Free microvascular rotationplasty with nerve repair for rhabdomyosarcoma in a 18-month-old patient.

Rotationplasty is a limb-sparing surgical option in lower limb malignancies. Sciatic or tibial nerve encasement has been considered an absolute contraindication to this procedure. We report a case of an 18-month-old girl with a rhabdomyosarcoma that affected the leg and popliteal fossa, with neurovascular involvement. Knee and proximal leg intercalary resection was performed followed by reconstruction with free microvascular rotationplasty and neurorraphy from tibial division of sciatic nerve to sural and tibial nerves, and from saphenous nerve to superficial peroneal nerve. Postoperative course was uneventful and ambulation with a provisional prosthesis was restarted during the sixth week after surgery. Bone consolidation was observed after two months. Eighteen months later, the patient had a good gait pattern with a below-knee prosthesis and had recovered sensation in the whole foot and ankle area. This case shows that rotationplasty with nerve repair may provide a sensate stump, which is vital for successful prosthetic adaptation. We believe it may be considered as an alternative to above-knee amputation in tumors with sciatic involvement.

Presentation and Long-term Outcome of High-grade Osteosarcoma: A Single-institution Experience.

PURPOSE: To evaluate clinicopathologic characteristics, prognostic factors, and treatment outcome of pediatric/adolescent high-grade osteosarcoma patients. METHODS/PATIENTS: Retrospective evaluation of patients 21 years of age or younger with newly diagnosed high-grade osteosarcoma treated in a single institution. Effects of variables on event-free survival and overall survival (OS) were determined by using Kaplan-Meier survival analysis. Variables found to be significant were evaluated with multivariable Cox regression analysis. RESULTS: Seventy-seven patients diagnosed between January 1985 and December 2011 were included. Median follow-up time was 11.0 years (range, 1.6 to 26.4 y). Event-free survival at 5 and 10 years was 38%±11% and 38%±11%, respectively. OS at 5 and 10 years was 51%±12% and 45%±12%, respectively. Metastatic disease, prolonged time interval to resumption of chemotherapy, lower tumor necrosis rate, and lack of achievement of complete response at the end of first-line chemotherapy treatment were associated with inferior OS probabilities in univariate analysis. Upon multivariate analysis, only achievement of complete response at the end of first-line chemotherapy and tumor necrosis rate retained independent prognostic significance. CONCLUSIONS: Prognostic factors and long-term survival are similar to those previously described. Reduction of global time interval to resumption of chemotherapy as well as a more specific and validated definition of pulmonary metastases at diagnosis are needed.

Surgical resection of massive liposarcomas at the extremities: a 10-year experience in a referral musculoskeletal sarcoma unit.

BACKGROUND: There is still no consensus regarding the management of patients with massive liposarcomas located in the extremities. Several discrepancies related to the aggressiveness of the surgery and the application of concomitant radiotherapy and chemotherapy treatments remain controversial. The purpose of this study was to analyse the clinicopathological characteristics, prognostic factors and outcomes of a series of patients with massive liposarcomas of the extremities who were treated at a referral hospital specializing in musculoskeletal oncology. METHODS: This was an observational, descriptive and retrospective case series covering 10 years of clinical practice. The records of 26 adults, 14 men and 12 women, with localized massive liposarcomas in the extremities were studied. The average age was 53 years. The patients were treated from January 2003 until January 2012. Wide surgical resections with limb-sparing surgeries were performed for most patients (96.2 %). RESULTS: The average tumour size was 15.1 ± 6.8 cm in the greatest dimension. Regarding the histological subtypes, there were 11 well-differentiated or atypical lipomatous tumours (42.3 %), 10 myxoid (38.5 %) and 5 (19.2 %) round cell and pleomorphic liposarcomas. Regarding the malignancy grades, 19 cases (73 %) were classified as low grade. Among these low-grade tumours predominated the well-differentiated subtype (57.9 %). Within high-grade tumours, the round cell and pleomorphic subtype was most frequent (57.1 %; p = 0.011). Radiotherapy was additionally applied to 12 patients (46.2 %) and adjuvant chemotherapy to 5 (19.3 %). Tumour recurrence was observed in only 2 cases (7.7 %). Only 1 of these cases developed lung metastatic dissemination. CONCLUSIONS: Across the entire series, these massive tumours did not compromise the survival of the patients. The histologic subtype and the malignancy degree were closely related. Proper and early diagnosis and therapeutic management of these patients via the application of wide-margin surgical excision are essential to ensure long-term survival.

