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Idiopathic granulomatous hypophysitis presenting as pituitary apoplexy
510
Case Reports / Journal of Clinical Neuroscience 21 (2014) 510–512
Idiopathic granulomatous hypophysitis presenting as pituitary apoplexy Qasim Husain a, Alexandros Zouzias b, Vivek V. Kanumuri a, Jean Anderson Eloy a,b,c, James K. Liu a,b,c,⇑ a
Department of Otolaryngology-Head and Neck Surgery, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, NJ, USA Department of Neurological Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, 90 Bergen Street, Suite 8100, Newark, NJ 07103, USA c Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Department of Neurological Surgery, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, 185 S Orange Avenue, Newark, NJ 07103, USA b
a r t i c l e
i n f o
Article history: Received 29 April 2013 Accepted 5 May 2013
Keywords: Granulomatous hypophysitis Macroadenoma Pituitary apoplexy Skull base Transsphenoidal
a b s t r a c t Idiopathic granulomatous hypophysitis (IGH) is an extremely rare chronic inflammatory lesion of the pituitary gland. This condition typically presents with chronic onset of headache and slow development of visual deficits. Pituitary apoplexy is a clinical syndrome characterized by sudden onset of headache, vision loss, opthalmoplegia, and signs of meningeal irritation. Although IGH has been previously described in the literature, its presentation as clinical pituitary apoplexy is novel. We report, to our knowledge, the first patient with IGH manifesting as clinical pituitary apoplexy. Physicians involved in the treatment of pituitary disease should be aware of this rare entity. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Hypophysitis, a rare inflammatory disorder of the pituitary gland that can often be misdiagnosed as a mass lesion, represents <1% of patients who present with headache, hypopituitarism, and visual disturbances [1]. This entity upon microscopic examination can either be found to be lymphocytic or granulomatous. Granulomatous hypophysitis is characterized by the formation of intrasellar giant-cell granulomata. In the absence of underlying systemic disease, such as histiocytosis X, sarcoidosis, tuberculosis, or syphilis, it is classified as idiopathic [1,2]. Idiopathic granulomatous hypophysitis (IGH) typically presents with chronic onset of headache and slow development of visual deficits. To date, ante mortem patients with IGH have been scarce, with even fewer demonstrating preoperative imaging [2]. While there have been articles in the literature discussing this rare disease, to our knowledge IGH has not been previously reported in the context of clinical pituitary apoplexy requiring emergent surgery. Pituitary apoplexy is a clinical syndrome characterized by sudden onset of headache, vision loss, ophthalmoplegia, and signs of meningeal irritation. We report, to our knowledge, the first patient with IGH manifesting as clinical pituitary apoplexy. 2. Case report A 36-year-old woman presented with the sudden onset of leftsided retrorbital headaches, diplopia, and left eye ptosis. On neurologic examination, she had partial left oculomotor nerve palsy. On further investigation, the patient reported menstrual irregularities over the preceding 8 months and intermittent galactorrhea. MRI of the brain demonstrated an intensely enhancing 1.9 1.1 1.3 cm sellar mass with mild suprasellar extension and mass effect on the left cavernous sinus consistent with a macroadenoma. The central aspect of the lesion displayed a smaller non-enhancing area of low signal on T1-weighted imaging, consistent with necrosis (Fig. 1A, B). Endocrinological findings demonstrated a mildly elevated serum prolactin level of 46.9 ng/ml (normal range 4.8–23.3 ng/ml), ⇑ Corresponding author. Tel.: +1 973 972 2906; fax: +1 973 972 2333. E-mail address: [email protected] (J.K. Liu).
