A clinical study of nasopharyngeal masses

Out of 36511 patients attending the ENT OHD of R. G,. Aur Medical Callege & Hospital. Kolkata, in one year 0.08% patients were found tuning naspharyngeal mass. 30 cases of nasopharyngeal mass were studied. The peak incidence of nasopharyngeal mass was in the age group between II to 20 years (40%). The incidence in males (73%) was more than females(27%). The maximum ineldence was found among students (74%). Commonest ntuopharyngeal nuns teas antrochoanal polyp (30%). Next commlon masses were adenoids (23%)Juvenile angwfibroma(20%) and nasophartngeal carcinoma (13%). Nasal obstruction was the main presenting symptom(83%) followed by epistaxis (40%).     

Unusual course of an epidural rhabdomyosarcoma of the upper thoracic spine

Summary This report deals with a case of rhabdomyosarcoma in the upper thoracic spine. It is of particular interest, not only for the rarity of type and location of this tumour, but for its clinical course, which presented fluctuations of neurological status, included an acute demonstration of complete paraplegia followed by full recovery after conservative treatment, and gradual relapsing of neurological deficit, one year later.     

Characteristics of rhabdomyosarcoma cell lines derived from uterine carcinosarcomas

 Rhabdomyosarcoma (RMS) is occasionally found in the female genital tract, and mostly appears as one of the heterologous mesenchymal components in uterine carcinosarcoma designated as malignant mixed müllerian tumour (MMMT). We examined the biological properties of a pure rhabdomyosarcoma (RMS) cell line designated FU-MMT-3, which was newly established from a surgical specimen taken from a patient with uterine MMMT. We also evaluated c-myc and MYCN gene amplification in three RMS cell lines (including FU-MMT-3) derived from three MMMTs by Southern blot analysis. FU-MMT-3 cells were propagated continuously for 57 serial passages over a 2-year period in vitro. FU-MMT-3 was able to produce tumours demonstrating pure RMS in athymic nude mice. Cytogenetically, FU-MMT-3 showed a triploidy pattern, with complex karyotypic abnormalities including trisomy of chromosome 8. All three RMS cell lines, including FU-MMT-3, showed amplification of the c-myc gene (approximately fourfold to eightfold), while no cell lines demonstrated MYCN gene amplification. FU-MMT-3 is considered to provide a useful system for the study of the biological behaviour of RMS in MMMTs. Extra copies of chromosome 8 and c-myc gene amplification may be associated with the rhabdomyoblastic differentiation in MMMT. Received: 7 January 1997 / Accepted: 2 May 1997     

Embryonal rhabdomyosarcoma of foot with expansive growth between metatarsals

 The case of a 14-year-old girl with rhabdomyosarcoma of the right foot is reported. Plain radiography showed a large nonspecific soft tissue tumor between the metatarsals with bowing of the metatarsals away from the mass. MR imaging showed a large soft tissue mass involving the metatarsals. The findings were conflicting, because the tumor had an infiltrative soft tissue mass and bowing of the metatarsals more suggestive of slow expansive growth. Bowing of short tubular bones may be a process similar to cortical saucerization, which is typically seen in Ewing’s sarcoma, and it can be one of the findings of high grade neoplasms, such as embryonal rhabdomyosarcoma.     

Indium111 antimyosin for the detection of leiomyosarcoma and rhabdomyosarcoma

¹¹¹In-antimyosin monoclonal antibody complex passes through damaged myocardial cell membranes and binds to the intracellular myosin. Normal myocardial and other muscle cells show no uptake.Rhabdomyosarcoma and Leiomyosarcoma cells also contain intracellular myosin.and the cell membrane permeability is greater than normal. Significant uptake of ¹¹¹In-antimyosin was observed in patient with Leiomyosarcoma and Rhabdomyosarcoma suggesting that the reagent has a potential for the in vivo detection of these tumour types. Tumour to background ratios of 10:1 were measured in one case and in view of the fact that the site of accumulation is intracellular, antimyosin may have a potential as a carrier for therapeutic agents.     

Rhabdomyosarcomatous differentiation in a neuroblastoma: a potential pitfall in the cytologic diagnosis of small round-cell tumors of childhood

Rhabdomyoblasts demonstrating immunoreactivity for muscle-specific actin, desmin, and myoglobin were identified in smears obtained by aspiration from a large retroperitoneal mass in a 14-mo-old girl. Following a tentative diagnosis of a rhabdomyogenous neoplasm, retroperitoneal exploration and adrenalectomy demonstrated a stromal poor neuroblastoma with extensive rhabdomyogenous differentiation. The presence of a subpopulation of rhabdomyoblasts was not diagnostic of rhabdomyosarcoma when obtained by fine-needle aspiration from a retroperitoneal tumor composed predominantly of primitive small round cells.     

