Manifestaciones paraneoplásicas a propósito de un caso con sarcoma de partes blandas y metástasis ósea / Paraneoplastic manifestations regarding a case with soft tissue sarcoma and bone metastases

Introducción: El sarcoma de tejidos blandos (STB) constituye un grupo variado de más de 60 neoplasias que solo suponen 1 por ciento del total de los tumores. Objetivo: Describir el caso de un paciente con sarcoma de partes blandas con metástasis ósea y manifestaciones paraneoplásicas. Caso clínico: Se presenta el caso de un paciente masculino de 55 años de edad con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que refiere que hace 50 días inició con decaimiento marcado, luego de permanecer 1 mes con este síntoma inició con dolor en la columna en la parte más baja de la espalda, conjuntamente iniciando con un cuadro febril. Además, tuvo pérdida de la fuerza principalmente en las piernas, y hablaba cosas incoherentes sin sentido, gran inapetencia, con pérdida de peso de 50 lb en 20 días. Ante cuadro de pérdida de la conciencia y de la fuerza principalmente en las piernas se decidió su remisión hacia el Hospital Clínico Quirúrgico Lucía Íñiguez Landín de Holguín donde se concluyó como sarcoma de partes blandas con manifestaciones paraneoplásicas y metástasis ósea. Conclusiones: El caso presentado de sarcoma de partes blandas pretende aportar información sobre esta enfermedad tan infrecuente en nuestro medio(AU)

Introduction: Soft tissue sarcoma (STB) constitutes a varied group of more than 60 malignancies that only accounts for 1% of all tumors. Objective: To report a case of a patient with soft tissue sarcoma with bone metastases and paraneoplastic manifestations. Clinical case report: We report the case of a 55-year-old male patient with a history of type 2 diabetes mellitus and hypertension, who stated that 50 days ago he began to feel markedly listless. And after a month he began to suffer pain in the spine, in the lower back, and have a fever. In addition he had loss of strength mainly in the legs, and spoke inconsistently, nonsense, great loss of appetite, with weight loss of 50 lbs in 20 days. Given the condition of loss of consciousness and strength, mainly in the legs, it was decided to refer him to Lucía Iñiguez Landín Surgical Clinical Hospital in Holguín, where a soft tissue sarcoma with paraneoplastic manifestations and bone metastases was diagnosed. Conclusions: The report of this case of soft tissue sarcoma aims to provide information about this disease, which is so rare in our setting(AU)

Alveolar soft-part sarcoma: Case demonstrating principles for uncommon vascular lesions.

BACKGROUND: Alveolar soft-part sarcoma (ASPS) is an uncommon malignancy that may present in a manner similar to benign vascular tumors. METHODS: A 6-year-old boy with autism spectrum disorder was referred to the Johns Hopkins All Children's Hospital vascular anomalies clinic for the evaluation of a tongue mass. RESULTS: Prior to the presentation, at 5 years of age, neck computed tomography (CT) was performed. This showed a well circumscribed, enhancing mass at the anterior aspect of the tongue. The radiologic impression was that this lesion was most likely a hemangioma. Two years later, the patient was evaluated in the vascular anomalies clinic. At that examination a 2-3 cm swelling was noted on the dorsal aspect of the tongue. The mass was fleshy and firm with discrete borders. Handheld Doppler examination indicated a high-flow lesion. The patient underwent an excisional biopsy. The lesion was identified as an alveolar soft-part sarcoma based on pathologic characteristics. CONCLUSION: Familiarity with common vascular tumors and malformations allows providers to diagnose the majority of these lesions on a combination of clinical history and physical examination. Atypical and combined lesions do benefit from imaging to help characterize and aid in the differential diagnosis. Biopsy enables definitive diagnosis but is necessary in the minority of cases. When in doubt, referral to a specialized, multidisciplinary vascular anomalies clinic will ensure that these patients receive management for this challenging collection of conditions.

A retrospective analysis of lung metastasis in 64 patients with alveolar soft part sarcoma

Objectives. To analyse the lung metastasis and possible factors influencing lung metastasis in alveolar soft part sarcoma (ASPS) patients. Methods. The medical records of 64 consecutive ASPS patients were reviewed to analyse their treatments, features of lung metastasis, and possible factors influencing lung metastasis. Results. Thirty-six females and 28 males with a median age of 27 years were included. The primary disease sites were the extremities in 51 patients and other locations in 13 patients. The median primary tumour size was 5 cm. Wide local excision of the primary tumour was performed on 56 patients (87.5 %). Thirteen patients (20.3 %) received postoperative adjuvant radiotherapy, and nine patients (14.1 %) underwent adjuvant chemotherapy. Twelve patients (18.8 %) presented with metastatic lung disease. Twenty-nine patients (45.3 %) developed metastatic lung disease during follow-up. Lung metastasis occurred in 64.1 % of the patients. Lung metastasis was detected at a median interval of 20 months after primary ASPS diagnosis. Being male, >20 years of age, having a primary tumour size ≥5 cm, and local recurrence were associated with a greater rate of lung metastasis. Median survival after the diagnosis of lung metastasis was 34 months. The 5-year survival rates were 64.1 and 95.2 % for patients with and without lung metastasis (P < 0.001). Thirty-seven patients with metastatic lung disease received anthracycline- and ifosfamide-based chemotherapy. One patient experienced a partial remission. Conclusions. ASPS patients have a high prevalence of lung metastasis. Sex, age, primary tumour size, and local recurrence are major factors influencing lung metastasis. Chemotherapy is not efficacious in ASPS patients with lung metastasis (AU)

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Gastric metastasis from an alveolar soft part sarcoma in a child: case report and review of the literature.

