Journal of Neuro-Ophthalmology, September 2014, Volume 34, Issue 3

A 16-Year-Old Boy With a Suprasellar Mass

A 16-Year-Old Boy With a Suprasellar Mass Madhura A. Tamhankar, MD, Michele E. Paessler, DO, Julia N. Kharlip, MD, Karuna V. Shekdar, MD, Jon M. Burnham, MD, MSCE, Kristina A. Cole, MD, PhD Dr. Tamhankar: A16-year-old man had headaches and nausea for 6 months. He denied visual symptoms, fevers, chills, abdominal pain, weight loss, or loss of appetite; however, he had been experiencing increased thirst and urination for 2 years. Magnetic resonance imaging (MRI) was performed. Dr. Shekdar: Initial MRI of the brain reveals a suprasellar mass that is 1.1 · 1.1 · 0.7 cm in dimension, extending along the infundibu-lum into the suprasellar cistern and abutting the optic chiasm (Fig. 1A, B). This mass appears to extend into the hypothal-amus. It is isointense on T1-weighted images and enhances diffusely with gadolinium. The pituitary gland is flattened and displaced, and there is mild displacement of the chiasm. The rest of the brain MRI is normal. The radiologic differential of this mass causing pituitary stalk thickening and diabetes insipidus includes conditions such as Langerhans cell histiocytosis, sarcoidosis, Wegener granulo-matosis, lymphocytic hypophysitis, tuberculosis, and neoplastic lesions such as lymphoma, leukemia, germ cell tumor, craniopharyngioma, and hypothalamic/chiasmal glioma (1,2). Drs. Tamhankar and Kharlip: The patient subsequently underwent an endocrinologic evaluation that revealed hypopituitarism characterized by central hypothyroidism, hypogonadism, growth hormone deficiency, and diabetes insipidus. He was placed on desmopressin acetate and levothyroxine as well as testoster-one. Growth hormone replacement was withheld until the nature of the pituitary lesion could be ascertained. He was referred for a neuro-ophthalmologic examination. The patient's visual acuity was 20/20 bilaterally with normal color vision and normal pupillary reactions to light stimulation; there was no relative afferent pupillary defect. Automated perimetry (Humphrey 24-2) showed an incom-plete bitemporal hemianopia (Fig. 2A). There was bilateral optic disc pallor. The patient underwent a lumbar puncture with cytology, a bone scan, and a whole-body positron emission tomography/ computed tomography (PET/CT), all of which gave normal results. Serum and cerebrospinal fluid (CSF) tumor markers a-fetoprotein (aFP) and b-human chorionic gonadotropin levels were normal. Shortly afterward, the patient underwent a transsphenoidal biopsy of the mass. Dr. Paessler: The specimen obtained at biopsy demonstrates a dense lymphoplasmacytic infiltrate with scattered plasma cells and few histiocytes with residual pituitary acini in the background and rare germinal centers. The lymphocytes are predominantly small, although there are occasional large transformed lympho-cytes (Fig. 3A). No neoplasm is seen. The findings were thought to be most consistent with lymphocytic hypophysitis. Dr. Tamhankar: After the diagnosis of lymphocytic hypophysitis, the patient was started on 60 mg of oral prednisone per day for 2 weeks followed by a 4-week taper. His visual fields initially improved (Fig. 2B). Two weeks later, visual acuity in the left eye declined to 20/70, whereas acuity in the right eye remained 20/20. Worsening of visual fields occurred after discontinuation of steroids (Fig. 2C), and anotherMRI of the brain was obtained. Dr. Shekdar: MRI reveals persistence of the mass, which is essentially unchanged compared with the initial scan, except for a new area of enhancement and thickening of the left side of the optic chiasm (Fig. 4). Dr. Tamhankar: It was thought that the chiasmal changes represented spread of contiguous inflammation from an inflamed pituitary gland. Given the rapidity of visual loss, the decision was made to treat the patient with intravenous methylprednisolone 250 mg every 4 hours for 3 days. On this regimen, visual acuity in the Department of Ophthalmology (MAT), Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Endocri-nology (JNK), University of Pennsylvania, Philadelphia, Pennsylvania; Departments of Pathology (MEP), Radiology (KVS), Rheumatology (JMB), and Oncology (KAC), Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania. The authors report no conflicts of interest. Address correspondence to Madhura A. Tamhankar, MD, Division of Neuro-Ophthalmology, Scheie Eye Institute, 51 N, 39th Street, Philadelphia, PA 19104; E-mail: [e-mail] Tamhankar et al: J Neuro-Ophthalmol 2014; 34: 295-300 295 Clinical-Pathological Case Study Section Editors: Neil R. Miller, MD Janet Rucker, MD Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. left eye improved to 20/40 along with improvement in visual fields (Fig. 2D). Accordingly, a slower taper of prednisone was instituted over the next 3 weeks from 80 to 60 mg. Follow-up MRI showed reduction in the size of the sellar mass; however, there was persistent, unchanged enhancement, and thickening of the left side of the optic chiasm. Due to the unusual clinical course necessitating prolonged steroid therapy, a review of the original pathology was requested. Dr. Paessler: The original histology slides were reviewed and additional immunohistochemical stains were performed. These dem-onstrated that the diffuse infiltrate was composed pre-dominantly of CD20+, CD79a+ B cells (Fig. 3B) and admixture of small CD3- and CD5-positive T cells. Kappa and lambda immunoglobulin light chain stains did not reveal a monoclonal population. CD1a stain was nega-tive, and CD3 and CD5 stains highlighted reactive T cells. Ki67 was positive in a cluster of cells consistent with germinal center B cells. Stains for CD10, BCL1, and BCL6 were non-diagnostic. Given the diffuse B-lymphocytic infiltrate, a small B-cell lymphoma was considered in the differential diagnosis. Attempts were made to test for IgH clonality with molecular methods; however, the tiny focus containing atypical lymphocytes was not present in additional sections, and the test could not be performed. Dr. Tamhankar: Although lymphoma could not be excluded based on the morphology and immunophenotype of the infiltrate, the findings in this clinical context, coupled with the location of lesion, relative stability of the lesion on MRI over a 5- month period, age of the patient, long duration of previous symptoms, and negative whole-body PET scan were still thought to be compatible with a marked inflammatory response, as seen in lymphocytic hypophysitis. Drs. Burnham and Tamhankar: Because this was thought to be an aggressive form of lymphocytic hypophysitis, the patient was maintained on 60 mg/d of prednisone for 3 weeks, and then a slow taper of 5 mg of prednisone per week was instituted. He began to experience side effects from chronic steroid use, and it was decided to switch him to a steroid-sparing agent. He was first given mycophenolate mofetil. As further attempts to wean him from prednisone led to visual decline, he underwent plasmapheresis and then given 2 cycles of cyclophosphamide. In addition, because the majority of lymphocytes were CD20 positive, rituximab was added, and FIG. 2. A-E, Results of serial visual field testing. prednisone was tapered gradually. FIG. 1. Initial magnetic resonance imaging. Postcontrast sagittal (A) and coronal (B) T1 scans show a sellar mass extending into the suprasellar cistern and compressing the optic chiasm. 296 Tamhankar et al: J Neuro-Ophthalmol 2014; 34: 295-300 Clinical-Pathological Case Study Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. During the next 8 weeks, the patient's visual fields fluc-tuated. Vision remained 20/20, right eye, but decreased to 20/50, left eye. On repeat MRI, there was a slight decrease in chiasmal thickening and enhancement (Fig. 5). On tapering the prednisone to 30 mg/d, the patient experienced an acute drop in visual acuity in the left eye to 20/100, associated with worsening of his visual fields (Fig. 2E). Once again, the original biopsy specimen was reviewed. FIG. 3. Transsphenoidal biopsy specimen. A. The tissue demonstrates a predominance of lymphocytes (hematoxylin and