Metachondromatosis: A Confusing Disease.

Metachondromatosis is a rare autosomal dominant genetic disease with incomplete penetrance that involves abnormal function of the PTPN11 gene. Differentiation between chondrogenic tumors is a challenge for orthopedists. We report a case of a 5 year-old girl with metachondromatosis, a disease that shares attributes with osteochondromas and enchondromas. We found multiple osteochondroma-like lesions with the atypical characteristic of guiding its growth toward the neighboring joint (epyphisis) instead of moving away from it. Furthermore, columnar enchondroma-like lesions were clearly visible in the right distal radius, in the proximal femoral cervix and in the iliac crests. The patient reported that some other tumor had disappeared or downsized with time. This case was debated between a multidisciplinary skeletal dysplasia group. The aforementioned clinical and radiographic findings reinforced the hypothetical diagnosis of metachondromatosis. Definitive diagnosis of metachondromatosis requires a combination of clinical, radiographical and histopathological findings. Differential diagnosis between enchondromas, osteochondromas and metachondromatosis is vital due to differences in malignization and natural history. When a patient has multiple enchondromas and osteochondromas with regression of some lesions and atypical radiographical characteristic of the osteochondroma-like lesions pointing toward the epiphysis, metachondromatosis, a rare disease, must be considered. Surgical treatment is reserved for painful lesions Risk of malignization is insignificant and genetic advice must be given due it is an autosomal dominant disease.

Embarazo ectópico intersticial tratado con metotrexato y cirugía: caso clínico / Interstitial ectopic pregnancy treated with methotrexate and surgery: clinical case

RESUMEN Antecedentes: El embarazo intersticial es muy inusual y representa <2,4% de todos las gestaciones ectópicas. Objetivo: Se presenta el caso de un embarazo ectópico intersticial tratado de forma médica y quirúrgica. Se realiza una revisión literaria sobre las opciones de manejo y tratamiento. Caso clínico: Paciente primigesta de 36 años con gestación ectópica cornual derecha tras 11 días de la transferencia de un embrión criopreservado. Se decide tratamiento con metotrexato (MTX) sin éxito y con progresión del embarazo, obligándonos por lo tanto a realizar una evacuación quirúrgica de la gestación, exponiendo a la paciente tanto a los efectos secundarios del tratamiento médico como a los del abordaje quirúrgico. Conclusión: El embarazo ectópico intersticial sigue siendo un reto para el ginecólogo. El diagnóstico muy temprano de estas patologías, aunque difícil, podría evitar la opción quirúrgica, siendo el MTX más eficaz en los casos tratados precozmente.

ABSTRACT Background: Interstitial pregnancy is very unusual, and it represents <2,4% of all ectopic pregnancies. Objective: We present the case of an interstitial ectopic pregnancy treated medically and surgically. A literary review is also made about the management and treatment options. Clinical case: A 36-year-old patient with a diagnosis of right cornual ectopic pregnancy after 11 days of a cryopreserved embryo's transfer. Methotrexate (MTX) treatment was applied as the initial step but without lasting results. Because of the progression of the pregnancy, further therapy was focused on its surgical evacuation exposing the patient to the side effects of both medical treatment and surgical approach. Conclusion: Interstitial ectopic pregnancy remains a challenge for the gynecologist. Very early diagnosis of these pathologies, although difficult, could avoid the surgical outcome, being MTX more effective in cases intervened initially.

