Subject(s)
Anemia/etiology , Colon, Sigmoid/pathology , Gastrointestinal Hemorrhage/etiology , Sarcoma, Clear Cell/diagnosis , Sigmoid Neoplasms/diagnosis , Adult , Anemia/therapy , Biomarkers, Tumor/analysis , Biomarkers, Tumor/genetics , Biopsy , Colectomy , Colon, Sigmoid/diagnostic imaging , Colon, Sigmoid/surgery , Colonoscopy , Cytogenetic Analysis , Diagnosis, Differential , Erythrocyte Transfusion , Gastrointestinal Hemorrhage/therapy , Humans , Ileostomy , Immunohistochemistry , Intestinal Mucosa/diagnostic imaging , Intestinal Mucosa/pathology , Intestinal Mucosa/surgery , Male , Rectum , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/genetics , Sarcoma, Clear Cell/surgery , Sigmoid Neoplasms/complications , Sigmoid Neoplasms/genetics , Sigmoid Neoplasms/surgery , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
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).
Subject(s)
Humans , Male , Young Adult , Tendons/abnormalities , Sarcoma, Clear Cell/epidemiology , Aponeurosis/abnormalities , Patients/psychology , Disease/classification , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Epidermis/injuries , Amputation, Surgical/rehabilitationABSTRACT
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).
Subject(s)
Humans , Male , Young Adult , Tendons/abnormalities , Sarcoma, Clear Cell/epidemiology , Aponeurosis/abnormalities , Patients/psychology , Disease/classification , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Epidermis/injuries , Amputation, Surgical/rehabilitationSubject(s)
Sarcoma, Clear Cell/diagnostic imaging , Skin Neoplasms/diagnostic imaging , Child , Chronic Disease , Female , Foot Ulcer/etiology , Humans , Magnetic Resonance Imaging , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/pathology , Skin Neoplasms/complications , Skin Neoplasms/pathologyABSTRACT
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.
Subject(s)
Kidney Neoplasms/diagnosis , Kidney Neoplasms/pathology , Kidney/pathology , Neuroblastoma/complications , Neuroblastoma/diagnosis , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Biomarkers, Tumor/analysis , Histocytochemistry , Humans , Immunohistochemistry , Infant , Male , Microscopy , Neuroblastoma/pathology , Sarcoma, Clear Cell/pathologyABSTRACT
No disponible
Subject(s)
Humans , Male , Aged, 80 and over , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Sarcoma, Clear Cell/pathology , Immunohistochemistry/methods , Frontal Bone/pathology , Dermis/anatomy & histology , Dermis/pathology , Mitosis , Diagnosis, DifferentialSubject(s)
Lung Neoplasms/complications , Lung Neoplasms/secondary , Pulmonary Veins , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/secondary , Soft Tissue Neoplasms/pathology , Adult , Constriction, Pathologic/etiology , Female , Humans , Lung Neoplasms/pathology , Neoplasm Invasiveness , Sarcoma, Clear Cell/pathologyABSTRACT
No disponible
Subject(s)
Humans , Female , Middle Aged , Sarcoma/complications , Sarcoma , Neoplasm Metastasis/diagnosis , Neoplasm Metastasis/physiopathology , Carcinoma, Renal Cell/complications , Carcinoma, Renal Cell , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/pathology , Sarcoma, Clear Cell , Radiography, Thoracic/instrumentation , Radiography, Thoracic/methods , Radiography, Thoracic , Lung Neoplasms/complications , Lung Neoplasms/psychology , Lung NeoplasmsABSTRACT
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)
Subject(s)
Humans , Male , Female , Adolescent , Young Adult , Adult , Sarcoma , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Positron-Emission Tomography/instrumentation , Positron-Emission Tomography/methods , Positron-Emission Tomography , Prognosis , Sarcoma, Clear Cell , Liposarcoma , Leiomyosarcoma , Liposarcoma, Myxoid , Nuclear Medicine/methodsABSTRACT
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.
