乳头状肾细胞癌4例报道及文献复习

目的:探讨乳头状肾细胞癌(PRCC)的临床和病理特征。方法:对4例乳头状肾细胞癌临床表现、病理特征及免疫组化表型进行观察分析并文献复习。结果:肿瘤组织呈乳头状或管状结构排列,乳头中心及间质内有泡沫细胞浸润,有明显出血坏死。结论:乳头状肾细胞癌诊断依赖病理特征及免疫表型,并需与其他有乳头状结构的肾恶性肿瘤进行鉴别。     

多学科综合治疗转移性肾细胞癌:一例报告及文献复习

目的:结合文献复习,探讨多学科综合治疗转移性肾细胞癌的价值。方法:采用手术、局部放疗、全身放射性核素治疗、介入治疗、双膦酸盐及干扰素-α等方法对1例转移性肾细胞癌的原发肿瘤和多发骨转移癌及右肾上腺转移癌进行治疗,随访67个月,观察疾病进展时间和生存时间。结果:原发肿瘤无复发,疾病进展时间为54个月,骨相关事件的发生向后推迟了67个月,至今已生存74个月。结论:多学科综合治疗转移性肾细胞癌能够最大限度地减轻患者的痛苦,提高生存质量,延长生存期。     

Rectal small cell carcinoma: a case report and review of the literature

We present a rare case of small cell carcinoma of the rectum presenting with rectal bleeding and discomfort in a fit 51-year-old gentleman. Our patient was treated with a combination of chemotherapy and radiotherapy and remains alive and free of disease 6 years after diagnosis. Our patient experience and review of the literature is presented highlighting the uncertainties regarding the biological behaviour and management.     

Primary small cell carcinoma of kidney after renal transplantation：a case report and literature review

Extrapulmonary small cell carcinoma （EPSCC） is a rare neoplasm comprising 2.5％ to 5％ of small cell carcinomas （SCCs）.Bladder SCC is the most common site of genitourinary tract.Primary renal SCC is extremely rare.We report a case of primary SCC of the kidney which is rarely reported in the urinary tract and presents an aggressive clinical picture.A 59-year-old female visited a urologic clinic with complaint of persistent left flank soreness 10 years after undergoing renal transplantation.Abdominal computed tomography showed a left renal pelvis tumor.After the patient received left nephroureterectomy with bladder cuff resection,her pathology results showed SCC.After surgery,she received adjuvant systemic chemotherapy,and her recovery has been uneventful as of 8 months.Primary renal SCC presents with an advanced tumor stage and a short median survival period,therefore early intervention and close follow-up are recommended.     

肾透明细胞癌阴道转移1例报告及文献回顾

肾细胞癌阴道转移临床少见,肾细胞癌阴道转移的常见途径是泌尿系统、淋巴循环,血行转移少见.转移部位多见于肺、脑、肾上腺等,阴道转移罕见.本例肾癌术后1年余发现阴道转移,且病理类型与术前基本相似,主要为透明细胞癌,考虑应为血行转移.我院2015年收治1例,现报告如下. 1 病例资料 患者女,51岁,既往于2014年6月主因查体发现左肾占位性病变10天来我院求治,既往发现糖尿病病史2年,血糖控制尚可.行CT检查示:左肾类圆形软组织密度影,根据强化方式考虑肾癌.大小约5 cm×6 cm.B超检查示:左肾中下极可见大小约5.0cm×6.2cm,向包膜外突起,内部回声分布不均匀.彩色多普勒超声(CDFI):其内可见短条状血流信号.遂在全麻下行经腹腹腔镜下左肾癌根治术,术后病理回报示:左肾透明细胞性肾细胞癌,核分级1-2级,癌组织未侵及肾被膜.     

以尿崩症为首发症状的多系统郎格汉斯细胞组织细胞增生症一例并文献复习

目的 探讨郎格汉斯细胞组织细胞增生症（LCH）的临床特点、诊断及治疗,进一步提高对LCH的认识.方法 回顾分析1例以中枢性尿崩症为首发症状的多系统LCH患者的临床症状、实验室检查、影像学检查及病理和免疫组织化学结果,并复习相关文献.结果 患者早期以中枢性尿崩症起病8年,数年间逐步发展为肋骨、肢体长骨多发损害,肝、脾、淋巴结侵犯,通过胫骨及淋巴结活组织检查示正常组织结构破坏,具有典型形态学特征的郎格汉斯细胞局灶性增生,该细胞核型不规则,扭曲、分叶,可见特征性的核沟纹（咖啡豆样核）,免疫组织化学示表达S-100、CD1a、Langerin,明确诊断为多系统LCH.结论 LCH临床表现及生物学行为呈明显的异质性,既可以表现为孤立的溶骨性损害（如嗜酸性肉芽肿）,也可表现为白血病样侵袭性的病程.LCH损害可累及全身所有器官,典型的LCH累及骨骼较为多见.以累及垂体前后叶并以中枢性尿崩症起病的多系统LCH症状较为隐匿,容易延误诊断,LCH导致的神经内分泌损害需要终生的激素替代治疗.     

