Infundibular Thickening Research Articles

7480 Isolated IgG4-Related Infundibulo-Hypophysitis

Abstract Disclosure: M. Allen: None. R.T. Beck: None. N.T. Zwagerman: None. R.J. Ryzka: None. D. Coss: None. A. Fisco: None. A.G. Ioachimescu: None. Background: 130 cases of IgG4-related hypophysitis (IgG4-RH) have been reported, usually in context of multi-organ IgG4-disease (e.g., siladenitis, pancreatitis, retroperitoneal fibrosis). Among patients with IgG4-disease, up to 5% develop hypophysitis. Presentation entails mass effects, AVP-D and anterior hypopituitarism.Case report: A 72-year-old man with history of hyperlipidemia presented with gradual decrease in strength, energy, appetite, libido, and depressed moods. Laboratory tests (8 AM) indicated undetectable AM cortisol, ACTH 6.8 pg/mL (7.2-63.3), TSH 0.20 mIU/mL (0.35-4.94), free thyroxine 0.64 ng/dL (0.7—1.48), prolactin 50.9 ng/mL (3.46-19.4), IGF-1 57 ng/mL (53-222), testosterone 7 ng/dL (240-950), LH 0.16 mIU/mL (0.57-12.07), and FSH 1.5 mIU/mL (1.0-12). MRI revealed a homogenously enhancing nodular suprasellar mass adjacent to the hypothalamus (0.8x0.7x0.8 cm) and contiguous with a 0.5 cm area of infundibular thickening. Neuro-ophthalmological evaluation was normal. In absence of mass effects, patient was monitored conservatively and treated with hydrocortisone, levothyroxine and testosterone replacement. Six months later, the mass enlarged to 1.2x0.8x1.1 cm. Patient underwent a transsphenoidal biopsy which revealed a dense fibrous mass with lymphoplasmacytic infiltration. Most cells stained for CD138 and IgG4. Postoperatively, serum IgG and complement levels, inflammatory markers, hemogram, serum protein electrophoresis, amylase and lipase were normal. CT chest and abdomen and thyroid ultrasound were unremarkable. After 4 weeks of prednisone 40 mg daily, the nodular lesion significantly decreased on MRI (0.6x0.5x0.4 cm). Prednisone was gradually tapered to 5 mg daily over 3 months. After 2 months of physiologic prednisone therapy, imaging remained stable. During follow-up of 1.5 years, no systemic IgG-4 disease was apparent and patient remained on glucocorticoid, thyroid and testosterone replacement.Discussion: Suprasellar masses can be inflammatory, infiltrative, infectious or neoplastic. The possibility of IgG4-RH should be considered even in absence of IgG4 systemic disease, as it happened in our case. Primary involvement of the infundibulo-hypophysis was suggested by a retrospective review study that found isolated IgG4-RH in 41% specimens initially diagnosed as primary autoimmune hypophysitis. Our case maintained a good radiological response after prednisone taper to physiologic doses. Response to prednisone is generally good in IgG4-RH, but relapse can occur upon taper, and long-term studies are lacking. Conclusion: We present a histologically-confirmed case of isolated IgG4-RH presenting with clinical manifestations of anterior hypopituitarism. Diagnosis and management of IgG4-RH requires multidisciplinary collaboration (endocrinology, rheumatology, neurosurgery, neuropathology and neuroradiology). Presentation: 6/3/2024

Preoperative differentiation of hypophysitis and pituitary adenomas using a novel clinicoradiologic scoring system

