Suprasellar and intrasellar paragangliomas

Neoplasms of the sellar region are entities with a large differential diagnosis. Although paraganglionic cells have not been demonstrated in the pituitary or adjacent structures, the existence of sellar region paragangliomas is well-documented. To elucidate, in this area the nature of these unusual tumors is relatively difficult. Clinical history, physical examination, radiographic investigation as well as intraoperative gross observation are the same as those of sellar meningioma or pituitary adenoma. Immunohistochemistry, using neuroendocrine markers and electron microscopy are the two definitive diagnostic methods to differentiate among these entities. The clinical management, the possible pathogenesis of the tumor, the importance of immunohistochemistry in making the diagnosis and the clinical outcome of these patients are discussed.     

A giant retroclival Rathke’s cleft cyst

Rathke’s cleft cyst (RCC) is a rare sellar tumor that can extend to the retroclival region, resulting in brain stem compression. We report a male with short stature who experienced repeated syncope due to compression of the brain stem by a giant retroclival RCC. MRI revealed a large homogeneous sellar tumor with anterior displacement of the pituitary gland that extended to the left retroclival space. The tumor differed from ordinary RCCs because of its unusual clinical presentation, the direction of extension, and its imaging characteristics. The retromastoid surgical approach was used for cyst marsupialization. Intraoperative appearance and histological examination confirmed the diagnosis. A follow-up MRI, 2 years after resection of the cyst, showed no recurrence.     

Colloid cysts of the pituitary gland

Colloid cysts of the pituitary gland are very rare pathological lesions occurring in sellar region. Their pathogenesis is not clear. They are located between the anterior and posterior lobe of the pituitary. Colloid cysts of the pituitary gland are space occupying lesions and induce hypopituitarism, diabetes insipidus, visual disturbances, etc. They cause pituitary apoplexy too. Magnetic resonance imaging is the preferred neurodiagnostic method in evaluating these lesions. 503 transsphenoidal procedures were performed from January 1996 to February 2001. Hypothalamo-hypophyseal dysfunction was caused by colloid cyst in seven cases. The most frequent presenting symptoms were headaches, oligomenorrhea and loss of libido. Diabetes insipidus was found in two cases before surgery. Colloid cyst induced pituitary apoplexy in one patient. Two patients presented visual disturbances. All our patients were operated on. We performed six transsphenoidal procedures and one transcranial operation. Postoperatively, the endocrine function of the pituitary gland was evaluated. We concluded that transsphenoidal operation is a safe method for treating colloid cyst located in the sellar region.     

Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature

Intrasellar dermoid cysts are extremely unusual lesions, with only four cases reported to date, and have not been previously reported in association with sudden-onset symptoms. Here, we present the case of an intrasellar dermoid cyst with sudden-onset symptoms mimicking pituitary apoplexy in an elderly woman. A 69 year-old woman presented with sudden onset of headache, dizziness, and decreased visual acuity. Magnetic resonance imaging of the sellar region showed an intrasellar lesion, which showed mixed hyper- and hypointense signal on T1-weighted and T2-weighted images and enhanced peripherally. Endocrine workup showed pituitary hormones within normal levels. According to these findings, the initial diagnosis of nonsecreting pituitary macroadenoma apoplexy was made. Intraoperatively, a large amount of whitish-yellow purulent material was found in the mass and the lesion was partially removed, owing to tight adhesion between remanent mass and surrounding neurovascular structures. Pathology showed a dermoid cyst with abundant neutrophil infiltrations.     

经额外侧入路手术治疗鞍区中线病变

目的探讨经额外侧入路手术治疗鞍区中线病变的临床疗效。方法1999年10月～2003年4月经额外侧入路施行显微外科手术治疗鞍区中线部位病变患者128例,其中垂体腺瘤57例、颅咽管瘤34例、鞍结节和蝶骨平台脑膜瘤15例、前床突脑膜瘤6例、拉克囊肿4例以及其它病变12例。结果128例患者手术后恢复良好,未发生颅内感染,无严重神经功能障碍,无一例死亡。肿瘤全切除者中垂体腺瘤52例(91.2%)、颅咽管瘤30例(88.2%)、鞍结节和蝶骨平台脑膜瘤15例(100%)、前床突脑膜瘤6例(100%)、拉克囊肿4例(100%),前交通动脉动脉瘤3例夹闭顺利。手术所用时间比常规翼点入路缩短20～30min。结论额外侧入路显露鞍区中线部位病变的效果与翼点入路并无差别。     

