Sarcomatous variant of Wilms' tumor. A light and immunohistochemical study of four cases

Four cases of infantile sarcomatous renal tumors are reported. Rhabdomyoblastic differentiation was investigated in these tumors by the immunoperoxidase method using antimyoglobin sera. Rhabdomyoblastic cells were found in 2 cases and in a metastasis of one case in which the primary tumor was composed by undifferentiated cells negative for myoglobin stain. The histogenesis of these neoplasias is discussed, and it is suggested that they are a sarcomatous variant of Wilms' tumor.     

Primary carcinoid tumor of the common hepatic duct: A rare case with immunohistochemical and molecular findings

We report immunohistochemical (IHC) and molecular findings in a rare case of a carcinoid tumor of the extrahepatic bile ducts in a 33-year-old woman, who presented with a 3.9x2.8x2.6 cm mass within the right and common hepatic ducts. She underwent surgery and a carcinoid tumor was identified. This lesion is of interest because in addition to the morphological and cytological features of a typical carcinoid, it demonstrated a distinct pleomorphic area immunoreactive for gastrin. By molecular analysis, loss of heterozygosity (LOH) with opposite allelic patterns between the gastrin-positive and gastrin-negative areas of the tumor was identified. The molecular studies for LOH along with the morphology and IHC profiling suggest that this second population of gastrin-positive carcinoid cells may represent a new clone within the carcinoid tumor with differentiation toward gastrin production or may represent the next step in the carcinogenic process with a gradual emergence of a more aggressive clone.     

Epithelioid trophoblastic tumor: a clinicopathological and immunohistochemical study of seven cases

The objective of this study is to evaluate the clinicopathological features and immunohistochemical characteristics of epithelioid trophoblastic tumor (ETT). Seven cases of epithelioid trophoblastic tumor treated in the Women’s Hospital of Zhejiang University from 2004 September to 2008 December were retrospectively analyzed. Immunohistochemical study was performed. The most common presenting symptom was vaginal bleeding. Four patients had prior evidence of molar pregnancy and three patients presented with metastases. Mean age at diagnosis was 34.7 years. Mean pregnancy interval was 3.39 years. Human chorionic gonadotropin levels were 33.25–174315.5 IU/l. One case died from metastasis in lungs. The remaining six patients survived without recurrence. Immunohistochemistry revealed diffusely positive for CK18, and focally positive for β-hCG, HPL, Mel-CAM (CD146) and inhibin-alpha. Nuclear staining of Ki67 and p63 were seen. The confirmation of epithelioid trophoblastic tumor diagnosis is difficult before surgery. Surgical intervention is the recommended primary treatment.     

Thymic carcinoid. A histologic, immunohistochemical, and ultrastructural study of 12 cases

Twelve cases of thymic carcinoid were studied by electron microscopy and immunohistochemistry. Clinically, all of them presented either as asymptomatic, radiographically detected mediastinal masses or as neoplasms producing symptoms of thoracic structural displacement or Cushing's syndrome. By light microscopic examination, the carcinoids manifested an organoid growth pattern, being composed of uniform polygonal cells with central, oval nuclei and finely dispersed chromatin. Immunohistochemical analysis disclosed the presence of cytoplasmic adrenocorticotropic hormone in four cases (three associated with Cushing's syndrome), of somatostatin in seven cases, and of serotonin in one case. Ultrastructurally, these tumors generally showed short, blunt, interdigitating cell processes; focal, scanty basement membrane material; infrequent but well-formed junctional complexes; moderate to large numbers of dense-core granules; and lipoautophagosomes. Six tumors contained small numbers of type I microfilaments, and three demonstrated perinuclear whorls of type II microfilaments. Rough and smooth endoplasmic reticulum was well developed in each tumor, the smooth form being prominent in cases with clinical Cushing's syndrome.     

