Xanthomatous hypophysitis

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.

Xanthomatous hypophysitis

Disclosure: J. John: None. S. Graybill: None. Lymphocytic hypophysitis is the most common presentation of primary pituitary gland inflammation. It typically presents with headache, vision changes, and sequelae of hypopituitarism, including central diabetes insipidus, anterior pituitary dysfunction, and hyperprolactinemia. Lymphocytic hypophysitis has a propensity to affect women in association with pregnancy due to an unknown underlying mechanism. A 38-year-old female presented with intractable headache and peripheral vision changes 3 weeks after giving birth to twin boys. Her post-partum course was complicated by preeclampsia with severe features. She also began experiencing blurry vision during this period. Seventeen days after delivery, she developed an acute onset headache and peripheral vision loss. She also noted sudden cessation of lactation with the onset of her headache. Initial CT head demonstrated a 1.6 centimeter enhancing pituitary mass suspicious for macroadenoma, and subsequent MRI sella revealed a 2.0 x 1.7 x 1.5 cm heterogeneously enhancing mass arising from the pituitary adenohypophysis extending to the undersurface of the optic chiasm. Initial laboratory evaluation demonstrated central hypothyroidism and inappropriately normal prolactin for a recently post-partum female. Given the patient’s impending vision loss, she was taken to the operating room for resection of suspected pituitary macroadenoma. Upon gross dissection, the abnormal appearance of the suspected mass prompted biopsy and immediate frozen section evaluation demonstrated significant lymphocytic infiltration. The resection was subsequently aborted. The patient was placed on levothyroxine and prednisone 80 milligrams daily for suspected lymphocytic hypophysitis, which was confirmed on tissue pathology. The patient’s headache and vision changes improved and after six days, she was discharged on a prednisone taper. Following three months of prednisone therapy, a repeat MRI sella demonstrated a reduction in the overall size of the pituitary gland to 1.7 x 1.2 x 1.1 cm. The patient was maintained on physiologic hydrocortisone and levothyroxine while monitoring for potential pituitary function recovery. Twelve months following her initial diagnosis, the patient did not demonstrate normalization of anterior pituitary function and required ongoing treatment for hypopituitarism. While hypoprolactinemia is atypical of hypophysitis, it is imperative to consider alternative pituitary pathology such as lymphocytic hypophysitis in the post-partum female when the clinical history is suspicious. Presentation: Friday, June 16, 2023

We report a rare case of recurrent lymphocytic hypophysitis (LyH) after transsphenoidal surgery and high dose methylprednisolone pulse therapy (HDMPT). The patient was treated with azathioprine, and achieved spontaneous pregnancy. A 22-year-old nulliparous female was seen in June 2004 due to headaches, diplopia and left eye ptosis, and bilateral milky nipple discharge. Six months prior she had undergone transsphenoidal pituitary tumor resection at another hospital, and the histopathological diagnosis was ‘‘pituitary tumor with inflammatory response.’’ The patient had no significant medical history, menarche at age 16, and regular cycles until March 2004. Physical examination was normal except for left eye ptosis and adduction limitation, and bilateral milky nipple discharge. Routine laboratory tests and T3, T4, TSH, LH, FSH, E2, prolactin, ACTH, cortisol, and 24 h urinary free cortisol were within normal limits. Thyroid autoantibodies (TGAb and TPOAb) and TSH receptor antibody were negative. Magnetic resonance imaging (MRI) showed abnormal, enhancing, irregular soft tissue signals with unclear boundaries in the sellar region and suprasellar cistern. Histopathological examination revealed lymphocyte, histiocyte, and neutrophil infiltration in the pituitary tissue, consistent with LyH. High dose methylprednisolone pulse therapy (HDMPT) was begun, and her symptoms rapidly improved. MRI showed the pituitary significantly reduced in size and the lesions had disappeared. The patient was asymptomatic until December 2005 when she experienced headaches, diplopia, and nausea and vomiting. MRI showed normal hypothalamic and pituitary morphology, and abnormal enhancement on the right side of the dorsum sellae and near the tentorium cerebelli. She was treated with azathioprine 100 mg/day for 16 weeks, and prednisone 30 mg/ day (decreased 5 mg/week until withdrawal). After treatment, she experienced regular menstrual cycles and conceived spontaneously in April 2009. Her pregnancy and delivery were uneventful with no LyH relapse during pregnancy or to date. The natural course of LyH can include sellar massoccupying effects, pituitary disorders, diabetes insipidus, and hyperprolactinemia [1]. MRI findings are not specific enough to distinguish LyH from pituitary adenomas; thus careful correlation with clinical and laboratory findings is critical [1]. Transsphenoidal resection of pituitary lesions, drugs (corticosteroids, azathioprine, and methotrexate), and radiotherapy have all been used to treat LyH; however, none are effective in all cases [1, 2]. Lecube et al. [3] first reported the successful treatment of LyH using azathioprine in a patient who failed corticosteroid treatment, and subsequently other authors have reported success with azathioprine [4]. Lymphocytic hypophysitis (LyH) is significantly correlated with pregnancy; of 210 reported cases in females, 120 (57%) occurred during pregnancy or the postpartum period, primarily in the final month of pregnancy or first 2 months postpartum [5]. If onset is during pregnancy, complications or adverse effects on the fetus are unlikely, and the condition may gradually resolve after delivery. A history of pregnancy-related LyH is not associated with relapse Z. Lu and G. Yang contributed equally.