鞍区生殖细胞瘤的早期诊断及综合治疗

目的 总结颅内鞍区生殖细胞瘤的早期诊断及综合治疗的经验.方法 回顾性分析23例高度怀疑鞍区生殖细胞瘤病人的临床资料.9例病人经手术证实为生殖细胞瘤后进一步行放化疗;14例通过临床特点、血清学和影像学检查、诊断性放疗后明确为生殖细胞瘤,直接进行放化疗.结果 随访23例,时间11～57个月,平均23.8个月.肿瘤复发1例,化疗后肿瘤消失.8例血HCG升高病人均恢复正常.所有病人头痛、视力减退等症状显著缓解,多饮多尿等内分泌异常症状仍存在.结论 鞍区生殖细胞瘤通过早期诊断和放化疗等综合治疗达到满意疗效.     

Subfrontal approach in sellar and suprasellar lesions

A variety of mass lesions are placed in and around of the sella turcica. Although their common location these lesions have distinguishing features and different approaches can be selected. There are conventional transcranial and sophisticated skull base approaches. We report our experience with a subfrontal route to the sellar region. Between February 1997 and March 1998, 19 tumors placed around the sella have been treated surgically by this method. Eleven of them were pituitary tumors, 3 were craniopharyngeomas, 2 were meningiomas, and 1 was germinoma. Total removal was achieved in 17 cases. There was not operative mortality. Postoperative complications included twelve cases of unilateral anosmia and four cases of transitory diabetes insipidus. Progressive stroke occurred in one patient. These results stress the importance of the subfrontal approach to reach such lesions in sellar region.     

Cerebral germinoma with marked granulomatous inflammation: Granulomatous germinoma

Cerebral germinomas with granulomatous inflammation are rare lesions that can present diagnostic difficulties. Four cases (two male and two female) of germinomas with pronounced inflammatory reaction are presented. The age ranged from 14 to 21 years (mean 18). Three patients with vision defects had masses around the sellar region, and a long duration of symptoms (2, 4 and 5 years). The fourth patient had a mass in the temporal lobe; she had convulsions and the duration of her symptoms was short (3 weeks). All lesions consisted of inflammatory changes with scattered neoplastic germinoma cells that expressed placental alkaline phosphatase. The inflammation area occupied more than two-thirds of the mass, and consisted of macrophages and their syncytial forms of mutinucleated giant cells, T-cells, B-cells, plasma cells, fibroblasts, and histiocytes or glial cells. Sarcoid granulomata were frequently seen, and multinucleated giant cells with Schaumann's bodies were also visible. The term ‘cerebral granulomatous germinoma’ is proposed for these unusual tumors. Moreover, it is likely that the study of cerebral granulomatous germinoma may provide some important clues towards the understanding of granulomatous inflammation in organs in general.     

Germ cell tumors (germinoma and yolk sac tumor) in unusual sites in the brain

This report presents three cases of primary intracranial germ cell tumor encountered in unusual sites, or essentially non-midline structures. The three provided the opportunity to examine surgically obtained tissues with the electron microscope. The histological diagnosis was initially made by light microscopic observations. The first case was a 10-year-old boy in whom the tumor occupied the right thalamic and basal ganglionic region and was diagnosed as a yolk sac tumor. The second an 11-year-old boy who presented with a mass lesion on the left thalamic and basal ganglionic region, diagnosed as germinoma. The third was a 39-year-old man who presented with multiple tumors in the ventricular system and posterior fossa, also diagnosed as germinoma. Among the three cases, neither diabetes insipidus nor ophthalmologic disorder was manifested. Extensive examination and autopsy findings indicated that these intracranial lesions had not metastasized from primary extracranial tumors, including those of the genital organs.     

