Rabdomiosarcoma laríngeo en un adulto, descripción de un caso y revisión de la literatura / Adult laryngeal rhabdomyosarcoma. Case report and literature review

Resumen El rabdomiosarcoma laríngeo es un cáncer infrecuente en cabeza y cuello, y aún más en adultos. Describimos el caso de un varón de 55 años con un rabdomiosarcoma del músculo cricoaritenoideo posterior izquierdo tratado mediante laringectomía total y linfadenectomía funcional bilateral.

Abstract Laryngeal rhabdomyosarcoma is an uncommon cancer in head and neck, especially in adults. We report a 55 years old male with a rhabdomyosarcoma from the left posterior cricoarytenoid muscle treated with a total laryngectomy and double functional cervical lymphadenectomy.

Rabdomiossarcoma embrionário de colo uterino: relato de caso em adolescente / Embryonic rabdomyosarcoma of the cervix: case report in a teenager

O rabdomiossarcoma embrionário, variante botrioide, é uma neoplasia maligna dos tecidos moles que deriva de células musculares mesenquimais embrionárias. Alguns fatores de risco genéticos são conhecidos, mas a doença geralmente se apresenta de forma esporádica. É raro manifestar-se em adolescentes, assim como é raro ser primário do colo uterino. Cursa com a presença de pólipos e até massas que se sobressaem na vagina com casos de sangramento vaginal anormal. O diagnóstico é realizado essencialmente pela história e exame anatomopatológico. Quanto maior o tempo para confirmação do diagnóstico, pior o prognóstico. Há várias modalidades de tratamento ­ que deve ser individualizado e envolver uma equipe multidisciplinar ­, que, basicamente, incluem quimioterapia, radioterapia e cirurgia. Os resultados geralmente são menos favoráveis em adolescentes, quando comparados com os de crianças com a mesma neoplasia.(AU)

Embryonic rhabdomyosarcoma, a botryoid variant, is a malignant neoplasm of soft tissues that derives from embryonic mesenchymal muscle cells. Some genetic risk factors are known, but the disease usually presents itself sporadically. It's rarely manifested in adolescents, just as it is rare to be primary in the cervix. It occurs with the presence of polyps and even masses that protrude in the vagina with cases of abnormal vaginal bleeding. The diagnosis is made essentially by history and anatomopathological examination. The longer the time to confirm the diagnosis, the worse the prognosis. There are several treatment modalities ­ involving a multidisciplinary team ­ that must be individualized and basically include chemotherapy, radiotherapy and surgery. The results are generally less favorable in adolescents, when compared with those of children with the same neoplasia.(AU)

Bacteremia due to Weeksella virosa in a pediatric patient with embryonal rhabdomyosarcoma / Bacteriemia por Weeksella virosa en un paciente pediátrico con rabdomiosarcoma embrionario

Abstract Background: Weeksella virosa is one of the two species of the genus Weeksella. Clinical disease due to this bacterium in humans is rare, for which only nine cases have been reported in the literature. Case report: A 4-year-old male patient was diagnosed with a left orbit rhabdomyosarcoma Stage III and was admitted to a northeast third level referral center in Mexico. Aerobic, non-pigmented, Gram-negative rod was isolated from a blood culture. W. virosa was identified by Sensititre™ ARIS. This organism has been described in cases of spontaneous bacterial peritonitis, sepsis, pneumonia, ventriculitis, and urinary tract infection. Conclusions: Clinicians should consider the diagnosis of W. virosa bacteremia in cases involving immunocompromised patients with oral lesions, although it is infrequent. To the best of our knowledge, this is the first clinical report of W. virosa bacteremia described in an immunocompromised pediatric patient.

Resumen Introducción: Weeksella virosa es una de las dos especies del género Weeksella. En los humanos, los reportes de infección por este microorganismo son raros. Solo se han reportado nueve casos en la literatura. Caso clínico: Paciente masculino de 4 años con diagnóstico de rabdomiosarcoma embrionario de órbita izquierda en estadio III, que fue atendido en un hospital de tercer nivel en el noreste de México. Se realizó un hemocultivo, a partir del cual se aisló un bacilo gramnegativo aerobio, no pigmentado. Se identificó W. virosa mediante Sensititre™ ARIS. Este microorganismo ha sido descrito en casos de peritonitis bacteriana espontánea, sepsis, neumonía, ventriculitis e infección del tracto urinario. Conclusiones: Aunque los casos de bacteriemia por W. virosa son raros, los clínicos deben considerar este agente en pacientes inmunocomprometidos con lesiones orales. Hasta donde se conoce, este es el primer caso que se describe de bacteriemia por W. virosa en un paciente pediátrico inmunocomprometido.

