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1/564. First cytogenetic study of a recurrent familial chordoma of the clivus.

    Two recurrences of a familial clivus chordoma, arisen from a patient who developed the primary tumor at age of 8 years, were investigated by cytogenetic and the fluorescence in situ hybridization (FISH) approach. Of the patient's 3 daughters, 2 developed, respectively, a clivus chordoma and an astrocytoma in infancy, a familial aggregation highly suggestive of a genetic background. After a 31-year hiatus, 2 tumor recurrences, developed over 17 months, were removed surgically. Both were hypo- or nearly diploid, and had a pronounced karyotypic heterogeneity with clonal and non-clonal rearrangements affecting several chromosomes. The same rearrangement, a dic(1;9)(p36.1;p21), was shared in both tumor specimens and, in 90% of the cells, chromosome 1p appeared to be involved in unbalanced translocations with different chromosomes, leading to variable losses of 1p. Previous cytogenetic data concerning chordoma are limited to 10 sporadic tumors with an abnormal karyotype; although no tumor-specific rearrangements have been identified, chromosome 1p appears to be involved frequently.
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2/564. A glomus tumour with four recurrences.

    A case of a glomus tumour of the distal segment of the ring finger, with four apparent recurrences in an 8-year period, is described. The patient was treated by two different surgeons (two and three times respectively) and obtained pain free intervals of between 4 and 11 months before recurrence. Histological examination confirmed the diagnosis of a glomus tumour in all five procedures. The location of the glomus tumour was defined preoperatively by high resolution MR imaging.
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ranking = 3.1969369640963
keywords = pain
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3/564. Ductal carcinoma in situ recurrent on the chest wall after mastectomy.

    Two patients with local recurrence on the chest wall subsequent to mastectomy for ductal carcinoma in situ (DCIS) are presented. One recurrence was invasive carcinoma and the second was DCIS. Excision and chest wall irradiation, together with chemotherapy in the first patient, have provided subsequent disease-free survivals of 6 and 12 years respectively. Although mastectomy for DCIS is almost always curative, the possibility of local recurrence requires careful surveillance.
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ranking = 44.699837337159
keywords = chest
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4/564. Soft tissue masses of the chest wall and axilla: has metastatic melanoma been considered?

    Isolated axillary and chest wall soft tissue masses are an uncommon presentation of metastatic cancer. The authors present three patients in whom malignant melanomas metastatic to these sites had been misdiagnosed, leading to inappropriate oncologic treatment planning in all three cases. The presumed diagnoses, even after fine-needle aspiration or trucut biopsies, were soft-tissue sarcoma (n = 2) and undifferentiated breast cancer (n = 1). The combination of taking a thorough history and performing proper immunohistochemical analysis of the biopsy material would have suggested the presence of malignant melanoma in all cases. As the disease appeared locoregionally limited in all patients, radical surgical resection with extended lymphadenectomy was performed without significant dysfunction of the upper extremity. One patient agreed to postoperative immunotherapy with interferon-alpha. Two patients are currently alive 17 and 14 months after operation. One patient was found to have systemic recurrence at 5 months, one experienced two isolated local recurrences in a prior operative site that were amenable to reresection and presently has no evidence of disease 12 months after resection, and one patient remains free of disease at 14 months. Clinical presentation, suggested diagnostic workup, and therapeutic implications are discussed to avoid misdiagnoses in this setting of possible clinical presentations of metastatic melanoma.
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ranking = 37.249864447632
keywords = chest
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5/564. Primary orbital leiomyoma and leiomyosarcoma.

    A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.
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keywords = pain
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6/564. Desmoplastic fibroma of the calcaneus treated with a microvascular bone graft. Case report.

    A calcaneal desmoplastic fibroma in a 24-year-old woman had previously been treated with repeated curettage and bone grafting. Because of a larger recurrence a microvascular bone reconstruction was carried out using an osteofascial radial forearm flap. She could walk without pain in one year. It has not recurred during a five-year follow-up period.
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keywords = pain
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7/564. Recurrent pleomorphic solitary angiomyxoma of the face.

    The historical background of solitary and multiple myxoid tumors of the dermis is accompanied by a confusing conglomeration of names. We report a case whose histologic features do not fit exactly with previous classifications. It appeared as a solitary angiomyxoma of the face containing stellate pleomorphic cells and exhibiting a locally infiltrative behavior with tendency for recurrences over more than 25 years. This recurrent pleomorphic solitary angiomyxoma appears to be distinct from cutaneous myxomas that exhibit a very low local recurrence rate and from malignant tumors with myxoid deposits. The treatment consisted of a wide surgical excision.
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8/564. A leptomeningeal metastasis revealed by sciatica.

    Meningeal metastatic disease usually occurs as a complication of a brain tumor and is exceptionally isolated in patients with solid tumors. We report the case of a 74-year-old woman admitted for mechanical S1 sciatica refractory to drug therapy. She had been treated for breast cancer three years earlier. Physical findings were pain upon hyperextension of the lumbar spine and absence of the ankle jerks. Analysis of cerebrospinal fluid sampled during an intrathecal glucocorticoid injection showed 1 g/L of protein and 11 normal cells per mm3. Grade 3 L5-S1 spondylolisthesis was seen on plain radiographs, computed tomography scans, and magnetic resonance imaging scans. At that point, the patient developed sphincter dysfunction and motor loss in the left lower limb in the distribution of several nerve roots. Findings were normal from a myelogram and a magnetic resonance imaging study of the brain. A repeat cerebrospinal fluid analysis showed 1.1 g/L of protein and 5 cells/mm3. Because of the discrepancy between the clinical and imaging study findings, the patient was transferred to a neurology department. A third cerebrospinal fluid study showed numerous adenocarcinoma cells, and a repeat magnetic resonance imaging demonstrated a mass in the dural sac opposite L2. A program of monthly intrathecal methotrexate injections was started. A fatal meningeal relapse occurred eight months later. CONCLUSION: This case shows that a leptomeningeal metastasis can cause isolated nerve root pain, and demonstrates the diagnostic value of magnetic resonance imaging and cerebrospinal fluid cytology in patients with atypical symptoms, particularly when there is a history of malignant disease.
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ranking = 6.3938739281927
keywords = pain
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9/564. 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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10/564. meningioma with sarcomatous change and hepatic metastasis.

    A 72-year-old patient had a meningotheliomatous meningioma that invaded through the skull and into temporalis muscle. One year following craniotomy for removal of the neoplasm, he developed headaches, diplopia, and proptosis of the left eye. biopsy of the orbital contents revealed a malignant supporting tissue neoplasm having a resemblance to the previous meningioma. No curative therapy was possible and the patient died 33 months after diagnosis. autopsy examination showed extensive residual intracranial neoplasm and a 3-cm metastasis in the liver. The metastatic tumor appeared similar to the meningioma and did not appear malignant histologically. The case illustrates the distinct histologic variations in meningiomas and the difficulties in predicting their biologic activity. Aggressive local behavior may indicate possible malignant areas in the neoplasm. Therefore, examination of the neoplasm should be thorough. Such a correlation may suggest malignant biologic potential.
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ranking = 2.3946534904039
keywords = headache
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