Hypophysitis can clinically and radiologically mimic other nonfunctioning masses of the sella turcica, complicating preoperative diagnosis. While sellar masses may be treated surgically, hypophysitis is often treated medically, so differentiating between them facilitates optimal management. The objective of our study was to develop a scoring system for the preoperative diagnosis of hypophysitis. A thorough literature review identified published hypophysitis cases, which were compared to a retrospective group of non-functioning pituitary adenomas (NFA) from our institution. A preoperative hypophysitis scoring system was developed and internally validated. Fifty-six pathologically confirmed hypophysitis cases were identified in the literature. After excluding individual cases with missing values, 18 hypophysitis cases were compared to an age- and sex-matched control group of 56 NFAs. Diabetes insipidus (DI) (p < 0.001), infundibular thickening (p < 0.001), absence of cavernous sinus invasion (CSI) (p < 0.001), relation to pregnancy (p = 0.002), and absence of visual symptoms (p = 0.007) were significantly associated with hypophysitis. Stepwise logistic regression identified DI and infundibular thickening as positive predictors of hypophysitis. CSI and visual symptoms were negative predictors. A 6-point hypophysitis-risk scoring system was derived: + 2 for DI, + 2 for absence of CSI, + 1 for infundibular thickening, + 1 for absence of visual symptoms. Scores ≥ 3 supported a diagnosis of hypophysitis (AUC 0.96, sensitivity 100%, specificity 75%). The scoring system identified 100% of hypophysitis cases at our institution with an estimated 24.7% false-positive rate. The proposed scoring system may aid preoperative diagnosis of hypophysitis, preventing unnecessary surgery in these patients.

Clinical Characteristics of Primary Hypophysitis - A Single-Centre Series of 60 Cases.

Clinical data on primary hypophysitis are still scarce. Especially non-surgical cases are underreported. We sought to analyse clinical characteristics of primary hypophysitis, particularly in clinically diagnosed patients. Retrospective single centre study in 60 patients with primary hypophysitis. Symptoms, MRI, histopathological findings, treatment and outcomes were analysed in 12 histopathologically and 48 clinically diagnosed patients. Diagnostic criteria for clinical diagnosis were: a) MRI findings compatible with primary hypophysitis; b) course of disease excluding other differential diagnoses. Mean duration of follow-up was 69 months. Female sex was predominant (73%). Fatigue (52%), headache (38%) and diabetes insipidus (38%) were the most frequent symptoms. 42% had a concomitant autoimmune disease. The corticotropic, thyrotropic, gonadotropic, somatotropic axis was impaired in 67%, 57%, 52%, 20%, respectively. Men had a higher number of impaired hormone axes (p=0.022) with the gonadotropic axis being affected more frequently in men (p=0.001). Infundibular thickening (56%) and space occupying lesions (46%) were typical MRI findings. Pituitary size was frequently enlarged at presentation (37%) but diminished during observation (p=0.029). Histopathologically and clinically diagnosed cases did not differ. The cohort of clinically diagnosed patients did not differ from our histopathologically diagnosed patients or from published cohorts with predominantly surgical patients. Thus, diagnosis of primary hypophysitis using clinical criteria seems feasible.

MON-246 A Diagnostic Dilemma of Primary Versus Secondary Thyroid and Adrenal Hormone Insufficiencies: A Unique Case of Widely Metastatic Renal Cell Carcinoma After Brain Radiation

Background Identifying the etiology of hormonal insufficiency is important for correct diagnosis and for appropriate hormone supplementation. Usually multiple hormone deficiencies are driven by pituitary pathology, but here we present a case that poses a unique diagnostic and therapeutic dilemma. Clinical Case A 55 y/o lady with HTN and recently diagnosed metastatic Renal Cell Carcinoma (RCC) presented with weakness, dizziness, altered mental status, polydipsia and polyuria. Her metastases included the bilateral adrenal glands, thyroid, brain, and she had a sellar lesion displacing the pituitary as well as infundibular thickening. She had been treated with dexamethasone due to vasogenic edema, whole brain radiation, and was going to start immunotherapy (Ipilumimab&Nivolumimab). On exam she had relative hypotension and tachycardia (BP 115/58, HR 108). She was diagnosed with non-PTH mediated hypercalcemia (Ca 13.9/ 15.3 corrected for albumin 2.3, PTH 6.5, PTH-RP 69, Vitamin D-25 19.4). Her calcium normalized after fluid resuscitation and bisphosphonate treatment, but weakness and hypotension persisted. We tested thyroid and adrenal function given the location of her lesions, recent whole brain radiation, and recent steroid use. Her TSH was 0.017 (0.270 - 4.200 µIU/mL), fT4 0.84 (0.80 - 1.50 ng/dL), T3 0.59 (72.0 - 153.0 ng/dL), ACTH was 1.7 (7.2-63.3 pg/ml), cortisol 1.3 (6.0 - 18.4 ug/dL), aldosterone 3 (< or = 28 ng/dL), renin 7.87 (0.25 - 5.82 ng/mL/h), renin/aldosterone 0.4 (0.9 - 28.9). At 30 minutes after cosyntropin administration cortisol was 7.7 ug/dL, aldosterone 26 ng/dL, and at 60 minutes cortisol was 10.3 ug/dL. Hydrocortisone was initiated while tapering off dexamethasone, and levothyroxine was offered but declined. She opted to postpone further workup. Conclusion This patient presents a dilemma in identifying the primary causes of hormonal abnormalities given potential pituitary and primary thyroid and adrenal disease. The thyroid abnormalities could represent sick euthyroid physiology with lower T4 to T3 conversion or the effects of steroids which can also suppress TSH vs. hypothalamic or pituitary disease. Primary hypothyroidism from gland destruction was unlikely. The low ACTH and cortisol levels were expected since she was on dexamethasone, but low aldosterone with raised renin activity was concerning for primary adrenal insufficiency. However, after cosyntropin the suboptimal cortisol yet preserved aldosterone response supported secondary or tertiary adrenal insufficiency, and only cortisol was supplemented. Understanding her endocrine disease had implications for longer term management given that replacement may only need to be temporary and adrenal recovery might be possible. Further, the use of a 30 minute aldosterone level can be helpful in cases where multiple factors and discrepant laboratory findings may exist.

