Nasopharyngeal alveolar rhabdomyosarcoma expressing CD56: a mimicker of extranodal natural killer/T-cell lymphoma

Alveolar rhabdomyosarcoma (ARMS) is remarkably rare in adults older than 45 years. Histologically, the tumor is composed of blue round cells with frequent expression of CD56 in addition to myogenic markers. Recent studies of ARMS have shown two specific recurrent translocations: PAX3-FKHR [t(2;13)(q35;q14)] or PAX7-FKHR [t(1;13)(p36;q14)]. Extranodal natural killer (NK)/T-cell lymphoma (ENKTL) occurs most frequently in the upper aerodigestive tract with a male preference in East Asia and Central and South Americas with neoplastic cells frequently expressing CD56. We report a 53-year-old Taiwanese man presenting with a nasopharyngeal mass, cervical lymphadenopathy, and multiple bone metastases. Histologically, the nasopharyngeal biopsy revealed diffuse sheets of small blue round tumor cells without obvious alveolar pattern, angioinvasion or tumor necrosis. An initial erroneous diagnosis of ENKTL was made due to CD56 expression using fresh tumor tissue with flow cytometric analysis and the patient was treated accordingly. Retrospective study showed that the tumor cells expressed CD56, desmin, and myogenin. Fluorescence in situ hybridization revealed that the tumor cells were positive for FKHR gene rearrangement, confirming the diagnosis of ARMS. Our case illustrates that a diagnosis of ENKTL based solely on CD56 expression can be misleading for a nasopharyngeal small blue round cell tumor. ARMS should be included as a differential diagnosis, and a correct diagnosis can be reached only after a high index of suspicion and a thorough histological examination with the aid of ancillary studies.     

CD56与神经内分泌检测对小细胞肺癌诊断应用价值的比较

目的比较CD56与常用神经内分泌标记对小细胞肺癌诊断应用价值。方法应用免疫组织化学法检测72例经支气管内窥镜和穿刺的小细胞肺癌（SCLC）和20例非小细胞肺癌（NSCLC）活检标本、12例肺炎性假瘤、10例淋巴细胞性胸腺瘤、10例淋巴结转移性小细胞肺癌和20例小B细胞性淋巴瘤标本中CD56、神经元特异性烯醇化酶（NSE）、嗜铬粒蛋白A（CgA），突触素（Syn）和其他抗体的表达。结果SCLC中CD56与NSE、CgA和Syn之间阳性表达率差异有统计学意义（P〈0．01）。CD56和其他抗体可以作为SCLC诊断和鉴别诊断的标记。结论CD56对活检的小细胞肺癌阳性率高，可作为临床病理诊断重要的标记物。     

Inner ear immunology

Previously the authors proposed that the perilymphatic inner ear immune system is independent of that of the cerebrospinal fluid. In the present study, the effect of dilatation of blood vessels surrounding the cochlea of chinchillas on the transfer of serum antibodies to the perilymph was tested. The possibility of local production of antibodies in the perilymphatic space was also investigated by antigen introduction into the inner ear through the facial nerve canal. The dilatation of blood vessels accelerated the transfer of serum antibodies to the perilymph, even though this transfer activity was limited. Results of the study, showing the effect of antibody production in the perilymphatic space upon introduction of antigen into the inner ear through the facial nerve canal, would deny local antibody production in the inner ear, particularly in the perilymphatic space.     

