Embryonal rhabdomyosarcoma of the uterine cervix in an adult female

ABSTRACT Embryonal rhabdomyosarcoma (RMS) of the female genital tract is an uncommon malignancy, presenting mainly in the pediatric and adolescent populations, primarily affecting the first two decades of life. This malignancy presentation in adulthood is rare and is seldom seen. The incidence of this tumor affecting adult females is approximately 0.4 - 1%, with the common site being the vagina. This tumor infrequently involves the cervix. RMS has a poor survival rate and once diagnosed, it requires aggressive management by radical surgery accompanied by chemoradiation. We present a case of an anaplastic variant of embryonal RMS of the uterine cervix presenting as a cervical polyp in a 36-year-old female who complained of dyspareunia and post-coital bleeding.

Anaplasia in childhood rhabdomyosarcoma: An under reported entity

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Anaplasia is a rare phenomenon seen in childhood RMS. The most common histologic subtype was Embryonal followed by Alveolar and spindle subtype. Design: A total of 11 cases of pediatric RMS were selected from January 2017 to June 2019 presenting at various sites. Out of 11 cases, 2 were further diagnosed as Embryonal, 2 as Alveolar, 2 as Pleomorphic, 1 as Spindle subtype and rest 4 as RMS-NOS based on morphology. All cases were positive for Desmin. The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the tumor cell (anaplastic cells) was selected as the main criterion to diagnose Anaplasia. Results: Out of the total 11 cases, anaplasia was seen in 7 cases. Out of these seven, five cases showed Focal Anaplasia (FA) (71.4%) and 2 cases showed Diffuse Anaplasia (DA) (28.6%). Out of 2 cases of Embryonal RMS one exhibited focal anaplasia (50%). One case of Spindle RMS showed diffuse anaplasia, 2 cases of pleomorphic RMS showed focal anaplasia. Out of 3 cases of RMS- NOS, 2 exhibited focal anaplaisa and one displayed Diffuse anaplasia. Both Alveolar RMS had no features of anaplasia. Conclusion: Presence of Anaplasia is a frequent observation in pediatric RMS. Anaplasia is often under reported in pediatric RMS. Pathologist should be more aware of this rare phenomenon.

Rabdomiosarcoma pulmonar primario: hallazgos clínico-patológicos e inmunohistoquímicos / Primary pulmonary rabdomyosarcoma: clinical-pathological and immunohistochemical findings

Resumen El rabdomiosarcoma pulmonar es una entidad rara y muy poco frecuente, más aún en la población adulta, lo que puede dificultar el diagnóstico correcto. Se presenta el caso de un varón de 48 años con un tumor pulmonar. El estudio histológico reveló que se trataba de un rabdomiosaroma embrionario pulmonar primario. Dada la ausencia de lesiones tumorales en otros sitios fue catalogado como primario pulmonar. El pa ciente realizó quimioterapia y posteriormente fue sometido a una neumonectomía por recidiva de la neoplasia. Al año de la cirugía inicial presentó una metástasis en glándula suprarrenal derecha. Falleció al cabo de 20 meses del diagnóstico original. Se enfatiza la importancia de la inmunohistoquímica en el diagnóstico y se analizan las distintas teorías vigentes que intentan explicar la histogénesis de estos tumores en sitios no habituales.

Abstract Pulmonary rhabdomyosarcona is a rare entity and the histopatological differential diagnosis can be difficult. We report on a case of a 48-year-old male patient with a neoplasm located at the hilum of the right lung. The histological study of the lobectomy specimen allowed the diagnosis of embryonal rhabdomyosarcoma to be made. Given the absence of tumor lesions in other sites, it was classified as primary pulmonary neoplasm. The patient underwent chemotherapy and subsequently a completion pneumonectomy for recurrence of the tumor. One year after the initial surgery, he presented a metastasis in the right adrenal gland. He died 20 months after the original diagnosis. The importance of immunohistochemistry in the diagnosis is emphasized and the different theories that attempt to explain the histogénesis of these tumors in unusual sites are analyzed.

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 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.

