垂体脓肿三例报道

垂体脓肿是一种罕见疾病,具有致命性,以头痛、视力障碍和垂体功能减退为主要表现。垂体脓肿在MRI上表现为蝶鞍区可见圆囊性病变,T1像为低或等信号,T2像为高或等信号,增强时环形强化。手术发现囊肿包裹脓液时可确诊。治疗主要经蝶窦手术联合抗生素抗感染,在特定条件下可行开颅手术。现在报道本院3例垂体脓肿,并进行文献复习。     

A case of anterior hypopituitarism showing recurrent pituitary mass associated with central diabetes insipidus

We report a case of anterior hypopituitarism showing recurrent pituitary mass associated with central diabetes insipidus. A 76-year old woman was hospitalized with general fatigue and 5 kg body weight loss. Endocrinological examinations and pituitary provocative tests demonstrated hypopituitarism and central diabetes insipidus. T1-weighted image of magnetic resonance imaging (MRI) revealed an intrasellar cystic mass with ring enhancement suggesting pituitary abscess. MRI films subsequently obtained from another hospital and studied retrospectively showed intrasellar cystic mass with ring enhancement 4 years earlier, and a mass shape that was decreased after 2 years. Over the subsequent years, the patient has remained asymptomatic with hormone replacement therapy only. Cystic pituitary adenoma or Rathke's cleft cyst with repeated infection may be involved in the repeated change of pituitary mass shape although neither pituitary surgery nor a pituitary biopsy was performed because of the patient's age and condition. It is reported that apparent recurrence of Rathke's cleft cysts after initially successful surgery was higher than suggested by previous reports, and that long-term follow-up with pituitary imaging and neuroophthalmological assessment is essential. Careful evaluation by follow-up brain MRI is needed in the present case to prevent future recurrence of pituitary abscess.     

自身免疫性垂体炎的研究进展

自身免疫性垂体炎是一种罕见的内分泌疾病，多发生于妊娠或产后。其发病机制不明，临床表现多变，主要包括垂体压迫、腺垂体功能减退症、尿崩症和高催乳素血症等相关症状。治疗目的主要是减小垂体肿块的大小和（或）替代有缺陷的内分泌功能。多数患者需要长期使用激素替代治疗。     

Pituitary abscess presenting with cranial nerve paresis. Case report and review of literature

Non-adenomatosus lesions of the pituitary represent a small part of the intrasellar processes and they have heterogeneous presentation. Making a precise diagnosis is of great importance, as it may lead to more efficient management. A 65-year-old man was admitted to the hospital because of headache and right cranial nerve III palsy. Basic laboratory work-up was normal whereas endocrinological assessment revealed hypopituitarism without diabetes insipidus. Plain radiography showed an enlarged sella and frontal and paranasal sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) of the sella revealed an intrasellar lesion with extension to the sphenoid and cavernous sinuses as well as the suprasellar region, exerting pressure on the optic chiasm. On T1-weighted images the mass had a low-intensity signal with a smooth enhancing rim with bright signal. Given the presence of multiple sinusitis and imaging characteristics a pre-operative diagnosis of pituitary abscess was made. The patient was operated via transphenoidal route and purulent material was drained out. Cultures of the material were positive for Staphylococcus aureus. Antibiotics as well as cortisol replacement therapy were given. Three months later hypopituitarism persisted but there was significant improvement in the neurological findings. We report a case of an unusual presentation of a pituitary abscess. High index of suspicion, the presence of associated conditions such as pituitary tumors, meningitis or sinusitis, as well as diabetes insipidus and specific imaging features are the main diagnostic clues. Pre-operative diagnosis, which will lead to prompt antibiotic therapy and transphenoidal drainage, can decrease high mortality and morbidity associated with this disease.     

