Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A review was conducted of 335 malignant melanomas to identify variant morphologic patterns that might be confused with other tumors. In all, 27 predominantly amelanotic neoplasms with unusual histologic features were selected for additional study. These included nine with an adenoid or pseudopapillary pattern, seven small cell neoplasms, five with prominent myxoid stroma, four with a hemangiopericytoma-like appearance, and two composed of neoplastic cells with a signet-ring configuration. A diagnosis of melanoma was confirmed in all cases by Fontana-Masson strains for melanin pigment, electron microscopic examination, or the results of immunohistochemical analyses for cytokeratin, vimentin, S-100 protein, and the HMB-45 antigen. One tumor was associated with a congenital hairy melanocytic nevus, five were vulvovaginal lesions, four arose in the sinonasal tract, and one occurred in the rectum. Four of the specified microscopic patterns were observed in both primary and secondary neoplasms; the two signet-ring cell melanomas were recurrent lesions. The authors conclude that malignant melanomas may assume the histologic guise of adenocarcinomas, small cell carcinomas, and sarcomas, in a variety of tissue sites. Special studies designed to detect melanocytic differentiation are therefore appropriate in diverse differential diagnostic settings.
 ...
PMID:Morphologic diversity in malignant melanomas. 224 3

Congenital smooth muscle hamartoma (CSMH) represents a proliferation of randomly oriented dermal smooth-muscle bundles. Six patients with CSMH were observed, the largest series to date, and the literature was reviewed. Congenital smooth muscle hamartoma has presented as congenital patches or slightly indurated plaques with prominent overlying hair (88% of cases), or rarely as patches with perifollicular papules without prominent hair (12% of cases). Most lesions (61% of cases) have been somewhat hyperpigmented, but 39% of cases have been flesh colored. Congenital smooth muscle hamartoma has occurred on the torso and proximal extremities, except for one case on the eyebrow and eyelid (present study). A positive pseudo-Darier's sign (temporary induration or piloerection after rubbing) helped to differentiate CSMH from congenital hairy nevo-cellular nevus. Congenital smooth muscle hamartoma is a distinct entity that is at one end of a spectrum that includes Becker's nevus, and should be considered in the differential diagnosis of any congenital hairy lesion.
 ...
PMID:Congenital smooth muscle hamartoma. A report of six cases and a review of the literature. 265 46

Congenital giant hairy nevi represent a special group of melanocytic lesions which generally cover large areas of the body surface. Giant hairy nevi assume special significance because of their predisposition to malignant transformation. Adequate treatment of this lesion involves complete surgical excision as early in the child's life as possible.
 ...
PMID:Congenital giant hairy nevus: implications for treatment. 270 86

Iris melanosis is an unusual condition characterised by the presence of minute, discrete, pigmented elevations arising from the anterior surface of the iris. We encountered two unrelated Mexican families in which all children, but no parent, had varying degrees of the condition bilaterally. Some family members also gave findings suggestive of ocular hypertension. No family member had any other ocular or cutaneous pigmentary changes with the exception of a hairy naevus on the thigh of one member. To our knowledge these are the first families reported with more than one member having isolated iris melanosis. This is also the first report of a possible relationship with ocular hypertension. Finally we suggest that the term 'melanosis' may be a misnomer, since the condition is characterised not by abnormal iris hyperpigmentation but by discrete, round elevations on the anterior iris surface.
 ...
PMID:Familial iris melanosis--a misnomer? 271 8

Congenital giant nevi of neuroectodermal origin are composed predominantly of melanocytes and occasional neural elements. Neoplasms arising in such nevi may be morphologically heterogeneous with variable clinical behavior. We report the case of a female infant born with multiple pigmented nevi, including a giant hairy nevus of bathing trunk distribution in which arose a large pedunculated mass involving the sacral region, buttocks, perineum, and left thigh. Within this neoplasm, mixtures of neural crest-derived tissues having both melanocytic (nevus) cell and neural supportive (neuroid of Schwannian) cell differentiation were identified. The neural differentiation, which was seen in the dermis and subcutaneous tissue, had a neurofibromatous appearance and demonstrated multiple pseudomeissnerian corpuscles. In addition, at 16 months of age the child developed a malignant melanoma in the flank region of this congenital giant nevus. The morphologic pluripotentiality of the primitive neural crest derivatives in congenital nevi has only recently been elucidated. These lesions should be regarded as neuroectodermal neoplasms and subclassified according to their differentiating histologic characteristics because prognosis and management are influenced by morphologic determinants.
 ...
PMID:Neuroectodermal neoplasms arising in congenital nevi. 285 58