an elevated thyroid-stimulating hormone level of 5.88 mU/L (normal range .27–4.2 mU/L), and normal thyroxine, follicle-stimulating hormone, lutenizing hormone and growth hormone levels. A urine pregnancy test was negative. 2.1. Operation Clinical pituitary tumor apoplexy was suspected and the patient underwent an emergent endoscopic trannssphenoidal removal of the tumor to decompress the optic nerves and the left cavernous sinus. At surgery, a firm, rubbery, yellowish-white mass was noted. The mass was adherent to surrounding arachnoid and to the left cavernous sinus. Gross total resection of the tumor was achieved. 2.2. Pathological findings and postoperative course Histopathologic examination demonstrated chronic granulomatous inflammation with extensive caseous necrosis (Fig. 2). There was palisading of epithelioid cells around the zones of necrosis as well as multinucleated giant cells within the zones. Staining for organisms, including Gram, acid-fast, and Grocott-Gomori methenamine-silver nitrate, was negative for bacteria, mycobacteria, and fungi, respectively. A skin purified protein derivative test for tuberculosis was negative, as was a Quantiferon Gold tuberculosis assay (Cellestis, Valencia, CA, USA). Serum rapid plasma regain (RPR) levels for syphilis were negative, as was a cerebrospinal fluid (CSF) RPR test. Angiotensin-converting enzyme levels were found to be normal as part of the workup for sarcoidosis. CSF fluid cultures for bacteria and acid-fast bacilli showed no growth. No mycobacterium tuberculosis complex rRNA was detected on polymerase chain reaction assay of CSF. Postoperatively, the patient’s diplopia and oculomotor palsy resolved completely; visual fields and acuity were normal. She experienced an episode of transient diabetes insipidus following surgery, which responded to pitressin and oral desmopressin. An extensive systemic workup for sarcoidosis, tertiary syphilis, and Wegener’s granulomatosis was carried out; all results were negative. Endocrinological evaluation was notable for hypothyroidism. She was discharged in excellent condition on L-thyroxine replacement therapy. Postoperative MRI at 37 months demonstrated excellent resolution of the lesion with decompression of the optic
Case Reports / Journal of Clinical Neuroscience 21 (2014) 510–512
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Fig. 1. Preoperative sagittal (A) and coronal (B) T1-weighted gadolinium-enhanced MRI demonstrating a well-circumscribed and intensely enhancing lesion with a necroticappearing core. Note the involvement of the left cavernous sinus, with downward displacement of the left internal carotid artery. Postoperative sagittal (C) and coronal (D) T1-weighted gadolinium-enhanced MRI over 1 year following surgery showing completely resolved mass effect on the optic chiasm and left cavernous sinus.
Fig. 2. (A) Histopathology showing necrotizing granuloma, with epithelioid cells, elongated nuclei and with a multinucleated giant cell in the upper part of the image. Cells surrounding the granuloma are lymphocytes (hematoxylin and eosin, original magnification 10). (B) Immunohistochemistry showing granuloma with multinucleated giant cell and epithelioid cells, with prolactin-positive clusters of pituicytes. Unstained smaller cells outside the granuloma are lymphocytes (using an antibody to prolactin, with diaminobenzidine as chromogen and light hematoxylin counterstain, original magnification 10) (This figure is available in colour at www.sciencedirect.com).
