颅内原发生殖细胞瘤的临床特点

目的总结颅内原发生殖细胞瘤临床特点及治疗效果。方法回顾性分析1991-2003年在我院住院的颅内原发生殖细胞瘤的临床表现、生化检查(如人绒毛膜促性腺激素β亚单位、甲胎蛋白等)的变化、影像学检查(如MRI、CT)的表现及在诊断中的作用、治疗方法及效果。结果1991-2003年在我院住院的颅内生殖细胞肿瘤26例,占颅内肿瘤的0.9%(同期颅内肿瘤3020例),其中颅内原发生殖细胞瘤为19例(73.1%)。19例中女性9例(47.4%),男性10例(52.6%),20岁以下13例,占68.4%,20岁以上6例,占31.6%。有颅内压增高症状者14例(73.7%),尿崩症8例(42.1%),垂体前叶功能减退5例(26.3%)。肿瘤部位分布在松果体区9例(47.3%),鞍区及鞍上区7例(38.8%)。治疗上有14例(73.7%)接受放射治疗(其中7例为术后再接受放射治疗),放疗后患者病情均缓解出院。10例经手术不同程度切除肿瘤,有2例患者术后死亡。结论颅内原发生殖细胞瘤以儿童、青少年多见,松果体区及鞍区为其好发部位,颅内压增高症状、尿崩症及垂体前叶功能减退是颅内原发生殖细胞瘤的常见临床表现,放射治疗能取得较好临床效果。     

儿童鞍区肿瘤术后垂体功能减退合并非酒精性脂肪性肝病的临床特征分析

目的 总结儿童鞍区肿瘤术后垂体功能减退合并非酒精性脂肪性肝病(NAFLD)的临床特征，探索儿童垂体功能减退与NAFLD之间的临床联系。方法 回顾性分析2017年1月—2021年12月于华中科技大学同济医学院附属武汉儿童医院规律随访的鞍区肿瘤术后垂体功能减退出现NAFLD的患儿临床资料，分析临床特点。结果 共32例规律随访且临床资料完整的鞍区肿瘤术后患者，其中10例(31.25%)出现NAFLD,其中男5例，女5例，9例为颅咽管瘤，均手术治疗，1例为生殖细胞瘤，局部放射治疗。10例患儿诊断垂体功能减退时的中位年龄为8.4(6.2～9.8)岁；诊断NAFLD的中位年龄为11.9(8.7～12.6)岁。从诊断垂体功能减退至诊断NAFLD的中位年数为2.0(1.4～4.0)年。诊断NAFLD时，10例患者均为肥胖，BMI较诊断垂体功能减退时平均增加(7.26±4.25)kg/m²;10例患者空腹血糖均正常，平均为(4.67±0.55)mmol/L,平均空腹胰岛素水平为(25.40±5.93)μIU/mL;胰岛素抵抗指数(HOMA-IR)平均为(5.26±1.29);9例患...     

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。     

颅内生殖细胞瘤45例临床分析

目的分析颅内生殖细胞瘤临床表现、检查、诊断和治疗的特点。方法对1993—2005年解放军总医院收治的45例颅内生殖细胞瘤进行回顾性分析。结果男性30例,女性15例;发病部位鞍区19例(42.2%),松果体区13例(28.9%),其他部位13例(28.9%)。临床表现颅内高压症状(53%)、视力下降及视野缺损(40%)、尿崩症(38%)、垂体前叶功能减退(27%)。影像学检查CT显示病变呈类圆形略高或高密度影,增强后明显均匀或不均匀强化。MRI显示长T1长T2或等T1等T2异常信号影,多数病变增强后明显强化。6例检查血清和脑脊液,血β-人绒毛膜促性腺激素(β-hCG)增高4例,其中2例脑脊液β-hCG也增高。诊断经手术或脑组织活检病理诊断确诊12例,经诊断性放疗确诊11例,其余诊断均根据影像结果及临床表现。治疗11例行肿瘤部分或全部切除术;9例合并梗阻性脑积水,行脑室分流术;多数患者行放疗,其中3例联合化疗,症状均有不同程度缓解。结论颅内生殖细胞瘤多发于青少年,男性多于女性,病变多在鞍区和松果体区,主要表现为颅内压升高和视力障碍,影像学检查有显著意义,血浆和脑脊液β-hCG测定有助于诊断,放射治疗是有效的治疗方法,压迫症状明显患者可行手术治疗。     

