Giant Congenital Pigmented Nevi Research Articles

Clinical study on the role of platelet-rich plasma in human acellular dermal matrix with razor autologous skin graft repair of giant congenital pigmented nevus in children

BackgroundNA ObjectiveEvaluate the safety and feasibility of platelet-rich plasma (PRP) in the treatment of giant congenital melanocytic nevi (GCMN) in children with human acellular dermal matrix (HADM) transplantation. Patients and MethodsA total of 22 children with GCMN were included in the study. They were divided into an experimental and a control group. The experimental group used the method of HADM with Razor Autologous Skin Graft combined with PRP to repair skin and soft tissue defects after giant nevus resection (Group A, n = 11). The control group was treated with HADM with Razor Autologous Skin Graft (Group B, n = 11) only. To compare the survival rate of skin grafts, we used the Vancouver Scar Scale (VSS) for the postoperative skin graft area and the Patient and Observer Scar Assessment Scale (POSAS) to compare the two groups of patients. ResultsThere was no statistically significant difference in age, gender, location of giant nevi, and pathological classification between Group A and Group (P > 0.05). The survival rate of skin grafting and the VSS and POSAS scores of scar tissue in group A were superior to those of group B (P < 0.05). ConclusionsPRP has improved the survival rate of composite skin grafting in children with GCMN, and long-term satisfactory prognosis of scar healing. Therefore, we consider this treatment method a valuable contribution to clinical practice.

NEUROFIBROMATOSIS TYPE 1 OR GIANT MELANOCYTIC NEVUS: PROBLEMS OF DIAGNOSTIC

Neurofibromatosis type 1 (NF-1) is a hereditary disease mainly affecting skin and peripheral nervous system. Individual signs of cutaneous manifestations of NF-1 can imitate or be combined with other neurocutaneous syndromes. At present on the outpatient phase is not always possible to conduct a detailed examination of the patients with NF-1 and to determine the indications for modern diagnostic examination in a specialized hospital. It can be important to verify the diagnosis. The authors presented short review of russian and foreign literature and clinical case of the patient with a specific lesion of the skin against the background of congenital giant melanocytic nevus. Problems of differential diagnosis of NF-1 and congenital giant pigmented nevus were analyzed.

Real‐time in vivo confocal laser scanning microscopy of melanin‐containing cells: A promising diagnostic intervention

The use of noninvasive imaging techniques to evaluate different types of skin lesions is increasing popular. In vivo confocal laser scanning microscopy (CLSM) is a new method for high resolution non-invasive imaging of intact skin in situ and in vivo. Although many studies have investigated melanin-containing cells in lesions by in vivo CLSM, few studies have systematically characterized melanin-containing cells based on their morphology, size, arrangement, density, borders, and brightness. In this study, the characteristics of melanin-containing cells were further investigated by in vivo CLSM. A total of 130 lesions, including common nevi, giant congenital pigmented nevi, vitiligo, melasma, melanoma, and chronic eczema, were imaged by in vivo CLSM. This research helps dermatologists understand the characteristics of melanin-containing cells and facilitate the clinical application of melanin-containing cells in the investigation of dermatological disease. In summary, melanin-containing cells include keratinocytes, melanocytes, macrophages, and melanocytic skin tumor cells. Our study presents the CLSM characteristics of melanin-containing cells to potentially facilitate in vivo diagnosis based on shape, size, arrangement, density, borders, and brightness.

Congenital Giant Pigmented Nevi

Congenital melanocytic nevus – represents a benign proliferation of dermal melanocytes, which clinically is observed at birth or appears during the first weeks of life. Congenital nevi vary in size, macroscopic manifestations and histological characteristics [1], they appearing in about 1% of newborns. Most nevi are characterized by small size, but there are large (LCN) and giant congenital nevi (GCN) as well, which have a large area, and sometimes extend to the entire segment. GCN are relatively infrequent congenital malformation which under macroscopic study typically characterized by intense pigmentation and often can have a differently expressed hair cover [2].

先天性巨大色素性母斑に生じたproliferative noduleの1例

22歳，女性。生下時より左肩から背部にかけて広範な色素性母斑あり。左肩の色素斑の中に小学生の頃に皮下結節出現。徐々に増大してきたため紹介受診。現症：左肩の色素斑の中に1.5 cm大の皮下結節あり。局所麻酔下に皮下結節を摘出した。病理組織学的に結節を作る細胞と周囲の母斑細胞との間に移行像がみられること，細胞の異型性は高度ではないこと，分裂像は少なく，異常核分裂像がみられないこと，壊死像がみられないこと，Ki－67陽性細胞が7％と高くないことが観察された。以上より先天性巨大色素性母斑に生じたproliferative noduleと診断した。術後1年6ヵ月経つが，再発，転移は認められていない。

Congenital giant pigmented nevus

Giant congenital pigmented nevi (<1/20,000 births) occur most commonly on the posterior trunk, but may also appear on the head or the extremities. The appearance of pigmented nevi at birth is not common place. Only 3% of infants are in born with visible nevi. Most of these are small in size, averaging just above 1.5 cm in diameter. We are reporting a case of congenital giant pigmented nevus in a neonate with sacral sinus.

Traitement des nævi géants congénitaux de l’extrémité céphalique par expansion cutanée

Giant congenital pigmented nevi are benign melanocytic tumors. The lesion transformation to a malignant melanoma is a rare but severe condition. Treatment is complex and often difficult. The purpose of this study was to analyze the results and discuss the contribution of expansion technique in the treatment of giant nevi of the cephalic extremity. Six giant nevi (five children and one adult) were treated by skin expansion between 1993 and 2010. The nevus had a fronto-temporal region and vertex location (one case), and hemifacial in five cases. Subtotal resection of the nevus was performed in every case. The defect was filled by a double rotation flap of expanded scalp (one case), and an expanded total skin graft (five cases). Two complications were observed: a depression of the frontal bone in a 6-year-old child who had received a total prosthesis volume of 2000 mL; and migration of the valve under a prosthesis, which required repositioning. These complications did not alter the final results. The risk of evolution to a malignant melanoma is considerably decreased after exeresis of congenital nevi. Tissue expansion is a true skin bank. It allows treating giant congenital nevi of the scalp and face, with good esthetic results and less morbidity.

