International Journal of Surgery Open 2 (2016) 19–21
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International Journal of Surgery Open j o u r n a l h o m e p a g e : w w w. e l s e v i e r. c o m / l o c a t e / i j s o
Case Report
Huge aneurysmal bone cyst mimicking a kidney tumor: Case report and review of literature Salah Termos a,*, Ahmad Alsaleh a, Haithem Rifaii b, Nijmeh Hammoud-Termos a, Bilal Aoun c a
Department of Surgery, Rafic Hariri University hospital (RHUH), Beirut, Lebanon Department of Orthopedic Surgery, Beirut Arab University (BAU), Beirut, Lebanon c Department of Pediatrics, Rafic Hariri University hospital (RHUH), Beirut, Lebanon b
A R T I C L E
I N F O
Article history: Received 12 February 2016 Received in revised form 2 April 2016 Accepted 2 April 2016 Available online 6 April 2016 Keywords: Aneurysmal bone cyst (ABC) Complete resection Two stage procedure Angiography
A B S T R A C T
Introduction: Aneurysmal bone cyst (ABC) is an uncommon osteolytic lesion that occurs in 1.4 per 100,000 patients (Leithner et al. [1]). ABCs are benign tumors with low risk of malignant transformation (osteosarcoma). Case report: We report a case of a large ABC of the 4th lumbar vertebra resembling a kidney mass that was presented with flank pain, urinary incontinence and neurological symptoms. Discussion: Recurrence rate in ABC is 25% if not completely removed. Aggressive curettage with bone grafting or en bloc resection is usually recommended. Angiography was initially performed, and due to the absence of mass hypervascularity, embolization was not considered and the patient underwent a complete resection in two stages. Conclusion: ABC can mimic renal cell carcinoma and other malignant bone tumors. In huge tumors with a difficult location as in our case, we recommend a two stage procedure for huge tumors to ensure a complete resection and hence lower recurrence rate and better outcome. © 2016 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction
2. Case report
Aneurysmal bone cyst (ABC) is a rare osteolytic bone lesion representing 1% of all primary benign bone tumors [1]. It affects younger population (about 60% of reported patients are younger than 20 years old) with a female predominance [2–5]. It is characterized by an expanding locally destructive vascular bone lesion. Histologically, it typically shows cavernous channels surrounded by a spindle cell stroma with osteoclast-like giant cells and osteoid production [6]. ABCs can be primary or secondary [7] accounting for 15% of all primitive tumors of the spine. Jaffe and Lichtenstein were the first to use distinct clinico-pathological features to separate (ABC), a tumorlike lesion of the bone from other bone cysts and giant-cell tumors [8–10]. We report a case of spine ABC in a 12 year old girl, presenting with atypical symptoms (flank mass with associated pain and urinary incontinence) manifested as unusual tumor-like lesions of the vertebral column resembling a kidney tumor.
A 12 year old girl, previously healthy, presented to our hospital for a left flank pain of 6 months duration. She described an intermittent pain of moderate to severe intensity, localized in the left flank region, radiating to the left lower extremity, increasing at night and with ambulation. Pain is partially relieved by rest, supine position or anti-inflammatory drugs and is lately associated with a limp. The child started to experience urinary incontinence (day and night) with a weight loss of 8 kg during the last 2 months. On physical exam, the child looked pale, with stable vital signs, weighing 48 kg (75th percentile). A left flank mass of around 6 cm was palpated, extending from the superior anterior iliac spine to the back. The mass was hard, immobile, and tender on palpation. The rest of the physical exam was irrelevant except for absent deep tendon reflexes on the left lower extremity and ipsilateral distal weakness (Motor power 3/5). Sensory function was preserved. Laboratory investigations including creatinine and urine analysis were within normal range except for mild anemia (Hemoglobin: 10 g/dl). CT-scan of the abdomen and pelvis with IV contrast showed a large, left para-spinal, heterogeneous enhancing space occupying the lesion in the lower abdomen extending to the pelvis, measuring around 10.5 × 9.5 × 7.5 cm. Tumor infiltrates the surrounding soft tissues, left psoas, left quadratus lumborum and erector spinae
* Corresponding author. Department of Surgery, RHUH, Beirut, Lebanon. Tel.: +96567782204, +9613985316, fax: +0096522464727. E-mail address:
[email protected] (S. Termos).
http://dx.doi.org/10.1016/j.ijso.2016.04.002 2405-8572/© 2016 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
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S. Termos et al./International Journal of Surgery Open 2 (2016) 19–21
pletely normal with intact reflexes on both lower extremities. X-ray spine and an abdominal ultrasound were performed 3, 6, 12 and 24 months respectively, which showed neither kyphosis, spondylolisthesis nor other abnormal findings. 3. Discussion
Fig. 1. CT scan of the spine at the level of 4th lumbar vertebra. It shows osteolytic lesion involving the left half of the body and the left pedicle, lamina and transverse process. The lesion extends into the anteriorly, laterally and posteriorly. [R] right side, [L] left side; the scale on the left side is in mm.
