Sarcoma de células claras del pie. Presentación de un caso / Clear cell sarcoma of the foot. Case presentation

RESUMEN El sarcoma de células claras fue descrito por primera vez por Franz M. Enzinger en 1965. Está íntimamente asociado a tendones y aponeurosis, excepcionalmente compromete la epidermis. Afecta fundamentalmente a pacientes jóvenes y se caracteriza por múltiples recurrencias locales y metástasis tardías. Se presenta un paciente de 22 años de edad, masculino que fue sometido a tratamiento quirúrgico radical (amputación transmetatarseana del 1er y 2do rayo). Los estudios anatomopatológicos confirmaron el diagnóstico de un sarcoma de células claras. El paciente se encuentra libre de la enfermedad después de 6 años de operado e incorporado a su vida social (AU).

ABSTRACT The clear cell sarcoma was firstly described by Franz M. Enzinger in 1965. It is intimately associated to tendons and aponeurosis, exceptionally compromising the epidermis. It mainly affects young patients and is characterized by multiple local recurrences and late metastases. We present a male patient, aged 22 years, who underwent a radical surgical treatment (transmetatarsal amputation of the 1st and 2nd rays). The anatomic-pathological studies confirmed the diagnosis of clear cell sarcoma. 6 years after surgery, the patients is free of the disease and reincorporated to his social life (AU).

Sarcoma de células claras del pie. Presentación de un caso / Clear cell sarcoma of the foot. Case presentation

RESUMEN El sarcoma de células claras fue descrito por primera vez por Franz M. Enzinger en 1965. Está íntimamente asociado a tendones y aponeurosis, excepcionalmente compromete la epidermis. Afecta fundamentalmente a pacientes jóvenes y se caracteriza por múltiples recurrencias locales y metástasis tardías. Se presenta un paciente de 22 años de edad, masculino que fue sometido a tratamiento quirúrgico radical (amputación transmetatarseana del 1er y 2do rayo). Los estudios anatomopatológicos confirmaron el diagnóstico de un sarcoma de células claras. El paciente se encuentra libre de la enfermedad después de 6 años de operado e incorporado a su vida social (AU).

ABSTRACT The clear cell sarcoma was firstly described by Franz M. Enzinger in 1965. It is intimately associated to tendons and aponeurosis, exceptionally compromising the epidermis. It mainly affects young patients and is characterized by multiple local recurrences and late metastases. We present a male patient, aged 22 years, who underwent a radical surgical treatment (transmetatarsal amputation of the 1st and 2nd rays). The anatomic-pathological studies confirmed the diagnosis of clear cell sarcoma. 6 years after surgery, the patients is free of the disease and reincorporated to his social life (AU).

Dual tumor - Clear cell sarcoma of kidney with differentiating neuroblastoma: A rare case with review of literature.

Occurrence of two distinct synchronous primary tumors is a rare event in children as well as in adults. Here, we report an extremely rare case of an infant found to have two synchronous Tumours namely clear cell sarcoma of kidney(CCSK) and differentiating neuroblastoma of preaortic region. To our knowledge, this may be the first case of synchronous CCSK and differentiating neuroblastoma being reported.

Tumor de lento crecimiento en región frontal / A slow-growing tumor on the forehead

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Afectación metastásica pulmonar de sarcoma renal de células claras en paciente de 70 años / Pulmonary Metastatic Involvement of Clear Cell Sarcoma of the Kidney in a 70-Year-Old Patient

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FDG PET/CT findings in rare sarcomas / Hallazgos de FDG PET/CT en sarcomas raros

Hasta la fecha se ha descrito en muchos estudios el papel de la FDG PET/TAC en el manejo de los sarcomas de tejido blando y hueso. Sin embargo, la contribución de la PET/TAC al diagnóstico y al tratamiento de muchos tipos de sarcomas con baja incidencia no ha sido identificada aún. El sarcoma de células claras, el sarcoma sinovial en tórax y el liposarcoma mixoide constituyen tipos raros de sarcomas. Tratamos de describir las pautas de captación de la FDG en estos tumores raros y de averiguar el papel de la FDG PET/TAC en el manejo de la enfermedad(AU)

The role of FDG PET/CT in management of soft tissue and bone sarcomas has been described in many studies up-to-date. However, contribution of PET/CT to diagnosis and treatment in some types of sarcomas that are seen with low incidence has not been identified properly yet. Clear cell sarcoma, synovial sarcoma of chest and myxoid lyposarcoma are rare types of sarcomas. We aimed to describe the FDG uptake patterns of these rare tumors and find out the role of FDG PET/CT in management of disease(AU)

Spinal anaesthesia for caesarean section in the presence of respiratory failure and spinal metastases from a soft tissue clear cell sarcoma.

Spinal metastases occur in up to 70% of all patients with cancer. However, only 10% are symptomatic. Before considering central neuraxial blockade in patients with malignancy, a history of back pain should be excluded. Anaesthetists should be aware that intrathecal and epidural injections could cause paraplegia if metastases are impinging on the spinal cord. Failure to achieve adequate sensory anaesthesia after central neuraxial blockade or presentation with postoperative paraplegia may indicate the presence of asymptomatic vertebral canal metastases. In this report, the anaesthetic management of a patient with respiratory failure and spinal metastases from a soft tissue sarcoma, requiring caesarean section is described. Sensory anaesthesia extending above a level of imminent cord compression was achieved despite loss of cerebrospinal fluid signal on magnetic resonance imaging.

