Pseudotumor inflamatorio del saco endolinfatico: Reporte de un caso

Inflammatory pseudotumor of the endolymphatic sac: A case report

• Bernard Tan, MBBS^{a, ,} ,
• Alwin Loh, MBChB (Hons), FRCPath^b,
• Tan Tiong Yong, MBBS, FAMS, FRCR (UK)^c,
• Mahalakshmi Rangabashyam, MBBS^d,
• Low Wong Kein, MBBS, PhD, FRCS^d

• ^a Yong Loo Lin School of Medicine, National University of Singapore, Singapore 1922828
• ^b Department of Pathology, Singapore General Hospital, Singapore, Singapore 169608
• ^c Department of Diagnostic Radiology, Changi General Hospital, Singapore 169608
• ^d Department of Otolaryngology, Singapore General Hospital, Singapore, Singapore 169608

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Abstract

The authors present a case of Endolymphatic Sac Inflammatory Pseudotumour treated at our institution and reviewed the relevant literature. 

This is a benign lesion which arises due to chronic inflammation. 

It has similar clinical and radiological features as endolymphatic sac tumors and is diagnosed through histopathological examination. 

To date, there have been over 200 case reports of ELSTs but only 2 case reports of endolymphatic sac pseudotumour. 

This report highlights the clinical presentation, radiological features, histological characteristics, treatment, and clinical outcomes of endolymphatic sac inflammatory pseudotumours and explores their likely etiology and discusses possible treatment options. 

Endolymphatic sac inflammatory pseudotumour may represent the end of a spectrum of immune-mediated inflammatory disease of the endolymphatic sac. 

Based on the experience of managing inflammatory pseudo-tumours elsewhere in the body, there may be a role for high dose steroids in its management.

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Figures and tables from this article:

Fig. 1. (A) Computed Tomography scan of the left temporal bone showing an irregular osteolytic lesion in the left retrolabyrinthine region measuring 1.2×1.2×0.7 cm, associated with internal bony spiculation. (B) T2 MRI of the patient showing high signal intensity of the left sided retrolabyrinthine lesion.

Fig. 2. Endolymphatic sac inflammatory pseudotumour. Microscopic examination of the excised specimen showing normal endolymphatic sac epithelium with an area of fibrosis, chronic inflammation and foreign body giant cell reaction with cholesterol clefts (H&E, × 200 magnification).

Fig. 3. Postoperative MRI showing no recurrence of the pseudotumor, with obliteration of mastoid cavity by the autologous fat graft.

Fig. 4. Treatment regimens and reported outcomes of temporal bone inflammatory pseudotumours.

Corresponding author. Department of Otolaryngology, Singapore General Hospital, Singapore 169608, Singapore. Tel.: + 65 92953571.

 

Fuente:  American Journal of Otolaryngology