Lymphocytic hypophysitis successfully treated with azathioprine: first case report

An aggressive case of lymphocytic hypophysitis is described which was successfully treated with azathioprine after failure of corticosteroids. The patient, aged 53, had frontal headache, diplopia, and diabetes insipidus. Cranial magnetic resonance imaging (MRI) showed an intrasellar and suprasellar contrast enhancing mass with involvement of the left cavernous sinus and an enlarged pituitary stalk. A putative diagnosis of lymphocytic hypophysitis was made and prednisone was prescribed. Symptoms improved but recurred after the dose was reduced. Trans-sphenoidal surgery was attempted but the suprasellar portion of the mass could not be pulled through the pituitary fossa. Histological examination confirmed the diagnosis of lymphocytic hypophysitis. Two months later he developed aseptic meningoencephalitis which was treated with high dose methylprednisolone pulse therapy. MRI revealed a progression of suprasellar mass. At this stage azathioprine treatment was begun. Four weeks later MRI shown no evidence of residual lesion and no pituitary stalk enlargement. After follow up of 18 months without azathioprine there was no clinical or radiological evidence of the disease. This is the first evidence of the efficacy of azathioprine treatment in a patient with lymphocytic hypophysitis.     

A case of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome]

A 48-year-old female was seen because of left orbital pain. The neurological findings were normal at her first visit. She presented temporary double vision during conservative period. Plain CT revealed no mass around the sellar region. Enhanced CT revealed enhanced mass in the left cavernous sinus. MRI revealed low intensity lesion on both T1 and T2 weighted images. Enhanced MRI showed strongly enhanced mass extended from the left cavernous sinus to the dura of sellar floor, the contralateral cavernous sinus, and cerebellar tentorium. Angiography showed stenosis of the left internal cerebral artery. Biopsy from the dura of the tentorium was performed via anterior temporal approach. Abundant collagen fibers with hyalinization were seen in the thickened dura. A final diagnosis of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome was made. Symptoms were free just after the surgery, however, the regrowth of the tentorial lesion was found on MRI after one year. The size of the lesion decreased after administration of steroid.     

Diagnostic value of super-selective bilateral cavernous sinus sampling with hypothalamic stimulating hormone loading in patients with ACTH-producing pituitary adenoma

AIMS: Early diagnosis and early treatment by transsphenoidal surgery is desirable for ACTH-producing pituitary microadenoma, but accurate localization of the functional lesion is not always possible before surgery because magnetic resonance (MR) imaging may provide false negative and/or positive findings. The diagnostic value of super-selective bilateral cavernous sinus sampling with the administration of corticotropin-releasing hormone (CRH) was assessed in patients with functioning ACTH-producing pituitary adenoma. METHODS: Fifteen patients with pituitary adenoma (14 with microadenoma) aged from 23 to 74 years (mean 46.7 years) underwent cavernous sinus sampling with or without the CRH loading test and subsequent transsphenoidal surgery in our institute from October 1997 through to November 2002. MR imaging including dynamic scan failed to detect the adenomatous lesion in all patients. To eliminate the bias due to uneven blood flow in the cavernous sinuses and the multi-hormonal response to CRH administration, the ACTH/FSH ratios were evaluated. The inter-cavernous gradient (ICG) was calculated as the higher/lower ACTH venous blood levels in the right and left cavernous sinuses with or without CRH loading. The adjusted ICG was calculated using the ACTH/FSH ratios. The results were compared with the surgical findings. An ICG of 1.4 or greater was considered to indicate the localization of the responsible lesion. RESULTS: Transsphenoidal surgery revealed the functioning lesion on the right in five cases, the left in six, the midline in three and the bilateral lateral wings (double adenoma) in one. Adjusted ICG with CRH loading had a localization accuracy of 93.3% (14/15), which was significantly higher than that of 73.3% (11/15) using ICG without hypothalamic stimulating hormone loading (p = 0.0402). CONCLUSIONS: Super-selective cavernous sinus sampling with hypothalamic stimulating hormone administration can provide accurate localization of the responsible lesion in patients with ACTH-producing pituitary adenoma.     