Rabdomiosarcoma del ala nasal en un paciente pediátrico. Un reporte de caso / Nasal ala Alveolar Rhabdomyosarcoma in a pediatric patient. A rare case report

Dada la respiración nasal preferencial que tiene la población pediátrica en los primeros meses de vida, la obstrucción nasal tiene más repercusiones a nivel clínico a diferencia de un adulto, lo cual hace más prioritario su diagnóstico y manejo. Una de las principales causas de obstrucción nasal en niños son las masas nasales, las cuales debido a sus múltiples etiologías causan bastante incertidumbre diagnóstica en el equipo médico. En este artículo se presenta el caso de una paciente de 10 meses con una masa en el ala nasal de crecimiento rápido; se realiza un rápido diagnóstico de rabdomiosarcoma que requiere un adecuado manejo. El rabdomiosarcoma con patrón alveolar se presenta de forma inusual en esta ubicación, es por esto por lo que se considera importante presentar este caso para mantenerlo en el algoritmo diagnóstico como una posibilidad y que, así como en el caso presentado, se pueda tener un diagnóstico temprano, un tratamiento adecuado y un resultado estético y funcional favorable.

Given preferential nasal breathing in the first months of life in the pediatric population, nasal obstruction has more clinical repercussions than it would have in adults, hence the need to give higher priority to its diagnosis and management. One of the main causes of nasal obstruction in children is the presence of nasal masses, which cause considerable diagnostic uncertainty in the medical team, because of their multiple etiologies. In this article, the case of a 10-month-old patient with a rapidly growing mass in the nasal ala is presented. A rhabdomyosarcoma was promptly diagnosed and adequately managed. Rhabdomyosarcoma with an alveolar pattern occurs in an unusual way in this location; for this reason, this case is important in order to include this possibility in the diagnostic algorithm and, as was the case in this patient, reach an early diagnosis and institute adequate treatment resulting in favorable aesthetic and functional results.

Morphological analysis of bone marrow metastases from neuroblastoma and rhabdomyosarcoma in children / 中华检验医学杂志

Objective:To retrospectively analyze the bone marrow smears of neuroblastoma and rhabdomyosarcoma in children and summarize the morphological characteristics of the two types of tumors invading the bone marrow to provide reference for the identification and differential diagnosis.Method:A total of 908 bone marrow specimens were collected from the outpatient and inpatient children who were diagnosed as Neuroblastoma and Rhabdomyosarcoma by pathological tissue or lymph node biopsy in Sun Yat-sen University Cancer Center from January 2013 to July 2020. Of which, 231 cases of tumor bone marrow metastasis were detected. Bone marrow smears were observed and analyzed, classified and summarized according to the morphological characteristics of tumor cells.Result:A total of 231 cases of bone marrow metastases were detected, including two types of tumors, 217 cases Neuroblastoma, with an invasion rate of 34.23%; 14 cases Rhabdomyosarcoma, with an invasion rate of 5.11%. The tumor cells of neuroblastoma were arranged in a pseudo-chrysanthemum or wall-like arrangement and most of them were surrounded by nerve fibers. According to cell size, they could be divided into large cell type and small cell type. Rhabdomyosarcoma cells were mainly medium in size, with vacuoles in the nucleus, and double, triple and multinucleated cells can be seen. The cytoplasm was gauze-like, with bead-like vacuoles at the edges. According to the morphological characteristics of neuroblastoma and rhabdomyosarcoma, they can be differentiated from acute leukemia.Conclusion:Among two malignant solid tumors in children, Neuroblastoma had a higher bone marrow invasion rate, while Rhabdomyosarcoma had a lower bone marrow invasion rate. Rhabdomyosarcoma can be initially divided into embryonal type and acinar type according to whether tumor cells were fused or not.

Clinical and prognostic analysis of rhabdomyosarcoma in infants: a single-center report / 中华实用儿科临床杂志

