Spinal lymphangiomas: Case-based review of a chameleonic disease entity

Abstract

Purpose: Lymphangiomas are benign hamartomas in the spectrum of lymphatic malformations, exhibiting multifaceted clinical features. Spinal involvement is exceedingly rare, with only 35 cases reported to date. Both due to their rarity and chameleonic radiologic features, spinal lymphangiomas (SLs) are usually misdiagnosed; postoperatively, surgeons are thus confronted with an unexpected histopathological diagnosis with sparse pertinent literature and no treatment guidelines available.

Methods: Here, we report the case of a 67-year-old female who underwent surgery for a T6-T7 epidural SL with transforaminal extension, manifesting with spastic paraparesis. Then, we present the results of the first systematic review of the literature on this subject, delineating the clinical and imaging features and the therapeutic implications of this rare disease entity.

Results: Our patient was treated with T6-T7 hemilaminectomy and resection of the epidural mass, with complete recovery of her neurological picture. No recurrence was evident at 18 months. In the literature, 35 cases of SL were reported that can be classified as vertebral SL (n = 18), epidural SL (n = 10), intradural SL (n = 3), or intrathoracic lymphangiomas with secondary spinal involvement (n = 4). Specific treatment strategies (both surgical and nonsurgical) were adopted in relation to each of these categories.

Conclusion: Gathering knowledge about SL is fundamental to promote both correct preoperative identification and appropriate perioperative management of this rare disease entity. By reviewing the literature and discussing an exemplary case, we delineate a framework that can guide surgeons facing such an unfamiliar diagnosis.

Keywords: Cystic hygroma; lymphangioma; lymphatic malformation; review; spine surgery; spine tumor.

Figure 1

Preoperative magnetic resonance imaging. (a) T1, (b) postcontrast T1, (c) Paramedian postcontrast T1, showing transforaminal extension (arrowhead) and the unrelated subcutaneous lipoma (arrow), (d) axial postcontrast T1

Figure 2

Magnification: 10x (left), 20x (right), H/E-stained microscopic sections of the extradural lesion, showing vascular structures of lymphatic and capillary appearance. The endothelium of these channels reacted extensively with D2-40 antibodies, which react with podoplanin and are used as a selective marker of lymphatic structures

Figure 3

Early postoperative magnetic resonance imaging (MRI) (a and b), 18-month follow-up MRI (c and d)