Granulomatous hypophysitis （GRH） is extremely rare and commonly presents with chronic inflammatory of the enlarged pituitary gland. In our study, 66-year-old and 57-year-old women, both Chinese, were diagnosed with ...Granulomatous hypophysitis （GRH） is extremely rare and commonly presents with chronic inflammatory of the enlarged pituitary gland. In our study, 66-year-old and 57-year-old women, both Chinese, were diagnosed with GRH presenting preoperatively definite imageology characters as pituitary adenoma. The 66-year-old woman presented with a year of headache, half a year of gradual decrease of visual acuity, and one month of right ptosis. Serum prolaetin level was slightly elevated. Screening computed tomography （CT） scanning revealed typical low density mass found on the enlarged sella, which demonstrated invasive extension from the sella to the right cavernous sinus by contrast enhanced magnetic resonance imaging （MRI）. Consequently, the patient was diagnosed with probable invasive pituitary adenoma. The other 57-year-old woman complained a light headache and had been previously treated as nonfunctional pituitary adenoma in other hospital. Finally these two patients underwent transsphenoidal microsurgery and were diagnosed with GRH according to postoperative histopathology. They then were treated with steroid. During the follow-up, the clinical symptoms such as headache, visual damage, and ptosis vanished, and the mass of the sellae dramatically shrank on repeated MR images. Clinically and radiologically, GRH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma. Trans-sphenoidal surgery can decompress the optical nerve or oculomotornerve as a therapeutic strategy, and support biopsy or further pathological diagnosis. However, the hormonal therapy should be emphasized both as diagnostic and therapeutic strategies. Conservative and tentative steroid treatment should be performed in preoperative period without acute nerve damage.展开更多
BACKGROUND Immunoglobin G4(IgG4)-related hypophysitis(IgG4-RH)is a rare form of IgG4-related disease(IgG4-RD),which often manifests as a single organ disease and is easily misdiagnosed as a pituitary tumor clinically ...BACKGROUND Immunoglobin G4(IgG4)-related hypophysitis(IgG4-RH)is a rare form of IgG4-related disease(IgG4-RD),which often manifests as a single organ disease and is easily misdiagnosed as a pituitary tumor clinically and by imaging.There are few reports of imaging findings of IgG4-RH.Therefore,we describe a case of IgG4-RH,which mimicked a pituitary macroadenoma,that was detected by computed tomography(CT)and magnetic resonance imaging(MRI),and review the previous literature in order to further the understanding of IgG4-RH.CASE SUMMARY A 47-year-old man presented with a history of blurred vision for more than 2 mo,without other symptoms.A preoperative unenhanced CT scan revealed a slightly hyperdense mass in the sellar region measuring 2.5 cm×2.3 cm×1.8 cm,with a CT value of 45 HU.T1-weighted imaging(T1WI)and T2-weighted imaging showed iso-hypointensity,and gadolinium contrast-enhanced T1WI showed obvious homogeneous enhancement.The MRI revealed involvement of the pituitary gland and stalk.Preoperative laboratory tests revealed abnormal pituitary hormone levels,including an increased prolactin level,and decreased levels of insulin-like growth factor,dehydroepiandrosterone,and testosterone.The lesion was surgically resected.Postoperative histopathological examination of a tissue sample and an elevated serum IgG4 level confirmed the diagnosis of IgG4-RH.The patient was treated with cortisone acetate postoperatively and made a good recovery without developing any neurological deficit.CONCLUSION An elevated serum IgG4 concentration is the main clue for diagnosis of IgG4-RD.Imaging combined with laboratory testing is useful for preoperative diagnosis of IgG4-RH.展开更多
BACKGROUND Lymphocytic hypophysitis(LYH)is an important condition to consider in the differential diagnosis of patients with a pituitary mass.The main clinical manifestations of LYH include headache,symptoms related t...BACKGROUND Lymphocytic hypophysitis(LYH)is an important condition to consider in the differential diagnosis of patients with a pituitary mass.The main clinical manifestations of LYH include headache,symptoms related to sellar compression,hypopituitarism,diabetes insipidus and hyperprolactinemia.Headache,which is a frequent complaint of patients with LYH,is thought to be related to the occupying effect of the pituitary mass and is rapidly resolved with a good outcome after timely and adequate glucocorticoid treatment