Primary carcinoid tumor of the liver: Report of four resected cases including one with gastrin production

Four cases of primary hepatic carcinoid were identified during a retrospective study of liver resections for primary tumor. The cases included two adult males, one adult female, and a 9-year-old boy in whom gastrin levels were documented. The estimation of gastrin levels was prompted by symptoms suggestive of acid-peptic disease. One patient died postoperatively. The other three are alive and well at 3 years, 2 years, and at 1 year, respectively, after surgery, outcomes distinctly different from hepatocellular carcinomas. Diagnostic difficulties may be experienced in histologic assessment, and this may require recourse to immunohistochemistry and electron microscopy. Long-term follow-up and careful exclusion of a possible primary elsewhere are necessary for establishing the primary nature of liver carcinoids. © 1996 Wiley-Liss, Inc.     

胃肠道类癌：免疫组织化和组织化学研究

Fourteen gastrointestinal carcinoid tumors were studied by immunohistochemical and histochemical methods. Neuro-specific enolase (NSE) was detected in all 14 cases with patterns of homogeneously moderate to strongly diffuse cytoplasmic stain. 12 of 14 neoplasms (85.7%) were positive for Grimelius technic. 5 (35.7%) were positive for Masson-Fontana stain. The results indicate that the immunohistochemical demonstration of NSE is a marker more sensitive than the silver stain for gastrointestinal carcinoid tumors. Distribution and significance of silver stain are discussed.     

直肠类癌临床病理及免疫组织化学分析

目的探讨原发性直肠类癌的临床病理、免疫组织化学特点。方法对11例原发性直肠类癌临床及病理资料进行回顾分析,应用S-P法免疫组织化学染色。结果 11例直肠肿瘤均为典型类癌。免疫组织化学结果Secretagogin 100.0%（11/11）,Syn 90.9%（10/11）,CgA 63.6%（7/11）,CEA 0.0%（0/11）,p53 0%（0/11）阳性。11例均行肠镜下黏膜切除术,其中2例再行肠段切除术。11例获随访者均无瘤生存。结论直肠类癌是一种低度恶性潜能的内分泌肿瘤,多数病例可以在内镜下行黏膜切除获得治愈。联合应用免疫组织化学Secretagogin、Syn、CgA、CEA和p53可作为直肠类癌诊断和预后的标志物。     

Glioblastoma multiforme in four siblings: A cytogenetic and molecular genetic study

Summary The familial occurrence of gliomas, in the absence of well-defined neurological tumor syndromes such as the neurofibromatoses, is uncommon. We present a family of ten children in which the four eldest suffered from gliomas. Three of these siblings had histologically verified glioblastoma multiforme, and one patient also had an intestinal non-Hodgkin's lymphoma, but there were no stigmata or family history of a neurological tumor syndrome. Cytogenetic studies of the proband revealed a normal karyotype. Molecular genetic analysis of the proband's glioblastoma revealed two mutations in the p53 tumor suppressor gene, but these were not present in the germline DNA, mutations were not detected in the MTS1 gene in the tumors or in the germline DNA. These findings suggest that a genetic factor may be responsible for the clustering of glial tumors in this family, but it is unlikely that the genetic alteration is mutation of the p53 gene. The data are discussed in light of the literature on familial brain tumors.     

胃肠道间质瘤78例临床病理及免疫组织化学特征

目的探讨胃肠道间质瘤（GIST）的临床病理特点及免疫组织化学特征。方法对78例GIST病例进行分析,并对其进行CD117、CD34、SMA、Desmin、S-100共5种免疫组织化学指标分析。结果本组肿瘤发生部位为胃27例（34.6%）,小肠26例（33.3%）,其它部位25例（32.1%）。镜下梭形细胞为主型73例（93.6%）,上皮样细胞为主型1例（1.3%）,混合细胞型4例（5.1%）。免疫组织化学阳性表达结果：78例GIST中CD117、CD34、SMA、Desmin及S-100蛋白阳性表达分别为77例（98.7%）、58例（74.4%）、27例（34.6%）、1例（1.3%）及7例（9.0%）。结论 GIST是消化道较常见的间叶性肿瘤,CD117、CD34等联合检测可协助鉴别诊断GIST。     

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。     

Malignant giant cell tumor of soft parts. An ultrastructural study of four cases.

