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1/172. Sialoblastoma: clinicopathological/immunohistochemical study.

    Sialoblastoma is an extremely rare salivary tumor diagnosed at birth or shortly thereafter with significant variability in histologic range and clinical course, so that for an individual case it may be difficult to predict the most appropriate therapy. We detail the case of a toddler noted to have a firm 1-2-cm mass in the left cheek at 21 months. Parotidectomy was performed at 26 months, revealing a sialoblastoma; the resection margins were positive. During the next 10 months, the mass recurred or persisted, necessitating numerous procedures. The tumor was composed of basaloid cells with fine chromatin and other more mature cuboidal epithelial cells. Ductules and solid organoid nests with some tendency toward peripheral pallisading were also noted. There was no perineural invasion; necrosis initially was sparse but increased over time. The mitotic rate also increased from 6 to 7/10 high-power fields in the first resection to 20/10 high-power fields in the last resection. Nuclear pleomorphism increased with time. The MiB1 proliferative index revealed a dramatic increase in the number of labeled nuclei: from 3 cells/10 high-power fields in the first specimen to 94 cells/10 high-power fields for the last specimen. Cytokeratin accentuated the ductal structures. S-100 showed a diffuse staining pattern, with darker staining of the spindled myoepithelial cells. The Her-2-neu protein showed moderate cytoplasmic staining, whereas the p53 showed only occasional labeling of nuclei. This is the first case of sialoblastoma with evidence of increasing anaplasia based on increasing proliferative capacity. Therefore, the distinction between benign and malignant sialoblastomas may not be as well defined as previously thought. The patient's prognosis is likely to be determined by the tumor grade as well as the stage at presentation and the extent of resection.
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ranking = 1
keywords = spindle
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2/172. Desmoplastic spindle-cell melanoma of the eyelid with orbital invasion.

    PURPOSE: To describe the clinicopathologic features of a patient with a spindle-cell melanoma of the eyelid that exhibited orbital invasion. methods: Case report. RESULTS: A slowly enlarging mass developed in the eyelid of a 50-year-old woman. Excision of the mass showed desmoplastic spindle-cell melanoma. The tumor recurred in the orbit, and an exenteration was performed. A second recurrence 7 months later was treated with radiation therapy. CONCLUSIONS: There is a continuum of spindle-cell to desmoplastic melanoma. The prognosis of patients with orbital invasion of melanoma is poor, and adjuvant treatment including radiation and chemotherapy has little benefit.
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ranking = 7
keywords = spindle
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3/172. Malignant fibrous histiocytoma of the conjunctiva.

    We report the clinical and pathological findings in a case of malignant fibrous histiocytoma in the conjunctiva of a 60-year-old man. The patient initially had an atypical limbal lesion, resembling a pterygium, which was excised. Two local recurrences, noted during the following year, were treated by surgical excision followed by cryotherapy. Histopathologic examination of the conjunctival lesions showed a stromal neoplastic infiltrate composed of atypical spindle cells and histiocytelike cells. The immunohistochemical and ultrastructural studies suggested that the tumor was composed of various cellular elements: fibroblasts, myofibroblasts, and histiocytes.
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ranking = 3.7153409168688
keywords = spindle cell, spindle
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4/172. Sarcoma botryoides of the cervix. Report of a case with cytopathologic findings.

    BACKGROUND: Cytologic findings of sarcoma botryoides were still equivocal because sarcoma botryoides of the uterine cervix is an extremely rare neoplasm, and few cases have been reported to date. CASE: A 17-year-old female was diagnosed with sarcoma botryoides of the uterine cervix. The entire vaginal canal was occupied with polypoid masses, which arose from the anterior lip of the uterine cervix, and the tumor was classified as group I (Intergroup rhabdomyosarcoma Study). After wedge resection and six courses of combination chemotherapy, the tumor recurred in the same location of the cervix as the primary lesion. touch smear of the polypoid mass formed loose clusters and also showed short spindle cells in a necrotic background. The nucleus of the tumor cells had a thin nuclear membrane, fine chromatin pattern and partly clear nucleolus, showing mild nuclear atypia. Immunohistochemically, some of the tumor cells showed positive staining for myoglobin and desmin. CONCLUSION: The cytologic findings of sarcoma botryoides of the female genital tract are typical features of nonepithelial malignant tumor. Immunohistochemical study is useful for the diagnosis of rhabdomyosarcoma.
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ranking = 3.7153409168688
keywords = spindle cell, spindle
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5/172. Sarcoma of the main pulmonary artery: an unusual etiology for recurrent pulmonary emboli.

    We describe a case of primary pulmonary artery (PA) trunk spindle cell sarcoma in an 86 year old female presenting clinically with debilitating signs of recurrent pulmonary embolism. Further extensive work aroused suspicion for pulmonary artery malignancy. Palliative wide surgical resection, pulmonary artery tumor embolectomy and reconstruction of the proximal pulmonary artery and right ventricle outflow tract (RVOT) with bovine pericardial tissue were performed. She survived the procedure with an improved quality of life, but expired due to recurrence at 6 months postoperatively. Albeit uncommon, pulmonary artery sarcoma is nowadays a more frequently preoperatively diagnosed and surgically treated malignancy. With a modern low perioperative mortality, aggressive surgical resection remains as the single most effective modality for its treatment and can result in short term palliation in selected patients.
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ranking = 3.7153409168688
keywords = spindle cell, spindle
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6/172. A solitary fibrous tumor in the perianal region with a 13-year follow-up: report of a case.

