Diagnosis Of Lymphocytic Hypophysitis Research Articles

6530 A Case of Lymphocytic Hypophysitis Followed by Acromegaly

Abstract Disclosure: L. Medina Mora: None. S. Htoo: None. A. Sanchez Ruiz: None. M. Harshan: None. M. Liu: None. Introduction: Acromegaly, a disorder with an estimated incidence of 0.28-0.4 cases per 100,000 individuals, is characterized by overproduction of growth hormone. Features include acral enlargement, frontal bossing, macrognathia, headaches, and hyperhidrosis. Comorbidities include hypertension, sleep apnea, type 2 diabetes, and cardiomyopathies. Hypophysitis, with an incidence of 1 in 9 million individuals, is an even rarer condition that involves pituitary gland inflammation. It can often lead to hormonal dysfunction and compression of surrounding structures. It is extremely rare for both acromegaly and hypophysitis to occur in the same individual. In this case report, we present a patient who developed lymphocytic hypophysitis followed by acromegaly. Case Report: A 58-year-old woman with a history of hypertension and lymphocytic hypophysitis (discovered during a workup of headaches without hormone derangement) returned to the clinic two years after complaining of progressive enlargement of her hands and feet, fragmented sleep, insomnia, and coarsening of facial features. She was found to have elevated IGF-1 (886 ng/mL; reference range: 48-235 ng/mL) and growth hormone levels (86.9 ng/mL; reference range: 0.12-9.88 ng/mL). An MRI showed a 2.9 x 3.2 x 3.8 cm suprasellar/sellar mass causing pressure on the optic chiasm, hypothalamus, third ventricle and midbrain. The patient underwent transsphenoidal resection (TSR), which was incomplete because the tumor was located behind the pituitary gland, followed by a right craniotomy to remove the remaining tumor. The tumor specimen stained positive for GH and PIT 1, exhibiting elevated proliferative indices (>2 mitotic figures per 10HPF, Ki-67 20%) and p53 labeling (5%). After the surgery, the patient's IGF-1 and GH levels normalized. However, her postoperative course was complicated by third cranial nerve palsy, diabetes insipidus, hypothyroidism, and secondary adrenal insufficiency. Discussion: Although TSR is the first-line treatment for most patients with acromegaly, it may not always result in a complete cure, as almost 50% of patients with macroadenomas do not achieve full surgical recovery. For persistent disease, medical therapy or transcranial surgery may be required. The tumor in this case was atypically proliferative, which was likely why GH and IGF-1 levels rose rapidly, manifesting in acromegalic symptoms in our patient two years after diagnosis of lymphocytic hypophysitis. The natural history of hypophysitis typically progresses from inflammation to fibrosis and subsequently to atrophy of the pituitary cells. This atrophy may cause changes in regulatory hormones that promote tumorigenesis. Therefore, hypophysitis may stimulate adenoma formation leading to acromegaly. More research is needed to characterize the relationship between these two rare entities. Presentation: 6/2/2024