垂体腺瘤与鞍区非肿瘤性病变

垂体腺瘤是颅内常见肿瘤 ,其发病率较高。依据肿瘤细胞的分类不同 ,临床上可分为多种类型 ,常见的有催乳素型、生长激素型、促肾上腺皮质激素型等 ;其相应表现分别为闭经 ,泌乳 ;肢端肥大 ;肾上腺皮质激素分泌过多等症状。依据上述症状 ,结合影像学及内分泌检查 ,典型的垂体腺瘤可与鞍区其他常见肿瘤鉴别 ,如鞍结节脑膜瘤、颅咽管瘤、异位生殖细胞瘤等。但因鞍区还有某些非肿瘤性的肿瘤样病变 ,且这些病变临床不常见 ,故有时与垂体腺瘤难以鉴别。本文将与垂体腺瘤相混淆的两种鞍区非肿瘤性病变作一介绍 ,以便于二者的鉴别。1　垂体增生垂体…     

甲状腺癌垂体转移一例报告

垂体转移是癌症的罕见并发症,甲状腺乳头状癌又是血行播散倾向较低的肿瘤。本文报告1例罕见的甲状腺乳头状癌垂体转移。该例患者43岁,因"右眼视物模糊"入院。8年前因甲状腺肿物行甲状腺部分切除术病理为甲状腺乳头状癌,没有进一步治疗。入院后MRI检查发现垂体肿物经鞍底突入右侧蝶窦内,为缓解视力障碍行神经内镜下经鼻蝶鞍区肿物切除...     

淋巴细胞性垂体炎的诊断与治疗

淋巴细胞性垂体炎(LYH)是一种较少见的自身免疫性内分泌疾病,以垂体组织内淋巴细胞浸润为特征,主要表现为颅内占位、垂体前叶功能减退和(或)中枢性尿崩症,需与其他类型的鞍区占位性病变鉴别.糖皮质激素治疗可以有效缩小病变并改善垂体功能.     

促甲状腺激素垂体瘤病例报道并文献复习

对2例促甲状腺激素(TSH)垂体瘤患者的临床资料进行回顾性分析,并进行文献复习.2例患者都存在甲状腺毒症,均有甲状腺激素水平增高同时不伴TSH抑制,1例大腺瘤患者存在视野缺损.2例患者在确诊之前均误诊为原发性甲状腺功能亢进而接受抗甲状腺药物治疗4～7年,1例患者还曾行甲状腺大部切除术.确诊后大腺瘤患者行垂体瘤手术联合垂体放射治疗,另1例为微腺瘤患者,行垂体放射治疗.术后随访,2例患者的甲状腺功能亢进均较前改善.甲状腺毒症患者血清TSH水平不被抑制时应警惕TSH垂体瘤的存在,鞍区影像学检查可以进一步明确诊断,早期诊断和手术联合放射治疗可以取得较好的治疗效果.     