Objective:To analyze the clinical characteristics and prognosis of infants with rhabdomyosarcoma (RMS), thus enhancing the understanding of this disease.Methods:Clinical data of all RMS patients younger than 12 months treated in the Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University from May 2006 to June 2019 were retrospectively analyzed, including the age, gender, histological type, tumor primary site, tumor size, and the prognosis.Patients were followed up until December 31, 2019.The 3-year event free survival (EFS) rate of children was performed by plotting the Kaplan-Meier survival curves.Results:A total of 15 RMS children younger than 12 months were enrolled, accounting for 4.9% of all RMS cases in the same period, including 6 males and 9 females.The median age at diagnosis was 7.0 months (3.0-11.5 months). Classified by the primary site, 40.0% (6 cases) located in the head and neck, followed by 26.7% (4 cases) located in the limbs, 26.7% (4 cases) located in other parts, and 6.7% (1 case) located in the urogenital system.Embryonal RMS, alveolar RMS and spindle cell RMS accounted for 46.6% (7 cases), 26.7% (4 cases), and 26.7% (4 cases), respectively.Ten cases (66.7%) were stage Intergroup Rhabdomyosarcoma Study (IRS)-Ⅲ and 1 case (6.7%) was in stage Ⅳ.There were 10 cases (66.6%) in the middle-risk group, 4 cases (26.7%) in the low-risk group, and 1 case (6.7%) in the high-risk group.Two cases had a larger than 5 cm primary tumor; lymph node involvement was confirmed in 3 cases, and pulmonary metastasis occurred in 1 case at the time of diagnosis.All children were treated with chemotherapy, and 13 cases received postoperative chemotherapy and 1 case received preoperative chemotherapy.One case were not operated.Only 3 children underwent radiotherapy, including 1 case underwent particle implantation and 2 cases received external radiotherapy.Among the 15 children with RMS, 5 cases had relapse and disease progression with the 3-year EFS rate of (59.1±14.5)%, and 2 died with the 3-year overall survival rate of (80.8±12.6)%.Conclusions:The median age of diagnosis of RMS in single-center infants is 7 months.Head and neck are the most common primary sites of RMS.Nearly 50% of the children have the primary site of RMS with poor prognosis.More than a quarter of the pathological subtypes are the spindle cell type.Local treatment significantly influences the local progression or recurrence of RMS.

A RARE CASE REPORT OF ALVEOLAR RHABDOMYOSARCOMA IN VAGINA OF YOUNG FEMALES

Alveolar rhabdomyosarcoma in a rare aggressive tumour which manifests in children and young adults. The site of alveolar rhabdomyosarcoma in vagina is also extremely rare. We presented a case report of 38 yrs.lady who was having complain of intermenstrual bleeding & discharge per vaginum from last 6 months. A large fleshy infiltrative bluish friable mass (3*3cm) present at the junction of middle rd rd and upper 1/3 and upper 1/3 of posterior vaginal wall . Another similar mass (2* 2cm) was also present in the lateral vaginal wall. Wide excision of both growth was done & postoperatively chemotherapy was given.

Rabdomiosarcoma alveolar con metástasis cardíaca en un paciente pediátrico / Alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient

El rabdomiosarcoma es el tumor maligno de partes blandas más frecuente en la edad pediátrica. Puede afectar cualquier localización anatómica. El subtipo histológico alveolar suele causar lesiones en las extremidades en niños de mayor edad. Los sitios metástasicos más frecuentes son el pulmón, la médula ósea, el hueso y los ganglios linfáticos. Describimos el caso de un paciente con rabdomiosarcoma alveolar (RA) con metástasis cardíaca, una presentación poco frecuente de la patología.

Rhabdomyosarcoma is the most common malignant soft tissue tumor in pediatric age. It can affect any anatomical location. Alveolar histological subtype usually presents lesions on the extremities in older children. The most common metastatic sites are the lung, bone marrow, bone and lymph node. We describe a case of alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient, a rare presentation of the pathology.

Rabdomiosarcoma paratesticular: Reporte de caso y revisión de la literatura / Paratesticular rhabdomyosarcoma: Case report and literature review

RESUMEN Objetivo: Ofrecer un alcance de la relevancia del diagnóstico oportuno del cáncer testicular, a partir de un caso infrecuente de rabdomiosarcoma paratesticular en un adulto. Reporte de Caso: Se trata de un paciente varón de 39 años, que ingresa al servicio de cirugía - urología por un aumento de volumen de contenido escrotal, de forma progresiva desde hace 2 años, con crecimiento paulatino de la masa. En la ecografía testicular se evidencia un notorio aumento de volumen de bolsas escrotales por aparente tumoración o masa de consistencia dura, de estructura heterogénea y aspecto sólido de bordes mal definidos. Considerando los hallazgos clínicos, marcadores testiculares e imagenológicos se diagnosticó un tumor paratesticular derecho. Se realizó una orquiectomía derecha más exéresis de tumor paratesticular derecho. Patología concluyó en rabdomiosarcoma embrionario paratesticular con cápsula rota. Paciente fue dado de alta con seguimiento por consulta externa y referido a hospital oncológico de cuarto nivel.

