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1/164. Antiangiogenic therapy of a recurrent giant cell tumor of the mandible with interferon alfa-2a.

    We report a 5-year-old girl with a large rapidly growing giant cell tumor of the mandible that recurred 2 months after the first surgical excision and 3 months after a second resection. An angiogenic protein, (bFGF), was abnormally elevated in her urine. The patient was treated with interferon alfa-2a for 1 year because this agent inhibits angiogenesis by suppressing bFGF overexpression in infantile hemangiomas and in other human tumors. During this time the bone tumor regressed and disappeared, the urinary bFGF fell to normal levels, and the mandible regenerated. She has remained tumor-free and has been off therapy for 3 years at this writing. This first successful use of interferon alfa-2a to treat a mandibular tumor in a child demonstrates: 1) low grade tumors that overexpress bFGF may respond to interferon alfa-2a, in a manner similar to life-threatening infantile hemangiomas; 2) antiangiogenic therapy, given without interruption for 1 year, was safe and effective in this patient; and 3) treatment may be continued for 1 year without the development of drug resistance.
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2/164. Clinical and morphological features of undifferentiated monomorphous GH/TSH-secreting pituitary adenoma.

    A 41-year-old male presented with progressive visual defects, acromegaly and hyperthyroidism. After clinical evaluation a giant GH/TSH-secreting pituitary adenoma was diagnosed. Administration of the somatostatin analog octreotide at doses of 150 microg s.c. per day inhibited the secretion of both GH and TSH. A three-week treatment with octreotide prior to surgery led to slight visual improvement and CT scan showed some new necrotic areas within the tumor mass. Transcranial surgery was performed. By immunohistochemical analyses of the adenoma tissue GH, prolactin and beta-chorionic gonadotropin were detected; TSH was negative. Electron microscopy revealed an undifferentiated, monomorphous adenoma with morphological features of an acidophil stem cell adenoma such as the presence of misplaced exocytoses, fibrous bodies and mitochondrial gigantism. However, the tumor cells contained small secretory granules (up to 250 nm) accumulated along the cell membrane characteristic of thyrotrope cells. Furthermore, some adenoma cells were fusiform with long cytoplasmic processes resembling thyrotropes. Two months after the operation CT scan revealed a large residual tumor. serum GH and TSH levels had increased again and the TSH level was even higher than before the treatment. The patient died suddenly, most probably of lethal arrhythmia. Specimens of the adenoma tissue obtained at autopsy confirmed the previous findings with the exception of positive immunostaining for TSH which was found in less than 1% of the adenoma cells. This undifferentiated, monomorphous GH/TSH-secreting pituitary adenoma represents an entity that is unusual both in its ultrastructural features and clinical manifestations suggesting a cytogenesis from an early, undifferentiated stem cell.
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3/164. Use of polymethylmethacrylate in large osseous defects in the foot and ankle following tumor excision.

    foot and ankle surgeons are occasionally confronted with having to fill large defects following excision of osseous lesions. This can prove to be quite challenging to the surgeon in regards to the requirement of large amounts of autogenous, allographic, or synthetic bone graft material. The amount of time spent nonweightbearing postoperatively can be quite prolonged, and the evaluation for tumor recurrence at the graft--host interface is difficult to ascertain. Polymethylmethacrylate has been used extensively in orthopedic surgery for many years in a safe manner for total joint replacement. It has also been used to fill large defects following tumor excision (i.e., giant cell tumor) and as an alternative to bone graft. This article briefly reviews the concepts of using polymethylmethacrylate in this manner and presents the use of polymethylmethacrylate in the treatment of foot and ankle lesions with three case presentations. The authors' purpose for this paper is to simply expand on the current medical literature available regarding the use of polymethylmethacrylate in the foot and ankle and to increase the awareness of foot and ankle surgeons regarding its use as a treatment alternative. A follow-up to this article is planned to present a larger patient population, longer term follow-up, and outcomes data.
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4/164. 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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5/164. Sarcomatoid carcinoma of the prostate: progression from adenocarcinoma is associated with p53 over-expression.

    BACKGROUND: The pathogenesis of sarcomatoid metaplasia of prostatic adenocarcinoma is uncertain. The histologic features of sarcomatoid carcinoma arising in two patients with previously irradiated prostatic adenocarcinoma are reported and the relationship between prostatic adenocarcinoma and subsequent sarcomatoid carcinoma is investigated by immunohistochemical detection of epithelial and soft tissue tumor markers, and p53 protein. methods AND RESULTS: Two patients, aged 72 and 67 years, underwent localized radiotherapy for prostatic adenocarcinoma and re-presented with sarcomatoid carcinoma 41 months and 60 months later, respectively. In both cases the tumor consisted of anaplastic spindle cells with occasional osteoclast-like giant cells. The initial tumors showed immunohistochemical staining typical of prostatic adenocarcinoma with absence of expression of p53 protein. The subsequent sarcomatoid carcinomas were positive for vimentin and negative for epithelial cell markers. In both cases serial biopsies showed a temporal increase in tumor expression of p53 protein. CONCLUSIONS: The development of sarcomatoid carcinoma in prostatic adenocarcinoma is associated with progressive accumulation of p53. This is suggestive of increasing clonal dominance of dedifferentiated tumor cells carrying p53 mutations.
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6/164. Florid juvenile (cellular) fibroadenomatosis in the adolescent: a case for subcutaneous mastectomy?

