Small Congenital Nevi Research Articles

A gyermekkori nagyméretű és óriás festéksejtes anyajegyek eltávolításának szempontjai

The removal of common nevi is necessary only in case of irritation, changes causing complaints, or dermatological recommendations, in addition to regular inspections. In case of the need for surgical removal of special birthmarks (e.g. Spitz nevus, Halo nevus, etc.), a dermatological examination and recommendation are also essential. Congenital melanocytic nevus is a special entity. In 1-3% of newborn cases we encounter the small (<1.5 cm) congenital melanocytic nevus, and less often a giant (≥40 cm) nevus. The number, localization and morphology of congenital melanocytic nevi are more important than size in terms of the probability of melanoma occurrence. The treatment of these nevi varies individually and requires teamwork (e.g. dermatologist, oncologist, surgeon, anesthesiologist, pediatrician). All skin incisions leave a scar, and the characteristics of lifelong scars are influenced by many factors. These include age and surgical technique. For plastic surgery performed in childhood, it is advisable to choose the simplest technique – but the one that leads to the most favorable later results – when possible, to avoid complicated flap plasties and skin replacement procedures. When developing the surgical strategy, it is essential to involve the parent and the child in addition to the allied professions, as well as keeping in mind the characteristics of childhood. In the optimal case, the completion of the reconstruction should carry out before get into community, but at the latest by the teenage years.

Black tumor of the umbilicus

Pigmented nevi, still called melanocytic nevi or more improperly "naevus naevocellulaires" are benign melanocytic tumors characterized by a proliferation of melanocytes near the dermoepidermal junction. They are grouped in clusters or in theca cells that differentiate them from normal melanocytes. They occur at birth in approximately 1% of newborns with no sex predominance. Their occurrence is mostly sporadic, although rare family forms have also been reported. They can be separated in an arbitrary manner according to their diameter on the day of the birth: small congenital nevi <1.5cm, medium-sized 1.5 to 20cm and large-sized >20cm. Umbilical nevi are rare. We here report an interesting case of atypical and rare congenital nevus. The study involved a young girl presenting with pigmented umbilical nodule occurred at birth which had recently become problematic. We suspected a nevus, a melanoma or an endometriosis on the basis of the clinical appearance of this nodule. Resection was performed. The diagnosis of benign melanocytic tumor was confirmed.

Clinical and dermoscopic characteristics of congenital melanocytic naevi.

Prompted by the limited data, we conducted this study to gather more information on dermoscopic features of CN in children, in order to optimize clinical care and management. All children with congenital nevi (CN) attending our Pediatric Pigmented Skin Lesion Unit during a 2-year period were included in the study. Clinical data were collected, and all children underwent clinical and dermoscopic examination. Dermoscopic patterns and specific features were recorded. Three hundred and thirty CN were examined in a population of 276 children, aged from 6 months to 14 years. The majority (85.14%) had only one congenital naevus, and 43.12% had a family history of congenital nevi. Children with multiple congenital nevi were more likely to have a positive family history of a CN (P = 0.012). Only, in 23 children, neurological/developmental abnormalities were reported. Small CN were the commonest in our cohort (167) followed by the medium-sized (160), whereas large CN (>20 cm) were only three. Thirty-eight CN were located on the volar skin. The globular was the commonest dermoscopic pattern, followed by the reticular, whereas the parallel furrow pattern was the commonest pattern on palms and soles. CN on the trunk were more likely to be globular on the limbs, and reticular and homogeneous on the head and neck (P < 0.001). The commonest dermoscopic findings were haloed and target globules, blotches and perifollicular hypopigmentation, whereas globules and dots around cristae on volar skin. CN located on the limbs were more likely to demonstrate an atypical network (P = 0.001) and a target network with globules (P = 0.020), whereas haloed and target globules (P < 0.001), blotches (P = 0.023) and dots (P = 0.004) were found with an increased frequency in CN on the trunk. Given that there is much controversy on the management and accurate classification of CN, our findings may provide useful information.