ABSTRACT Objetive: Offer a scope of the relevance of the timely diagnosis of testicular cancer, based on a infrequent case of paratesticular rhabdomyosarcoma in an adult. Case report: The patient is a 39-year-old male who was admitted to the urology-surgery department because of an increase in scrotal volume, which has been progressive over the past two years, with gradual growth of the mass. Testicular ultrasound shows a notorious increase in volume of scrotal bags due to an apparent tumour or mass of hard consistency, of heterogeneous structure and solid aspect with ill-defined edges. Considering the clinical findings, testicular markers and imaging, a right paratesticular tumor was diagnosed. A right orchiectomy plus right paratesticular tumor exeresis was performed. Pathology concluded in paratesticular embryonic rhabdomyosarcoma with a broken capsule. Patient was discharged with outpatient follow-up and referred to the fourth level cancer hospital.

Clinical analysis of testicular rhabdomyosarcoma / 北京大学学报(医学版)

Testicular rhabdomyosarcoma is relatively rare in testicular tumors, but the age of patient is relatively young and the degree of malignancy is high. Therefore, this article introduces 4 cases of testicular rhabdomyosarcoma who were admitted to Peking University Third Hospital from May 1994 to February 2019, and reviews the literature to improve the diagnosis and treatment of this disease. The average age of the 4 patients was 17.5 years (14-21 years), the average hospital stay was 22.0 d (17-31 d), and the average body mass index was 19.6 kg/m2 (14.7-25.8 kg/m2). All the patients underwent routine preoperative blood and urine routine, biochemical tests, as well as serum tumor markers. Preoperative examinations also included chest radiograph, electrocardiogram, ultrasound of the scrotum and groin, and abdominal enhanced CT. Lung CT or other examinations were performed if necessary. The median serum human chorionic gonadotropin (HCG) of the 4 patients was 0.20 IU/L (0.06-0.86 IU/L) (all normal), and the median serum alpha-fetoprotein (AFP) was 1.03 g/L (0.65-1.66 g/L) (all normal). The average maximum diameter of the tumor was 10.0 cm (4.5-15.0 cm). Testicular rhabdomyosarcoma was mainly diagnosed by pathology. The main treatment was radical orchiectomy combined with retroperitoneal lymph node dissection, with or without postoperative adjuvant chemotherapy. The clinical manifestations of the patients with testicular rhabdomyosarcoma had no specific characteristics, but most patients were young at onset with mainly painless masses in the testicles, which were already large when they were found. Patients with testicular rhabdomyosarcoma have a poor prognosis, most of whom recur within two years. Because of the small number of cases of testicular rhabdomyosarcoma, there is no standard treatment currently. It is recommended that patients with testicular rhabdomyosarcoma undergo radical testicular resection combined with retroperitoneal lymph node dissection. Retroperitoneal lymph node metastasis is an important prognostic factor, and patients with postoperative adjuvant chemotherapy can still survive for a longer time. If local recurrence or limited metastasis is found after operation, local resection and salvage radiotherapy are feasible.

Expression of JMJD2B and HIF-1α in Rhabdomyosarcoma Tissues and Their Prognostic Significance / 肿瘤防治研究

Objective To explore the expression and prognostic significance of histone demethylase JMJD2B and HIF-1α in rhabdomyosarcoma(RMS) tissues. Methods A total of 78 RMS patients were followed up to obtain survival time, and immunohistochemical staining was used to detect the expression of JMJD2B and HIF-1α in RMS tissues and analyze their relation with clinicopathological characteristics and prognosis. Results Among the 78 samples, there were 46 (59.0%) and 44 (56.4%) cases of positive expression of JMJD2B and HIF-1α, respectively. The expression of JMJD2B and HIF-1α were positively correlated (P < 0.001, r=0.518) and related to tumor risk, metastasis, IRS staging and neoadjuvant chemotherapy (all P < 0.05). The Kaplan-Meier survival curve and univariate Cox regression analysis showed that the risk level, neoadjuvant chemotherapy, the expression of HIF-1α and JMJD2B were related to the prognosis of patients (all P < 0.05). Multivariate Cox regression analysis showed that JMJD2B (HR=3.161, P=0.039) and risk degree (HR=2.925, P=0.001) could be used as independent prognostic factors for RMS patients. Conclusion JMJD2B and HIF-1α expression are both significantly related to the overall survival of rhabdomyosarcoma patients, and JMJD2B is an independent prognostic indicator.