眶上和经眶上锁孔入路在鞍区显微手术中的应用比较

目的比较眶上入路与经眶上入路在鞍区显微外科手术中的应用情况。方法总结25例鞍区肿瘤的手术经验,其中垂体腺瘤9例,颅咽管瘤6例,鞍结节脑膜瘤3例,蛛网膜囊肿4例,Rathke囊肿2例,上皮样囊肿1例。采用眶上入路17例,经眶上入路8例。部分病人术中采用神经内镜辅助技术。结果术后复查MRI,示眶上入路全切除13例,次全切除3例,部分切除1例;经眶上入路全切除和次全切除各为4例。眶上入路开颅操作简便,适用于大多数鞍区病变的手术。经眶上入路将锁孔技术与颅底外科技术结合运用,不仅将额叶的牵拉降至最低限度,而且对鞍区的显露较眶上入路视角向上增加了20°,操作径路也相应缩短。结论眶上入路适用于大多数鞍区肿瘤和囊性病变的手术切除,经眶上入路则适用于体积较大的鞍区肿瘤。内镜辅助技术可帮助术中确认手术效果,减少肿瘤残留机会。     

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.     

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.     

儿童鞍区肿瘤的外科治疗

目的 探索儿童鞍区肿瘤的有效手术治疗方法 .方法 回顾性分析45例儿童鞍区肿瘤患者的临床资料.结果 肿瘤全切除29例,次全切除9例,大部切除7例.术中16例可见垂体柄,均予保留.术后出现多饮多尿21例,其中17例在术后2周内恢复正常;出现高钠血症5例,低钠血症3例,均在2周内恢复正常,术后死亡1例.随访33例患者1～3年(平均1.5年),其中2例术后1年内复发而再次手术,13例行γ-刀治疗,31例患儿术后可参加正常学习.结论 严格把握手术适应证,选择合适的手术入路,并注意保护下丘脑结构,防止供血动脉损伤,是儿童鞍区肿瘤手术治疗的原则和取得较好效果的关键.     

Primary medulla oblongata germinoma – an unusual posterior fossa tumors in young adults

We encountered 2 patients with germinoma arising from the medulla oblongata in whom preoperative radiological diagnosis was difficult. A 30-year-old woman presented due to aspiration pneumonia caused by bilateral lower cranial nerve palsies, and a 24-year-old man presented with headache caused by obstructive hydrocephalus. In both patients, there was a midline tumor that extended from the lower part of the fourth ventricle to the C1 lamina level. It was well-demarcated and homogeneously enhanced tumor with a slightly high density on plain CT scan. The preoperative diagnosis for both patients was ependymoma. The former patient had persistent lower cranial nerve palsies due to brain stem injury after tumor resection. Both patients achieved complete remission with adjuvant therapy. Fewer than 10 cases of germinoma affecting the medulla oblongata have been reported. Radiological findings resembling those of the pineal region germinoma were observed in the two patients reported here. We would like to stress the importance of remembering germinoma when making a preoperative differential diagnosis of fourth ventricular tumors in young adults.     

Rathke's cleft cysts: surgical-MRI correlation in 16 symptomatic cases

Rathke's cleft cysts (RCCs) are non neoplastic epithelial lesions of the sellar region that have been rarely reported as a clinical entity. We retrospectively reviewed the magnetic resonance imaging (MRI), intraoperative, and pathological findings of a series of 16 cases of RCCs operated at our institution since 1992. Concurrently, we discussed the different hypotheses about their embryological origin. The patients included 12 females and 4 males, 11 to 73 years of age. Endocrine disturbance was the most common presentation, followed by headaches and visual impairment. The location of the cyst was intrasellar in 7 cases, intrasellar and suprasellar in 6 cases, and suprasellar in 3 cases. The size of the cyst ranged from 8 to 26 mm (mean 12 mm). MRI signal intensity was quite variable on T1-weighted images. The cyst appeared hyperintense in 6 cases, hypointense in 6 cases, isointence in 3 cases, and heterogeneous in one case. On T2-weighted images (available in 13 cases), the signal intensity was more constant and appeared hyperintense in 11 cases and hypointense in 2 cases. After Gd-DTPA, we did not observe enhancement either of the cyst contents or of the cyst wall, but only of the pituitary gland in all patient. Most often, the pituitary gland was displaced inferiorly by the cyst located above showing a typical image of "an egg in a cup". Fifteen patients were operated upon via the transsphenoidal approach and one upon a frontal craniotomy. Intraoperatively, the cyst contents were gelatinous or thick, and dark colored. In 2 cases, it was cerebrospinal fluid-like corresponding to the signal observed on MRI. The position of the pituitary gland confirmed by surgery in 15 cases coincided with enhancement seen and MR imaging. In 13 cases where biopsy of the cyst wall was performed, it confirmed focally ciliated columnar or cuboid epithelium. A coexistent adenoma was found in one case. In conclusion, we consider that RCCs have varied MRI characteristics so that no pathognomonic sign may be observed. Except in few cases, there were no correlation between MRI and intraoperative findings. Therefore, even with MRI studies, differential diagnoses with others cystic lesions of the sellar region remains extremely difficult. The most interesting findings on MRI studies of RCCs were to locate the pituitary gland to help the surgeon to preserve pituitary tissue during surgery.     