or surgery.CASE SUMMARY Here,we report a patient with LYH whose initial symptom was headache and whose pituitary function assessment showed the presence of secondary hypoadrenalism,central hypothyroidism and hypogonadotropic hypogonadism.Pituitary magnetic resonance imaging showed symmetrical enlargement of the pituitary gland with suprasellar extension in a dumbbell shape with significant homogeneous enhancement after gadolinium enhancement.The size of the gland was approximately 17.7 mm×14.3 mm×13.8 mm.The pituitary stalk was thickened without deviation,and there was an elevation of the optimal crossing.The lesion grew bilaterally toward the cavernous sinuses,and the parasternal dural caudal sign was visible.The patient presented with repeatedly worsening and prolonged headaches three times even though the hypopituitarism had fully resolved after glucocorticoid treatment during this course.CONCLUSION This rare headache regression suggests that patients with chronic headaches should also be alerted to the possibility of LYH.展开更多
BACKGROUND Hypophysitis induced by programmed cell death 1 protein(PD-1)immune checkpoint inhibitors is rare and poorly described.We report three patients with non-small cell lung cancer who developed hypophysitis aft...BACKGROUND Hypophysitis induced by programmed cell death 1 protein(PD-1)immune checkpoint inhibitors is rare and poorly described.We report three patients with non-small cell lung cancer who developed hypophysitis after anti-PD-1 immunotherapy.CASE SUMMARY Both case 1 and case 2 presented with common symptoms of fatigue,nausea,and vomiting.However,case 3 showed rare acute severe symptoms such as hoarse voice,bucking,and difficulty in breathing even when sitting.Following two cycles of immunotherapy in case 3,the above severe symptoms and pituitary gland enlargement were found on magnetic resonance imaging at the onset of hypophysitis.These symptoms were relieved after 10 d of steroid treatment.Case 3 was the first patient with these specific symptoms,which provided a new insight into the diagnosis of hypophysitis.In addition,we found that the clinical prognosis of patients with hypophysitis was related to the dose of steroid therapy.Case 3 was treated with high-dose hormone therapy and her pituitary-corticotropic axis dysfunction returned to normal after more than 6 mo of steroid treatment.Cases 1 and 2 were treated with the low-dose hormone,and dysfunction of the pituitary-corticotropic axis was still present after up to 7 mo of steroid treatment.CONCLUSION The clinical symptoms described in this study provide a valuable reference for the diagnosis and treatment of immune-related hypophysitis.展开更多
A 39-year-old woman consulted in the postpartum period because of agalactia, polyuria, polydipsia, and headache and decreased visual acuity. The initial analysis was compatible with a panhypopituitarism. The magnetic ...A 39-year-old woman consulted in the postpartum period because of agalactia, polyuria, polydipsia, and headache and decreased visual acuity. The initial analysis was compatible with a panhypopituitarism. The magnetic resonance (MRI) showed an expansive sellar and suprasellar lesion of high density in the basal study and enhancement after contrast administration, in contact with the optic chiasm, so urgent surgery was indicated. While waiting for the surgery, intravenous corticosteroids were initiated and the symptoms rapidly resolved. Most of the pituitary axes were recovered, except the diabetes insipidus. The control MRI showed complete resolution of the sellar mass. The surgery was canceled and the diagnosis of lymphocyte hypohpyisitis was made. Lymphocyte hypophysitis is an uncommon disease in which inflammation of the pituitary gland occurs. This results in a permanent or transient hormonal insufficiency. Lymphocyte hypophysitis is a very rare condition that occurs preferentially in women in the peripartum period. Early initiation of high doses of systemic corticosteroids may avoid unnecessary surgery in some cases.展开更多