Report of a malignant melanoma arising in a small congenital nevus in a 3-year-old child

Report of a malignant melanoma arising in a small congenital nevus in a 3-year-old child

Small and Medium-Sized Congenital Nevi in Children: A Comparison of the Costs of Excision and Long-Term Follow-Up

Small and Medium-Sized Congenital Nevi in Children: A Comparison of the Costs of Excision and Long-Term Follow-Up

Nodular melanomas associated with nevi

To the Editor: Nodular melanoma (NM) represents approximately 9% to 15% of invasive melanomas. The histopathologic definition of NM is based on the absence of a radial growth phase. Most frequently, it manifests itself in midlife with a median age at presentation of 53 years, and it is more common in males than in females. In 11% to 25% of the cases, NM is histologically associated with nevi. We report the experience of the Melanoma Unit of University Hospital Spedali Civili of Brescia, Italy. Between January 1, 1982 and October 1, 2009, 95 patients with cutaneous NM were identified from a group of 1865 patients with histologically confirmed melanoma; the incidence was 5.1% (95/1865). Of the 95 patients evaluated, 49 (51.6%) were males and 46 (48.4%) were females. All patients were white. The average age was 54.5 years (range, 15-91). Primary melanomas were found in different body sites as follows: eight (8.4%) lesions were on the head and neck, 32 (33.7%) on the trunk, 22 (23.2%) on the upper extremities, and 33 (34.7%) on the lower extremities. The lesions appeared most frequently on the trunk in male patients and on the lower extremities, upper extremities, and trunk in female patients. None of our 95 cases had a documented family history of melanoma. With regard to invasive melanomas, Breslow thickness ranged from 0.5 mm to 11 mm, with a mean thickness of 3.9 mm. The mean Breslow thickness was 4.4 mm in male patients and 3.4 mm in female patients. Ten out of 95 patients (10.5%) showed histologic evidence of an associated nevus; five patients were male and five were female. The average age of these 10 patients was 55.9 years. In seven patients, melanoma arose in association with an acquired nevus (2 dysplastic nevus and 5 other acquired nevi). In the remaining three patients, melanoma arose in association with a small congenital nevus. When examined by anatomic location, one lesion was on the head and neck, five were on the trunk, one was on the upper extremities, and three were on the lower extremities. The mean Breslow thickness for these 10 patients was 2.3 mm. When comparing the two groups (NM vs NM associated with nevi), the second group showed a thinner Breslow thickness (4.04 mm vs 2.3 mm). Our findings support the belief that in most cases, NM arises in absence of a precursor lesion. NM often fails to exhibit the original ABCD diagnostic criteria,

A Case of Multiple Small Congenital Nevus Cell Nevi, and a Classification and Statistical Observation of Congenital Nevus Cell Nevi

A Case of Multiple Small Congenital Nevus Cell Nevi, and a Classification and Statistical Observation of Congenital Nevus Cell Nevi

Giant Congenital Melanocytic Nevi

Giant or large congenital melanocytic nevi (CMNs) measuring more than 20 cm in diameter are present in 1 out of 20 000 newborns, with an estimated 6.3% lifetime risk of developing malignant melanoma. Approximately half of all melanomas that arise within a giant congenital nevus do so by 5 years of age. They occur most commonly on the posterior trunk but may also appear on the head or the extremities. Congenital pigmented nevi have been categorized by size: Giant congenital nevi are more than 20 cm in diameter; small congenital nevi are less than 2 cm in diameter; and intermediate nevi are in between in size. Giant nevi frequently lie in the distribution of a dermatome and will cover areas like an arm, leg, or a significant part of the trunk. These lesions are usually termed in a descriptive manner, for example, coat-sleeve, stocking, bathing-trunk, or giant hairy nevi. Giant hairy nevi are associated with neurological disorders. Nevi on the scalp and neck are associated with an increased incidence of leptomeningeal melanocytosis, epilepsy, and other focal neurological abnormalities. Those located over the vertebral column are associated with increased risk of spina bifida and meningomyelocele. The presence of ‘‘satellite’’ melanocytic nevi increases the risk of leptomeningeal involvement, particularly when located on the head or midline on the trunk. Nevus cells within the leptomeninges may cause increased intracranial pressure with subsequent hydrocephalus, seizures, and motor deficits. We describe a newborn with a bathing-trunk giant congenital pigmented nevus. Case Report