Biopsy proven pituitary sarcoidosis presenting as a possible adenoma

Sarcoidosis is a well-recognized systemic granulomatous process which involves the central nervous system in 5–15% of patients. One of the more frequent sites of central nervous system involvement is the pituitary and hypothalamic region. Involvement of the sellar region by sarcoidosis is overall an infrequent occurrence, comprising less than 1% of all intrasellar lesions. Patients typically present with an infiltrative lesion on imaging studies and clinically with symptoms related to diabetes insipidus or hyperprolactinemia. This report describes a 38-year-old woman who initially presented with a variety of symptoms including headaches, light sensitivity, nausea and vomiting, acute visual changes, cold intolerance, amenorrhea, decreased libido, fatigue and galactorrhea. She had an elevated serum prolactin level and evidence of oligoclonal bands in the cerebrospinal fluid. Imaging studies discovered a 1.8 cm mass involving the pituitary gland and compressing the optic chiasm. The lesion was excised and microscopically was marked by a chronic inflammatory cell infiltrate and scattered nonnecrotizing granulomas. Stains and microbiologic cultures failed to demonstrate microorganisms. There was no evidence of other organ involvement on postoperative imaging. She was treated with prednisone with improvement of symptoms and subsequently required methotrexate to treat left eye pain and blurred vision, 29 months after her surgery. Achieving treatment control in patients with pituitary and hypothalamic improvement in sarcoidosis still remains a challenge.     

Case report of spontaneous cervical epidural hematoma in elderly]

Spontaneous spinal epidural hematomas (SSEH) are relatively rare clinical entities and associated with coagulopathies, tumors, or vascular malformation. In addition, these are often neurosurgical emergencies; therefore, prompt diagnosis and treatment are paramount. We reported a case of an 87-year-old woman with spontaneous cervical epidural hematoma. She presented with the sudden onset of neck pain, rt. upper arm sensory disturbance and rt. hemiparesis. MRI revealed a C3-Th1 dorsally placed extradural lesion. The lesion was iso-to hypointense on T1-weighted images and was hyperintense on T2-weighted images. She underwent surgery within 9 hours after symptom onset, removal of hematoma through the right hemilaminectomy was performed. She was a very high age, but she was discharged from the hospital without neurological deficit. To obtain good prognosis for patients with SSEH, early diagnosis and treatment are important. We also review the current literature concerning diagnosis and treatment of SSEH.     

海绵窦间隙在侵袭海绵窦垂体腺瘤神经内镜经鼻蝶入路切除术中的应用

目的 探讨海绵窦间隙在侵袭海绵窦垂体腺瘤神经内镜经鼻蝶入路切除术中的应用价值。方法 回顾性分析2017年1月至2020年10月经鼻蝶入路神经内镜手术治疗的15例侵袭海绵窦垂体腺瘤的临床资料。基于颈内动脉的自然走形,把海绵窦分为上、下、后、外侧间隙,术中对不同间隙内肿瘤采用不同切除方法。结果 肿瘤全切除11例,次全切除4例。术后发生脑脊液鼻漏1例,尿崩6例,垂体功能减退危象1例,眼球外展障碍1例,动眼神经麻痹1例。15例术后随访3~36个月;11例肿瘤全切除中,10例无复发,1例无功能垂体腺瘤复发并动态观察;4例次全切除中,2例无进展;2例术后6个月内行伽玛刀治疗。结论 神经内镜下经鼻入路手术切除侵袭海绵窦垂体腺瘤的效果良好,基于颈内动脉的自然走形的海绵窦间隙划分方法为侵袭海绵窦垂体腺瘤的手术治疗提供了新思路。     

Cerebral venous thrombosis: a young woman case study

Cerebral venous sinus thrombosis is a rare, serious cerebrovascular disease with poor prognosis. It can be a sequel to various coagulation disturbances, head injuries or local inflammations. We report a case of a young woman with no risk factors detected, who developed a massive cerebral venous sinus thrombosis. She had progressively worsening symptoms, including left hemiplegia, aphasia, tonic-clonic seizures and unconsciousness. The diagnosis was supported by CT, MRI and angio-MRI findings. The intensive i.v. heparin and streptokinase treatment, as well as antibiotics, resulted in full remission of all patient's symptoms. The case emphasizes the necessity of early diagnosis and management of cerebral venous sinus thrombosis.     