Magnetic resonance imaging and computerized tomography images in a case of pituitary abscess

A 29-year-old male patient with clinical manifestations of panhypopituitarism and diabetes insipidus is presented. The clinical and laboratory evaluation of the pituitary reserve confirmed a total pituitary insufficiency. Computed tomography (CT) scan demonstrated a low-density lesion suggesting a pituitary tumor with suprasellar extension, and magnetic resonance imaging (MRI) revealed a pituitary mass with decreased signal intensity on T1-weighted images and capsular contrast enhancement after gadolinium injection. The surgical procedure was the treatment of choice. During surgery a large amount of purulent material was removed. With light microscopy, chronic non-specific inflammation and a pituitary abscess capsule were demonstrated in the tissue. The results of the cultures revealed coagulase negative staphylococcus. The post-operative course was uneventful and, as the patient was pituitary-deficient, he was on replacement therapy and was being followed-up. Pituitary abscess is an exceptional lesion in the literature and despite the advent of CT and MRI, its preoperative diagnosis still remains difficult. However, the presence of an intrasellar expansive process with liquid center and contrast enhanced outline should suggest the possibility of an abscess. Sellar round cystic mass isointense or hypointense to grey matter on T1, high intensity signal on T2, or opposite pattern with a peripheral rim enhancement following gadolinium injection, and diabetes insipidus may all be suggestive of a pituitary abscess.     

A case of cystic lymphocytic hypophysitis with cacosmia and hypopituitarism

Lymphocytic hypophysitis is a rare inflammatory disease of the pituitary gland that is being increasingly recognized as a cause of hypopituitarism. This condition may be due to an autoimmune pituitary destruction which usually occurs in young women during pregnancy or in the immediate postpartum period. We describe a case of cystic pituitary mass in a thirty-eight year-old woman presenting with nausea, vomiting, cold intolerance, blurring of vision and the presence of disagreeable odors for a one-month period. She had secondary amenorrhea and galactorrhea for three months. Combined anterior pituitary stimulation test confirmed the diagnosis of hypopituitarism. Magnetic resonance imaging scan with enhancement showed a huge cystic sellar mass with suprasellar extension and thickening of the pituitary stalk. Transsphenoidal exploration was performed with preoperative diagnosis of pituitary macroadenoma with cystic necrosis. Histological examination revealed lymphocytic hypophysitis characteristic of diffuse, dense lymphocytes and plasma cells infiltration with surrounding interstitial reactive fibrosis. Postoperatively, the patient's olfactory function returned to normal but combined anterior pituitary stimulation test showed persistence of hypopituitarism with mild hyperprolactinemia. Prednisolone, thyroxine and estrogen replacements were started and clinical symptoms were much improved. In summary, we report an extremely rare case of a woman with cystic lymphocytic hypophysitis with cacosmia and hypopituitarism, confirmed by histological examination.     

伴尿崩症的鞍区颗粒细胞瘤

少年起病、伴尿崩症的颗粒细胞瘤目前国内外尚无文献报道。本文1例患者14岁,男性,以多尿为主要表现,头颅MRI示鞍区实性占位病变,术前垂体功能基本正常,曾考虑淋巴细胞垂体炎和生殖细胞瘤,分别行甲基强的松龙冲击治疗和鞍区放疗均无效果。行经蝶肿瘤切除,病理为颗粒细胞瘤。术后患者病情平稳,因垂体功能低下行替代治疗。     

Panhypopituitarism due to Wegener's granulomatosis

Wegener's granulomatosis (WG) is a multi-system necrotizing granulomatous vasculitis which classically affects the upper respiratory tract, lungs and kidneys. Pituitary participation has been described in 24 patients in the literature to date. The aim of this article is to report a case of pituitary involvement in WG, and to present a literature review on this association. We present a female patient with WG who evolved with central diabetes insipidus (CDI), panhypopituitarism, and mild hyperprolactinemia. MRI showed an infiltrative pattern. Pituitary involvement has been reported in around 1% of patients with WG, mostly in women. It is represented by CDI and hypopituitarism. MRI generally shows pituitary enlargement, stalk thickening and loss of hyperintensity of the neurohypophysis. Permanent endocrine therapy is generally needed. WG should be considered in cases of CDI and hypopituitarism, essentially if a vasculitis is suspected and more common sellar disorders have been ruled out.     