chiasm and cavernous sinus (Fig. 1C, D). Clinical follow-up at 39 months was unremarkable. 3. Discussion Primary or idiopathic hypophysitis is an inflammatory pituitary reaction that is histologically characterized by lymphocytic, granulomatous, and xanthomatous subtypes [3]. IGH is the rarest entity in this group. Most cases of granulomatous hypophysitis are secondary to systemic granulomatous diseases, notably sarcoidosis, syphilis, and tuberculosis. It is considered idiopathic if no definite etiology is found. The largest review of granulomatous hypophysitis carried out was performed by Rickards and Harvey in 1954 with 115 documented patients and 23 idiopathic patients; however, all were diagnosed post mortem by autopsy [4]. Brisman et al. in 1996
performed a review of ante mortem diagnosis of IGH and identified 13 patients. However, only four of these patients had preoperative MRI and there was no report of acute neurologic deterioration [5]. The typical presentation of IGH in several reviews has been hypopituitarism with sellar compression symptoms, such as headache and visual changes [5]. The compressive effects can cause visual field defects as well as dysfunction of extraocular muscles [3]. Other presentations of IGH have been reported including diabetes insipidis and elevated prolactin [6]. Our patient with pituitary apoplexy due to IGH is a notable exception. Pituitary apoplexy is caused by acute hemorrhage or infarction into a pre-existing pituitary tumor. It presents most often with sudden onset of headache, nausea/vomiting, and low blood pressure, but can also present with visual field defects, ocular palsy (usually unilateral as seen in this patient), and/or altered mental
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Case Reports / Journal of Clinical Neuroscience 21 (2014) 510–512
status. The differential diagnosis for pituitary apoplexy includes migraine, meningitis, stroke, temporal arteritis, optic neuritis, subarachnoid hemorrhage, Rathke cleft cysts, or pituitary adenoma [7]. The most common type of hypophysitis, lymphocytic hypophysitis, has also been previously shown in rare cases to present as pituitary apoplexy [8]. Based on this and other evidence, previous studies have suggested that granulomatous hypophysitis should be included in the differential for pituitary apoplexy [9]. It is important to consider granulomatous hypophysitis when faced with the clinical picture of severe headaches, visual disturbancs, and nausea/vomiting. The radiographic findings of granulomatous hypophysitis are often indistinguishable from pituitary adenomas. Hypophysitis on CT scans present as a low density intrasellar mass with cystic areas and ring enhancement [3,10]. MRI findings include isointensity with gray matter on T1-weighted images with heterogenous intensity on T2-weighted imaging [10]. Other radiographic features include a well-circumscribed mass lesion with infundibular thickening [10]. It has also been suggested that hypophysitis tends to preferentially spread laterally, as it did in this current patient, which may be seen on MRI [3,9]. However, many of these findings remain consistent with the much more common pituitary adenoma, which may explain why the most common preoperative diagnosis in these patients is pituitary adenoma [3]. Thus, pathological examination of resected tissue is important for definitive diagnosis. Histological exam of granulomatous hypophysitis typically shows granulomatous inflammation with nodular aggregates of epithelioid histiocytes and multinucleate giant cells along with lymphocytes and other inflammatory cells. Fibrotic destruction of the pituitary stroma is also seen [3]. Lymphocytic hypophysitis differs from granulomatous hypophysitis due to the lack of these nodular aggregates. In order to complete the diagnosis of primary idiopathic hypophysitis, systemic granulomatous diseases such as sarcoidosis, syphilis, tuberculosis, fungal infections, or systemic vasculitides must be ruled out [3]. The management of IGH in patients with obvious nerve compression or visual defects is urgent transsphenoidal surgical exploration and decompression to maximize postoperative visual and cranial nerve function [3]. This is particularly true in this patient because of the life-threatening complications that can potentially arise from pituitary apoplexy. Immediate corticosteroid replacement should also be initiated in the setting of suspected pituitary apoplexy [7]. Postoperative results are generally favorable for visual dysfunction but not endocrine disturbances [3]. Therefore, hormone replacement may be necessary to compensate for loss of pituitary function [7]. http://dx.doi.org/10.1016/j.jocn.2013.05.002
4. Conclusion IGH is a rare lesion of the pituitary gland that may present in a similar fashion to pituitary apoplexy. Since this entity can appear identical to pituitary adenoma both radiographically and clinically, a high index of suspicion is warranted for accurate diagnosis. In the presence of caseous necrotizing granulomas, a thorough work-up for tuberculosis, sarcoidosis, and other granulomatous etiologies is essential. Treatment consists of transsphenoidal decompression in the face of acute neurological deterioration and evidence of mass effect, with hormone replacement therapy commonly required pre- or post-operatively for hypopituitarism.
Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. This paper was presented at the Combined Sections Meeting of The Triological Society, Scottsdale, AZ, January 27-29, 2011.
Acknowledgement We would like to thank Dr. Leroy Sharer for providing the pathological images.
References [1] Chung CH, Song MS, Cho HD, et al. A case of idiopathic granulomatous hypophysitis. Korean J Intern Med 2012;27:346–9. [2] Bhansali A, Velayutham P, Radotra BD, et al. Idiopathic granulomatous hypophysitis presenting as non-functioning pituitary adenoma: description of six cases and review of literature. Br J Neurosurg 2004;18:489–94. [3] Su SB, Zhang DJ, Yue SY, et al. Primary granulomatous hypophysitis: a case report and literature review. Endocr J 2011;58:467–73. [4] Rickards AG, Harvey PW. Giant-cell granuloma and the other pituitary granulomata. Q J Med 1954;23:425–39. [5] Brisman MH, Morgello S, Silvers A, et al. Idiopathic granulomatous hypophysitis. Neurosurg Focus 1996;1:e7. [6] Scanarini M, d’Avella D, Rotilio A, et al. Giant-cell granulomatous hypophysitis: a distinct clinicopathological entity. J Neurosurg 1989;71:681–6. [7] Verrees M, Arafah BM, Selman WR. Pituitary tumor apoplexy: characteristics, treatment, and outcomes. Neurosurg Focus 2004;16:E6. [8] Dan NG, Feiner RI, Houang MT, et al. Pituitary apoplexy in association with lymphocytic hypophysitis. J Clin Neurosci 2002;9:577–80. [9] Inoue T, Kaneko Y, Mannoji H, et al. Giant cell granulomatous hypophysitis manifesting as an intrasellar mass with unilateral ophthalmoplegia–case report. Neurol Med Chir (Tokyo) 1997;37:766–70. [10] Vasile M, Marsot-Dupuch K, Kujas M, et al. Idiopathic granulomatous hypophysitis: clinical and imaging features. Neuroradiology 1997;39:7–11.
Case Reports / Journal of Clinical Neuroscience 21 (2014) 510–512
Idiopathic granulomatous hypophysitis presenting as pituitary apoplexy Qasim Husain a, Alexandros Zouzias b, Vivek V. Kanumuri a, Jean Anderson Eloy a,b,c, James K. Liu a,b,c,⇑ a
Department of Otolaryngology-Head and Neck Surgery, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, NJ, USA Department of Neurological Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, 90 Bergen Street, Suite 8100, Newark, NJ 07103, USA c Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Department of Neurological Surgery, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, 185 S Orange Avenue, Newark, NJ 07103, USA b
a r t i c l e
i n f o
Article history: Received 29 April 2013 Accepted 5 May 2013
Keywords: Granulomatous hypophysitis Macroadenoma Pituitary apoplexy Skull base Transsphenoidal
a b s t r a c t Idiopathic granulomatous hypophysitis (IGH) is an extremely rare chronic inflammatory lesion of the pituitary gland. This condition typically presents with chronic onset of headache and slow development of visual deficits. Pituitary apoplexy is a clinical syndrome characterized by sudden onset of headache, vision loss, opthalmoplegia, and signs of meningeal irritation. Although IGH has been previously described in the literature, its presentation as clinical pituitary apoplexy is novel. We report, to our knowledge, the first patient with IGH manifesting as clinical pituitary apoplexy. Physicians involved in the treatment of pituitary disease should be aware of this rare entity. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Hypophysitis, a rare inflammatory disorder of the pituitary gland that can often be misdiagnosed as a mass lesion, represents <1% of patients who present with headache, hypopituitarism, and visual disturbances [1]. This entity upon microscopic examination can either be found to be lymphocytic or granulomatous. Granulomatous hypophysitis is characterized by the formation of intrasellar giant-cell granulomata. In the absence of underlying systemic disease, such as histiocytosis X, sarcoidosis, tuberculosis, or syphilis, it is classified as idiopathic [1,2]. Idiopathic granulomatous hypophysitis (IGH) typically presents with chronic onset of headache and slow development of visual deficits. To date, ante mortem patients with IGH have been scarce, with even fewer demonstrating preoperative imaging [2]. While there have been articles in the literature discussing this rare disease, to our knowledge IGH has not been previously reported in the context of clinical pituitary apoplexy requiring emergent surgery. Pituitary apoplexy is a clinical syndrome characterized by sudden onset of headache, vision loss, ophthalmoplegia, and signs of meningeal irritation. We report, to our knowledge, the first patient with IGH manifesting as clinical pituitary apoplexy. 