A detailed pituitary profile always matters

Abstract 29-year-old female with known hypothyroidism and positive thyroid auto antibodies, followed up throughout pregnancy for adjusted doses of thyroid replacement therapy, presented 2 months post-partum with gradually increasing fatigue, repeated pre-syncope experience, inability to cope with daily home and family needs and headache. Adequacy of thyroid replacement therapy was questioned, TSH has been performed: 0.18 mIU/ml. Checking the blood pressure in lying and standing position has showed Postural hypotension. An urgent measurement of Cortisol (am) showed a result of1.06 ug/dl. Preliminary Differential Diagnoses have been suggested; Pituitary apoplexy, Pituitary adenoma, Autoimmune adrenalitis (Autoimmune Polyglandular Syndrome) or Others. On further detailed history, the patient affirmed dizziness and fainting liability on standing from lying position, bitemporal headache more in the morning. However, she denied frequent urination throughout the day, neither repeated times at night. In addition, she confirmed efficient breast feeding. Continued Lab investigations: ACTH (am) (Low); FT4 (Low; on replacement therapy); IGF1 (Normal) Serum osmolality (Normal); Urine osmolality (Normal); E2 / LH / FSH (normal physiological levels) Prolactin (normal for breast feeding) MRI pituitary and sella: Diffuse isointense enlargement of anterior pituitary gland. Homogenous and strong diffuse pituitary enhancement with gadolinium contrast. History and investigations were not fully matching with any of the previously proposed differential diagnoses, especially in regards of selective corticotroph and thyrotroph affection, sparing other pituitary cell types. Moreover, the autoimmune predisposition, the nature of the clinical presentation and its timing during early postpartum period, together with the laboratory and imaging investigations were all matching with the diagnosis of Lymphocytic Hypophysitis. The Patient got pregnant again 14 months post-partum, reflecting spared gonadotroph function and highlighting the importance to identify this uncommon diagnosis and to notify the patient about normal gonadal function and the normal possibility of pregnancy. However, she remained on hydrocortisone and thyroxine replacement. Finally, it is important that this condition should not be misdiagnosed in the first place as postpartum thyroid disturbance and TSH results, especially during and after pregnancy, shall always be interpreted in the clinical context and with the overall thyroid profile.

A Case of Pituitary Germinoma Misdiagnosed as Lymphocytic Hypophysitis

Pituitary germinomas are difficult to be differentiated from lymphocytic hypophysitis due to similar clinical, radiological and even histological findings. However, it is of great importance to make the distinction between them because of different therapeutic approaches of the two diseases. We report a case of neurohypophysial germinoma who initially was misdiagnosed as lymphocytic hypophysitis. The patient was a 26-year-old man who first presented with central diabetes insipidus and subsequent pan-hypopituitarism. Magnetic resonance imaging (MRI) of the pituitary which first had shown mild pituitary enlargement, revealed a pituitary mass of unusual feature after 1 year. Pathological diagnosis of the lesion, biopsied through trans-sphenoidal route, was lymphocytic hypophysitis. Corticosteroid was initiated for him. Despite, intensive treatment clinical symptoms worsened and the patient developed visual field defect. Follow-up MRI revealed enlargement of the pituitary mass with suprasellar extension and involvement of optic chiasm. Surgical resection of the mass was performed, and the second histological examination confirmed the diagnosis of germinoma. Subsequently, the patient underwent radiotherapy, and complete remission was achieved; however, pan-hypopituitarism persisted. In conclusion, the possibility of neurohypophyseal germinoma should be considered in patients with the diagnosis of lymphocytic hypophysitis who do not respond to corticosteroid therapy. J Endocrinol Metab. 2018;8(5):113-118 doi: https://doi.org/10.14740/jem530w

Corticosteroids in the management of lymphocytic hypophysitis: Case series.

The diagnosis of lymphocytic hypophysitis (LYH) is a clinical challenge. Medical management with steroids may result in complete resolution of the symptoms and radiological features. We report our approach to the diagnosis and treatment of LYH. Retrospective analysis of data of nine consecutive patients of LYH from August 2013 to August 2015 was done. The average age of the study population was 38.4 ± 19.8 years, with five (55.5%) women. Presumptive diagnosis of LYH was made in six patients who were treated with steroids. Among the medical intervention group, one patient developed progressive ocular symptoms involving the third cranial nerve, which required surgical intervention. Out of the five remaining patients under medical treatment, four patients showed improvement in clinical symptoms and resolution of radiological features and one patient showed recurrence of the lesion. In the surgical group, the diagnosis was made after the histopathological confirmation. In the surgical group, the diagnosis was made after the histopathological confirmation in all patients except one. All the patients in this group presented with optic nerve compression requiring surgical decompression. All the patients in this group showed symptomatic improvement postoperatively. Symptoms for deficiency of hormones required supplementation dose of steroids. Steroids should be considered as the first line of management in LYH. Surgical resection should be considered in the presence of optic nerve and/or chiasmal compression and for lesions nonresponsive to medical management.