颅内生殖细胞瘤45例临床分析

目的 分析颅内生殖细胞瘤临床表现、检查、诊断和治疗的特点。方法 对1993-2005年解放军总医院收治的45例颅内生殖细胞瘤进行回顾性分析。结果 男性30例，女性15例；发病部位：鞍区19例（42．2％），松果体区13例（28．9％），其他部位13例（28．9％）。临床表现：颅内高压症状（53％）、视力下降及视野缺损（40％）、尿崩症（38％）、垂体前叶功能减退（27％）。影像学检查：CT显示病变呈类圆形略高或高密度影，增强后明显均匀或不均匀强化。MRI显示长T1长T2或等T1等T2异常信号影，多数病变增强后明显强化。6例检查血清和脑脊液，血β-人绒毛膜促性腺激素（β-hCG）增高4例，其中2例脑脊液β-hCG也增高。诊断：经手术或脑组织活检病理诊断确诊12例，经诊断性放疗确诊11例，其余诊断均根据影像结果及临床表现。治疗：11例行肿瘤部分或全部切除术；9例合并梗阻性脑积水，行脑室分流术；多数患者行放疗，其中3例联合化疗，症状均有不同程度缓解。结论 颅内生殖细胞瘤多发于青少年，男性多于女性，病变多在鞍区和松果体区，主要表现为颅内压升高和视力障碍，影像学检查有显著意义，血浆和脑脊液β-hCG测定有助于诊断，放射治疗是有效的治疗方法，压迫症状明显患者可行手术治疗。     

双侧肾上腺及垂体肿瘤切除术后合并妊娠1例报告

患者30岁,妊娠39周,皮肤呈黑褐色,心慌、憋气,诊断为妊娠合并肾上腺皮质功能不全。患者曾于10年前患柯兴氏综合征行双极肾上腺切除术,术后每日口服强的松7.5mg行替代治疗。2年前患垂体嫌色细胞瘤行垂体肿瘤切除术。本次停经6周,出现恶心、呕吐等妊娠反应,停经18周感胎动,检查胎儿大小附合妊娠周,仍每日口服强的松7.5mg。     

Sellar and suprasellar mixed germ cell tumor mimicking a pituitary adenoma

Germ cell tumors (GCT) are a heterogeneous group of lesions whose origin is not well established. Several cases of primary intrasellar germinomas have been reported, however non-germinomatous GCT have rarely been described. We report the case of a young adult male patient with a mixed GCT that presented with a sellar tumor with suprasellar extension. The patient seeked medical attention because of seizures and magnetic resonance imaging evidenced a tumor of the sellar region. Hyperprolactinemia was also present and dopamine agonist therapy was started. As there was a rapid tumor growth and the patient had concomitant central diabetes insipidus and elevated testosterone levels, a GCT was suspected and confirmed by elevated serum concentration of β-human chorionic gonadotrophin. Patient underwent surgical resection of the tumor and histopathological examination confirmed the diagnosis of a mixed GCT. Chemotherapy was initiated, followed by conventional radiotherapy. In conclusion, although pituitary adenomas respond for the vast majority of sellar tumors, concomitant symptoms such as central diabetes insipidus and rapid tumor growth should raise the suspicion of a diverse diagnosis. The present report intend not only to show a rare case of sellar and suprasellar mixed GCT but also to remind clinicians that if laboratory findings do not fit into patient’s diagnosis (such as high testosterone levels in our patient), then the diagnosis should be reviewed.     

Surgical resection of granular cell tumor of the sellar region: three indications

Pituitary - This case series evaluates the surgical management of granular cell tumor (GCT) of the sellar region. This rare entity presents a unique diagnostic and surgical challenge. Institutional...     