2. Case report A 36-year-old woman presented with the sudden onset of leftsided retrorbital headaches, diplopia, and left eye ptosis. On neurologic examination, she had partial left oculomotor nerve palsy. On further investigation, the patient reported menstrual irregularities over the preceding 8 months and intermittent galactorrhea. MRI of the brain demonstrated an intensely enhancing 1.9 1.1 1.3 cm sellar mass with mild suprasellar extension and mass effect on the left cavernous sinus consistent with a macroadenoma. The central aspect of the lesion displayed a smaller non-enhancing area of low signal on T1-weighted imaging, consistent with necrosis (Fig. 1A, B). Endocrinological findings demonstrated a mildly elevated serum prolactin level of 46.9 ng/ml (normal range 4.8–23.3 ng/ml), ⇑ Corresponding author. Tel.: +1 973 972 2906; fax: +1 973 972 2333. E-mail address: [email protected] (J.K. Liu).
an elevated thyroid-stimulating hormone level of 5.88 mU/L (normal range .27–4.2 mU/L), and normal thyroxine, follicle-stimulating hormone, lutenizing hormone and growth hormone levels. A urine pregnancy test was negative. 2.1. Operation Clinical pituitary tumor apoplexy was suspected and the patient underwent an emergent endoscopic trannssphenoidal removal of the tumor to decompress the optic nerves and the left cavernous sinus. At surgery, a firm, rubbery, yellowish-white mass was noted. The mass was adherent to surrounding arachnoid and to the left cavernous sinus. Gross total resection of the tumor was achieved. 2.2. Pathological findings and postoperative course Histopathologic examination demonstrated chronic granulomatous inflammation with extensive caseous necrosis (Fig. 2). There was palisading of epithelioid cells around the zones of necrosis as well as multinucleated giant cells within the zones. Staining for organisms, including Gram, acid-fast, and Grocott-Gomori methenamine-silver nitrate, was negative for bacteria, mycobacteria, and fungi, respectively. A skin purified protein derivative test for tuberculosis was negative, as was a Quantiferon Gold tuberculosis assay (Cellestis, Valencia, CA, USA). Serum rapid plasma regain (RPR) levels for syphilis were negative, as was a cerebrospinal fluid (CSF) RPR test. Angiotensin-converting enzyme levels were found to be normal as part of the workup for sarcoidosis. CSF fluid cultures for bacteria and acid-fast bacilli showed no growth. No mycobacterium tuberculosis complex rRNA was detected on polymerase chain reaction assay of CSF. Postoperatively, the patient’s diplopia and oculomotor palsy resolved completely; visual fields and acuity were normal. She experienced an episode of transient diabetes insipidus following surgery, which responded to pitressin and oral desmopressin. An extensive systemic workup for sarcoidosis, tertiary syphilis, and Wegener’s granulomatosis was carried out; all results were negative. Endocrinological evaluation was notable for hypothyroidism. She was discharged in excellent condition on L-thyroxine replacement therapy. Postoperative MRI at 37 months demonstrated excellent resolution of the lesion with decompression of the optic
Case Reports / Journal of Clinical Neuroscience 21 (2014) 510–512
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Fig. 1. Preoperative sagittal (A) and coronal (B) T1-weighted gadolinium-enhanced MRI demonstrating a well-circumscribed and intensely enhancing lesion with a necroticappearing core. Note the involvement of the left cavernous sinus, with downward displacement of the left internal carotid artery. Postoperative sagittal (C) and coronal (D) T1-weighted gadolinium-enhanced MRI over 1 year following surgery showing completely resolved mass effect on the optic chiasm and left cavernous sinus.
