Cordoma sacral metastático / Metastatic sacral chordoma

Os cordomas sacrais (CS) são tumores ósseos malignos primários da coluna vertebral de ocorrência rara, com incidência entre 0,000005-0,000027%. O objetivo deste estudo é relatar um caso de CS metastático. Homem de 41 anos, sem comorbidades, chega ao serviço de referência apresentando lesão sacral. Ressonância magnética mostrou tratar-se de tumor com 9,3 cm sugestivo de mieloma ou cordoma. Realizou-se biópsia e histopatológico, confirmando o diagnóstico de CS. O paciente submeteu-se à excisão cirúrgica do tumor. Seis meses após a cirurgia, evoluiu com recidiva e implantes metastáticos em coluna vertebral, partes moles da parede torácica, fígado e espa-ço pleural, evoluindo com paraplegia. Não havia indicação de radioterapia e/ou quimioterapia adjuvante. Não havia também possibilidade de liberação de imatinibe pelo Sistema Único de Saúde. Em cerca de 28 meses de seguimento clínico mensal, o paciente foi a óbito. O caso apresentado mostrou um CS sem sucesso cirúrgico, o que é associa-do a pior prognóstico. O paciente apresentou disseminação sistêmica do tumor e paraplegia poucos meses após a cirurgia, indo a óbito em 28 meses de seguimento. (AU)

Sacral chordomas (SC) are rare primary malignant bone tumors of the vertebral column, with an incidence between 0.000005-0.000027%. This study aims to describe a case of metastatic SC. A 42-year-old man without comorbid conditions, arrived at the referral center, presenting with a sacral lesion. MRI showed a tumor measuring 9.3 cm that was suggestive of myeloma or chordoma. A biopsy with histopathology study was performed, confirming the diagnosis of SC. The patient underwent surgical tumor excision. Six months after surgery, the tumor recurred with metastatic vertebral column implants, soft tissues of the chest wall, liver, and pleural space, and the patient developed paraplegia. There was no indication of adjuvant radiotherapy and/or chemotherapy. There was also no possibility that the Unified Health System would approve imatinib. At about 28 months of monthly clinical follow-up, the patient died. The case presented showed unsuccessful SC surgery, which is associated with a worse prognosis. The patient had systemic tumor dissemination and paraplegia a few months after surgery, dying at 28 months of follow-up. (AU)

Sacroileítis tuberculosa: Reporte de un caso y revisión bibliográfica / Sacroiliac joint tuberculosis. Case report and bibliographic review

La tuberculosis es una de las principales causas infecciosas de muerte en el mundo, pese a que existe un tratamiento específico. La sacroileítis representa el 10% de la afectación ósea en la tuberculosis y sigue siendo una entidad de difícil diagnóstico y de tratamiento controvertido. Presentamos el caso de una mujer de 40 años, que comienza con dolor lumbar bajo, asociado a tumoración y fiebre. Luego de una demora inicial se llega al diagnóstico por punción biopsia. Recibe tratamiento médico. El conocimiento de esta enfermedad facilitará el diagnóstico temprano y su tratamiento. Se discuten la presentación clínica, los métodos auxiliares de diagnóstico y el tratamiento, y se presenta una revisión bibliográfica de la enfermedad. Nivel de Evidencia: IV

Tuberculosis is a worldwide known cause of death, even nowadays with specific treatment. Tuberculosis of the sacroiliac joint represents 10% of bone involvement in this disease; diagnosis still remains a challenge, and treatment is controversial. We present a 40-year-old woman with lower back pain, fever and a lower back mass. After an initial delay, diagnosis of tuberculosis of the sacroiliac joint was established by fine-needle aspiration of the joint. Awareness of this particular presentation will facilitate early diagnosis and treatment. Clinical presentation, diagnostic methods and treatment are discussed, and a bibliographic review of this entity is made. Level of Evidence: IV

Hibernoma dorsal gigante: presentación de caso / Hibernoma giant dorsal: case report

El hibernoma o lipoma de grasa parda, es un tumor benigno extremadamente infrecuente. Presentamos el caso, de un paciente femenino de 12 años de edad con una tumoración gigante en región dorsal, de origen congénito, con una extensión desde la región cervical baja hasta el sacro, presentaba con ausencia de los músculos de la región glútea, se realizó estudios de imagen, así como química sanguínea, donde se evidencio anemia severa y desnutrición proteico energética, se organizó un equipo multidisciplinario centrado al tratamiento definitivo de la lesión, la cual provocaba además seria deformidad de la columna lumbar (cifoescoliosis), se logró la resección completa de la lesión, con un peso total de 12 Kg, con reconstrucción de los bordes anales sin afectar el esfínter anal, se mantuvo bajo seguimiento durante 1 años sin presentar ninguna recidiva de la lesión.

