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1/21. Desmoplastic malignant melanoma on the buttock of an 18-year-old girl: differentiation from desmoplastic nevus.

    Melanocytic proliferation in young people may sometimes pose a diagnostic dilemma. This is particularly so when a desmoplastic component is present. Because the two main differential diagnoses, desmoplastic malignant melanoma and desmoplastic Spitz nevus, share some morphologic features, the diagnosis of desmoplastic malignant melanoma may be overlooked. Distinction between the two is important because they show completely different biological behavior. The age of patient, site of lesion, histologic findings of melanocytic atypia, neurotropism, mitosis, and maturation help to distinguish the two entities. We report a case of desmoplastic malignant melanoma occurring in the buttock of an 18-year-old Chinese girl. Histologically, it had typical features of desmoplastic malignant melanoma with junctional melanocytic atypia and prominent neurotropism. Clinical and histologic differences between desmoplastic malignant melanoma and desmoplastic Spitz nevus are reviewed. We conclude that although desmoplastic Spitz nevus occurs much more commonly in adolescents, desmoplastic malignant melanoma can occur in this age group and even in non-sunexposed skin. Microscopic findings remain the mainstay that guides the final diagnosis.
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ranking = 1
keywords = malignant melanoma
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2/21. Polypoid Spitz naevus: the benign counterpart of polypoid malignant melanoma.

    Polypoid malignant melanoma is a peculiar morphological variant of melanoma with a distinct exophytic pattern of growth. This form of melanoma is usually very thick and the prognosis is accordingly poor. We present here a previously undescribed form of Spitz naevus which had a similar polypoid exophytic silhouette and marked cytological atypia. Despite these close morphological similarities, polypoid Spitz naevus evolves in a completely benign manner. Morphologically, polypoid Spitz naevus can be distinguished from polypoid melanoma by the absence of mitoses and by the prominent stromal reaction throughout the lesion.
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ranking = 0.5
keywords = malignant melanoma
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3/21. Prepubertal malignant melanoma: report of three cases.

    Prepubertal malignant melanoma (MM) is an extremely rare tumor. In slovenia, 13 MM cases were registered between 1968 and 1996 by the Cancer Registry of slovenia. The diagnosis of MM was confirmed by histology in 3 children. In 3 other children the lesions initially diagnosed as MM were reclassified as Spitz nevus. In the remaining cases, the slides were not accessible for histological review, and the clinical course of disease corroborated the diagnosis of a benign nevus. In the present report, 3 of 13 cases with histologically confirmed prepubertal MM are described. The difficulties encountered in the diagnosis and management of this rare condition are discussed.
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ranking = 0.5
keywords = malignant melanoma
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4/21. Spitzoid malignant melanoma in teenagers: an entity with no better prognosis than that of other forms of melanoma.

    AIMS: A rare form of melanoma in teenagers closely simulates Spitz naevus and is claimed to have a good prognosis. The aim of this study is to identify the clues for a confident diagnosis of this entity and to confirm the peculiarly good prognosis. methods AND RESULTS: Two cases of melanoma with Spitzoid features were compared with Spitz naevus and it was found that the major distinctive criteria are: mitoses and single cell necrosis in the deepest part of the lesion, cellular and particularly nuclear and nucleolar pleomorphism, and growth pattern in solid sheets of cells. More subtle clues were the asymmetric distribution of pigment and the thinning of the epidermis with parakeratosis and exudate in the cornified layer. Both of the lesions reached the mid-dermis. There was a fatal outcome in both patients after generalized metastatic spread. The metastatic disease in one of the cases appeared 15 years after the excision of the primary lesion. CONCLUSIONS: Spitzoid melanoma in teenagers can be distinguished from Spitz naevus if strict criteria are followed. Spitzoid melanoma does not show a better prognosis than other types of melanoma if the follow-up is prolonged enough.
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ranking = 0.4
keywords = malignant melanoma
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5/21. Spitzoid malignant melanoma with lymph-node metastasis. Is a copy-number loss on chromosome 6q a marker of malignancy?

    Distinction of spitzoid malignant melanomas (SMM) from Spitz nevi may be difficult or even impossible on the basis of conventional histology. In this report, a patient suffering from a primary lesion diagnosed as a Spitz nevus and a metastatic malignant melanoma approximately 4 years thereafter is described. A diagnosis of SMM was made subsequently upon review of the primary lesion. In the present analysis, we used comparative genomic hybridization (CGH) to define markers characteristic of SMM. The primary lesion revealed deletions on chromosomes 6q and 9p. In the metastasis, additional deletions on chromosomes 10p and 10q and gains of chromosome 7 were found. To our knowledge, no chromosomal aberration on chromosome 6 was hitherto demonstrated in benign melanocytic nevi. Findings reported in the literature suggest that human melanoma metastasis suppressor gene maps to 6q. In contrast, losses on chromosome 9p seem to be an early event in the development of melanoma. However, they are not only found in melanomas but are occasionally present in Spitz nevi as well as in atypical nevi. The CGH result with deletion of 6q in this difficult to diagnose primary melanocytic lesion strongly supports the diagnosis of malignant melanoma. To demonstrate the reliability of loss on chromosome 6q as a marker of SMM, a larger number of lesions must be investigated.
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ranking = 0.7
keywords = malignant melanoma
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6/21. Multiple agminated Spitz nevi arising on a cafe au lait macule: review of the literature with contribution of another case.