Seudotumor hemofílico: complicación poco frecuente en pacientes con deficiencia del factor VIII y IX que asemeja sarcoma de partes blandas / Haemophilic pseudotumour: a rare complication of factor VIII and IX deficiency which can mimic soft tissue sarcoma. A case report and review of the literature

El seudotumor hemofílico es una complicación infrecuente en pacientes hemofílicos y es básicamente un hematoma encapsulado en diferentes estadios de organización, que muchas veces llega a confundirse clínica y radiográficamente como un sarcoma osteogénico o de tejidos blandos. Informamos el caso de un varón de 30 años con hemofilia A y diagnóstico de seudotumor hemofílico en miembro pélvico izquierdo con evolución de un año. Se realizó amputación supracondílea en su manejo multidisciplinario, así como un estudio inmunohistoquímico. Se revisó la literatura(AU)

A rare complication of haemophilia is haemophilic pseudotumour which is an encapsulated haematoma in different stages of organization. However, it often resembling osteogenic or soft tissue sarcoma, both clinically and radiologically. We report a case of a 30 year old male with haemophilia A and a one year history of haemophilic pseudotumour of the left leg. A multidisciplinary team was responsible for his management. A supracondylar amputation was performed and immunohistochemical studies were carried out. The literature was reviewed(AU)

Pediatric clear cell sarcoma of the kidney with cavoatrial thrombus.

Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor. Only 4 cases of CCSK with vascular thrombus have been reported, and 2 of these were pediatric cases. One of the children had an intraatrial thrombus as well. We describe a 3-year-old boy who was diagnosed as having a Wilms tumor but did not respond to preresection chemotherapy. He underwent complete resection of the tumor under cardiopulmonary bypass. Histologic examination indicated that the tumor was a CCSK. The patient was then managed with appropriate chemotherapy and radiation therapy and is well 16 months after diagnosis.

Síndrome de Rud: observación de un nuevo caso / Rud´s syndrome: report of a new case

La asociación de la tríada oligofrenia, ictiosis congénita e hipogonadismo constituye el síndrome de Rud, entidad con muy pocos casos en la literatura médica mundial. Se han descrito en estos pacientes otras alteraciones menos frecuentes, como crisis convulsivas, epilepsia, talla baja, retinitis pigmentosa, polineuropatía hipertrófica o sordera neurosensorial. Forma parte de los denominados síndromes neurocutáneos queratósicos, junto con otros mejor definidos, como el síndrome de Sjögren-Larson o la enfermedad de Refsum. Se expone el caso de un paciente de 13 años de edad, con criterios clínicos compatibles con el síndrome de Rud, que asocia una agenesia renal unilateral y un sarcoma de partes blandas

The triad of oligophrenia, congenital ictiosis and hypogonadism constitutes Rud´s syndrome, and entity that is rarely reported in the world medical literature. Other less common festures such as seizures, epilepsy, short stature, retinitis pigmentosa, hypertrophic plyneuropathy or neurosensory hearing loss have also been reported in these patients. Rud´s syndrome is one of the so-called keratotic neurocutaneous syndromes, which include other better defined entities such as Sjögren-Larsson syndrome and Refsum disease. We report the case of a 13-years-old boy who met the clinical criteria for Rud´s syndrome, which was associated with unilateral renal agenesis and the development of a soft tissue sarcoma

Tricoblastoma gigante / Giant trichoblastoma

El tricoblastoma es una neoplasia cutánea benigna con diferenciación hacia células germinativas foliculares y con un estroma densamente fibrocítico que reproduce la papila folicular. Actualmente se utiliza la clasificación de Ackerman et al para denominar este grupo de neoplasias benignas. Generalmente la lesión aparece como un nódulo solitario, con mayor frecuencia en cuero cabelludo y cara, que se enuclea fácilmente durante la extirpación quirúrgica y lo más habitual es que mida entre 1 y 2 cm. Describimos el caso de un tricoblastoma de gran tamaño localizado en nalga

Trichoblastoma is a benign tumour that differentiates towards the hair germ epithelium with a dense fibrous stroma that gives a hair bulb-like appearance. Currently it is being used the classification proposed by Ackerman and colleagues. The lesion usually presents as a solitary nodule, 1-2 cm in diameter, more frequently located on the scalp and face, that is easily removed during surgery. We describe the case of a large trichoblastoma located on the buttock

Clear cell sarcoma of the kidney with cavo-atrial tumor thrombus: complete resection in a child.

Clear cell sarcoma is one of the rarely occurring renal tumors during childhood, and transvenous tumor extension into the right atrium is even more unusual. In this report, we describe the presentation, treatment, technique of complete resection, and outcome of a 23-month-old girl with clear cell sarcoma of the right kidney with cavo-atrial tumor thrombus.