Fig. 2. (A) Histopathology showing necrotizing granuloma, with epithelioid cells, elongated nuclei and with a multinucleated giant cell in the upper part of the image. Cells surrounding the granuloma are lymphocytes (hematoxylin and eosin, original magnification 10). (B) Immunohistochemistry showing granuloma with multinucleated giant cell and epithelioid cells, with prolactin-positive clusters of pituicytes. Unstained smaller cells outside the granuloma are lymphocytes (using an antibody to prolactin, with diaminobenzidine as chromogen and light hematoxylin counterstain, original magnification 10) (This figure is available in colour at www.sciencedirect.com).
chiasm and cavernous sinus (Fig. 1C, D). Clinical follow-up at 39 months was unremarkable. 3. Discussion Primary or idiopathic hypophysitis is an inflammatory pituitary reaction that is histologically characterized by lymphocytic, granulomatous, and xanthomatous subtypes [3]. IGH is the rarest entity in this group. Most cases of granulomatous hypophysitis are secondary to systemic granulomatous diseases, notably sarcoidosis, syphilis, and tuberculosis. It is considered idiopathic if no definite etiology is found. The largest review of granulomatous hypophysitis carried out was performed by Rickards and Harvey in 1954 with 115 documented patients and 23 idiopathic patients; however, all were diagnosed post mortem by autopsy [4]. Brisman et al. in 1996
performed a review of ante mortem diagnosis of IGH and identified 13 patients. However, only four of these patients had preoperative MRI and there was no report of acute neurologic deterioration [5]. The typical presentation of IGH in several reviews has been hypopituitarism with sellar compression symptoms, such as headache and visual changes [5]. The compressive effects can cause visual field defects as well as dysfunction of extraocular muscles [3]. Other presentations of IGH have been reported including diabetes insipidis and elevated prolactin [6]. Our patient with pituitary apoplexy due to IGH is a notable exception. Pituitary apoplexy is caused by acute hemorrhage or infarction into a pre-existing pituitary tumor. It presents most often with sudden onset of headache, nausea/vomiting, and low blood pressure, but can also present with visual field defects, ocular palsy (usually unilateral as seen in this patient), and/or altered mental
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Case Reports / Journal of Clinical Neuroscience 21 (2014) 510–512
status. The differential diagnosis for pituitary apoplexy includes migraine, meningitis, stroke, temporal arteritis, optic neuritis, subarachnoid hemorrhage, Rathke cleft cysts, or pituitary adenoma [7]. The most common type of hypophysitis, lymphocytic hypophysitis, has also been previously shown in rare cases to present as pituitary apoplexy [8]. Based on this and other evidence, previous studies have suggested that granulomatous hypophysitis should be included in the differential for pituitary apoplexy [9]. It is important to consider granulomatous hypophysitis when faced with the clinical picture of severe headaches, visual disturbancs, and nausea/vomiting. The radiographic findings of granulomatous hypophysitis are often indistinguishable from pituitary adenomas. Hypophysitis on CT scans present as a low density intrasellar mass with cystic areas and ring enhancement [3,10]. MRI findings include isointensity with gray matter on T1-weighted images with heterogenous intensity on T2-weighted imaging [10]. Other radiographic features include a well-circumscribed mass lesion with infundibular thickening [10]. It has also been suggested that hypophysitis tends to preferentially spread laterally, as it did in this current patient, which may be seen on MRI [3,9]. However, many of these findings remain consistent with the much more common pituitary adenoma, which may explain why