Cases reported "Retroperitoneal Neoplasms"

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1/11. Two cases of neuroblastoma presenting to the emergency department.

    neuroblastoma is the second most common malignant solid tumor in the pediatric population. Recent advances in treatment options and identification of prognostic subsets have made early detection important. Early classification into a favorable stage and subset may carry a 10-year survival of about 90% (1). With newer treatment regimens, the probability of long-term survival in patients with metastatic disease has also increased about fourfold since 1979 (2). Emergency physicians can contribute to improved morbidity and mortality with appropriate evaluation and disposition of the patient presenting with neuroblastoma. Two patients, a 6-month-old and a 2-week-old, presented to the Emergency Department with weakness. Both had a presumptive diagnosis of neuroblastoma made by the emergency physician. A brief discussion of the incidence, presentation, and diagnosis of neuroblastoma follows.
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2/11. Retroperitoneal schwannoma presenting as an adrenal tumor.

    A retroperitoneal tumor in the region of the adrenal gland was diagnosed in a 56-year-old woman. The patient had been suffering from a dull abdominal pain for nearly four weeks before consulting her family physician. Ultrasound, CT and MRI scans revealed a giant tumor of the right adrenal gland. Endocrine activity could not be demonstrated. The size of the tumor was suggestive of an adrenal carcinoma. The patient was referred for adrenalectomy and complete exstirpation of the retroperitoneal mass. The histological examination revealed characteristical findings of a benign schwannoma.
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3/11. Extrarenal Wilms' tumor.

    The extrarenal location of Wilms' tumor is extremely rare. These tumors can arise from other neoplasms, generally teratomas, or they can present without any associated teratomatous elements. We encountered only 19 well-documented cases of isolated extrarenal nephroblastoma, and we describe three previously unreported patients with this disease. Two of the three children presented also with horseshoe kidneys, an association that may have clinical and embryologic significance. The presence of tumor cephalad to a horseshoe kidney is easier to explain when we consider that they originate from primitive mesodermal tissue, probably mesonephric rests, and not from metanephric remnants. Also, this association should alert physicians to the possible diagnosis of extrarenal nephroblastoma in patients with a retroperitoneal mass and horseshoe kidneys. These patients should be treated according to the same protocols as those for patients with intrarenal Wilms' tumor.
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4/11. A new onset of fatigue in an active elderly man.

    fatigue is often perceived as part of normal aging. Yet for many active elderly, a complaint of generalized, non-specific weakness should alert the physician to the existence of possible underlying pathology, as this case demonstrates.
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5/11. Contralateral displacement of abdominal viscera by a retroperitoneal liposarcoma: ultrasonic demonstration.

    Occasional reports have described contralateral displacement of the kidney, gall bladder, or liver by masses, but no ultrasonographic description of this phenomenon has been published. To our knowledge, this is the first report of contralateral displacement of all three structures by an encapsulated, well-differentiated low-grade liposarcoma. The significance to the ultrasonographer lies in the fact that he may be the first physician, as we were in this case, to have the opportunity to recognize the true anatomical relationships of all three organs and to suggest that this tumor is more likely to be benign or slowly growing rather than a highly infiltrative process.
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6/11. Malignant schwannoma: report of a case mimicking lumbar disk disease.

    Primary neurogenic retroperitoneal tumors in the adult are rare. This case presentation reviews an unusual history of a patient who presented with pain and neurologic deficits suggesting lumbar disk disease but who was unrelieved by laminectomy and disk removal. Later he was found to have a malignant schwannoma arising from the right L nerve root. Experience such as this reminds physicians that when the result of treatment is not as expected the original diagnosis must be questioned.
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7/11. Mesenchymal chondrosarcoma of the retroperitoneum. Report of a case diagnosed by fine needle aspiration biopsy with immunohistochemical, electron microscopic demonstration of S-100 protein in undifferentiated cells.

    BACKGROUND: Mesenchymal chondrosarcoma (MC), first described in 1959 by Lichtenstein and Bernstein, is an uncommon tumor of the soft tissues and bone defined by the coexistence of nests of well-defined cartilaginous tissue within a proliferation of primitive mesenchymal cells. CASE: A 27-year-old, white female consulted a physician for a left lumbar mass associated with sciatica-type pain, asthenia, anorexia and weight loss. Abdominal computed tomography showed a 20-cm, retroperitoneal neoplasm that occupied the lower half of the left abdominal flank. Fine needle aspiration biopsy showed clusters of small round cells mixed with islets of mature cartilage. Immunomarking for electron microscopy, carried out with 15 nm of A protein/colloidal gold, showed specific immunoreactivity to S-100 protein in the nuclear chromatin, nucleoli, golgi apparatus, plasma membrane and cytosol of cells with more developed organelles. CONCLUSION: The diagnosis of MC rests on recognition of the biphasic component of small, undifferentiated cells and cartilage islets. In aspirates with only undifferentiated cells, demonstration of ultrastructure-specific immunoreactivity to S-100 protein may contribute to the final diagnosis.
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8/11. Primary retroperitoneal synovial sarcoma.

    We present a patient with primary retroperitoneal synovial sarcoma which showed a monophasic fibrous pattern, the fourth such case to be described, and a review of the literature. Synovial sarcoma cells in the present case were stained positive for cytokeratin and epithelial membrane antigen (EMA), of which histology was differentiated from other spindle cell sarcoma with similar light microscopic features. Retroperitoneal synovial sarcoma is usually treated surgically, however only one of 16 cases identified in the literature survived five years after resection. Due to the high fatality rate, physicians should be alerted to the possibility of this disorder in the differential diagnosis of an abdominal mass.
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9/11. Long-term lumboureteral shunt removed secondary to iatrogenic meningitis.

    With the advent of ventriculoperitoneal cerebrospinal fluid shunts for communicating hydrocephalus, rarely does the physician see patients with alternate types of shunting devices. The once popularized lumboureteral shunt is unique for its potential complications of dehydration, electrolyte imbalances, infection, and the sacrifice of a functioning kidney. This article presents the case of a woman with a longstanding lumboureteral shunt that was removed after the onset of iatrogenic meningitis secondary to an ascending urinary tract infection.
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10/11. lymphangioleiomyomatosis.

    Lymphangiomyomatosis (LAM) is a progressive disease afflicting women of childbearing age. LAM remains a rare disease, unfamiliar to many clinicians. It usually poses a diagnostic dilemma for the primary physician, possibly resulting in a delayed or missed diagnosis, unnecessary operative intervention, and inappropriate therapy. LAM is characterized microscopically by abnormal smooth muscle proliferation causing gradual obstruction of small airways, lymphatics, and vasculature. The proliferation eventually results in a characteristic clinical syndrome of progressive dyspnea, recurrent pneumothorax, chylous effusion, and hemoptysis. Despite a variety of treatment regimens developed since the first description of LAM, patient survival has not improved appreciably. Most patients die within 10 years of the time of diagnosis. This report presents a patient with LAM and a review of the literature.
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