In this study, we selected adult normal pituitary gland tissues from six patients during operations for pituitary microadenomas via the transsphenoidal approach for extended normal pituitary tissue resection around th...In this study, we selected adult normal pituitary gland tissues from six patients during operations for pituitary microadenomas via the transsphenoidal approach for extended normal pituitary tissue resection around the tumor, and analyzed the protein expression of human normal pituitary using two-dimensional high-performance liquid chromatography combined with LTQ-Orbitrap mass spectrometry proteomics technology. The ten most highly expressed proteins in normal human pituitary were： alpha 3 type VI collagen isoform 5 precursor （abundance among tall pituitary proteins 1.30%）, fibrinogen beta chain preproprotein （0.99%）, vimentin （0.73%）, prolactin （0.69%）, ATP synthase, H~ transporting and mitochondrial F1 complex beta subunit precursor （0.52%）, keratin I （0.49%）, growth hormone （0.45%）, carbonic anhydrase I （0.40%）, heat shock protein 90 kDa I （0.31%）, and annexin V （0.30%）. Based on the biological function classifications of these proteins, the top three categories by content were neuroendocrine proteins （abundance among all pituitary proteins, 40.1%）, catalytic and metabolic proteins （28.3%）, and cell signal transduction proteins （9.8%）. Based on cell positioning classification, the top three categories were cell organelle （24.5%） membrane （20.8%）, and cytoplasm （13.0%）. Based on biological process classification, the top three categories of proteins are involved in physiological processes （42.9%）, cellular processes （40.4%）, and regulation of biological processes （9.1%）. Our experimental findings indicate that a protein expression profile database of normal human pituitary can be precisely and efficiently established by proteomics technology.展开更多
A 68-year-old female patient with previous history of transsphenoidal hypophysectomy operation underwent three-vessel coronary artery bypass graft (CABG) surgery for extensive coronary artery disease. Preoperative neu...A 68-year-old female patient with previous history of transsphenoidal hypophysectomy operation underwent three-vessel coronary artery bypass graft (CABG) surgery for extensive coronary artery disease. Preoperative neurological examination revealed sequelae visual loss at right temporal visual field. Follow-up Magnetic Resonance Imaging studies showed a residual hypophyseal tumor tissue extending to suprasellar area. No additional pathology was detected in the early postoperative cranial control CT, but aggravation of visual field defect was determined. Coincidently, cranial magnetic resonance imaging (MRI) showed hemorrhage into the tumor tissue. We decided to follow-up the patient who exhibited no additional symptoms and was discharged well on the fifth day due to the signs of resolution of hemorrhage. Follow-up controls of the patient at sixth and twelfth months demonstrated normal hormone levels without any additional clinical complaints. We present preoperative assessment, perioperative anesthesia management, and postoperative clinical follow-up of a patient with a residual hypophyseal tumor.展开更多
Immune checkpoint inhibition against advance malignancies was named breakthrough discovery by the science magazine in 2013.In numerous clinical studies,monoclonal antibodies against the immune checkpoints,CTLA4,PD1 an...Immune checkpoint inhibition against advance malignancies was named breakthrough discovery by the science magazine in 2013.In numerous clinical studies,monoclonal antibodies against the immune checkpoints,CTLA4,PD1 and PD1 ligand PDL1 have shown promising tumor response in different type of metastatic malignancies.The adverse events are autoimmune-related.The endocrine disorders,hypophysitis and thyroiditis are among the most common side effects associated with immune checkpoint inhibition treatment.Hypophysitis,a very rare endocrine disorder occurs in about one tenth of the patients receiving anti-CTLA4 treatment.Thyroiditis,on the other hand,is more commonly seen in patients receiving anti-PD1 treatment.In addition,both thyroiditis and hypophysitis are common in patients receiving combination treatment with anti-CTLA4 and anti-PD1 treatment.The time to onset of hypophysitis and thyroiditis is short.Most of the endocrine disorders occur within 12 weeks after initiation of the immune checkpoint inhibition therapy.Hypophysitis can manifest as total anterior pituitary hormone deficiency or isolated pituitary hormone deficiency.Diabetes insipidus is rare.TSH and gonadotropin deficiencies may be reversible but ACTH deficiency appears permanent.Thyroiditis can present as hypothyroidism or thyrotoxicosis followed by hypothyroidism.Hypothyroidism appears irreversible.Early identifying the onset of hypophysitis and thyroiditis and proper management of these endocrine disorders will improve the quality of the life and the outcome of this novel immunotherapy.展开更多
Idiopathetic central diabetes insipidus （CDI） is a heterogeneous hypothalamus-pituitary disease due to the absence or deficiency of arginine vasopressin （AVP）.
