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Tenosynovial Giant Cell Tumors in Children

A Similar Entity Compared With Adults

Mastboom, Monique J. L. MD; Verspoor, Floortje G. M. MD; Uittenbogaard, Daniël MD; Schaap, Gerard R. MD; Jutte, Paul C. MD, PhD; Schreuder, H. W. Bart MD, Prof; van de Sande, Michiel A. J. MD, PhD

Clinical Orthopaedics and Related Research®: September 2018 - Volume 476 - Issue 9 - p 1803–1812
doi: 10.1007/s11999.0000000000000102
2017 INTERNATIONAL SOCIETY OF LIMB SALVAGE PROCEEDINGS
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Background Tenosynovial giant cell tumor (TGCT) is a rare, benign, monoarticular entity. Many case-series in adults are described, whereas TGCT is only incidentally reported in children. Therefore, its incidence rate and natural history in children are unknown.

Questions/purposes (1) How many cases have been reported of this condition, and what were their characteristics? (2) What is the standardized pediatric incidence rate for TGCT? (3) Is there a clinical difference in TGCT between children and adults? (4) What is the risk of recurrence after open resection in children compared with adults?

Methods Data were derived from three sources: (1) a systematic review on TGCT in children, seeking sources published between 1990 and 2016, included 17 heterogeneous, small case-series; (2) the nationwide TGCT incidence study: the Dutch pediatric incidence rate was extracted from this nationwide study by including patients younger than 18 years of age. This registry-based study, in which eligible patients with TGCT were clinically verified, calculated Dutch incidence rates for localized and diffuse-type TGCT in a 5-year timeframe. Standardized pediatric incidence rates were obtained by using the direct method; (3) from our nationwide bone and soft tissue tumor data registry, a clinical data set was derived. Fifty-seven children with histologically proven TGCT of large joints, diagnosed and treated between 1995 and 2015, in all four tertiary sarcoma centers in The Netherlands, were included. These clinically collected data were compared with a retrospective database of 423 adults with TGCT. Chi-square test and independent t-test were used to compare children and adults for TGCT type, sex, localization, symptoms before diagnosis, first treatment, recurrent disease, followup status, duration of symptoms, and time to followup. The Kaplan-Meier method was used to evaluate recurrence-free survival at 2.5 years.

Results TGCT is seldom reported because only 76 pediatric patients (39 female), 29 localized, 38 diffuse, and nine unknown type, were identified from our systematic review. The standardized pediatric TGCT incidence rate of large joints was 2.42 and 1.09 per million person-years in localized and diffuse types, respectively. From our clinical data set, symptoms both in children and adults were swelling, pain, and limited ROM with a median time before diagnosis of 12 months (range, 1-72 months). With the numbers available, we did not observe differences in presentation between children and adults in terms of sex, symptoms before diagnosis, first treatment, recurrent disease, followup status, or median time to followup. The 2.5-year recurrence-free TGCT survival rate after open resection was not different with the numbers available between children and adults: 85% (95% confidence interval [CI], 67%-100%) versus 89% (95% CI, 83%-96%) in localized, respectively (p = 0.527) and 53% (95% CI, 35%-79%) versus 56% (95% CI, 49%-64%) in diffuse type, respectively (p = 0.691).

Conclusions Although the incidence of pediatric TGCT is low, it should be considered in the differential diagnosis in children with chronic monoarticular joint effusions. Recurrent disease after surgical treatment of this orphan disease seems comparable between children and adults. With targeted therapies being developed, future research should define the most effective treatment strategies for this heterogeneous disease.

Level of Evidence Level III, therapeutic study.

M. J. L. Mastboom, D. Uittenbogaard, M. A. J. v. d. Sande, Orthopaedics Oncology Department, Leiden University Medical Center, Leiden, The Netherlands

F. G. M. Verspoor, H.W. B. Schreuder, Orthopaedics Oncology Department, Radboud University Medical Center, Radboud, The Netherlands

G. R. Schaap, Orthopaedics Oncology Department, Academic Medical Center Amsterdam, Amsterdam, The Netherlands

P. C. Jutte, Orthopaedics Oncology Department, University Medical Center, Groningen, Groningen, The Netherlands

M. J. L. Mastboom, Leiden University Medical Center Albinusdreef 2 2333 ZA Leiden The Netherlands email: mjlmastboom@lumc.nl

Each author certifies that neither he or she, nor any member of his or her immediate family, has funding or commercial associations (consultancies, stock ownership, equity interest, patent/licensing arrangements, etc) that might pose a conflict of interest in connection with the submitted article.

All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research® editors and board members are on file with the publication and can be viewed on request.

Each author certifies that his or her institution approved the human protocol for this investigation and that all investigations were conducted in conformity with ethical principles of research.

This work was performed at Leiden University Medical Center, Leiden, The Netherlands.

Received August 02, 2017

Received in revised form September 26, 2017

Accepted November 22, 2017

© 2018 Lippincott Williams & Wilkins LWW
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