利用比较基因组杂交技术分析横纹肌肉瘤中染色体基因组的变化特征

目的 探讨横纹肌肉瘤(RMS)基因组DNA的变化特征.方法 在一步法RT-PCR检测所有标本PAX3/PAX7-FKHR mRNA融合基因表达情况的基础上,应用比较基因组杂交技术分析25例原发性RMS患者,其中腺泡状横纹肌肉瘤(ARMS)10例,胚胎性横纹肌肉瘤(ERMS)12例,多形性横纹肌肉瘤(PRMS)3例.根据融合基因表达情况、组织学分型、临床分期、组织学分级、性别和年龄进行分组比较.同时收集RMS细胞株A204(腺泡型)、RD(胚胎型)作为对照.结果 25例RMSCGH分析结果显示:(1)RMS中发生DNA拷贝数扩增最常见的部位是2p和12q,其他依次为6p、9q、10q、1p、2q、6q、8q、15q和18q(30%),发生DNA拷贝数丢失最常见的部位是3p、11P和6p(30%).(2)ARMS扩增最常见的染色体臂是12q、2p、6、2q、4q、10q和15q(30%),缺失最常见的染色体臂是3p、6p、1q、5q(30%);ERMS扩增最常见的染色体臂是7p、9q、2p、18q和1 p、8q(30%),缺失最常见的染色体臂是11p.基因组变化在不同的组织学分类中差异无统计学意义(P0.05).(3)伴有融合基因组扩增最常见的染色体臂是12q、2、6、10q、4q和15q(30%),缺失最常见的染色体臂是3 p、6p、5q(30%);不伴有融合基因组扩增最常见的染色体臂是2p、9q、18q(30%),基因组缺失最常见的染色体臂是11p和14q(30%).12q扩增在这两组间的差异有统计学意义(P<0.05),并多见于伴有融合基因的RMS.(4)在临床分期分组中,9q扩增在Ⅱ期和Ⅲ～Ⅳ期间差异有统计学意义(P<0.05),且多见于Ⅱ期患者.结论 (1)2p、12q、6p、9q、10q、lp、2q、6q、8q、15q、18q扩增及3p、11p、6p缺失可能与RMS发病相关;(2)12q扩增可能与融合基因相关;(3)9q扩增可能与RMS发病早期有关.     

RHABDOMYOSARCOMA WITH BONE MARROW METASTASIS SIMULATING ACUTE LEUKEMIA

Rhabdomyosarcoma manifested as a systemic disease is very rare and cases showing diffuse metastasis in the bone marrow are most unusual. Recently we encountered two cases of rhabdomyosarcoma with diffuse bone marrow metastasis which were clinically manifested as acute leukemia. The first patient was a 15-year-old female, who was admitted in 1982 with pancytopenia and many large primitive cells in bone marrow aspirates, hematological malignancy being diagnosed. Thereafter the bilateral breasts showed rapid swelling and a biopsy specimen revealed the histological features of typical alveolar rhabdomyosarcoma. The primary site of the neoplasm remained undetermined during the course. At autopsy, it was disclosed that the neoplasm originated from the left thigh and showed generalized metastasis. The second patient was a 38-year-old man, who was admitted in 1986 because of a nasal polyp obstructing the nasal cavity, and persistent nasal bleeding. Peripheral blood samples showed leucoerythroblastosis and thrombocytopenia, and large primitive cells were found In bone marrow aspirates, so that hematological malignancy was initially diagnosed. A biopsy specimen of the nasal polyp showed proliferation of large round cells and electron microscopy demonstrated the ultrastructural features of rhabdomyosarcoma.     

三磷酸腺苷对人横纹肌肉瘤细胞系诱导分化作用的研究

为了探讨三磷酸腺苷（ＡＴＰ０对人横纹肌肉瘤细胞增殖和分化的影响，用ＡＴＰ作用于人横纹肌肉瘤细胞亚系（ＲＤＬ６）细胞，观察到ＡＴＰ可抑制ＲＤＬ６细胞的增殖，使其生长速度明显减慢，作用第５ｄ时增殖抑制率为８１％，流式光度术检测；观察到ＡＴＰ ＲＤＬ６细胞Ｓ期的细胞数明显增多，说明细胞停滞在Ｓ期，用罗氏黄荧光染料传法实，ＡＴ家恢复ＲＤＬ６细胞间隙加接通讯功能的作用。用 光细胞化学方法观察到经ＡＴＰ处理后     

儿童横纹肌肉瘤"上海儿童医学中心Rs-99方案"临床报告

目的　改善儿童横纹肌肉瘤、生殖细胞性肿瘤、Ewing’s肉瘤等恶性实体瘤预后。 方法　对 1998年10月至 2 0 0 1年 10月明确诊断的横纹肌肉瘤及其它软组织肉瘤、生殖细胞性肿瘤、Ewing’s肉瘤患儿共 17例 ,采用上海儿童医学中心Rs 99方案 ,即外科手术、内科化疗、选择性放疗、病理科与影像学科协作诊断、综合治疗。按方案中条件根据危险因素进行分组 ,并按分组给予分级治疗。结果　 17例恶性实体瘤患儿中 4例自行终止治疗并失访 ,12例获完全缓解 ,1例部分缓解 6个月后进展死亡。随访至 2 0 0 2年 9月 ,12例获缓解者中 1例 2 2个月时复发后死亡 ;1例 2 4个月时复发带病生存至今 35个月。另 10例持续缓解 15～ 4 6个月 ,平均为 2 8个月 ,其中 9例已停药平均 17个月。结论　Rs 99方案诊断及治疗对横纹肌肉瘤、生殖细胞性肿瘤、Ewing’s肉瘤有效。