横纹肌肉瘤双侧乳腺转移一例

患者女,14岁.因发现右侧鼻翼肿物1年余,于2006年12月收入我院头颈外科治疗.查体:右鼻翼肿物,色红,约4 cm×3 cm,肿物侵及右鼻前庭、下鼻甲前端及双侧颌下,颈深Ⅱ、Ⅲ区可触及肿大淋巴结,直径约0.5～1 cm不等.北京协和医院皮肤科活检病理提示:间叶组织肿瘤,横纹肌肉瘤可能大.免疫组化:vimentin(++),desmin(+),Ki67抗原标记指数为30%,AE1/AE3(-),EMA(-),CEA(-).     

Recent experience in the treatment of rhabdomyosarcoma.

Twenty-two patients with rhabdomyosarcoma have presented in the years 1970--1977. Of 8 patients who had inadequate postoperative chemotherapy and radiotherapy, or none at all, only 2 patients with small completely resectable tumors are alive and well at 6 and 4 yr after surgery. Fourteen patients had intensive combination chemotherapy in addition to appropriate surgery and radiotherapy. Five have died and the remaining nine are well and free of disease at follow up ranging from 9 mo to over 5 yr. A continued place in the treatment regime is advocated for early surgery aimed at total excision of the primary tumor where this is feasible.     

Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation

Twenty-three patients with parameningeal (including orbital rhabdomyosarcoma (RMS)) were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and January 1983. Twenty were children with a mean age of 6 and 3 were adults. In 6 patients, the primary tumor was from the orbit, whereas the remaining 17 had other parameningeal primary sites. The tumors were in a very progressive local stage, with extensive destruction of the facial bones in 19 patients. Eight patients were treated with T2 chemotherapy protocol and 15 received T6. Seven patients received 5,000 to 7,200 rad delivered to the primary tumor in 11-16 weeks, 15 patients received between 4,500 to 5,000 rad in 4-7 weeks, and 1 patient received 3,000 rad in 3 weeks for residual microscopic disease following surgery. Two patients were treated with radiation to the whole brain; no patients received radiation of the whole central nervous axis (CNA). Fifteen of the 23 patients (65%) are alive and well with a medical follow-up time of 5 years. Two patients died of therapeutic complications and six died of tumor spread. In five patients, involvement of the central nervous system (CNS) was the cause of death. The prognosis of orbital RMS with parameningeal involvement is no better than in other tumors of parameningeal sites. In those patients who had impaired vision because of optic nerve damage prior to treatment, the vision did not improve following treatment. There was no impaired vision seen due to radiation damage of eye structures except in the lens.     

Vaginal tumors in childhood: the experience of St. Jude Children's Research Hospital

Background/Purpose

The aim of this study was to retrospectively analyze the clinical presentation, histology, treatment, and outcomes of children with vaginal tumors who were treated at a single institution.

Methods

A retrospective review of medical records and pathologic materials of all children with vaginal tumors treated at St Jude Children's Research Hospital between 1970 and 2009 was conducted.

Results

Eighteen patients (median age, 3.7 years; range, 0.1-15 years) were identified. Three different histologies were found: rhabdomyosarcoma (RMS; n = 13), germ cell tumor (n = 3), and clear cell adenocarcinoma (n = 2). Bleeding or blood-tinged discharge was the most common clinical presentation (66%), followed by a protruding mass (39%). Vaginal and uterine salvage was 44.4% (8 of 18 patients). Thirteen patients (72.2%) remain disease-free, with a median follow-up of 23.2 years (range, 2-39 years). Four patients (22.2%) died of disease progression (1 RMS, 2 germ cell tumor, and 1 clear cell adenocarcinoma), and 1 patient with RMS died of colon cancer 12 years after the primary diagnosis had been made.

Conclusions

Vaginal tumors are extremely rare in the pediatric population. Early recognition of symptoms like bleeding and a protruding vaginal mass may prevent morbidity and mortality. Our findings confirm the good prognosis of vaginal RMS.     

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     

Major resection for embryonal rhabdomyosarcoma of the biliary tree

Embryonal rhabdomyosarcoma of the biliary tree is a very rare tumor. Only 56 patients have been reported in the literature; 17 of them were long-term survivors. The recommended therapy is multidisciplinary: according to the Intergroup Rhabdomyosarcoma Study I and II (IRS I and II), the combination of major resection, chemotherapy, and radiation of the porta hepatis could improve the results. This report reviews our experience with four consecutive patients from a surgical point of view. We conclude that the classical definition of resectability cannot be applied to rhabdomyosarcomas of the biliary tree, because these polypoid tumors as a rule extend into the liver sectors and often affect both halves of the liver. Major resection with atypical reconstruction of the biliary tree is necessary in order to provide some promise of success. The problem of preoperatively determining the extent of the tumor is very important, as even intraoperative cholangiography cannot accurately demonstrate the true dimensions of the tumor in the sectorial bile ducts. The advantages and disadvantages of the procedures that come into question are discussed.     

Primary renal botryoid rhabdomyosarcoma: diagnosis and outcome

Primary renal rhabdomyosarcoma is a rare entity. We report on a pediatric patient who, despite having multiple metastases to the lung on presentation, is free of disease 28 months after radical nephrectomy combined with chemotherapy and radiation therapy.     

Efficacy of topotecan plus vincristine and doxorubicin in children with recurrent/refractory rhabdomyosarcoma

Background This study investigates the efficacy and the feasibility of a chemotherapy regimen with topotecan plus vincristine and doxorubicin (TVD) given on an individually tailored basis to patients with refractory/recurrent rhabdomyosarcoma (RMS). Patients and methods Nine patients received TVD therapy at relapse, and six were assessable for response. Results All the six patients experienced objective response after two cycles of chemotherapy: one minor response, four partial response, and one complete response. Conclusions The value of our study is severely limited by the small number of cases, the single-institutional setting and the individually tailored treatment, but we nonetheless confirmed the feasibility and tolerability of topotecan-based chemotherapy in RMS.