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     

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.     

Pelvic rhabdomyosarcoma in children

From 1961 to 1986, 29 patients with pelvic rhabdomyosarcoma (including pelvic retroperitoneal rhabdomyosarcoma) were admitted to the paediatric surgical service at the Red Cross Children's Hospital, Cape Town. During the period 1961–1970, the mainstay of treatment was radical surgery with or without subsquent chemotheraphy and radiotherapy. Of the 13 patients, only 2 have survived long-term (15%). From 1971 a new management policy of pre-treatment with chemotherapy and in some instances radiotherapy followed by conservative limited surgery was commenced. Twelve of the 16 patients have survived (75%). One patient with stage III prostatic tumour had treatment with only chemotherapy and radiotherapy and is surviving 8 years after diagnosis; another with a vaginal tumour, also stage III, whose parents refused surgery is alive tumour-free 4 years after similar treatment. Chemotherapy has not only significantly improved survival in pelvic rhabdomyosarcoma but has permitted limited surgery and in some instances has resulted in survival without surgical excision. Offprint requests to: S. Cywes     

Neoplastic diseases of the head & neck in children

Pediatric neoplasm is next only to trauma as the most common cause of death. The cervicofacial malignancies presents a unique challenge owing to potential adverse effects of both the disease process and the treatment employed on critical developing head and neck structures. This study comprised of 106 children below 12 years age group conducted during 1999–2003 at JNMC, Aligarh. Of 106 cases 70 were benign neoplasms and 36 malignant neoplasms. Nasopharynx was the commonest site of involvement. Nasopharyngeal angiofibroma was the commonest benign tumour and lymphoma was the commonest malignant neoplasm. Team approach is required for mitigation of the disease process.     

眼眶胚胎性横纹肌肉瘤一例

患者男,11岁。2002年10月发现左眼眶下侧肿胀,自行扪及下眶缘一绿豆大小硬结,右眼无异常。在外院手术切除肿物后,未予治疗。2个月后再次发现左下眼眶肿胀,并扪及一约0．5 cm×0．3 cm大小结节,再次于外院手术切除,并     

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.