Cases reported "Lip Neoplasms"

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1/56. Malignant mesothelioma in subjects with Marfan's syndrome and ehlers-danlos syndrome: only an apparent association?

    Malignant mesothelioma is a rare neoplasm which could be favored by an hereditary predisposing factor. So far, malignant mesothelioma have never been described in patients with hereditary diseases of the connective tissue. Here, we report some cases of mesothelioma affecting subjects who were not exposed to inhalation of asbestos. One of these subjects was affected by ehlers-danlos syndrome, whereas in two brothers, mesothelioma was associated with Marfan's syndrome. The observation of the same histologic subtype of mesothelioma in two brothers and the coexistence of two pathologic conditions of mesodermal origin indicate the presence of hereditary factors predisposing to the cancerogenic action of even small amounts of asbestos. Structural alterations of collagen and primary immunodeficiency may represent the host factor inducing development of the neoplasm. We conclude that the association between these rare disorders of the connective tissue and mesothelioma may not be coincidental, but could be the result of the exposition to small amounts of asbestos in predisposed individuals.
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2/56. Trichoblastic carcinoma: a report of two cases of a deeply infiltrative trichoblastic neoplasm.

    BACKGROUND: Trichoblastoma is a benign neoplasm with primitive hair follicle differentiation. The tumor is characterized by nests and cords of epithelial cells in an organized relationship with stroma. Some of the epithelial cells resemble follicular germ and other cells show follicular sheath differentiation. The neoplasm is symmetrical and well circumscribed. In addition, cytologic examination shows no atypia. Concept of its malignant counterpart is poorly understood. OBJECTIVE: We report two trichoblastic neoplasms that show histologic features of malignancy. methods: skin biopsies from the lip region of a 53-year-old female and a 74-year-old male who had presented with the neoplasms in that location for several years were reviewed. Excised specimens measured 2 x 1 x 1 cm and 1.6 x 0.7 x 0.8 cm respectively. RESULTS: The histologic sections revealed locally aggressive neoplasms showing prominent differentiation towards hair germ and follicular sheath with formation of papillary mesenchymal bodies, follicular bulb-like structures, small keratin cysts and rare malformed hair shaft. The neoplasm showed asymmetry of architecture and massive subcutaneous and muscular infiltration. CONCLUSION: With demonstration of these two cases and references of few other reported cases we raise the possibility of existence of trichoblastic carcinoma that is distinct from basal cell carcinoma.
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3/56. Basal cell carcinoma on the vermilion border of the lip: a study of six cases.

    BACKGROUND: The vermilion border of the lower lip is a frequent location of squamous cell carcinoma (SCC), but it is very rarely mentioned within the published series of basal cell carcinomas (BCCs). OBJECTIVE: We present 6 cases of BCC involving either mainly or exclusively the vermilion border of a lip. methods: We reviewed from our files all the cases of BCC diagnosed in a period of 11 years. RESULTS: A number of 3,477 BCCs were histologically diagnosed in that period; 2,872 (82.6%) of them were located on the head, and 66 (2.3%) of the latter (1.9% of all BCCs) were on the lips. In 6 cases, the neoplasm involved either mainly or exclusively the vermilion border of either the lower (5 cases) or the upper (1 case) lip. CONCLUSION: Not every carcinoma of the vermilion border of the lip is a SCC.
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4/56. Onset of oral extranodal large B-cell non-Hodgkin's lymphoma in a patient with polycythemia vera: a rare presentation.

    polycythemia vera (PV) is a hematologic malignancy characterized by excessive proliferation of erythroid, myeloid and megakaryocytic elements in the bone marrow. patients suffering from PV may subsequently be affected by other neoplasms of the haematopoietic system, but lymphomas are very rare and no cases of oral lymphoma have yet been reported. We report the case of a patient with PV in whom a primary non-Hodgkin's lymphoma (high grade malignancy on the Kiel scale) of the oral cavity subsequently developed. The case is unusual for its extranodal onset, its location in the oral cavity and the clinical presentation.
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5/56. Desmoplastic malignant melanoma of the lip: a report of 6 cases and review of the literature.

