Idiopathic Granulomatous Hypophysitis Research Articles

ODP318 Idiopathic Granulomatous Hypophysitis : A Rare Cause of Hypopituitarism

Abstract Introduction Pituitary hypophysitis is an inflammatory disorder classified as primary or secondary. The five histologic subtypes of primary hypophysitis are lymphocytic, granulomatous, xanthomatous, IgG4-related, and necrotizing. Idiopathic granulomatous hypophysitis (IGH) has an incidence of 1 in 10 million and accounts for < 1% of cases involving panhypopituitarism, headache, and visual disturbances. We present the case of IGH in a patient initially suspected to have pituitary adenoma. Case Presentation A 54-year-old female with a history of hypertension presented to the emergency department with nausea, vomiting, and acute worsening of a chronic headache of 1-week duration. Her past medical history was significant for a suspected pituitary macroadenoma of 1.7 cm, which was incidentally detected 7 months before the current presentation. She reported a history of 9 lb weight gain in one month, hot flashes, and diplopia, but denied hirsutism, polyuria, polydipsia, and galactorrhea. Prior evaluation revealed normal prolactin, TSH, and visual field testing. Her medication and familial history were unremarkable. Physical exam during the current presentation revealed stable vital signs, left eye exophthalmos, left anisocoria, decreased sensation in the left V1 and V2 trigeminal nerve distribution, and right abducens nerve palsy. Her initial laboratory workup was consistent with panhypopituitarism. Brain Magnetic Resonance Imaging (MRI) revealed an extensive, locally invasive, exuberantly enhancing infiltrative process measuring at least 5.6 cm in maximal transverse diameter involving the pituitary gland with bilateral cavernous sinus and Meckel's caves invasion and extension into bilateral superior orbital fissures. The patient underwent supraorbital craniotomy with subtotal resection of the tumor. Tuberculosis, sarcoidosis, and neoplasm were ruled out by appropriate tests, and the patient was diagnosed to have IGH on histology. Her symptoms improved postoperatively and the patient was discharged on levothyroxine and hydrocortisone. Conclusion Clinically and radiologically, IGH is a rare pathology that can closely mimic a pituitary adenoma. Through this case, we highlight the importance of performing a comprehensive evaluation with a full hormonal workup and imaging, with the exclusion of secondary causes while assessing patients with a sellar mass. Presentation: No date and time listed

Idiopathic granulomatous hypophysitis mimicking adenoma

Hypophysitis is a rare disease of pituitary gland, which, although it is usually a primary lesion, can also occur secondary to systemic conditions. Granulomatous hypophysitis is an inflammatory disease condition which accounts for less than 1% of all cellular lesions and can mimic adenoma.A 32-year-old woman presented with weight gain, galactorrhea and blurred vision. The MRI showed a cystic, nodular lesion in the intermediate lobe of the pituitary gland and the initial diagnosis was adenoma. She underwent surgery and the histopathology revealed granulomas composed of epithelioid histiocytes, multinuclear giant cells and mononuclear inflammatory cells.Inflammatory diseases of the pituitary gland are much less frequent than pituitary adenomas and idiopathic granulomatous hypophysitis is extremely rare. Histopathology and the ruling out of a systemic cause are the gold standards for its diagnosis.

Idiopathic granulomatous hypophysitis presenting with galactorrhea, headache, and nausea in a woman: a case report and review of the literature