Pituitary abscess in a pregnant woman.

Pituitary abscess is a rare and potentially lethal condition. Pituitary abscess in a pregnant woman has not been previously described. A 38-year-old pregnant woman (34 weeks gestation) with a pituitary mass complained of a progressive headache and sudden visual impairment. She was afebrile and had no inflammatory symptoms on admission. On MRI, the preoperative diagnosis was pituitary adenoma with sphenoid sinusitis. She underwent an uncomplicated transsphenoidal procedure for removal of the pituitary mass. The next day, labor commenced and a healthy preterm baby was delivered. Pathologic examination of the intrasellar mass showed polymorphonuclear cells, debris and no tumor cells. The sellar contents were cultured and Streptococcus viridans was grown. To our knowledge this is the first case of pituitary abscess reported during pregnancy. Although the patient was pregnant, the transsphenoidal approach was safe for the mother and the fetus. Surgical drainage and antibiotic therapy are required for the definitive treatment of this condition.     

T‐cell lymphoblastic lymphoma/leukemia presenting as a pituitary mass lesion: A case report and review of the literature

We present a rare case of primary T‐cell lymphoblastic lymphoma of the pituitary gland. A 58‐year‐old woman presented with headaches, right‐sided ptosis and cranial nerve III palsy. She subsequently developed polyuria, polydipsia, and hyperglycemia and was found to have hypopituitarism. MRI revealed a large, heterogeneously enhancing intrasellar/suprasellar lesion displacing the optic chiasm and extending into the right cavernous sinus. Radiologically, these findings were thought to represent an invasive pituitary adenoma. Pterional craniotomy was performed with subtotal tumor resection. Histopathological examination revealed a T‐cell lymphoblastic lymphoma/leukemia (T‐LBL) admixed with pituitary corticotrophic cell hyperplasia. CT scans of the chest, abdomen and pelvis showed no evidence of systemic disease. Analysis of peripheral blood and bone marrow, including flow cytometry, demonstrated no involvement by T‐LBL. Follow‐up MRI of the spine revealed abnormalities in the distal thoracic spinal cord and conus medullaris, raising suspicions of leptomeningeal dissemination. Only five case reports of T‐cell primary pituitary lymphoma (PPL) have been previously described, four of which were associated with hypopituitarism and/or concurrent pituitary adenoma. We present the first report of a T‐cell PPL associated with adenohypophyseal hyperplasia and the third documented occurrence of a primary pituitary T‐LBL.     

MRI and CT in a case of pituitary abscess.

We report the case of a woman admitted for hypopituitarism of sudden onset, in whom conventional radiography, CT and MRT suggested a pituitary tumour with supra- and intrasellar extensions. The surgical findings and the clinical course under antibiotic therapy transformed this diagnosis into one of pituitary abscess by a pyogenic micro-organism. Pituitary abscess is an exceptional lesion. Despite the advent of CT and MRI, its preoperative diagnosis remains difficult. However, the presence of an intrasellar expansive process with liquid centre and contrast-enhanced outline should suggest the possibility of an abscess, particularly when the pituitary lesion is associated with a sphenoidal sinus effusion.     

Subarachnoid hemorrhage from dissecting aneurysm of the posterior communicating artery

A 36-year-old woman on chronic hemodialysis presented with sudden onset of headache and deterioration of consciousness. She was sent to our hospital on day 1, with subarachnoid hemorrhage. Cerebral angiography showed pearl and string sign on the left posterior communicating artery, which was consistent with a diagnosis of hemorrhage from a dissecting aneurysm of the left posterior communicating artery. She underwent parent artery occlusion via endovascular treatment on day 2. Although cone beam computed tomography before embolization showed a perforator from the lesion, there were no ischemic lesions on diffusion-weighted imaging after the procedure. She was discharged without any neurological deficits. It is important to recognize that dissecting aneurysm of the posterior communicating artery is one cause of subarachnoid hemorrhage. We also discuss the utility of cone beam computed tomography in formulating the treatment plan for such patients.     