Rhabdomyosarcoma of the common bile duct mimicking choledochal cyst: a rare cause of obstructive jaundice / 中国当代儿科杂志

Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.

Uterine cavity embryonal rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a rare solid tumor in childhood and adolescence. The higher incidence is predominant during the first two decades of life. According to the Intergroup RMS Study Group, the embryonal RMS (ERMS), botryoidal variant, constitutes a histological subtype characterized as a "grape-like" lesion of 2.0 cm to 9.5 cm. The treatment involves chemotherapy, surgery, and/or radiotherapy. We present the case of a 14-year-old female patient diagnosed with ERMS, botryoidal variant, which originated in the uterine cervix with vaginal externalization. The initial therapeutic approach comprised an initial prolapsed mass excision followed by Wertheim­Meigs surgery due to the tumor extension. No consensual protocol to ERMS treatment is found in the medical literature; however, a combined approach seems to offer a better result. The postoperative time period was uneventful and the patient followed an adjuvant therapy with vincristine, d-actinomycin, and cyclophosphamide. A comprehensive evaluation of the therapeutic options preserving the reproductive function­unfortunately not always possible­is part of a multi-disciplined care team concerning the pediatric patients.

Metastasis of Rhabdomyosarcoma to the Male Breast: a Case Report with Magnetic Resonance Imaging Findings

Metastasis of rhabdomysarcoma to the breast is a very rare manifestation in adult males. Herein, we report a case of metastasis from embryonal rhabdomyosarcoma in the left hypothenar muscle that presented as a breast mass in a 38-year-old man, who four months later expired because of multiple bone metastases related to pancytopenia. We describe the various imaging findings, including mammograms, ultrasonography, computerized tomography (CT), positron emission tomography-computed tomography (PET-CT), and magnetic resonance imaging (MRI) of this rare disease. The various imaging findings of this lesion could be helpful for future diagnosis of male breast lesions.

Clinicopathological Characteristics of Urinary Bladder Tumors in Korean Patients 20 Years or Younger

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.

Embryonal rhabdomyosarcoma of nasopharyngeal: a case report / 临床耳鼻咽喉头颈外科杂志

This case reports of nasopharyngeal embryonal rhabdomyosarcoma mainly for a stuffy nose, runny nose with blood, and without typical clinical manifestations. Electronic laryngoscopy tip: nasopharyngeal neoplasm. MRI tip: nasopharyngeal carcinoma. By pathological and immunohistochemical examinations, it finally was diagnosed with nasopharyngeal embryonal rhabdomyosarcoma.

successful pregnancy during the treatment of cervical sarcoma botryoides and advantage of fertility sparing management: a case report

Sarcoma botryoides of cervix is a rare variant of rhabdomyosarcomas [RMS] of female genital tract. It is usually diagnosed in first or second decade of life. In this case report, we aimed to present a 21 year-old nulligravid patient who was diagnosed with embryonal RMS of the cervix, to discuss the treatment options that have been stated in the literature, and to highlight the advantage of fertility sparing management in these young patients. We report a 21-year-old nulligravid woman complaining about a mass protruding from introitus, which was represented with a 8x7 cm "grape-like" cervical polyp on speculum examination. The histopathologic examination of the biopsy taken was combined with immunohistochemical staining with desmin, myogenin, S100, vimentin, and myoglobin. Colposcopy, second biopsy, and positron emission tomography were used during the follow-up. The histopathologic examination revealed embryonal RMS of cervix. She received three cycles of combination chemotherapy, doxorubicin and ifosfamide. She refused to have a surgery because of an unplanned, desired pregnancy at two months after the chemotherapy. She was lost during the follow-up. After having an uneventful pregnancy and a successful delivery, she reapplied at postpartum 6[th] month. Colposcopic evaluation revealed a local polypoid area, the histopathologic examination of biopsy suggested recurrence even though positron emission tomography scans were unremarkable. Therefore complementary treatment was planned as conization and pelvic lymphadenectomy. The histopathology revealed no residual tumor on the conization material and no involvement of pelvic lymph nodes. Fertility sparing management, including doxorubicin and ifosfamid combination in chemotherapy step, can be management option. Pregnancy and successful delivery is possible during the treatment. Colposcopy has importance for early detection of recurrences

