A 37-year-old woman presented with a 6 months history of headaches and memory impairment. Examination showed no neurological deficit with normal vision. MRI scans showed an enlarged optic chiasm. There was no dural or leptomeningeal enhancement or hydrocephalus. Open biopsy of the suprasellar mass showed non-caseating chronic granulomatous inflammation compatible with sarcoidosis. Systemic features of sarcoid were absent. Patient showed marked improvement on steroid therapy

Shaikh, Farheen

Siddiqui, Arshad Ali

Tahir, Muhammad Zubair

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eCommons@AKUSection of Neurosurgery Department of SurgeryOctober 2010Primary chiasmal sarcoid granuloma masqueradingas glioma of the optic chiasmMuhammad Zubair TahirAga Khan UniversityFarheen ShaikhAga Khan UniversityArshad Ali SiddiquiJinnah Postgraduate Medical CentreFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_neurosurgPart of the Ophthalmology Commons, and the Surgery CommonsRecommended CitationTahir, M., Shaikh, F., Siddiqui, A. (2010). Primary chiasmal sarcoid granuloma masquerading as glioma of the optic chiasm. Journal ofthe College of Physicians and Surgeons Pakistan, 20(10), 695-6.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_neurosurg/4Journal of the College of Physicians and Surgeons Pakistan  2010, Vol. 20 (10): 695-696 695INTRODUCTIONNeurosarcoidosis is a rare disease, seen in adultsoften with the evidence of systemic disease. Its clinicalspectrum includes a diverse range of conditions includingmeningitis, cranial nerve palsies, epilepsy, hydro-cephalus and transverse myelitis.1,2 MRI is a usefulradiological investigation for its evaluation active lesionsshow contrast enhancement. Despite this, the MRI appearances of neurosarcoidosisare seldom distinctive and it may be mistaken for otherdiseases.3,4We report a rare case of primary sarcoid granuloma ofoptic chiasm masquerading as chiasmal glioma.CASE REPORTA 37-year-old lady of Yemeni origin presented withheadaches and memory impairment over the last 6months. She denied any visual or other constitutionalsymptoms. Her neurological examination includingvisual acuity, cranial nerves and fundoscopy wasunremarkable. There was no adenopathy. Chestradiograph, basic haematological investigations werenormal. Her endocrine workup showed that serum T3level of 0.05 n mol/L, T4 level of 4.2 microgm/dl, TSHlevel of 4.28 micro IU/ml, growth hormone level of 0.05ng/ml, serum cortisol level of 0.89  micrograms/dl andserum prolactin level of 143 ng/ml. All these values werebelow normal and suggestive of hypopituitarism. Herserum angiotensin converting enzyme (ACE) level wasmarginally high at 28 U/L.She underwent MRI scan of brain which showed anenlarged chiasma with swelling extending to the inferiorpart of the hypothalamus. The mass had a low signal onboth T1 and T2 weighted images and showed strongcontrast enhancement. No dural or leptomeningeal enhance-ment or hydrocephalus was present (Figures 1 and 2).The patient underwent open biopsy of the suprasellarmass. Frozen section specimens revealed a granulo-matous lesion. Histopathology showed multiple non-caseating granuloma and inflammatory cell infiltrate withperivascular cuffing but no acid-fast bacilli, a diagnosisof sarcoidosis was made. The patient was commencedon oral steroids 5 mg of prednisolone  for 6 weeks. Sheshowed a definite slow progressive improvement anddischarged from hospital on the 14th postoperative day.Patient was advised to follow-up in her country of originwith her referring physician and have a follow-up MRimaging at least after 3 months from date of surgery.Unfortunately, she lost follow-up and repeat MR studycould not be documented.DISCUSSIONNeurosarcoidosis, accounts for approximately 5% of allcases of sarcoidosis.5,6 Of these, have reported thatABSTRACTA 37-year-old woman presented with a 6 months history of headaches and memory impairment. Examination showed noneurological deficit with normal vision. MRI scans showed an enlarged optic chiasm. There was no dural or leptomeningealenhancement or hydrocephalus. Open biopsy of the suprasellar mass showed non-caseating chronic granulomatousinflammation compatible with sarcoidosis. Systemic features of sarcoid were absent. Patient showed marked improvementon steroid therapy.Key words: Optic chiasm.   