肺癌组织中MOC-31和CD56的检测及其意义

背景与目的：国外多数研究发现,由于MOC－3在肺癌组织中的阳性率明显高于胸膜间皮瘤的阳性率,因此认为MOC－3对两者鉴别诊断有重要意义,但也有少数相反的报道。CD56（神经细胞粘附分子neural cell adhesion molecule)对小细胞肺癌的敏感性高,对非小细胞肺癌的敏感性则报道不五。本研究旨在探讨MOC－3及CD56检测在肺癌诊断中的意义。方法：采用免疫组织化学（S－P）方法对92例肺癌组织吕MOC－31及CD56进行同步检测。结果：MOC－31在肺癌中的阳性率为98．9％（91／92）,其中在非小细胞肺癌（non-small cell lung carcinoma NSCLC)中的阳性率为98．6％（72／73）,在小细胞肺癌（smal cell lung carcinoma,SCLC)中的阳性率为100％（19／19）。CD56在肺癌中的阳性表达率为30．4％（28／92）,其中在NSCLC中的阳性表达率为12．3％（9／73）,在SCLC中的阳性表达率为100％（19／19）,MOC－31及CD56在癌旁肺组织及正常肺组织中均未见阳性反应。结论：MOC－31是检测肺癌的一个敏感性高特异性较强抗体。而CD56仅对SCLC有敏感性。MOC－31与CD56联合检测,对诊断SCLC更有意义。     

小儿CD56~+急性髓系白血病临床生物学特征

目的研究CD56+小儿急性髓细胞白血病(AML)的生物学特征及临床意义。方法对80例初治小儿AML进行细胞形态学、免疫表型、多药耐药P糖蛋白(P170)检测、临床观察、并常规采用HAE方案诱导治疗,判定疗效。结果综合我中心8年间小儿急性髓系白血病资料分析,CD56阳性率20.69%(30145)。CD56+患儿在FAB分型中以M2b、M5、M7多见,CD56+组中免疫表型特征为多表达CD34(85.00%和44.11%P=0.01)。HLA-DR(76.66%和42.00%P=0.003)。和PgP(85.71%和25.58%P=0.001)。此外,CD56+AML患儿髓外浸润现象明显(77.27%和45.00%P=0.017),尤其是淋巴结(59.09%和22.50%P=0.006)和脾脏(63.63%和22.50%P=0.002)受累显著,CD56表达与年龄、性别、白细胞计数、血红蛋白含量、血小板计数、及外周血幼稚细胞数无关,也不影响CR率和无病生存时间(DFS),但达首次缓解时间较长,中位时间56天。结论CD56+AML具有独特的临床生物学特征,生物学侵袭性较强,多表达耐药蛋白P170,预后较差。建议初诊时监测CD56分子表达以判断预后。     

The 56 kd platelet-derived growth factor (PDGF)-related protein is phosphorylated and the most stable form in human glioma cells

We report herein the presence of a 56 kd platelet derived growth factor (PDGF)-related protein as a phosphorylated form in human glioma cells. The phosphorylation of the 56 kd form was found to be the longest of all PDGF-related proteins. By Western blotting using a monoclonal anti-PDGF B-chain, the 80 kd, 56 kd, 40 kd, 28 kd and 17 kd PDGF-related proteins were detected, while after treatment among the nitrocellulose membrane transblotted cell extracts with alkaline phosphatase, 40 kd was the most densely observed while the 56 kd and 80 kd PDGF-related proteins were also detected. In a ³²P flush labeling study, it was revealed that PDGF-related proteins incorporated with ³²P were detected at 28, 32, 35, 40, 56 and 80 kd but the 17 kd monomer was not labeled. Among the labeled PDGF-related proteins, the 56 kd PDGF-related protein alone remained intracellularly for at least 16 hours. These results indicated that the PDGF-related proteins in human glioma cells are synthesized in a phosphorylated form and partly remain in a 56 kd phosphorylated form intracellularly. The 56 kd form may thus be the most stable form and likely has a substantial biological effect.     

