Cases reported "Thoracic Neoplasms"

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11/18. Acquired ichthyosis in a child with rhabdomyosarcoma.

    A generalized ichthyosiform eruption developed in a 13-year-old boy three weeks before presentation with a paravertebral rhabdomyosarcoma. His skin returned to normal shortly after the onset of chemotherapy and remission of the tumor. This case is unusual because the ichthyosis developed acutely, following a generalized urticarial eruption, and also involved the palms and soles.
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ranking = 1
keywords = rhabdomyosarcoma
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12/18. methotrexate as relapse therapy for rhabdomyosarcoma.

    Four patients had a local relapse after standard therapy for rhabdomyosarcoma and were treated with high-dose 42-hour MTX infusions. All patients responded to this therapy, one patient had a complete, and two patients a partial remission. Long duration MTX infusion should be part of a combination chemotherapy for relapsed rhabdomyosarcomas.
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ranking = 1.2
keywords = rhabdomyosarcoma
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13/18. Modified technique for radical transmediastinal forequarter amputation and chest wall resection.

    Forequarter or interscapulothoracic amputation is a major surgical procedure indicated primarily in the treatment of malignant lesions involving the bony and soft tissue parieties of the upper part of the arm, shoulder, and scapula. It is also indicated in extensive trauma with irreparable damage to the shoulder area and as a palliative measure in intractable pain caused by incurable tumors of the shoulder girdle. Several operative techniques have been described: the classical Berger approach, an anterior pectoral approach, and posterior retroscapular approaches. A radical transthoracic approach has been described in cases in which the tumor had spread through the chest wall. The two main goals of these approaches have been early ligation of the subclavian vessels and immediate exploration for operability. This report details our experience with a modified technique for radical forequarter amputation and chest wall resection in which a transmediastinal approach is employed. This approach was used in two patients: One had a radiation-induced fibrosarcoma of the left axilla and adjacent chest wall following a radical mastectomy 19 years earlier, and the other patient had a recurrent rhabdomyosarcoma of the right axilla with invasion of the chest wall. This technique avoids time-consuming and individual excision of ribs and minimizes the amount of blood loss by early ligation of the internal mammary vessels. Safe and excellent exposure and division of the subclavian vessels and early exploration for mediastinal and intrathoracic involvement are made possible. Details of the procedure with illustrations are described.
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ranking = 0.2
keywords = rhabdomyosarcoma
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14/18. Primitive neuroectodermal tumour of the chest wall.

    The concept of primitive neuroectodermal tumour (PNET) has been evolving for many years, as has its nomenclature. It was first described as a tumour arising in peripheral nerve, when it was called neuroepithelioma. These tumours are part of the differential diagnosis of malignant small round cell tumours, which include Ewing's sarcoma, rhabdomyosarcoma, neuroblastoma and lymphoma, and which appear as sheets of monotonous small round cells on light microscopy, staining dark blue with haematoxylin and eosin. In the 1970's, reports of tumours displaying neural features introduced new terms such as primitive neuroectodermal tumour and peripheral neuroectodermal tumour. A relationship to the undifferentiated primitive neuroectodermal tumours of the central nervous system in children was assumed by the title of these tumours, although it was recognised that they were not necessarily related to peripheral nerves.
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ranking = 0.2
keywords = rhabdomyosarcoma
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15/18. Primary carcinoma of the choroid plexus in li-fraumeni syndrome: case report.

    An 8-month-old female infant with a primary carcinoma of the choroid plexus developed a rhabdomyosarcoma in the anterior chest wall at the age of 1 year and 2 months. Her mother had developed a liposarcoma in her left thigh at the age of 17 years. One of the patient's siblings had a rhabdomyosarcoma of the epipharynx at the age of 1 year. This is the fourth reported case of a choroid plexus carcinoma occurring in li-fraumeni syndrome.
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ranking = 0.4
keywords = rhabdomyosarcoma
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16/18. Intracerebral recurrence of primary intrathoracic rhabdomyosarcoma.

    We present two cases of intracerebral recurrence of primary intrathoracic rhabdomyosarcoma, occurring within 2 months of each other. Both had embryonal histological features. One (case 1) almost certainly arose within the lung adjacent to a developmental cyst; the intrathoracic site of origin of the other (case 2) was difficult to ascertain. Both were treated on SIOP MMT protocols. The intracerebral deposits became clinically apparent soon after completing chemotherapy. They were removed surgically and second-line chemotherapy was given both systemically and intrathecally. Neither received radiotherapy. The disease recurred in both children whilst on therapy and they died as a consequence of raised intracranial pressure.
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ranking = 1
keywords = rhabdomyosarcoma
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17/18. Cerebral metastases of alveolar rhabdomyosarcoma in an infant with multiple skin nodules.

    PURPOSE: This report describes extremely rare cases of infantile rhabdomyosarcoma with multiple skin nodules. They are of interest not only for their anatomic sites, but also for subsequent cerebral metastases with sudden cranial hypertension. patients: Two infants had multiple skin nodules and excisional biopsy revealed alveolar type rhabdomyosarcomas. The patients were treated with tumor resection and combined chemotherapy without any clinical progression for 9 and 16 months, respectively. RESULTS: Evidence of cerebral metastases developed with sudden vomiting and convulsion as the first manifestation. In one patient, urgent radiographic examinations failed to reveal lesions except for dilated cerebral ventricles. Seven weeks after the onset of the neurologic symptoms, only Gd-DPTA-enhanced magnetic resonance imaging (MRI) revealed multiple punctate metastatic lesions hyperintense to the surrounding cerebral tissue. Despite appropriate chemotherapy, both patients had disease progression and died of central nervous system metastases. CONCLUSIONS: The authors emphasize the need to recognize the multiple cutaneous presentation of infantile rhabdomyosarcoma and the association of cerebral metastases as a potential and fatal complication. The diagnosis is facilitated by Gd-DPTA-enhanced MRI, particularly when cerebral computed tomography scans fail to disclose metastatic lesions.
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ranking = 1.4019097399098
keywords = rhabdomyosarcoma, alveolar
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18/18. Thoracic manifestations of renal cell carcinoma.

    Over 1/3 of patients with renal cell carcinoma will present with metastases with no symptoms referable to the kidney. Early metastatic disease is a result of the unique accessibility of the kidney to lymphohematogenous pathways. The thorax is particularly vulnerable. Several of the more unusual manifestations involving the thorax are illustrated, including 1 case with intra-alveolar metastases.
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ranking = 0.00038194798195421
keywords = alveolar
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