European Spine Journal
Solitary juvenile xanthogranuloma in the spine pretreated
with neoadjuvant denosumab therapy followed by surgical resection
in a 5‑year‑old child: case report and literature review
· Minna K. Laitinen
· Jyrki Parkkinen
· Jacob Engellau
· Marko H. Neva
Received: 13 February 2018 / Revised: 16 April 2018 / Accepted: 1 June 2018
© Springer-Verlag GmbH Germany, part of Springer Nature 2018
Purpose We present a case report that describes neoadjuvant denosumab therapy initiated in a child with a solitary giant
cell-rich juvenile xanthogranuloma tumor involving the spine, and review the current literature.
Methods A giant cell-rich histiocytic lesion involving the 11th thoracic vertebral body was identiﬁed in a healthy 5-year-old
girl with persistent back and pelvic pain for several months. Imaging examinations and an open biopsy were performed to
obtain a deﬁnite pathologic diagnosis. As the tumor appeared to be aggressive in nature, we administered adjuvant therapy
with denosumab preoperatively and then performed a total spondylectomy.
Results Histopathology conﬁrmed that the tumor was juvenile xanthogranuloma. No tumor metastases or recurrence were
detected at the 3-year follow-up, and the patient was asymptomatic.
Conclusions In giant cell-rich tumors, denosumab is occasionally used as neoadjuvant or adjuvant therapy, especially for
tumors in diﬃcult locations or with substantial soft tissue extensions. Rare adverse events in children include skin infections
and disruption of calcium homeostasis. Surgical treatment is aimed at removing the tumor and relieving the symptomatic
spinal cord compression. Use of denosumab as neoadjuvant therapy for juvenile xanthogranuloma involving the spine has
not been reported previously.
Keywords Spine · Tumor · Xanthogranuloma · Denosumab · Hypercalcemia
Spine tumors, including giant cell-rich tumors, are rare in
children, accounting for only 2% of childhood tumors [1, 2].
Tumors rich in giant cells, such as an aneurysmal bone cyst
(ABC), which is the most common bone tumor containing
giant cells in children; a true, benign giant cell tumor of bone
(GCTB), ﬁbrous metaphyseal defects, chondroblastomas,
giant cell-rich osteosarcomas, brown tumors associated
with hyperparathyroidism, giant-cell reparative granulomas,
and, in rare cases, solitary juvenile xanthogranuloma (JXG)
Management of pediatric spine tumors is challenging.
Deﬁnitive treatment is determined on the basis of the tumor
histology, location, and extent of involvement. Benign
tumors in the spine are typically treated with intralesional
resection aimed at removing the tumor and relieving symp-
tomatic spinal cord compression . Originally, giant cell-
rich tumors were treated with an intralesional surgical resec-
tion. Currently, cystic ABCs are treated with polidocanol
injections [6, 7]. Rare tumors, like extracutaneous JXG, are
still treated with surgery .
Denosumab is a fully human monoclonal antibody that
inhibits ligand binding to receptor activator of NF-kappa
B (RANK) . Denosumab is eﬀective and safe for treat-
ing osteoporosis , skeletal metastases , and GCTB
—particularly inoperable or metastatic GCTBs . In
GCTB, denosumab treatment reduces the number of giant
* Tero Irmola
Coxa, Hospital for Joint Replacement, Biokatu 6,
33521 Tampere, Finland
Department of Orthopedics and Traumatology, Unit
of Musculoskeletal Surgery, Tampere University Hospital,
Fimlab Laboratories, Tampere, Finland
Department of Oncology, Skane University Hospital, Lund,