文摘Granulomatous hypophysitis （GRH） is extremely rare and commonly presents with chronic inflammatory of the enlarged pituitary gland. In our study, 66-year-old and 57-year-old women, both Chinese, were diagnosed with GRH presenting preoperatively definite imageology characters as pituitary adenoma. The 66-year-old woman presented with a year of headache, half a year of gradual decrease of visual acuity, and one month of right ptosis. Serum prolaetin level was slightly elevated. Screening computed tomography （CT） scanning revealed typical low density mass found on the enlarged sella, which demonstrated invasive extension from the sella to the right cavernous sinus by contrast enhanced magnetic resonance imaging （MRI）. Consequently, the patient was diagnosed with probable invasive pituitary adenoma. The other 57-year-old woman complained a light headache and had been previously treated as nonfunctional pituitary adenoma in other hospital. Finally these two patients underwent transsphenoidal microsurgery and were diagnosed with GRH according to postoperative histopathology. They then were treated with steroid. During the follow-up, the clinical symptoms such as headache, visual damage, and ptosis vanished, and the mass of the sellae dramatically shrank on repeated MR images. Clinically and radiologically, GRH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma. Trans-sphenoidal surgery can decompress the optical nerve or oculomotornerve as a therapeutic strategy, and support biopsy or further pathological diagnosis. However, the hormonal therapy should be emphasized both as diagnostic and therapeutic strategies. Conservative and tentative steroid treatment should be performed in preoperative period without acute nerve damage.
基金Supported by National Key R&D Program of China,No.2019YFC0120602Clinical Research Plan of SHDC,No.SHDC2020CR3020A+2 种基金Research Startup Fund of Huashan Hospital,Fudan University,No.2021QD035Shanghai Municipal Commission of Science and Technology,No.22S31905300Greater Bay Area Institute of Precision Medicine(Guangzhou),No.KCH2310094。
文摘BACKGROUND Immunoglobin G4(IgG4)-related hypophysitis(IgG4-RH)is a rare form of IgG4-related disease(IgG4-RD),which often manifests as a single organ disease and is easily misdiagnosed as a pituitary tumor clinically and by imaging.There are few reports of imaging findings of IgG4-RH.Therefore,we describe a case of IgG4-RH,which mimicked a pituitary macroadenoma,that was detected by computed tomography(CT)and magnetic resonance imaging(MRI),and review the previous literature in order to further the understanding of IgG4-RH.CASE SUMMARY A 47-year-old man presented with a history of blurred vision for more than 2 mo,without other symptoms.A preoperative unenhanced CT scan revealed a slightly hyperdense mass in the sellar region measuring 2.5 cm×2.3 cm×1.8 cm,with a CT value of 45 HU.T1-weighted imaging(T1WI)and T2-weighted imaging showed iso-hypointensity,and gadolinium contrast-enhanced T1WI showed obvious homogeneous enhancement.The MRI revealed involvement of the pituitary gland and stalk.Preoperative laboratory tests revealed abnormal pituitary hormone levels,including an increased prolactin level,and decreased levels of insulin-like growth factor,dehydroepiandrosterone,and testosterone.The lesion was surgically resected.Postoperative histopathological examination of a tissue sample and an elevated serum IgG4 level confirmed the diagnosis of IgG4-RH.The patient was treated with cortisone acetate postoperatively and made a good recovery without developing any neurological deficit.CONCLUSION An elevated serum IgG4 concentration is the main clue for diagnosis of IgG4-RD.Imaging combined with laboratory testing is useful for preoperative diagnosis of IgG4-RH.