MRI revealed enlargement of the pituitary stalk in two patients with different clinical presentations. A 60 y/o female had autoimmune hypothyroidism associated with blunted cortisol response to ACTH, while the other, a 30 y/o female had amenorrhea and polyuria with high plasma and low urinary osmolality.

Lymphocytic hypophysitis is an uncommon autoimmune condition that often results in significant morbidity. Although most cases resolve spontaneously or after a short course of steroids, rarely, refractory cases can cause persistent neurological deficits despite aggressive medical and surgical management. A 41-year-old woman presented with progressive visual loss in the left eye and was found to have a sellar mass. She underwent transsphenoidal surgery because of lesion enlargement. Histopathology was consistent with adenohypophysitis with B-cell predominance. Despite steroid treatment, her neurological condition worsened and she experienced loss of vision in the right eye. Craniotomy with decompression of the right optic nerve was performed. Her condition improved initially, but she continued to have progressive visual compromise over the following months. She was therefore treated with rituximab, a monoclonal antibody against B cells. Her vision improved significantly within a few weeks. There was no clinical or radiographic exacerbation 2 years after starting immunotherapy. Rituximab, an anti-CD20 antibody that specifically depletes B lymphocytes, can be an effective treatment strategy for patients with steroid-refractory, B cell-predominant lymphocytic hypophysitis.

Disclosure: C. Kelly: None. L. Ghalib: None. Hypophysitis is rare; accounting for 0.24-0.39% of all pituitary diseases. Hypophysitis can be primary or secondary in nature. Granulomatous disease rarely causes pituitary enlargement, but Sarcoidosis and Granulomatosis with Polyangiitis have been documented to cause granulomatous infiltration of the pituitary. This case regards a man with granulomatous infiltration of the pituitary not consistent with either of the aforementioned disease processes. A 40 year old male presented to Urgent Care with headaches and blurry vision. Headaches were unresponsive to analgesics. The patient then presented to his PCP with the same complaints. MRI was then performed which showed a heterogeneously enhancing sellar and suprasellar mass. He was evaluated by Neurosurgery who recommended he undergo endoscopic resection of the pituitary lesion. Preoperative evaluation of pituitary axes were concerning for central hypothyroidism with a TSH of 1.046 uIU/mL (0.550-4.780) and free T4 of 0.76 ng/dL (0.89-1.76),as well as central hypogonadism with a FSH of 0.4 mIU/mL (<18.1), LH of 0.18 mIU/mL (1.5-9.3),and testosterone of 17 ng/dL (87-814). All other axes normal. Resection of the pituitary mass occurred approximately one week later at which time he was evaluated by Endocrinology for the first time. Prior to discharge, the patient was started on levothyroxine, but gonadal replacement was deferred to outpatient setting. Grossly the pituitary mass was concerning for lymphocytic hypophysitis (LH), and thus the patient was discharged on prednisone for empiric therapy. At the time of his endocrinology follow up, his headaches and visual changes had improved, but he was experiencing polydipsia, polyuria, and nocturia. Labs were consistent with Diabetes Insipidus (DI), and he was started on desmopressin. Surgical pathology had returned “acute and chronically inflamed granulation-like tissue associated with necrosis” confirming LH. CT chest was performed to evaluate for secondary causes of granulomatous disease which showed mediastinal lymphadenopathy without parenchymal lung disease. Serologic evaluation was positive for ANA, myeloperoxidase antibody, and SS-B antibody. While attempting to wean his prednisone the patient experienced recurrence of his headaches, which resolved when he returned to higher doses. The patient was evaluated by Pulmonology given the mediastinally mphadenopathy, who recommended bronchoscopy. When specimens from the mediastinal lymph nodes were evaluated by pathology, it was again significant for necrotizing granulomatous disease, but ACE and IL-2 were negative which argued against a diagnosis of sarcoidosis. He was evaluated by Rheumatology who recommended further infectious work upas well as consideration of initiation of a steroid-sparing agent for granulomatous disease. Workup of secondary etiologies for LH is ongoing given the systemic involvement. Presentation: Friday, June 16, 2023