eosin, ·40). B. Immunstaining shows that the majority of lymphocytes are C20 positive (·40). C. Atypical cells are present (arrows) (hematoxylin and eosin, ·20). D. The large atypical cells show positive staining for CD117 confirming the diagnosis of germinoma (·20). E. Characteristic 2-cell pattern of large polygonal germinoma cells (arrows) adjacent to lymphocytes (hematoxylin and eosin, ·63). FIG. 4. Magnetic resonance imaging 5 months after initial presentation. Postcontrast sagittal (A) and coronal (B) T1 images reveal that the sellar mass is unchanged but there is thickening and enhancement of the left side of the optic chiasm (arrow). Tamhankar et al: J Neuro-Ophthalmol 2014; 34: 295-300 297 Clinical-Pathological Case Study Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Dr. Paessler: We now noted a focal area of large atypical cells amidst the diffuse lymphocytic infiltrate on one of the fragments of tissue. This raised concern for a neoplasm (Fig. 3C). The specimen was sent to the National Cancer Institute, where the original hematoxylin and eosin slides were destained and then restained with CD117. There was positive staining in the large atypical cells (Fig. 3D), consistent with the diag-nosis of a germinoma (Fig. 3E). Dr. Cole: The patient underwent a repeat metastatic workup, includ-ing CSF cytology and MRI of the spine. No evidence of metastases was found, and serum and CSF germ cell markers remained negative. MRI revealed interval enlarge-ment of the chiasmal mass with extension into the left optic nerve and hypothalamus. Given the imaging findings and a decline in the patient's visual acuity to 20/100, left eye, he underwent proton beam radiation therapy (total dose: 4,500 cGy). This resulted in significant reduction in the size and enhancement of the sellar and chiasmal lesion and stabilization of his vision (Fig. 6). Drs. Tamhankar and Cole: The present case emphasizes the challenges sometimes encountered in establishing a correct etiology for suprasellar and sellar masses when they share similar clinical, imaging, and pathologic features. Lymphocytic hypophysitis and germinoma share similarities not just in their clinical and imaging presentations, but also in pathology, rendering the diagnosis difficult in some patients. Lymphocytic hypophysitis is a rare autoimmune condi-tion that can involve the anterior pituitary, posterior pituitary, or the entire gland. It accounts for 0.38%- 1.1% of sellar masses excised during transsphenoidal surgery (3). Although it is most often seen in young women in the antepartum or postpartum period (4), a variant of lympho-cytic hypophysitis involving the infundibulum and neuro-hypophysis causing diabetes insipidus has been reported in men and children (5,6). Intracranial germinomas comprise 0.1%-3.4% of all intracranial neoplasms, occurring most commonly in the second decade of life in boys (average age: 12 years). They typically arise from the pineal region and less commonly in suprasellar and ventricular locations (7,8). The incidence of intracranial germinoma is higher in Japan than in America (3.4% vs ,1%) (9,10). Lymphocytic hypophysitis and germinoma can present with similar neurological symptoms and signs including headache, visual loss, hypopituitarism, and diabetes insipidus (4,11,12). The mechanism of visual loss in lymphocytic hypophy-sitis is usually mechanical compression of the visual path-ways (13-15) or an inflammatory optic neuropathy (5,16,17), as was presumed to be the case in our patient. Inflammatory thickening of the optic nerves can be seen on MRI in patients with optic neuropathy. However, the degree of chiasmal enlargement and enhancement in our patient was unusual for lymphocytic hypophysitis (5,16). Involvement of the visual pathways by germinoma is rare and generally occurs from suprasellar extension (18-21) or, FIG. 5. Magnetic resonance imaging 7 months after initial presentation. Postcontrast coronal T1 scan shows improvement in the left-sided chiasmal thickening and enhancement. FIG. 6. Magnetic resonance imaging 6 months after proton beam radiation. Postcontrast sagittal (A) and coronal (B) scans show reduction of the sellar mass and chiasmal thickening and enhancement. 