Benign brain tumors: sellar/parasellar tumors

Neoplasms of the sellar region include pituitary adenomas, craniopharyngiomas, Rathke's cleft cysts, and, less commonly, meningiomas, germinomas, and hamartomas. Each of these entities has unique diagnostic and treatment considerations. Pituitary adenomas are the most common sellar mass found in adults, whereas craniopharyngiomas account for the majority of pediatric sellar masses. The diagnosis of sellar lesions involves a multidisciplinary effort; detailed endocrinologic, ophthalmologic, and neurologic tests are critical. The management of pituitary tumors varies. For most tumors, transsphenoidal resection remains the mainstay of treatment. Less invasive modalities, such as endoscopic transsphenoidal surgery, specific chemotherapeutic drugs, and stereotactic radiosurgery, show promise as adjuvant treatment modalities.     

Imaging of sellar and parasellar lesions

The sellar and parasellar region is an anatomically complex area where a number of neoplastic, infectious, inflammatory, developmental and vascular pathologies can occur. Differentiation among various etiologies may not always be easy, since many of these lesions may mimic the clinical, endocrinologic and radiologic presentations of pituitary adenomas. The diagnosis of sellar lesions involves a multidisciplinary effort, and detailed endocrinologic, ophthalmologic and neurologic testing are essential. CT and, mainly, MRI are the imaging modalities to study and characterise normal anatomy and the majority of pathologic processes in this region. We here provide an overview of the most relevant MRI and CT characteristics together with clinical findings of pituitary tumors, vascular, inflammatory and infectious lesions found in the sellar/parasellar region in order to propose an appropriate differential diagnosis.     

鞍区肿瘤的临床特征与术中解剖研究

目的 探讨鞍区解剖特点及垂体瘤、颅咽管瘤、Rathke囊肿、鞍结节脑膜瘤的临床特征.方法 分析115例鞍区肿瘤手术治疗的病例资料,观察不同肿瘤的临床特点及术中鞍区解剖.结果 垂体瘤为鞍内或鞍上肿瘤,发病率居于鞍区第1位,主要表现为垂体前叶功能障碍、肿瘤部分囊性变;颅咽管瘤发病年龄较小,肿瘤主体在鞍上,多表现为钙化或囊变;Rathke囊肿为主体在鞍内的圆形或椭圆形肿物,边界清楚,肿瘤大小1 cm左右,与周围垂体组织存在较明显边界;鞍结节脑膜瘤以视力障碍为首发症状,鞍结节及其附近蝶骨平台骨质结节状增生为特征.结论 鞍区肿瘤的临床特征各具有特异性,熟悉鞍区解剖结构对鞍区肿瘤切除具有重要意义.     

Tuberculomas of the hypophysis cerebri: report of five cases.

Tuberculomas involving the hypophysis cerebri are extremely rare lesions, and these may occur even in the absence of systemic tuberculosis. This report presents the clinical data of 5 patients harbouring tuberculomas of the pituitary gland. Sellar tuberculomas commonly mimic pituitary adenoma. Histological diagnosis of a sellar lesion is mandatory irrespective of the clinical presentation and radiological findings. A history of extracranial tuberculosis in the past associated with radiological findings like leptomeningeal enhancement, parenchymatous brain tuberculomas or a thickened pituitary stalk on contrast MRI, are indicative of the possibility of a sellar tuberculoma. The aim of surgery is tissue diagnosis and tumour debulking. The response to long-term antituberculous chemotherapy is excellent. There is often a complete resolution of the granuloma and a satisfactory recovery of visual and endocrinological function.     

鞍区海绵状血管瘤误诊为侵袭性垂体瘤一例报告并文献复习

目的通过一例鞍区海绵状血管瘤误诊分析和相关文献复习,以降低鞍区海绵状血管瘤的误诊、误治率。方法结合相关文献,在对鞍区海绵状血管瘤的特点进行系统复习的基础上,对1例鞍区海绵状血管瘤的误诊原因进行分析,并对其与侵袭性垂体瘤进行鉴别。结果术中发现肿瘤颜色暗红,光滑质韧,切开后瘤体回缩、不能被刮出,出血明显,仅取小块组织行病理检查为海绵状血管瘤。结论鞍区海绵状血管瘤有其独特的临床特征,术前明确诊断避免误诊、误治。     

