No abstract available.
Radiotherapy* ; Retinoblastoma* ; Rhabdomyosarcoma*

Rhabdomyosarcoma originating from the chest wall is a rare malignant tumor in children and was considered to be guarded in prognosis previously. However, recent advances in multidisciplinary treatment of rhabdomyosarcoma in children have improved the disease free survival rate. Authors report a case of alveolar rhabdomyosarcoma, stage III, originating from the chest wall who is surviving free of disease for 15 months with aggressive chemotherapy and radiotherapy.
Child ; Disease-Free Survival ; Drug Therapy ; Humans ; Prognosis ; Radiotherapy ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar* ; Thoracic Wall* ; Thorax*

Rhabdomyosarcoma which was first described by Weber has generally been considered an uncommon tumor of striated muscle. Recently it was classified as four types as embryonal, alveolar, pleomorphic, and botryoid type by Horn and Enterline. A fourth type, sarcoma botryoides was generally recognized as a variant of the embryonal type. Al1 of these tumors tend to have a short clinical course, but the survival time seems to be slightly improved by sugical excision followed by radiotherapy and chemotherapy. Authors report one case of rhabdomyosarcoma, which was seen in the right lower leg and showed a rapid growing nature to die, which experienced in Busan National University Hospital, February, 1983.
Animals ; Busan ; Drug Therapy ; Horns ; Humans ; Infant, Newborn ; Leg ; Muscle, Striated ; Radiotherapy ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal ; Sarcoma

Embryonic rhabdomyosarcoma is an extremely malignant tumor of the lower genitourinary tract which arises from striated muscle. It characteristically presents as a lobular grape-like mass. Rhabdomyosarcoma was subclassified by Stout, Horn and Enterline, into 3 basic type; embryonal, alveolar and pleomorphic The onset of irritative, painful and obstructed symptoms predominants in this type of tumor. Combined therapy is the key to proper treatment of this disease utilizing chemotherapy, surgery and radiotherapy, so the survival rate has continued to rise. We report a case of embryonal rhabdomyosarcoma of the bladder developed in a 6 year old boy and the relevant literature has been reviewed.
Animals ; Child* ; Drug Therapy ; Horns ; Humans ; Male ; Muscle, Striated ; Radiotherapy ; Rhabdomyosarcoma* ; Rhabdomyosarcoma, Embryonal ; Survival Rate ; Urinary Bladder*

The primary rhabdomyosarcoma of the brain is very rare. There are only 14 cases reported in the literatures till 1975, and the majority of them were arised in the cerebellum. The intracranial rhabdomyosarcomas may be originated from the multipotent mesenchymal cells of aberrent muscle tissue in the leptomeninges. The histological 3 types are adult pleomorphic, alveolar and embryonal type. The demonstration of cross-striation is confirmed for diagnosis of this tumor. All of these tumors tend to have a short clinical course, but the survival time seems to be slightly improved by surgical excision followed by radiotherapy. We have recently experienced one case of primary rhabdomyosarcoma in the left cerebellopontine angle of 9 year-old girl, which was confirmed by operation and complete autopsy.
Adult ; Autopsy ; Brain ; Cerebellopontine Angle ; Cerebellum ; Child ; Diagnosis ; Female ; Humans ; Radiotherapy ; Rhabdomyosarcoma*

We report a case of embryonal rhabdomyosarcoma with palpable right inguinal lymph node in a 6-year-old girl which developed rapidly on the right labia minora over a period of 2 montha. Histopathological study showed characteristic findings of spindle shaped rhabdomyoblast with hyperchromatic nuclei and cytoplasmicprocesses. After a preoperative chemotherapy with vincristine, actinomycin D, and cytoxan, the size of the mass was reduced, and lymph nodes were not palpable. And then, simple vulvectomy. postoperhtive cheirnatherapy and radiotherapy were done.
Child ; Cyclophosphamide ; Dactinomycin ; Drug Therapy ; Female ; Female* ; Genitalia, Female* ; Humans ; Lymph Nodes ; Radiotherapy ; Rhabdomyosarcoma, Embryonal* ; Vincristine

Malignant Rhabdoid tumor is a rare malignant neoplasm and is morphologically similar to rhabdomyosarcoma, but is different immunohistochemically. Most malignant rhabdoid tumors occur in the kidney of infants, and are rarely reported at extrarenal sites. Since Frierson and his collagues first described the malignant extrarenal rhabdoid tumor(MRT) in children in 1985, it has rarely been reported. The neoplasm is histologically and immunohistochemically identical to a renal malignant rhabdoid tumor. There have been no previous reports of this neoplasm in Korea. An MRT of the sacroiliac bone in a 11-year-old girl is described. Five months after the initial presentation, the patient is still alive with partial response to combination chemotherapy and radiotherapy. The authors review previous reports of extrarenal MRT, and discuss the pathologic characteristics, differential diagnosis, and treatment of this rare neoplasm.
Child ; Diagnosis, Differential ; Drug Therapy, Combination ; Female ; Humans ; Infant ; Kidney ; Korea ; Radiotherapy ; Rhabdoid Tumor* ; Rhabdomyosarcoma