Lymphocytic hypophysitis in the elderly: A case presentation and review of the literature

Abstract Lymphocytic hypophysitis (LH), an autoimmune mediated chronic inflammation of the pituitary gland, is uncommon in the elderly population greater than 70 years old. It most commonly occurs in peripartum women and classically presents with mass-effect symptoms, hyperprolactinemia, or with symptoms of adenohypophysial or neurohypophysial involvement. We report a case of an elderly female who presented with headaches, visual defects, panhypopituitarism, and a sellar mass thought to be a non-functioning pituitary macroadenoma. On surgical pathology the diagnosis of LH was made. In a comprehensive literature search, we have found only sixteen cases of LH in the elderly. A comparison of the clinical differences between the pediatric, adult, and elderly populations with LH revealed notable differences between the clinical presentations and hormonal deficiencies present in the pediatric versus the adult and elderly populations. A much larger portion of adults and the elderly present with headache and visual disturbances, while a majority of the pediatric population presents with diabetes insipidus and growth hormone deficiency. Adults and elderly with LH have a much higher association with autoimmune disease than children. There was no gender predilection found in the elderly population, which is a notable difference from the adult population in which female cases of LH are much more common.

Lymphocytic hypophysitis presenting as a visual threatening sellar mass and its complete recovery with oral steroids: think beyond surgery

A 37-years female patient presented to us with hypothyroidism. She was started on oral thyroid replacement and sent back to home. Four weeks later she again came with history of progressive vision loss and headache. Ophthalmological evaluation revealed bitemporal hemianopia with bilateral papilloedema. Non-contrast computed tomography revealed mass lesion in pituitary fossa. Hormone profile showed secondary hypocortisolism and secondary hypothyroidism. Magnetic resonance imaging (MRI) brain showed diffusely enlarged sellar mass with suprasellar extension with enhancement of both anterior, posterior pituitary andinfundibulam. Neurologist advised urgent surgical intervention. A diagnosis of lymphocytic hypophysitis was made based on clinical and radiological evidences and surgery was deferred. Patient was given oral steroid on OPD basis and she improved dramatically. The mass also vanished subsequently on follow up MRI brain. This case illustrates the potential benefit of trial of medical management when there is a strong possibility even with vision loss due to mass effect in lymphocytic hypophysitis.

Lymphocytic hypophysitis masquerading as pituitary adenoma

Introduction:Pituitary hypophysitis (PH) is characterized by pituitary infiltration of lymphocytes, macrophages, and plasma cells that could lead to loss of pituitary function. Hypophysitis may be autoimmune or secondary to systemic diseases or infections. Based on the histopathological findings PH is classified into lymphocytic, granulomatous, xanthomatous, mixed forms (lymphogranulomatous, xanthogranulomatous), necrotizing and Immunoglobulin- G4 (IgG4) plasmacytic types.Objective:To report a case of lymphocytic hypophysitis (LH).Case Report:A 15-year-old girl presented with history of headache, amenorrhea, and history of polyuria for past 4 months. Initial evaluation had suppressed follicular stimulating hormone (<0.01 mIU/ml), high prolactin levels (110.85 ng/ml) and diabetes insipidus (DI). Magnetic resonance imaging of sella was suggestive of pituitary macroadenoma with partial compression over optic chiasma. Patient underwent surgical decompression. Yellowish firm tissue was evacuated and xanthochromic fluid was aspirated. Histopathology was suggestive of LH. She resumed her cycles postoperatively after 4 months, prolactin levels normalized, however, she continues to have DI and is on desmopressin spray. This case has been presented here for its rare presentation in an adolescent girl because it is mostly seen in young females and postpartum period and its unique presentation as an expanding pituitary mass with optic chiasma compression.Conclusion:Definitive diagnosis of LH is based on histopathological evaluation. Therapeutic approach should be based on the grade of suspicion and clinical manifestations of LH.