原发性高颅压中垂体和蝶鞍形态演变

目的 :证实原发性高颅压中垂体和蝶鞍形态改变的演化规律。方法 :10例经临床确诊的原发性高颅压病人 ,通过比较复查前后 MR正中矢状面垂体和蝶鞍出现的形态学改变。结果 :全部病人在首次 MR检查中均发现不同程度的空蝶鞍或出现蝶鞍扩大。在复查 MR中 ,有 9例病人的垂体形态和蝶鞍扩大程度出现改变 ,1例空蝶鞍和蝶鞍稍扩大病人在复查 MR中未见明显变化。结论 :原发性高颅压可引起的鞍区形态学改变 ,是与颅压增高程度和病程长短有关的动态演变过程。垂体形态改变要早于蝶鞍改变 ;而蝶鞍扩大出现较晚 ,提示已经存在长时期的明显的颅压增高     

垂体脓肿的诊断、治疗和长期随访

目的垂体脓肿是一种少见的鞍区感染性疾病,其临床表现没有特异性,术前诊断比较困难。为了更好地发现其临床特点以助于临床诊断,为了评估垂体脓肿手术治疗的疗效及其长期转归,我们分析总结了我院手术治疗的垂体脓肿病例资料。方法回顾性分析总结北京协和医院1991年～2007年17年间手术证实和治疗的30例垂体脓肿病例,对所有病例的详细病史资料、术前术后的影像学资料和内分泌检查结果及长期随访资料均进行了仔细分析和总结。结果垂体脓肿患者往往因为垂体占位性病变的相关症状而就诊,很少表现为感染相关的症状。中枢性尿崩、垂体功能低下、头痛是最常见的临床症状;MRI表现多样,但多数具有脓肿的典型表现即鞍区囊性占位,静脉注射对比剂后可见病灶边缘环形强化;尽管术后复发并不少见,大多数垂体脓肿通过手术引流可以治愈,但垂体功能低下往往很难恢复,需要长期激素替代治疗。结论临床上表现为尿崩、垂体功能低下伴有环形强化的鞍区占位往往提示垂体脓肿的诊断;治疗上首选经蝶脓肿引流手术,尽管伴有一定的复发率,手术往往可以治愈脓肿,但垂体功能低下往往不能恢复,需要重视长期替代治疗。     

Hemangiopericytoma of the sella mimicking pituitary adenoma: case report and review of the literature

OBJECTIVE: Hemangiopericytoma (HPC) is a potentially malignant vascular neoplasm that in rare cases presents as a primary intracranial lesion, where most often it is meningeal in origin. Hemangiopericytoma arising within the sella turcica is an even more sporadic event. To our knowledge, only 9 cases of HPC presenting as a sellar or suprasellar mass have been reported in the literature. Often, these cases can mimic and be mistaken for a pituitary adenoma. MATERIAL AND METHODS: We report a case of an 18-year-old woman presenting with a sellar mass which was thought both clinically and radiologically to be a pituitary adenoma. RESULTS: Based on histologic, immunohistochemical and electron-microscopic studies, the diagnosis of sellar HPC was made. CONCLUSION: Hemangiopericytoma should be considered in the differential diagnosis of sellar or suprasellar masses.     

Paraganglioma in pituitary fossa

Paragangliomas are neuroendocrine tumors of paraganglionic tissue which are extremely rare in the sellar area. We present a case of sellar paraganglioma with parasellar and suprasellar extension in a 70‐year‐old man who presented with headache. CT demonstrated sellar mass with suprasellar and right parasellar extension. The lesion was removed subtotally via a trans‐sphenoidal approach. Histopathological diagnosis was paraganglioma. Differential diagnosis between paragangliomas and unusual types of pituitary adenomas, especially null‐cell adenomas, is made by presence of cell nests (Zellballen) in paraganglioma, lack of immunopositivity for pituitary hormones, cytoplasmic immunopositivity for chromogranin A and neuron‐specific enolase, and immunonegativity for cytokeratin 19, cytokeratin 20 and endomysial antibody. In the case of a mass in the sellar region, paraganglioma, although rare, should be included in the differential diagnosis.     

Intrasellar plasmacytoma: an illustrative case and literature review

Intrasellar plasmacytoma is a rare pituitary pathology. Pre-operative diagnosis remains a challenge as a sellar plasmacytoma mimics a pituitary adenoma in clinical and radiological features. We report a 45-year-old woman, known to have multiple myeloma, presenting to our clinic with a pituitary mass eventually diagnosed as a plasmacytoma. We review 26 similar cases reported in the literature and divide them into three categories, based on their presentation and follow-up data. Pitfalls in diagnosis and reported treatment modalities are also discussed. We conclude that presentation with cranial nerve palsies and an aggressive sellar mass on imaging, and without gross anterior hypophyseal hormonal imbalance, are features of a pituitary plasmacytoma. The presentation and follow-up characteristics may help to determine the prognosis of such patients.     

