Case Study: March 2011
80 year lady presented with a mass in the orbit. Imaging confirmed that the mass was originating from the inferior rectus.
Fig 1: H and E
Fig 2: immunostaining
Fig 3: immunostaining
- What is the diagnosis?
- What is the differential diagnoses?
- What are the immunohistochemical stains shown in Figure 2 and 3? What other special stains would be useful?
Click to show answers
- Granular cell tumour (GCT) of the orbit. This is a benign tumour of peripheral nerve sheath origin which rarely undergoes malignant change. It has been reported in the orbital soft tissues, particularly involving the extraocular muscles. Granular cell tumours are infiltrative lesions with an ill-defined margin consisting of polygonal or spindle shaped cells which have a characteristic eosinophilic granular cytoplasm and bland nuclei (Figure 1: HE x10).Chronic inflammatory infiltrates have been reported in association with GCT and in this case, occurred as lymphoid aggregates.
- Although the microscopic appearance of GCT is distinctive, the differential diagnosis of this tumour may include atypical histiocytic inflammatory lesions and the presence of the lymphocytic infiltration may be misleading.
- Granular cell tumours are immmunoreactive to S100 (Figure 2) and CD68 (Figure 3) and are negative for epithelial, melanocytic, smooth muscle, dendritic cell, and endothelial markers. These tumours are also PAS positive and diastase resistant.
- Orbital granular cell tumor in a patient with Churg Strauss syndrome: the importance of biopsy.Guerriero S, Giancipoli G, Sborgia A, Fiore MG, Rossi R, Piscitelli D. Orbit. 2011 Jan;30(1):30-3.
- Poyraz CE, Kiratli H, Söylemezo?lu F.Korean J Ophthalmol. 2009 Mar;23(1):43-5. Granular cell tumor of the inferior rectus muscle.
- Karcioglu ZA, Hemphill GL, Wool BM. Granular cell tumour of the orbit: case report and review of the literature. Ophthalmic Surg 1983; 14: 125-9.
- Jaeger MJ, Green WR, Miller NR, Harris GJ. Granular cell tumour of the orbit and ocular adnexae. Surv Ophthalmol 1987; 31: 417-23
- Mazur MT, Shultz JJ, Myers JL. Granular cell tumor. Immunohistochemical analysis of 21 benign tumors and one malignant tumor. Arch Pathol Lab Med. Jul 1990;114(7):692-6.