Primary extramedullary plasmacytoma and multiple myeloma: phenotypic differences revealed by immunohistochemical analysis

Primary extramedullary plasmacytomas are infrequent, typically solitary, plasma cell neoplasms that generally pursue an indolent clinical course but may, rarely, convert to multiple myeloma. Phenotypic differences between these two entities are not well defined. Twenty-eight cases of primary extramedullary plasmacytoma and 26 cases of both medullary (n = 17) and extramedullary (n = 9) multiple myeloma were analysed for the expression of proteins known to play a role in the biology of multiple myeloma. Immunohistochemistry was performed on paraffin wax sections using antibodies against cyclin D1, Bcl-2, Bcl-xL, p27, p21, p53, MIB1, CD20, and CD56. Twenty-three extramedullary plasmacytomas were localized in the upper aerodigestive tract, four in the lymph nodes, and one in the testis. There was a strong male predominance (M : F = 6 : 1). None of the patients died from the disease or progressed to multiple myeloma (mean follow-up 50 months). Nine patients developed local relapse and one patient's tumour evolved into a B-cell non-Hodgkin's lymphoma. In contrast to both intra- and extra-medullary multiple myeloma, extramedullary plasmacytoma showed absence of cyclin D1 (p < 0.001) and infrequent expression of CD56 (p < 0.001). Furthermore, extramedullary plasmacytomas were characterized by weaker staining for Bcl-2 protein and rare overexpression of p21 and p53. In comparison to extramedullary multiple myeloma, extramedullary plasmacytoma showed a more mature morphology and lower proliferation indices (p = 0.008). There was no association between the phenotypic parameters investigated and clinical outcome in extramedullary plasmacytoma. In summary, extramedullary plasmacytoma and multiple myeloma show significant immunophenotypic differences, some of which may be of both diagnostic utility and biological relevance.     

Influence of iron (56Fe2O3 or 54Fe2O3) in the upregulation of cytochrome P4501A1 by benzo[a]pyrene in the respiratory tract of Sprague-Dawley rats

Any influence of iron in polycyclic aromatic hydrocarbon (PAH)/iron oxide mixtures on the capacity of PAHs to induce metabolizing enzymes will be one of the ways that iron oxides can affect PAH carcinogenicity. Because cytochromes P450 (CYPs) are haemoproteins, it will be of interest to investigate the possible involvement of Fe(2)O(3) in benzo[a]pyrene (BaP)/Fe(2)O(3) mixtures on the induction of CYP1A1 enzymes in the lung. Male Sprague-Dawley rats were instilled intratracheally with haematite ((56)Fe(2)O(3) or (54)Fe(2)O(3), 3 mg), BaP (3 mg) or BaP (3 mg) coated onto haematite ((56)Fe(2)O(3) or (54)Fe(2)O(3)) particles (3 mg). Firstly, mRNA expressions of cyp1a1 were studied. Secondly, protein concentrations and catalytic activities (7-ethoxyresorufin O-deethylase: EROD) of CYP1A1 were determined. Thirdly, (54)Fe from BaP/(54)Fe(2)O(3) mixtures in microsomal proteins was studied using time-of- flight laser microprobe mass spectrometry (ToF-LMMS). Statistically significant increases in mRNA expressions, protein concentrations and catalytic activities of CYP1A1 were observed in animals exposed to BaP, to BaP coated onto (56)Fe(2)O(3) particles or to BaP coated onto (54)Fe(2)O(3) particles versus controls. Both of the BaP/Fe(2)O(3) mixtures induced higher CYP1A1 protein levels and EROD activities than BaP alone. Iron oxide particles per se did not affect mRNA levels of cyp1a1 but only enhanced BaP-mediated increases of CYP1A1 protein levels and activity. The ToF-LMMS spectrum pro fi les showed that the (54)Fe/(56)Fe ratio in the microsomes of BaP coated onto (54)Fe(2)O(3) particle-instilled animals was 1.3 instead of the theoretical ratio (i.e. 0.063) observed in BaP coated onto (56)Fe(2)O(3) particle-instilled animals. Taken together, these novel data support the hypothesis that the Fe(2)O(3)-induced increases of the metabolic activation of BaP might rely on the property of Fe(2)O(3) particles to enhance the BaP-induced translation rate of the cyp1a1 gene into functional haemoproteins.