A case of granulomatous hypophysitis with hypopituitarism and minimal pituitary enlargement.

A case of hypopituitarism and minimal sellar enlargement was found at hypophysectomy to have a giant cell granuloma of the pituitary. The clinical and histopathological features of this rare entity are reviewed. It is proposed that hypopituitarism which is out of proportion to minimal sellar enlargement may be a suggestive clue to the preoperative diagnosis of giant cell granulous which normally simulates a pituitary tumour.     

Diagnostic and clinical implications of pituicytoma

Pituicytoma is a rare, indolent, benign tumor of the sellar and suprasellar region arising from pituicytes of the neurohypophysis. It is most often diagnosed pre-operatively as a pituitary adenoma. We report two patients with pituicytoma operated on at our Institute over 8 years. Imaging of one patient showed a parasellar and medial temporal extension, which has not been reported to our knowledge. The radiological features that help distinguish this low-grade tumor from other sellar and parasellar tumors are discussed along with its distinct histological findings. The authors also review the literature on its clinical presentation, diagnosis, surgical management and outcome.     

Sellar abnormalities in female first-degree relatives

Non-pituitary lesions account for a minority of sellar region abnormalities. We report the unusual occurrence of non-pituitary sellar/suprasellar lesions in a mother and her two daughters. Each of these cases was diagnosed and managed differently, illustrating the relative importance of radiographic imaging, tumor markers and histopathologic examination in the diagnosis and treatment of intracranial disease. The mother had histologically confirmed Rathke's cleft cyst (RCC) with typical radiographic and histologic appearance. One daughter was treated presumptively for germinoma based on characteristic radiographic studies and slightly elevated tumor marker. The other daughter's lesion exhibited radiographic characteristics concerning for pituitary macroadenoma but with slightly elevated germ cell tumor marker, raising the suspicion for germinoma. Biopsy of the intrasellar mass revealed only proteinaceous material and normal anterior pituitary, consistent with cyst content without evidence of neoplasm. Without a clear unifying diagnosis it is difficult to posit an underlying pathology or genetic mechanisms in this unusual set of cases. At least two of the patients had benign cysts. The diagnosis of the third patient is unclear as there was no tissue biopsy. However, it is highly improbable that three female first-degree relatives would develop such lesions in the same brain region simply by chance.     

Pituitary metastasis: From pathology to clinical and radiological considerations

PurposeA review of the literature with respect to pituitary metastases (PM) with clinical and radiological considerations are summarized to facilitate clinical decision making in the management of PMMethodsA review of literature associated with PM and tumour to tumour metastases in the English literature was reviewed and summarizedResultsPituitary metastases account for 1.0–3.6% of all surgically treated pituitary lesions. Often identified in parallel with extensive disseminated disease, once diagnosed, the prognosis is generally poor, although survival is highly heterogeneous and dependent on the primary tumor histology. Within this anatomical region is also the observation of tumor-to-tumor metastases and collision tumours. Both the tumor macro- and microenvironment play central roles to the progression of disease with distinctive radiological features that may suggest a metastatic sellar lesion as opposed to a primary pituitary lesion. Surgical resection is the first line of therapy followed by adjuvant chemoradiotherapy and endocrinological evaluation for hormonal supplementationConclusionPMs are relatively rare but important oncological entities representing disseminated disease in the majority of cases. Careful consideration of the relevant clinical history and radiological features can aid the clinician differentiate between a metastatic lesion to the pituitary region and a primary pituitary tumor. While surgical resection is first line therapy, stereotactic radiosurgery in carefully selected patients is emerging as a viable alternative.     

