A rare case of giant solitary spinal B cell lymphoblastic lymphoma
© The Author(s) 2016
Received: 15 April 2016
Accepted: 8 September 2016
Published: 2 November 2016
Common B cell lymphoma locations are lymph nodes, skin, bone, mediastinum and it accounts for 2 % of NHL. Giant solitary spinal B-LBL has not yet been described.
A 15-years old boy was admitted for a painful swelling of the thoracolumbar spine and paraplegia. MRI showed a voluminous mass of the thoracolumbar spine. Initial treatment consisted of surgical decompression and stabilization, followed by chemotherapy and radiotherapy. Histological examination showed a B-LBL. The quality of life improved significantly.
To the best of our knowledge, this is the first case of giant solitary spinal B-LBL ever reported. Indication to choose a treatment method over another should weigh on individual priorities. Surgery should be considered as initial treatment option in this atypical lesion.
B cell lymphoblastic lymphoma (B-LBL) is a subtype of non Hodgkin’s (NHL) lymphoma with great tropism for central nervous system. It is an aggressive tumour and accounts for 2 % of all NHL . Lymphoma is the term used when the process is confined as a mass lesion without evidence of blood involvement.
Common locations of B cell lymphoblastic lymphoma are lymph nodes, skin, bone, mediastinum  and soft tissues . However, rare and uncommon sites such as gall bladder , nasopharynx , great omentum  and orbit  have been reported. As far as we know, giant spinal B-LBL as a primary location has not yet been described.
We report a case of giant spinal primary B-LBL revealed as a spinal cord compression and thoracolumbar mass syndrome. Surgical decompression and stabilization were warranted at the initial stage to improve quality of life. We present this successful management of an atypical giant lesion of this area.
He received 4 cycles of hyperfractionned CVAD regimen as follows: cyclophosphamide administrated at the dose of 300 mg/m2 intravenously on days 1 to 3; vincristine administrated at the dose of 2 mg intravenously on days 4 and 11; doxorubicin administrated at the dose of 50 mg/m2 intravenously on day 4 and 40 mg dexamethasone administrated orally and daily on days 1 to 4 and days 11 to 14. This intensive chemotherapy was followed by 30 Gy radiotherapy in 6 fractions. Maintenance chemotherapy was then administrated for 12 months with vincristine 2 mg intravenously every month and prednisone 200 mg daily for 5 days every month orally.
There was no change in his neurological status but his general condition improved significantly, sitting pain-free and enjoying a better quality of life. Two years after surgery, chemotherapy and radiotherapy, his condition remains improved.
B-LBL is a solid tumour without blood involvement. The accepted definition of bone lymphoma is a tumour with bone involvement without evidence of systemic involvement. . When there is more than 25 % blast in the bone marrow with or without mass lesion, the lesion is defined as lymphoid leukemia . Primary vertebral location of non-Hodgkin lymphomas is rare and accounts for 1.7 % of all primary bone lymphoma . Spinal lymphomas are more commonly found to take origin in the epidural space and derive from normal lymphoid tissue in the epidural space . Spinal giant primary solitary B-LBL has not yet been described. To our knowledge, the treatment of this atypical lymphoma is not yet codified.
For Peng et al.  the ideal treatment of vertebral lymphoma is chemotherapy combined with radiotherapy. Park et al.  however argue that treatment of spinal lymphoma must be multifactorial including surgery, radiotherapy and chemotherapy.
In this particular case, the size of the tumour was important, so much so that prone position was made impossible. Moreover, it was very aggressive for the vertebras, causing kyphosis of the spine and a serious neurological status.
Surgery was warranted as initial treatment option, for decompression, stabilization and to improve the quality of life immediately. Chemotherapy and radiotherapy followed surgery with good outcome.
We think that the management of the vertebral B-LBL must be guided by the clinical state of the patient, and particularly by the therapeutic purpose to be reached. The indications to choose a treatment with regard to another one have to depend on priorities case by case.
We report a unique case of giant spinal primary B-LBL lymphoma with high bone aggressivity on presentation. Decompressive surgery and stabilization can be successfully used as an initial treatment option in this rare tumour.
No funding was available in support of this study.
Availability of data and materials
All data can be found in pubmed.
HAN has performed surgery, drafted and revised the manuscript. LKD an LD assisted HAN for surgery. SAM revised the English language. DNO and VBZ as our heads accepted the final version for publication. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
The father was informed that the case would be submitted for publication and he provided consent. The consent form is available upon request.
Ethics approval and consent participate
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
- Kim HJ, Lee TJ, Choi YS. Primary B Lymphoblastic lymphoma of gallbladder involving mandibular bone. Int J of Hematol. 2014;99:790–3.View ArticleGoogle Scholar
- Epstein JB, Epstein JD, Le ND, Gorsky M. Characteristics of oral and paraoral malignant lymphoma : a population based review of 361 cases. Oral Surg Oral Med Oral Path Oral Radiol Endod. 2001;92:519–25.View ArticleGoogle Scholar
- Alford MA, Nerad JA, Conlan RM, Comito M, Giller RH. Precursor B-cell lymphoblastic lymphoma presenting as an orbital mass. Orbit. 1999;18:17–24.View ArticlePubMedGoogle Scholar
- Gravel J, Lallier M, Garel L, Brochu P, Champagne J, et al. Primary non hodgkin lymphoma of the extrahepatic biliary tract and gallbladder in a child. J Pediatr Gastroenterol Nutr. 2001;32:598–601.View ArticlePubMedGoogle Scholar
- Lopes Da Silva R, Fernandes T, Santos S, Rodrigues AS, de Sousa AB. B lymphoblastic lymphoma presenting as a tumor of the nasopharynx in an adult patient. Head Neck Pathol. 2010;4:318–23.View ArticlePubMedPubMed CentralGoogle Scholar
- Kantekure K, Cossor F, Miller KB, Pilichowska ME. A unique case of relapsed B acute lymphoblastic lymphoma. Case Rep hematol. 2014; dx.doi.org/10.1155/2014/425163.Google Scholar
- Farrès MT, Dock W, Augustin I, Windhager R, Riegler M, et al. The radiologic clinical picture of primary bone lymphoma. Rofol. 1993;58:589–93.View ArticleGoogle Scholar
- Roy CA, Hawkes EA, Bardy P, Woo T, Ting K, et al. Rare case of precursor B-cell acute lymphoblastic leukemia presenting as a solitary paraspinal mass. J Clin Oncol. 2013;22:e383–5.View ArticleGoogle Scholar
- Park DA, Park SG, Kim SW. Solitary lymphoblastic lymphoma of the thoracic spine. J Korean Neurosurg Soc. 2012;52:564–6.View ArticlePubMedPubMed CentralGoogle Scholar
- Peng X, Wan Y, Chen Y, Chen I, He A, et al. Primary non hodgkin’s lymphoma of the spine with neurologic compression treated by radiotherapy and chemotherapy alone or combined with surgical decompression. Oncol Rep. 2009;21:1269–75.View ArticlePubMedGoogle Scholar