High Frequency of BRAFV600E Mutation in Acquired Nevi and Small Congenital Nevi, but Low Frequency of Mutation in Medium-Sized Congenital Nevi

To investigate whether the frequency of the BRAF(V600E) (V-raf murine sarcoma virus oncogene homolog B1) mutation in melanocytic nevi is associated with sun exposure patterns, we examined 120 acquired melanocytic nevi excised from various anatomic sites, including glabrous skin, as well as 62 congenital nevi. We used a new mutation detection system based on the shifted termination assay, called Mutector, which was able to detect only 5% of heterozygous mutant cells within the samples. We detected the mutation in 105/120 (87.5%) acquired nevi and 43/62 (69.4%) congenital nevi. Notably, we found the mutation in 35/43 (81.4%) acquired nevi excised from glabrous skin and genitalia. These results strongly suggest that UV light is not necessarily required for the acquisition of the BRAF(V600E) mutation, and suggest that non-mutagenic effects of UV light to melanocytes may be more important in the nevogenesis. Additionally, we showed heterogeneous distribution of BRAF-mutated cells within the lesions of small congenital nevi by a combination of laser microdissection and direct sequencing. Finally, we found low frequency of BRAF(V600E) mutation (6/20, 30.0%) in medium-sized congenital nevi. Most of these nevi with wild-type BRAF had neroblastoma ras viral oncogene homolog mutations (9/14, 64.3%), suggesting different pathogenesis of medium-sized congenital nevi from acquired nevi and small congenital nevi.

Moles and melanoma.

The melanoma epidemic in adults is well documented, and there is now evidence that the incidence of malignant melanoma in teenagers is increasing. Risk factors for melanoma are recognizable in children and include congenital nevi, numerous common nevi, and atypical nevi. Large congenital nevi overlying the head or spine also carry risk for central nervous system involvement, which, if symptomatic, carries a grave prognosis. Laser therapy has recently been advocated for small congenital nevi but often yields only temporary improvement. Adjuvant therapy with interferon alfa-2b holds promise for patients with metastatic melanoma. Melanoma risk is also linked to sun sensitivity and childhood exposures, and sunscreen use has been promoted for prevention of skin cancer. Because many sunscreens offer protection from ultraviolet (UV) B but not UVA, spectra that may be involved in melanoma induction, pediatricians should counsel their families to practice a full program of sun protection that includes sun avoidance and protective clothing and eyeware in addition to sunscreens.

The risk of melanoma in patients with congenital nevi: A cohort study

Background: Giant congenital nevi are associated with a greatly increased risk of melanoma, but this has not been quantified. Smaller congenital nevi are believed by some authors to be associated with increased risk, but this is uncertain and needs to be clarified. Objective: Our purpose was to analyze cause-specific mortality and cancer incidence risks in patients with congenital nevi according to size of the nevi. Methods: We followed up 265 patients with congenital nevi first treated at the Hospital for Sick Children or at St. John's Hospital in London during 1950 to 1984 for mortality to mid-1993 and for cancer incidence from 1971 to 1989. Mortality and cancer incidence rates in the cohort were compared with expectations from national mortality and cancer incidence rates by sex, age, and calendar period. Results: Among the 33 patients with a congenital nevus covering at least 5% of the body area, two melanomas occurred during follow-up; both were fatal. The relative risk of melanoma mortality in these patients was 1046 (95% confidence interval, 127 to 3779). In the remaining 232 patients, 68 of whom had a nevus covering 1% to 4% of the body, and 164 with nevi smaller than 1% of body area, no melanomas occurred (0.18 melanoma deaths expected). The difference in melanoma mortality risk between the group with a nevus covering at least 5% of the body and the group with smaller nevi was significant ( p < 0.05). There was not a significantly increased risk of nonmelanoma mortality or of nonmelanoma cancer incidence overall in the cohort, although two lymphohematopoietic malignancies occurred. Conclusion: The data show the large risk of melanoma in patients with nevi covering more than 5% of the body surface area. The results do not support the hypothesis of greatly increased risk in persons with congenital nevi smaller than this, but because the confidence intervals of the result were wide, the data are compatible with a sizable risk. Much larger studies than those that have so far been undertaken, or combined analysis of data from several studies, are needed to quantify more precisely the risk of melanoma in relation to size of nevi and to determine the appropriate clinical management of these lesions.

