by postoperative MRI. CONCLUSION: SEC is hard to be differentiated from other epidural spinal lesions before intervention but should be considered in the list of differential diagnosis regarding its favorable outcome.

Marfan syndrome and symptomatic sacral cyst: report of two cases.

CONTEXT: Meningeal abnormalities such as dural ectasia are seen in Marfan syndrome, but spinal meningeal cysts are rarely seen. These cysts usually asymptomatic and often found incidentally on magnetic resonance imaging, large cysts may cause neurological deficits and pain secondary to nerve root compression. DESIGN: Case reports. FINDINGS: Two patients with Marfan syndrome presented with urinary symptoms secondary to dural ectasia and sacral cysts. Patient 1 had a history of low back pain, erectile dysfunction, and occasional urinary incontinence and groin pain with recent symptom worsening. He underwent L5 partial laminectomy and S1-S2 laminectomy with sacral cyst decompression. Nine weeks later, he underwent drainage of a sacral pseudomeningocele. Pain and urinary symptoms resolved, and he remains neurologically normal 2 years after surgery. Patient 2 presented after a fall on his tailbone, complaining of low back pain and difficulty urinating. Physical therapy was implemented, but after 4 weeks, urinary retention had not improved. He then underwent resection of the sacral cyst and S1-S3 laminectomy. Pain and paresthesias resolved and bowel function returned to normal. Other than needing intermittent self-catheterization, all other neurologic findings were normal 30 months after surgery. CONCLUSION/CLINICAL RELEVANCE: Surgical goals for sacral cysts include resection as well as closure of the dura, which can be challenging due to thinning from ectasia. Neurosurgical intervention in Marfan syndrome is associated with a high risk of dural tears and osseous complications, and should be performed only when symptoms are severe.

Anterior sacral meningocele in a patient with currarino syndrome as a cause of ileus.

Currarino's syndrome (CS) is characterized by a triad of a sacral bony defect, anorectal malformations and presacral mass, most commonly an anterior sacral meningocele. Since it was first described as a syndrome by Currarino et al. in 1981, approximately 300 cases have been reported in the literature. Diagnosis of CS in adulthood is rare. We present an adult patient with CS, manifesting by an acute intestinal obstruction. To our knowledge, acute intestinal obstruction in an adult as a presentation of CS has not been reported previously. Colostomy was performed first by the general surgery team to relieve intestinal obstruction caused by the giant cyst. After the final diagnosis of anterior sacral meningocele was established, a second operation was performed for the ligation of the cyst neck through a posterior approach. The size of the cyst gradually reduced over time. A staged approach and the multidisciplinary management, with the collaboration of the general surgery and neurosurgery teams, provided a satisfactory clinical outcome.

Sacral schwannoma found incidentally--report of a case.

Sacral schwannoma is a rare retrorectal tumor in adults. Postoperative sacral neurological deficit is difficult to avoid. Currently, there is no established consensus regarding best treatment options. We present a case of a 33 years old patient with atypical discomfort in lower abdomen and no neurological complaints who was diagnosed with a pelvic mass by abdominal ultrasound. CT, MRI and MSCT showed an inhomogeneous presacral mass involving right S1 sacral foramen. Although there were no neurological complaints, EMG and ENG showed a minor chronic lesion of L5 root bilaterally, more on the right side, affecting the fibers to the small muscles of the feet. We treated this patient with total extirpation of the mass without additional curretage. No radiotherapy was applied and postoperative neurological functions were preserved.

Management of Chordoma of the Sacrum and Mobile Spine.

➢: Chordomas account for 1% to 4% of primary tumors of the spine and sacrum. ➢: En bloc resection is the preferred surgical treatment for the management of chordomas. ➢: Proton beam radiation is increasingly being used as a postoperative radiation modality for the treatment of chordomas.

Sacral fractures after lumbosacral fusion: a characteristic fracture pattern.

OBJECTIVE: The purpose of this study was to describe the radiologic pattern of sacral fractures after lumbosacral fusion and to identify clinical characteristics relevant to the radiologic diagnosis. MATERIALS AND METHODS: A search of CT, nuclear medicine, and MRI radiology reports over a 5-year period at our institution revealed a total of 23 patients with sacral fractures after lumbosacral fusion. Two radiologists reviewed all of the images to determine the sacral fracture pattern. The clinical records of these patients were reviewed for interval after surgery, fusion length, hardware, approach, preoperative diagnosis, symptoms, treatment, and risk factors. RESULTS: All 23 sacral fractures were horizontal through the sacral body, involved the screw holes, and exited through the posterosuperior sacral alae. The fractures occurred within 3 months of fusion in 19 of 23 patients. All 23 patients had symptoms at the time of fracture. Seventeen of 23 fusions were long (more than four vertebrae). Four of 23 patients had osteoporosis. Eleven of 23 fractures healed without surgery, and 12 were managed with transiliac fixation. CONCLUSION: Sacral fractures after lumbosacral fusion have a characteristic transverse pattern through the sacral screw holes that differs from the configuration of more common sacral insufficiency fractures. Most of these fractures occur within 3 months after surgery, and many of the patients need additional surgical fixation. Because few of the patients had osteoporosis and most underwent long fusion, the fractures might have been caused by hardware-related stress raisers in the sacrum.

