Goblet cell carcinoid of the appendix

A 50-year-old man was admitted with a diagnosis of acute appendicitis on January, 5, 1984 and an appendectomy was performed on the same day. The acutely inflamed appendix measured 7 cm in length, and a gross tumor, was seen at the apex. Histologic examination of the gross tumor, which was 1.7 cm in diameter, revealed a neoplasm representing a goblet cell carcinoid. Silver impregnation by both the Grimelius and Masson-Fontana methods disclosed a positive reaction in some neoplastic cells. Electron microscopically, endocrine granules with both limiting membranes and ahlos, 100-210 nm in diameter, were present in the neoplastic cells, Because of the relatively large size of the tumor and neoplastic invasion to the mesoappedix in this case and, in addition, the possible malignant nature of carcinoid tumor, ileoascendocolectomy was performed as an additional operation.     

Goblet cell carcinoid tumors of the appendix: An overview

Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.     

阑尾杯状细胞类癌临床病理分析（附3例）

目的:探讨阑尾杯状细胞类癌（goblet cell carcinoid,GCC）的临床表现、组织病理学特征、免疫组化及特殊染色的特点,提高临床及病理医师对阑尾GCC的认识。方法:回顾性分析2018年7月至2019年4月我院收治的经临床及病理确诊为阑尾GCC的患者资料,对所有病例进行临床资料收集、组织病理学观察、免疫组化与特殊染色检查及预后随访,并结合相关文献进行分析讨论。结果:3例阑尾GCC患者,男性2例,女性1例,平均年龄57岁。3例患者临床表现均为急性阑尾炎。病理组织学观察3例均可见阑尾GCC的经典特征:肿瘤细胞轻度异型,排列成巢状,簇状、腺泡样结构,浸润阑尾肌层,直达浆膜层。1例表现为肿瘤细胞弥漫破坏肌纤维,伴黏液湖形成,灶区可见黏液腺癌成分存在。免疫表型:肿瘤细胞 CKpan、CK19、CK18、CK20、EMA、CEA、Syn、CD56、S-100阳性,CgA、CD68阴性。AB-PAS染色阳性。术后分别随访15个月、7个月和8个月,均生存良好,无复发和转移。结论:阑尾GCC临床易与急性阑尾炎相混淆,确诊需病理学检查。手术推荐右半结肠切除术。     

抗纤方中药制剂防治肺辐射损伤的临床研究

 目的 探讨抗纤方中药制剂对胸部肿瘤放疗所致肺辐射损伤的预防作用。方法 148例胸部肿瘤患者随机分为实验组70例，对照组78例；均接受胸部肿瘤常规放疗；实验组病例在放疗同时应用抗纤方中药制剂治疗至放疗结束；对照组患者则接受安慰剂治疗。结果 两组放疗后放射性肺炎及放射性肺纤维化的发生率分别为38.4 %，12.8 %和29.4 %，8.5 %，两组比较差异有统计学意义（P＜0.05）。结论 抗纤方中药制剂能够降低胸部肿瘤放疗所致放射性肺炎及肺纤维化的发生率，对肺辐射损伤有明显的预防作用。     

Sertoli cell tumors of ovary: light microscopic and ultrastructural study with histogenetic considerations.

Two cases of pure Sertoli cell tumors of the ovary are presented. The tumors were grey to brown and generally solid, but with several cystic areas. They showed a highly differentiated, but variable, histologic patterns with solid cords of neoplastic cells, as well as tubular formations. Ultrastructurally, the basal part of the cells rested on a nonfibrillary basement membrane layer while the free border showed occasional cilia. Laterally, the cells showed tight junctions and desmosomes. Abundant rough endoplasmic reticulum and some cystically dilated smooth endoplasmic reticulum were evident. The theories regarding the histogenesis are discussed. Enough similarities, both by light and by electron microscopy, are presented between these tumors and the Sertoli cell to justify classifying these ovarian tumors as Sertoli cell in type. The development of cilia is considered to be a manifestation of focal metaplasia of the neoplastic Sertoli cell.     