Lymphocytic Hypophysitis Accompanied by Aseptic Meningitis Mimics Subacute Meningoencephalitis

A 66-year-old man was admitted to our hospital with muscle pain, fatigue and appetite loss that had lasted for a month. The patient was somnolent and had nuchal stiffness with a high fever. Cerebrospinal fluid (CSF) tests demonstrated lymphocytic pleocytosis with a decreased CSF-blood glucose ratio. The tests for tuberculous, fungal and carcinomatous meningitis and herpetic meningoencephalitis were negative. Endocrinological exams showed hypopituitarism and diabetes insipidus. A pituitary MRI showed an enlargement of the pituitary stalk. A diagnosis of lymphocytic hypophysitis with aseptic meningitis was made after the exclusion of secondary hypophysitis. Lymphocytic hypophysitis with aseptic meningitis can mimic subacute meningoencephalitis.

Lymphocytic Hypophysitis with a Long Latent Period from Onset of Central Diabetes Insipidus to Development of Pituitary Enlargement

A 61-year-old man presented with central lymphocytic hypophysitis. Initial pituitary MRI imaging was normal, except for loss of the "bright spot" of the posterior lobe. A diagnosis of idiopathic diabetes insipidus was made. Two years later, pituitary gland enlargement with panhypopituitarism was detected. Eight months after commencing a replacement dose of corticosteroid, the pituitary enlargement was reduced in size. These findings resulted in a diagnosis of lymphocytic hypophysitis. In patients with idiopathic diabetes insipidus, it is important to suspect lymphocytic hypophysitis and to perform a long follow-up to repeat endocrinological examinations and pituitary imaging.

Bilateral Dacryoadenitis Complicated by Lymphocytic Hypophysitis

Three patients developed dacryoadenitis (DA) or lymphocytic pneumonitis before the diagnosis of lymphocytic hypophysitis (LyH). There were two previous reports of concurrence of DA and LyH. Our patients add support to the idea that DA and LyH are manifestations of a systemic autoimmune disease. We suggest that the discovery of DA should prompt imaging and endocrine investigation of LyH.

Lymphocytic Hypophysitis Occurring Simultaneously with a Functioning Pituitary Adenoma

Lymphocytic Hypophysitis (LH) is a rare and previously under-recognised disorder, most commonly affecting young females in the post-partum period. It presents clinically with symptoms and signs related to either a pituitary mass or hypopituitarism, frequently mimicking a pituitary adenoma; the diagnosis of LH can only be made histologically with the presence of a dense lymphocytic infiltration usually confined to the anterior pituitary. We present two case histories of patients who presented with symptoms suggestive of a functioning pituitary adenoma who also had concomitant LH confirmed histologically. The first case was a 39 year old lady, with a history of primary hypothyroidism, who presented with weight gain and hirsutism and clinical and biochemical features of Cushing's syndrome. The second case was a 61 year old male, also with a history of primary hypothyroidism, who presented with visual field loss and biochemically with hyperprolactinaemia. In both patients, magnetic resonance (MR) imaging of the pituitary demonstrated an enlarged partially cystic pituitary mass with slight suprasellar extension. Both patients were treated surgically with transphenoidal drainage and excision and histological examination of the surgical specimens demonstrated a mixture of pathologies with fragments of adenohypophyseal tissue (staining positive for ACTH and prolactin respectively) with a dense chronic inflammatory cell infiltrate suggestive of LH in nearby normal anterior pituitary. In both cases a joint diagnosis of a functioning pituitary adenoma with LH was made. There have been only several reported cases of this combination of pathologies but LH even in isolation is becoming increasingly recognised.