Spontaneous pituitary adenoma occurring after resection of a Rathke’s cleft cyst

Rathke’s cleft cysts (RCC) are benign cystic lesions that originate from remnants of the epithelial lining of Rathke’s pouch. RCC are known rarely to occur together with a concomitant pituitary adenoma. Here, we report a patient with a pituitary adenoma arising in the same location as a previously-resected RCC, 3 years post-operatively, and review the literature of “collision” sellar lesions. Consecutive transsphenoidal operations from a single-center between 2008 and 2016 were reviewed to identify patients with pituitary adenoma arising after surgical resection of RCC, and a systematic search of the literature was also performed to identify such patient reports, as well as reports of concomitant pituitary adenoma and RCC. Of 837 transsphenoidal operations from our own experience, one patient with pituitary adenoma occurring after RCC resection was identified and is reported here. A systematic review of the literature resulted in identification of 34 patients with concomitant RCC and pituitary adenoma and no incidents of pituitary adenoma occurring after resection of RCC. Concomitant occurrence of RCC and pituitary adenoma was more commonly diagnosed in women (61%), at a median age of diagnosis of 44 years. The RCC histological analysis in these patients consistently described ciliated columnar or cuboidal epithelium. Although rare, the presence of a new, pathologically-distinct lesions in the sella after prior surgical treatment, is possible. During post-operative monitoring, physicians should consider that what appears as a “recurrent” lesion may actually be growth of a new and entirely different lesion.     

岩下窦静脉取血在疑难ACTH依赖性库欣综合征诊断中的应用

目的 探讨岩下窦静脉取血(IPSS)在疑难库欣综合征诊断中的价值.方法 对20例疑难库欣综合征行岩下窦和外周静脉取血,测定血ACTH浓度比,评价其对库欣病诊断的敏感性和特异性.结果 18例患者的岩下窦与外周静脉血ACTH的比值＞2,其中13例行经蝶窦垂体术后病理为垂体ACTH腺瘤;1例行鞍区γ-刀治疗、4例行生长抑素治疗后病情缓解.2例岩下窦与外周静脉血ACTH的比值＜2,其中1例为右肺类癌,另1例为垂体ACTH腺瘤.本组IPSS诊断库欣病的敏感性和特异性分别为93%和100%.结论 IPSS操作安全,并发症少,可作为疑难库欣综合征的重要鉴别方法.     

垂体脓肿的诊断和经蝶显微手术治疗

目的探讨垂体脓肿的诊断及治疗方法。方法回顾性分析我院15例垂体脓肿患者的临床表现、影像学特征、诊断和治疗。结果15例患者中术前主要表现为头痛8例,视力下降或颞侧偏盲6例,垂体前叶功能低下7例,尿崩4例,发热1例。MRI增强示病灶均呈环形强化。15例患者均采用经鼻蝶手术入路清除脓肿。术后1W,8例头痛患者症状均消失,6例视力下降患者中4例改善;术后随访至第3个月,4例尿崩患者有2例恢复正常,7例垂体功能减退患者中4例好转,另3例患者继续予以药物替代治疗;术后6个月,另2例仍多饮多尿患者和3例垂体功能仍低下者均恢复正常。15例患者均无复发。结论垂体脓肿术前诊断较困难,对鞍区囊性病变应考虑到垂体脓肿的可能性。及早采用微创手术、合理应用抗生素及恰当的对症治疗是治疗垂体脓肿的关键。     

Intrasellar cavernous hemangioma.

Reports of intrasellar cavernous hemangioma are rare. They are usually incidental findings at autopsy, or initially mistaken for pituitary adenoma and treated accordingly. There are no specific symptoms. Cranial nerve palsy has occasionally been reported in patients with cavernous hemangioma, with or without sellar extension. However, intrasellar cavernous hemangioma with extension into the cavernous sinus resulting in oculomotor palsy has not been reported. We present a rare case of intrasellar cavernous hemangioma and discuss the diagnosis and management. Total surgical removal is recommended. However, attempts to resect the parasellar component of the lesion may be associated with high morbidity. Therefore, surgical cranial nerve decompression in the acute stage followed by stereotactic radiosurgery for the residual lesion may be an alternative.     