298 Tamhankar et al: J Neuro-Ophthalmol 2014; 34: 295-300 Clinical-Pathological Case Study Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. rarely, from primary tumor infiltration leading to massive enlargement of the optic nerves or chiasm (22-27). On neuroimaging, both lymphocytic hypophysitis and germinoma can demonstrate thickening of the infundibu-lum. This finding also can be seen in other entities such as Langerhans cell histiocytosis, sarcoidosis, and other neo-plastic lesions (1,2). Germinomas in this region typically arise in the suprasellar space and secondarily involve the pituitary stalk. They are isointense or slightly hyperintense to adjacent brain and diffusely enhance with gadolinium. Cystic areas along with variable enhancement can be seen in germinomas (28). In most patients presenting with an iso-lated sellar mass, endoscopic biopsy is generally required to make the correct diagnosis (29). Histologically, lymphocytic hypophysitis is composed of a dense lymphoplasmacytic infiltrate in a background of normal hypophyseal tissue with a variable number of B and T lymphocytes, neutrophils, eosinophils, and histiocytes (30). In contrast, in 75% of cases, pathologic examination of germinomas reveals infiltrating lymphocytes adjacent to nests of neoplastic cells in a 2-cell pattern (Fig. 3E). The germinoma cells are large polygonal cells with pale eosino-philic cytoplasm with vacuolation, round nuclei, and prom-inent nucleoli (31). In 25% of cases, lymphocytes largely outnumber the few isolated neoplastic cells. In such cases, the diagnosis can be extremely challenging, as was the case in our patient (32,33). Germinomas are highly immuno-genic tumors and can exhibit the highest degree of tumor-infiltrating lymphocytes among human neoplasia (34). Because germinoma cells were not seen on initial examina-tion, germinoma-specific stains were not obtained. There are fewer than a dozen case reports of an occult germinoma being diagnosed initially as lymphocytic hypo-physitis (4,34-41). In some, there was initial clinical suspi-cion for lymphocytic hypophysitis, but failure to improve with steroids led to a diagnosis of germinoma that was con-firmed by biopsy (36,37). In other cases, special stains such a CD117 and OCT4 led to the diagnosis of germinoma after failure of the sellar mass to regress with steroids (34,38,39), whereas in others, the initial biopsy was interpreted as lym-phocytic hypophysitis and later disproved on repeat biopsy performed due to clinical (40,41) or imaging progression (4). Another diagnostic clue was that the mass either did not respond to steroids, or the response was transient and increase in size of the tumor occurred despite steroid treatment. Our patient was unusual in that there was regression of the sellar mass with prolonged steroid treatment. It is likely that the intense lymphocytic infiltration was steroid responsive, and thus contributed to tumor shrinkage and led us to believe that the mass was inflammatory. Although there are reports of spontaneous (42,43) or steroid-induced regression (44,45) of intracranial germinomas, this is rare (44). The persistent steroid-refractory chiasmal thickening and enhancement in our patient may represent seeding of germinoma cells within the chiasm (18,19,46). In summary, it is important to remember that germi-nomas can be accompanied by an intense lymphocytic infiltration that can make the neoplastic cells difficult to identify on histopathological examination (41). It is impor-tant to maintain a high index of suspicion for a germ cell tumor in a young male presenting with a suprasellar mass, diabetes mellitus, and hypopituitarism, particularly as de-layed diagnosis and treatment may adversely affect overall survival because of tumor dissemination (47,48). REFERENCES 1. Kanagaki M, Miki Y, Takahashi JA, Shibamoto Y, Takahashi T, Ueba T, Hashimoto N, Konishi J. MRI and CT findings of neurohypophyseal germinoma. Eur J Radiol. 