Angiolipoma sacro. Reporte de un caso / Sacral angiolipoma. A case report

Fundamento: Los angiolipomas son tumores benignos que se presentan en adultos con una localización, preferentemente, en el espacio epidural posterior torácico. Objetivo: Presentar un caso que debutó con un traumatismo axial lumbosacro donde se evidenció una localización poco común de la lesión y sin relación con las estructuras del canal raquídeo. Presentación del caso: Hombre de 25 años que se cayó y debido a ello se le hizo un traumatismo directo en la región sacrococcígea con dolor y aumento de volumen regional, asociado a parestesias glúteas. Los estudios radiológicos evidenciaron una fractura del cóccix y la presencia de una lesión ubicada en las partes blandas, de aspecto redondeado, homogéneo, sólido, de poco más de 50 mm de diámetro. Se le realizó tratamiento quirúrgico que consistió en coccigectomía subperióstica y exéresis macroscópica de la masa. El estudio histológico concluyó el diagnóstico de un angiolipoma. Conclusiones: Los angiolipomas son tumores raros que tienen características radiológicas peculiares, requieren de alta sospecha clínico-imagenológica para indicar los estudios y el tratamiento. La exéresis total es recomendada para evitar la recurrencia y mejorar el pronóstico.

Background: Angiolipomas are benign tumors that appear in adults with special location in the posterior thoracic epidural position. Objective: To present a case that appeared with a lumbosacral axial trauma where a non-common lesion location was evidenced with no relation among the structures of the spinal canal. Case presentation: 25-year-old man who fell down, consequently suffered a painful direct trauma to the sacrococcygeal region and increased regional volume, associated with gluteal paresthesias. Radiological studies showed a fracture of the coccyx and presence of a lesion located in the soft tissues, with a rounded, homogeneous, solid aspect, a little more than 50 mm in diameter. Surgical treatment consisted of subperiosteal coccygectomy and macroscopic excision of the mass. Histological study concluded the diagnosis of an angiolipoma. Conclusions: Angiolipomas are rare tumors with peculiar radiological features, they require high clinical-imaging suspicion for studies and treatment. Total excision is recommended to avoid recurrence and improve prognosis.

Pure yolk sac tumor of sacrococcygeal region.

The sacrococcygeal region is the most common site for the extragonadal germ cell tumors comprising seminomatous and non-seminomatous tumors. Seminomatous tumors are seminomas, and non-seminomatous tumors comprise mainly teratoma (mature and immature), yolk sac tumor (YST), embryonal carcinoma (EC), and choriocarcinoma. These tumors occur in newborns, infants, and adolescents. Other common sites for extragonadal germ cell tumors are the brain and mediastinum, although they may occur anywhere in the body. These tumors may occur in mixed as well as pure form. So, sectioning from different areas should be done before labeling them as pure germ cell tumors. YST, in its pure form, is rare and therefore should not be missed as it is chemosensitive. The patient should be thoroughly assessed clinically. Imaging also becomes necessary while evaluating swelling in the sacrococcygeal region and can aid in differentials. When the clinical and imaging suspicion of either Sacrococcygeal teratoma or other germ cell tumor is high, serum biomarkers as alfa-fetoprotein should be requested. The serum levels are necessary and should be done preoperatively, postoperatively, and during the course of chemotherapy as follow-up. However, the final diagnosis rests on the histopathological diagnosis. We report one such case of pure YST in the sacrococcygeal region in a 9-month-old female child. The imaging suggested sacrococcygeal teratoma type 4, and high alfa-fetoprotein levels were determined postoperatively.

Diagnóstico prenatal del síndrome de Currarino completo. Presentación de un caso / Prenatal diagnosis of complete Currarino syndrome. Case report

RESUMEN Fundamento: El síndrome de Currarino es una enfermedad poco frecuente, presenta varias malformaciones conformadas por una tríada: estenosis anal, malformación sacrococcígea y masa presacra; su diagnóstico se realiza con frecuencia en edad adulta. Objetivo: Reportar un caso que se diagnosticó con síndrome de Currarino en etapa fetal. Caso clínico: Se reportó un feto del sexo masculino de 22 semanas de gestación, con síndrome de Currarino que al realizarle la necropsia se constató la presencia de: defecto sacro coccígeo (ausencia total del sacro), masa o tumoración presacra (de aspecto quístico), ano imperforado y ausencia de pliegue interglúteo, estenosis del sigmoide y bolsa escrotal única, riñón único, pélvico y poliquístico, con salida de 2 uréteres. Conclusiones: El síndrome de Currarino se caracteriza por una tríada de presentaciones, en muchos casos se puede pasar por alto y existir subdiagnósticos, por lo que su detección precoz permite evitar complicaciones en la etapa adulta y mejorar la calidad de vida.

