Castleman's disease with autoimmune hypophysitis

Dear Editor,Castleman’s disease is an uncommon lymphoproliferativedisorder, usually accompanied with other autoimmunediseases. Autoimmune hypophysitis (AH) is a rare diseaseof the pituitary gland that presents as a sellar mass lesionand/or hypopituitarism, which is speculated to have anautoimmune basis. We reported the first case of Castle-man’s disease accompanied with LH.This is a 34-year-old woman, who suffered fromheadache, diuresis, and irregular menses for 2 years since2005. Laboratory examination showed hyperprolactinemia,anemia, and diabetes. Computed tomography (CT) scan ofthe lung revealed a lymph node enlargement in themediastinum and multiple nodes in the lung. CT scan ofthe abdominal cavity showed a neoplasm located in theposterior abdominal wall. Cerebral magnetic resonanceimaging (MRI) revealed intrasellar lesions (Fig. 1a, b).The patient underwent a transnasal surgery and anothersurgery of the posterior abdominal wall neoplasm. Thepostoperative course was uneventful with partial recoverytreated with desmopressin acetate tablets.The neoplasm of the posterior abdominal wall wascharacterized by typical histological presentations of Castle-man’s disease: large follicles with intervening sheets ofplasma cells. As is well known, Castleman’sdiseaseisclassified into two histopathological types: the hyalinevascular type and the plasma cell type [1]. The plasma celltype of Castleman’s disease is less common and clinicallymore aggressive [2], which can have systemic manifestationsincluding fever, excessive sweating, fatigue, arthralgia,anemia, loss of weight, and bone marrow plasmacytosis.Histological examination of the intrasellar mass showedthe defining pathological feature of AH: the infiltration ofthe pituitary gland mainly with plasma cells, which diffusedthroughout the gland, distorting the normal architecture. OnMRI of AH, the symmetry of pituitary enhancement, thelack of erosive changes of the sellar floor, the homogeneityof the pituitary mass, and its intense enhancement aftergadolinium can be diagnostic in the proper clinical context,which could be seen in this patient’s MRI images [1]. Theclinical presentation of AH comprises four categories ofsymptoms: sellar compression, hypopituitarism, diabetesinsipidus, and hyperprolactinemia [1].As to this patient, based on previous histologicaldiagnosis of Castleman’s disease, considering the clinicalhistory, imaging features characteristic of AH, endocrinefindings and histological findings of intrasellar mass, wemade the final diagnosis as Castleman’s disease with AH.Patients with Castleman’s disease could be treated withcorticosteroid and antineoplastic chemotherapy [1, 3], butall these treatments have only demonstrated a limited effect.Appropriate management remains controversial for AH.Corticosteroid therapy has been advocated as a means ofattenuating inflammation, but such therapy does not seemjustified for most patients. Partial resection of the mass foraccurate diagnosis and to decompress the surroundingstructures is an optional surgical strategy [1].We suggest that AH should be considered in thedifferential diagnosis of any pituitary mass, especially ifpresenting with Castleman’s disease. In the absence ofsurgical emergency, medical management combined withsequential MRI is preferable.