2004;49: 204-211. 2. Shin JH, Lee HK, Choi CG, Suh DC, Kim CJ, Hong SK, Na DG. MR imaging of central diabetes insipidus: a pictorial essay. Korean J Radiol. 2001;2:222-230. 3. Buxton N, Robertson I. Lymphocytic and granulocytic hypophysitis: a single centre experience. Br J Neurosurg. 2001;15:242-245. 4. Caturegli P, Lupi I, Landek-Salgado M, Kimura H, Rose NR. Pituitary autoimmunity: 30 years later. Autoimmun Rev. 2008;7:631-637. 5. Al-Mujaini A, Ganesh A, Al-Zuhaibi S, Al-Dhuhli H, Al-Mashani A, Al-Kindi H, Al-Memari A, Al-Futaisi A, Al-Asmi A. Lymphocytic infundibulo-neurohypophysitis: an unusual cause of recurrent optic neuropathy in a child. J AAPOS. 2009;13:207-209. 6. Maghnie M, Genovese E, Sommaruga MG, Aricò M, Locatelli D, Arbustini E, Pezzotta S, Severi F. Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is "lymphocytic infundibuloneurohypophysitis" in children a different entity? Eur J Endocrinol. 1998;139:635-640. 7. Horowitz MB, Hall WA. Central nervous system germinomas. A review. Arch Neurol. 1991;48:652-657. 8. Packer RJ, Sutton LN, Rosenstock JG, Rorke LB, Bilaniuk LT, Zimmerman RA, Littman PA, Bruce DA, Schut L. Pineal region tumors of childhood. Pediatrics. 1984;74:97-102. 9. Villano JL, Propp JM, Porter KR, Stewart AK, Valyi-Nagy T, Li X. Malignant pineal germ-cell tumors: an analysis of cases from three tumor registries. Neuro Oncol. 2008;10:121-130. 10. Goodwin TL, Sainani K, Fisher PG. Incidence patterns of central nervous system germ cell tumors: a SEER Study. J Pediatr Hematol Oncol. 2009;31:541-544. 11. Packer RJ, Cohen BH, Cooney K. Intracranial germ cell tumors. Oncologist. 2000;5:312-320. 12. Kageyama N, Belsky R. Ectopic pinealoma in the chiasma region. Neurology. 1961;11:318-327. 13. Cosman F, Post KD, Holub DA, Wardlaw SL. Lymphocytic hypophysitis. Report of 3 new cases and review of the literature. Medicine (Baltimore). 1989;68:240-256. 14. Leung GK, Lopes MB, Thorner MO, Vance ML, Laws ER Jr. Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg. 2004;101:262-271. 15. Gutenberg A, Hans V, Puchner MJ, Kreutzer J, Brück W, Caturegli P, Buchfelder M. Primary hypophysitis: clinical-pathological correlations. Eur J Endocrinol. 2006;155: 101-107. 16. Tamiya A, Saeki N, Mizota A. Lymphocytic infundibulo-neurohypophysitis associated with recurrent optic neuritis. Br J Neurosurg. 2001;15:180-183. 17. Zoeller GK, Benveniste RJ, Farhadi FA, Bruce JH. Lymphocytic hypophysitis in a patient presenting with sequential episodes of optic neuritis. Pituitary. 2012;15:101-105. 18. Bowman CB, Farris BK. Primary chiasmal germinoma. A case report and review of the literature. J Clin Neuroophthalmol. 1990;10:9-17. Tamhankar et al: J Neuro-Ophthalmol 2014; 34: 295-300 299 Clinical-Pathological Case Study Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. 19. Cohen DN, Steinberg M, Buchwald R. Suprasellar germinomas: diagnostic confusion with optic gliomas. Case report. J Neurosurg. 1974;41:490-493. 20. Kageyama N. Ectopic pinealoma in the region of the optic chiasm. Report of five cases. J Neurosurg. 1971;35:755-759. 21. Simson LR, Lampe I, Abell MR. Suprasellar germinomas. Cancer. 1968;22:533-544. 22. Wilson JT, Wald SL, Aitken PA, Mastromateo J, Vieco PT. Primary diffuse chiasmatic germinomas: differentiation from optic chiasm gliomas. Pediatr Neurosurg. 1995;23:1-5. 23. Iizuka H, Nojima T, Kadoya S. Germinoma of the optic nerve: case report. Noshuyo Byori. 1996;13:95-98. 24. Nadkarni TD, Fattepurkar SC, Desai KI, Goel A. Intracranial optic nerve germinoma. J Clin Neurosci. 2004;11:559-561. 25. Chavy B, Rossazza C, Jan M, Bazeze V, Tabouy P. Germinoma of the optic pathway (apropos of a case) (article in French). Bull Soc Ophtalmol Fr. 1988;88:145-148. 26. DiLuna ML, Two AM, Levy GH, Patel T, Huttner AJ, Duncan CC, Piepmeier JM. Primary, non-exophytic, optic nerve germ cell tumors. J Neurooncol. 2009;95:437-443. 27. Xia C, Liu Z, Zhang R, Mao Y, Wang Y. Primary intrinsic optic chiasm germinoma. J Clin Neurosci. 2011;18:860-862. 28. Liang L, Korogi Y, Sugahara T. MRI of intracranial germ-cell tumours. Neuroradiology. 