MON-263 Renal Cell Cancer Metastasis to the Pituitary Masquerading as a Non-Functioning Adenoma

A 47 yr. old male with history of RCC and hypertension was seen in pituitary clinic for management of a pituitary mass. He initially presented to an outside hospital with persistent headaches, unintentional weight loss, severe fatigue, dizziness, cold intolerance, low libido and difficulty with erections. CT head showed a pituitary mass. Lab work revealed pan-hypopituitarism with adrenal insufficiency [random cortisol 0.7 ug/dL (4- 19 ug/dL); ACTH 13 pg/mL (7-63 pg/mL)], hypothyroidism [T4 0.52 ng/dL (0.7-1.5 ng/dL); TSH 0.1 mIU/L (0.45-4.5 mIU/L)] and hypogonadism [total testosterone 15 ng/dL (200-1000 ng/dL); LH of 0.6 mIU/mL (1.5-9.3 mIU/mL); FSH of 3.5 mIU/mL (1.6- 8.0 mIU/mL)]. Prolactin was slightly elevated at 34 ng/mL (2- 18 ng/mL) from stalk compression. IGF-1 was 78 ng/mL (Z score -1.2).When we saw him, he was already on hormone replacement with significant improvement in symptoms. He had no visual field defects or symptoms of diabetes insipidus (DI). As for his history of RCC, he underwent a partial left nephrectomy in 2015, followed by a completion nephrectomy shortly after. Pathology was consistent with clear cell RCC. He did not receive any adjuvant therapy; Follow up imaging studies were reportedly negative for recurrence. Pituitary MRI showed a 1.6 X 1.4 X 1.5 cm enhancing sellar and suprasellar mass without compression of optic chiasm. He was thought to have a presumed non-functioning adenoma, and chose to proceed with trans-spenoidal surgery (TSS). Intra operatively, a very vascular tumor was encountered, which could only be debulked safely. Pathology was consistent with metastatic clear cell RCC. A follow up CT showed innumerable lung nodules and a 3.2 cm right adrenal nodule consistent with metastases. He completed stereotactic radiation to the sella and is currently on Ipilimumab and Nivolumab for immunotherapy.MP is uncommon, and seen in 1-4% of patients with cancer in autopsy studies. Breast (40%) and Lung cancer (25%) are the most common tumors causing MP in women and men respectively, with very few case reports of RCC MP (2.6%). DI is the most common symptom of MP, which is thought to be due to direct vascular supply from hypophyseal arteries to the posterior pituitary. Interestingly in RCC MP, anterior pituitary dysfunction is more prevalent (90%) compared to DI (24%). There is also a tendency for suprasellar extension, causing vision loss. Radiologic features that could help distinguish MP from an adenoma include a rapidly enhancing sellar mass, erosion of sellar floor or posterior clinoid process, infundibular thickening, and the presence of intra-tumoral vascular flow voids. There reportedly is no benefit to using FDG- PET to diagnose MP from RCC. There are no standardized treatment guidelines and the choice of treatment is also influenced by the extent of systemic disease.