Primary tumors of the posterior pituitary: A systematic review

In 2017, the World Health Organization established that pituicytoma, granular cell tumor (GCT), spindle cell oncocytoma (SCO) and sellar ependymomas (SE) are posterior pituitary tumors (PPT). They probably arise from the pituicytes and may constitute a unique histopathological entity. We carried out a systematic review using PubMed’s database. A total of 266 patients with pathological diagnosis of PPT (135 pituicytomas, 69 GCT, 47 SCO, 8 SE and 7 mixed histology tumors) were analyzed. Gender distribution was identical and median age at diagnosis was 48?±?21.8 years. Main presentation symptoms were visual disorders (n?=?142; 58.1%), headache (n?=?99; 40.5%), hypopituitarism (n?=?84; 34.4%), hypercortisolism (n?=?10; 4.1%), polyuriapolydipsia (n?=?6; 2.4%) and acromegaly features (n?=?5; 2.0%). On MRI, 122 (47.6%) patients showed sellar with suprasellar extension masses, 67 (23.1%) were suprasellar and 63 (24.6%) exclusively sellar. Median tumor size was 22.0?±?14.2 mm. Two hundred sixty four patients underwent surgery, transphenoidal access was selected in 132 (64.4%) and craniotomy in 58 (28.3%). Complications were hypopituitarism (n?=?70; 42.1%), diabetes insipidus (n?=?55; 33.1%) and hemorrhage (n?=?50; 30.1%). Tumor persisted in 93 patients (45.6%) and recurred in 13 (6.4%). Regarding comparison between main types of PPT, SCO patients were diagnosed later (60.0 vs 47.0 vs 47.0 years, p?=?0.023), the tumor was larger 25.0 mm [10.8] vs 20.0 mm [14.2] vs 2.0 mm [15.0] and they were frequently sellar with suprasellar extension tumors (71.7% vs 46.2% vs 32.8%, p?=?0.003) compared to pituicytoma and GCT. In conclusion, PPT are rare tumors and have been misdiagnosed mainly as non-functioning pituitary adenomas. Different types of PPT share similar epidemiology, clinical manifestations and surgical outcomes. Surgery is the only curative option but complications and subtotal resection are common.

     

Granular cell tumor of the cecum with extensive hyalinization and calcification： A case report

A granular cell tumor （GCT） is a benign neoplasm of unclear histogenesis that is generally believed to be of nerve sheath origin. GCT is not common and most often affects the tongue, skin and soft tissue, although it may occur anywhere in the body. We experienced a case of GCT that arose in the cecum of a 55-year- old man. The GCT was removed by laparoscopic resection. In addition to the tumor, endoscopic examination revealed the presence of a 5-mm-polyp in the descending colon and multiple tiny polyps in the sigmoid colon and rectum. Histological examination demonstrated a cecal tumor 1.5 cm × 1.0 cm × 0.7 cm with a hard consistency; in cut sections, mixed cells with yellowish and whitish portions were seen. The tumor was located between the mucosa and subserosa, and was composed of plump histiocyte-like tumor cells with abundant granular eosinophilic cytoplasm, which were immunoreactive for S-100 protein, vimentin, neuron-specific enolase, inhibin-α and calretinin. The tumor showed extensive hyalinization and focal dystrophic calcification. Immunohistochemical profiles did not confirm any particular cell type for the histogenetic origin of the GCT, including a nerve sheath origin. Extensive hyalinization and calcification showing involution of tumor cells suggest benign clinical behavior of GCT.     

Granular cell tumor of the duodenum: a case report

Granular cell tumor (GCT) in the duodenum is an extremely rare disease: only one case has been listed in a review, to date. We reported a 47-yr-old Japanese male case with GCT of the duodenum. Clinically, melena caused by bleeding from the tumor was the only symptom. The tumor cells showed abundant, granular eosinophilic cytoplasm. Although this tumor was clinically and histologically benign, highly developed tumor microvessels were demonstrated both angiographically and histologically, suggesting malignant potential of the tumor.     

Granular cell tumor of colon： Report of a case and review of literature

Granular cell tumor (GCT) is uncommon in the colon and rectum. Here we report a case of GCT in the transverse colon. A 48-year-old male patient underwent a screening colonoscopy. A yellowish sessile lesion, about 4 mm in diameter, was found in the transverse colon. An endoscopic snare resection was performed without complication. Histological examination revealed the tumor consisted of plump neoplastic cells with abundant granular eosinophilic cytoplasm containing acidophilic periodic acid Schiff-positive, diastase-resistant granules. Immunohistochemical analysis showed the tumor cells expressed S-100 protein and neuron-specific enolase. Thus, the resected tumor was diagnosed as a GCT. Since GCTs are usually benign, endoscopic resection constitutes an easy and safe treatment. Colonoscopists should consider the possibility of GCT in the differential diagnosis of submucosal tumors of the colon.     

