Recurring Primary Xanthomatous Hypophysitis Behaving Like Pituitary Adenoma: Additional Case and Literature Review.

BACKGROUND: Xanthomatous hypophysitis (XH) is the rarest histologic type of primary hypophysitis. It is nonlymphocytic and characterized by an infiltration of the pituitary gland by lipid-laden histiocytes and macrophages. The clinical and radiologic features overlap heavily with pituitary adenomas and are prone to misdiagnosis. We describe a rare case of XH recurrence at 1 year, treated surgically. Moreover, we provide an updated review of the literature to further elucidate useful management and detection strategies. CASE DESCRIPTION: A 45-year-old woman presented with a history of menstrual irregularity for 9 months, amenorrhea, galactorrhea, and headache for 2 months duration. Preoperative endocrinologic studies showed increased prolactin levels. Magnetic resonance imaging of the sella showed a cystic lesion with suprasellar extension suggestive of a pituitary adenoma. The patient underwent transsphenoidal resection, showing a thick yellowish colloidal material. Histopathology showed necrotic tissue with no definitive diagnosis and no identified microorganisms. At 2 months after surgery, prolactin levels and menstrual cycle normalized. At 1 year after surgery, her menstrual cycle again became irregular. Repeat magnetic resonance imaging showed a recurrent mass and a second transsphenoidal resection was undertaken. Repeat histopathology was consistent with XH. The patient is 9 years postoperative and is doing well without evidence of recurrence. CONCLUSIONS: XH presents similarly to nonfunctional adenomas and diagnosis remains difficult without surgical pathology, requiring meticulous immunohistochemistry to prevent misdiagnosis. Thus, XH should be considered as a rare cause in the differential of disease of the sellar region. Management can parallel that of pituitary neoplasm, with a focus on radiosurgery, re-resection, and high-dose steroids.

Clinical and hormonal characteristics of patients with different types of hypophysitis: a single-center experience.

OBJECTIVE: The inflammation of the pituitary gland is known as hypophysitis. It is a rare disease accounting for approximately 0.24%-0.88% of all pituitary diseases. The natural course of hypophysitis is variable. Main forms are histologically classified as lymphocytic, granulomatous, IgG4 related and xanthomatous. We aim to present our patients with hypophysitis and compare clinical, laboratory and radiological features. SUBJECTS AND METHODS: We retrospectively reviewed our database of 1.293 patients diagnosed with pituitary diseases between 2010 and 2017. Twelve patients with hypophysitis were identified. Demographical data, clinical features, endocrinological dysfunction, imaging findings, treatment courses and follow-up periods were evaluated. RESULTS: The frequency of hypophysitis was found 0.93% in all cases of the pituitary disease. Twelve patients (nine females and three males), ages between 17-61 years, were evaluated. The characteristic features of our patients tended to be predominantly female and young. Diagnosis of hypophysitis was made after pituitary biopsy in four patients, and in eight patients after pituitary operation due to adenoma. Headache (67%) and visual problems (33%) were the most frequent nonendocrine symptoms. Anterior pituitary hormone deficiencies (63.7%) and/or diabetes insipidus (17%) were seen among patients. According to histopathological forms, four had lymphocytic, seven had granulomatous and one had xanthogranulomatous types. Contrast enhancement heterogeneous and thickened pituitary stalk were the most common radiological alterations. CONCLUSION: Hypophysitis should be considered in the differential diagnosis of sellar masses. It can mimic pituitary adenomas in radiological and endocrinological aspects. The different patterns of pituitary hormone deficiencies may be seen in the course of the disease.

Clinical and hormonal characteristics of patients with different types of hypophysitis: a single-center experience

ABSTRACT Objective: The inflammation of the pituitary gland is known as hypophysitis. It is a rare disease accounting for approximately 0.24%-0.88% of all pituitary diseases. The natural course of hypophysitis is variable. Main forms are histologically classified as lymphocytic, granulomatous, IgG4 related and xanthomatous. We aim to present our patients with hypophysitis and compare clinical, laboratory and radiological features. Subjects and methods: We retrospectively reviewed our database of 1.293 patients diagnosed with pituitary diseases between 2010 and 2017. Twelve patients with hypophysitis were identified. Demographical data, clinical features, endocrinological dysfunction, imaging findings, treatment courses and follow-up periods were evaluated. Results: The frequency of hypophysitis was found 0.93% in all cases of the pituitary disease. Twelve patients (nine females and three males), ages between 17-61 years, were evaluated. The characteristic features of our patients tended to be predominantly female and young. Diagnosis of hypophysitis was made after pituitary biopsy in four patients, and in eight patients after pituitary operation due to adenoma. Headache (67%) and visual problems (33%) were the most frequent nonendocrine symptoms. Anterior pituitary hormone deficiencies (63.7%) and/or diabetes insipidus (17%) were seen among patients. According to histopathological forms, four had lymphocytic, seven had granulomatous and one had xanthogranulomatous types. Contrast enhancement heterogeneous and thickened pituitary stalk were the most common radiological alterations. Conclusion: Hypophysitis should be considered in the differential diagnosis of sellar masses. It can mimic pituitary adenomas in radiological and endocrinological aspects. The different patterns of pituitary hormone deficiencies may be seen in the course of the disease.

