鼻腔胚胎型横纹肌肉瘤6例并文献复习

<正>横纹肌肉瘤(rhabdomyosarcoma,RMS)是最常见的软组织恶性肿瘤之一,好发于儿童和青少年,主要发生于头颈部、腹膜后和泌尿生殖系统。成人横纹肌肉瘤很少见[1],而胚胎型横纹肌肉瘤(embryonalrhab-domyosarcoma,ERMS)是最常见的亚型,占横纹肌肉瘤的50豫~60豫,而成人鼻腔ERMS临床上更为罕     

All-trans-retinoic acid counteract the tumor-stimulating effect of hepatectomy and increases survival of rats bearing liver metastases

Background

We previously demonstrated a stimulating effect of hepatectomy on residual tumor cells after resection of liver metastases. The aim of this study was to analyze the effect of all-trans-retinoic acid (ATRA) on the protumor effect of hepatectomy and survival of hepatectomized rats bearing liver metastases. We also explored whether ATRA interfered with the tumor promoting effect of hepatotropic growth factors (GFs).

Methods

The in vitro effect of ATRA on proliferation of S4MH rhabdomyosarcoma tumor cells was assessed when cultured with laparotomized or hepatectomized rat serum (HRS), or in the presence of GFs (hepatocyte growth factor, insulin growth factor 2, Platelet Derived Growth Factor (PDGF)-BB, and vascular endothelial growth factor). For the in vivo studies, rats were partially hepatectomized on day 10 after metastasis induction, one group being treated with ATRA from day 7 to 14, and a second receiving cyclophosphamide (CY; on days 10 and 14) alone or with ATRA. We determined the size and number of liver and lung metastases. Finally, we analyzed the effect of treatments on rat survival.

Results

Hepatotropic GFs increased cell proliferation in a similar manner to HRS. In vitro, ATRA blocked the protumor effect of both HRS and GFs. In vivo, ATRA reduced the size and number of liver and lung metastases, and significantly increased rat survival. Furthermore, adding ATRA to CY significantly increased survival compared with CY alone.

Conclusions

In our model, ATRA minimizes the tumor-stimulating effect of hepatectomy, reducing the number and size of liver metastases and improving survival. The results suggest that the ATRA may be useful for blocking the growth-promoting effect of hepatotropic GFs released after liver metastasis resection.     

Hepatobiliary rhabdomyosarcoma mimicking choledochal cyst: Lessons learned

INTRODUCTION

The differential diagnosis of hepatic cystic lesions is a challenging process especially in case of hepatic rhabdomyosarcoma (HRMS) presenting as hepatic cyst.

PRESENTATION OF CASE

We introduce our experience with a case of HRMS in a 3-year-old female patient who was misdiagnosed to have type IV-A choledochal cyst and definitive correct diagnosis was reached after the pathological and immunohistochemical examination of the surgically resected lesion. This case presentation is followed by important practical messages to hepatobiliary surgeons regarding HRMS.

DISCUSSION

HRMS is a rare pediatric tumor. Jaundice is the most common presentation of HRMS followed by abdominal pain and vomiting. Great effort is needed to differentiate the tumor from choledochal cyst and infectious hepatitis. Through evaluation using available imaging studies together with clinical anticipation is mandatory for establishing the correct diagnosis.

CONCLUSION

Differentiation of HRMs from choledochal cyst mandates through evaluation and clinical anticipation. HRMS should be suspected in any child with obstructive jaundice. Once diagnosis is established, multidisciplinary treatment is the best management strategy and it has proved better surgical outcome and long term survival.     

Overexpression of GEFT,a Rho family guanine nucleotide exchange factor,predicts poor prognosis in patients with rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is one of the most common soft-tissue sarcomas in children and adolescents with poor prognosis. Yet, there is lack of effective prognostic biomarkers for RMS. The present study, therefore, aimed to explore potential biomarkers for RMS based on our previous findings using array comparative genomic hybridization. We investigated guanine nucleotide exchange factor, GEFT, at expression level in 45 RMS patients and 36 normal striated muscle controls using immunohistochemistry using tissue microarrays. The expression rate of GEFT in RMS samples (42/45, 93.33%) was significantly higher (P<0.05) than that in normal controls (5/36, 13.89%). Moreover, the overexpression rate of GEFT in RMS (31/45, 68.89%) was also significantly higher (P<0.05) than that in normal controls (0/36, 0.00%). Increased expression of GEFT correlated significantly with advanced disease stages (stages III/IV) (P=0.001), lymph node metastasis (P=0.019), and distant metastasis (P=0.004), respectively, in RMS patients. In addition, RMS patients having overexpressed GEFT experienced worse overall survival (OS) than those having low levels of GEFT (P=0.001). GEFT overexpression was determined to be an independent prognostic factor for poor OS in RMS patients (hazard ratio: 3.491, 95% confidence interval: 1.121-10.871, P=0.004). In conclusion, these observations provide the first evidence of GEFT overexpression in RMS and its correlations with disease aggressiveness and metastasis. These findings suggest that GEFT may serve as a promising biomarker predicting poor prognosis in RMS patients, thus implying its potential as a therapeutic target.     