PURPOSE: Radiotherapy (RT) is considered a mainstay of treatment in parameningeal rhabdomyosarcoma (PM-RMS). We aim to determine the treatment outcomes and prognostic factors for PM-RMS patients who treated with RT. In addition, we tried to evaluate the adequate dose and timing of RT. MATERIALS AND METHODS: Twenty-two patients with PM-RMS from 1995 to 2013 were evaluated. Seven patients had intracranial extension (ICE) and 17 patients had skull base bony erosion (SBBE). Five patients showed distant metastases at the time of diagnosis. All patients underwent chemotherapy and RT. The median radiation dose was 50.4 Gy (range, 40.0 to 56.0 Gy). RESULTS: The median follow-up was 28.7 months. Twelve patients (54.5%) experienced failure after treatment; 4 local, 2 regional, and 6 distant failures. The 5-year local control (LC) and overall survival (OS) were 77.7% and 38.5%, respectively. The 5-year OS rate was 50.8% for patients without distant metastases and 0% for patients with metastases (p < 0.001). Radiation dose (<50 Gy vs. ≥50 Gy) did not compromise the LC (p = 0.645). However, LC was affected by ICE (p = 0.031). Delayed administration (>22 weeks) of RT was related to a higher rate of local failure (40.0%). CONCLUSION: RT resulted in a higher rate of local control in PM-RMS. However, it was not extended to survival outcome. A more effective treatment for PM-RMS is warranted.
Diagnosis ; Drug Therapy ; Follow-Up Studies ; Humans ; Ice ; Meninges ; Neoplasm Metastasis ; Radiotherapy* ; Rhabdomyosarcoma* ; Skull Base

BACKGROUND: To investigate the clinicopathological characteristics of urinary bladder tumors, a rare malignancy, in patients 20 years or younger. METHODS: Using a retrospective chart review among patients who received bladder surgery at 2 institutions between July 1996 and January 2013, we analyzed the clinicopathological characteristics of urinary bladder tumors in 21 pediatric patients (male:female = 4.25:1.00; mean age, 12.1 years). RESULTS: Pathology revealed 9 urothelial tumors, 6 rhabdomyosarcomas, 1 low-grade leiomyosarcoma, 1 large cell neuroendocrine carcinoma, 1 inflammatory myofibroblastic tumor, and 3 cases of chronic inflammation without tumors (including 1 xanthogranulomatous inflammation). Urothelial tumors (mean patient age, 16.0 years) were benign or low-grade; and only transurethral resection of the bladder tumor was necessary for treatment. Patients with rhabdomyosarcomas (mean age, 5 years) underwent radiotherapy (if unresectable) or transurethral resection of the bladder tumor (if resectable), after chemotherapy. Of these patients, 2 underwent radical cystectomy, with the remaining patients not receiving a cystectomy. With the exception of one patient, all patients are currently alive and recurrence-free. CONCLUSION: Urothelial tumors were the most commonly found pediatric bladder tumor, with embryonal rhabdomyosarcoma being the second most common. Urothelial tumors are common in relatively older age. Since urothelial tumors in children typically have a good prognosis and rarely recur, transurethral resection of the bladder tumor is the treatment of choice. Rhabdomyosarcomas are common in younger patients. Since rhabdomyosarcoma is generally chemosensitive, chemotherapy and radiotherapy are the treatment of choice for bladder preservation in these patients.
Carcinoma, Neuroendocrine ; Child ; Cystectomy ; Drug Therapy ; Humans ; Inflammation ; Leiomyosarcoma ; Myofibroblasts ; Pathology ; Prognosis ; Radiotherapy ; Retrospective Studies ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal ; Urinary Bladder Neoplasms ; Urinary Bladder*

PURPOSE: We report a case of rhabdomyosarcoma misdiagnosed as a chalazion. METHODS: 11-year-old boy, complaining of tumoral lesion on his left upper eyelid that was suspected to be a chalazion, visited our clinic with progressed lid swelling and edema. The mass lesion was removed surgically, and demonstrated a well-demarcated solid mass of 2 X 2cm in diameter within the medial part the left upper lid. Histopathologic examination showed infiltrative, small round cells and he was diagnosed with embryonal type rhabdomyosarcoma by the positive responses of Desmin, SMA and Ki-67 on immunohistochemical study. Following diagnosis as IRS group IIA because the adjacent fatty tissues were involved with the tumor cells, radiotherapy and chemotherapy were done. RESULTS: After chemotherapy for 47 weeks and radiotherapy for 4 weeks, the patient had only mild signs of keratitis and there was no evidence of the tumor in his left upper lid throughout 12 months of follow-up. CONCLUSIONS: We experienced a case of rhabdomyosarcoma misdiagnosed as a chalazion that was successfully treated by surgical excision, radiotherapy and chemotherapy. Because the presenting signs and symptoms of orbital rhabdomyosarcoma sometimes show a misleading variety including chalazion, the physician should consider the possibility of a rhabdomyosarcoma in any recent onset of rapidly progressing mass of the lid in childhood.
Chalazion* ; Child ; Desmin ; Diagnosis ; Drug Therapy ; Edema ; Eyelids ; Follow-Up Studies ; Humans ; Keratitis ; Male ; Orbit ; Radiotherapy ; Rhabdomyosarcoma*