Control of presacral venous bleeding with helical tacks on PTFE pledgets combined with pelvic packing.

Persistent and torrential bleeding from presacral veins is an uncommon complication during rectal resection. Control of bleeding is often difficult. We report an effective technique for controlling sacral bleeding using endoscopic helical tackers applied over pledgets of expanded polytetrafluoroethylene.

Distal sacrectomy for recurrent rectal cancer.

Surgical treatment of recurrent rectal tumors is a challenge for cancer surgeons. Due to the high risk of intraoperative and postoperative complications, such tumors are often considered inoperable. We present a clinical case of 56-years-old male patient with local recurrence and pathological fracture of the sacrum at S5 level to whom en-bloc distal sacrectomy was performed. Such surgical technique for recurrence of rectal cancer provides significant improvement in local and general condition and creates a favorable prognosis for the patient's survival, which is demonstrated by the described clinical case.

Nerve Root Sparing En Bloc Resection of Sacral Chondrosarcoma: Technical Note and Review of the Literature.

BACKGROUND: Total en bloc sacrectomy provides the best long-term local control for large primary bony sacral tumors, but often requires lumbosacral nerve root sacrifice leading to loss of ambulation and/or bowel, bladder, and/or sexual dysfunction. Nerve-sparing techniques may be an option for some patients that avoid these outcomes and accordingly improve postoperative quality of life. OBJECTIVE: To describe the technique for a posterior-only en bloc hemisacrectomy with maximal nerve root preservation and to summarize the available literature. METHODS: A 38-yr-old woman with a 7.7 × 5.4 × 4.5 cm biopsy-proven grade 2 chondrosarcoma involving the left L5-S2 posterior elements underwent a posterior-only left hemisacrectomy tri-rod L3-pelvis fusion. A systematic review of the English literature was also conducted to identify other descriptions of high sacrectomy with distal sacral nerve root preservation. RESULTS: Computer-aided navigation facilitated an extracapsular resection that allowed preservation of the left-sided L5 and S3-Co roots. Negative margins were achieved and postoperatively the patient retained ambulation and good bowel/bladder function. Imaging at 9-mo follow-up showed no evidence of recurrence. The systematic review identified 4 prior publications describing 6 total patients who underwent nerve-sparing sacral resection. Enneking-appropriate resection was only obtained in 1 case though. CONCLUSION: Here we describe a technique for distal sacral nerve root preservation during en bloc hemisacrectomy for a primary sacral tumor. Few prior descriptions exist, and the present technique may help to reduce the neurological morbidity of sacral tumor surgery.

Radiofrequency sensory ablation as a treatment for symptomatic unilateral lumbosacral junction pseudarticulation (Bertolotti's syndrome): a case report.

OBJECTIVE: Describe the clinical presentation, diagnostic evaluation, and successful treatment of a case of symptomatic unilateral lumbosacral junction pseudarticulation using a novel radiofrequency nerve ablation technique. CASE: A 56-year-old female patient who had suffered with low back and right upper buttock pain for 16 years experienced incomplete relief with L4/5 facet joint radiofrequency ablation. She was found to have an elongated right L5 transverse process that articulated with the sacral ala (Bertolotti's syndrome). Fluoroscopically guided local anesthetic/corticosteroid injection into the pseudarthrosis eliminated her residual right buttock pain for the duration of the local anesthetic only. Complete pain relief was achieved by injecting local anesthetic circumferentially around the posterior pseudarthrosis articular margin. Accordingly, bipolar radiofrequency strip thermal lesions were created at the same locations. Complete pain relief and full restoration of function was achieved for 16 months postprocedure. CONCLUSION: This case report describes a novel radiofrequency technique for treating symptomatic lumbosacral junction pseudarticulation that warrants further evaluation.

Giant extradural sacral meningioma.

We present the first case of primary benign fibrous meningioma restricted to the sacrum with no sign of recurrence after a long follow-up duration. This occurred in a 36-year-old man who initially presented with a 3-month history of right-sided sciatica. The tumour was successfully resected after three surgical procedures due to its high vascularity and technical difficulties. Sacroiliac joint stability was not disrupted, and he is still doing well clinically at 8 years follow-up.