the most common preoperative diagnosis in these patients is pituitary adenoma [3]. Thus, pathological examination of resected tissue is important for definitive diagnosis. Histological exam of granulomatous hypophysitis typically shows granulomatous inflammation with nodular aggregates of epithelioid histiocytes and multinucleate giant cells along with lymphocytes and other inflammatory cells. Fibrotic destruction of the pituitary stroma is also seen [3]. Lymphocytic hypophysitis differs from granulomatous hypophysitis due to the lack of these nodular aggregates. In order to complete the diagnosis of primary idiopathic hypophysitis, systemic granulomatous diseases such as sarcoidosis, syphilis, tuberculosis, fungal infections, or systemic vasculitides must be ruled out [3]. The management of IGH in patients with obvious nerve compression or visual defects is urgent transsphenoidal surgical exploration and decompression to maximize postoperative visual and cranial nerve function [3]. This is particularly true in this patient because of the life-threatening complications that can potentially arise from pituitary apoplexy. Immediate corticosteroid replacement should also be initiated in the setting of suspected pituitary apoplexy [7]. Postoperative results are generally favorable for visual dysfunction but not endocrine disturbances [3]. Therefore, hormone replacement may be necessary to compensate for loss of pituitary function [7]. http://dx.doi.org/10.1016/j.jocn.2013.05.002
4. Conclusion IGH is a rare lesion of the pituitary gland that may present in a similar fashion to pituitary apoplexy. Since this entity can appear identical to pituitary adenoma both radiographically and clinically, a high index of suspicion is warranted for accurate diagnosis. In the presence of caseous necrotizing granulomas, a thorough work-up for tuberculosis, sarcoidosis, and other granulomatous etiologies is essential. Treatment consists of transsphenoidal decompression in the face of acute neurological deterioration and evidence of mass effect, with hormone replacement therapy commonly required pre- or post-operatively for hypopituitarism.
Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. This paper was presented at the Combined Sections Meeting of The Triological Society, Scottsdale, AZ, January 27-29, 2011.
Acknowledgement We would like to thank Dr. Leroy Sharer for providing the pathological images.
References [1] Chung CH, Song MS, Cho HD, et al. A case of idiopathic granulomatous hypophysitis. Korean J Intern Med 2012;27:346–9. [2] Bhansali A, Velayutham P, Radotra BD, et al. Idiopathic granulomatous hypophysitis presenting as non-functioning pituitary adenoma: description of six cases and review of literature. Br J Neurosurg 2004;18:489–94. [3] Su SB, Zhang DJ, Yue SY, et al. Primary granulomatous hypophysitis: a case report and literature review. Endocr J 2011;58:467–73. [4] Rickards AG, Harvey PW. Giant-cell granuloma and the other pituitary granulomata. Q J Med 1954;23:425–39. [5] Brisman MH, Morgello S, Silvers A, et al. Idiopathic granulomatous hypophysitis. Neurosurg Focus 1996;1:e7. [6] Scanarini M, d’Avella D, Rotilio A, et al. Giant-cell granulomatous hypophysitis: a distinct clinicopathological entity. J Neurosurg 1989;71:681–6. [7] Verrees M, Arafah BM, Selman WR. Pituitary tumor apoplexy: characteristics, treatment, and outcomes. Neurosurg Focus 2004;16:E6. [8] Dan NG, Feiner RI, Houang MT, et al. Pituitary apoplexy in association with lymphocytic hypophysitis. J Clin Neurosci 2002;9:577–80. [9] Inoue T, Kaneko Y, Mannoji H, et al. Giant cell granulomatous hypophysitis manifesting as an intrasellar mass with unilateral ophthalmoplegia–case report. Neurol Med Chir (Tokyo) 1997;37:766–70. [10] Vasile M, Marsot-Dupuch K, Kujas M, et al. Idiopathic granulomatous hypophysitis: clinical and imaging features. Neuroradiology 1997;39:7–11.