Papel actual del acetato de ulipristal en el tratamiento de los síntomas asociados a los miomas uterinos / Current role of ulipristal acetate in the management of uterine fibroids

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Acetato de ulipristal después del proceso de la Agencia Europea del Medicamento. ¿Qué ha sucedido y cuál es la visión actual? / Ulipristal acetate after the process of the European Medicines Agency. What has happened and what is the current vision?

Los miomas uterinos son tumores benignos en la pared miometrial del útero. El acetato de ulipristal fue autorizado en la Unión Europea por primera vez el 23 de febrero de 2012; desde entonces, se han tratado con él unas 765.000 mujeres. Durante estos años de uso se han reportado algunos escasos cuadros de lesión hepática, lo que ha supuesto un análisis exhaustivo de los análisis clínicos previos. Presentamos una evaluación de los estudios realizados con el fármaco respecto a su seguridad y a los estudios reportados en la Agencia Europea del Medicamento, así como nuestro punto de vista sobre estos hallazgos, los beneficios del tratamiento y las implicaciones en la práctica clínica

Uterine fibroids are benign tumours within the uterine wall affecting women. Ulipristal acetate was first authorized in the European Union on 23 February 2012, with a post-marketing exposure estimated to be more than 765,000 patients so far. During the post-marketing experience, sporadic cases of liver injury and hepatic failure were reported. A detailed review of the clinical trials carried out in the development of Ulipristal acetate was undertaken to further assess the liver safety data reported during the clinical trials. The experts present an evaluation of the liver safety findings observed during the clinical development and during the evaluation from the European Medicines Agency, and their views on the role of these findings in predicting the occurrence of drug-induced liver injury, the benefits of the treatment and the implications to the current clinical practice

Diagnóstico de Metástasis Ováricas de un linfoma B diseminado mediante ecografía 3D / Diagnosis of Ovarian Metastases from disseminated B lymphoma using 3D ultrasound

RESUMEN La participación del aparato genitourinario en el contexto de la enfermedad hematológica maligna es bien conocida. No obstante, la afectación ovárica por linfomas es inusual, ya sea de novo o como manifestación tardía de linfoma diseminado. El objetivo de esta comunicación es exponer la aportación diagnóstica de la ecografía tridimensional (3D) en la detección de metástasis ováricas, cuando todavía algunas de las pruebas de imagen estándar como la tomografía computarizada (TAC) son normales. En este caso presentamos una paciente con linfoma B difuso de células grandes mediastínico de crecimiento rápido y diseminación precoz a aparato gastrointestinal y genitourinario.

ABSTRACT Genitourinary participation in the context of malignant hematologic disease is well known. However, ovarian involvement by lymphoma is unusual, either de novo or as late manifestation of disseminated lymphoma. The purpose of this communication is to show the diagnostic contribution of three-dimensional ultrasound (3D) in the detection of ovarian metastases, when still some of the standard imaging tests such as computed tomography (CT) are normal. Here we present a patient with primary mediastinal large B-cell lymphoma diffuse with fast growing and early dissemination to gastrointestinal and genitourinary tract.

Septo uterino completo acompañado de doble cérvix y tabique vaginal / Complete septate uterus with double cervix and septate vagina

El objetivo del siguiente trabajo consiste en presentar a 2 pacientes con las mismas alteraciones en el sistema genital que no se incluyen en la clasificación clásica de las malformaciones müllerianas debido a que presentan estas alteraciones de manera combinada. El correcto diagnóstico de las mismas es fundamental para un manejo adecuado, ya que en estos 2 casos saber que las pacientes tienen un útero septo completo con doble cuello y tabique vaginal nos permitirá realizar la resección del septo uterino y del tabique vaginal respetando el cuello, y así favorecer su pronóstico ginecológico y obstétrico (AU)