    A case of a solitary fibrous tumor (SFT) of the perianal region in a 62-year-old man is reported herein. The patient had undergone an abdominoperineal excision of the rectum for an anorectal tumor 13 years previously, and had been referred to our hospital for a perineal mass. Computerized tomography and angiography revealed a markedly hypervascular tumor measuring 11 x 8 cm in size in the pelvic cavity. After preoperative radiotherapy (total 58 Gy) and the embolization of the feeding arteries, he underwent an en bloc excision of the tumor. Microscopically, the tumor was composed of spindle shaped cells with a "patternless" arrangement in a collagenous background. There was immunohistochemical evidence that these cells were strongly positive for CD34, thus suggesting the tumor to be SFT. The previously resected anorectal tumor showed similar histological and immunohistochemical findings. The patient's recovery was uneventful.
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ranking = 1
keywords = spindle
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7/172. High-grade extraskeletal myxoid chondrosarcoma: a high-grade epithelioid malignancy.

    AIMS: Extraskeletal myxoid chondrosarcoma is typically a low-to-intermediate grade sarcoma that is associated with a prolonged clinical course. High-grade forms are rare and not well characterized. In this series we report the clinicopathological, immunohistochemical and ultrastructural findings in four cases of high-grade extraskeletal myxoid chondrosarcoma. methods AND RESULTS: The patients were three men and one woman (ages 34-73 years) with tumours located in the thigh (two cases), paraspinal soft tissue and perineum. Three patients had metastases, one at 12 weeks, one at 10 months, and one at presentation of recurrent tumour. In the latter case the original tumour was low grade and became high grade when it recurred 3.5 years later. All three patients died of disease. One patient was lost to follow-up. The most striking histological feature in all four tumours was the presence of numerous large epithelioid cells. These cells were arranged in cords within myxoid matrix and in sheets devoid of matrix. Two tumours had areas of conventional extraskeletal myxoid chondrosarcoma intermixed with the high-grade areas. One tumour showed transition to high-grade spindle cell sarcoma. One tumour had cells with rhabdoid features. Immunohistochemically, two tumours focally expressed S100 protein, and one focally expressed EMA. All were negative with cytokeratin, desmin, smooth muscle actin, HMB45, CD31 and CD34. Ultrastructural features in three cases were compatible with chondrosarcoma; one tumour had aggregates of microtubules within rough endoplasmic reticulum, a characteristic feature of this tumour. CONCLUSIONS: High-grade extraskeletal myxoid chondrosaroma is a rare and aggressive soft tissue sarcoma, and should be included in the differential diagnosis of other epithelioid malignancies.
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ranking = 3.7153409168688
keywords = spindle cell, spindle
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8/172. Chondromyxoid fibroma of skull base: a tumour prone to local recurrence.

    Chondromyxoid fibroma of the skull base is extremely uncommon. Sometimes involvement of the nasal cavity may occur and the patients may present with nasal symptoms. The biological behaviour of this tumour has not been well studied, primarily because of the limited number of reported cases and the short duration of follow-up. We report a histologically confirmed case of chondromyxoid fibroma of the skull base that recurred repeatedly over a 10-year period after the initial operation. Histologically it showed identical morphology to the original tumour with no evidence of histological progression or dedifferentiation. Ultrastructurally, the spindle tumour cells in the fibromyxoid area showed dual chondroblastic and fibroblastic differentiation, suggesting that these spindle fibroblastic cells and the better differentiated chondroid cells were of the same cell type with different histological morphology.
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ranking = 2
keywords = spindle
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9/172. Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy.

    A demarcated 6 x 5 cm right paravertebral tumor at the level of T6 in a 39-year-old male was removed surgically. Histologically, the tumor consisted of monomorphous benign-looking, low-cellular spindle cells embedded in desmoplastic stroma. Ten years later, the tumor recurred locally with metastasis to systemic organs, including the occipital skin. Malignancy was histologically evident by the increased cellularity, cellular atypia and mitotic activity. The patient died of respiratory failure at the age of 49. Retrospectively reviewed, the primary lesion was low-grade fibrosarcoma-like spindle cell tumor, with secondary transformation into a highly malignant form. The differential diagnoses included sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma and malignant peripheral nerve sheath tumor. Immunohistochemically, the spindle cells in the primary and recurrent tumors consistently expressed epithelial membrane antigen, vimentin, type 4 collagen and laminin. The tumor cells in the present case showed a differentiation toward perineurial cells, which are normally positive for these immunohistochemical markers. Hence, the appropriate diagnostic term should be 'malignant perineurioma', a subtype of malignant peripheral nerve sheath tumor.
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ranking = 11.146022750606
keywords = spindle cell, spindle
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10/172. Fine needle aspiration cytology of plexiform fibrohistiocytic tumor. A case report.

    BACKGROUND: Plexiform fibrohistiocytic tumors are rare lesions of proposed myofibroblastic origin occurring primarily in infants and children. While the histologic, immunohistochemical and ultrastructural findings have been well described, cytologic description has been limited. CASE: An 8-month-old, male infant presented with a posterior chest wall mass and decreased use of his left arm. Fine needle aspiration biopsy showed a spectrum of plump fibroblastic spindle cells and histiocytelike cells within a finely granular myxoid background. Osteoclastlike giant cells were also noted. CONCLUSION: We report here the cytologic findings of a plexiform fibrohistiocytic tumor from fine needle aspiration biopsy studied using Papanicolaou, Ultrafast Papanicolaou and Diff-Quik stain, with the cytologic differential diagnosis of other spindled and histiocytelike tumors.
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ranking = 4.7153409168688
keywords = spindle cell, spindle
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