Germ cell tumors of the thalamus and the basal ganglia

Two cases of germ cell tumors (GCTs) of the basal ganglia are presented and 40 previously reported cases are reviewed. The incidence of GCTs of the basal ganglia and thalamus was estimated as less than 14% of all intracranial GCTs. All patients except for two (95%) were male, aged 7–19 years. The clinical course was usually slow. The major symptoms were hemiparesis, mental deterioration such as dementia or character change, precocious puberty, diabetes insipidus, oculomotor palsy, speech disturbance, and hemianopsia. Signs of intracranial hypertension did not occur until the late stages of the disease. The plain CT finding was characterized by an irregularly defined, slightly high-density area frequently accompanied by central low-density areas without significant mass effect. The tumors showed mild to moderate and nonhomogeneous contrast enhancement. An ipsilateral cerebral hemiatrophy was often found. MR images demonstrated the corresponding findings. GCTs of the basal ganglia had a high possibility of containing components other than germinoma such as choriocarcinoma, endodermal sinus tumor, and embryonal carcinoma. Thus, tumor markers in the serum, CSF, or cyst fluid were frequently positive. With recent refinement of microsurgical techniques as well as immunohistochemical study and measurements of tumor markers of serum, CSF, and cyst fluid, major resections of tumor, accurate pretreatment histologic diagnosis, and early determination of the specific types of this tumor appear to be readily possible. This is essential for effective treatment of patients not only with radiosensitive germinoma, but also those with radioinsensitive nongerminoma variants and a combination of them located in this region.     

鞍区皮样囊肿的诊断及治疗

目的探讨鞍区皮样囊肿的临床特征、诊断及治疗。方法对经手术及病理证实的11例鞍区皮样囊肿进行总结分析。结果11例中9例位于鞍上,1例位于鞍旁,1例位于鞍上—鞍旁,其中9例全切,1例近全切除,1例大部切除。本组无死亡及其他并发症。结论鞍区皮样囊肿少见,显微手术全切除效果理想,依据囊肿的不同位置及生长方式选择合理的手术入路。     

Sellar lymphoma mimicking sphenoid infection presenting with cavernous sinus syndrome

Lymphomas can occur throughout the body, but are relatively rare in the sphenoid region. The clinical presentations are atypical, ranging from nasal symptoms, pituitary hormone dysfunction and neurological impairment. We report a 63-year-old female patient who suffered nasal discharge, retro-ocular pain and left lateral gaze palsy for 3 months. Cranial MRI revealed sellar and suprasellar lesions involving the clivus, cavernous sinus and sphenoid sinus. The patient was treated for chronic sphenoid sinusitis. Histopathological review for possible misdiagnosis was arranged owing to treatment failure 1 month later. The final diagnosis was primary large B-cell lymphoma, which was confirmed by immunohistochemical study and systemic survey. The symptoms of sellar lymphoma resemble those of chronic sphenoid sinusitis. MRI and hematoxylin and eosin histological examination can accurately diagnose most cases. Immunohistochemistry assay is recommended in equivocal cases and provides a more detailed tumor classification.     

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.     

A rare case of pituitary adenoma with calcification: a case report

Though craniopharyngioma remains the most common sellar suprasellar tumor with calcification, it can be seen very rarely in pituitary adenomas and Rathke's cleft cysts. Appreciation of the pattern of calcification in a sellar suprasellar lesion can give a clue to the nature of the lesion. The authors describe a 53-yearold male with a cystic sellar suprasellar lesion and curvilinear calcification that was managed by successful transsphenoidal surgery. The present case highlights the occurrence of calcification in a pituitary adenoma and the importance of recognizing the calcification pattern for pre-operative diagnosis of sellar suprasellar lesions that can help plan the surgical strategy and management.