Castleman's disease of the sacral spine

doi:10.1016/j.clineuro.2011.10.033

Clinical Neurology and Neurosurgery

Volume 114, Issue 3, April 2012, Pages 287-289

https://doi.org/10.1016/j.clineuro.2011.10.033 Get rights and content

Introduction

Angiofollicular lymph node hyperplasia, also known as Castleman's disease (CD), is a lymphoproliferative disorder of which there are three major types: hyaline vascular, plasma cell and mixed cellular variants [1]. The hyaline vascular type is comprised of atrophic or regressing germinal centers which contain small, hyalinized vessels and numerous follicular dendritic cells. It is also defined by broadened mantle zones with concentric rings of small lymphocytes in an onion skin composition and an interfollicular zone in which sinuses are rarely seen. In the plasma cell variant, there are hyperplastic germinal centers with an interfollicular region containing sheets of plasma cells and patent sinuses. The disease usually occurs either in a localized, unicentric pattern (unicentric CD, UCD) of a predominantly hyalinized pathology, or a multicentric pattern (multicentric CD, MCD) of a predominantly plasma cell pathology. MCD is often associated with more systemic symptoms and has a much poorer prognosis [1].

Castleman's disease is usually seen as an isolated UCD mass of the mediastinum, but can also occur in the abdomen, neck and axillae [2]. Although rare, CD can even manifest in the spine [2], [3]. Prior reports have described CD occurring as epidural lesions involving the thoracic spine. There are no reports in the literature of CD involving the sacral spine. We report the case of a patient with CD of the sacral spine and describe the surgical techniques used to facilitate en bloc resection of this lesion.

Section snippets

Case report

Preoperative: A 60-year-old woman was diagnosed with a sacral lesion (Fig. 1) after undergoing workup for progressive sacral pain. An MRI demonstrated a 4.5 cm × 3.5 cm × 1.5 cm homogenously enhancing lesion involving the right side of S4, S5 and the coccyx (Fig. 1). The lesion demonstrated low signal intensity on T1 and T2-weighted imaging. After undergoing two non-diagnostic percutaneous needle biopsies, the patient requested definitive management of the lesion. We recommended en bloc resection of

Discussion

We present the first reported case of CD of the sacrum. To date, there have been seven documented cases of CD within the spine, and all but one case presented with spinal cord compression and myelopathy [2], [3]. In these cases, six of seven were of the hyaline vascular type. Four of the cases have been treated with full surgical resection, two of which were en bloc resections. Of the other cases, one case was managed solely by partial resection, one case by partial resection with radiotherapy

Conclusion

CD of the spine is a rare lesion that must be considered in the differential for spinal lesions. At present, surgical resection of CD is the gold standard for management. For CD of the sacral spine, en bloc resection should be considered on a case by case basis as a treatment option to ensure negative surgical margins.