基金Supported by the Nature Science Foundation of Jilin Province(No.2018010113JC).
文摘BACKGROUND Lymphocytic hypophysitis(LYH)is an important condition to consider in the differential diagnosis of patients with a pituitary mass.The main clinical manifestations of LYH include headache,symptoms related to sellar compression,hypopituitarism,diabetes insipidus and hyperprolactinemia.Headache,which is a frequent complaint of patients with LYH,is thought to be related to the occupying effect of the pituitary mass and is rapidly resolved with a good outcome after timely and adequate glucocorticoid treatment or surgery.CASE SUMMARY Here,we report a patient with LYH whose initial symptom was headache and whose pituitary function assessment showed the presence of secondary hypoadrenalism,central hypothyroidism and hypogonadotropic hypogonadism.Pituitary magnetic resonance imaging showed symmetrical enlargement of the pituitary gland with suprasellar extension in a dumbbell shape with significant homogeneous enhancement after gadolinium enhancement.The size of the gland was approximately 17.7 mm×14.3 mm×13.8 mm.The pituitary stalk was thickened without deviation,and there was an elevation of the optimal crossing.The lesion grew bilaterally toward the cavernous sinuses,and the parasternal dural caudal sign was visible.The patient presented with repeatedly worsening and prolonged headaches three times even though the hypopituitarism had fully resolved after glucocorticoid treatment during this course.CONCLUSION This rare headache regression suggests that patients with chronic headaches should also be alerted to the possibility of LYH.
文摘BACKGROUND Hypophysitis induced by programmed cell death 1 protein(PD-1)immune checkpoint inhibitors is rare and poorly described.We report three patients with non-small cell lung cancer who developed hypophysitis after anti-PD-1 immunotherapy.CASE SUMMARY Both case 1 and case 2 presented with common symptoms of fatigue,nausea,and vomiting.However,case 3 showed rare acute severe symptoms such as hoarse voice,bucking,and difficulty in breathing even when sitting.Following two cycles of immunotherapy in case 3,the above severe symptoms and pituitary gland enlargement were found on magnetic resonance imaging at the onset of hypophysitis.These symptoms were relieved after 10 d of steroid treatment.Case 3 was the first patient with these specific symptoms,which provided a new insight into the diagnosis of hypophysitis.In addition,we found that the clinical prognosis of patients with hypophysitis was related to the dose of steroid therapy.Case 3 was treated with high-dose hormone therapy and her pituitary-corticotropic axis dysfunction returned to normal after more than 6 mo of steroid treatment.Cases 1 and 2 were treated with the low-dose hormone,and dysfunction of the pituitary-corticotropic axis was still present after up to 7 mo of steroid treatment.CONCLUSION The clinical symptoms described in this study provide a valuable reference for the diagnosis and treatment of immune-related hypophysitis.
文摘A 39-year-old woman consulted in the postpartum period because of agalactia, polyuria, polydipsia, and headache and decreased visual acuity. The initial analysis was compatible with a panhypopituitarism. The magnetic resonance (MRI) showed an expansive sellar and suprasellar lesion of high density in the basal study and enhancement after contrast administration, in contact with the optic chiasm, so urgent surgery was indicated. While waiting for the surgery, intravenous corticosteroids were initiated and the symptoms rapidly resolved. Most of the pituitary axes were recovered, except the diabetes insipidus. The control MRI showed complete resolution of the sellar mass. The surgery was canceled and the diagnosis of lymphocyte hypohpyisitis was made. Lymphocyte hypophysitis is an uncommon disease in which inflammation of the pituitary gland occurs. This results in a permanent or transient hormonal insufficiency. Lymphocyte hypophysitis is a very rare condition that occurs preferentially in women in the peripartum period. Early initiation of high doses of systemic corticosteroids may avoid unnecessary surgery in some cases.