阑尾杯状细胞类癌的临床病理分析

目的 探讨阑尾杯状细胞类癌（GCC）的临床病理特征、诊断标准及治疗预后.方法 分析郑州大学附属洛阳市中心医院6例GCC患者的临床病理资料.结果 6例患者平均发病年龄为48.67岁,临床上4例表现为急性阑尾炎的症状和体征,2例表现为腹部疼痛;手术方式：4例为阑尾单纯切除术,2例为阑尾切除术＋右半结肠切除术;术后平均随访30个月,5例健康生存,1例失访.大体观察阑尾无明显肿块.显微镜下观察：4例肿瘤细胞似肠腺杯状细胞,无异型性和坏死,缺乏核分裂象,2例肿瘤细胞呈圆形及多边形,中等大小,胞质丰富,富含黏液,细胞核偏于一侧,呈印戒细胞样,瘤细胞呈巢状、花环状、腺样弥漫散在排列.免疫组织化学示：Syn阳性6例,CgA阳性4例,NSE、CD56、CK7、CK20均有患者阳性,5例Ki-67≤2％,1例Ki-67为3％,6例均未发现淋巴结、肠道、卵巢转移.结论 GCC是一种少见的神经内分泌肿瘤,较经典类癌更具有侵袭性.Syn、CgA阳性为诊断所必需的,Ki-67阳性指数对肿瘤分级有指导意义.     

Mixed Brenner and adenomatoid tumor of the testis: an ultrastructural study and histogenetic considerations.

A previously unreported association of Brenner and adenomatoid tumor found in the tunica vaginalis testis is presented. Many ultrastructural features found in mesothelial cells such as intercellular spaces, deeply indented nuclei, tonofilaments and tight desmosomes, were also shared by cells present in both neoplastic patterns. The previous histogenetic origins ascribed to testicular Brenner tumors are discussed and the evidence for their origin in the mesothelium considered.     

阑尾类癌13例报告

回顾并结合文献分析13例阑尾类癌患者的临床病理特征、治疗和预后。13例阑尾类癌患者，6例肿瘤直径〈1cm，局限于浆膜内无转移，单纯阑尾切除术后无瘤生存10年者5例，非肿瘤性死亡1例。3例肿瘤直径1～2cm，局限于浆膜内无淋巴结转移，回盲部切除术后生存5年者3例，生存10年者1例。4例肿瘤直径〉2cm，1例侵犯浆膜外无淋巴结转移者，回盲部切除术后生存满5年；2例侵犯浆膜外并淋巴结转移，回盲部切除术＋术后化疗，生存5年者1例；合并肝脏转移者1例，右半结肠切除术＋术后化疗，2年内死亡。初步研究结果提示，阑尾切除术中应常规纵形切开阑尾标本检查有无微小肿瘤；肿瘤直径〈1cm者可单行阑尾切除术；直径1～2cm者回盲部切除，术后辅以化疗；肿瘤直径≥2cm可有浆膜受侵和淋巴结转移，宜行右半结肠切除术，术后辅以化疗。     

阑尾类癌13例报告

回顾并结合文献分析13例阑尾类癌患者的临床病理特征、治疗和预后。13例阑尾类癌患者,6例肿瘤直径<1cm,局限于浆膜内无转移,单纯阑尾切除术后无瘤生存10年者5例,非肿瘤性死亡1例。3例肿瘤直径1~2cm,局限于浆膜内无淋巴结转移,回盲部切除术后生存5年者3例,生存10年者1例。4例肿瘤直径>2cm,1例侵犯浆膜外无淋巴结转移者,回盲部切除术后生存满5年;2例侵犯浆膜外并淋巴结转移,回盲部切除术+术后化疗,生存5年者1例;合并肝脏转移者1例,右半结肠切除术+术后化疗,2年内死亡。初步研究结果提示,阑尾切除术中应常规纵形切开阑尾标本检查有无微小肿瘤;肿瘤直径<1cm者可单行阑尾切除术;直径1~2cm者回盲部切除,术后辅以化疗;肿瘤直径≥2cm可有浆膜受侵和淋巴结转移,宜行右半结肠切除术,术后辅以化疗。     

Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). I. Adenocarcinoma of the appendix.

