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1/147. Malignant melanoma of the choroid in neurofibromatosis.

    A 60-year-old white woman with generalized neurofibromatosis and multiple melanocytic hamartomas of the iris developed an unusual choroidal mass, with secondary sensory retinal separation in the left eye. Ophthalmoscopically the tumor had a peculiar donut configuration that was caused by a large focus of central necrosis within a spindle B melanoma.
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ranking = 1
keywords = melanocytic
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2/147. A 42-year-old woman with liver masses and long-term use of oral contraceptives.

    A 42-year-old woman with a history of 25-year oral contraceptive use presented with abdominal pain and was found to have two exophytic liver masses. She had no known prior liver diseases, and her serum liver enzyme and AFP levels were normal. One of the masses was a hepatocellular adenoma and the other was a pigmented hepatocellular carcinoma. The exophytic appearance of both lesions was unusual. This case, once more, demonstrated the risk of hepatocellular adenomas to undergo malignant transformation. The reason for the brown pigment deposition in the hepatocellular carcinoma was not clear. The prognosis was expected to be excellent following complete surgical resection.
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ranking = 0.11661483090264
keywords = pigmented
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3/147. Simultaneous occurrence of multiple melanoma in situ on sun-damaged skin (lentigo maligna), solar lentigo and labial melanosis: the value of dermoscopy in diagnosis.

    We report on a patient developing simultaneous occurrence of lentigo maligna lesions, solar lentigines and an extensive melanosis of the oral mucosa. Diagnostically, epiluminescence microscopy had a relevant role in the preoperative assessment and selection of suspicious pigmented lesions, as the lesions histologically labelled as lentigo maligna and solar lentigo were clinically indistinguishable. We review the clinical, dermoscopic and histopathologic differential diagnosis of solar lentigo, malignant lentigo and mucosal melanosis with other melanocytic and keratinocytic lesions and discuss the possible relationship between these entities.
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ranking = 1.1166148309026
keywords = melanocytic, pigmented
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4/147. An unusual melanocytic lesion associated with eccrine duct fibroadenomatosis and syringoid features.

    The intimate association of nevomelanocytic nevi with eccrine ducts commonly seen in congenital nevi was emphasized by Mishima, who described as eccrine-centered nevi those lesions characterized by nevomelanocytic cells predominantly proliferating around and within the eccrine sweat duct walls. However, there were no changes in the overlying epidermis, dermis, or eccrine acrosyringeal or dermal duct proliferation in these lesions. We present the case of a 16-year-old boy with a 1-year-history of a 0.6-cm diameter single tan papule on the right heel, clinically thought to be a Spitz nevus. Histopathologic examination revealed a compound nevomelanocytic nevus associated with epidermal hyperplasia, thin anastomosing cords of acrosyringeal epithelium extending within the dermis, and eccrine ductal proliferation in a syringoma-like pattern associated with a dense fibrous stroma. Features that distinguish our case from eccrine-centered nevus are that the latter lacks epidermal and eccrine duct hyperplasia and a dense fibrous stroma. The location of the lesion on the heel in our case suggests the possibility that the pathologic changes observed could result from repetitive trauma.
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ranking = 7
keywords = melanocytic
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5/147. carney complex--an unexpected finding during puerperium.

    carney complex is an extremely rare, autosomal dominant, multi-system disorder characterized by multiple neoplasias and lentiginosis. The genetic defect responsible for this complex has been localized to the short arm of chromosome 2 (2p16). The most prevalent clinical manifestations in patients with carney complex are spotty skin pigmentation, skin and cardiac myxomas, Cushing's syndrome and acromegaly. Here we report the case of a 31-year-old woman with a spontaneous pregnancy. At 32 weeks of gestation, she was admitted to our Department of obstetrics with hypertension and severe back pain. In addition, she had unusual pigmentation and typical cushingoid features. One day after admission, the pregnancy was terminated by emergency cesarian section because of preeclampsia and pathological CTG. During the postoperative period the severe back pain persisted, and radiographic evaluation revealed a collapse of L(2)/L(3) with severe osteopenia. A CT scan showed a mass in the right suprarenal area. Histopathological examination revealed a primary pigmented nodular adrenocortical disease. After biochemical confirmation of the diagnosis of Cushing's syndrome, it was recognized that the patient met the diagnostic criteria for carney complex.
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ranking = 0.11661483090264
keywords = pigmented
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6/147. Basal cell carcinoma in association with multiple trichoepitheliomas.