Hypophysitis Due to Paranasal Sinusitis: Neurosurgical Perspective from Developing World.

BACKGROUND: Sinusitis is a common clinical condition, but sphenoid sinusitis is a less common form and even rarer is hypophysitis as a complication of the latter. Clinically, hypophysitis may mimic a pituitary neoplasm in presenting with mass effect and pituitary hormone dysfunction. CASE DESCRIPTION: We present 5 cases of sinusitis-related hypophysitis treated at the Royal Care International Hospital in Khartoum, Sudan. Clinical symptoms at presentation included headache, fever, ptosis, ophthalmoplegia, and history of sinusitis with running nose (nasal discharge). None of the patients were immunocompromised or showed signs of meningitis. Laboratory tests indicated neutrophilia and elevated inflammatory indices, namely C-reactive protein and erythrocyte sedimentation rate. There was also a disturbance of the hypothalamic-pituitary hormone axis, particularly impaired cortisol level. Magnetic resonance imaging scans on all patients revealed swollen masses in the pituitary fossa and enhancement of the sellar region and paranasal sinuses, especially the sphenoid sinus. All cases were empirically treated with hydrocortisone and amoxicillin-clavulanate, resulting in reversal of symptoms. CONCLUSIONS: Sinusitis is common in tropical regions where the climate is usually warm and often hot and dry. Here, the condition is considered a common incidental finding in magnetic resonance imaging examinations done for various indications. Hence it is not considered to be a serious health problem. Though our cohort of cases is small, we emphasize the importance of keeping a high index of suspicion for the diagnosis of hypophysitis in relevant case settings. This would help make an early diagnosis and ensure appropriate medical, perhaps nonsurgical, management.

Xanthomatous Hypophysitis Presenting with Diabetes Insipidus Completely Cured Through Transsphenoidal Surgery: Case Report and Literature Review.

BACKGROUND: Xanthomatous hypophysitis (XH) is extremely rare. Only 27 cases have been reported in the literature. No XH patient presenting with diabetes insipidus (DI) has been completely cured through surgery. Here, we describe the first XH case of a DI patient whose pituitary function was normalized postoperatively, without hormone replacement therapy. CASE DESCRIPTION: A 41-year-old woman suffered from polydipsia, DI, headache, and breast discharge. Laboratory investigation revealed hyperprolactinemia. Pituitary magnetic resonance imaging showed a 2.0-cm × 1.4-cm × 1.6-cm lesion that demonstrated heterogeneous intensity on T1-weighted imaging and peripheral ring enhancement following contrast; the lesion was totally removed through transsphenoidal surgery. Histopathologic and immunohistochemical examinations confirmed the diagnosis of XH. At the 4- and 15-month follow-up visits, all pituitary-related hormones were normal, and the patient was not taking medication. A repeat pituitary magnetic resonance imaging showed no evidence of recurrence. CONCLUSIONS: To the best of our knowledge, this case is the first documented occurrence of XH with DI completely cured through surgery. If XH is suspected, total surgical resection of the lesion is recommended and normal pituitary tissue should be carefully protected intraoperatively.

Endoscopic Endonasal Management of Rare Sellar Lesions: Clinical and Surgical Experience of 78 Cases and Review of the Literature.

OBJECTIVE: In the present study we aim to provide further definition of a group of rare sellar diseases treated by the endoscopic endonasal approach. METHODS: The study was a retrospective analysis of data obtained from a series of 1729 patients who underwent endoscopic endonasal surgery at 2 academic institutions (Università degli Studi di Napoli Federico II, Naples, Italy between January 1997 and December 2013 and the Wexner Medical Center at The Ohio State University between July 2010 and September 2015). Clinical charts, operative notes, and pathology reports were examined. RESULTS: A total of 346 cases were identified to have nonadenomatous diseases. Applying the Rosner test for outliers assisted in excluding relatively frequent lesions. The final cohort of rare sellar diseases comprised 78 patients. Arachnoid cysts were the most frequently encountered sellar lesion (12 patients, 15%), followed by metastasis (11 cases, 14%), followed by hypophysitis (8 cases, 10%), oncocytoma, and glioma (6 cases, 8% each). The most frequent clinical findings were headache (28%) and visual disorders (80%). A standard endoscopic endonasal approach was performed in 44 patients (56%), and an extended approach was carried out in 34 patients (44%). Tumor removal was gross total in 53% of patients, subtotal in 19%, and partial in 21%. Postoperative endocrinologic and visual deficit evaluation showed improvements in endocrine function in 8 patients (10%) and in visual disorders in 13 (16%). Postoperative complications arose in 28% of cases, mostly represented by diabetes insipidus (10%). CONCLUSIONS: Endoscopic endonasal approaches offer some specific benefits in the treatment of these patients.

Clinical Interrogation for Unveiling an Isolated Hypophysitis Mimicking Pituitary Adenoma.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.