Primary pituitary lymphoma in immunocompetent patient: diagnostic problems and prolonged follow-up

Primary pituitary lymphoma in immunocompetent patients is a rare disease and has been described in less than 20 cases. Moreover, low-grade lymphomas constitute only 3% of all primary central nervous system lymphoma. The objective of this report is to report a low-grade primary pituitary lymphoma, diagnostic problems and to give more evidence about the evolution of this rare disease. A 49 y.o. woman was referred to our clinic with symptoms of hypopituitarism. A diagnostic work-up showed mild anemia, an erythrocyte sedimentation rate of 122 mm/h and a negative Elisa test for HIV. Panhypopituitarism was confirmed and the MRI showed a 20 mm sellar and suprasellar enhancing mass with a thickening of the pituitary stalk, chiasmal compression and bitemporal hemianopsia. She underwent transsphenoidal resection only 10 months later for non medical reasons. During this period she was clinically asymptomatic on hormonal replacement therapy. A new MRI showed regression of the suprasellar extension and invasion to the left cavernous sinus. A firm and infiltrative mass was found during transsphenoidal surgery, and only partial resection was performed. Biopsy showed a low-grade lymphoplasmacytic lymphoma. Staging was negative for other localizations. She was given chemotherapy and localized radiotherapy. Four years after surgery, the sellar MRI showed a 10 mm residual sellar mass with the persistence of a cavernous sinus invasion and she is considered to be in remission. The neurosurgeon and clinician should consider primary pituitary lymphoma as a potential cause of a sellar mass, especially in the presence of diabetes insipidus and an enhancing invasive mass. Neurosurgical biopsy is crucial for a correct diagnosis and prognosis could be better than classic CNS primary lymphomas.     

Tumors metastatic to the pituitary gland: case report and literature review

Tumors metastatic to the pituitary gland are an unusual complication of systemic cancer typically seen in elderly patients with diffuse malignant disease. Breast and lung are the commonest sites of the primary tumor, whereas diabetes insipidus is the most frequent symptom at presentation. Their rarity and usually indolent course, as well as the lack of specific clinical and radiological features, impede their differentiation from other more common sellar area lesions, particularly when history of malignancy is absent. Management of these patients may also be very difficult because the prognosis depends on the course of the primary neoplasm. A 68-yr-old man, with no history of malignancy, presented with recent onset of hypopituitarism, mild diabetes insipidus, headaches, left oculomotor nerve palsy, and progressive bilateral deterioration of visual acuity and visual fields. Magnetic resonance imaging revealed a large sellar mass compressing the optic chiasm and invading the left cavernous sinus, whereas a prolactin elevation at 438.6 ng/ml (19.73 nmol/liter) was noted. Decompression of the sellar region was attempted, and pathology disclosed a metastatic hepatocellular carcinoma. On postoperative investigation, primary liver tumor was identified and confirmed by biopsy. The patient improved transiently but died 3 months after diagnosis because of deterioration of the liver disease. The relevant literature is reviewed in light of this unusual case, illustrating the problems in the diagnosis and management of patients with metastasis to the pituitary.     

Pituitary abscess: our experience with a case and a review of the literature

Pituitary abscess is a rare disease. Due to its potentially high mortality and morbidity rate, it should be considered in the differential diagnosis of sellar masses. Despite recent advances in radiological investigations, making a definitive diagnosis preoperatively remains challenging. This 45-year-old Indian gentleman presented with severe throbbing headache for six months. Magnetic resonance imaging of the brain revealed a sellar mass with peripheral enhancement that was misdiagnosed as a pituitary macroadenoma. A trans-sphenoidal excision was done, which surprisingly yielded only pus that was drained accordingly from the lesion. Fungal and bacterial cultures were negative. However this patient subsequently lost complete function of the pituitary gland, resulting in central diabetes insipidus and required permanent hormonal replacement therapy in the deficient endocrine axes. A Medline search was conducted and a review of 24 cases reported in the last 5 years discussed.     

Metastatic tumors of the sella turcica masquerading as primary pituitary tumors

Tumors metastatic to the pituitary gland are uncommon, but may mimic a typical pituitary adenoma and are an important part of the differential diagnosis of sellar mass lesions. Pituitary metastases were treated by transsphenoidal microsurgical removal in 14 patients. Most tumors appeared in men in the sixth and seventh decades of life. Half of the patients presented with visual loss, 6 had anterior hypopituitarism, and 4 had diabetes insipidus. Primary cancer had been diagnosed and treated previously in 5 patients. In the remaining 9 patients, the transsphenoidal operation provided the initial diagnosis of cancer, and primary lesions subsequently were detected in all but 2. Transsphenoidal surgery also provided satisfactory decompression of the mass effect related to the tumors, improving the presenting symptoms in the majority of patients, and the surgery was free of mortality or serious complications.     