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)

Risk factors for disease progression and prognosis of pediatric head and neck rhabdomyosarcoma / 中华实用儿科临床杂志

Objective:To explore the risk factors for disease progression and the prognosis with different disease events in a historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma (HNRMS).Methods:From June 1, 2013 to June 30, 2019, 56 pediatric patients diagnosed as HNRMS treated in the Department of Pedia-trics, Beijing Tongren Hospital, Capital Medical University were recruited in the present study.Clinical data, including general information about the diagnosis, treatment, prognostic factors, and follow-up results were collected.Patients were divided into non disease-progression (NPD) groups, including non-event (NE) group and disease-relapse (RD) group, and disease-progression (PD) group based on the disease event.Clinical data and the prognosis in NE group, RD group and PD group were compared.Risk factors for disease progression in pediatric patients with HNRMS were further analyzed by univariate and multivariate analyses of NPD group and PD group.Results:The median follow-up time of all 56 patients was 31.8 months (3.5-74.6 months). There were 30, 12, and 14 patients in NE group, RD group and PD group, respectively.The estimated 5-year overall survival rate was 100.0%, 82.5%, and 11.9% in NE group, RD group and PD group, respectively, which was statistically significant ( P<0.001). Compared with those of NE group, significantly higher rates of patients with a minimum tumor size of 5 cm ( P=0.008) and non-initial radiation therapy ( P=0.001) were detected in PD group.In addition, a significantly higher rate of patients with a minimum tumor size of 5 cm ( P=0.002), and more advanced postoperative pathological stage ( H=12.982, P=0.009) and risk of disease severity( H=18.679, P<0.001) were detected in PD group than those of RD group.The univariate analysis demonstrated that tumor size > 5 cm, alveolar rhabdomyosarcoma, non-initial radiation therapy, advanced postoperative pathological stage and high risk of disease severity were significant risk factors for disease progression of HNRMS.The multivariate analysis result showed that tumor size > 5 cm ( OR=6.75, 95% CI: 1.02-44.78), alveolar rhabdomyosarcoma ( OR=7.10, 95% CI: 1.08-46.57), and non-initial radiation therapy ( OR=15.26, 95% CI: 2.38-97.70) were independent risk factors for disease progression of HNRMS. Conclusions:Pediatric patients with HNRMS in disease progression have a significantly worse overall survival rate, and their prognosis quite differs from those with disease relapse or non-disease events.A minimum tumor size of 5 cm, alveolar rhabdomyosarcoma and non-initial radiation therapy are independent risk factors for disease progression of HNRMS.Pediatric patients with disease progression of HNRMS are susceptible to advanced pathological stage and risk of disease severity.

Gran tumor cardiaco de cavidades derechas con infiltración pulmonar en una mujer joven / Large right-cavity cardiac tumor with pulmonary infiltration in a young woman

Introducción: El diagnóstico diferencial de una masa intracavitaria incluye las variantes normales, los trombos, las vegetaciones y las neoplasias. Los tumores cardíacos primarios son poco frecuentes, con una incidencia que varía del 0,0017 por ciento al 0,28 por ciento y las metástasis aparecen entre el 1,5 por ciento al 20,6 por ciento de las necropsias de los enfermos neoplásicos. Objetivo: Presentar una paciente con masa en ventrículo derecho y vértice pulmonar derecho, con diagnóstico de rabdomiosarcoma cardíaco con metástasis pulmonar. Caso clínico: Paciente femenina de 46 años sin enfermedad previa ni hábitos tóxicos, que ingresa en el servicio de Medicina Interna del Hospital Militar Dr. Carlos J. Finlay, por falta de aire y síntomas dispépticos. Evoluciona de forma tórpida en 48 días, con progresión de la disnea, asociada a hipotensión y taquicardia. Hubo una sospecha inicial de tromboeembolismo pulmonar y luego de enfermedad neoplásica, intracavitaria o pulmonar. Conclusiones: El rabdomiosarcoma cardíaco es poco frecuente y se caracteriza por su crecimiento rápido que conduce a la muerte en semanas o meses, a partir del momento de su presentación clínica(AU)