Small Bowel Perforation Caused by Metastasis from an Extra-Abdominal Malignancy: Report of Three Cases

Small bowel perforation is rarely caused by metastasis from an extra-abdominal malignancy. This report describes three cases of small bowel perforation that occurred secondary to a metastatic tumor. The first case involved a 72-year-old man with malignant lymphoma of the larynx that had been treated with chemo- and radiation therapy; the second involved a 70-year-old man with rhabdomyosarcoma of the mediastinum that had been treated with radiation therapy; and the third involved a 41-year-old man with lung carcinoma that had been treated with surgery 10 months prior to perforation. Each patient presented with acute abdominal pain, had X-ray findings of free air in the abdomen, and underwent limited emergency surgery. Wedge resection and closure of the ileum was performed for the first patient and partial bowel resection with the creation of an intestinal stoma was performed for the second and third patients. In each case, the histologic findings of the resected specimens were consistent with the extra-abdominal primary tumors. Although the patients recovered sufficiently to begin eating and moving about, all three died of cancer or cancer-related complications within 45 days of surgery. We conclude that surgeons should be aware of the poor prognosis of such patients and perform only the minimal surgery required. Received: April 7, 2000 / Accepted: September 26, 2000     

Embryonal rhabdomyosarcoma of the ampulla of Vater in early childhood: report of a case and review of literature

Embryonal rhabdomyosarcoma of the ampullary region is a very rare childhood tumor (2 reported cases), and herein we describe a child presenting with obstructive jaundice at early age owing to such tumor in the ampullary region. Successful management with multidisciplinary approach is discussed with reference to the literature.     

Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases

Rhabdomyosarcoma (RMS) is currently classified into embryonal RMS, including its botryoid and spindle cell variants, alveolar RMS, including a solid variant, and pleomorphic RMS. In children and adolescents embryonal RMS occurs in a younger age group than alveolar RMS, and pleomorphic RMS is almost always seen in older adults. Most recently rare spindle cell and sclerosing, pseudovascular RMS have been reported in adults as well. We analysed the clinicopathological and immunohistochemical features of seven new cases of spindle cell RMS arising in adult patients. Five patients were male and two were female and the age of the patients ranged from 38 to 76 years. Four neoplasms arose on the lower extremities and one case each on the forearm, the lateral aspect of the neck and the penis. Five neoplasms were completely excised, in one incompletely excised neoplasm additional chemotherapy was given, and in one patient a biopsy was done only so far. All neoplasms arose in subcutaneous and deep soft tissues with dermal involvement in one case, and the size of the neoplasms ranged from 4 to 19 cm in largest diameter. Histologically, a plump or diffuse infiltration was seen, and all neoplasms were mainly composed of cellular bands and fascicles of atypical spindle-shaped tumour cells containing enlarged and atypical nuclei associated with a variable number of rhabdomyoblasts. In addition, focal areas reminiscent of sclerosing, pseudovascular RMS were noted in three cases, and in two cases each small solid areas with pleomorphic tumour cells as well as scattered round tumour cells were present. Proliferative activity ranged from 1 to 60 mitoses in 10 high-power fields and tumour necrosis was evident in four cases. Immunohistochemically, all neoplasms tested stained variably positive for desmin, myf-4, WT1 and CD 99, whereas fast myosin was positive in only two out of seven cases. In addition, five out of seven cases tested stained focally positive for alpha-smooth muscle actin. The remaining antibodies (h-caldesmon, S-100 protein, CD 34, pancytokeratin and epithelial membrane antigen) were all negative. Follow-up information was available in five patients (range from 10 to 48 months) and revealed lung metastases in two patients who died of disease within a short period. In summary, spindle cell rhabdomyosarcoma represents a rare neoplasm in adulthood characterized clinically by a rather poor prognosis, and shows a broad morphological spectrum including most likely the sclerosing, pseudovascular variant. Immunohistochemically, tumour cells in RMS stain positively for CD 99 and WT1 as well, which is of importance in the differential diagnosis to other mesenchymal neoplasms, whereas fast myosin does not represent a reliable marker for RMS in adults.     

Embryonal rhabdomyosarcoma of the omentum: two cases occurring in children

Rhabdomyosarcoma is a soft-tissue malignancy that represents approximately 4–8% of all solid tumours in children and commonly arises from the head and neck and genitourinary system. Intraperitoneal rhabdomyosarcoma, in particular with omental involvement, has been rarely reported in the literature. Furthermore, reports of omental rhabdomyosarcoma of embryonal origin do not exist, to our knowledge. We report two cases of omental embryonal rhabdomyosarcoma affecting children and illustrate the imaging characteristics of this rare tumour.     

Literature Review of Benign Müllerian Papilloma Contrasted With Vaginal Rhabdomyosarcoma

Study ObjectivesBenign müllerian papillomas of the genital tract are rare and, hence, can be mistaken for vaginal rhabdomyosarcoma on initial clinical review. This review of the literature will consolidate the previous cases of müllerian papilloma reported and looks for clues to differentiate the 2 entities.Design and SettingWe provide a case report and literature review, with patients from a pediatric adolescent gynecology clinic in a tertiary center.MethodsWe conducted a search of English-language publications from 1951 (the first case report) until January 2014 by using the search words “Müllerian papilloma” and “prepubertal bleeding.” References from previous published reports were also obtained for completeness.Main OutcomeLiterature review of benign müllerian papilloma.ResultsSince 1951, 56 cases of müllerian papilloma were reported, including 4 cases at our institution. Comorbid conditions were found in 31.5% of cases (with 3 cases associated with mesenchymal tumors). The average length of time from onset of symptoms (primarily vaginal bleeding) to diagnosis was 6.7 months (range, 1 day to 3 years), with only 1 case diagnosed incidentally. Median age of presentation was 5 years (range, 1 day to 52 years). Most cases were localized and resected with ease. Histology reveals complex papillary lesions without cytologic atypia.ConclusionBenign müllerian papilloma is distinguished from the more significant diagnosis of vaginal rhabdomyosarcoma by initial length of vaginal bleeding at presentation, lack of vaginal wall extension, ease of resection, and histopathology. This is compared with vaginal rhabdomyosarcoma which commonly exhibits both localized and distant spread.     

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