ABSTRACT Background: Currarino syndrome is a non-frequently disease, presenting several malformations consisting of a triad: anal stenosis, sacrococcygeal malformation and presacral mass; its diagnosis is habitually performed in adulthood. Objective: To report a case diagnosed with Currarino syndrome in the fetal stage. Case report: A 22-week gestation male fetus with Currarino syndrome, at necropsy he was found to have: sacrococcygeal defect (total absence of the sacrum), presacral mass or tumors (cystic appearance), non-perforated anus and absence of intergluteal fold, sigmoid stenosis and single scrotal pouch, single, pelvic and polycystic kidney, with exit of 2 ureters. Conclusions: Currarino syndrome is characterized by a triad of appearances, in many cases it can be overlooked and underdiagnosed, so early detection can prevent complications in adulthood and improve life quality.

Pure yolk sac tumor of sacrococcygeal region

The sacrococcygeal region is the most common site for the extragonadal germ cell tumors comprising seminomatous and non-seminomatous tumors. Seminomatous tumors are seminomas, and non-seminomatous tumors comprise mainly teratoma (mature and immature), yolk sac tumor (YST), embryonal carcinoma (EC), and choriocarcinoma. These tumors occur in newborns, infants, and adolescents. Other common sites for extragonadal germ cell tumors are the brain and mediastinum, although they may occur anywhere in the body. These tumors may occur in mixed as well as pure form. So, sectioning from different areas should be done before labeling them as pure germ cell tumors. YST, in its pure form, is rare and therefore should not be missed as it is chemosensitive. The patient should be thoroughly assessed clinically. Imaging also becomes necessary while evaluating swelling in the sacrococcygeal region and can aid in differentials. When the clinical and imaging suspicion of either Sacrococcygeal teratoma or other germ cell tumor is high, serum biomarkers as alfa-fetoprotein should be requested. The serum levels are necessary and should be done preoperatively, postoperatively, and during the course of chemotherapy as follow-up. However, the final diagnosis rests on the histopathological diagnosis. We report one such case of pure YST in the sacrococcygeal region in a 9-month-old female child. The imaging suggested sacrococcygeal teratoma type 4, and high alfa-fetoprotein levels were determined postoperatively.

Squamous cell carcinoma arising in a sacrococcygeal tailgut cyst

Abstract: We report a case of squamous cell carcinoma originated from a sacrococcygeal tailgut cyst in a 73-year-old female patient. Tailgut cysts are generally multilocal and have a layer of either columnar, squamous or transitional epithelium, or a combination of these. This case was treated with surgical excision and radiotherapy. Cancer presentation of a congenital abnormality in old age is a rare entity. This report is the first case of squamous cell carcinoma developing in a tailgut cyst without any synchronization, as an isolated (pure) pathology.

Generalidades de los tumores de la región sacrococcígea / General aspects of sacrococcygeal tumors

Fundamento: Los tumores que se originan en la región sacrococcígea son raros, representan del uno al siete por ciento de los tumores espinales primarios. Sin embargo, su diagnóstico y tratamiento imponen un reto para el equipo quirúrgico, por la complejidad de las relaciones anatómicas y las características biológicas de las lesiones más frecuentes. Objetivo: Actualizar y sistematizar los conocimientos sobre las generalidades de los tumores de la región sacrococcígea. Desarrollo: Son más frecuentes en adultos jóvenes del sexo masculino, debutan con dolor inespecífico en la espalda baja, a lo que se suman progresivamente la debilidad muscular, el déficit sensitivo y los trastornos esfinterianos. Pueden ser epidurales o intradurales, primarios o secundarios, neoplásicos o no y originarse en las partes blandas o en las estructuras óseas. La edad y la localización deben ser consideradas para evaluar el potencial de malignidad. La tomografía axial computarizada y la resonancia magnética nuclear son los estudios confirmatorios y permiten la selección del abordaje quirúrgico. Las lesiones que con más frecuencia se reportan son el cordoma, el quiste óseo aneurismático y el tumor de células gigantes. La radioterapia y la quimioterapia pueden complementar la cirugía en dependencia de la histología. Conclusiones: El diagnóstico precoz necesita la sospecha ante síntomas inespecíficos. El tratamiento de los tumores de la región sacrococcígea debe ser multidisciplinario y multimodal, para garantizar un mejor pronóstico y mayor supervivencia de los pacientes.

