Clinical Overview > Condition/ Syndrome > Hypophysitis

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Jaya Sujatha Gopal-Kothandapani Department of Human Metabolism, University of Sheffield, Sheffield, UK

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Veejay Bagga Department of Neurosurgery, Royal Hallamshire Hospital, Sheffield, UK

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Stephen B Wharton Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK

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Daniel J Connolly Department of Neuroradiology, Royal Hallamshire Hospital, Sheffield, UK
Department of Neuroradiology, Sheffield Children's Hospital, Sheffield, S10 2TH, UK

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Saurabh Sinha Department of Neurosurgery, Royal Hallamshire Hospital, Sheffield, UK
Department of Neurosurgery, Sheffield Children's Hospital, Sheffield, S10 2TH, UK

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Paul J Dimitri Department of Paediatric Endocrinology, Sheffield Children's Hospital, Sheffield, S10 2TH, UK

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Summary

Xanthogranulomatous hypophysitis (XGH) is a very rare form of pituitary hypophysitis that may present both clinically and radiologically as a neoplastic lesion. It may either be primary with an autoimmune aetiology and can occur in isolation or as a part of autoimmune systemic disease or secondary as a reactive degenerative response to an epithelial lesion (e.g. craniopharyngioma (CP), Rathke's cleft cyst, germinoma and pituitary adenomas) or as a part of a multiorgan systemic involvement such as tuberculosis, sarcoidosis or granulomatosis. It may also present with a variation of symptoms in children and adults. Our case series compares the paediatric and adult presentations of XGH and the differential diagnoses considered in one child and two adult patients, highlighting the wide spectrum of this condition. Endocrine investigations suggested panhypopituitarism in all three patients and imaging revealed a suprasellar mass compressing the optic chiasm suggestive of CP or Rathke's cleft cyst in one patient and non-functioning pituitary macroadenoma in two patients. Magnetic resonance imaging (MRI) demonstrated mixed signal intensities on T1- and T2-weighted sequences. Following endoscopic transsphenoidal surgery, histological analysis revealed necrotic material with a xanthogranulomatous reaction confirming XGH in two patients and a necrobiotic granulomatous chronic inflammatory infiltrate with neutrophils in one patient, which is not typical of current descriptions of this disorder. This case series describes the wide spectrum of XGH disease that is yet to be defined. Mixed signal intensities on T1- and T2-weighted MRI sequences may indicate XGH and diagnosis is confirmed by histology. Histological variation may indicate an underlying systemic process.

Learning points

  • XGH is a rare form of pituitary hypophysitis with a wide clinical and histological spectrum and can mimic a neoplastic lesion.

  • XGH primarily presents with growth arrest in children and pubertal arrest in adolescents. In adults, the presentation may vary.

  • A combination of hypopituitarism and mixed signal intensity lesion on MRI is suggestive of XGH and should be considered in the differential diagnosis of sellar lesions.

  • Radical surgery is the treatment of choice and carries an excellent prognosis with no recurrence.

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Beverly T Rodrigues Department of Diabetes and Endocrinology, The Townsville Hospital, Townsville, Queensland, Australia
School of Medicine and Dentistry, James Cook University, Douglas, Queensland, Australia

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Zulfiquer Otty Department of Oncology, The Townsville Hospital, Townsville, Queensland, Australia

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Kunwarjit Sangla Department of Diabetes and Endocrinology, The Townsville Hospital, Townsville, Queensland, Australia

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Vasant V Shenoy Department of Diabetes and Endocrinology, The Townsville Hospital, Townsville, Queensland, Australia
School of Medicine and Dentistry, James Cook University, Douglas, Queensland, Australia

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Summary

Autoimmune hypophysitis (AH) has been previously described in a typical demographic population, primarily women in the reproductive age group and perinatal period. The era of immune modulation using anti-cytotoxic T-lymphocyte-associated antigen 4 biological therapy (ipilimumab) against advanced cancers like metastatic melanomas has now resulted in a new form of hypophysitis being increasingly recognised under a spectrum of immune-related adverse events. Drug-related AH often presents with subtle symptoms and a pituitary mass, with the potential for fatality necessitating wide awareness and a high index of clinical suspicion given that it is usually treatable. We describe below two cases of AH within the last three months at our centre, which were treated with different regimens and produced good endocrine outcomes.

Learning points

  • AH is a new and defined clinical entity occurring as a side effect of ipilimumab, which enhances immune-mediated destruction of metastatic melanoma.