Primary pituitary abscess followed by empty sella syndrome in an adolescent girl

Primary pituitary abscess is a rare pituitary pathology, particularly at a young age and is characterized by atypical clinical features making the diagnosis difficult. Correct diagnosis and therapy are mandatory due to the potentially lethal outcome of pituitary infection. We report the case of an adolescent girl presenting with headache, diabetes insipidus and central thyro-gonadic insufficiency with no history of infection, in whom the intra-operative diagnosis of primary pituitary abscess was made. Bacterial cultures indicated infection with Streptococcus spp. One year after neurosurgery and antibiotic therapy, recovery of diabetes insipidus and pituitary insufficiency was documented except for persistence of subnormal growth hormone secretion. Post-surgery, pituitary magnetic resonance imaging revealed an empty sella syndrome.     

Primary pituitary tubercular abscess mimicking as pituitary adenoma

Tubercular abscess of the pituitary fossa is rare and may lead to diagnostic uncertainty in a patient with absence of tuberculosis elsewhere in the body. We present a rare case report of a young lady who presented with sellar and suprasellar cystic mass. She was diagnosed as a case of pituitary macroadenoma and was intraoperatively found to harbor pus in the lesion. She did not have any symptoms of infection. The case underlines the importance of considering such a possibility in the differential diagnosis of cystic sellar lesions and further diagnostic tests should be done for confirmation and treatment of this rare and potential life threatening illness.     

Pituitary tuberculoma: an unusual lesion of sellar region

Pituitary tuberculomas are extremely rare lesions, with only few cases described in the literature, usually mistaken as pituitary tumors. Its heterogeneous clinical and imaging profile preclude preoperative diagnosis which ultimately relies on the histopathological examination. We describe a 46 years old woman who presented with an episode of confusion and hypopituitarism with no evidence of systemic tuberculosis. Computed tomography (CT) showed a central calcified and enhancing sellar mass. Magnetic resonance imaging (MRI) showed a sellar mass with suprasellar extension and associated optic chiasm compression. She was submitted a craniotomy for biopsy and resection. A caseous material was found at the center of the lesion involved by a thick wall. Due to the wall adherence to the optic chiasm and the inflammatory aspects of the lesion, subtotal removal was achieved and the patient followed on anti-tuberculous and hormonal replacement therapy. Sellar tuberculomas should be considered in the differential diagnosis of sellar tumors in order to offer appropriate treatment.     

Hypopituitarism in older adults. The report of five cases with different presentations

Patients with hypopituitarism may present with myriad signs and symptoms which result from reduced secretion of one or more pituitary hormones. Severe hyponatremia and hypoglycemia are rare, but potentially life threatening presenting clinical features of hypopituitarism. As these abnormalities are completely restored with replacement therapy and patients are often undiagnosed until admission, a high clinical suspicion is necessary to diagnose a patient with hypopituitarism. We present five cases, each exhibiting rare symptoms of hypopituitarism--severe hyponatremia, hypoglycemia and osteoporosis--as the only presenting feature; these cases illustrate that hypopituitarism must be considered in the differential diagnosis of these abnormalities, especially in older patients.     

Waxing and waning of a pituitary mass in a young woman with combined pituitary hormone deficiency (CPHD) due to a PROP-1 mutation

We describe a 23-yr old woman with congenital combined pituitary hormone deficiency (CPHD) diagnosed at 10 years of age and a large sellar mass discovered at the age of 19 years, when her first pituitary MRI was performed. The mass (height: 13 mm) extended to the suprasellar region, close to the optic chiasm, showed signal hyperintensity in T1- and hypointensity in T2-weighted images, with no enhancement after gadolinium injection. Although these MRI features were suggestive of Rathke’s cleft cyst, cystic craniopharyngioma or previous hemorrhage, no visual symptoms, diabetes insipidus and/or hyperprolactinemia were present. In addition, similar MRI findings had been previously described in a few cases of CPHD due to PROP-1 mutations, which prompted us to carry out a molecular study before any therapeutic decision was made. A 301302delAG PROP-1 mutation was found in her DNA and the patient was closely followed through ophthalmologic evaluation and pituitary MRI scans. During a 3.6-year follow-up, we were able to document a marked initial growth followed by shrinkage and recurrent growth of the PROP-1 sellar mass. The patient remains free of compressive neuro-ophthalmological signs, suggesting that surgical intervention is unnecessary in these cases. However, they must be followed closely with sellar MRIs and campimetry until the mass completely regresses.     

