1/9. Primary malignant melanoma of the gallbladder in dysplastic naevus syndrome.A case of gallbladder involvement by malignant melanoma in a 57-year-old woman is reported. The gallbladder, resected for cholelithiasis, harboured a pedunculated polypoid dark mass, which histologically revealed sheets and nests of epithelioid cells with hyperchromatic nuclei in the lamina propria and at the junctional level. These cells were pigmented (with positive reaction with Schmorl's stain and bleaching with peroxide) and showed immunohistochemical positivity for S-100, gp 100 antigen (HMB-45 antibody) and vimentin. The patient, affected by dysplastic naevus syndrome, had a melanoma in situ excised from the scalp 8 years earlier. The features of the investigated lesion address towards a diagnosis of primary gallbladder melanoma. Furthermore, this is the first time that the existence of such a controversial entity is sustained by the ultrastructural investigation of melanosomes, demonstrating the presence of two melanocitary populations, a typical one exclusively junctional and an atypical one both at the junctional level and in the lamina propria.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
2/9. The triad of bilateral retinoblastoma, dysplastic naevus syndrome and multiple cutaneous malignant melanomas: a case report and review of the literature.We report a case of a patient with the triad of retinoblastoma, dysplastic naevus syndrome (DNS) and multiple cutaneous melanomas. The combination of retinoblastoma and DNS is a significant risk factor for the development of cutaneous melanoma. This risk extends to family members. We recommend that survivors of (inherited) retinoblastoma and their relatives are closely screened for the presence of dysplastic naevi.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
3/9. Utility of the wood's light: five cases from a pigmented lesion clinic.We demonstrate the utility of the wood's light in a practice that specializes in the evaluation of pigmented lesions. The wood's light assisted the physician in locating the site of a completely regressed primary cutaneous melanoma, determining the clinical borders of a lentigo maligna melanoma, differentiating between agminated naevi and a naevus spilus and detecting the recurrence of pigmentation after the excision of a dysplastic naevus, and also proved useful in monitoring a large segmental speckled atypical lentiginous naevus for change. Despite the availability of many 'high-tech' imaging and diagnostic devices designed to evaluate skin lesions, the relatively simple wood's lamp continues to be of great value. We encourage physicians not to abandon the use of the wood's light in their clinical practice.- - - - - - - - - - ranking = 0.6keywords = naevus (Clic here for more details about this article) |
4/9. Multiple primary melanomas in a patient with familial-type DNS during clomiphene-induced pregnancy.A 35-year old woman developed six primary cutaneous melanomas during her third pregnancy. She had received clomiphene treatment for nearly 2 years previously. She developed two more primary melanomas 15 and 21 months after giving birth. All melanomas were histologically associated with preexisting dysplastic naevi. The patient showed the characteristic phenotype of the dysplastic naevus syndrome; a cousin and an aunt were treated for malignant melanoma and the patient's brother had histologically confirmed dysplastic naevi. The course of her first two pregnancies was not complicated by the development of melanomas. We suggest that clomiphene may have played a role in the activation or progression of these 'precursor lesions' into malignant melanoma.- - - - - - - - - - ranking = 0.2keywords = naevus (Clic here for more details about this article) |
5/9. Dysplastic naevi in association with partial deletion of chromosome 11.A patient is reported who developed dysplastic naevi at an early age. He also suffered from a syndrome including Wilms' tumour, aniridia, mental retardation and diabetes mellitus in association with an interstitial deletion of the short arm of chromosome 11. It is suggested that genetic factors may be important in sporadic as well as familial cases of the dysplastic naevus syndrome.- - - - - - - - - - ranking = 0.2keywords = naevus (Clic here for more details about this article) |
6/9. Malignant melanoma of skin and eye in the same family: more than a coincidence?Hereditary cutaneous malignant melanomas are usually associated with the Dysplastic naevus syndrome. Whether these patients have an increased risk to choroidal malignant melanomas is doubtful. The existence of an hereditary form of malignant uveal melanoma is still uncertain.- - - - - - - - - - ranking = 0.2keywords = naevus (Clic here for more details about this article) |
7/9. Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome.The linear naevus sebaceous syndrome of Feuerstein-Mims belongs to the phacomatoses and classically is composed of a linear cutaneous lesion associated with mental retardation and epilepsy, sometimes also with ocular dystrophies. During an unusually long follow-up of 32 years of such a case, which is reported here, the late occurrence of additional ocular and intracranial pathology are demonstrated: conjunctival and bulbar tumours with progressive deterioration of visual acuity, bilateral intracranial arachnoidal cysts, dilatation and tortuosity of the middle cerebral artery. As a consequence of these observations the prognosis in cases with this syndrome has to be given with special caution.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
8/9. Malignant melanoma of the choroid in an infant with the dysplastic naevus syndrome.A 7-month-old Algerian boy with numerous skin naevi from birth was found to have a malignant melanoma of the choroid, giving symptoms from the age of 3 months. The ocular and cutaneous tumours were shown to be melanocytic by a battery of histochemical tests and by electron microscopy. The rarity of the case is discussed on basis of the literature, and it is concluded that the case is unique because of the early age at which both the ocular tumour and the dysplastic naevus syndrome occurred.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
9/9. Three cases of primary acquired melanosis of the conjunctiva as a manifestation of the atypical mole syndrome.We report three patients with the atypical mole syndrome (AMS) [also known as dysplastic naevus or FAMMM syndrome] who presented with primary acquired melanosis (PAM). PAM is a melanocytic lesion of the conjunctiva which may progress to conjunctival melanoma. The association of this rare condition with the AMS phenotype in three individuals suggests that PAM may be a conjunctival manifestation of the AMS.- - - - - - - - - - ranking = 0.2keywords = naevus (Clic here for more details about this article) |