Background: tumors arising from sacrococcygeal region are weird and represent from one to seven percent of primary spinal tumors. However diagnosis and treatment are a challenge for the surgical staff because of the anatomic relations complexity and the more frequent lesions biological features. Objective: a review of the literature was done to update and systematize the knowledge about general aspects of sacrococcygeal tumors. Development: they are more frequent in male young adults, unspecific low back pain is the onset symptom and muscle weakness, sense deficit and sphincter disturbances join up later progressively. They can be epidural or intradural, primary or secondary, neoplastics or not and they could arise from soft tissues or bone structure. Age and location most be considered to evaluate malignant potential. CT scan and magnetic resonance imaging are the confirmatory tests and allow surgical approach selection. The most frequent affections are chordoma, aneurismal bone cyst and giant cell tumor. Radiotherapy and chemotherapy can support surgery according to histology. Conclusions: precocious diagnosis needs to be suspected before unspecific symptoms. Sacrococcygeal tumors treatment most be multidisciplinary and multimodal to assure better prognosis and patient´s life expectancy increasing.

Sacrococcygeal teratoma: case report.

We present a male newborn child with a sacrococcygeal mass who was sent to clinic 46 of the Mexican Social Security Institute located in Gomez Palacio, Durango, Mexico for pediatric/neonatal surgical resolution. The mass was detected on gestation week 24 in the sacrococcygeal area and was initially interpreted as a myelomeningocele. On gestation week 32, the mass had grown, so the diagnosis of cystic hygroma was posed. The child was born at 38 weeks of gestational age with a large tumor in the sacrococcygeal area. Images were obtained, and tumor resection was performed without complications. Pathologic examination confirmed the diagnosis of sacrococcygeal teratoma. The postoperative course was uneventful and there were no further complications.

Se presenta el caso de un recién nacido del género masculino que es enviado a la clínica 46 del Instituto Mexicano del Seguro Social de Gómez Palacio, Durango, México para manejo por cirugía pediátrica y neonatología, por la presencia de una masa en región sacrococígea que fue detectada en la semana 24 de gestación como probable mielomeningocele. A las 32 semanas de gestación se observó un mayor crecimiento y se sospechó de un higroma quístico. Se programa cesárea a las 38 semanas de gestación y, después de exámenes imagenológicos, se realiza resección del tumor sin complicaciones. El estudio anatomopatológico confirmó el diagnóstico de teratoma sacrococcígeo. La evolución posoperatoria inmediata y su condición en la actualidad, son satisfactorias.

Relato de dois casos de tumores retro-retais e revisão da literatura / Report of two cases of retrorectal tumors and literature review

Os tumores retro-retais são neoplasias raras no adulto. No espaço retro-retal, existem diversas estruturas embriológicas, que podem desenvolver grupos heterogêneos de tumores benignos e malignos. A apresentação clínica desses tumores é inespecífica, variando de queixas urinárias ou intestinais a ausência de sintomas, e a via de acesso cirúrgica ainda é alvo de discussão na literatura. Os autores relatam dois casos de tumores retro-retais, suas manifestações clínicas, diagnóstico e aspectos cirúrgicos

The retro-rectal tumors are rare neoplasms in adults. In the retro-rectal space, there are various embryological structures, which can develop a heterogeneous group of benign and malignant tumors. The clinical presentation of these tumors is nonspecific, ranging from urinary or intestinal complaints to absence of symptoms, and the surgical approach is still a matter of discussion in the literature. The authors report two cases of retro-rectal tumors, their clinical manifestations, diagnosis and surgical aspects

Teratoma sacrococcígeo: reporte de un caso, desde el diagnóstico antenatal a la resección y reconstrucción primaria / Sacrococcygeal teratoma: case report, from prenatal diagnosis to resection and primary reconstruction