A case of lymphocytic infundibuloneurohypophysitis showing diabetes insipidus followed by anterior hypopituitarism associated with thrombasthenia

We report a case of a 42-year old male patient with diabetes insipidus followed by anterior hypopituitarism associated with thrombasthenia. The patient had been diagnosed with thrombasthenia since the age of 19. He was admitted and diagnosed as diabetes insipidus in 1995. Although T1-weighted image of magnetic resonance imaging (MRI) showed empty sella and partial pituitary stalk hypertrophy, the anterior pituitary functions were normal at that time. Three years later, he was re-admitted after an episode of general malaise and impotence in 1998. Endocrinological studies revealed adrenal insufficiency, hypothyroidism and hypogonadism. T1-weighted image of MRI demonstrated the thickening of pituitary stalk and neurohypophysis. Analysis of anti-pituitary antibodies by immunoblotting identified a major band at 61.5 kDa. The diabetes insipidus was controlled by desmopressin acetate and the shrinkage of pituitary stalk was seen after hormonal replacement therapy including glucocorticoid and thyroid hormone. We suggested that this case represented lymphocytic infundibuloneurohypophysitis, in which a chronic inflammatory process occurred in infundibulum and/or neurohypophysis and that hypopituitarism developed possibly due to damage to the pituitary portal vessels caused by a thickened pituitary stalk, although a pituitary biopsy was not done because of the risk of bleeding in thrombasthenia. The pituitary autoantibodies in sera from patients with hypopituitarism may be helpful to characterize the patient with lymphocytic hypophysitis.     

鞍区肿瘤术后内分泌代谢紊乱的防治和管理

随着神经外科经鼻蝶内镜技术的进步，鞍区肿瘤手术全切率和安全性已明显提高。但由于肿瘤类型、大小、生物特性多变，且鞍区位置特殊，术中有时难免损伤下丘脑、垂体，造成垂体内分泌和水、盐代谢紊乱，导致患者术后出现记忆力下降、生长发育停滞、不孕不育、肥胖、多尿、电解质紊乱等，并且医患存在对下丘脑、垂体和靶腺激素替代认识不足，治疗不规范，随访不积极，严重时危及生命。本文从内分泌专科医生角度，详细阐述鞍区肿瘤术后垂体功能减退、下丘脑综合征及水、盐代谢紊乱的防治和管理，希望通过本文规范激素替代治疗，促进患者垂体功能重建，改善患者预后，提高远期生活质量，减少死亡率。     

Idiopathic giant-cell granulomatous hypophysitis mimicking acute meningitis.

A 32-year-old woman presented with severe headache, photophobia, fever, nausea, vomiting, and worsening vision. She had also noted several months of amenorrhea. She was febrile to 38.9 degrees C. Laboratory evaluation revealed a markedly elevated erythrocyte sedimentation rate. Lumbar puncture revealed a cerebrospinal fluid lymphocytic pleocytosis and an elevated protein level. Endocrine studies revealed evidence of panhypopituitarism without diabetes insipidus. A magnetic resonance imaging study showed a 2-cm pituitary mass with optic chiasmal compression. The patient had a trans-sphenoidal resection of the mass. Pathology revealed multinucleated giant cells in necrotic debris, but no evidence of pituitary tumor. Studies looking for evidence of systemic granulomatous disease were negative. The patient was considered to have idiopathic giant-cell granulomatous hypophysitis. After surgery, the patient's vision improved and hormone replacement therapy was initiated. This case illustrates that idiopathic giant-cell granulomatous hypophysitis should be considered in the differential diagnosis of a patient presenting with a pituitary mass, hypopituitarism, and meningitis-like symptoms.