Introduction: The differential diagnosis of an intracavitary mass includes normal variants, thrombi, vegetations and neoplasms. Primary cardiac tumors are rare, with an incidence ranging from 0.0017 percent to 0.28 percent and metastases appear in 1.5 percent to 20.6 percent of necropsies in neoplastic patients. Objective: To present a patient with a right ventricular and right lung apex mass diagnosed with cardiac rhabdomyosarcoma with pulmonary metastases. Clinical case: A 46-year-old female patient with no previous illness or toxic habits was admitted to the Internal Medicine Service of Military Hospital Dr. Carlos J. Finlay due to lack of air and dyspeptic symptoms. Torpid evolution in 48 days with progression of dyspnea associated with hypotension and tachycardia. Initial suspicion of pulmonary thromboeembolism and then neoplastic, intracavitary or pulmonary disease. Conclusions: Cardiac rhabdomyosarcoma is rare and characterized by rapid growth leading to death within weeks or months from the time of clinical presentation(AU)

Sarcomas durante el embarazo: reporte de dos casos y revisión de la literatura / Sarcomas in pregnancy: Report of two cases and literature review / Sarcomas in pregnancy: Report of two cases and literature review

RESUMEN Objetivo. Presentar los resultados perinatales de dos pacientes con diagnóstico de sarcoma de Ewing y rabdomiosarcoma alveolar. Metodología. Se revisaron dos casos de pacientes con diagnóstico de sarcoma atendidas en el Instituto Nacional de Perinatología de la Ciudad de México. Resultados. Caso 1 femenino de 22 años, con embarazo de 23,0 semanas y tumoración en región glútea izquierda de 20 cm, dolor y dificultad para deambular. La radiografía de tórax informó múltiples nódulos pulmonares y la resonancia magnética, tumor en región glútea con compromiso extenso. El diagnóstico fue sarcoma de Ewing metastático en etapa IV. El manejo consistió en tratamiento sintomático con resolución del embarazo a las 28 semanas. Caso 2 femenino de 22 años con embarazo de 12,0 semanas y diagnóstico de síndrome medular metastásico. Se realizó descompresión T9-11. Se evidenció ausencia de frecuencia cardiaca fetal, por lo que se realizó manejo médico de aborto diferido.

ABSTRACT Objective: To present the perinatal results of two patients diagnosed with Ewing's sarcoma and alveolar rhabdomyosarcoma. Methodology: Two cases of patients with a diagnosis of sarcoma treated at the National Institute of Perinatology in Mexico City were reviewed. Results: Case 1, a 22-year-old female, with a 23.0-week pregnancy and a 20-cm tumor in the left gluteal region, pain and difficulty walking. The chest radiograph revealed multiple pulmonary nodules and the magnetic resonance imaging revealed a tumor in the gluteal region with extensive involvement. The diagnosis was stage IV metastatic Ewing sarcoma. Management consisted of symptomatic treatment with resolution of the pregnancy at 28 weeks. Case 2, a 22-year-old female with a 12.0-week pregnancy and a diagnosis of metastatic spinal cord syndrome. T9-11 decompression was performed. Absence of fetal heart rate was evidenced, so medical management of deferred abortion was performed.

Rhabdomyosarcoma of lip - issues of subtyping, morphology and immunohistochemistry versus molecular studies: a case report with review of literature

Rhabdomyosarcoma (RMS) is a malignant soft tissue tumour occurring most frequently in younger age groups. Study presented a rare case of spindle cell Rhabdomyosarcoma of the upper lip involving the commissure area. In this case report after successful surgical excision, the patient underwent extensive postoperative histopathological studies including special stains and immunohistochemistry. Genotype studies (reverse transcription polymerase chain reaction) were done to help in subtyping and prognostication, and it turned out to be a surprise as it showed positivity for PAX3-FOXO1 t (2;13) translocation which is almost exclusively seen with alveolar RMS. After that he successfully underwent chemotherapy and radiotherapy as it was an aggressive variant and has been disease free for the past 2 years. The author hopes that this case report will highlight the importance of high clinical suspicion in head and neck masses presenting in pediatric age group and to not rely solely on biopsy reports to confirm diagnosis, so that early detection will lead to successful therapy and outcome as exemplified in this case.

Primary biatrial cardiac Rhabdomyosarcoma

Abstract Primary malignant neoplasms of the heart are rare. Cardiac rhabdomyosarcoma is the second most common primary sarcoma. We report a rare case of a 49-year-old woman with a huge biatrial cardiac rhabdomyosarcoma treated by performing surgical resection followed by salvage chemotherapy for local recurrence. Cardiac sarcoma that occupy both atria are extremely rare. Although the prognosis of cardiac rhabdomyosarcoma is dismal, surgical resection should be recommended as a first line therapy to clarify the diagnosis and to relieve symptoms associated with the tumor.