Intradural extramedullary eosinophilic granuloma of the spine with emergency presentation: A case report

Abstract

Background: Intradural extramedullary spinal eosinophilic granuloma is a very unusual manifestation of Langerhans Cell Histiocytosis (LCH) that is typically misdiagnosed due to its nonspecific clinical and radiological features.

Case description: We report a case of a 22-year-old female patient who presented with acute onset paraplegia. The magnetic resonance imaging was initially suggestive of tuberculoma, which is prevalent in tuberculosis-endemic regions. Intraoperative findings and histopathology, however, established the diagnosis of LCH. The lesion was intradural, and CD1a and S-100 protein staining demonstrated classic Langerhans cells.

Conclusion: The paper stresses the need for a multidisciplinary team in the proper diagnosis and handling of spinal LCH. Further research is needed to develop optimal management protocols for this rare condition.

Keywords: Histopathological diagnosis; Intradural extramedullary eosinophilic granuloma; Langerhans cell histiocytosis; Spinal lesion; Tuberculoma.

Figure 1:

(a) Preoperative (Preop) Sag-short tau inversion recovery. Shows the mass at the T9 level, with suspicion of extradural as there is an involvement of the spinous process and paraspinal soft tissues. (b) Preop Sag-T2 shows a mass in the T9, which is an intradural extramedullary lesion. (c) Preop Sag-T1-postcontrast shows the enhancement of the mass in a heterogeneous pattern with the enhancement of the spinous process and surrounding muscles up to the fascia. (d) Preop. The pattern of enhancement is ring enhancing, raising suspicion of infection and, most likely, TB, as it is endemic in our country.

Figure 2:

Preop axial-T2 showing the mass is hypointense and left-sided, displacing the spinal cord to the right.

Figure 3:

(a) Intraoperative image demonstrating the tumor (black arrows) compressing and displacing the spinal roots. (b) Intraoperative image showing meticulous dissection and preservation of an intact spinal root (green and blue arrows). (c) Intraoperative image revealing a fibrous granuloma that was initially biopsied for pathological evaluation. (d) Intraoperative image depicting the gross total resection of the lesion, ensuring decompression of the spinal cord.

Figure 4:

(a) Hematoxylin and eosin (H&E) staining showing an infiltrate of eosinophils mixed with histiocytic cells. A Langerhans cell with a characteristic grooved, coffee-bean-shaped nucleus is indicated by the arrow, supporting the diagnosis of Langerhans cell histiocytosis (LCH), ×400 (Arrow). (b) Immunohistochemistry (IHC) demonstrating strong diffuse positivity for S-100 protein in lesional cells, confirming the presence of Langerhans cells, ×200. (c) CD1a IHC stain showing strong membrane positivity in lesional cells, a hallmark feature of LCH, ×200. (d) Glial fibrillary acidic protein (GFAP) IHC stain demonstrating negative staining, excluding glial-derived tumors, ×200. (e) Epithelial membrane antigen (EMA) IHC stain showing negative staining, helping to exclude metastatic carcinoma from the differential diagnosis, ×200.