A case of pseudotumor cerebri associated with colon cancer

A case of pseudotumor cerebri associated with iron deficiency anemia due to colon cancer is reported in a 37-year-old woman. Her initial symptoms were vomiting and severe headache. On physical examination, no lymph nodes and abdominal mass were palpable but marked anemia was noted in her skin and conjunctiva . Neurological examination revealed papilledema in her both eyes and stiff neck. There was no abnormal findings on CT scan on admission. Spinal puncture revealed CSF pressure as high as 620 mmH2O with normal cells, protein, sugar and chloride levels. Hematological examination revealed iron deficiency anemia and thrombocytosis. Angiography at third day revealed no sinus occlusion, but retention of contrast media was seen on the cortical vein of parietal lobe and right transverse sinus. Brain scintigram at sixth day revealed mild accumulation in left parietal lobe, so small venous infarction was suggested. There were two circumscribed stenotic lesions of right ascending colon in the barium enema, and right hemicolectomy was achieved. The pathological diagnosis was adenocarcinoma. The symptoms of pseudotumor cerebri was completely disappeared soon after the surgery together with resolution of anemia. She lives with no deficits now 1 year 3 months after surgery. In conclusion much attention is necessary to a patient of pseudotumor cerebri with iron deficiency anemia for the presence of cancer, because not only this central nervous system lesion is reversible and curable but also the cancer itself may be curable by surgery.     

Isolated histiocytosis X of the pituitary stalk

A case of histiocytosis X granuloma localized in the pituitary stalk is reported. Coronal and sagittal magnetic resonance imaging views were useful to determine the precise size and location of the mass lesion. The diagnosis was established immunohistochemically and the patient was treated with low-dose irradiation therapy. After irradiation, the patient improved well without endocrine replacement treatment. The pituitary stalk recovered its normal size with no evidence of recurrence on MRI at 7-year follow-up. We emphasize the importance of MRI before initiating therapy to evaluate the pituitary mass lesion and the effectiveness of low-dose irradiation for isolated histiocytosis X.     

术中 X-线定位在神经内镜切除甲介型蝶窦垂体瘤手术中的应用简

目的探讨术中x-线定位在经神经内镜下甲介型蝶窦垂体瘤手术的应用。方法回顾性分析6例甲介型蝶窦垂体瘤病人的临床资料,均采用神经内镜下垂体瘤切除结合术中X-线定位方法。结果术中X-线定位准确4例;2例发生纵向偏差,均纠正。肿瘤全切除5例,次全切除1例。术后激素水平正常,视力视野改善。出现暂时性尿崩1例,治疗后痊愈。无脑脊液鼻漏、垂体功能低下、蝶窦炎发生,无死亡病例。6例病人随访3年,均无复发。结论神经内镜技术结合术中X-线定位是一种治疗甲介型蝶窦垂体瘤的安全、有效方法。     

Cavernous angioma of the second cranial nerve and chiasmatic apoplexy

We present the case of a female patient who developed chiasmatic apoplexy and menstrual alterations. CT scanning showed a suprasellar hemorrhage. She underwent surgery with the presumptive diagnosis of pituitary tumor. At surgery, we find a brown-grayish lesion involving left optic nerve and chiasm. Cavernous angioma was diagnosed by histopathology. Cavernous angiomas constitute nearly 15% of all central nervous system vascular malformations. Location at the optic pathway is very rare, but must to be ruled out in the diagnosis of a patient with chiasmatic and/or optic apoplexy. Surgery is useful in preventing worsening of the previous deficit or a new visual defect.     

Diabetes insipidus in sellar-suprasellar tuberculoma.

Tuberculoma involving the sellar and suprasellar region is extremely rare. Sellar region tuberculoma usually presents with endocrinopathy of hypofunction, rarely hyperfunction or normal function of the anterior pituitary. However, sellar-suprasellar tuberculoma presenting with diabetes insipidus (DI) is very rare. We report the case of a 32 year old housewife presenting with DI and secondary amenorrhea, who had a sellar-suprasellar mass on MRI. She underwent a transnasal transsphenoidal surgical removal of the pituitary mass, which was tubercular in nature on histology. She received antitubercular treatment and hormonal replacement therapy. She was well at last follow-up, 3 years after surgery.