    Desmoplastic malignant melanoma is a rare neoplasm consisting primarily of spindle-shaped melanoma cells embedded in a fibrous stroma, with approximately 6% of cases occurring in the lip. A literature search revealed that most reported cases occur in sun-exposed areas in older men. Few cases of desmoplastic malignant melanoma of the lip in young people (in their mid-twenties) have been described. We report 6 previously undocumented cases of labial desmoplastic malignant melanoma occurring in young individuals and review 20 cases from the literature. physicians should consider the possibility of desmoplastic malignant melanoma in young people who present with atypical lip lesions, which on histopathologic analysis demonstrate spindle-cell hyperplasia. Although this diagnosis is rare and there have not been reports of such patients in the literature thus far, our findings should alert clinicians to the possibility of desmoplastic malignant melanoma as a diagnosis for lip lesions in young people.
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6/56. Microcystic adnexal carcinoma: an unusual cause of swelling and paraesthesia of the lower lip.

    Microcystic adnexal carcinoma (MAC) is an uncommon, recently described, cutaneous adnexal malignant neoplasm, associated with significant morbidity as a consequence of its propensity for perineural invasion. The present report details the clinical and histological features of MAC in a young female presenting with lower labial swelling and paraesthesia.
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7/56. carney complex: report of a Japanese case associated with cutaneous superficial angiomyxomas, labial lentigines, and a pituitary adenoma.

    We report the case of a 12-year-old female patient who manifested multiple cutaneous angiomyxomas and labial pigmented lesions. Although the familial history was not confirmed in the present case, autosomal dominant inheritance has been reported to be involved in the pathogenesis of this condition. In addition to the cutaneous complications, magnetic resonance (MR) images revealed the presence of a pituitary adenoma, which provoked an elevation of serum growth hormone (GH) level. On the other hand, no significant symptoms such as cardiac myxoma, myxoid fibroadenoma of the breast, or adrenocortical complaints suggesting cushing syndrome, were detected. In the Japanese literature, only a few cases of this disorder have been described in the form of brief reports. There have been only a few similar cases described in the dermatological field, except for one report diagnosed as carney complex in 1990. Therefore, the present case seems to be the first Japanese case of typical carney complex manifesting major clinical complications, including angiomyxomas, lentigines, and a pituitary adenoma, which induced endocrine overactivity.
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keywords = breast
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8/56. The therapy of virus-associated epithelial tumors of the face and the lips in organ transplant recipients.

    The risk of developing malignant cutaneous neoplasms is increased after organ transplantation. We report three patients with malignant tumors of the epithelium of the facial skin and the lips after kidney and heart transplantation, respectively. They showed an aggressive course of the disease with more than five synchronous or metachronous basal cell and squamous cell carcinomas. Tissue samples were Epstein-Barr virus (EBV) positive by PCR. Using an in situ hybridization technique EBV-encoded rna (EBER) was detected in tumor-infiltrating lymphocytes. The aggressive course was not alone controllable by surgical or radiological therapy. The systemic and topical application of cidofovir (Vistide) led to remarkable remissions, to a better confinement and operability of the tumors, and to a cessation of tumor pain. The photodynamic therapy represents another opportunity for managing superficial local recurrences and multiple tumors. In conclusion, the results of these case reports demonstrate that combined antiviral, photodynamic and surgical therapy may be used successfully to treat aggressive cutaneous malignancies in patients after organ transplantation.
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9/56. Polypoid S-100-negative granular cell tumor of the oral cavity: a case report and review of literature.

    This is a report of a case of an S-100-negative polypoid granular cell tumor arising in the lower lip of a 4-year-old patient that may represent the entity primitive polypoid granular cell tumor. Unlike the usual granular cell tumors arising in the tongue or buccal mucosa, such lesions are not S-100 positive and can exhibit mitoses and cytologic atypia, features that may suggest a low-grade mesenchymal malignancy necessitating aggressive therapy. However, this is in fact a nonaggressive neoplasm that is not associated with significant morbidity and therefore should be treated with simple excision.
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10/56. mucus-producing adenopapillary carcinoma of the oral cavity.

    mucus-producing adenopapillary carcinoma is a rare neoplasm of the oral cavity. The literature is reviewed and a case described, with histological and ultrastructural findings of a tumour which involved the upper lip and recurred following local excision.
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