A case of infant with giant parapharyngeal space rhabdomyosarcoma / 临床耳鼻咽喉头颈外科杂志

Rhabdomyosarcoma is a kind of skeletal muscle ifferentiation tendency of primitive mesenchymal malignant tumor. It is common in pediatric malignant pleomorphic rhabdomyosarcoma, and its prognosis is poor. It is easy to relapse, and its predilection sites in head and face were around the eyes, the external auditory meatus, nasal cavity and paranasal sinuses. We should take comprehensive treatment including surgical operation, radiotherapy and chemotherapy drug combination for embryonal rhabdomyosarcoma.

Embryonal rhabdomyosarcoma in the abdominal cavity of an aged Sprague-Dawley rat / 대한수의학회지

We report a spontaneous embryonal rhabdomyosarcoma in the abdominal cavity of an aged (88-week-old) Sprague-Dawley rat. The animal had a firm lobulated 5 x 5 x 4.5 cm mass in the abdominal cavity that was whitish to tan with necrotic and hemorrhagic plaques. Microscopically, the mass contained nodules with spindle or globoid shaped neoplastic cells with abundant eosinophilic cytoplasm and round or elongated nuclei mixed with other spindle cells with a filamentous appearance and scanty cytoplasm. Multinucleated cells and cross-striations were also observed. The neoplastic cells were positive for vimentin, desmin, and alpha-smooth muscle actin, especially the small spindle cells.

Rabdomiossarcoma Embrionário: Relato de Caso com 15 Anos de Sobrevida e Revisão de Literatura / Embryonal Rhabdomyosarcoma: Case Report with a 15-year Survival and Literature Review

O rabdomiossarcoma de cabeça e pescoço é o sarcoma mais comum de tecido mole em crianças. O planejamento do tratamento depende da localização do tumor, extensão da doença e presença ou não de metástases.O tratamento pode causar diversas sequelas tardias na região de cabeça e pescoço, principalmente na cavidade oral. A prevenção e o controle dessas sequelas proporcionam uma melhor qualidade de vida para o paciente. Relato de caso: Este relato descreve um caso de rabdomiossarcoma embrionário parameníngeo em região de parótida diagnosticadoem um paciente do sexo masculino aos 3 anos de idade, tratado com quimioterapia e radioterapia. Esse paciente recebeu atendimento odontológico como parte integrante do tratamento multidisciplinar. Apresentou boa resposta ao tratamento, permanecendo em controle clínico sem evidência de doença por 15 anos. As sequelas tardias em face e cavidade oral do tratamento oncológico foram: fechamento precoce das raízes; rizogênese incompleta em todos os elementos dentários; agenesias dentárias; múltiplos dentes inclusos; hipoplasia dos ossos da face e trismo. Entretanto,teve qualidade de vida satisfatória, com manutenção da capacidade mastigatória, tendo frequentado a escola edesenvolvido um bom convívio social. Conclusão: O rabdomiossarcoma de cabeça e pescoço está associado a sequelas tardias decorrentes tratamento. A abordagem multidisciplinar é importante para a prevenção e o controle das sequelas e obtenção de uma melhor qualidade de vida para os pacientes...

Extracardial rhabdomyoma: a clinicopathologic analysis of 9 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic characteristics, differential diagnosis and biological behavior of extracardiac rhabdomyoma.</p><p><b>METHODS</b>Nine cases of extracardiac rhabdomyoma diagnosed between January of 1997 and July of 2014 were reviewed. The clinical, pathologic and immunohistochemical profiles were evaluated.</p><p><b>RESULTS</b>There were 5 males and 4 females at diagnosis with age ranging from 2 years and three months to 59 years (mean, 37.6 years). Sites included the head and neck region (7 cases), chest (1 case ) and vagina wall (1 case). Clinically, most cases manifested as a subcutaneous nodule or as a submucosal polypoid lesion with a mean diameter of 3.2 cm. Histologically, 4 were adult-type rhabdomyoma characterized by tightly packed large round or polygonal rhabdomyoblasts with abundant eosinophilic to clear cytoplasm; 3 were myxoid variant of fetal rhabdomyoma composed of immature myofibrils, spindled and primitive mesenchymal cells embedded in a myxoid background, 1 was an intermediate form of fetal rhabdomyoma consisting of densely arranged differentiated myoblasts with little myxoid stroma; 1 was a genital rhabdomyoma composed of elongated or strap-like myoblasts scattered in loose fibrous connective tissue. By immunohistochemistry, they showed diffuse and strong positivity for desmin, MSA and myoglobin with variable expression of myogenin. A case of intermediate type also stained for α-smooth muscle actin. Follow up data (2 months ~ 17 years) showed local recurrence in one patient 6 months after surgery.</p><p><b>CONCLUSIONS</b>Rhabdomyoma is a distinctively rare benign mesenchymal tumor showing skeletal muscle differentiation, which may occassionally recur if incompletely excised. Familiarity with its clinical and morphological variants is essential to avoid misdiagnosing this benign lesion as embryonal rhabdomyosarcoma.</p>

Ureteral Substitution Using Appendix for a Ureteral Defect Caused by a Retroperitoneal Rhabdomyosarcoma in a Child

A 7-year-old boy was diagnosed with a recurrent embryonal rhabdomyosarcoma in the retroperitoneum. After resection of the mass, direct end-to-end anastomosis of the ureter was not possible owing to the length of the resected segment. Accordingly, we performed ureteral substitution by using the appendix to repair the ureteral defect.

Primary embryonal rhabdomyosarcoma of the liver in a young male

Rhabdomyosarcoma [RMS] occurs infrequently in the liver. Rhabdomyosarcomas are malignant tumours that display features of striated muscle differentiation. They are the most common soft-tissue sarcomas among children. In adults however, these are very rare. We report a case of a primary embryonal rhabdomyosarcoma of the liver in a 17 years old boy. This was confirmed by histological examination using immunohistochemical analysis [LCA negative, desmin positive, myogenin focally positive and cytokeratin negative] and site was confirmed by PET CT scan. He received multiple chemotherapies including [doxorubicin, ifosfamide, dacarbazine; gemcitabine, paclitaxel; vincristine, actinomycin D, cyclophosphamide] but longest sustained stable disease was seen with gemcitabine-paclitaxel regimen. The patient died 31 months after the first presentation, secondary to complicated abundant abdominal progressive disease. The poor prognosis and early death of most previously reported cases imply the need for investigation of a more effective treatment method of this uncommon tumour

Naturally Occurring Mediastinal Teratoma with Malignant Transformation in an Adult Male

We report a rare case of rhabdomyosarcoma spontaneously arising in an anterior mediastinal teratoma in a 47-year-old male. The patient was found to have an anterior mediastinal mass on a chest X-ray, which was taken two months before his presentation to Asan Medical Center. A subsequent computed tomography scan revealed an 8.9x7.1x8.0 cm heterogeneous mass in the anterior mediastinum. He underwent an excision via median sternotomy. The histopathologic study identified a mature teratoma with embryonal rhabdomyosarcoma.

Embryonal Rhabdomyosarcoma Arising from a Mediastinal Teratoma: An Unusual Case Report

We report an unusual case of 9.5-cm-sized embryonal rhabdomyosarcoma arose from a mediastinal mature teratoma in a 46-yr-old man. A man presented with chest trauma as a result of an accident at 10 September 2011. On chest X-ray, an anterior mediastinal mass was detected. To obtain further information, chest computed tomography (CT) with contrast enhancement was performed, revealing an anterior mediastinal mass. Complete surgical excision was performed and entire specimen was evaluated. Pathologic diagnosis was embryonal rhabdomyosarcoma arising in mature cystic teratoma. After surgical excision, two cycles of dactinomycin-based chemotherapy were performed. Lung metastasis was detected on follow up CT in September 2012, and wedge resection was performed. Pathological finding of the lung lesion showed same feature with that of primary rhabdomyosarcoma.