Lymphocytic hypophysitis: long-time follow-up of clinical and MR findings

Objectives: We evaluated clinical and MR findings of hypophysitis in the course of a long-term follow-up period. Methods: We retrospectively studied 11 patients, aged 7–67 years (average 37.3), three men and eight women. Five patients presented diabetes insipidus (DI), two anterior lobe dysfunction, two headache, one double vision, and one visual disturbance. The patients were divided into two groups: six patients with adenohypophysitis and five patients with neurohypophysitis presenting DI. Clinical and MR findings were followed in these patients from 16 to 101 months (average 44.5). Results: In adenohypophysitis, enlargement of the pituitary was found in all patients on initial image and continued from 1 to 17 months (average 6.8). Enlargement of the stalk was found in five patients on initial image and continued from 3 to 17 months (average 5.2). In two patients, recurrence was seen at 52 and 3 months. In neurohypophysitis, DI disappeared in only one patient at 14 months. However, loss of high intensity of the posterior lobe was found in all patients and continued on all follow-up images. Enlargement of the stalk was found in all patients on initial image and continued more than 12 months (average 18.4). Enlargement of the pituitary was not found in any patient on initial image, and atrophy of the pituitary was seen in three on follow-up image. Any recurrences were not found during follow-up period. Conclusions: Since diagnosis of lymphocytic hypophysitis (LH) is difficult to make when only using the initial clinical and MR findings, it is important to follow-up clinical and MR findings regarding confirmation and recurrence.

Lymphocytic Hypophysitis: Late Recurrence Following Successful Transsphenoidal Surgery

Lymphocytic hypophysitis (LH) is an inflammatory dis-ease of the anterior pituitary. The varying clinical presentation and the short-term outcome of LH have been extensively described in several case reports or small cohort studies. However, little is known about the long-term outcome of this disease. It is currently believed that if left untreated it may run a self-limited course followed by full resolution of the mass with or without persisting pituitary failure. We describe a 29-yr-old female who presented with secondary amenorrhea, headaches, visual defects, and a pituitary mass, which was removed by transsphenoidal surgery. Histology was consistent with the diagnosis of LH. Following surgery the patient demonstrated a gradual recovery of gonadotroph function with restoration of menses and a successful pregnancy. However, 3 yr after delivery and 6 yr following her initial presentation she developed amenorrhea, headaches, and a pituitary mass. Institution of steroid therapy resulted in resolution of the pituitary mass. In summary, this case illustrates that similarly to many other disorders of autoimmune origin LH may run a fluctuating course and late recurrence is possible even after the successful removal of the inflammatory mass, thus necessitating long-term follow-up of these patients.

Transient Pituitary Enlargement and Dysfunction due to Lymphocytic Hypophysitis

The patient is a 23-year-old man, presenting with headache, central hypogonadism, and diffuse pituitary enlargement seen with magnetic resonance imaging (MRI). Because symptoms related to a pituitary mass were lacking, and after major etiologies of a pituitary fossa solid mass were ruled out with exception of an inflammatory pituitary disorder, we determined to follow the patient closely. Serum antipituitary antibodies, measured by an immunofluorescence technique using rodent pituitary cell lines GH3 and AtT-20, were strongly positive, suggesting lymphocytic hypophysitis. Three months after first consultation, a pituitary MRI showed a normal-sized gland. Prolactin deficiency was demonstrated using dynamic hormone testing. Antipituitary antibodies continued to be positive to GH3 and AtT-20 rodent pituitary cell lines. These findings remained until 20 months after presentation when pituitary function became normal. Antipituitary antibodies, however, remained positive until 48 months after presentation. Several points illustrated by our case are worthy of note. First, the diagnosis of lymphocytic hypophysitis was suspected and then confirmed without pituitary surgical exploration and biopsy. Second, in this case the disease was selflimited. Third, it suggests that pituitary autoantibodies can be a useful tool in diagnosing a noninvasive evaluation in suspected cases of lymphocytic hypophysitis.