Intraosseous sacral paraganglioma with extradural extension: case report.

BACKGROUND: Paragangliomas are tumors that arise from the paraganglion system, which is a component of the neuroendocrine system. Approximately 10% are located in the extra-adrenal paraganglion system. Paragangliomas of the spine, however, are rare. They usually present as an intradural tumor in the cauda equina. There are only three reports of primary intraosseous paragangliomas of the sacrum. CASE DESCRIPTION: A 69-year-old man presented with low back pain and urinary incontinence. Imaging revealed a large intraosseous mass at S2, S3 and S4. Surgical resection was accomplished through a posterior midline incision exposing the spine from L5 to the coccyx. The tumor was located in the extradural space. It was friable, grayish and bleeding. Total tumor removal was performed, with normal bone margins. Follow-up at 2 years showed complete resolution of the preoperative symptoms and no evidence of local recurrence. CONCLUSION: Although rare, the possibility of paraganglioma should be included in the differential diagnosis of sacral tumors. The majority of the spinal paragangliomas are benign, slowly growing tumors with low proliferative activity. Despite these characteristics, local recurrence has been reported in cases of both macroscopically total and subtotal resection. Postoperative radiation therapy for patients with incomplete excision may not prevent recurrence, so gross tumor removal should be the goal of surgery.

Sacroplasty in a patient with bone metastases from hepatocellular carcinoma. A case report.

Bone metastases account for 10% to 30% of secondary tumors in all cancer types. In patients with primary hepatocellular carcinoma (HCC), bone metastases are usually treated by nonoperative procedures including pain medication, radiotherapy, hormone therapy, chemotherapy, and bisphosphonates. Surgical treatments include vertebrectomy, reconstruction with a cage or polymethylmethacrylate bone cement, and stabilization with pedicle screws. Sacroplasty to treat bone metastases from HCC has been rarely reported in the literature. We describe the case of a patient with vertebral metastases of HCC treated by this approach. A 65-year-old man had undergone a hepatic segmentectomy in 2004. In May 2008, after several weeks of back pain and bed rest, the patient underwent computed tomography and magnetic resonance imaging of the abdominal and pelvic spine, which revealed metastatic lesions in S1-S5 on the right and S1-S2 on the left. Sacroplasty was performed on all lesions without complications. The patient was discharged from the hospital the same day of the procedure. Two months later, he reported pain relief and improvement in walking. Due to the technical feasibility, low complication rate, and immediate relief of symptoms, sacroplasty for HCC metastases of the sacrum may be considered a valid therapeutic option.

Sacral mesenchymal chondrosarcoma in childhood: a case report and review of the literature.

Mesenchymal chondrosarcomas are rare malignant tumors in pediatric age group. The authors present a case of mesenchymal chondrosarcoma located in the sacrum in a 10-year-old-girl that was successfully treated with chemotherapy and radiotherapy after surgical excision. According to the authors' literature search, the patient is the first reported case of pediatric sacral primary mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma cases in pediatric age group published in English literature was reviewed.

Results of Chordoma Patients Treated by Different Approaches in a Single Institution.

AIM: To discuss the optimal and recent treatment options based on clinical review of 16 chordoma patients. MATERIAL AND METHODS: Data of the patients diagnosed and treated between 1999 and 2017 in Gazi University School of Medicine has been collected through patients’ files and the electronic database of hospital records. Statistical analysis was applied to evaluate the correlation between the progression free survival and treatment modalities. RESULTS: Nine of the 16 patients were women (56.3%). Half of the patients had intracranially located tumors, whereas the other 50% of the sample had spinal (n=5) and sacral (n=3) chordomas. The median follow-up time was 51.7 months. Recurrence was observed in 50% of patients, while the median recurrence time equaled to 27.6 months. Multivariate analysis results showed that age, gender tumor size, intra or extracranial location of tumor, treatment modalities, subtotal or grosstotal resection of tumor, radiotherapy dose, and techniques were not associated with recurrence. On the other hand, 2 patients are still under chemoterapy (imatinib, bevacizumab) without evident of recurrent disease. CONCLUSION: Despite the fact that surgery remains to be the cornerstone of treatment, total resection is not reasonable for all patients with chordomas. For this reason, adjuvant treatment for ensuring local control is highly important. If the residual tumor is of a small volume, SBRT may provide more advantages. Targeted treatment or chemotheapeutic agents may also be benificial for maintanence therapy. As the clinical awareness about chordomas is based on our series, aggressive multi-modality treatment options should be applied in the adjuvant therapy.