The authors report a child with alveolar soft part sarcoma who developed significant anemia due to gastrointestinal blood loss. Evaluation revealed the source of bleeding as a gastric metastasis, which was successfully removed. A brief review of gastrointestinal involvement by alveolar soft part sarcoma is discussed.

Alveolar soft part sarcoma of the uterine cervix in a woman presenting with postmenopausal bleeding: a case report and literature review.

INTRODUCTION: Alveolar soft part sarcoma (ASPS) of the uterine cervix is a rare mesenchymal malignancy that occurs in adolescents and young adults. CASE REPORT: A 52-year-old postmenopausal woman presented with profuse vaginal bleeding of one month's duration with severe anemia. The pelvic examination revealed a 3 cm mass on the posterior lip of the uterine cervix. On magnetic resonance imaging, the tumor had high signal intensity on T1- and T2-weighted images. A modified radical hysterectomy and bilateral salpingo-oophorectomy were performed. Immunohistochemical staining for TFE3 and electron microscopic examination revealed an ASPS of the uterine cervix. DISCUSSION: The better prognosis of cervical ASPS, compared to the soft counterparts, may be related to early clinical detection, small size, resectability, and demarcation of the tumor.

Leiomiosarcoma cutáneo primario desarrollado sobre cicatriz de quemadura: reporte de un caso / Primary cutaneus leiomyosarcoma developed on burn scar: report of a case

El leiomiosarcoma cutáneo primario es una neoplasia maligna originada de músculo liso, es infrecuente y de forma esporádica se ha encontrado asociado a trauma o exposición a radiación. Por factores pronósticos se subdividen en dérmicos y subcutáneos, el tipo dérmico tiende a crecer lentamente y tiene un mejor pronóstico que el tipo subcutáneo. Se presenta el caso de un paciente masculino de 87 años de edad con antecedente de quemadura con agua caliente en el 1/3 distal de pierna izquierda hace 20 años. Sobre la misma se desarrolló tumor de 3 años de evolución, con ulceración en los últimos meses, no dolorosa. Por sospecha clínica de carcinoma de células escamosas se realizó biopsia en la que se observó tumor dérmico con extensión a tejido celular subcutáneo que correspondióa leiomiosarcoma cutáneo, el cual se confirmó con estudio de inmunohistoquímica...(AU)

Leiomiosarcoma cutáneo primario desarrollado sobre cicatriz de quemadura: reporte de un caso / Primary cutaneus leiomyosarcoma developed on burn scar: report of a case

El leiomiosarcoma cutáneo primario es una neoplasia maligna originada de músculo liso, es infrecuente y de forma esporádica se ha encontrado asociado a trauma o exposición a radiación. Por factores pronósticos se subdividen en dérmicos y subcutáneos, el tipo dérmico tiende a crecer lentamente y tiene un mejor pronóstico que el tipo subcutáneo. Se presenta el caso de un paciente masculino de 87 años de edad con antecedente de quemadura con agua caliente en el 1/3 distal de pierna izquierda hace 20 años. Sobre la misma se desarrolló tumor de 3 años de evolución, con ulceración en los últimos meses, no dolorosa. Por sospecha clínica de carcinoma de células escamosas se realizó biopsia en la que se observó tumor dérmico con extensión a tejido celular subcutáneo que correspondióa leiomiosarcoma cutáneo, el cual se confirmó con estudio de inmunohistoquímica...

Alveolar soft-part sarcoma presenting with eosinophilia and shunt.

Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumour found in young adults that usually arises in skeletal muscle and occurs most frequently in the lower limbs. Radiological and pathological findings of ASPS in a 34-year-old man who presented with increasing shortness of breath over a period of four to six weeks with peripheral blood eosinophilia, hypoxemia and a significant arteriovenous shunt are reported. The present article is the fourth report of eosinophilia in association with sarcoma, and the first involving ASPS.

Deep venous thrombosis revealed during ultrasound-guided femoral nerve block.

Ultrasound imaging used to facilitate performance of a femoral nerve block also affords imaging of adjacent anatomical structures. Following a fracture of the femur, an ultrasound guided femoral nerve block (UGFNB) was performed to provide analgesia; this led to the incidental finding of a previously undiagnosed femoral vein thrombosis (DVT), resulting in a change in patient management before surgery. An inferior vena cava (IVC) filter was placed before intramedullary nailing of the fracture.

Alveolar soft-part sarcoma presenting with multiple intracranial metastases.

A 28-year-old man presented with history of raised intracranial pressure and one episode of generalized tonic clonic seizures. Computed Tomogram revealed multiple contrast enhancing intracranial lesions. Biopsy of one of the lesions was reported as metastatic alveolar soft part sarcoma. He was advised whole brain radiotherapy.

Alveolar soft part sarcoma of the vagina. A case report.

We present a case of alveolar soft part sarcoma of the vagina in an 18-year-old woman. The presenting symptoms were menometrorrhagia and a painless vaginal mass. The tumor was in the vaginal stroma with intact mucosa. Periodic acid-Schiff stain showed positive diastase-resistant crystals in some of the tumor cells. Electron microscopy demonstrated characteristic intracytoplasmic crystals. Formaldehyde-induced fluorescence stain showed no catecholamines, and Grimelius stain showed no argyrophilic granules. Black pigment was found within some tumor cells; it proved to be melanin pigment by Masson-Fontana stain. The tumor mass recurred four months after local excision. Following the second surgical removal, the patient received adjuvant radiotherapy to the whole pelvis, including the vagina. At this writing she was currently alive, without evidence of recurrence, five years after the primary diagnosis.