Rabdomiosarcoma embrionario botrioide de útero / Embryonal botryoid rhabdomyosarcoma of the uterus

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.

Rabdomiosarcoma botrioide del aparato genital femenino en una adolescente virgen / Botryoid Rhabdomyosarcoma of the Female Genital Tract in a Virgin Adolescent

El rabdomiosarcoma es un tumor complejo y de gran malignidad que se origina en las células de la mesénquima embrionaria con capacidad para diferenciarse en células musculares esqueléticas. Este es el tumor maligno de tejido blando más frecuente. Representa aproximadamente 3,5 por ciento de los casos de cáncer en niños de 0 a 14 años de edad. Se presenta una paciente adolescente y virgen en la que se diagnostica histológicamente rabdomiosarcoma botrioide de la vagina. La presentación clínica del rabdomiosarcoma embrionario variedad botrioide es, en general, una masa que protruye por la uretra o el introito vaginal, o por la presencia de flujo fétido o sangrado vaginal en niñas menores de 2 años. En estas lesiones el apoyo diagnóstico con inmunohistoquímica es de vital importancia. El pronóstico de esta enfermedad está determinado por variables como el tamaño tumoral, órgano comprometido, edad del paciente, resultado quirúrgico (R0/R1) y presencia de metástasis. La evaluación médica multidisciplinaria precoz y oportuna permitirá siempre establecer un diagnóstico y tratamiento adecuados(AU)

Rhabdomyosarcoma is a complex and highly malignant tumor that originates in the cells of the embryonic mesenchyme with the ability to differentiate into skeletal muscle cells. This is the most common malignant soft tissue tumor. It represents approximately 3.5 percent of cancer cases in children from zero to 14 years of age. A case of a virgin adolescent patient is presented. A botryoid rhabdomyosarcoma of the vagina was diagnosed histologically. The clinical presentation of embryonic botryoid rhabdomyosarcoma variety is, in general, a mass that protrudes through the urethra or vaginal introitus, or the presence of fetid flow or vaginal bleeding in girls under 2 years. In these lesions, diagnostic support with immunohistochemistry is of vital importance. The prognosis of this disease is determined by variables such as tumor size, compromised organ, age of the patient, surgical result (R0 / R1) and presence of metastasis. Early and timely multidisciplinary medical evaluation will always allow an adequate diagnosis and treatment to be established(AU)

Pitfalls in the diagnosis of lesions of sinonasal tract both on clinical and histopathological grounds

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.

Rabdomiosarcoma botrioide del aparato genital femenino en paciente adolescente virgen / Botryoid rhabdomyosarcoma of the female genital apparatus in a virgin adolescent

El rabdomiosarcoma es un tumor complejo y de gran malignidad que se origina en las células del mesénquima embrionario con capacidad para diferenciarse en células musculares esqueléticas. Este es el tumor maligno de tejido blando más frecuente. Representa aproximadamente 3,5 por ciento de los casos de cáncer en niños de 0 a 14 años de edad. La presentación clínica del rabdomiosarcoma embrionario variedad botrioides es, en general, una masa que protruye por la uretra o el introito vaginal, o por la presencia de flujo fétido o sangrado vaginal en niñas menores de 2 años. Se presenta el caso de un rabdomiosarcoma botrioides de la vagina diagnosticado es una paciente de 16 años y virgen. El apoyo diagnóstico con inmunohistoquímica es de vital importancia y la evaluación médica multidisciplinaria precoz y oportuna permitirá siempre establecer un diagnóstico y tratamiento adecuados que mejoren el pronóstico de quienes padecen esta enfermedad(AU)

Rhabdomyosarcoma is a complex tumor of great malignity that originates in the embryonary mesenchymal cells with the capacity of differentiating into skeletal muscle cells. This is the most frequent malignant tumor in the soft tissue. It roughly represents 3.5 percent of cancer in children aged 0 to 14 years. Generally, the clinical presentation of botryoid-type embryonary rhabdomyosarcoma is a mass that protrudes from the urethra or the vaginal introit or the presence of fetid fluid or vaginal bleeding in girls under 2 years-old. This is the case of a 16 years-old virgin female patient diagnosed with botryoid rhabdomyosarcoma of the vagina. The diagnostic support with immunohistochemistry is of vital importance in addition to the early and timely multidisciplinary medical assessment for setting adequate diagnosis and treatment that improve the prognosis of persons suffering this disease(AU)

A Case Report of Botryoid Wilms Tumor: Has Different Clinical Manifestations / 임상소아혈액종양

A previously healthy, 16-month-old boy was admitted to hospital due to fever, vomiting, and persistent gross hematuria for 2 weeks. His imaging studies showed an inhomogeneous mass, in a left dilated renal pelvis and proximal ureter, which was suggestive of a kidney neoplasm. A stage I Wilms tumor limited to the nephrectomised kidney was diagnosed by histology. The margin of ureter and renal vessels were clear. There was no evidence of anaplasia, angiolymphatic invasion, or skeletal muscle differentiation. In addition, there were polypoid protrusions of tumor into the pelvicaliceal system, resulting in such a 'botryoid' appearance. The patient had been treated with adjuvant chemotherapy using 7 cycles of intravenous actinomycin-D and vincristine (NWTS regimen EE4A) for 5 months. During a 22-month follow-up period, there has been no evidence of tumor recurrence.

A Case Report of Botryoid Wilms Tumor: Has Different Clinical Manifestations / 임상소아혈액종양

A previously healthy, 16-month-old boy was admitted to hospital due to fever, vomiting, and persistent gross hematuria for 2 weeks. His imaging studies showed an inhomogeneous mass, in a left dilated renal pelvis and proximal ureter, which was suggestive of a kidney neoplasm. A stage I Wilms tumor limited to the nephrectomised kidney was diagnosed by histology. The margin of ureter and renal vessels were clear. There was no evidence of anaplasia, angiolymphatic invasion, or skeletal muscle differentiation. In addition, there were polypoid protrusions of tumor into the pelvicaliceal system, resulting in such a 'botryoid' appearance. The patient had been treated with adjuvant chemotherapy using 7 cycles of intravenous actinomycin-D and vincristine (NWTS regimen EE4A) for 5 months. During a 22-month follow-up period, there has been no evidence of tumor recurrence.

Botryoid Odontogenic Cyst on Mandibular Anterior and Both Body Area: a Case Report

Clinical and histopathologic analysis of glandular odontogenic cysts of the jaws

Rabdomiosarcoma botrioide del aparato genital en una mujer de 50 años: reporte de un caso / Botryoid rhabdomyosarcoma of the genital tract in a 50-year-old woman: a case report

El rabdomiosarcoma botrioide del cérvix uterino es en términos generales una neoplasia poco frecuente, generalmente ocurre en pacientes en la primera y segunda décadas de la vida, rara vez afecta a individuos de mayor edad. Recientemente se ha descrito que esta neoplasia cuando se encuentra localizada en el cérvix tiene un pronóstico relativamente favorable, pudiendo efectuarse inclusive un tratamiento quirúrgico conservador. Sin embargo otros artículos mencionan que esto no siempre es así.

The uterus cervical botryoid rabdomiosarcom is a very rare tumor, and in general it occur in patients of first or second decade of age, it is not frequent in older persons. Recently it has been described localized in uterus cérvix and it has a favorable prognosis, and it is possible to treat surgically. However it is not always so.