INTRODUCCIÓN: Los teratomas sacrococcígeos (TSC) constituyen una patología infrecuente en la edad pediátrica (1/27.000 recién nacidos vivos), aunque es la neoplasia más frecuente de diagnóstico fetal. La disponibilidad ecográfica ha permitido el diagnóstico antenatal de muchas de estas lesiones, lográndose planificación oportuna, estudio y tratamiento multidisciplinario de estos pacientes. PRESENTACIÓN DEL CASO: Al control ecográfico prenatal, el paciente presenta masa quística heterogénea en polo caudal, decidiéndose cesárea electiva al término por sospecha de TSC. Nace en buenas condiciones destacando masa sacrococcígea de 15 cm de diámetro. Las imágenes son compatibles con TSC Altman II. Luego de completado el estudio con marcadores tumorales, se opera al 9º día de vida extirpándose el tumor y cóccix en su totalidad, se reconstruye el piso pelviano y se logra cierre primario. El paciente evoluciona favorablemente sin complicaciones neurológicas ni digestivas. La histopatología informa teratoma maduro con áreas de neuroepitelio inmaduro, sin atipia celular. DISCUSIÓN: El diagnóstico obstétrico de TSC permite la consejería y derivación oportuna, la planificación quirúrgica, y selección de pacientes que podrían beneficiarse de interrupción precoz, considerando la mortalidad fetal que alcanza 16 por ciento. La clasificación preoperatoria tiene implicancias terapéuticas y pronósticas para estos pacientes. No existe hoy suficiente evidencia de que el tratamiento citotóxico aporte beneficios para teratomas benignos, maduros o inmaduros. En este caso, cabe al seguimiento alejado evaluar las consecuencias del tratamiento, en cuanto a recurrencia, incontinencia urinaria y fecal.

INTRODUCTION: Sacrococcygeal teratoma (SCT) is an uncommon pediatric condition (1 case per 27,000 live births), nevertheless, it is the most frequent neoplasia diagnosed at fetal age. Higher access to obstetric ultrasound allows prenatal diagnosis, appropriate planning, study and multidisciplinary treatment of these patients. CASE REPORT: In a routine obstetric evaluation, patient presented a heterogeneous, cystic mass in the sacrococcygeal region. A SCT was suspected, and an elective cesarean delivery was planned to term gestational age. The patient was delivered without complications, with a 15 cm sacrococcygeal mass. Imaging revealed an Altman II SCT, and serum tumor markers were measured. By the ninth day of life, surgery was performed with tumor and coccyx complete resection, pelvic floor reconstruction and primary closure. Postoperatively, the patient made a good recovery without digestive or neurological complications. The biopsy revealed a mature teratoma with minor immature neuroepithelial foci, without atypical cells. DISCUSSION: The prenatal diagnosis in SCT allows appropriate counseling of parents, derivation to specialized centers, and could identify high-risk patients for early interruption of gestation, taking into account a mortality rate as high as 16 percent. Preoperative classification is important for both treatment and prognosis. There is not enough evidence to recommend chemotherapy for the treatment of mature or immature benign teratomas. In this case, close long-term follow-up will be important to rule out recurrence, and urinary and fecal incontinence.

Sacrococcygeal hernia: a challenge for the coloproctologist

Sacrococcygeal hernia consists of the protrusion of abdominal and pelvic structures through the sacrococcygeal region, an uncommom complication of coccygectomy and sacral coccygectomy. Its surgical treatment is based on perineal hernia repair, by means of abdominal, perineal or abdominoperineal access. Perineal (local or sacrococcygeal) access avoids the laparotomy morbidity and is indicated to patients that are not exposed to radiation or those who had not undergone oncological surgery, allowing local tissue to reconstruct, as in myocutaneous advancement flaps, associated or not to prosthetic mesh, because of the low complication rates and favourable outcomes. The aim of this article is to report the case of a female patient who had undergone sacral coccygectomy due to refractory coccygodynia and developed a symptomatic sacrococcygeal hernia. She underwent polytetrafluoroethylene mesh herniorrhaphy followed by soft tissue closure and gluteal myocutaneous V-Y advancement flap. The authors emphasize technical details and the difficulty of the procedure itself. After three years of follow-up, no recurrence was found. (AU)

As hérnias sacrococcígeas são protrusões de estruturas pélvicas e abdominais pela topografia do sacro e cóccix, sendo complicação incomum após coccigectomias ou sacrococcigectomias. O tratamento é cirúrgico e baseado na correção das hérnias perineais, e pode ser realizado por acesso perineal, abdominal ou abdominoperineal. O perineal (local ou sacrococcígeo), sem a morbidade da laparotomia, é viável para os pacientes não submetidos à cirurgia oncológica ou radioterapia, permitindo a reconstrução com tecidos locais, como no avanço miocutâneo, associado ou não ao uso de telas, com baixa incidência de complicações e bons resultados. O objetivo deste artigo é apresentar o caso de uma paciente submetida à sacrococcigectomia por coccigodinia refratária ao tratamento clínico, que evoluiu com hérnia sacrococcígea sintomática tratada com a correção do defeito com tela de politetrafluoretileno e síntese das camadas suprajacentes, associada ao avanço miocutâneo bilateral tipo V-Y, com o glúteo maior. Serão enfatizados detalhes técnicos e o grau de dificuldade da reconstrução. Após seguimento de três anos, a paciente se encontra em acompanhamento ambulatorial sem recidiva. (AU)