2002;44:382-388. 29. Reddy AT, Wellons JC III, Allen JC, et al. Refining the staging evaluation of pineal region germinoma using neuroendoscopy and the presence of preoperative diabetes insipidus. Neuro Oncol. 2004;6:127-133. 30. Waki K, Yamada S, Ozawa Y, Seki K, Endo Y. A case of lymphocytic infundibuloneurohypophysitis: histophathological studies. Pituitary. 1999;1:285-290. 31. Hirato J, Nakazato Y. Pathology of pineal region tumors. J Neurooncol. 2001;54:239-249. 32. Kraichoke S, Cosgrove M, Chandrasoma PT. Granulomatous inflammation in pineal germinoma. A cause of diagnostic failure at stereotaxic brain biopsy. Am J Surg Pathol. 1988;12:655-660. 33. Utsuki S, Oka H, Tanizaki Y, Kondo K, Kawano N, Fujii K. Histological features of intracranial germinomas not disappearing immediately after radiotherapy. Neurol Med Chir (Tokyo). 2006;46:429-433. 34. Gutenberg A, Bell JJ, Lupi I, Tzou SC, Landek-Salgado MA, Kimura H, Su J, Karaviti LP, Salvatori R, Caturegli P. Pituitary and systemic autoimmunity in a case of intrasellar germinoma. Pituitary. 2011;14:388-394. 35. Bettendorf M, Fehn M, Grulich-Henn J, Selle B, Darge K, Lüdecke DK, Heinrich UE, Saeger W. Lymphocytic hypophysitis with central diabetes insipidus and consequent panhypopituitarism preceding a multifocal, intracranial germinoma in a prepubertal girl. Eur J Pediatr. 1999;158:288-292. 36. Ozbey N, Sencer A, Tanyolac S, Kurt R, Sencer S, Bilgic B, Turantan I, Molvalilar S. An intrasellar germinoma with normal cerebrospinal fluid beta-HCG concentrations misdiagnosed as hypophysitis. Hormones (Athens). 2006;5:67-71. 37. Nishiuchi T, Imachi H, Murao K, Fujiwara M, Sato M, Nishiuchi Y, Kushida Y, Haba R, Shindo A, Tamiya T, Ishida T. Suprasellar germinoma masquerading as lymphocytic hypophysitis associated with central diabetes insipidus, delayed sexual development, and subsequent hypopituitarism. Am J Med Sci. 2010;339:195-199. 38. Torremocha F, Hadjadj S, Menet E, Kas A, Bourgeois H, Levillain P, Bataille B, Maréchaud R. Pituitary germinoma presenting as a pseudotumoral lymphocytic hypophysitis in a man (article in French). Ann Endocrinol (Paris). 2002;63: 13-17. 39. Houdouin L, Polivka M, Henegar C, Blanquet A, Delalande O, Mikol J. Pituitary germinoma and lymphocytic hypophysitis: a pitfall. Report of two cases (article in French). Ann Pathol. 2003;23:349-354. 40. Mikami-Terao Y, Akiyama M, Yanagisawa T, Takahashi- Fujigasaki J, Yokoi K, Fukuoka K, Sakuma M, Miyata I, Fujisawa K, Oi S, Eto Y. Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl. Childs Nerv Syst. 2006;22:1338-1343. 41. Fehn M, Bettendorf M, Lüdecke DK, Sommer C, Saeger W. Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl: a case report. Pituitary. 1999;1:303-307. 42. Ide M, Jimbo M, Yamamoto M, Hagiwara S, Aiba M, Kubo O. Spontaneous regression of primary intracranial germinoma. A case report. Cancer. 1997;79:558-563. 43. Fujimaki T, Mishima K, Asai A, Suzuki I, Kirino T. Spontaneous regression of a residual pineal tumor after resection of a cerebellar vermian germinoma. J Neurooncol. 1999;41:65-70. 44. Mascalchi M, Roncaroli F, Salvi F, Frank G. Transient regression of an intracranial germ cell tumour after intravenous steroid administration: a case report. J Neurol Neurosurg Psychiatry. 1998;64:670-672. 45. Masoudi A, Amini E, Wolff JE, Fuller GN, Ketonen L, Mahajan A. Shrinkage of germinoma by dexamethasone only. Pediatr Blood Cancer. 2008;50:1079. 46. Pereira LS, Green AJ, Hwang TN, McCulley TJ. Suprasellar germinoma and late perioptic seeding. Eur J Ophthalmol. 2008;18:159-161. 47. Mootha SL, Barkovich AJ, Grumbach MM, Edwards MS, Gitelman SE, Kaplan SL, Conte FA. Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. J Clin Endocrinol Metab. 1997;82:1362-1367. 48. Sethi RV, Marino R, Niemierko A, Tarbell NJ, Yock TI, MacDonald SM. Delayed diagnosis in children with intracranial germ cell tumors. J Pediatr. 2013;163:1448-1453. 300 Tamhankar et al: J Neuro-Ophthalmol 2014; 34: 295-300 Clinical-Pathological Case Study Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.