Background: Pituitary adenomas are the most common sellar masses in adults with magnetic resonance imaging (MRI) being the imaging modality of choice. Inflammatory pituitary lesions such as lymphocytic hypophysitis (LH) can mimic pituitary macroadenoma on imaging and are often misdiagnosed as such. Although the imaging appearance on most of the sequences on MRI has similar findings, LH has a characteristic dark signal on T2 images (called dark T2 sign) which can be very helpful to reliably differentiate the two conditions.Case Description: A 68-year-old woman diagnosed with a “pituitary mass” on the MR study done at an outside facility was referred to our neurosurgery department. The case was discussed at our multidisciplinary tumor board, where the possibility of an inflammatory condition mimicking tumor was considered, given the very dark signal on T2-weighted sequences. Transsphenoidal endoscopic biopsy revealed a firm rubbery mass, which histopathology demonstrated fibrous connective tissue with inflammatory cells consistent with LH.Conclusion: Dark T2 signal on MR imaging can be very helpful in demarcating inflammatory pituitary conditions like LH from pituitary macroadenomas.

We report the first case of pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroids without surgery. A 27-year-old woman had been monitored for chronic headache 13 months after giving birth, associated with amenorrhea and galactorrhea. Cranial magnetic resonance imaging revealed a markedly enlarged pituitary gland with a suprasellar extension; the only biochemical abnormality was a mild hyperprolactinemia. Because of a putative diagnosis of prolactinoma, bromocriptine was prescribed at a dose of 5 mg daily, soon followed by the transitory appearance of menstruation. Two years later, panhypopituitarism was present and was revealed by acute adrenal insufficiency. Magnetic resonance imaging revealed that the pituitary mass was the same as previously described, but hormonal investigation showed evidence of complete hypopituitarism and no hyperprolactinemia. Nuclear antibodies were negative as well as other autoantibodies. Human leukocyte antigen serological Class II typing was DR3/DR4. Lymphocytic hypophysitis was then suspected; in the absence of visual complication and because this patient refused surgery, corticosteroids were attempted at a daily dose of 60 mg of prednisone for 3 months, progressively decreased for the next 6 months. Under this treatment, a gradual recovery of all pituitary hormones was observed and magnetic resonance imaging showed a reduction of two-thirds in pituitary mass. Five months after the end of corticoid treatment, our patient relapsed with panhypopituitarism and an increase of pituitary volume. She underwent steroid treatment, and a biopsy was performed and confirmed the diagnosis of autoimmune hypophysitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Total quality culture in radiotherapy departments across Ontario