The hibernoma or fetal brown fat, is an uncommon benign tumor. We report a case with an unusual mass in a 12 years old female, in dorsal region, congenital origin, with extension from down cervical region until sacral region, absence of gluteal region muscle. We did image study and blood test, with diagnosis of severe anemia and malnutrition. We organized multidisciplinary team to do final treatment for the lesion that caused important deformity to lumbar spine (kyphoscoliosis), the surgical team achieved gross total resection of tumor, with 12 kg of total weight and reconstruction of anal edge without damage of sphincter, follow up during one year without recurrence.

Tumor maligno de vaina de nervio periférico: reporte de caso / Malignant peripheral nerve sheath tumor: case report

Se presenta un caso de tumor sacro voluminoso con características de Tumor Maligno de Vaina de Nervio Periférico (MPNST) que tras una resección inicial y recidiva se reintervino y trató con radioterapia complementaria. Tras ocho años se ha observado una buena evolución.

A case of voluminous sacral tumor with Malignant Peripheral Nerve Sheath Tumor (MPNST) characteristics is reported, that after a first resection and later recurrence is reoperated and treated with complementary radiotherapy. After eight years a good evolution has been observed.

Carcinomatous degeneration of pilonidal cyst with sacrum destruction and invasion of the rectum / Degeneracao carcinomatosa do cisto pilonidal com destruicao do sacro e invasao da parede do reto

Malignant degeneration of pilonidal cysts is rare. The most common histologic type is the squamous cell carcinoma, triggered by the chronic inflammatory process. The growth of the lesion is typically slow. The diagnosis must be early and the appropriate treatment is ample surgical resection, including the presacral fascia. In some cases, as the present one, the diagnosis is made at a stage when the disease has progressed and invaded adjacent structures. In these cases the surgery involves multiple organ resection. We report the case of a patient with carcinomatous degeneration of pilonidal cyst, with bulky disease that extended up to the wall of the rectum. The treatment was extended resection, sacrectomy and abominoperineal resection of the rectosigmoid with permanent colostomy.

A degeneração maligna do cisto pilonidal é rara. O tipo histológico mais freqüente é o carcinoma epidermóide e tem como fator desencadeante o processo inflamatório crônico. O tumor é de crescimento lento. O diagnóstico deve ser precoce e o tratamento cirúrgico adequado é a ressecção ampla incluindo a fáscia pré-sacral. Em alguns casos, como o que apresentamos, o diagnóstico é feito numa fase em que a doença progrediu e invadiu as estruturas adjacentes. Nestes a cirurgia necessária envolve a ressecção multiorgânica. Apresentamos paciente com degeneração carcinomatosa do cisto pilonidal, com doença volumosa que se estendia até a parede do reto. O tratamento realizado foi ressecção alargada, sacralectomia e ressecção abominoperineal do retossigmóide com colostomia definitiva.

Sacroplastia: una nueva esperanza para el dolor por metástasis sacra / Sacroplasty: new hope for pain from sacral metastasis

La cementoplastía en aplastamientos o fracturas vertebrales es un proceder que se utiliza con mayor frecuencia en la sociedad moderna, basado en la inyección percutánea de polimetil-metacrilato. El objetivo de este artículo es presentar una experiencia de nuevo abordaje en el tratamiento del dolor oncológico; en este caso por metástasis en el cuerpo vertebral de S2, a partir de un cáncer de próstata, que refería un dolor incapacitante. Creemos que el proceder fue novedoso y que existen pocos casos reportados en la bibliografía mundial sobre la sacroplastia.

The cementoplasty in squashing or vertebral fractures is an action that is utilized with high frequency in the modern society, based in the percutaneous injection of polimetil-metacrylate. The objective of this presentation is to show you an experience to a new perspective in the treatment of the cancer pain; In this case for metastasis in S2's vertebral body, from a prostate cancer, that presented a crippling pain. That is why we thought the way it was conducted was innovative and there are few cases that have been reported in the worldwide bibliography on the sacroplasty.