Merkel细胞癌术后复发1例及相关文献复习

目的：探讨Merkel细胞癌的诊断、治疗及预后。方法：一位老年Merkel细胞癌患者，于2006年8月初发时先行化疗及放射治疗，1年后局部再发肿瘤，再次接受扩大性切除术及放疗和化疗。结果：术后1月Merkel细胞癌再次复发。结论：Merkel细胞癌是一种罕见且进展快的皮肤肿瘤。诊断主要依靠病理（包括免疫组化方法），即使综合治疗后仍有局部复发和远处转移的倾向，预后差。     

Primary squamous cell carcinoma of thyroid: a case report and review of literature

Background

Oral epithelial dysplasia (OED) has a malignant potential. Therapeutic options for OED remain both limited and without good evidence. Despite surgery being the most common method of treating OED, recurrence and potentially significant morbidity remain problematic. Consequently, there has been much interest in non-surgical treatments for OED. Cyclo-oxygenase (COX) up-regulation is known to occur in the dysplasia-carcinoma sequence and evidence now exists that COX-2 is a prognostic marker of malignant transformation in OED. COX-inhibitors are therefore considered a potential therapeutic strategy for treating this condition. We aimed to provide both proof of principal evidence supporting the effect of topical COX inhibition, and determine the feasibility of recruitment to an OED chemoprevention trial in the UK.

Methods

Recruitment of 40 patients with oral leukoplakia to 4 study arms was planned. The total daily dose of Aspirin would increase in each group and be used in the period between initial diagnostic and follow-up biopsies.

Results

During the 15-month recruitment period, 15/50 screened patients were eligible for recruitment, and 13 (87%) consented. Only 1 had OED diagnosed on biopsy. 16 patients were intolerant of, or already taking Aspirin and 16 patients required no biopsy. Initial recruitment was slow, as detection relied on clinicians identifying potentially eligible patients. Pre-screening new patient letters and directly contacting patients listed for biopsies improved screening of potentially eligible patients. However, as the incidence of OED was so low, it had little impact on trial recruitment. The trial was terminated, as recruitment was unlikely to be achieved in a single centre.

Conclusion

This feasibility trial has demonstrated the low incidence of OED in the UK and the difficulties in conducting a study because of this. With an incidence of around 1.5/100,000/year and a high proportion of those patients already taking or intolerant of Aspirin, a large multi-centred trial would be required to fulfil the recruitment for this study. The ability of topical non-steroidal anti-inflammatory drugs to modify COX and prostaglandin expression remains an important but unanswered question. Collaboration with centres in other parts of the world with higher incidences of the disease may be required to ensure adequate recruitment.

ISRCTN

31503555.     

Unique pineal gland metastasis of clear cell renal carcinoma: case report and review of the literature

The metastatic involvement of the pineal gland is an extremely unusual event; it has a 4% incidence in patients with disseminated neoplasias. Most metastatic pineal lesions are asymptomatic. Only in a small number of cases the symptoms produced by metastatic involvement of this organ precede those of the primary tumor or those of another metastatic site. To our knowledge the herein reported case is the first in which the pineal gland was apparently the unique metastatic site of a primitive kidney carcinoma and where the symptoms produced by metastasis in the pineal region were the first sign of the disease.     

多房囊性肾细胞癌1例报告及文献复习

目的:探讨多房性囊性肾细胞癌的临床病理特征及其鉴别诊断。方法:对1例多房性囊性肾细胞癌的临床表现、病理特征及免疫组化表型进行观察分析并文献复习。结果:男55岁,因体检时发现右肾肿瘤伴尿急、尿频和尿痛3天入院,CT示:①右肾上极占位性病变,考虑肾癌;②右肾上腺囊肿;③左肾囊肿。镜下可见囊腔内衬单层或数层异型小的透明细胞,囊壁之间可见透明细胞巢,部分囊腔内有粉染液体,内含大量红细胞。免疫组化CK(++)、EMA(++)CD68(-)。病变符合多房性囊性肾细胞癌。结论:多房囊性肾细胞癌的诊断依赖病理特征及免疫表型,并需与其他肾囊性病变相鉴别。     

Metastasising clear cell odontogenic carcinoma: a case report and review of the literature

Primary odontogenic carcinomas are rare and examples which have metastasised are even more uncommon. We describe the first reported case of a clear cell odontogenic carcinoma which metastasised to distant bones, namely the 5th lumbar vertebra and hip, 3 years after initial diagnosis. The initial incisional biopsy was thought to represent a calcifying epithelial odontogenic tumour, but in the subsequent resection the tumour showed a prominent clear cell component admixed with squamous cells showing peripheral palisading, widespread infiltration and necrosis indicating a malignant neoplasm. Radiologically guided biopsy revealed a metastatic lesion in L5 vertebrae and left hip, confirmed by immunohistochemistry. The metastatic lesion had similar appearances to the first biopsy, and diagnosis was confirmed by comparison of histological features, immunohistochemistry and exclusion of a second primary lesion by clinical examination and imaging. The diagnosis of clear cell odontogenic carcinoma is a difficult one to make. The behaviour of these tumours is unpredictable. This case confirms that clear cell odontogenic carcinomas have the potential for distant metastasis and require long-term follow up.     