基金supported by the National NaturalScience Foundation of China, No. 81200890
文摘In this study, we selected adult normal pituitary gland tissues from six patients during operations for pituitary microadenomas via the transsphenoidal approach for extended normal pituitary tissue resection around the tumor, and analyzed the protein expression of human normal pituitary using two-dimensional high-performance liquid chromatography combined with LTQ-Orbitrap mass spectrometry proteomics technology. The ten most highly expressed proteins in normal human pituitary were： alpha 3 type VI collagen isoform 5 precursor （abundance among tall pituitary proteins 1.30%）, fibrinogen beta chain preproprotein （0.99%）, vimentin （0.73%）, prolactin （0.69%）, ATP synthase, H~ transporting and mitochondrial F1 complex beta subunit precursor （0.52%）, keratin I （0.49%）, growth hormone （0.45%）, carbonic anhydrase I （0.40%）, heat shock protein 90 kDa I （0.31%）, and annexin V （0.30%）. Based on the biological function classifications of these proteins, the top three categories by content were neuroendocrine proteins （abundance among all pituitary proteins, 40.1%）, catalytic and metabolic proteins （28.3%）, and cell signal transduction proteins （9.8%）. Based on cell positioning classification, the top three categories were cell organelle （24.5%） membrane （20.8%）, and cytoplasm （13.0%）. Based on biological process classification, the top three categories of proteins are involved in physiological processes （42.9%）, cellular processes （40.4%）, and regulation of biological processes （9.1%）. Our experimental findings indicate that a protein expression profile database of normal human pituitary can be precisely and efficiently established by proteomics technology.
文摘A 68-year-old female patient with previous history of transsphenoidal hypophysectomy operation underwent three-vessel coronary artery bypass graft (CABG) surgery for extensive coronary artery disease. Preoperative neurological examination revealed sequelae visual loss at right temporal visual field. Follow-up Magnetic Resonance Imaging studies showed a residual hypophyseal tumor tissue extending to suprasellar area. No additional pathology was detected in the early postoperative cranial control CT, but aggravation of visual field defect was determined. Coincidently, cranial magnetic resonance imaging (MRI) showed hemorrhage into the tumor tissue. We decided to follow-up the patient who exhibited no additional symptoms and was discharged well on the fifth day due to the signs of resolution of hemorrhage. Follow-up controls of the patient at sixth and twelfth months demonstrated normal hormone levels without any additional clinical complaints. We present preoperative assessment, perioperative anesthesia management, and postoperative clinical follow-up of a patient with a residual hypophyseal tumor.
基金This review is supported by NICHD/NIH K08 HD070957.
文摘Immune checkpoint inhibition against advance malignancies was named breakthrough discovery by the science magazine in 2013.In numerous clinical studies,monoclonal antibodies against the immune checkpoints,CTLA4,PD1 and PD1 ligand PDL1 have shown promising tumor response in different type of metastatic malignancies.The adverse events are autoimmune-related.The endocrine disorders,hypophysitis and thyroiditis are among the most common side effects associated with immune checkpoint inhibition treatment.Hypophysitis,a very rare endocrine disorder occurs in about one tenth of the patients receiving anti-CTLA4 treatment.Thyroiditis,on the other hand,is more commonly seen in patients receiving anti-PD1 treatment.In addition,both thyroiditis and hypophysitis are common in patients receiving combination treatment with anti-CTLA4 and anti-PD1 treatment.The time to onset of hypophysitis and thyroiditis is short.Most of the endocrine disorders occur within 12 weeks after initiation of the immune checkpoint inhibition therapy.Hypophysitis can manifest as total anterior pituitary hormone deficiency or isolated pituitary hormone deficiency.Diabetes insipidus is rare.TSH and gonadotropin deficiencies may be reversible but ACTH deficiency appears permanent.Thyroiditis can present as hypothyroidism or thyrotoxicosis followed by hypothyroidism.Hypothyroidism appears irreversible.Early identifying the onset of hypophysitis and thyroiditis and proper management of these endocrine disorders will improve the quality of the life and the outcome of this novel immunotherapy.
文摘Idiopathetic central diabetes insipidus （CDI） is a heterogeneous hypothalamus-pituitary disease due to the absence or deficiency of arginine vasopressin （AVP）.