Cordoma sacrococcígeo tratado com ressecção e radioterapia complementar: relato de caso / Sacrococcygeal chordoma treated with resection and radiotherapy: a case report

Cordoma sacrococcígeo que é uma neoplasia malignade grau baixo a intermediário, remanescente da notocorda.Apresenta crescimento indolente, entretanto comcomportamento local agressivo. Trata-se de uma neoplasiarara e que apresenta diagnóstico tardio visto suapobre sintomatologia no início da doença. A localizaçãoé mais comum no esqueleto axial, e neste relato descrevemoso tratamento de um cordoma sacrococcígeo.

Sacrococcygeal chordoma is a malign neoplasia oflow degree remainder of the notocorda. Presents indolentgrowth, meantime with aggressive local behavior. Itis a rare neoplasia and that it presents seen late diagnosishis poor symptomatology in the beginning of thedisease. The location is more common in the axial skeleton,and in this report we described the treatment of asacrococcygeal chordoma.

Teratoma sacrococcigeo neonatal, reporte de un caso de autopsia / Neonatal sacrococcygeal teratomas, autopsy case report

Introducción: Los teratomas sacrococcígeos, constituyen una neoplasia que pese a su baja frecuencia, esuna de las más comunes en neonatos. Se describe un caso de un feto muerto con hallazgos histopatológicosde un teratoma sacro coccígeo inmaduro. Pacientes y Métodos: Mujer de 26 años de edad en su cuartoembarazo de 32 semanas de gestación, quien presenta amenaza de parto pre término. Se le realiza unaecografía obstétrica que informa la presencia de una masa sacra fetal, e hidrops fetal. Se realizó cesáreay se obtiene un óbito fetal de sexo femenino. El estudio anatomopatológico evidenció un teratomasacrococcígeo inmaduro. Conclusión: En los teratomas sacrococcígeos fetales, el desarrollo de hidropsfetal, insuficiencia cardiaca y gran tamaño tumoral son indicadores de mal pronóstico. El diagnósticoprenatal, la planificación del parto, la estabilización perinatal y la resección quirúrgica del tumor, puedenhacer posible la sobre vida en estos pacientes.

Introduction: Sacrococcygeal teratomas are neoplasm that despite their low frequency, are one of the most common in newborns. We describe a case of a dead fetus with an immature sacrococcygeal teratoma,its radiological and histological findings. Patients and Methods: A 26 years old woman in the fourthpregnancy of 32 weeks’ of gestation, who presented the threat of pre-term birth. We performed obstetric ultrasound reporting the presence of a fetal sacral mass and fetal hydrops. Caesarean section was run andgot a female fetal death. Pathologic study showed immature sacrococcygeal teratoma. Conclusion: In fetalsacrococcygeal teratomas, the development of fetal hydrops, heart failure and large tumor size are indicators of poor prognosis. Prenatal diagnosis, planning delivery, the postnatal stabilization period and surgical resection of the tumor, can make survival possible in these patients.

Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida / Sacrococcygeal agenesis association and anal atresia in mixed breed cats

O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.

This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

Cordoma Sacrococcígeo gigante: relato de caso / Giant Sacrococcygeal chordoma: case report

Cordoma sacrococcígeo é uma neoplasia maligna rara que se origina de remanescentes da notocorda. A localização crítica, comportamento localmente agressivo, reconhecida resistência à radioterapia, significativa morbimortalidade cirúrgica e elevada taxa de recidiva tornam seu tratamento um desafio. Descrevemos um caso de cordoma sacrococcígeo gigante.

Sacrococcygeal chordoma is a rare malignant neoplasm arised from the remmants of the notochord. The critical localization, locally aggressive behavior, well-known resistance to radiation therapy, meaningful surgical morbimortality and increased recurrence rate become its treatment a challenge. We describe a case of a giant unresectable sacrococcygeal chordoma.

Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida

This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.

Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida

This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.