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1/411. Acral dermatofibrosarcoma protuberans with metastases.

    dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor that occurs rarely on the extremities. This tumor has a particular propensity for local recurrence following seemingly adequate excision. Metastases are rare, and usually follow repeated local recurrences. On rare occasions, fibrosarcomatous change may arise in a DFSP, and appears to affect adversely the prognosis. The adequacy of the initial resection is the most important prognostic factor; however, suggested margins vary between 1.5 and 5 cm. The ideal margin of resection on the foot is unknown. We report a patient with recurrent, acral DFSP with fibrosarcomatous change and pulmonary parenchymal metastases in a 48-year-old black male.
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2/411. 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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3/411. A pathologic and clinical study of adenosquamous carcinoma of the larynx. Report of four cases and review of the literature.

    Four cases of adenosquamous carcinoma are described. Emphasis is put on the rarity of this histological type of tumour which was first described in the larynx by Gerughty et al. (1968) who reported three cases. An exhaustive histological documentation of the neoplasm accompanies the discussion of its pathomorphological characteristics, histogenesis, differential diagnosis, prognosis and treatment. Stress is laid on the extreme aggressiveness of the neoplasm and on its high biologic malignancy. Elective treatment is radical surgery. The tumour in question is a distinct anatomo-clinical entity with its own connotations and therefore must be distinguished from the most common squamous cell carcinoma of the larynx.
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4/411. Peripheral primitive neuroectodermal tumors of bone. A review of three cases.

    The recently individualized and still incompletely understood family of peripheral neuroectodermal tumors encompasses several tumor types, of which some have a predilection for bone. Immunocytochemical studies are essential and usually provide the diagnosis. A t(11;22)(q24;q12) translocation is present in over 80% of cases. Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. Contradictory data have been reported regarding the comparative prognosis of peripheral neuroectodermal tumor and Ewing's sarcoma, indicating a need for further studies in large numbers of patients. We illustrate these points by three case-reports, two in girls diagnosed with a vertebral primary at five and nine years of age, respectively, and one in a man diagnosed with a pelvic primary at 29 years of age.
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5/411. 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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6/411. Bellini duct (collecting duct) carcinoma of the kidney.

    carcinoma of the collecting ducts, or Bellini carcinoma, is a rare renal tumour and, unlike most renal cell carcinomas, it derives from distal tubules. It displays highly aggressive behaviour and has a poor prognosis. In this study, the authors present three cases which they observed over the past three years.
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7/411. Metachronous cancers or late recurrences after resection of stage I lung cancer.

    In 2 patients with stage I lung cancer, tumors recurred at their resection lines 10 years after the original surgical resections. These cases suggest that the prognosis of late cancer occurrences after resected primary lung malignancies might be related to the interval of time between primary and subsequent cancers rather than to their categorization as recurrent or metachronous cancers.
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8/411. Surgical treatment for recurrent tumors of primary malignant melanoma of the esophagus: a case report and review of the literature.

    The purpose of this communication is to present a case of resection performed for local recurrent tumors of primary malignant melanoma of the esophagus (PMME) and to review the relevant literature. The patient was a 54 year-old man who had received an intraabdominal esophagectomy with a total gastrectomy for primary malignant melanoma of the abdominal esophagus in another hospital, in November 1995. After the initial operation, he was treated as an outpatient. In August 1997, computed tomography and ultrasonography revealed recurrent tumors in the dorsal pancreatic lymph node and in the right adrenal gland. The recurrent tumor of the dorsal pancreas directly invaded the dorsal pancreas parenchyma and occluded the superior mesenteric vein and splenic vein, and the other metastatic tumor in the right adrenal gland existed in the absence of circumference invasion. Metastases of the PMME were confirmed in the dorsal pancreas, the superior mesenteric vein, splenic vein, and right adrenal gland, and were removed by a total pancreatectomy on October 7, 1997. By immunohistochemical staining, we found that the focal areas expressed S-100 protein and HMB-45 antibody. Currently (February 1998), the patient is alive and disease-free. PMME is an extremely rare tumor with a poor prognosis for survival. Only 2 cases of removal of recurrent tumors, including the present case, have been reported. The treatment of choice is surgical resection, even in cases of recurrence, because radiotherapy and/or chemotherapy have not been proven to be beneficial; however, they may play a palliative role if surgery is not possible.
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9/411. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood.

    Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma (ALCL) is an unusual tumor in the pediatric population. However, the nodal-based form of the disease compared with other histologic subsets of childhood non-Hodgkin's lymphomas (NHL) more frequently involves skin, soft tissue, and bone. The objective of this article is to determine the histologic and immunologic characteristics of childhood primary cutaneous Ki-1(CD30) positive ALCL and its prognosis. The clinical data, histologic features and immunohistochemical profiles of skin biopsy specimens from 3 children with cutaneous Ki-1(CD30) positive lymphoma were reviewed. A literature search was performed and disclosed information on 5 childhood cases. The 3 patients with primary cutaneous Ki-1(CD30) positive ALCL all presented similarly as rapidly growing masses initially and clinically believed to be infectious/reactive processes. The diagnosis was established on the basis of histopathologic examination and immunohistochemical studies. Histologic sections revealed an extensive infiltrate of tumor cells extending throughout the entire dermis into the subcutaneous fat with frank ulceration in 1 patient. No significant epidermotropism was noted. Tumor cells exhibited striking cellular pleomorphism and a high mitotic rate with numerous atypical mitoses. Inflammatory cells were present in all patients. The tumor cells stained positively for Ki-1 antigen (CD30), epithelial membrane antigen, and for T-cell markers (UCHL-1, CD3). One of 3 cases, however, failed to stain for leukocyte common antigen (LCA). No clinically apparent adenopathy was observed in any of the patients. In all instances the patients developed recurrent disease in the skin at sites separate from the primary location. None of the patients demonstrated any involvement of lymph nodes, bone marrow, or other organ systems. All patients were treated with chemotherapy with good response. Primary cutaneous Ki-1(CD30) positive lymphoma is rare in children and is characterized by recurrences. The prognosis seems to be favorable.
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10/411. liposarcoma of the hypopharynx. A case report and review of the literature.

    A new case of well differentiated hypopharyngeal liposarcoma is reported. The author reviews the literature about the clinical and histologic features of these tumors. From the 93 head and neck liposarcomas reported 13 are located in the hypopharynx. The mean age of presentation is 61 years and males are largely predominant. Etiology is still unknown. Tumor size does not seem to affect the prognosis. Usually patients do not present with cervical lymph node metastasis nor distant metastasis. Histologic diagnosis according to Enzinger and Weis's classification could be difficult especially to distinguish between lipoma and well differentiated liposarcoma. The main prognostic factor is histologic grade but early recognition combined with a complete surgical excision can result in a decreased local recurrence rate and high survival rate. Low grade tumors often recur locally but distant metastases are rare. From the 8 well differentiated tumors reviewed 6 presented a local recurrence 2 months to 20 years after surgery but only 1 patient died without disease. High grade tumors are much more aggressive locally and metastasize frequently. radiotherapy and chemotherapy are proposed in selected cases without evidence to be of value.
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