On Friday, May 14, 2021, the Health Service Executive, the organization providing public health services in the Republic of Ireland, was the victim of a significant cyberattack on its information technology systems. All systems were subsequently shut down to prevent further damage and to allow cybersecurity experts to investigate the attack. As a result, oncology services were severely disrupted, with the cessation of radiation therapy treatments in all public radiation therapy departments. Ireland has 5 large public and 6 smaller private radiation therapy centers in total. Because of the widespread adoption of electronic medical records in radiation therapy departments, it wasn't possible to retrieve patient details of those who were undergoing radiation therapy at the time of the cyberattack. In total, 513 patients nationally had their radiation therapy interrupted.A national radiation therapy cyberattack response team was formed immediately to oversee the response to the attack. The immediate concerns were radiation therapy emergencies and category 1 patients where gaps in treatment would have an adverse effect on outcome. Communication with patients and the public was also established as a priority and agreements were reached with the private sector for the treatment of patients affected by the cyberattack. The national media was used to alert patients of the need to communicate with their radiation therapy department. Dedicated phone lines were established. Locally, radiation therapy departments held daily crisis meetings with key staff members, including information technology personnel. Individual centers employed different technologies for treatment planning and data storage, so local solutions to the cyberattack to reestablish radiation therapy for patients were developed. In addition, national documentation on prioritization of patients to resume treatment was produced and a national approach was made to compensate for gaps in treatment caused by the attack. All 5 centers had reestablished radiation therapy by May 30, although there has been a long aftermath to the cyberattack. In this article, we provide an overview of the effects of the cyberattack on our national radiation therapy service and our strategy to resume patient treatment in a timely fashion.

Disclosure: H.C. Davuluri: None. K. Kaur: None. P. Chaulagain: None. E.I. Krug: None. A 16-year-old female presented with a history of headaches of 1 year duration and was initially diagnosed with migraine without aura. At the same time, she developed menstrual irregularity and eventual amenorrhea, was prescribed a 3 months course of OCPs. Amenorrhea persisted following discontinuation of OCP regimen. She was seen by a pediatric neurologist for worsening headaches, associated with light sensitivity and worsening of pain with noise, coughing or sneezing. Brain MRI revealed a 1.5 cm pituitary mass with displacement of the optic chiasm. Fortunately, ophthalmologic evaluation was unremarkable. Endocrine evaluation revealed evidence of central hypogonadism and mild hyperprolactinemia with prolactin level of 57.6 ng/ml [4.8-33.4 ng/ml]. ACTH, cortisol, growth hormone, IGF-I, TSH, free T4 levels were within normal limits. Her growth and development were normal for her age. Cabergoline (0.5 mg twice weekly) was started, reducing prolactin to 0.2 ng/ml, with no significant change in headache frequency and no resolution of amenorrhea. Subsequently, she underwent transnasal transsphenoidal biopsy of the suspected pituitary mass. Intraoperative findings revealed a thickened dura with fibrous appearance and a mass-like lesion adherent to the dura. Frozen sections analysis revealed no evidence of a neoplasm; the findings are suggestive of lymphocytic hypophysitis. Histopathology confirmed the diagnosis of lymphocytic hypophysitis. Further underwent a rheumatological workup, including ESR, CRP, ANA, anti-SSA/SSB, anti-dsDNA, and ANCA, all of which were unremarkable. She received glucocorticoid therapy with prednisone, 25 mg daily with prolonged steroid taper over a period of 12 months. Serial brain MRIs revealed a reduction in the size of the pituitary gland to 6 mm after 4 months of treatment. Repeat imaging at 3 and 6, 9 and 12 months showed continued resolution. Her menstrual cycle normalized after 4 months of treatment, and headaches significantly improved. Glucocorticoid therapy has been discontinued after 12 months of treatment with no evidence of recurrence so far. Discussion: We present a case of histologically confirmed lymphocytic hypophysitis with complete radiologic and functional recovery following prolonged glucocorticoid therapy at a relatively low dose with very slow taper. Affected patients typically present with severe headaches and symptoms of pituitary dysfunction. Optimal treatment is not known at this time. There is a report describing clinical improvement in 4 patients following high dose methylprednisolone pulse therapy and another report describing resolution of symptoms after a short course of glucocorticoids in 2 patients. Additional research is needed to determine best treatment strategy, but it appears that high index of suspicion and early diagnosis may prevent progressive pituitary atrophy and hypopituitarism. Presentation: Monday, July 14, 2025