    Juvenile or giant fibroadenoma (JF) is an uncommon fibroadenoma variant usually presenting in adolescence. Although these masses are benign, when multiple and bilateral, they present a complex challenge to the attending surgeon, both in diagnosis, and in selection of the most appropriate therapy. Treatment is usually surgical and ranges from simple excision to subcutaneous mastectomy with reconstruction. We report an unusual case of refractory JF, initially treated with combined hormonal and surgical treatment but ultimately requiring bilateral subcutaneous mastectomies to prevent tumor regrowth. This case highlights the occasional difficulty in the management of macromastia in the adolescent female.
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7/164. Tenosynovial giant cell tumor of finger, localized type: a case report.

    The authors report a typical case of tenosynovial giant cell tumor of the right middle finger of a 31-year-old man. Histologically, this tumor is characterized by a discrete proliferation of rounded synovial-like cells accompanied by a variable number of multinucleated giant cells, inflammatory cells, and xanthoma cells. Clinicopathologically, this tumor is a benign lesion that nonetheless possesses a capacity for local recurrence. Local excision with a small cuff of normal tissue is the treatment of choice in this tumor.
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8/164. Warty (condylomatous) squamous cell carcinoma of the penis: a report of 11 cases and proposed classification of 'verruciform' penile tumors.

    Within the spectrum of penile squamous cell carcinomas, those that we descriptively refer to collectively as the "verruciform" lesions are particularly difficult to subclassify. In a review of 50 such tumors, we found 11 distinctive neoplasms with condylomatous features conforming to the appearance of so-called "warty (condylomatous) carcinoma." The average patient age was 55 years and the average duration of disease was 19 months. The primary tumor involved multiple anatomic sites (glans, coronal sulcus, and foreskin) in seven cases and a single site (glans or foreskin) in four cases. Grossly, white to gray cauliflower-like tumors typically measuring approximately 5 cm were noted. Histologically the tumors were mainly papillomatous with acanthosis and hyperkeratosis. The papillae had prominent fibrovascular cores. The most conspicuous microscopic findings were striking nuclear atypia of koilocytotic type and clear cytoplasm. The interface between tumor and stroma was irregular in the majority of cases; deep invasion of corpus cavernosum was noted in five cases. The differential diagnosis included verrucous carcinoma, low-grade papillary squamous cell carcinoma, not otherwise specified, and giant condyloma acuminatum. Among other differences, the first two lesions show no koilocytotic changes and the last lacks malignant features and irregular stromal invasion. Metastatic spread occurred in two patients; both are alive with evidence of recurrent disease 12 and 72 months after initial diagnosis. A third patient was alive with recurrent disease 12 months after diagnosis. Five patients were free of disease 8, 12, 24, 52, and 108 months after diagnosis. Three patients were lost to follow up. Warty (condylomatous) carcinomas of the penis are morphologically distinctive verruciform neoplasms with features of human papillomavirus-related lesions and should be distinguished from other verruciform tumors so that differences in behavior, if any, between these tumors will become established.
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9/164. Surgical approach to a giant fibrolipoma of the supraglottic larynx.

    This paper describes the surgical procedures for a fibrolipoma that first appeared as a giant tumour in the hypopharynx and extended to the cardiac antrum of the oesophagus. At the initial surgery, a pedunculated tumour originating from the left arytenoid of the larynx was found to occupy the cervical as well as thoracic oesophagus and was thus removed through a lateral pharyngectomy. A histological examination revealed fibrolipoma. However there was a recurrence of the tumour in the arytenoid and the patient suffered from dysponea. In addition, a submucosal tumour was also found in the left false vocal fold. At the second surgery, the masses in the arytenoid and false vocal fold were subtotally removed without damaging the mucosa. The mucosa of the arytenoid was sutured to the thyropharyngeal muscle on the same side and the arytenoid swelling disappeared almost completely. The post-operative course has been uneventful for more than two years.
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10/164. Giant schwannoma of the back.

    We treated a patient with giant schwannoma of the back. The tumor measured 35 x 25 x 12 cm and weighted 1840 g. Histological diagnosis was benign schwannoma. To our knowledge, there is no previous report of such a large schwannoma of the back.
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