scholarly journals Primary atlantoaxial bone tumors in children: management strategies and long-term follow-up

2014 ◽  
Vol 13 (3) ◽  
pp. 260-272 ◽  
Author(s):  
Arnold H. Menezes ◽  
Raheel Ahmed
Keyword(s):  
Fibrous Dysplasia ◽  
Needle Biopsy ◽  
Bone Tumors ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Eosinophilic Granuloma ◽  
Bone Cysts ◽  
Aneurysmal Bone Cysts

Object Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up. Methods: The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010. Results Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2–18 years). Chordomas received proton or LINAC irradiation, and as of 4–15 years of follow-up, no recurrence has been noted. Conclusions Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.

Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study.

1990 ◽  
Vol 8 (10) ◽  
pp. 1664-1674 ◽  
Author(s):  
M E Nesbit ◽  
E A Gehan ◽  
E O Burgert ◽  
T J Vietti ◽  
A Cangir ◽  
...  
Keyword(s):  
Overall Survival ◽  
Local Recurrence ◽  
Multimodal Therapy ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Patient Characteristics ◽  
Group I ◽  
Long Term Follow Up

A total of 342 previously untreated eligible children were entered into the first Intergroup Ewing's Sarcoma Study (IESS) between May 1973 and November 1978. In group I institutions, patients were randomized between treatment 1 (radiotherapy to primary lesion plus cyclophosphamide, vincristine, dactinomycin, and Adriamycin [doxorubicin; Adria Laboratories, Columbus, OH] [VAC plus ADR]) or treatment 2 (same as treatment 1 without ADR), and group II institutions randomized patients between treatment 2 or treatment 3 (same as treatment 2 plus bilateral pulmonary radiotherapy [VAC plus BPR]). The percentages of patients relapse-free and surviving (RFS) at 5 years for treatments 1, 2, and 3 were 60%, 24%, and 44%, respectively. There was strong statistical evidence of a significant advantage in RFS for treatment 1 (VAC plus ADR) versus 2 (VAC alone) (P less than .001) and 3 (P less than .05) and also of treatment 3 versus 2 (P less than .001). Similar significant results were observed with respect to overall survival. Patients with disease at pelvic sites have significantly poorer survival at 5 years than those with disease at nonpelvic sites (34% v 57%; P less than .001). Among pelvic cases, there was no evidence of differing survival by treatment (P = .81), but among nonpelvic cases, there was strong evidence of differing survival by treatment (P less than .001). The overall percentage of patients developing metastatic disease was 44%; the percentages by treatments 1, 2, and 3 were 30%, 72%, and 42%, respectively. The overall incidence of local recurrence was 15%, and there was no evidence that local recurrence rate differed by treatment. Patient characteristics related to prognosis, both with respect to RFS and overall survival experience, were primary site (nonpelvic patients were most favorable) and patient age (younger patients were more favorable).

Surgical treatment for primary spinal aneurysmal bone cysts: experience from Children's Hospital Boston

2012 ◽  
Vol 9 (3) ◽  
pp. 305-315 ◽  
Author(s):  
Georgios Zenonos ◽  
Osama Jamil ◽  
Lance S. Governale ◽  
Sarah Jernigan ◽  
Daniel Hedequist ◽  
...  
Keyword(s):  
Spinal Instrumentation ◽  
Neurological Deficits ◽  
Preoperative Embolization ◽  
Bone Cysts ◽  
Children's Hospital ◽  
Initial Intervention ◽  
Children’S Hospital ◽  
Aneurysmal Bone Cysts