    The majority of Spitz nevi are acquired solitary lesions. Multiple Spitz nevi are rare and may develop on hyperpigmented skin. We report a 16-year-old girl with multiple Spitz nevi arranged on a cafe au lait macule. immunohistochemistry showed positivity for S-100 and HMB-45. Of interest, expression of Polo-like kinase (PLK), a novel proliferation marker that recently proved to be positive in up to 98% of malignant melanoma cells, showed positivity in 40% of the nevus cells. The clinical development of multiple Spitz nevi is not yet clear, as they are preferentially excised. Reviewing 70 cases in the literature we found that multiple agminated Spitz nevi occur more frequently than reported previously. In about one-third of these cases Spitz nevi arose on congenitally hyperpigmented skin.
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ranking = 0.1
keywords = malignant melanoma
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7/21. A variant of junctional naevus of epithelioid and spindle cell type rich in melanophages.

    We describe a form of junctional melanocytic neoplasm with a massive production of melanin accumulated in the dermis. The pigment is stored in macrophages, which are by far the most numerous cellular component of the lesion. Another peculiar aspect is the occasional presence of a few melanocytes scattered in a pagetoid pattern above the dermo-epidermal junction in the spinous layer. The histological picture of this lesion is similar to a form of "tumoral melanosis" induced by a regressed malignant melanoma. The lesion had a worrisome clinical picture, its dark colour constituting a clinico-pathological diagnostic problem. The main clinical clues to the benign nature of this entity are the small lateral diameter, the uniform distribution of the pigment and the stability of the lesion over time; moreover, the patients are alive and well after a considerable length of time. Although a regressed dysplastic or malignant lesion cannot be totally excluded from a scientific point of view, we conclude that there is no sound morphological or clinical evidence that the lesion is other than biologically benign. The lesion is most likely another peculiar variant of epithelioid and spindle cell naevus.
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ranking = 0.1
keywords = malignant melanoma
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8/21. Two cases of unusual acral melanocytic tumors: illustration of molecular cytogenetics as a diagnostic tool.

    The differential diagnosis between benign Spitz nevus and malignant melanoma may present considerable difficulties in some cases. Here we report 2 unusual melanocytic tumors with spitzoid features developing in acral sites of Japanese patients to illustrate the use of comparative genomic hybridization (CGH) to classify these lesions. Case 1 was a 12-mm-thick, >2 cm-diameter nodule on the sole of a 37-year-old man. Case 2 was a subungual tumor of the left index finger in a 13-year-old boy. CGH showed absence of chromosomal aberrations in case 1 and multiple aberrations in case 2, including focused amplification as previously described in acral melanomas. Case 1 was free of disease after 2.5 years of follow-up, whereas case 2 developed lymph node metastasis. We conclude that molecular techniques such as CGH can be of diagnostic help in the classification of histologically ambiguous lesions.
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ranking = 0.1
keywords = malignant melanoma
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9/21. Malignant melanoma in an 8-year-old Caribbean girl: diagnostic criteria and utility of sentinel lymph node biopsy.

    The incidence of malignant melanoma (MM) is continuing to rise, although childhood MM remains rare. We describe an 8-year-old Afro-Caribbean girl who developed a non pigmented lesion on the tip of her left thumb, which persisted despite treatment in primary care with cryotherapy. At biopsy she was found to have an acquired acral MM. She underwent amputation of the distal phalanx of her thumb, together with positive sentinel lymph node (SLN) biopsy and subsequent axillary lymph node clearance and adjuvant chemotherapy. MMs are very rare in this age and skin-type group, therefore requiring strict diagnostic criteria. These criteria include the distinction from MM mimics, especially Spitz tumours, and an appropriate use of staging techniques such as SLN biopsy to influence management.
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ranking = 0.1
keywords = malignant melanoma
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10/21. Hyalinizing Spitz nevus: spindle and epithelioid cell nevus with paucicellular collagenous stroma.

    Hyalinizing spindle and epithelioid cell nevus (Spitz nevus) is an uncommon melanocytic lesion. The histologic features of this benign tumor can mimic those of certain benign (dermatofibroma and desmoplastic cellular blue nevus) and malignant (metastatic carcinoma and malignant melanoma) neoplasms. We report a woman with a hyalinizing Spitz nevus and review the clinical characteristics, histologic features, and differential diagnosis of this lesion. The lesion presented as an asymptomatic red firm nodule on her abdomen. Microscopic examination showed a symmetric lesion in the dermis. The neoplastic cells were large and epithelioid-shaped or plump and spindle-shaped. They were present as isolated individual cells, single cells in a linear pattern, small nests, and fascicles in a paucicellular hyalinized stroma. Neoplastic cells with prominent eosinophilic nucleoli were evenly exhibited from the superficial to deep lesion. Diffuse expression of S-100 protein and absence of staining with antibodies to cytokeratin and HMB-45 were observed. The features were diagnostic of a hyalinizing Spitz nevus. Hyalinizing Spitz nevus is a benign lesion whose histologic features may mimic dermatofibroma, desmoplastic cellular blue nevus, metastatic carcinoma, and malignant melanoma. Immunohistochemical studies are helpful to differentiate these lesions.
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ranking = 0.2
keywords = malignant melanoma
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