Subject(s)
Anesthesia, Obstetrical/methods , Anesthesia, Spinal/methods , Cesarean Section/methods , Respiratory Insufficiency/complications , Sarcoma, Clear Cell/secondary , Spinal Neoplasms/secondary , Adult , Apgar Score , Bone Neoplasms/pathology , Female , Humans , Infant, Newborn , Magnetic Resonance Imaging , Palliative Care , Pregnancy , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/pathology , Spinal Neoplasms/complications , Spinal Neoplasms/pathologyABSTRACT
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)
Subject(s)
Humans , Female , Hemophilia A/complications , Hemophilia A/diagnosis , Hemophilia A/pathology , Hemophilia B/complications , Hemophilia B/diagnosis , Hemophilia B/pathology , Factor IX , Soft Tissue Neoplasms/pathology , Sarcoma, Clear Cell/pathology , Immunohistochemistry/methods , Hemorrhage/complications , Soft Tissue Neoplasms/diagnosis , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Immunohistochemistry , Hemorrhage/diagnosis , Hemorrhage/pathology , Hemarthrosis/complications , Hemarthrosis/diagnosis , Hemarthrosis/pathologySubject(s)
Humans , Male , Aged, 80 and over , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Sarcoma, Clear Cell/pathology , Melanoma/diagnosis , Melanoma/pathology , Tongue Neoplasms/complications , Tongue Neoplasms/diagnosis , Tongue Neoplasms/pathology , Neoplasm Metastasis/diagnosis , Neoplasm Metastasis/pathology , Tongue/pathology , /methodsABSTRACT
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.
Subject(s)
Diagnostic Errors , Heart Atria/pathology , Kidney Neoplasms/diagnosis , Nephrectomy/methods , Sarcoma, Clear Cell/diagnosis , Thrombectomy , Vena Cava, Inferior/pathology , Venous Thrombosis/etiology , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cardiopulmonary Bypass , Child, Preschool , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Dactinomycin/administration & dosage , Doxorubicin/administration & dosage , Etoposide/administration & dosage , Heart Atria/diagnostic imaging , Heart Atria/surgery , Hepatic Veins/diagnostic imaging , Hepatic Veins/pathology , Hepatic Veins/surgery , Humans , Kidney Neoplasms/complications , Kidney Neoplasms/drug therapy , Kidney Neoplasms/pathology , Kidney Neoplasms/radiotherapy , Kidney Neoplasms/surgery , Male , Neoadjuvant Therapy , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/drug therapy , Sarcoma, Clear Cell/pathology , Sarcoma, Clear Cell/radiotherapy , Sarcoma, Clear Cell/surgery , Tomography, X-Ray Computed , Ultrasonography , Vena Cava, Inferior/diagnostic imaging , Vena Cava, Inferior/surgery , Venous Thrombosis/diagnostic imaging , Venous Thrombosis/surgery , Vincristine/administration & dosage , Wilms Tumor/diagnosisABSTRACT
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
Subject(s)
Male , Child , Humans , Mucopolysaccharidosis III/complications , Ichthyosis/complications , Keratosis/complications , Keratosis/surgery , Hypogonadism/complications , Hypogonadism/diagnosis , Dwarfism/complications , Biopsy/methods , Sarcoma, Clear Cell/complications , Sarcoma, Clear Cell/diagnosis , Abnormalities, Multiple/genetics , Sarcoma/complications , Keratosis/diagnosis , Sarcoma/diagnosis , Growth Disorders/genetics , Hyperbilirubinemia/complications , Hyperbilirubinemia/diagnosis , Intellectual Disability/complicationsABSTRACT
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
Subject(s)
Female , Middle Aged , Humans , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Neoplasms, Adnexal and Skin Appendage/diagnosis , Neoplasms, Adnexal and Skin Appendage/therapy , Histiocytoma, Benign Fibrous/complications , Histiocytoma, Benign Fibrous/diagnosis , Biopsy/methods , Mitosis/physiology , Sarcoma, Clear Cell/complications , Adenocarcinoma, Clear Cell/complicationsABSTRACT
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.