Object Spinal aneurysmal bone cysts (ABCs) constitute a rare and clinically challenging disease, primarily affecting the pediatric population. Information regarding the management of spinal ABCs remains sparse. In this study the authors review their experience with spinal ABCs at Children's Hospital Boston. Methods The medical records of all patients treated surgically for primary spinal ABCs between January 1998 and July 2010 were retrospectively reviewed. Results Fourteen cases were identified (6 males and 8 females, ages 5–19 years old). The ABCs were located throughout the spine, with an equal number in the thoracic and lumbar spine, and rarely in the cervical spine. The majority of patients presented with back pain, but neurological deficits and spinal deformity were common. A variety of radiographic techniques were used to establish the diagnosis, including needle biopsy. Preoperative selective arterial embolization was performed in 7 cases (50%), and the majority of cases required spinal instrumentation along with resection. Mean follow-up was 55.9 months (range 15–154 months) after initial intervention. Two ABCs recurred (14%), at 9 months and 8 years after incomplete initial resection, and the patients underwent reoperation. Complete resection was ultimately achieved in all cases. All patients were asymptomatic and neurologically intact at their last follow-up evaluation, and showed no evidence of deformity or recurrence on imaging. Conclusions Computed tomography and MR imaging are adequate for an initial evaluation of spinal ABCs, although solid variants can present a diagnostic challenge. Given the high rates of recurrence with residual disease, complete obliteration of the lesion should be the goal of treatment. Preoperative embolization is often performed, although in the authors' opinion the degree of bleeding tends not to support its routine use. Long-term follow-up is warranted as recurrences can occur years after initial intervention. However, gross-total excision in conjunction with spinal stabilization, as needed, usually provides cure of the ABC and excellent long-term spinal alignment.

Long-term follow-up of Ethibloc injection in aneurysmal bone cysts

2009 ◽  
Vol 18 (6) ◽  
pp. 375-380 ◽  
Author(s):  
Harvey L. George ◽  
Puthanveettil Nithin Unnikrishnan ◽  
Neeraj K. Garg ◽  
Jayanth Sundar Sampath ◽  
Alf Bass ◽  
...  
Keyword(s):  
Bone Cysts ◽  
Long Term Follow Up ◽  
Aneurysmal Bone Cysts

Ewing's sarcoma: Long-term follow-up in 49 patients treated from 1967 to 1989

1993 ◽  
Vol 25 (3) ◽  
pp. 431-438 ◽  
Author(s):  
Hedy Mameghan ◽  
Richard J. Fisher ◽  
Darcy O'Gorman-Hughes ◽  
Edward H. Bates ◽  
Ronald L. Huckstep ◽  
...  
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Long Term Follow Up

scholarly journals Outcome of Ewing's sarcoma of the scapula–a long-term follow-up study

2020 ◽  
Vol 106 (1) ◽  
pp. 25-30
Author(s):  
Shahbaz S. Malik ◽  
Muaaz Tahir ◽  
Usman Ahmed ◽  
Scott Evans ◽  
Lee Jeys ◽  
...  
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Follow Up Study ◽  
Long Term Follow Up

Cyclophosphamide-induced hemorrhagic cystitis in Ewing's sarcoma.

1988 ◽  
Vol 6 (1) ◽  
pp. 76-82 ◽  
Author(s):  
T J Stillwell ◽  
R C Benson ◽  
E O Burgert
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Hemorrhagic Cystitis ◽  
Significant Loss ◽  
Gross Hematuria ◽  
Primary Surgery ◽  
Cyclophosphamide Treatment ◽  
Long Term Follow Up

Recent improvements in survival of patients with Ewing's sarcoma have been made since the addition of cyclophosphamide-based adjuvant chemotherapy to primary surgery and radiation. A potential limitation to cyclophosphamide use is its urotoxicity, primarily in the form of hemorrhagic cystitis. The incidence of this adverse effect in patients treated for Ewing's sarcoma has not been established. In a Mayo Clinic series of 116 patients with Ewing's sarcoma treated with cyclophosphamide, 17 (15%) developed hemorrhagic cystitis diagnosed on the basis of gross hematuria or cystoscopic findings (or both). Microscopic hematuria also occurred in 53% of patients (56 of 105 examined). The dose and duration of therapy appeared to be unrelated to the development of urotoxicity. Most patients recovered uneventfully with or without discontinuation of cyclophosphamide therapy, but a significant loss of blood occurred in three patients, and one patient required a cystectomy because of bladder fibrosis. Long-term follow-up is mandatory in these patients because of late recurrences of hemorrhagic cystitis or the possibility of bladder carcinoma. New therapies, directed at protecting the bladder from urotoxicity during cyclophosphamide treatment, are available.

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