Síndrome de Currarino y enfermedad de Hirschsprung: una asociación poco frecuente / Currarino's syndrome and Hirschsprung's disease: a rare combination

The Currarino syndrome is an autosomal dominant partial sacral agenesis involving sacral vertebrae S2 to S5, with presevation of the S1 vertebrae. In the most severe form of the syndrome, the sacral agenesis is associated to presacral mass, anorrectal and urogenital malformations. Hirschsprung's disease is a congenital malformation of the hindgut determined by the absence if parasympathetic intrinsic ganglion cells in the submucosal and myenteric plexuses. We present a case of a 3 years old male child affected from Currarino syndrome associated to Hirschprung's disease.

El Síndrome de Currarino consiste en agenesia sacra parcial de herencia genética autosómica dominante parcial, que afecta las vértebras sacras S2 a S5, preservándose S1. En la forma más severa del síndrome, a la agenesia sacra se asocian masa presacra y malformaciones anorrectales y urogenitales. La enfermedad de Hirschsprung es una malformación congénita del intestino grueso debida a la ausencia de células ganglionares intrínsecas parasimpáticas de la submucosa y de los plexos mientéricos. Presentamos el caso de un niño de 3 años con Síndrome de Currarino asociado a enfermedad de Hirschsprung.

Evaluación de la frecuencia de espina bífida oculta y su posible relación con el arsénico ambiental en una muestra prehispánica de la Quebrada de Camarones, norte de Chile / Spina bifida occulta associated with environmental arsenic exposure in a prehispanic sample from northern Chile

Background: The Camarones River Valley, located in the extreme north of Chile, is characterized by high environmental arsenic levels and an arid desert. It has been inhabited by humans for the past 7,000 years. Evidence exists for chronic arsenic poisoning in both prehispanic and present populations residing in the area. Chronic arsenic exposure causes multi-systemic problems and can induce congenital malformations, in particular neural tube development defects such as spina bifda. Aim: To study the prevalence of spina bifda among prehispanic mummies of the area. Material and Methods: Onehundred and twenty prehistoric adult individuals were analyzed for evidence of spina bifda occulta of the sacrum in skeletal samples from the sites of Camarones 8, Camarones 9, Azapa 140 and Lluta 54, held in repository at the Museo Universidad de Tarapacá de Arica- San Miguel de Azapa. A diagnosis was considered positive when at least S1, S2 or S3 were affected. As controls, mummies of individuals that resided in Lluta and Azapa valley, with a low arsenic exposure, were analyzed. Results: The frequency of spina bifda occulta among samples from the Camarones coast and Lluta and Azapa Valley were 13.5 and 2.4 percent respectively. Conclusions: Considering these were contemporaneous samples, and are believed to have had no other differences in diet or other factors, the differential exposures to arsenic could have produced the observed differences in spina bifda frequencies.

Effectiveness of intravenous bisphosphonate in treatment of giant cell tumor: a case report and review of the literature.

Giant cell tumor is a benign locally aggressive tumor. The standard treatment is en bloc resection followed by major reconstructive surgery, or extended curettage conjunction with bone grafting or the use of bone cement implantations. Surgical treatment of giant cell tumor at the sacrum is associated with high morbidity, and local recurrence. The authors present a case of giant cell tumor at the sacrum treated with intravenous 4 mg zoledronate every 4 weeks for seven courses followed with curettage and cement implantation. At two years follow-up, the patient had no pain, no neurological deficit, and no local recurrence. The patient's gait was normal. From the present study, the authors demonstrate the effectiveness of zoledronate for treatment of giant cell tumor at the sacrum. It can reduce the morbidity from major surgery.

Sacral Intraspinal Bronchogenic Cyst: A Case Report

Intraspinal bronchogenic cysts are rare congenital cystic lesions. In all the reported cases, the cysts have been located in the cervical, upper thoracic or thoracolumbar segments. We report the case of an intraspinal bronchogenic cyst in the sacral location. We present the case of a 5-month-old female with a skin dimple in the midline over the sacral vertebra. Magnetic resonance image of the lumbar and sacral vertebra revealed a dermal sinus tract and an epidural cystic mass at the S2 level. The patient underwent the removal of the dermal sinus tract and the cyst. The cystic mass was shown to be connected to the subarachnoid space through a slender pedicle from the dura. The cyst was diagnosed to be a bronchogenic cyst based on the results of the histopathological examination. We conclude that intraspinal bronchogenic cysts may appear in the sacral location.