Radiation therapy for head and neck cancer frequently caused severe salivary gland dysfunction. The salivary gland dysfunction possibly decreased the protective function of saliva and caused dental caries. The purpose of this study was to obtain an illustration about DMF-T index in patient undergoing radiation therapy with LINAC X-ray radiation for head and neck cancer at Department of Radiotherapy, Dr. Hasan Sadikin Hospital in January-February 2007. The study was a simple descriptive. The study was conducted on 7 males and 9 females undergoing radiation therapy with LINAC X-ray radiation for head and neck cancer. The ages of patient are between 37 years and 77 years. The severity of caries was measured by DMF-T index. DMF-T index in 16 patient undergoing radiation therapy with LINAC X-ray radiation for head and neck cancer at Dr. Hasan Sadikin Hospital is 10.6 as the result of this study. The conclusion of this study showed that the DMF-T index in 16 patient undergoing radiation therapy with LINAC X-ray radiation for head and neck cancer at Dr. Hasan Sadikin Hospital had very high grade based on WHO classification, which the value was over 6.6.

Lymphocytic hypophysitis (LYH) is a rare neuroendocrine disorder characterized by autoimmune inflammation of the pituitary gland. Visual disturbance is one of the most common and serious symptoms of LYH. Most of the visual symptoms in LYH are secondary to compression of the optic chiasm and some reports have described direct inflammatory involvement of the optic pathways. We describe a 30-year-old man with a 9-day history of bilateral blurred vision. Ophthalmic examination demonstrated severely impaired vision without temporal hemianopsia. Hypothyroidism, hypocortisolism, and hypogonadism were detected in laboratory tests. Central diabetes insipidus was diagnosed by a hypertonic saline infusion test. MRI revealed thickening of the pituitary stalk and enlargement of the hypophysis, which was enhanced with gadolinium. High intensity of the posterior lobe was not recognized on T1-weighted images. These findings established a clinical diagnosis of lymphocytic panhypophysitis. Methylprednisolone pulse therapy was introduced and his visual acuity gradually recovered. The anterior pituitary function improved, but desmopressin was still required. Pattern-reversal visual evoked potentials (VEP) have been widely used to detect optic nerve lesions caused by multiple sclerosis and brain tumors. However, there have been no previous reports of their usefulness for LYH. The P100 latency in our case was slightly prolonged and the amplitude was markedly reduced. These findings are similar to ischemic optic neuropathy and other conditions in which axonal damage is prominent. The prolonged latency and low amplitude on VEP examination in this case showed rapid improvement in parallel with the recovery of visual acuity. Taken together, our case implies the usefulness of pattern-reversal VEP for the diagnosis of optic neuritis in LYH, especially for the evaluation of its pathogenic mechanisms.

In two patients with lymphocytic adenohypophysitis, images of the pituitary gland were serially observed by MRI. In both cases, the pituitary gland had swollen during the late stage of the first pregnancy. In case 1, MRI findings were representative of lymphocytic adenohypophysitis. After delivery, plasma levels of PRL, ACTH and cortisol decreased markedly. The height of the pituitary gland gradually decreased from 22 mm (14 days after delivery) to 13 mm (73 days) and became rapidly smaller (4.9 mm, 115 days) following administration of massive doses of hydrocortisone for the treatment of acute adrenal insufficiency induced by painless thyroiditis. Six years later, the height was 2.5 mm. Low plasma levels of PRL and cortisol persisted. Diabetes insipidus did not develop. In case 2, MRI revealed a pituitary mass accompanied by a cystic change. Lymphocytic adenohypophysitis was confirmed by histological examination. Because pituitary function tests indicated that ACTH, PRL, GH and TSH were of low levels, hydrocortisone and L-thyroxine were orally administered. No diabetes insipidus was demonstrated. MRI disclosed that the height of the pituitary gland was 23 mm (17 days after delivery) but decreased to 17 and 5.5 mm after 44 and 128 days, respectively. Four years later immediately after the second delivery, it was 1 mm, and the patient was diagnosed as having empty sella. Long-term observation of lymphocytic adenohypophysitis demonstrated that the pituitary gland was markedly atrophied, leading to empty sella. It is believed that some of the classic cases of Sheehan's syndrome associated with empty sella may include lymphocytic adenohypophysitis.