The aim of this study was to present two patients with the same alterations in their genital system. These alterations are not included in the classical classification of Müllerian malformations because of their co-occurrence. An accurate diagnosis is essential for correct management, because the identification of septate uterus with double cervix and septate vagina in these two patients will allow us to resect the two septa, leaving the cervix intact, thus improving gynecological and obstetric symptoms (AU)

Estudio de la torsión ovárica fetal intrauterina mediante ecografía 3D / Study of the intrauterine fetal ovarian torsion by 3D echography

Los quistes ováricos fetales son la causa más frecuente de masa quística intraabdominal diagnosticada prenatalmente, con una incidencia en torno al 30 por ciento. Normalmente son un hallazgo casual en una ecografía de rutina y no se encuentran anomalías asociadas. Pueden resolverse espontáneamente o presentar complicaciones como rotura del quiste, hemorragia intraquística y torsión ovárica. Las diferentes opciones de tratamiento incluyen el manejo conservador, la punción con aspiración del quiste antenatal o neonatal o la quistectomía laparoscópica o por laparotomía. Tras su diagnóstico se recomiendan estudios ecográficos seriados, donde nos será de utilidad la ecografía 3D, ya que esta modalidad de ecografía nos permitirá reconstruir en tres planos el aparato reproductor femenino y estudiar con detalle numerosas patologías tanto uterinas como anexiales. Su uso en combinación con la ecografía Doppler mejora sustancialmente el diagnóstico precoz de las tumoraciones ginecológicas.

Fetal ovarian cysts are the most common cause of prenatally diagnosed intra-abdominal cystic mass, with an incidence around 30 percent. They are usually an incidental finding on a routine ultrasound and anomalies are not associated. They may be resolved spontaneously or lead to complications such as cyst rupture, intracystic hemorrhage and ovarian torsión. Treatment options include conservative management, antenatal or neonatal cyst aspiration, laparoscopic cystectomy and laparotomy. Afterthe diagnosis, serial ultrasound studies are recommended, 3D ultrasound will be useful for us, since this form of ultrasound allows us to reconstruct in three planes the female reproductive system and study in detail a number of both uterine and adnexal pathology. Its use in combination with Doppler ultrasonography improves significantly the early diagnosis of gynecologic tumors.

Tumor de Brenner bilateral benigno / Bilateral benign Brenner tumor

El tumor de Brenner es un tumor raro que representa aproximadamente el 1,5 por ciento de los tumores de ovario. Se define como un tumor de células transicionales compuesto por células uroteliales dispuestas en agregados sólidos o quísticos embebidos en un estroma fibroso. Se clasifica en tumor de Brenner benigno (95 por ciento), borderline (3-4 por ciento) y maligno (1 por ciento). Presentamos el caso clínico de una paciente de 91 años intervenida por una tumoración anexial gigante en la que el estudio anatomopatológico reveló la presencia de un tumor de Brenner bilateral benigno asociado a dos cistoadenomas serosos de ovario derecho.

Brenner's tumor is a rare tumor that represents approximately 1.5 percent of the tumors of ovary. It is defined as a tumor of transitional cells composed by cells uroteliales arranged in solid or cystic acccumulations absorbed in a fibrous estroma. It qualifies in tumor of benign Brenner (95 percent), borderline (3-4 percent) and malignantly (1 percent). We present the clinical case of a 91-year-old patient controlled by a giant anexial mass in whom the pathology study (biopsy) revealed the presence of a tumor of bilateral benign Brenner associated with two cistoadenomas serous of right ovary.