[ report about a rare case of tail gut cyst [TGC] in a 90- year-old man with sacrum ulceration]

Tail gut cyst [TGC] is a non common cystic lesion with post anal origin which is occurred in retro rectal region. In this article we studied a 90-year-old man with a tail gut cyst who presented with post sacral ulcer. The patient did not cooperate well with taking history. For further evaluations the patient underwent surgical treatment for detriment and the region was drained off and repaired. At operation retro rectal cystic mass was excised for pathologic studies. Although the incidence of malignancy in TGC is extremely rare, it should be considered in differential diagnosis of retro rectal tumors especially in middle-aged women

Forensic identification for 16 cases with lumbar sacralization and lumbarization / 法医学杂志

Lumbar sacralization and lumbarization are congenital spine malformation. 16 cases with lumbar sacralization or lumbarization were reviewed. Through studying the etiopathogenisis and clinical manifestation we analyze the relationship between injury and disease. 6 cases of 16 have lumbar sacralization. 10 cases are attributed to lumbarization. Most of this cases have backleg pain more or less. As a forensic doctor we suggest that pay more attention to this problem in our identify practice and locate the sequence of vertebral body more exactly.

Sacro-iliac tuberculosis presenting as cauda equina syndrome

Tuberculosis affecting the bone and spine has been a common problem in the developing countries for quite some time. Though the incidence has decreased dramatically, it still continues to be an important differential diagnosis in certain clinical situations. We hereby describe a patient who presented with features of cauda equine syndrome; the diagnosis was ultimately tuberculosis primarily involving the sacroiliac joint extending through the neural foramen and causing extradural compression

Tratamento de dor em paciente com tumor sacral inoperável: relato de caso / Management of pain in patient with inoperable sacral tumor: case report

JUSTIFICATIVA E OBJETIVOS: Diversas técnicas podem ser utilizadas no controle de dor em pacientes oncológicos. O objetivo deste relato é mostrar o uso de algumas medidas terapêuticas empregadas para tratar um paciente com quadro doloroso importante de difícil controle. RELATO DO CASO: Paciente do sexo masculino, 70 anos, com cordoma sacral e com possibilidade terapêutica muito difícil. Apresentava quadro de dor importante associado ao tumor. São relatadas diversas técnicas utilizadas no seu tratamento e os resultados obtidos. CONCLUSÕES: Os bloqueios neurolíticos, para o controle da dor em paciente com tumores cuja possibilidade terapêutica é difícil, constituem técnica eficaz quando bem indicados e realizados dentro de critérios estabelecidos.

Adult sacrococcygeal teratomas

Sacrococcygeal SCT teratoma are rare in adults with an incidence of 1:87,000 and a female to male ratio of 10:1. Most of SCT are cystic and benign. Our patient was born with this mass that increased in size as she grew older. Being a precious child she was kept away from seeking medical advice by her parents. Initial work up included plain x-ray pelvis, computerized tomography scan and magnetic resonance imaging. At operation the mass was seen arising from sacrococcygeal region. It was excised completely along with coccyx. Pathologically, the specimen contained differentiated tissue from all 3 germ layers

Meningocele intra-sacral oculta: relato de caso / Occult intrasacal meningocele: case report

Meningoceles intra-sacrais säo lesöes raras, que podem determinar sintomas de compressäo radicular. Descrevemos o caso de uma paciente de 18 anos, com queixa de incontinência urinária, acometida por esta patologia. Após avaliaçäo neuro-urológica e estudo uro-dinâmico, foi demonstrada arreflexia do detrusor, sendo aventada a hipótese de disrafismo espinhal. O diagnóstico de meningocele intra-sacral oculta foi evidenciado através de ressonância magnética. A paciente foi submetida a abordagem cirúrgica com boa recuperaçäo pós-operatória. No presente artigo, é feita breve revisäo da literatura, e säo discutidas as apresentaçöes clínicas possíveis, os achados neuro-radiológicos e o tratamento cirúrgico