The nature of giant cell tumor of bone

Giant cell tumor of bone (GCT) is a locally osteolytic tumor with variable aggressiveness. In rare cases, pulmonary metastasis can be observed. The lesion most frequently occurs in the epiphysis of long tubular bones of the knee region, predominantly affecting young adults after closure of the growth plate. The characteristic histological appearance of GCT displays a high number of osteoclast-like multinucleated giant cells, which resulted in the classification "osteoclastoma" or "giant cell tumor". Apart from the multinucleated giant cells, there are two mononuclear cell types in GCT. The first one has a round morphology and resembles monocytes. The second cell type is the spindle-shaped, fibroblast-like stromal cell. Cell culture experiments with GCT cells revealed the stromal cell to be the proliferating component of the GCT. The other two cell types, the monocyte and the multinucleated giant cell, were lost after a few cell culture passages. Furthermore, latest results from GCT reveal that the stromal cells secrete a variety of cytokines and differentiation factors, including MCP1, ODF, and M-CSF. These molecules are monocyte chemoattractants and are essential for osteoclast differentiation, suggesting that the stromal cell stimulates blood monocyte immigration into tumor tissue and enhances their fusion into osteoclast-like, multinucleated giant cells. The multinucleated giant cell itself resembles a normal osteoclast that is able to resorb bone leading to extended osteolysis. This new model of GCT genesis supports the hypothesis that the stromal cell is the neoplastic component whilst the monocytes and the multinucleated giant cells are just reactive components of this tumor. Taking this into consideration, the nomenclature of the "giant cell tumor" needs to be reconsidered.     

结直肠颗粒细胞瘤

颗粒细胞瘤起源于雪旺细胞。结直肠颗粒细胞瘤发生率在消化道中仅次于食道。大多结直肠颗粒细胞瘤无临床症状,术前明确诊断比较困难,内镜下治疗是良性结直肠颗粒细胞瘤的首选治疗方法。恶性结直肠颗粒需要行局部广泛切除,必要时加上区域淋巴结清扫,预后较差。     

Giant cell tumor of the cervical spine treated by carbon ion radiotherapy: A case report

Introduction:Giant cell tumor (GCT) of the bone is a benign–malignant intermediate tumor with locally destructive growth and a relatively high local recurrence rate. Neurological symptoms may develop in patients with GCT of the spine, and surgical treatment is prioritized in cases where resection is possible. However, the local recurrence rate of GCT of the bone is higher than that of GCT at other sites owing to the associated surgical challenges, and treatment is often difficult. No study to date has reported long-term remission of recurrent tumors for more than 5 years by treatment with carbon ion beam radiotherapy after resection of GCT of the cervical spine.Patient concerns:A 14-year-old boy who experienced recurrence after surgery for GCT of the cervical spine.Diagnosis:The patient presented with cervical pain, and computed tomography revealed a mass of the C2 vertebral body. He underwent surgery for tumor resection and autologous bone grafting, and the final pathological diagnosis was GCT. The transplanted bone exhibited gradual progression of resorption, and recurrent tumors were observed on computed tomography and magnetic resonance imaging 1 year and 4 months after surgery.Interventions:The patient was started on denosumab at 15 years of age and received carbon ion beam therapy with 70.4 Gy administered in 32 sessions over 7 weeks.Outcomes:No progressive tumor growth was observed, there were no neurological symptoms such as paralysis or pain were noted, and the patient was in remission for 5 years after irradiation.Conclusion:These findings suggest that carbon ion radiotherapy is a safe and effective therapeutic option for patients with recurrent GCT of the cervical spine.