  • It can present insidiously and have life-threatening complications related to hypocortisolism, hence a high index of clinical suspicion must be exerted by treating physicians, and seems to result in resolution of pituitary masses and variable improvements of pituitary function.

  • Clinical improvement, radiological resolution of pituitary masses and variable normalisation of pituitary function are possible with early treatment with high-dose oral or i.v. steroids and hormone replacement therapy, although duration and dosing protocols are unclear at this stage.

  • Ipilimumab should continue to be prescribed as treatment for metastatic melanoma; however, close clinical observation of patient's progress must be maintained while they are on this drug.

  • Predictive factors for onset of AH remain unclear and it is imperative that AH is distinguished from pituitary metastases.

  • Further studies are required to determine the safety of continuing therapy with ipilimumab in patients who have developed AH while on treatment.

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K Majumdar University College London Hospitals NHS Foundation Trust, Diabetes and Endocrinology, 250 Euston Road, London NW1 2PG, UK

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M Barnard Whittington Hospital, London, UK

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S Ramachandra Whittington Hospital, London, UK

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M Berovic Whittington Hospital, London, UK

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M Powell National Hospital for Neurology and Neurosurgery, Neurosurgery, London, UK

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Summary

Tuberculosis (TB) is an important cause of mortality and morbidity across the world. In 2–5% of all cases of systemic TB, the C is affected, with lesions reported in the meninges, cortex and ventricles. Intrasellar tuberculomas, however, are extremely rare. We report the interesting case of a young female patient who presented with secondary hypothyroidism and hyperprolactinaemia. She was treated successfully for pituitary TB. We also highlight and discuss some interesting (and hitherto unreported) endocrine issues. Radiological and histological features and treatment of pituitary TB are discussed using this case as an example.

Learning points

  • Intrasellar TB continues to be a rare presentation, but incidence and prevalence are expected to grow with increasing numbers of multidrug-resistant TB and shrinking geographical boundaries across the world.

  • Pituitary TB can present with features of a typical adenoma, but has certain radiological and histological features that help to differentiate from an adenoma.

  • Patients can present with a variety of endocrine abnormalities at different times.

  • The presence of an intrasellar mass in individuals at a high risk of developing TB, or with a previous history of systemic TB, should prompt the diagnosis of pituitary TB. In such individuals, it may be worth considering a trial of anti-tuberculous therapy, before considering surgery.

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Gabriela Alejandra Sosa Departments of Endocrinology, Metabolism and Nuclear Medicine

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Soledad Bell Departments of Endocrinology, Metabolism and Nuclear Medicine

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Silvia Beatriz Christiansen Pathology

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Marcelo Pietrani Radiology

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Mariela Glerean Departments of Endocrinology, Metabolism and Nuclear Medicine

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Monica Loto Departments of Endocrinology, Metabolism and Nuclear Medicine

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Soledad Lovazzano Departments of Endocrinology, Metabolism and Nuclear Medicine

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Antonio Carrizo Neurosurgery, Hospital Italiano, Perón 4190, 1202 Buenos Aires, Argentina

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Pablo Ajler Neurosurgery, Hospital Italiano, Perón 4190, 1202 Buenos Aires, Argentina

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Patricia Fainstein Day Departments of Endocrinology, Metabolism and Nuclear Medicine

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Summary

IgG4-related hypophysitis is a recently described entity belonging to the group of IgG4-related diseases. Many other organs can also be affected, and it is more common in older men. To date, 32 cases of IgG4-related hypophysitis have been reported in the literature, 11 of which included confirmatory tissue biopsy and the majority affecting multiple organs. The aim of this report is to present two cases of biopsy-proven IgG4-related hypophysitis occurring in two young female patients with no evidence of involvement of other organs at the time of diagnosis.

Learning points

  • IgG4-related hypophysitis belongs to the group of IgG4-related diseases, and is a fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis.

  • It is more common in older men, but young women may also present this type of hypophysitis.

  • Although involvement of other organs is frequent, isolated pituitary disease is possible.

  • Frequent clinical manifestations include anterior hypopituitarism and/or diabetes insipidus.

  • The diagnosis may be confirmed with any of the following criteria: a pituitary biopsy with lymphoplasmacytic infiltrates, with more than ten IgG4-positive cells; a sellar mass and/or thickened pituitary stalk and a biopsy-proven involvement of another organ; a sellar mass and/or thickened pituitary stalk and IgG4 serum levels >140 mg/dl and sellar mass reduction and symptom improvement after corticosteroid treatment.

  • Glucocorticoids are recommended as first-line therapy.

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