Four cases of malignant giant cell tumor of soft parts (MGCT) were studied ultrastructurally. Most of the cells in three cases were identified as undifferentiated mesenchymal cells and mononuclear monohistiocytic cells. The osteoclastlike cells seemed to arise from fusion of monohistiocytic cells. Immature fibroblastic cells, occasionally containing filaments with densities, were also present. They were scarce in three cases and predominant in the fourth. It was concluded that MGCT is a mesenchymal sarcoma with a bimorphic--monohistiocytic and fibroblastic--differentiation. MGCT is therefore considered a special variant of malignant fibrous histiocytoma.     

Glomangiosarcoma in a glomus tumor. An immunohistochemical and ultrastructural study

An extremely rare case of glomangiosarcoma (GS) occurring in a glomus tumor (GT) was evaluated ultrastructurally and histochemically. A man 65 years of age who was suffering from back pain underwent resection of a deep cutaneous nodule. Cells of a solid type GT showed numerous subplasmalemmal pinocytotic vesicles, thin filaments with scattered dense bodies, and thick external lamina, but negative desmin staining and a lack of glycogen. Similar findings also were observed in the GS, but were less obvious. The GS compressed the surrounding GT, exhibited many mitotic figures, prominent nucleoli, elongated nuclei and cytoplasm, and reacted more strongly to vimentin staining than the GT. The GT contained S-100 protein-positive Schwann cells, a few substance P-positive nerve fibers, and moderate numbers of infiltrating mast cells. None of these findings were observed in the GS. Results were consistent with the view that GS was transformed possibly from the GT, and that the good prognosis for GS may be due to its small size that may be related to the preexistence of a pain-causing GT.     

Low incidence of KIT gene mutations and no PDGFRA gene mutations in primary cutaneous melanoma: an immunohistochemical and molecular genetic study of Japanese cases

Background  

Although several studies have reported the incidence of KIT and PDGFRA gene mutations in cutaneous melanomas of Caucasians, only one report is available for Mongoloids, including Japanese. In Japan, melanoma is an uncommon neoplasm compared with countries with Caucasian populations.     

An immunohistochemical and electron microscopic study of atypical teratoid/rhabdoid tumor

We report two infant cases with atypical teratoid/rhabdoid tumor (AT/RT) located in the cerebellar vermis and spinal cord. MRI showed the tumors were isointense on T₁-weighted images and mixed intensity of isointense and slight high intensity on T₂-weighted images. Postcontrast MRI demonstrated clear margin of tumor and heterogeneous strong enhancement. It was difficult to differentiate the tumor from medulloblastoma by hematoxylin and eosin staining. However, immunohistochemical staining showed that these tumor cells react positively for cytokeratin, smooth muscle actin (SMA), and epithelial membrane antigen (EMA) and helped us with the differentiation. Electron microscopic study has confirmed the presence of mesenchymal components, such as filaments and desmosome junctions in the rhabdoid cells, but no neuronal components. The tumors rapidly increased in size, showing high MIB-1 index, and the prognosis was gave.     

Differential expression of neurofilament triplet proteins in carcinoid tumours: an immunohistochemical study

Neurofilaments (NFs) are specific intermediate filaments to neural cells. Mammalian NFs are protein triplets composed of three major subunits with respective molecular weights of approximately 70, 150 and 200 kD. Using an immunohistochemical method, 13 carcinoid tumours from different sites were examined for the presence of these three subunits by means of monospecific antisera. All tumours contained cells that were positive for the 70 Kd subunit; nine cases contained cells immunoreactive for the 150 Kd subunit and only three of them for the 200 kD subunit. The results indicate that the 70 kD subunit is a good overall marker of carcinoid tumours. The 150 and 200 kD subunits are more likely to be absent in carcinoids, both typical and atypical.     