Sarcoidosis.   Magnetic resonance imaging.   Steroid.1 Department of Neurosurgery1/Opthalmology2, The Aga KhanUniversity Hospital, Karachi.3 Department of Neurosurgery, Jinnah Postgraduate MedicalCentre, Karachi.Correspondence: Dr. Arshad Ali Siddiqui, Neurosurgeri Klinik,Hospital Sultanah Aminah, Jalan Abu Bakar, Johar Bahru-80100, Johor-Malayisa..E-mail: doc4brains@yahoo.comReceived  January 30, 2010; accepted  May 17, 2010.Primary Chiasmal Sarcoid Granuloma Masquerading as Glioma of The Optic ChiasmMuhammad Zubair Tahir1, Farheen Shaikh2 and Arshad Ali Siddiqui3CASE REPORTFigure 1: Postcontrast T1-weightedimage shows sagittal section of theenlarged optic chiasm. The homo-genous contrast enhancement isexclusively within the chiasma.Figure 2: Postcontrast T-1 weighted(coronal section) shows enhance-ment of the chiasm, suggestive ofan intrinsic mass lesion. 48% initially present with neurological symptoms.6Ultimately majority of the patients develop othersystemic manifestations of the disease.6 Typically,sarcoid presents in other organs such as eyes, skin,lymph nodes, bones, lungs and parotid gland. When it isfound in the nervous system it seems to affect cranialnerves, promote a basal arachnoiditis or meningo-encephalitis, or organize in  posterior fossa nodules.7Histologically distinct, non-caseating granulomas withlymphocytic infilterates are found.8 Radiologicaldiagnosis of this entity is often indistinguishable fromlymphoma, metastasis or primary malignant disease.9Enlargement of optic chiasmal is commonly result ofoptic nerve glioma. It accounts for 2% gliomas in adults,and 7% in childern.10 They occur commonly in femalesand up to 25% of cases are associated with neuro-fibromatosis. Histologically there are infiltrations of theoptic pathways with glioblasts of various sizes andoccasional astrocytes. Hyperplasia of the overlyingarachnoid matter, known as arachnoid gliomatosis, is awell-recognized feature. Chiasmal tumours are generallynot treated surgically except for biopsy, CSF shunting orto remove the rare exophytic component to try andimprove vision. Chemotherapy or radiotherapy is usedfor chiasmal tumours especially in younger patients.10Neurosarcoidosis affecting the optic chiasm may closelyresemble chiasmal glioma. Chiasmal enlargement onMRI should suggest a diagnosis of sarcoidosis even inthe presence of mass lesion and absence of systemicfeatures. This case shows one of the many faces ofneurosarcoidosis, which are almost impossible todiagnose correctly without open biopsy.REFERENCES1. Zouaoui A, Maillard JC, Dermont D, Marsault C. MRI in neuro-sarcoidosis. J Neuroradiol 1992; 19:271-84.2. Douglas AC, Maloney AF. Sarcoidosis of the central nervoussystem. J  Neurol Neurosurg Psychiatry 1973; 36:1024-33.3. Hayes WS, Sherman JL, Stern BJ, Citrin CM, Pulaski PD. MRand CT evaluation of intracranial sarcoidosis. AJR Am J Reontgenol1987; 149:1043-9.4. Ng KL, McDermott N, Romanowski CA, Jackson A. Neuro-sarcoidosis masquerading as glioma of the optic chiasm in achild. Postgrad Med J 1995; 71:265-8.5. Chen RC, McLeod JG. Neurological complications ofsarcoidosis. Clin Exp Neurol 1989; 26:99-112.6. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosisand its neurological manifestations. Arch Neurol 1985; 42:909-17.7. Oksanen V. Neurosarcoidosis: clinical presentation and coursein 50 patients. Acta Neurol Scand 1986; 73:283-90.8. Burger PC, Scheithauer BW. Atlas of tumour pathology: tumoursof the central nervous system. Washington: AFIP; 1994.9. Leu NH, Chen CY, Wu CS, Lee KW, Hsiao HS, Zimmerman RA.Primary chiasmal sarcoid granuloma: MRI. Neuroradiology 1999;41:440-2.10. Greenberg MS. Handbook of neurosurgery. New York: Thieme;2001.696 Journal of the College of Physicians and Surgeons Pakistan  2010, Vol. 20 (10): 695-696Muhammad Zubair, Farheen Shaikh and Arshad Ali Siddiquil l l l lOl l l l l

Primary chiasmal sarcoid granuloma masquerading as glioma of the optic chiasm

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Primary chiasmal sarcoid granuloma masquerading as glioma of the optic chiasm

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