No abstract available.
Adult* ; Humans ; Lip* ; Rhabdomyosarcoma, Alveolar*

Uterine rhadomyosarcoma (RMS) is a rare tumor accounting for 2~6% of the patients with uterine malignant neoplasm and it happens under 1% of the patients with genitourinary malignancies. RMS can be classified in four types; embryonal, alveolar, pleomorphic, and undifferenciated. About twenty percent of RMS occurs in the genitourinary tract, with sligtly more than half being embryonal rhadomyosarcoma. Alveolar rhabdomyosarcoma (ARMS) of the uterine cervix is very rare. Emerich et al. reported the first case of ARMS of the cervix in 1996. So, little information is available regarding its prognosis and therapy. We report a case of a 56 year-old woman with ARMS of the uterine cervix with a review of the literature. She was received the operation and radiotherapy. She has since been disease-free. We herein report a case of very rare ARMS of the cervix.
Accounting ; Arm ; Cervix Uteri ; Female ; Humans ; Prognosis ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar

This paper reported a case of cervical intraspinal metastasis of alveolar rhabdomyosarcoma (ARMS). The clinicopathological features, surgical treatment, chemotherapy and prognosis were introduced and the current literature was reviewed. The diagnosis, differential diagnosis, treatment, molecular features and prognosis of the disease were comprehensively analyzed to improve clinicians' knowledge of this rare disease. The primary lesion appeared about 1 year ago which was painless mass of left hand whose size was about 2 cm×2 cm. After conservative treatment, the mass gradually enlarged and the mass was resected. Postoperative pathology revealed embryonic rhabdomyosarcoma. Postoperative chemotherapy with recombinant human endostatin, liposomal doxorubicin and ifosfamide was performed. The left neck mass was found about 3 months ago, and then the left neck mass was resected under general anesthesia. Postoperative pathological examination showed small round cell malignant tumors. Severe left upper extremity pain began about 2 weeks ago with nocturnal pain and supine pain. Non-steroidal anti-inflammatory drugs were needed to relieve pain which was accompanied by numbness and weakness of the left upper extremity. MRI showed a intraspinal tumor at C5. The left thumb and index finger were absent. Hypoesthesia, muscle atrophy and hypotonia of the left upper limb were confirmed. The muscle strength of biceps brachii and deltoid muscle of the left upper limb was grade 0, the muscle strength of extensor carpus and interphalangeal muscle was grade II, the muscle strength of intrinsic muscles of hands was grade I. The tendon reflex of the left upper limb disappeared. Intraspinal mass was removed and the pain was relieved. But there was no significant change in the muscle strength of the left upper limb. Pathological examination revealed small cell malignancies which were poorly differentiated with diffuse patchy distribution and disordered arrangement. The tumor cells had round, oval or irregular nuclei, and few cytoplasms were positive for Myogenin and MyoD1. FISH test of FOXO1 gene was positive. More than 50% of nuclei showed redgreen signal separation, and the distance between redgreen signals was larger than double diameter of the signal points, which supported ARMS. Total resection of intraspinal tumors was achieved and postoperative chemotherapy was admitted. But intraspinal disseminated metastasis occurred rapidly. ARMS was rare, aggressive tumor with poor prognosis. Subdural metastasis was rare. Correct diagnosis and classification can be made only with help of modern molecular diagnostic methods, which is effective to guide the treatment.
Humans ; Ifosfamide ; Muscle, Skeletal ; Myogenin ; Prognosis ; Rhabdomyosarcoma, Alveolar ; Spinal Neoplasms

Rhabdomyosarcoma is a malignant tumor of mesenchymal origin. It is the most common soft tissue sarcoma of childhood and approximately 250 new cases are diagnosed in the U.S. each year. The most common sites for rhabdomyosarcoma are the head and neck (parameningeal, orbit, paryngeal etc.), the extremities, and the genitourinary tract. Histologically, it can be classified into embryonal, alveolar, pleomorphic, and undifferentiated. Alveolar subtype accounts for approximately 25%. Alveolar rhabdomyosarcoma is very rare and its prognosis is very poor. Especially, cases that reported from vulva are extremely rare, and informations regarding treatment and prognosis are not standardized. We present a case of a girl with alveolar rhabdomyosarcoma of vulva with a review of a literature.
Extremities ; Female ; Head ; Humans ; Neck ; Orbit ; Prognosis ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar* ; Sarcoma ; Vulva*

Rhabdomyosarcoma, when it occurs in the head and neck, is primarily found in children. Alveolar rhabdomyosar-coma is rarely seen in the oral lesion, comparing to the embryonal and the pleomorphic variants. This is a report of a case of alveolar rhabdomyosarcoma in the mandible in a ten-year old girl who complained of a non-painful swelling on the right cheek. The right lower 1st molar was mobile. Her radiographs revealed an extensive radiolucency with somewhat irregular border on the right mandibular ramus. The right mandibular 1st and 2nd molars lost their lamina dura and were floating. CT images revealed smooth-outlined soft tissue mass occupying the pterygomandibular space, the infratemporal space, and the masseteric muscle with thinning and perforation of the right mandibular angle and ramus. Histopathological and immunohistochemical findings established the final diagnosis of alveolar rhabdomyosarcoma.
Cheek ; Child ; Diagnosis ; Female ; Head ; Humans ; Mandible ; Molar ; Neck ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar* ; Sarcoma

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*

Animals ; Brachytherapy ; Combined Modality Therapy ; Humans ; Male ; Prognosis ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar/drug therapy*

Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal(ERMS), alveolar(ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC(vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/ IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.
Adolescent ; Adult ; Arm ; Child ; Dactinomycin ; Extremities ; Humans ; Lymph Nodes ; Lymphatic Metastasis ; Neoplasm Metastasis ; Prognosis ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar ; Young Adult

Pediatric solid tumors have many similarity among different tumors. These tumors present small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the investigation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumor. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is an useful tool for a rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.
Cell Line ; Diagnosis* ; Diagnosis, Differential ; Humans ; Lymphoma ; Pathology ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar ; RNA ; Sarcoma ; Sarcoma, Ewing ; Translocation, Genetic ; Wilms Tumor

Rhabdomyosarcoma is a malignant soft tissue tumor which shows skeletal muscle differentiation. Leptomeningeal metastasis can occur as a late complication, but currently there are no reports that have documented the cytologic features in cerebrospinal fluid (CSF). We report a case of metastatic alveolar rhabdomyosarcoma diagnosed in the CSF of a 28-year-old male who was originally diagnosed with rhabdomyosarcoma on the neck, and that went through systemic therapy. The tumor was positive for anaplastic lymphoma kinase, but progressed despite additional therapy with crizotinib. The CSF specimen revealed small round cells, large atypical cells with abundant cytoplasm and eccentric nuclei, and cells with horseshoe-shaped nuclei. These cytologic findings were in agreement with previous literature and well-correlated with histopathology. This is the first report to document the cytologic feature of rhabdomyosarcoma in CSF. In many cases it is difficult to perform ancillary tests in a CSF specimen and cytopathologists should be aware of the cytomorphologic characteristics to avoid misdiagnosis.
Adult ; Cerebrospinal Fluid ; Cytoplasm ; Diagnosis ; Diagnostic Errors ; Humans ; Lymphoma ; Male ; Muscle, Skeletal ; Neck ; Neoplasm Metastasis ; Phosphotransferases ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar