ABSTRACT
Resumen El rabdomiosarcoma es una neoplasia maligna de origen mesenquimal con diferenciación muscular estriada esquelética. Es el sarcoma más común de la infancia y presenta cuatro subtipos: embrio nario, alveolar, pleomórfico y de células ahusadas/esclerosante. De todos ellos el embrionario es el de mayor prevalencia y presenta una variante, botrioide, que suele comprometer órganos huecos en forma de una masa polipoide multilobulada. Presentamos el caso de una mujer de 27 años que consultó por sangrado vaginal y en quien se evidenció en la colposcopia, una lesión polipoide blanquecina que se exteriorizaba a través del orificio cervical externo. El estudio histológico reveló sectores celulares alternados por áreas laxas, mixoides, junto a glándulas ístmico-endometriales típicas. La proliferación fusocelular atípica, se disponía en nidos, constituidos por células de amplio citoplasma eosinófilo con estriaciones citoplasmáticas transversales y núcleos excéntricos con cromatina homogénea. Se exhibían áreas de células densamente condensadas inmediatas y próximas al revestimiento epitelial intacto, pero separadas de él, por una fina capa de estroma laxo (capa cambial). El perfil de inmunomarcación resultó positivo para desmina, actina músculo específico y miogenina, y negativo para actina músculo liso. Se realizó diagnóstico de rabdomiosarcoma embrionario botroide de cuerpo uterino.
Abstract Rhabdomyosarcoma is a malignant neoplasm of mesenchymal origin with skeletal striated mus cular differentiation. It is the most common sarcoma of childhood and has four subtypes: embryonal, alveolar, pleomorphic and spindle cell/sclerosing. Of all of them, the embryonal one is the most prevalent and presents a variant, botryoid, which usually involves hollow organs in the form of a multilobed polypoid mass. We pres ent the case of a 27-year-old woman who consulted for vaginal bleeding and in whom colposcopy revealed a whitish polypoid lesion that was externalized through the external cervical os. Histological examination revealed cellular sectors alternated by lax, myxoid areas, together with typical isthmic-endometrial glands. The atypical spindle cell proliferation was arranged in nests, made up of ce lls with large eosinophilic cytoplasm with transverse cytoplasmic striations and eccentric nuclei with homogeneous chromatin. Areas of densely packed cells were exhibited immediately, but separated from, the intact epithelial lining by a thin layer of loose stroma (cambium layer). The immunostaining profile was positive for desmin, muscle-specific actin and myogenin, and negative for smooth muscle actin. A diagnosis of embryonal botryoid rhabdomyosarcoma of the uterine corpus was made.
ABSTRACT
Testicular tumors are relatively uncommon in children. The tumors of germ cell origin are still frequently encountered. The pure embryonal rhabdomyosarcomas of testis, not associated with sarcomatous component of the germ cell tumor, is a very rare entity. It is a highly aggressive tumor of childhood and young adolescents. In testes, it arises commonly from paratesticular tissue; primary intratesticular being extremely rare. To the best of authors knowledge, only fifteen cases of primary pure intratesticular tumor have been reported in the literature so far. We report a case of eight-year-old child who presented with a progressive painless right testicular enlargement over two months. Ultrasonography showed a heterogenous intratesticular mass. High orchidectomy was performed. Histopathological examination of the specimen assisted with immunohistochemistry revealed embryonal rhabdomyosarcoma.
ABSTRACT
Embryonal rhabdomyosarcoma (ERMS) is a rare tumor of the female genital tract. It tends to occur during childhood in the vagina and rarely it can arise in the uterine cervix, with a peak incidence in the second decade. We report a case of 15 year old adolescent girl who presented with recurrent vaginal discharge not responding to medical treatment. Examination under anesthesia showed friable growth arising from the cervix. Histopathological examination revealed embryonal rhabdomyosarcoma (botyroid variant) of the cervix. Patient underwent local excision of growth followed by IRS-4 protocol based chemotherapy and now patient is under follow up at our side and pediatric oncology and doing well. Young girls presenting with recurrent vaginal discharge not responding to medical treatment must undergo proper clinical examination and EUA and any suspicious lesions should be examined so as to avoid missing rare but aggressive etiology like rhabdomyosarcoma. Due to the young age of affected patients, embryonal rhabdomyosarcoma (sarcoma botyroides) poses a management challenge as the preservation of hormonal, sexual and reproductive function is essential. Awareness of such as uncommon lesion and its clinical implications is important for the counseling and management of the patient.
ABSTRACT
Objective To explore the nursing countermeasures of children with facial rhabdomyosarcoma treated by radioactive seed implantation. Methods Retrospective analysis was performed on the diagnosis, treatment and nursing process of a child orbital embryonal rhabdomyosarcoma admitted to the department of tumor radiotherapy, Peking University Third Hospital in February 2018. Results Mild wound swelling and pain occurred after treatment, and no acute complications such as increased intracranial pressure, particle displacement, wound bleeding and infection, or visual acuity change occurred. Conclusions Disease evaluation and preoperative preparation should be done strictly before the implantation of radioactive particles; changes of the disease should be monitored strictly during the operation to ensure the safety of treatment posture; postoperative complications observation and radiation protection guidance are the basis to ensure the smooth completion of the stage treatment of children.
ABSTRACT
Objective@#To explore the nursing countermeasures of children with facial rhabdomyosarcoma treated by radioactive seed implantation.@*Methods@#Retrospective analysis was performed on the diagnosis, treatment and nursing process of a child orbital embryonal rhabdomyosarcoma admitted to the department of tumor radiotherapy, Peking University Third Hospital in February 2018.@*Results@#Mild wound swelling and pain occurred after treatment, and no acute complications such as increased intracranial pressure, particle displacement, wound bleeding and infection, or visual acuity change occurred.@*Conclusions@#Disease evaluation and preoperative preparation should be done strictly before the implantation of radioactive particles; changes of the disease should be monitored strictly during the operation to ensure the safety of treatment posture; postoperative complications observation and radiation protection guidance are the basis to ensure the smooth completion of the stage treatment of children.
ABSTRACT
Background: The nasal cavity and paranasal sinuses are collectively referred to as the sinonasal tract, which is anatomically and embryologically distinct from the nasopharynx. Aim and objectives: To understand the variable clinical presentations of three unusual cases in the sinonasal tract, to study the histomorphology and analyze the pitfalls in their diagnosis, to study the differential diagnosis and review the literature. Materials and methods: Unusual presentations of three cases were encountered during the period from October 2016 to July 2017 in the Department of Pathology, Government ENT Hospital, Hyderabad. Excised specimens of all the three cases were received and subjected to routine processing, cutting, staining and histopathological features were analyzed. Special histochemical and immunohistochemical stains were done for confirmation of final diagnosis. Results: Case 1 was Rosai Dorfman Disease, Case 2 was Botryoid Embryonal Rhabdomyosarcoma and Case 3 was Invasive Aspergillosis. Conclusion: Rosai Dorfman Disease has to be differentiated from lymphoproliferative and other histiocytic disorders while the benign inflammatory pattern of Botryoid Embryonal Rhabdomyosarcoma is invariably mistaken for an inflammatory process. Aspergillosis mimics as a neoplastic lesion, by its expansile invasive nature. Thus an inflammatory lesion may be mistaken for neoplastic growth and vice versa which can lead to erroneous diagnosis, improper therapy and poor outcome.
ABSTRACT
Se muestra en el siguiente trabajo la forma de presentación, diagnóstico, tratamiento y evolución de una paciente de 47 años que fue referida por presentar lesión de tipo polipoide en cuello uterino diagnosticándose rabdiomiosarcoma embrionario, la cual fue intervenida quirúrgicamente y quien falleció poco tiempo después del diagnóstico.
In the following paper, the presentation, diagnosis, treatment and outcome of a 47-year-old patient referred due to a polypoid type lesion in cervical are showed. An embryonal rhabdomyosarcoma was diagnosed, which underwent surgery. The patient dies shortly after diagnosis.
ABSTRACT
<p><b>Objective</b>To investigate the pathological characteristics, diagnosis, and differential diagnosis of embryonal rhabdomyosarcoma (ERMS) in the male reproductive system.</p><p><b>METHODS</b>We obtained the clinicopathological features, immunophenotypes, and electron microscopic findings of 11 male patients with ERMS in the reproductive system from 2000 to 2015, analyzed the data, and reviewed relevant literature.</p><p><b>RESULTS</b>ERMS developed in these patients at a median age of 17 (9－58) years, 3 cases in the testis, 4 in the scrotum, 1 in the epididymis, and 3 in the prostate. ERMS presented no clinical specificity, which made it difficult to be differentiated from inflammatory and other benign lesions. Microscopically, the tumor cells were arranged in a diffuse or fascicular distribution and mainly composed of short spindle-like, round, or irregularly shaped cells with nuclear hyperchromatism, the cytoplasm strongly eosinophilic, with differentiation of the striated muscle. Some of the cells were naively differentiated or tennis racket-shaped and some exhibited vacuolar degeneration in the cytoplasm. The nuclei were round or short spindle-shaped with visible nucleoli and mitoses. Immunohistochemically, the tumor cells were positive for Myogenin (5/6), Desmin (11/11), MyoD1 (8/9), and Myosin (1/2). Electron microscopy revealed early myofibrils in the cytoplasm of the tumor cells.</p><p><b>CONCLUSIONS</b>ERMS is a rare and highly malignant tumor characterized by local invasion and early metastasis and apt to develop in the reproductive system of young males. The diagnosis of the malignancy is mainly based on its histopathological and immunohistochemical manifestations, combined with electron microscopy when necessary. Early surgical resection in combination with radio- and chemotherapy is recommended for its treatment, which could reduce the recurrence of the tumor and improve the survival of the patients.</p>
ABSTRACT
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.
Subject(s)
Animals , Rats , Abdominal Cavity , Actins , Cytoplasm , Desmin , Eosinophils , Rats, Sprague-Dawley , Rhabdomyosarcoma, Embryonal , Triacetoneamine-N-Oxyl , VimentinABSTRACT
El rabdomiosarcoma (RMS) del tracto genital inferior es una patología maligna relativamente frecuente en la infancia aunque muy poco prevalente en la edad adulta. Tan solo suponen el 2-4 por ciento de todos los sarcomas de partes blandas. Se trata de una neoplasia derivada de células progenitoras de miocitos de músculo estriado en distinto grado de diferenciación. En un elevado número de casos, el cuadro se presenta como un pólipo endocervical de apariencia benigna, lo cual retrasa el diagnóstico. El correcto manejo del RMS de tracto genital es controvertido. Un esquema agresivo de tratamiento con cirugía, poliquimioterapia y radioterapia en pacientes seleccionadas, ha demostrado aumentar la supervivencia e incluso conseguir la curación en estadios precoces.
Rhabdomyosarcoma (RMS) of the lower genital tract is a common childhood malignancy but a rare tumor in female adults. It accounting for around 2-4 percent of soft-tissue sarcomas. It is a malignant neoplasm originating from myogenic progenitors cells that shows variable stages of skeletal muscle differentiation. In many cases, the tumor appears as a benign endocervical polyp and this delays the correct diagnosis. Optimal management of adult genital tract RMS is uncertain. Aggressive primary therapy with local excition, poliche-motherapy and radiotherapy in selected patients may result in prolonged survival and cure in early stages.
Subject(s)
Humans , Female , Adolescent , Uterine Cervical Neoplasms/surgery , Uterine Cervical Neoplasms/diagnosis , Rhabdomyosarcoma, Embryonal/surgery , Rhabdomyosarcoma, Embryonal/diagnosis , Hysterectomy , Uterine Cervical Neoplasms/therapy , Chemotherapy, Adjuvant , Rhabdomyosarcoma, Embryonal/therapy , Radiotherapy, Adjuvant , Neoplasm Recurrence, LocalABSTRACT
Los sarcomas uterinos son relativamente raros. El tipo histológico más frecuente es el leiomiosarcoma, seguido por el sarcoma del estroma endometrial. Los rabdomiosarcomas (RMS) son neoplasias malignas con diferenciación muscular esquelética. El rabdomiosarcoma embrionario (RMSE) tipo botrioide es el sarcoma más común de la infancia; con escasos reportes en adultos, afectando la región de cabeza y cuello, tracto genitourinario y extremidades más frecuentemente. En el tracto genitourinario, la vagina es la localización mas frecuente, comprometiendo en raras ocasiones el cuello o fondo uterino. Se presentan las características clínicas y morfológicas de un caso de RMSE uterino tipo botrioide diagnosticado en una paciente de 58 años en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez Aravena de Temuco.
Uterine sarcomas are relatively rare. The most common histological type is leiomyosarcoma, followed by endometrial stromal sarcoma. The rhabdomyosarcoma (RMS) are malignant neoplasms with skeletal muscle differentiation. Embryonal rhabdomyosarcoma (RMSE) type botryoides is the most common in childhood, with few reports in adults, affecting the head and neck region, genitourinary tract and extremities more frequently. In the genitourinary tract, the vagina is the most common location, rarely involving cervix and fundus uterine. Clinical and morphological characteristics are presented of a case of uterine RMSE diagnosed in a 58 year-old woman in the Pathology Unit of the Hernán Henríquez Aravena Hospital in Temuco.
Subject(s)
Humans , Female , Middle Aged , Uterine Neoplasms/pathology , Rhabdomyosarcoma, Embryonal/pathology , Immunohistochemistry , Uterine Neoplasms/diagnosis , Rhabdomyosarcoma, Embryonal/diagnosisABSTRACT
Embryonal rhabdomyosarcoma of the head and neck is very rare in adults. We report a case of embryonal rhabdomyosarcoma in the nasal cavity, occuring in a 24-year-old male patient presenting with left nasal obstruction, ipsilateral eye and cheek pain and multiple neck nodules. Rhabdomyosarcoma should be considered in the differential diagnosis of poorly defined soft tissue mass of the nasal cavity not only in children, but also in adults.
Subject(s)
Adult , Child , Humans , Male , Young Adult , Cheek , Diagnosis, Differential , Eye , Head , Nasal Cavity , Nasal Obstruction , Neck , Rhabdomyosarcoma , Rhabdomyosarcoma, EmbryonalABSTRACT
Objective:To improve the understanding and diagnosis of embryonal rhabdomysarcoma of biliary tree in childhood.Methods:The management of 1 child with sarcoma botryoides of biliary tree and other 15 cases reported were reviewed.Results:11 cases of boys and 5 of girls,varying from 1 to 6 years old.Clinical symptoms at presentation included jaundice in 16/16,fever 8/16,abdominal pain 6/16,white feces 9/16 and hepatomegaly 13/16, etc.5 cases of which were misdiagnosed as having hepatitis.There were only 2 cases considered as rhabdomyosarcoma by ultrasound and 1 by abdominal CT scan.7 cases had not recurred in 8 months after combined treatment of complete surgical excision,chemotherapy and radiotherapy.Treated with combination of partial excision and chemotherapy,1 case's tumor turned smaller 3 months later.But 2 cases treated solely with partial excision died 2 months later and 1 case recurred 2 months later.Conclusion:All rhabdomyosarcoma of biliary tree in childhood are botryoid,embryonal rhabdomyosarcoma.The patients are aged from 1 to 6 years old,and boys are more common than girls.The main clinical symptoms are obstructive jaundice,fever and hepatomegaly,so it is easily misdiagnosed as hepatitis in clinic.Ultrasound and abdominal CT sacn are helpful in diagnosing embryonal rhabdomysarcoma (sarcoma botryoides) of biliary tree,but pathological examination is essential for confirming the disease.A combined approach of aggressive surgery,chemotherapy and radiotherapy may result in long-term survival.
ABSTRACT
Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and young adult. Rhabdomyosarcoma can be classified into one of four type; embryonal, alveolar, pleomorphic, undifferentiated. Embryonal rhabdomyosarcomas of the female genital tract are rare, malignant tumors derived from primitive myogenic precursor and generally occur during infancy and childhood. About twenty percent of rhabdomyosarcoma arise in the genitourinary tract, with slightly more than half being embryonal rhabdomyosarcoma. Vaginal primaries are 5-times more common than cervical primaries. Overall, cervical tumor may predominate in adolescent. In perimenopausal women, the uterus is the most common site of rhabdomyosarcoma. The management of rhabdomyosarcoma of genitourinary tract has changed slowly from pelvic exenteration without adjuvant therapy to neoadjuvant chemotherapy followed by less radical surgery and postoperative radiation. Embryonal rhabdomyosarcomas originates in the uterine cervix are extremely rare especially in the thirties. So, little information is available regarding its prognosis and therapy. We present a case of a woman 31 years old with embryonal rhabdomyosarcoma of the uterine cervix with a review of a literature.
Subject(s)
Adolescent , Adult , Female , Humans , Young Adult , Cervix Uteri , Drug Therapy , Pelvic Exenteration , Prognosis , Rhabdomyosarcoma , Rhabdomyosarcoma, Embryonal , Sarcoma , UterusABSTRACT
Embryonal rhabdomyosarcoma, called sarcoma botryoides, is the most common malignant tumor arising from in the infant vagina. This tumor is diagnosed before the age of 5 and highly malignant. We experienced a case of embryonal rhabdomyosarcoma arising from vaginal wall in 5-year-old girl. Authors presented this case with a brief review of literature.
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Rhabdomyosarcoma, Embryonal , Sarcoma , VaginaABSTRACT
A rare case of the nasal cavity and paranasal sinus is encountered and we report the case with the literature review of embryonal rhabdomyosarcoma. The occurrence of embryonal rhabdomyosarcoma in nasal cavity and paranasal sinus is rather uncommon. Most embryonal rhabdomyosarcoma occurs in children younger than 12 years of age, and in adult, the incidence is only 10%, Recently, we experienced a case of adult embryonal rhabdomyosarcoma originating in the nasal cavity. The clinical course and the possible management of this unusual neoplasm are discussed.
Subject(s)
Adult , Child , Humans , Incidence , Nasal Cavity , Rhabdomyosarcoma, EmbryonalABSTRACT
Rhabdomyosarcomas are found mainly in young patients, but rare in adults. A correlated cytological and histologic study of one case of embryonal rhabdomyosarcoma is presented. The cytologic appearance of the urine smear corresponded well with the histologic findings. Cytologically, two main cell types were distinguished; a predominant primitive, small round cell with scant cytoplasm and a large cell with abundant cytoplasm. The cytologic feature proving rhabdomyoblastic differenti-ation, such as cross-striation, was absent.
Subject(s)
Adult , Humans , Cytoplasm , Rhabdomyosarcoma , Rhabdomyosarcoma, Embryonal , Urinary BladderABSTRACT
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.
Subject(s)
Animals , Humans , Infant, Newborn , Drug Therapy , Horns , Leg , Muscle, Striated , Radiotherapy , Rhabdomyosarcoma , Rhabdomyosarcoma, Embryonal , SarcomaABSTRACT
Rhabdomyosarcoma is the most frequent cancer involving the bladder, prostate, vagina and paratesticular tissues in children, and is highly malignant neoplasm with a tendency toward early infiltration of adjacent structures and metastases to regional lymph nodes and distant organs. We report a case of paratesticular embryonal rhabdomyosarcoma developed in 20-year-old male with brief review of literatures.
Subject(s)
Child , Humans , Male , Young Adult , Lymph Nodes , Neoplasm Metastasis , Prostate , Rhabdomyosarcoma , Rhabdomyosarcoma, Embryonal , Urinary Bladder , VaginaABSTRACT
A case of embryonal rhabdomyosarcoma of the bladder in a 6-month-old male infant, who presented dysuria with straining and dribbling, and a suprapubic palpable hard mass distinct from the distended bladder, was reported with brief review of literatures.