BackgroundInflammation of the pituitary gland can occur in a variety of primary or secondary disorders. Idiopathic granulomatous hypophysitis is a rare inflammatory disease of the pituitary gland that can closely mimic a pituitary adenoma clinicoradiologically. Most authorities agree on minimally invasive transsphenoidal surgery as the mainstay in diagnosis and treatment of this disorder. There is still some controversy regarding pure medical management of idiopathic granulomatous hypophysitis in the literature.Case presentationA 47-year-old Iranian woman of Azeri ethnicity with a history of benign breast cysts with a chief complaint of galactorrhea presented to our endocrinology clinic. Her past medical history was negative for any menstrual irregularities, hirsutism, visual complaints, diplopia, polyuria and polydipsia or seizures. She was taking 100 mcg of levothyroxine daily. Her familial history and physical examination were unremarkable. Her initial laboratory work-up revealed hyperprolactinemia (82.4 ng/mL) with otherwise normal pituitary axes. Brain magnetic resonance imaging showed a pituitary macroadenoma for which she was treated with 0.5 mg of cabergoline weekly. Although her serum prolactin level dropped to 1.7 ng/mL and her galactorrhea was resolved, she continued to complain of headaches and nausea. Repeated imaging showed no decrease in size of the macroadenoma. Therefore, she underwent transsphenoidal surgery of the macroadenoma which was reported as chronic granulomatous hypophysitis by expert pathologists. Tuberculosis, sarcoidosis, Wegener’s granulomatosis, Langerhans cell histiocytosis, and syphilis were ruled out by appropriate tests and she was diagnosed as having idiopathic granulomatous hypophysitis. Fortunately, her condition was not complicated by hypopituitarism and she was symptom free 9 months after transsphenoidal surgery.ConclusionsIdiopathic granulomatous hypophysitis, a rare inflammatory disease of the pituitary gland, is a diagnosis of exclusion for which both medical and surgical management are reported in the literature. We present a case of idiopathic granulomatous hypophysitis who was symptom free with no complications of hypopituitarism following its transsphenoidal resection after 9 months of follow-up.

Abstract #912: Nine Year Follow Up of a 45 Year Old Female with Idiopathic Granulomatous Hypophysitis Maintaining Normal Pituitary Function

Abstract #912: Nine Year Follow Up of a 45 Year Old Female with Idiopathic Granulomatous Hypophysitis Maintaining Normal Pituitary Function

Hypophysitis : Types and differential diagnosis

Inflammatory findings in the pituitary glands account for approximately 1 % of operations in the sellar region. Primary inflammation (e.g. lymphocytic and idiopathic granulomatous hypophysitis) have to be differentiated from secondary types (e.g. concomittant inflammation with Rathke's cleft cysts, craniopharyngiomas and germinomas) and involvement of the pituitary in generalized inflammation (IgG4-related disease, sarcoidosis and septicopyemia). Langerhans cell histiocytosis also has to be considered for the differential diagnostics. Lymphocytic hypophysitis shows lymphocytic infiltrations of varying density, predominantly of the T‑cell type. Granulomatous hypophysitis has the features of sarcoidosis and can only be diagnosed by exclusion of generalized sarcoidosis. Secondary hypophysitis has a mixed cell infiltration, especially by histiocytic infiltration and predominantly originates from ruptures or bleeding from Rathke's cleft cysts. The frequently very sparse remnants of cyst epithelium should be confirmed by pankeratin immunostaining.

Idiopathic granulomatous hypophysitis presenting as pituitary apoplexy

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.

Idiopathic granulomatous hypophysitis: a systematic review of 82 cases in the literature

Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary. There is debate in the scientific literature as to whether IGH represents a continuum of disease with lymphocytic hypophysitis or has a distinct pathogenesis. Due to the rare nature of the disease, previous descriptions have been limited to single case reports or small series. In the present study, a systematic review of the literature was performed for cases of IGH. 82 cases met inclusion criteria. Data was gathered on IGH clinical aspects, in order to elucidate any associations useful in determining pathogenesis, appropriate clinical treatment, or prognosis. Univariate and multivariate analysis was performed on available data. Female sex was significantly associated with IGH (p < 0.0001). Fever (p = 0.002), nausea or vomiting at presentation (p = 0.031), and histological evidence of necrosis (p = 0.022) correlated with reduced time to presentation. Panhypopituitarism at presentation predicted need for long term hormone replacement (p = 0.014). Hyperprolactinaemia (p = 0.032), normal gonadal (p = 0.037) and thyroid axes (p = 0.001) were associated with reduced likelihood of long-term hormone replacement. Anorexia (p = 0.017), cold intolerance (p = 0.046), and fatigue (p = 0.0033) were associated with death from IGH. Patients who had excisional surgery alone trended towards increased rates of symptom resolution, compared with patients who received corticosteroids as an adjunct to excisional surgery (p = 0.11). This article details the first systematic review of IGH, and presents evidence for a female predilection of the disease. Implications for pathogenesis, and a suggested clinical approach are discussed. An online disease registry has been established to facilitate further IGH research.