鞍区的显微解剖学研究

目的 为临床神经外科提供鞍区显微解剖和解剖参数。方法 用汉族成人尸头湿标本、漂白干颅骨各30例,在显微镜下进行显微解剖观察并测量。结果 鞍区重要的解剖学结构有:①骨性标志有鞍结节、前后床突、视神经管、鞍底、蝶窦内视神经管隆起和颈内动脉隆起;②相关硬膜结构有鞍膈、海绵窦和海绵间窦;③相关蛛网膜结构有颈动脉池、视交叉池;④重要的神经血管结构有垂体和垂体柄、视神经和视交叉以及Willis环及其分支。结论 该显微解剖学研究提供了鞍区的重要解剖结构和解剖参数,对鞍区的临床手术具有重要价值。     

Melanoma of the sellar region mimicking pituitary adenoma

We report here the case of an 82‐year‐old woman who presented with visual disturbance. MRI demonstrated a sellar mass. The diagnosis of pituitary adenoma was made. She underwent transnasal surgery. Histologic, immunohistochemical and ultrastructural studies indicated that the tumor was a melanoma. Despite an exhaustive search for a primary lesion elsewhere, none was found. The sellar tumor was considered a primary lesion, although extrasellar primary tumor imaging cannot be excluded with 100% certainty. Reported examples of melanoma affecting the sellar region are few. They exhibit morphologic features identical to those of melanomas arising elsewhere. Although very rare, primary melanomas enter into the differential diagnosis of sellar lesions.     

垂体脓肿(附五例报告及文献复习)

目的 探讨垂体脓肿的临床特点、早期诊断和治疗选择。方法 5例垂体脓肿均行手术治疗，3例经额下入路，2例经蝶入路。本文逐一分析了对5例垂体脓肿的诊疗经验。结果 男性3例，女性2例，年龄在12～56岁，主要症状为感染史，头痛，视力减退，多饮多尿和垂体功能低下等。神经影像学表现为：强化的T1加权像可见鞍内／鞍上低密度肿物，周围有强化环。5例患者术后均恢复良好，随诊未见复发。结论 垂体脓肿早期明确诊断后，最佳选择为经蝶手术治疗。     

原发性高颅压的垂体及蝶鞍影像学形态变化(附28例分析)

目的　研究原发性高颅压病人垂体和蝶鞍形态变化与病程的关系。方法　回顾性分析原发性高颅压 2 8例病人的临床资料 ,并对垂体和蝶鞍形态变化分别分级。结果　本组垂体形态正常 3例 ,垂体 /垂体窝≥ 50 %者 6例 ,≤ 50 %者 1 0例 ,空蝶鞍 9例。蝶鞍形态正常 1 0例 ,蝶鞍稍扩大 1 2例 ,明显扩大 6例。病程小于 3个月者 ,3例出现垂体形态变化 ;3～ 1 2个月病程者均出现垂体形态改变 ,仅半数有蝶鞍形态改变 ;病程大于 1 2个月者均出现垂体和蝶鞍形态变化。结论　原发性高颅压患者垂体形态变化早于蝶鞍形态变化 ,垂体形态变化明显者的病程长于垂体形态正常或变化较轻者 ,此表现有助于早期提示和诊断原发性高颅压。     

内镜辅助经单鼻孔-蝶窦入路切除垂体瘤的解剖及临床研究

目的 进行内镜辅助经单鼻孔-蝶窦入路切除垂体瘤的解剖研究，探讨其临床应用手术经验。方法 10例甲醛溶液固定的成人尸头模拟内镜辅助经单鼻孔-蝶窦手术入路，并进行显微解剖学观察；临床16例垂体瘤经该入路手术切除。结果 内镜辅助经单鼻孔-蝶窦入路对蝶窦、鞍区等结构显露良好，内镜可弥补显微镜的盲区。临床16例垂体瘤中，手术全切13例，近全切3例，效果满意，无相关严重并发症发生。结论 解剖及临床应用表明内镜辅助下行经单鼻孔-蝶窦入路具有对鞍区结构显露良好、肿瘤全切率高、手术创伤小、并发症少、病人术后恢复快等优点，是切除垂体瘤的理想术式。     

A case of pituitary metastasis from breast cancer that presented as left visual disturbance

Tumors that metastasize to the pituitary gland are unusual, and are typically seen in elderly patients with diffuse malignant disease. The most common metastases to the pituitary are from primary breast and lung cancers. We report a 65-year-old woman with pituitary metastasis from breast cancer who presented with recent-onset left progressive deterioration of visual acuity and visual field. The clinical diagnosis was made after brain and sellar magnetic resonance imaging showed a large sellar mass compressing the optic chiasm and invading the pituitary stalk. An otorhinolaryngology and neurosurgery team removed the tumor via a transsphenoidal approach, and this procedure obtained symptomatic relief. Postoperatively, metastasis from breast invasive ductal adenocarcinoma was confirmed histologically. We report this unusual case with a review of the relevant literature.