Clinical Interrogation for Unveiling an Isolated Hypophysitis Mimicking Pituitary Adenoma

Hypophysitis is a rare disease entity mimicking a pituitary adenoma. Despite crucial disease involving a pituitary gland which is a main component of the hormonal axis, there have been minimal researches regarding the hypophysitis. In this study, we described the constellation of the preoperative findings including clinical, radiologic, and endocrinologic features, as well as postoperative outcomes of the hypophysitis. The authors reviewed the data retrospectively of a total of 2814 patients who underwent microsurgery at our institution to treat a pituitary mass lesion. Among them, the patients proven as hypophysitis in histologic examination were enrolled, followed by stratification into tumor-associated hypophysitis and isolated hypophysitis depending on the existence of associated tumor lesion. As a result, a total 21 cases were enrolled consisting of the 13 cases of tumor-associated and the 8 cases of isolated hypophysitis. In this study, the isolated hypophysitis was focused on the analysis. All patients with isolated hypophysitis were evaluated the clinical, radiologic, and endocrinologic tests preoperatively. After microsurgery, all patients had a minimum follow up of 6 months (6∼72 months). The clinical symptoms, endocrinologic tests, and brain MRI were checked at every 6 months during first 2 year, since then, at every 12 months. Among 2814 cases of pituitary mass lesion underwent microsurgery, the hypophysitis were found 21 cases (0.74 %). The 13 cases were the tumor-associated hypophysitis, consisting of rathke's cleft cyst (7 cases), craniopharyngioma (3 cases), pituitary adenoma (2 cases), and germinoma (1 case). The 8 cases were the isolated hypophysitis including lymphocytic (4 cases), granulomatous (3 cases), and IgG4-related (1 case) hypophysitis. The patients presented a wide range of clinical symptoms from general weakness to panhypopituitarism. In addition, the most remarkable radiologic features of the isolated hypophysitis were infundibular thickening and the low signal intensity in T1WI and T2WI. All 8 patients with isolated hypophysitis presented endocrinologic deficiency with various extent in a preoperative biochemistry. After surgery, the hormonal status were not changed in 4 patients (50%), however, aggravated in 4 patients (50%). The 3 patients (37.5%) complained visual field defects in preoperative ophthalmologic test, but improved all after surgery. In this study, the preoperative clinical manifestations, radiologic and endocrinologic features of hypophysitis were described comprehensively. In addition, we also revealed the long-term outcomes of hypophysitis in the aspect of visual function and endocrinologic outcomes. Preexisting visual field defects were improved in all patients (3 cases). However, all patients presented endocrinologic deficiency in preoperative biochemistry, among them, the 4 patients (50 %) were unchanged and the 4 patients (50 %) were exacerbated after surgery. In conclusion, the visual outcomes were satisfactory, however, the endocrinologic outcomes were vice versa, because the preoperative endocrinologic deterioration was unchanged or even aggravated after surgery in some cases.

More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders.

Although Langerhans cell histiocytosis (LCH) is a familiar entity to most radiologists and to pediatric radiologists in particular, it is but one of a group of disorders caused by the overproduction of histiocytes, a subtype of white blood cells. Other less familiar diseases in this category are Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG), Rosai-Dorfman disease (RDD), and hemophagocytic lymphohistiocytosis (HLH). This review describes the classification system, clinical manifestations, and pathophysiology of each disease, with particular attention to differential radiographic findings, including typical locations of involvement and varying appearances at radiography, computed tomography, magnetic resonance imaging, ultrasonography, and nuclear medicine imaging. Although LCH has a wide variety of manifestations and appearances, classic imaging findings include vertebra plana, skull lesions with a beveled edge, the "floating tooth" sign, bizarre lung cysts, and an absent posterior pituitary bright spot with infundibular thickening. The classic imaging findings of ECD are a perirenal rind of soft tissue and patchy long bone osteosclerosis. RDD has more nonspecific imaging findings, including lymphadenopathy (most commonly cervical) and intracranial lesions. Imaging findings in HLH are broad, with the most common abnormalities being hepatosplenomegaly, cerebral volume loss, and periventricular white matter abnormalities. JXG can manifest at imaging, but radiology does not play a major role in diagnosis. Familiarity with these disorders and their associated imaging findings facilitates correct and timely diagnosis. Imaging also features prominently in the assessment of treatment response.