Idiopathic Granulomatous Hypophysitis Presenting as Pituitary Apoplexy: A Rare Manifestation of a Rare Disorder

Idiopathic Granulomatous Hypophysitis Presenting as Pituitary Apoplexy: A Rare Manifestation of a Rare Disorder

Idiopathic Granulomatous Hypophysitis Presenting as Pituitary Apoplexy

Idiopathic Granulomatous Hypophysitis Presenting as Pituitary Apoplexy

Idiopathic Granulomatous Hypophysitis Presenting as Panhypopituitarism

Aim: The aim is to describe a very rare case of idiopathic granulomatous hypophysitis presenting as panhypopituitarism on the background of chronic renal failure and type 2 diabetes mellitus. The patient presented with fatigue and episodes of self-terminated hypoglycemia (while on sulfonylurea) as the sole manifestation of panhypopituitarism. The case also highlights the difficulties of identifying patients with hypophysitis in the setting of chronic renal failure. Results: We describe a middle-aged man who presented to us with the sole complaints of early morning fatigue and hypoglycemia (while on glimepiride), with a background history of type 2 diabetes and chronic renal failure. Before visiting our practice, he was repeatedly evaluated for decompensation of renal and cardiovascular disease without much success. When we saw the patient there was no obvious evidence of any endocrine disturbance, especially as the blood pressure was high without any postural drop. It was only when the patient persistently complained of an impaired quality of life due to the fatigue that we decided to chase an endocrine cause for the same. On endocrine evaluation, we diagnosed panhypopituitarism secondary to idiopathic granulomatous hypophysitis. Conclusion: Idiopathic granulomatous hypophysitis is a very rare form of inflammatory pituitary disease. Fewer than 35 cases of biopsy-proven idiopathic granulomatous hypophysitis have been reported in medical literature. This is only the third case of biopsy-proven idiopathic granulomatous hypophysitis reported from India.

Acute Onset of Polyarthralgia and High Anti-Cyclic Citrullinated Peptide Antibodies in a Case of Idiopathic Granulomatous Hypophysitis

Acute Onset of Polyarthralgia and High Anti-Cyclic Citrullinated Peptide Antibodies in a Case of Idiopathic Granulomatous Hypophysitis

Pituitary tuberculosis mimicking idiopathic granulomatous hypophysitis

An unusual case of tubercular granulomatous hypophysitis is reported. A sellar mass diagnosed as pituitary adenoma in MRI, showed non-caseating granulomas, glandular destruction and fibrosis in histology. Stain for acid-fast bacilli (AFB) were negative and the case was misdiagnosed as Idiopathic Granulomatous hypophysitis. The patient came back 2 weeks after trans-sphenoidal endoscopic resection with meningitis. CSF showed mixed pleocytosis. PCR for Mycobacterium tuberculosis was positive. The case is reported to highlight the need to maintain a high degree of suspicion for tuberculosis in any form of granulomatous hypophysitis, with or without caseous necrosis, in regions endemic for tuberculosis to prevent subsequent complications.

Idiopathic granulomatous hypophysitis presenting as non-functioning pituitary adenoma: description of six cases and review of literature

The aim of this study was to analyse the complete profile and outcome of patients with idiopathic giant cell granulomatous hypophysitis. Six consecutive cases of idiopathic giant cell granulomatous hypophysitis were studied from 1993 to 2002. Headache and visual disturbances were the most frequent presenting symptoms. All patients had hypogonadism, four had hypoadrenalism and three were hypothyroid at presentation. None of them had diabetes insipidus preoperatively. A sellar mass with suprasellar extension on MRI with loss of the posterior pituitary 'bright spot' was a consistent observation in all patients. All patients underwent surgical excision of the mass lesion with histopathological confirmation of giant cell granulomatous hypophysitis. Other systemic granulomatous diseases were excluded by appropriate investigations. Postoperatively, all patients became hypothyroid and hypogonad, five patients had adrenal insufficiency, while two developed permanent diabetes insipidus. The clinical presentation of giant cell granulomatous hypophysitis is that of an expanding sellar mass lesion with a varying degree of endocrine dysfunction. Preoperative diagnosis of 'hypophysitis' is usually difficult; however, stalk thickening and loss of posterior pituitary 'bright spot' on MR imaging are clues to the diagnosis.