muscles. Erosion of the L4 vertebra (body, pedicle and lamina) with extension over the left intervertebral foramen causes spinal cord compression (Fig. 1). Posteriorly, it is extending through the left quadratus lumborum muscle to involve the left erector spinae muscle. A renal cell carcinoma was included in the differential diagnosis and had to be ruled out. MRI of the abdomen and pelvis reveals a 10 × 8.5 × 7 cm left paravertebral multiloculated cystic mass with multiple fluid levels, involving L4 body and its posterior elements on the left with intraspinal extradural extension, suggestive of an ABC (Fig. 2A,B). CT chest and bone scans were normal. Two aspiration biopsies confirmed the MRI suspected diagnosis of ABC but could not exclude other malignancies. The management was planned by a multidisciplinary team including an orthopedist, a neurosurgeon, an interventional radiologist, a pediatric surgeon and a pediatrician. The decision was to do a preoperative arteriography of the aorta and lumbar arteries if possible to achieve embolization. As arteriography revealed a left paravertebral neovascularization at the level of L3–L4 with an absence of a hypervascularized mass, embolization was not done and a surgical option was considered (Decompression and Fusion). Bilateral L4 laminectomies were performed and the whole posterior component of the tumor was removed en bloc followed by anterior decompression and fusion L3–L5 (with pedicular screws, rods and bone graft) one week later. The patient was discharged home in a good condition with improvement of her neurological status. Histopathological examination of the excised mass was suggestive of aneurysmal bone cyst. The patient was followed up in our clinic where she regained weight. Moreover neurological exam was com-
ABC of the spine is a very rare entity involving the cervical spine in 22% of cases, the thoracic spine in 34% of cases, the lumbar spine in 31% and the sacrum in 13% [11]. Several articles described other rare localizations of bone aneurysm such as zygomatic bone or transorbital bone, reported mainly after trauma [12]. In our case, no history of trauma was noted and the localization of ABC was typical in the spine but the associated symptoms like anorexia, weight loss, and a huge abdominal mass leading to neurological symptoms were uncommon, thus requiring the exclusion of malignancy. A study done by Sawin et al. [13] described a patient with temporal bone cyst presenting with hearing loss. Still, radiological investigations of both CT and MRI are the main imaging techniques used to confirm the nature of the mass, but in our patient, due to the huge mass appearance on the CT and atypical findings on MRI, aspiration biopsies were done preoperatively. The origin of ABC is still unclear. Despite being a benign condition, there is ongoing debate about the possible neoplastic nature of those tumors. Kenney et al. [14] have assessed cytogenetically many of those lesions and found the presence of translocation on chromosome 17, which might lead to a neoplastic lesion later on. Thus, such interesting phenomenon might raise new questions about the real nature of such tumors and require cytogenetic studies in patients with ABC to distinguish those with benign from those with neoplastic origin. In our case, we could not perform cytogenetic studies due to financial limitations. In spite of the benign nature of the tumor as reported in the histopathological examination, a close follow up of the patient was attained by radiological and biological analysis. Moreover, both radiological and surgical interventions are considered as valid options in treating patients with ABC. The advantage of performing angioplasty prior to surgical excision is based on the theory of facilitating the resection by shrinking the mass first before its removal [15], but still carries disadvantages, mainly disrupting arterial supply to adjacent normal tissues and other embolic complications [16]. Different approaches to treat ABC were described in the literature. A review by Liu et al. [17] found that preoperative embolization of the mass is a good option followed by surgical removal. Other studies found that intralesional excision of the mass has 30% recurrence rate [18]; this complete marginal resection should be the goal, like in our case, to decrease the risk of recurrence. In our patient the mass was huge, requiring a two stage intervention: first, bilateral L4 laminectomies was performed and the whole posterior component of the tumor was
Fig. 2. MRI of the spine. (A) Axial T2-weighted image shows the osteolytic lesion involving the 4th lumber vertebra with extension into the surrounding soft tissue; it also reveals the fluid–fluid level, which is characteristic of ABC. (B) Coronal T2-weighted image shows destruction of the left half of the L4 extending beyond the psoas muscle laterally and superiorly reaching the lower pole of the left kidney.
S. Termos et al./International Journal of Surgery Open 2 (2016) 19–21
removed en bloc with the mass; and second, anterior vertebral decompression was done with spine fusion one week later. According to the literature, this approach is not widely used and usually a single step removal is more common. The role of radiotherapy is yet controversial; even some authors described the so called radio-induced tumors [19]. Follow up of the patient did not reveal any signs of recurrence as found after simple surgery without angioplasty. The risk of recurrence is highest during the first two years following surgery. Recurrence was reported to occur mostly after curettage, with lower incidence after complete surgical resection. A close follow up is recommended especially in children with ongoing growth.
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Conflict of interest There are no conflicts of interest. Guarantor No guarantor, no financial support. Research registration UIN Research registration UIN: researchregistry676. References
4. Conclusion ABC of the vertebral column is a very rare tumor, with an unclear etiology and pathophysiology. It can mimic renal cell carcinoma and other malignant bone tumors. Imaging studies mainly CT-scan and MRI help in the diagnosis, but biopsy is considered the gold standard. According to the literature review a complete excision of the ABC offers better symptom relief, higher chance of cure and lower risk of recurrence. In huge tumors with difficult location, we recommend a two stage procedure to ensure a complete resection and hence better outcome. Funding None. Ethical approval Written informed consent was obtained from the patient for publication of this case report and its accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. Author contribution Salah Termos was the major contributor in writing the case report and he was involved in the acquisition and analysis of literature review. Ahmad Alsaleh and Nijmeh Hammoud-Termos were involved in the review and preparation of the manuscript. Haythem Rifaii and Bilal Aoun provided clinical care of the patient during his treatment and further follow-up as well as supervising the writing of the case report.
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