A case of neurosarcoidosis with labyrinthine involvement.

Sarcoidosis is a chronic granulomatous disease of unknown aetiology, which may involve any organ system. It most commonly occurs in adults with childhood involvement being rare. Central nervous system involvement is seen in up to 25% and typically involves meningeal disease resulting in multiple cranial neuropathies. Other common clinical findings include seizures, headache, dementia, and pituitary dysfunction. Imaging plays a central role in the diagnosis with typical findings including pachymeningeal and leptomeningeal enhancing lesions. Other imaging findings include lacunar and major territory infarcts, hypothalamic and infundibular thickening, hydrocephalus, and cranial nerve enhancement. We present a case of an eight-year-old male patient with progressive headache, visual disturbance, unilateral sensory hearing loss, and multiple cranial neuropathies. Imaging findings demonstrated the classic pachymeningeal and leptomeningeal enhancement along much of the skull base, as well as enhancement of the right and left second and eighth cranial nerves. Extensive inflammatory changes were noted in the temporal bones and paranasal sinuses. There was also enhancement of the right and left labyrinths. Sinus biopsy confirmed sarcoidosis. We present the first case to our knowledge of sarcoid labyrinthitis.

Aspects IRM de la tuberculose de la région sellaire

Purpose Tuberculosis of the sellar region, especially the pituitary gland, is rare. The purpose of this article is to demonstrate through a review of five clinical cases the value of imaging, especially MR imaging, in the evaluation of this pathology. Patients and methods CT and MRI of the brain were obtained in all cases along with a chest radiograph. Results Four patterns were detected on MRI: pituitary tuberculoma mimicking adenoma; pituitary abscess, extending to the cavernous sinus in one case and associated with infundibulum thickening in another; hypophysitis with suprasellar extension in association with tuberculous meningoencephalitis; and infundibular thickening associated with tuberculous meningoencephalitis. Diagnosis was based on biopsy in two cases and combination of imaging and clinical data in three cases. Outcome was favorable with anti-tuberculosis drugs. Conclusion Irrespective of the imaging features, a history of travel to an endemic region combined to other findings such as infundibular thickening should raise concern for the possibility of tuberculosis even in the absence of signs of systemic infection.

Magnetic resonance findings in sellar and suprasellar tuberculoma with hemorrhage.

Background:Tuberculosis is endemic in many counteries like India. It can infect any site in the central nervous system. However, islolated involvement of the sellar and suprasellar region is rare. Sellar tuberculoma with hemorrhage is even more rare. We present magnetic resonance (MR) findings in case of sellar and suprasellar tuberculoma with hemorrhage.Case Description:A 40-year-old female patient presented with a 1-month history of persistent headache and blurred vision on the left side. A contrast-enhanced MR study revealed peripherally enhancing sellar and suprasellar mass with hemorrhage with compression of the left half of the optic chiasma. There was also evidence of infundibular thickening and enhancement of the adjacent dura. The mass was approached through a transphenoidal approach and was partially resected. Subsequent histopathology was suggestive of tuberculosis. The patient was put on anti-tubercular therapy. Patient reported significant improvement in symptoms. Follow-up MR done 8 months later confirmed complete regression of the mass.Conclusion:Because of its rarity, sellar tuberculoma is seldom considered in the differential diagnosis and is often mistaken for pituitary macroadenoma, which is the most common tumor in this region. Although rare, presence of infundibular thickening and enhancement of the adjacent dura should suggest the presence of a granulomatous lesion like tuberculoma.