Salivary gland carcinosarcoma: immunohistochemical, molecular genetic and electron microscopic findings

Salivary gland carcinosarcoma, or true malignant mixed tumor, is a very rare and extremely aggressive neoplasm. The clonality and clonal origin of this tumor are discussed controversially. We report a carcinosarcoma of the left parotid gland in a patient who subsequently died of cutaneous, lymphatic and pulmonary metastases. Immunohistochemical staining, electron micrograph analysis, loss of heterozygosity (LOH) analysis and sequence analysis were performed on this tumor with an adenocarcinomatous and a predominant spindle cell-like component. While smooth muscle actin was undetectable by immunohistochemistry, cytoplasmatic myoepithelial structures could be detected by electron microscopy. LOH analysis at 12 genomic locations detected complete deletion of one allele at 17p13.1, 17q21. 3, and 18q21.3 indicating allelic loss in both components of the tumor. Double strand sequencing of the remaining allele of the p53 tumor suppressor gene revealed a wild-type allele. Based on our results, we favor the hypothesis of monoclonal origin of this salivary gland carcinosarcoma with a common stem cell that could be the myoepithelial cell and an inactivated tumor suppressor gene on chromosome 17 other than p53.     

Solitary fibrous tumor: a clinico-morphological and immunohistochemical study

Solitary fibrous tumor is a rare neoplasm which may occur at any site although it is more frequent in the pleura, mediastinum and lung. The study used 4 cases of tumor localization in the pleura and orbit. Three cases presented as a "hemangiopericytic" variety of spindle cells; there were numerous giant cells in orbit tumor. Solitary fibrous tumor revealed enhanced expression of vimentin, CD34, bel-2 and CD99. Expression of S-100, desmine and non-striated muscle actin was found in few cells in some cases. Such features as large size (over 10 cm), necrosis, high cellularity, nuclear polymorphism and high mitotic index (more than 4 mitoses within 4 visual fields, at high magnification) were used as malignancy criteria:. Tumor histological pattern of "hemangiopericytic" variety could be reliably identified thanks to immunohistochemical procedure.     

Pulmonary carcinoid tumours: a clinico-pathological study of 35 cases

A clinico-pathological study of 35 bronchial carcinoid tumours was undertaken. Age, T stage, N stage, lymph node involvement, number of lymph nodes involved and number of cigarettes smoked per day were the clinical variables affecting survival. The histological variables related to survival were; mitotic count, necrosis, nuclear pleomorphism, vascular and lymphatic permeation and an undifferentiated growth pattern. All these features could be detected with routine histological stains, whereas immunocytochemical methods for demonstrating neuron specific enolase were of no help in assessing the prognosis. However there was a tendency for a well differentiated neuroendocrine carcinoma to stain strongly in some areas with carcinoembryonic antigen.     

Argyrophilic carcinoma "carcinoid tumor" of the breast: case report with electron microscopic and immunohistochemical investigations

A rare primary argyrophilic carcinoma "carcinoid tumor" of the breast in a 48-year-old woman was investigated by light and electron microscopy, and immunohistochemistry. Light microscopy showed the greater part of the tumor to have characteristic histological features of carcinoid tumor and Grimerius' stain revealed the presence of numerous argyrophilic granules in the tumor cells. Numerous neurosecretory granules and bundles of intermediate filaments were observed ultrastructurally in the cytoplasm. In addition, carcinoembryonic antigen (CEA) and neuronespecific enolase (NSE) were detected in the tumor cells using the immunoperoxidase method. From the results, it is speculated that the tumor cells have the ability to produce CEA as well as NSE in the cytoplasm. The observation of ductal spreading in parts of the tumor, and the detection of CEA, suggest the tumor cells to be derived from mammary epithelial cells.