    We report the case of a basal cell carcinoma (BCC) associated with multiple trichoepitheliomas (TEs) and include the results of histopathological and immunohistochemical studies. The patient had a large, pigmented nodule associated with 4 flesh-colored papules in the central facial area. Two of the 4 flesh-colored papules and the large pigmented nodule were excised. Histopathologically, the pigmented nodule was diagnosed as BCC and 2 of the papular lesions were diagnosed as TEs. In both the BCC and TEs, almost all of the basaloid aggregations were diffusely positive for cytokeratin (CK) 19, CK8 and CK8/18. Based on these clinicopathological observations and reports in the recent literature, these two neoplasms are considered to be highly related. BCC appears to differentiate toward follicular germinative cells, and TE is its benign counterpart.
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ranking = 0.34984449270793
keywords = pigmented
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7/147. Congenital giant melanocytic nevus with pigmented epithelioid cells: a variant of epithelioid blue nevus.

    Epithelioid-cell blue nevus is an unusual cytologic variant of blue nevus that has been recently described mostly in patients with carney complex, although the lesion may also appear in patients with no evidence of carney complex. This variant of blue nevus is composed of melanin laden large polygonal epithelioid melanocytes situated within the dermis. The neoplastic cells show no maturation with progressive descent and, in contrast with the usual stromal changes in blue nevi, epithelioid-cell blue nevus exhibits no dermal fibrosis. This report describes a congenital giant melanocytic nevus with pigmented epithelioid cells located on the back of a 2-year-old male. The lesion was present at birth and the patient had no evidence of carney complex. Histopathologically, the lesion consisted of a large and entirely intradermal melanocytic nevus composed of heavily pigmented epithelioid melanocytes involving the full-thickness of dermis, but extending also to the subcutaneous fat and underlying soft tissues. Immunohistochemically, epithelioid neoplastic melanocytes expressed immunoreactivity for S-100 protein, HMB-45, Melan-A, NK1C3, and microphthalmia transcription factor (MiTF) antibodies. MIB-1 cellular proliferation marker was expressed in the nuclei of only a few scattered epithelioid melanocytes. This report demonstrates that epithelioid-cell blue nevus is a distinctive histopathologic variant of blue nevus that may also appear as a giant congenital melanocytic nevus.
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ranking = 7.6996889854159
keywords = melanocytic, pigmented
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8/147. association of Fallot Tetralogy with Carney's complex.

    The carney complex is an inherited, autosomal disease of multicentric tumors in many organs. Some components of Carney's complex are cardiac myxoma, spotty pigmentation, and endocrine overactivity. Primary pigmented nodular adrenocortical dysplasia (PPNAD) is an exceedingly rare cause of Cushing's syndrome in infants, children, and young adults. PPNAD occurs sporadically or as part of a familial syndrome called Carney's complex. Up to our knowledge, the association of Fallot Tetralogy with Carney's complex has not been previously reported. We presented, a 20-year-old woman, who had been operated for Fallot Tetralogy at the age of 3 years, had Carney's complex, i.e. left atrial myxoma, two facial spotty pigmented areas, and PPNAD.
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ranking = 0.23322966180529
keywords = pigmented
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9/147. 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 = 0.23322966180529
keywords = pigmented
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10/147. Intracranial meningeal melanocytoma associated with ipsilateral nevus of ota. Case report.

    In this report, the authors review the case of a man with a neurocutaneous syndrome. He presented with an intracerebral melanocytoma associated with a blue nevus of the scalp; its location and its appearance during childhood supported the diagnosis of a nevus of ota. Meningeal melanocytomas are increasingly being diagnosed, but remain rare. Primary meningeal malignant melanoma is the first differential diagnosis to eliminate. Despite their common embryonic origin. the association of a melanocytoma with a nevus of ota is rare. A nevus of ota exhibits the same melanocytic proliferation and affects the trigeminal nerve territory. An ocular effect is not always observed. In contrast to an ocular lesion, a nevus of ota rarely transforms into a malignant melanoma. It is found only among caucasians. During 4 years of follow-up review after surgery, the patient remained asymptomatic. Other than antiepileptic therapy, he received no complementary treatment and cerebral imaging revealed no evidence of recurrence.
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ranking = 1
keywords = melanocytic
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