Precursors to skin cancer

Certain cutaneous lesions serve as both precursors of skin cancer and markers for increased risk. The solar or actinic keratosis serves such a role for the nonmelanoma (NMSC) forms of skin cancer (basal cell carcinoma and squamous cell carcinoma). Clinically, these keratoses manifest as rough, scaly, erythematous patches on chronically sun-exposed surfaces. Conversion to squamous cell carcinoma in an individual lesion is uncommon and has been estimated at 1 per 1000 per year. Individuals with actinic keratoses have had sufficient chronic photodamage to produce skin cancer, and regular surveillance is recommended. The second precursor for invasive NMSC is Bowen's disease (squamous cell carcinoma in situ). Invasion of the dermis results in frank squamous cell carcinoma. Some types of viral warts may develop into squamous cell carcinoma. The most important precursor/marker for melanoma is the clinically atypical mole (CAM) or dysplastic nevus. CAMs occur in 5-10% of the U.S. population. CAMs, under photographic follow-up, have been observed to evolve into cutaneous melanoma. The frequency of conversion to melanoma of any single CAM is quite low; however, in melanoma-prone families, prospectively diagnosed melanomas arise in association with a histopathologically observed dysplastic nevus in more than 80% of the cases. Giant congenital melanocytic nevi have an approximately 6% lifetime risk of melanoma development. The risk associated with small congenital nevi is uncertain. Lentigo maligna develop into invasive melanoma with a frequency reported in the literature ranging from 5-50%.

Congenital Nevi and Melanoma Risk

The degree of risk of developing melanoma in a congenital nevus remains controversial. Most authors now believe that the cumulative lifetime risk is up to 10% for patients with giant congenital nevi (greater than 20 cm in diameter), but there is no consensus about the risk in smaller congenital nevi. This study readdresses this topic, confirming previous findings without adding significant new data. The authors retrospectively …

Nevus cells and special nevomelanocytic lesions in children.

Nevomelanocytic lesions in children represent a wide spectrum of proliferative neurocristopathies, ranging from the common small and benign congenital nevi to the garment-type congenital lesions. Included in this spectrum are some rare entities like neurocutaneous melanocytosis, malignant melanoma simulants, and special variants of congenital pigmented nevi. Here, we analyze some tissue culture characteristics of nevomelanocytic cells from giant and small congenital nevi, neurocutaneous melanocytosis, and a rare variant of nevus named "bulky nevocytoma." Correlations with their histological, immunohistochemical, and ultrastructural features are addressed.

Malignant melanoma arising from a small congenital nevus in a black child

Malignant melanoma arising from a small congenital nevus in a black child

MALIGNANT MELANOMA IN CHILDREN

While significant risk factors for malignant melanoma may initially develop or are first seen in childhood, the actual occurrence of this neoplasm in prepubertal children is uncommon. A retrospective study of malignant melanoma in Puerto Ricans up to 16 years of age occurring from 1973 to 1990 was carried out by identifying those cases in the Puerto Rico Cancer Registry. A total of seven cases were found consisting of three boys and four girls with ages ranging from 22 months to 16 years and comprising 0.94% of the total melanomas. In three of the seven cases, there was a history of a previously existent small congenital melanocytic nevus on the area. Three cases were Clark's level I, two level II, and in two cases with proved metastatic disease, Clark's level of invasion were not reported. Those cases with Clark's level I and II had a 100% 5-year survival. Although rare, malignant melanoma in children can be as aggressive as in adults. Among the known factors predisposing to malignant melanoma, three out of seven cases developed within a small congenital nevus, two of which occurred during the first decade of life. Due to the rarity of this event in our population, it appears unreasonable to excise all small congenital nevi during the first decade of life. Even for those who advocate excision of all small congenital nevi, the evidence at present suggests that such small nevi very rarely undergo malignant change before puberty and therefore a policy of observation in childhood and offering excision around the time of puberty is perfectly logical.