Space occupying processes of the sellar region with emphasis on tumor-like lesions

Most space occupying lesions of the sellar region are tumors (e.g., pituitary adenomas, craniopharyngiomas, but also meningiomas). In approximately 8% of patients with sellar mass lesions, non-neoplastic disorders (e.g., inflammations, cysts, hyperplasia) are causal. Cystic lesions of the pituitary region are classified as Rathke's cysts, colloid cysts, arachnoidal cysts and epidermoid cysts. These have to be differentiated from cystic craniopharyngeomas or cystic pituitary adenomas. Autochthonous inflammations (lymphocyctic hypophysitis, idiopathic granulomatous hypophysitis, xanthomatous and peritumorous hypophysitis) have to be distinguished from generalized diseases involving the pituitary (e.g., sarcoidosis, tuberculosis). Hyperplasia can result in a doubling of pituitary size. Lactotroph and corticotroph hyperplasia are the most common and may be of a diffuse and nodular type. The nodular form of pituitary hyperplasia may show transition into adenoma. Other hyperplasias may be found adjacent to adenomas or other tumours. Furthermore, vascular (aneurysms) and bony lesions (especially fibrous dysplasia) can appear as space occupying lesions of the sellar region.

Hipofisitis granulomatosa idiopática. Estudio morfológico e inmunohistoquímico de una observación

Inflammatory diseases of the pituitary gland constitute a group of interest because of their scarce frequency, because the disorder presents with symptoms of hypopituitarism and expanding sellar mass and because of their therapeutics implications. We present one case of idiopathic granulomatous hypophysitis, in a 55-years-old patient with daily headaches, panhypopituitarism and a sellar mass lesion. Granulomatous hypophysitis is characterized by granulomas with epithelioid histiocytes and multinucleated giant cells but also shows lymphocyte collections. With respect to immunohistochemistry our results show histiocytes (CD68+) and an heterogeneous inflammatory infiltrate (CD45RO+ y CD20+). We analyze the differential diagnosis with another granulomatous processes, infectious or not infectious, and with the histiocytosis. We examine the possible relation with the lymphocytic hypophysitis.

Idiopathic Giant-Cell Granulomatous Hypophysitis Mimicking Acute Meningitis

A 32-year-old woman presented with severe headache, photophobia, fever, nausea, vomiting, and worsening vision. She had also noted several months of amenorrhea. She was febrile to 38.9 degrees C. Laboratory evaluation revealed a markedly elevated erythrocyte sedimentation rate. Lumbar puncture revealed a cerebrospinal fluid lymphocytic pleocytosis and an elevated protein level. Endocrine studies revealed evidence of panhypopituitarism without diabetes insipidus. A magnetic resonance imaging study showed a 2-cm pituitary mass with optic chiasmal compression. The patient had a trans-sphenoidal resection of the mass. Pathology revealed multinucleated giant cells in necrotic debris, but no evidence of pituitary tumor. Studies looking for evidence of systemic granulomatous disease were negative. The patient was considered to have idiopathic giant-cell granulomatous hypophysitis. After surgery, the patient's vision improved and hormone replacement therapy was initiated. This case illustrates that idiopathic giant-cell granulomatous hypophysitis should be considered in the differential diagnosis of a patient presenting with a pituitary mass, hypopituitarism, and meningitis-like symptoms.

Idiopathic granulomatous hypophysitis

A case is described of an elderly woman who presented with headaches, hypopituitarism, and visual disturbances and was found to have idiopathic granulomatous hypophysitis, a rare lesion of the pituitary gland. Preoperative magnetic resonance imaging demonstrated a well-circumscribed lesion, which was isointense on both T1- and T2-weighted imaging and enhanced uniformly with administration of gadolinium contrast enhancement, not unlike a macroadenoma. The present case and 12 other cases from the literature are reviewed.

Magnetic resonance imaging in the diagnosis of idiopathic giant-cell granulomatous hypophysitis: a rare cause of hyperprolactinaemia

Idiopathic giant cell granulomatous hypophysitis is a rare disorder of pituitary gland characterised by a chronic inflammatory process. It can also be an extremely rare cause of hyperprolactinaemia. In this paper, we present our experience with two cases of idiopathic giant cell granulomatous hypophysitis manifested by hyperprolactinaemia, and their neuroradiological evaluation including preoperative MRI studies in one of them, and discuss our findings in the light of the literature.