Cases reported "Soft Tissue Neoplasms"

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1/16. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

    rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.
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keywords = sacrum
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2/16. magnetic resonance imaging appearance of metastatic Merkel cell carcinoma to the sacrum and epidural space.

    Merkel cell carcinoma (MCC) is a rare malignant tumor of the skin and often is diagnosed histologically as lymphoma, melanoma and even metastatic small cell carcinoma of the lung (SCCL). Classified as a neuroendocrine tumor, clinically it originates in the head and neck region and may present with metastatic disease at the time of presentation [1]. Osseous involvement in the past has been described to involve regional facial bones only. We present the first reported MRI findings of distant osseous metastasis from a Merkel cell carcinoma to the lumbosacral spine with associated soft tissue and epidural involvement. Appropriate treatment and patient survival depend on prompt diagnostic imaging for establishment of metastatic disease. Previous reports have advocated CT for diagnosis and staging of distant metastases [2,3]. When spinal involvement is suspected, MRI may be a more suitable modality for assessment of the epidural space and appropriate staging and follow-up in such cases.
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keywords = sacrum
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3/16. Neurofibromatosis involving the urinary bladder.

    We present two interesting cases of a 24-year-old man and a 14-year-old boy, uncle and nephew, with lower urinary tract symptoms, cafe au lait patches and subcutaneous nodules. ultrasonography and computed tomography scans showed a large, irregular lobulated soft tissue mass between the bladder and sacrum. cystoscopy, laparotomy and biopsies revealed neurofibromatosis involving the urinary bladder. No enlargement of the tumor or upper urinary tract obstruction has occurred during the long-term follow up. We recommend meticulous follow up of patients with giant intrapelvic neurofibromatosis.
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keywords = sacrum
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4/16. Newborn with an open posterior hip dislocation and sciatic nerve injury after intrauterine radiofrequency ablation of a sacrococcygeal teratoma.

    Advanced prenatal ultrasonography techniques have allowed for better understanding of the natural history, treatment, and prognosis of sacrococcygeal teratomas. Several intrauterine surgical techniques to debulk the tumor when fetal and maternal life are in jeopardy have been described. Orthopaedic impairment, such as lower extremity weakness and swelling, also has been described in association with sacrococcygeal teratomas. The authors report on a newborn in whom a large soft tissue defect overlying the posterior hip region with direct exposure of the disarticulated hip joint existed at the time of birth, which resulted from intrauterine radiofrequency ablation of a sacrococcygeal teratoma. This unexpected complication has resulted in a loss of sciatic nerve function, malformation of the acetabulum and femoral head, and loss of the left ischium, coccyx, inferior sacrum, gluteal, adductor and piriformis muscles, and posterior hip capsule. At 16 months of age, the patient has a flaccid left lower extremity with a hypoplastic hip joint.
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keywords = sacrum
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5/16. Noninvasive sacral chordoma presenting as a benign soft tissue mass.

    In this case report, we describe a sacral chordoma, which had an atypical presentation as a mobile, encapsulated, benign soft tissue mass. The patient was asymptomatic, except for the slight enlargement of this lesion. biopsy of this mass showed a lobulated tumor with bland neoplastic cells in a rich myxoid matrix with the classical immunohistochemical profile of chordoma. Opposite to this classical histological picture of chordoma, the imaging studies (computed tomography and magnetic resonance imaging) could not find any sacral involvement or lytic destruction. Surgical excision of this chordoma confirmed all preoperative findings and diagnoses, showing an encapsulated mass in the sacral soft tissue that has not invaded into the sacrum. This chordoma originated from the sacrococcygeal joint and grew parallel to the sacrum and below the skin. At the same time, histological sections and immunostains reconfirmed diagnosis of chordoma.
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keywords = sacrum
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6/16. Inflammatory fibrous histiocytoma: response to non-surgical therapy: a case report.

    A case of inflammatory fibrous histiocytoma arising in soft tissue near the sacrum is presented. The patient's mode of presentation, clinical course, and tumor histology were typical of this disease. The tumor was inoperable, and radiotherapy combined with doxorubicin, cyclophosphamide, and intermediate-dose oral methotrexate produced a dramatic complete response lasting over 27 months. Favorable results with nonsurgical therapy have not previously been reported for this disease. The gratifying result obtained, although of relatively short duration to date, indicates that combined modality therapy may provide significant back-up to aggressive surgery. The use of these agents in an adjuvant setting merits study.
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ranking = 1
keywords = sacrum
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7/16. Two-stage therapy in the treatment of sacral tumors.

    Sacral tumors are rare and may be clinically overlooked for a long period, because the symptoms and signs are often mild and non-specific. This led to frequent errors in clinical diagnosis and a long delay between the onset of symptoms and treatment. On presentation the lesions frequently expanded the anterior cortex, however, in most patients the periosteum of the sacrum and the presacral fascia form an unbroken barrier for a tumor. The pelvic viscera are not infiltrated by the tumor until late. Wide excision is difficult and often causes urogenital and/or anorectal dysfunction, but preserving the sacral nerve roots often leads to local recurrence. Surgical wide excision with a combined anterior-posterior approach is considered the treatment of choice for large lesions with significant anterior intrapelvic extension. This paper reports data resulting from the treatment of five large sacral tumors with comments on the results.
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keywords = sacrum
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8/16. Presacral myxopapillary ependymoma presenting as an abdominal mass in a child.

    Ependymomas of the sacrococcygeal region almost always arise on the posterior aspect of the sacrum in the soft tissues and subcutaneous tissues of this region. The predominant histologic type of ependymoma in the sacrococcygeal area is myxopapillary. We report a case of myxopapillary ependymoma arising in the presacral area and presenting clinically as an abdominal mass. Ependymomas arising on the ventral aspect of the sacrum are exceedingly rare, and only 24 previous cases have been reported in the English literature. While dorsal myxopapillary ependymomas occasionally metastasize, there have been no reports of metastasis in the presacral tumors of this type.
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ranking = 2
keywords = sacrum
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9/16. Ectopic immature renal tissue over the dorsum of lumbar and sacral area in two infants.

    During reparative surgery for meningomyelocele of the lumbar area, a 7-week-old female infant was found to have a small, well-delineated, subcutaneous, renal blastema. A 3-month-old female infant was found to have immature renal tissue, consisting of glomeruli and tubules, in a soft tissue swelling dorsal to the sacrum. Neither of these patients showed neurologic or renal abnormalities. The second patient has had a disease-free follow-up period of six years. The possible etiology and significance of these benign findings and their relation to the origin of Wilms tumors are discussed.
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ranking = 1
keywords = sacrum
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10/16. Computed tomography of tumors of the musculoskeletal system in children. Clinical applications.

    Studies indicate that computed tomography (CT) has several important clinical applications in the evaluation of tumors of the musculoskeletal system. These include (a) diagnosis of lesions involving difficult areas such as the sacrum and ilium, (b) demonstration of soft-tissue components of bone tumors or primary soft-tissue masses, (c) assessment of bone-marrow involvement, and (d) follow-up after irradiation or chemotherapy. Several selected pediatric cases are described in detail.
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keywords = sacrum
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