Theoretically, Becker's melanosis or hairy epidermal nevus and smooth muscle hamartoma are two quite separate entities. In fact, there are cases which could be considered as intermediate. As a matter of fact, a slight underlying smooth muscle hyperplasia can be seen in Becker's nevus; on the other hand, hypermelanosis of the basal layer and hypertrichosis may be encountered in smooth muscle hamartoma. The 4 here reported cases are examples of diagnostic difficulties for which the sometimes not clear-cut limits of these 2 types of lesion can be responsible.
 ...
PMID:[Smooth muscle hamartoma or nevus of Becker? Apropos of 4 cases]. 305 Mar 31

The abdominal wall of the infant presents a significant skin excess. Applying techniques of aesthetic abdominoplasty, it is possible to excise extensive abdominal wall lesions, as often seen in children with congenital hairy nevi. Three cases of congenital nevi were handled in this manner and are presented. One case involved the upper abdomen, while the other two involved the lower abdomen.
 ...
PMID:Abdominoplasty in infants for removal of giant congenital nevi: a report of three cases. 315 65

DNA flow cytometry was performed on formalin fixed, paraffin embedded melanocytic naevi. DNA aneuploidy was detected in all three types of naevus but was significantly more frequent in those naevi accepted as precursors of malignancy: that is, dysplastic and congenital pigmented hairy naevi. It may be that the presence of DNA aneuploidy has prognostic significance in these naevi. Technical problems were encountered in the analysis of data from melanocytic lesions so that caution is recommended in interpretation of studies using formalin fixed tissue.
 ...
PMID:The flow cytometry of melanocytic skin lesions. 321 70

The authors reported the clinical course and the postmortem examination of a unique case of neurocutaneous melanosis with numerous anomalies and complications, which included congenital dislocation of lenses, hypogonadism, ectopia of prostatic duct, genuine phimose, retentio testis, psina bifida and neurogenic bladder. This 13-year-old boy with a large hairy nevus in a bathing trunk configulation and multiple small nevi over the whole body since his birth was admitted to our hospital for evaluation of headache and vomiting. Neurological examination showed bilateral papilledema and slight left hemiparesis. A CT scan revealed a large right frontal mass and craniotomy was performed with subtotal removal of this tumor which was confirmed as a malignant leptomeningeal melanoma. He initially made uneventful postoperative recovery, and two courses of chemotherapy with DTIC, ACNU and VCR were given; however, the currence of brain tumor ensued shortly thereafter, and he died in approximately six months after the onset of intracranial symptoms despite of the third course of chemotherapy. Thirty five cases of neurocutaneous melanosis associated with or without malignant melanoma have been reported in Japan. Twenty-eight cases were male and 7 female. Two cases showed the evidence of primary malignant melanoma outside of the central nervous system, whereas twenty eight leptomeningeal melanoma, in which 22 were solid and 6 diffuse, were shown intracranially. Other 5 cases had epileptic seizure and/or hydrocephalus caused by wide spreaded leptmeningeal melanosis. This high incidence of intracranial malignant melanoma in this disorder was remarkable compaired with the previous reports in other countries. Mean duration between deaths and the onset of symptoms of intracranial hypertension or focal neurological signs was 7 months, ranging from 1 to 24 months, showing the rapidly deteriorating course in this disorder.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[An autopsy case of neurocutaneous melanosis associated with intracerebral malignant melanoma]. 332 33

Macrodactyly is an uncommon congenital anomaly that affects the fingers and toes. Speculation as to cause is focused on the association with connective tissue abnormalities, such as neurofibromatosis. We report a highly unusual case of a patient with epidermal nevus syndrome, a specific connective tissue and skeletal disease, who also exhibited bilateral, four finger macrodactyly. Potential causes of linkage between these two specific and unusual syndromes are discussed. Treatment of the epidermal nevus may be nonoperative, differing from the philosophy of aggressive treatment of the similarly appearing premalignant congenital hairy nevus.
 ...
PMID:Macrodactyly and the epidermal nevus syndrome. 365 32


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>