Asymptomatic cerebellopontine angle and lateral ventricle metastases from renal cell carcinoma: case report and literature review

Tumors rarely metastasize to the cerebellopontine angle. We report the first instance of simultaneous metastasis of renal cell carcinoma to the cerebellopontine angle and the lateral ventricle. A 51-year-old man presented with anemia and fatigue owing to stomach metastases from renal cell carcinoma 8 years after undergoing partial left nephrectomy for grade II clear cell renal carcinoma and radical right nephrectomy for grade III clear cell renal carcinoma. He also suffered metastases to the lung, both adrenal glands, the L-3 vertebra, and the brain (asymptomatic, but revealed by magnetic resonance imaging): a 1-cm (maximum diameter) mass in the left medullary cistern and a 2-cm (maximum diameter) mass in the right lateral ventricle trigone. Both brain lesions were treated with stereotactic radiosurgery, followed by gross-total resection of the right trigonal mass 7 months later. Both tumors shrank significantly, but the patient died from progressive systemic cancer 1 year after diagnosis of brain metastasis.     

Giant renal cell carcinoma: a case report

Though renal cell carcinoma is a fairly common disease, it is extremely rare to encounter a case providing a resected mass as large as over 2,000 g in weight. We report a case in which a giant 2,200 g tumor was resected after renal arterial embolization and interferon treatment for reduction of its size. A forty-three-year-old woman was first seen at a certain hospital with a chief complaint of fever and right flank pain. Under suspicion of right renal tumor, nephrectomy by extraperitoneal flank incision was attempted. However, the tumor was too large to be resected. After transfer to our department, she was treated by renal arterial embolization with absolute ethanol and steel coil and by the administration of interferon (rIFN-alpha A). As the tumor shrank to 77% of its original size on CT measurements and became mobile, transperitoneal nephrectomy was performed. The resected mass was as large as 12 x 12 x 21 cm. Pathologically, it was a clear cell type tumor showing mucinous degeneration and hyaline degeneration of tumor cells. Interferon used amounted to as high as 864 x 10(6) units, but no particular side effect occurred. No recurrence has been detected so far.     

Primary signet ring cell mucinous ovarian carcinoma: a case report and literature review

A 24-year-old female patient presented with an extremely rare primary signet cell carcinoma of the right ovary 1 year after surgery for a mucinous borderline tumour of the left ovary. Relaparotomy was carried out with right adnexectomy, appendectomy and partial omentectomy. Surgery was followed by 6 courses of paclitaxel/carboplatinum chemotherapy. After an initial response, the patient again developed increasing ascites. The patient was transferred to our hospital and a re-relaparotomy was carried out, completing the operation. After 3 courses of pegylated doxorubicin/trabectedin, the clinical course showed a positive response and a decline of the tumour marker CEA in peripheral blood. After 5 months, ascites developed in the retroperitoneum so that the chemotherapy had to be changed. In spite of a positive response with the new chemotherapy, the patient died of a very rare pulmonary complication after 1 month within 2 days.     

替西罗莫司治疗索拉菲尼失败后转移性肾癌1例报道并文献复习

目的：本文通过病例报道及文献复习,探讨哺乳动物雷帕霉素靶蛋白（ mammalian target of rapamy-cin,mTOR）抑制剂治疗转移性肾癌（metastatic renal cell carcinoma,mRCC）的药理作用及临床研究进展。方法：回顾1例 mRCC 的临床资料,分析替西罗莫司治疗的疗效及不良反应,同时复习近5年关于 mRCC 及mTOR 抑制剂的143篇文献,深入了解 mTOR 抑制剂治疗 mRCC 的药理作用,总结临床应用现状。结果：患者无进展生存期（progression - free - survival,PFS）为7.5个月,主要不良反应为血红蛋白减少,胆固醇及甘油三酯均有升高。结论：患者经替西罗莫司治疗,不良反应少,耐受性较好,并取得了较好的客观疗效。因此, mTOR 抑制剂治疗在 mRCC 治疗中有一定的优势,值得临床进一步研究。     

Recovery of paraneoplastic hypercalcemia by sunitinib treatment for renal cell carcinoma: a case report and review of the literature

Sunitinib is a novel oral multitargeted tyrosine kinase inhibitor. İt has higher response rates and progression-free survival in patients with metastatic renal cell carcinoma (RCC) when compared with standard chemotherapy and interferon-α. We report a case of paraneoplastic hypercalcemia, resistant to conventional treatment but recovers by sunitinib treatment as the first case in the literature, in a 33-years-old man with metastatic RCC. At the sixth month of follow-up period, in this case, serum calcium level was still in normal ranges. Besides sunitinib is effective in symptom control, it is also helpful in management of paraneoplastic hypercalcemia, a life-threatening entity.