特異なリンパ節病変を伴う，先天性母斑に合併した悪性黒色腫の１例

A 30-year-old man with malignant melanoma of the left foot arising in a small congenital nevus was reported. Histopathologically, an expansile nodule was observed, which pushed the residual congenital vevus, in the dermis. The nodule was composed of monomorphic spindle cells with fine melanin granules and vesicular nuclei. Although no mitotic figures were found, we considered the nodular lesion as a nodular melanoma.In 3 out of 18 inguinal lymph nodes, there were nevus cell aggregates. They were situated mostly in the capsule and trabeculase. However, atypical large cells with melanin granules were found, intermingling with nevus cells, not only in the capsular area but also in the node parenchyma.

Precursor lesions to melanoma

T he field of precursor lesions to melanoma is subject to ongoing debate and evolving insights. Regarding congenital melanocytic nevi there is controversy about the management of these lesions. It has become apparent that patients with giant (“bathing trunk-type”) congenital nevi have an increased incidence of malignant melanoma developing in their lesions. Prophylactic excision is generally accepted treatment and these lesions should be removed in early childhood; however, the subject of debate has been enlarged to include all congenital nevi, including those of the “small” type. It is unclear to what extent melanoma develops in smaller congenital nevi and dermatologists disagree on the therapy of smaller lesions. The intensity of the controversy appears to be inversely proportional to the size of the nevus. Regarding dysplastic nevi there is controversy about diagnostic definitions both clinically and histologically. Some even deny the existence of dysplastic nevi! The Familial Atypical Multiple-Mole Melanoma (FAMMM) syndrome also has its contentions with regard to the genetic mechanism: Is a dominant gene localized on chromosome lp involved in all FAMMM families? Furthermore, the complete spectrum of the FAMMM syndrome including the occurrence of systemic cancer(s) and ocular melanoma is still debated. This review focuses on the aforementioned controversies in the field of precursor lesions to melanoma. Early detection of malignant melanoma reduces its morbidity and mortality. The most important method to achieve this is by professional and public education about the signs and symptoms of early melanoma and preven-

Evaluation of human monoclonal antibody (2-139-1) in cutaneous melanocytic neoplasms in fixed tissue sections.

Despite the growing list of xenogeneic monoclonal antibodies (MAb) that recognize malignant melanoma-associated antigens (MAA) in formalin-fixed, paraffin-embedded tissue, none has been able to detect epitopes found in malignant melanomas and not in melanocytic nevi. A human MAb, 2-139-1, that showed promise in this regard was evaluated against 85 melanocytic neoplasms, including malignant melanoma and histological simulators, particularly Spitz's nevus. MAb 2-139-1 stained 18 (53%) of 34 melanomas, eight (57%) of 14 dysplastic nevi, six (38%) of 16 Spitz's nevi, and three (14%) of 21 banal nevi, which included three small congenital nevi. We observed a significant increasing trend in reactivity (% positive cells x intensity) associated with the potential for malignancy (p for linear trend = 0.002). We conclude that human MAb 2-139-1 is applicable to the study of melanocytic neoplasms in routinely processed tissue. Although the ability of this MAb to separate benign from malignant cells is not absolute, our results suggest that the expression of the 2-139-1 epitope may be an early event in melanocytic tumor progression.

Nonepidermal origin of cutaneous melanoma in a small congenital nevus

Nonepidermal origin of cutaneous melanoma in a small congenital nevus