A study of primary epithelial neoplasms of the appendix (with the exception of classical carcinoids), produced 24 adenocarcinomas and 42 benign lesions from the files of the Laboratory of Surgical Pathology of Columbia University. Invasive adenoarcinomas were often complicated by appendicitis and were then unexpectedly discovered during appendectomy. In one third of the cases the carcinomas were found in juxtaposition to adenomatous lesions of the appendix. Well-differentiated adenocarcinomas of the colonic type generally had a better prognosis than signet-ring cell (or microglandular) tumors. The most reliable criterion for correlating prognosis was the Duke's method of staging. Since invasive adenocarcinomas of the appendix can metastasize to regional lymph nodes, treatment should ideally consist of ileocolectomy.     

Epithelioid sarcoma: case report with ultrastructural review, histogenetic discussion, and chemotherapeutic data.

A case of abdominal wall epithelioid sarcoma, studied by light and electron microscopy over a 3-year period, is presented. Ultrastructurally, there appear to be two types of tumor cells, light and dark, which differ by virtue of a heavier concentration of microfibrils and dilated rough endoplasmic reticulum in the dark cells. Both tumor cell types contain prominent Golgi systems, abundant free ribosomes, and numerous pinocytotic vesicles. The ultrastructural characteristics of the tumor cells resembel those of epithelioid cells of experimental human granulomas, as well as those of normal human synovium. A multifaceted relationship between histiocytes and synovial cells is demonstrated and it is concluded that the tumor is probably derived from mesenchymal reserve cells capable of differentiating a long histiocytic or synovial lines. Preliminary chemotherapeutic data are reviewed.     

Pathogenetic and histogenetic features of HIV-associated Hodgkin's disease

Compared with the cases in the general population, Hodgkin's disease (HD) arising in the HIV setting shows distinctive features in terms of epidemiology, aetiopathogenesis, histopathology and clinical behaviour. Although HD does not represent an AIDS-defining condition, recent evidence consistently indicates that HIV-infected individuals have a significantly increased risk of developing HD. HIV-related HD is characterised by the preponderance of aggressive histological subtypes, advanced stage at presentation, and highly malignant clinical course. Moreover, unlike HD in the general population, the large majority of HIV-related HD cases are pathogenetically linked to Epstein–Barr virus (EBV), with rates of EBV positivity ranging from 80 to 100%. Hodgkin and Reed-Sternberg cells of these cases invariably show a strong expression of the EBV-encoded latent membrane protein-1 (LMP-1), which functions as a constitutively activated tumour necrosis factor (TNF) receptor-like molecule. Usurpation of physiologically relevant pathways by LMP-1 may lead to the simultaneous or sequential activation of signalling pathways involved in the promotion of cell activation, growth, and survival, contributing thus to most of the features of HIV-related HD.     

Small cell tumor of cervix with neuroepithelial features: ultrastructural observations in two cases.

Two cases of small cell tumor of the uterine cervix are reported in which electron microscopic studies indicated that the tumor cells were of neural crest origin. Both tumors behaved aggressively, and metastatic involvement of pancreas was demonstrated in one patient, while the second patient developed liver metastasis. The need to distinguish these neoplasms from squamous cell carcinoma of the cervix is stressed. The tumors are compared with small cell neuroepithelial neoplasms previously reported as adult neuroblastomas.     

Malignant mixed müllerian tumors. An ultrastructural and immunohistochemical analysis with histogenetic considerations

In the female genital tract, malignant mixed müllerian tumors (MMTs) are uncommon neoplasms of uncertain histogenesis. We have examined 11 MMTs by both electron microscopy (EM) and immunoperoxidase techniques (IPX). Eight were of endometrial, two were of ovarian, and one of tubal origins. The IPX analysis included monoclonal antibodies to keratin (k) and vimentin (v) and a polyclonal antibody to myoglobin. Carcinomatous elements were always keratin positive (K+) and were focally positive for vimentin in six tumors. Homologous stromal sarcoma cells were vimentin positive (V+) and in three tumors were focally K+. Ultrastructurally, the epithelial cells were not highly differentiated and the sarcomatous elements generally resembled normal proliferative-phase stromal cells. The epithelial and stromal elements were separated by a thin basal lamina that only rarely and focally had discontinuities. No transitional cellular forms were identified. A definite positive myoglobin reaction was seen in two of the four neoplasms in which rhabdomyoblasts were identified by light microscopy. Myofilaments were identified by electron microscope in three neoplasms.