RADIOLOGICAL EVALUATION OF PATIENTS WITH PITUITARY LANGERHANS CELL HISTIOCYTOSIS AT DIAGNOSIS AND AT FOLLOW-UP

This study evaluated pituitary imaging findings in 13 patients with Langerhans cell histiocytosis (LCH) with diabetes insipidus. Nine patients were evaluated with pituitary magnetic resonance imaging (MRI), 3 with brain computed tomography, and 1 with brain MRI. The infundibulum was thickened in 11 (84.6%) patients, thread-like in 1 (7.7%), and normal in 1 (7.7%). Posterior pituitary intensity was absent in 10 patients (76.9%); in 4 patients, the pituitary gland was small in size, and 2 patients had atrophic pituitary. Three had a small sella. Infundibular thickening and absence of posterior pituitary intensity were the most common radiological findings. MRI imaging should be used to follow up patients with pituitary histiocytosis, and patients with LCH and diabetes insipidus should be followed for pituitary atrophy.

Atteinte hypothalamo-hypophysaire dans l’histiocytose de l’adulte

Langerhans cell histiocytosis is a rare entity. Involvement of the pituitary region is frequently delayed and rarely studied. We report some clinical and paraclinical particularities of the disease in four women. Diabetes insipidus was the initial symptom in all cases, accompanied by panhypopituitarism at initial presentation in one woman. Hypopituitarism was diagnosed from 6 months to 4 years after the diabetes insipidus in the other cases, and systemic lesions appeared 3 to 6 years thereafter. Magnetic resonance imaging of the hypothalamic-pituitary axis found tumors in two patients and infundibular thickening in the other two. One patient also had a partially empty sella. In adults, pituitary involvement seems to occur predominantly in women, and panhypopituitarism is possible at initial presentation. The clinical polymorphism of Langerhans cell histiocytosis makes this disease difficult and often slow to diagnose.

MRI and CT findings of neurohypophyseal germinoma

Objective: Magnetic resonance (MR) imaging and computed tomography (CT) findings of neurohypophyseal germinoma have not previously been described in detail. The purpose of the present study was to establish the spectrum of MR imaging and CT findings in neurohypophyseal germinomas. Materials and methods: MR and CT images of 13 consecutive patients (seven males, six females; mean age: 15 years; range: 6–31 years) with neurohypophyseal germinoma were retrospectively analyzed. The diagnosis had been made either histologically (n=8) or clinically according to established criteria (n=5). All patients had been examined using MR imaging and CT before treatment. Results: On MR imaging, infundibular thickening (up to 16 mm) was observed in all 13 cases. Hyperintensity of the posterior pituitary on T1-weighted image was absent in all 13 cases (100%) and 12 of the 13 displayed central diabetes insipidus. Ten germinomas (77%) were isointense to cerebral cortex on T1-weighted image, but variable intensities were exhibited on T2-weighted image. MR images revealed intratumoral cysts in six cases (46%), most of which demonstrated intra-third ventricular extension. Eleven of the 13 cases (85%) revealed hyperdense solid components on unenhanced CT. Calcification was absent in all cases (100%). Conclusion: Infundibular thickening, absence of the posterior pituitary high signal on T1-weighted image, lack of calcification and hyperdensity on unenhanced CT are common imaging features of neurohypophyseal germinoma.

Wegener granulomatosis: MR imaging findings in brain and meninges.

To determine the spectrum of intracranial magnetic resonance (MR) imaging appearances of Wegener granulomatosis. MR imaging studies in 19 patients with Wegener granulomatosis and possible central nervous system involvement were reviewed by two neuroradiologists. Intermediate-weighted and T2-weighted fast spin-echo MR images of the brain had been acquired in all patients, and spin-echo T1-weighted nonenhanced and gadolinium-enhanced images had been acquired in 18 patients. MR imaging findings included diffuse linear dural thickening and enhancement (n = 6); focal dural thickening and enhancement contiguous with orbital, nasal, or paranasal disease (n = 5); infarcts (n = 4); nonspecific white matter areas of high signal intensity on intermediate-weighted and T2-weighted images (n = 10); enlarged pituitary gland with infundibular thickening and enhancement (n = 2); a discrete cerebellar lesion that was probably granulomatous in origin (n = 1); and cerebral (n = 8) and cerebellar atrophy (n = 2). MR imaging demonstrated the wide spectrum of findings of central nervous system involvement in patients with Wegener granulomatosis and was particularly useful for the evaluation of direct intracranial spread from orbital, nasal, or paranasal disease.