Gestación heterotópica en un embarazo espontáneo / Heterotopic pregnancy in a spontaneous pregnancy

La gestación heterotópica es aquella en la que coexisten gestación intra y extrauterina. La incidencia es baja en embarazos espontáneos, aumentando con técnicas de reproducción asistida; por tanto, su diagnóstico precisa un alto índice de sospecha. Presentamos el caso clínico de una primigesta de 34 años, sin antecedentes, con gestación actual espontánea, que consultó por spotting e hipogastralgia. En la ecografía se objetivaron un saco gestacional intrauterino y una masa anexial izquierda compatible con gestación ectópica. Tras la sospecha de gestación heterotópica se realizó una salpingectomía laparoscópica y un legrado evacuador de la gestación intrauterina detenida. La anatomía patológica confirmó el diagnóstico (AU)

Heterotopic pregnancy consists of simultaneous intra and extrauterine pregnancy. The incidence is low in spontaneous pregnancies, but these pregnancies are more common when assisted reproductive technologies are used; therefore, diagnosis requires a high index of suspicion. We report the case of a 34-year-old woman with no history of interest and a spontaneous pregnancy, who presented with spotting and hypogastralgia. An ultrasound scan showed an intrauterine gestational sac and left adnexal mass consistent with ectopic pregnancy. As a heterotopic pregnancy was suspected, laparoscopic salpingectomy and curettage of the miscarriage were performed. Pathological analysis confirmed the diagnosis (AU)

Síndrome de Ogilvie: una complicación poscesárea / Ogilvie's syndrome, a caesarean complication

El síndrome de Ogilvie es una seudoobstrucción aguda de colón en ausencia de obstrucción mecánica, se trata típicamente de una complicación que aparece tras cirugías, sobretodo de cadera y cesáreas. Es de difícil diagnóstico, sobre todo por tener clínica similar al íleo paralítico, aunque con una gravedad muy superior. Sus complicaciones por excelencia son la isquemia y la perforación, que cuando se establecen pueden tener una mortalidad de hasta el 50%. Se trata de un caso con evolución tórpida poscesárea y que, a pesar del tratamiento médico, se complicó con perforación de ciego, acabando en una hemicolectomía derecha (AU)

Ogilvie's syndrome is acute colonic pseudo-obstruction without mechanical obstruction, is a tipical complication of caesareas and hips surgery. It is difficult to diagnose, mainly, by having clinic similar to paralytic ileus, but with a much higher gravity. Its complications are ischemia and perforation, which can have when establishing a mortality rate of up to 50%. This is a case with bad evolution after caesarea and that despite the medical treatment is complicated with cecal perforation, finishing in a right hemicolectomy (AU)

Neoplasia sincrónica de endometrio y ovario / Synchronous neoplasia of the endometrium and ovary

Éste es el caso de una paciente con neoplasia sincrónica de endometrio y ovario. Se presentó a una edad joven (45 años) y con signos clínicos de hipermenorrea. Posteriormente, mediante pruebas de imagen, hallamos la tumoración ovárica. Los tipos histológicos fueron endometrioides en ambas localizaciones y, como en la mayoría de este tipo de neoplasias sincrónicas, de bajo grado y estadio. Todos estos factores son de buen pronóstico. Dentro de las neoplasias sincrónicas, esta asociación de tumores es relativamente frecuente; se caracterizan por tener buen pronóstico y ser de bajo grado. Hay estudios en la actualidad cuyo objetivo es establecer criterios claros para diferenciar 2 neoplasias independientes de una sola neoplasia que afecta a 2 tejidos distintos, mediante sofisticadas técnicas de biología molecular

We report the case of a patient with synchronous neoplasms of the endometrium and ovary, which developed at a relatively young age (45 years). Clinical presentation consisted of hypermenorrhea. An ovarian tumor was subsequently detected through ultrasound and computed tomography. Pathological examination revealed endometrioid histology at both sites and, as in most synchronous tumors occurring in these locations, both the grade and the stage of the tumors were low. All these factors indicate a good prognosis. The association of these two tumors is relatively frequent in synchronous neoplasia in general. These neoplasms are low grade and have a good prognosis. Several current studies aim to establish clear criteria on how to differentiate two independent tumors from a single neoplasm affecting two distinct tissues by using sophisticated molecular biology techniques