Neurocutaneous melanosis: surgical pathological features of an apparently hamartomatous lesion in the amygdala

Neurocutaneous melanosis (NCM) is a rare, congenital phakomatosis characterized by the presence of congenital melanocytic nevi and a benign or malignant pigmented cell tumor of the leptomeninges of the CNS. Here the authors report the surgical pathological features of a lesion in the left amygdala in a 10-year-old girl with giant congenital pigmented nevi and mesial temporal lobe epilepsy. The lesion exhibited high intensity on T1-weighted MR images and low intensity to isointensity on T2-weighted images. A left anterior temporal lobectomy and hippocampectomy were performed. Histologically, the lesion was composed of melanin-containing polygonal cells arranged in solid alveolar or multiple lobular patterns. Immunohistochemically, the cells were immunoreactive for HMB45, S100 protein, and vimentin, the profiles being consistent with those of melanocytes. Bundles of astrocytic processes surrounded the nests of melanocytes. Melanin-containing and dysmorphic neurons were also scattered near the nests. In the temporal neocortex adjacent to the amygdaloid melanocytic lesion, cortical dysplasia with cortical laminar disorganization was evident. Based on the histopathological features, the parenchymal lesion appeared to be hamartomatous in nature rather than a neoplasm, involving aberrant migration of melanocytes into the developing neuroepithelial tissue. This case appears to represent an unusual CNS manifestation of NCM.

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

Surgical Management of Large and Giant Congenital Pigmented Nevi of the Lower Extremity

The surgical treatment of large and giant congenital pigmented nevi of the lower extremity is a challenging endeavor with limited reconstructive options. Fifty large (>10 cm) and giant (>20 cm) congenital pigmented nevi of the lower extremity treated by the senior author (B.S.B.) over a 25-year period were reviewed. All of these nevi were too large for serial excision or direct closure. A reconstructive algorithm based on the extent and location (thigh, knee, popliteal fossa, leg, and foot) of the nevus is proposed. The approach to each anatomical region is described in detail, along with nuances of tissue expansion in the extremities. Treating large and giant congenital pigmented nevi of the lower extremity requires careful planning and often multiple stages. An evolution of the authors' approach to these lesions has led to improved outcomes. Contour and the limiting of scar contracture around the joints are of paramount importance.

The expanded transposition flap: shifting paradigms based on experience gained from two decades of pediatric tissue expansion.

The authors present their experience with the design of expanded skin flaps gained over the past two decades in a large series of 995 expanded flap reconstructions performed in 626 operations in 430 patients. The indications for tissue expansion were giant congenital pigmented nevi (72.7 percent), scar contractures (11.2 percent), and a remainder for a variety of congenital and acquired deformities. Surgical strategies were reviewed retrospectively to determine the location in the body where the tissue expansion was performed, the number of procedures required to accomplish the reconstructive goal, and the design of the expanded flap that was used to reconstruct the involved area. Specific points that were noticed included contour deformities (such as webbing, dog-ears, or decreased limb circumference) following flap reconstruction, anatomic distortions (such as distortion of the eyebrow or the distance from the brow to hairline) following reconstruction, final position of the scars in relation to anatomic landmarks, borders of aesthetic units, and relaxed skin tension lines, and the potential for later scar contracture. Careful examination of reconstruction by region of involvement demonstrated significant advantages in the use of expanded transposition flaps over pure advancement. These advantages and the modifications in the design of expanded flaps for each body region are discussed in a series of representative cases. They emphasize the ability of transposition flaps to dissipate tension away from the flap apex and distribute it more proximally, thus redirecting the tension lines so there is less likelihood of anatomic distortion in the reconstructed area. Also, flaps designed in this manner allow improved contour by avoiding webbing, tenting across concavities, and bunching of skin laterally. The authors conclude that restricting the expanded flap design to advancement alone to minimize potential scarring severely limits the reconstructive capabilities of the added tissue and distracts from the surgeon's ability to accomplish the initial reconstructive goal. The cost of additional incisions is worthwhile to achieve better final contour of the reconstructed part, lesser risk of anatomic distortion, better position of the scars, and lowered risk of scar contracture.

Large and giant congenital pigmented nevi of the upper extremity: an algorithm to surgical management.

The timing and choice of treatment of congenital giant pigmented nevi continues to evolve under the influence of changing opinions regarding the risk of malignant degeneration and the impact of excision and reconstruction on the affected child. Many studies exist to support a notable enough risk of malignancy to warrant excision, yet other series and pigmented lesion clinics suggest that the risk of malignancy does not warrant the potential scarring and deformity that has followed the surgery necessary to remove these giant lesions. To satisfy both sides in this controversy, we have been challenged to modify our surgical techniques in a manner that minimizes the risk of malignant degeneration and at the same time provides optimal functional and aesthetic outcomes for these complex reconstructions. Thirty consecutive patients with large and giant nevi of the upper extremity were treated over a 23-year period (1979-2002) by the senior author. These patients represent a subset of 259 children (12%) with large or giant congenital pigmented nevi treated and followed during this period of time. In proximal upper extremity lesions, expanded transposition flaps from the upper back and shoulder have effectively eliminated contour defects or circumferential constriction in the upper arm and axilla. An expanded free transverse rectus abdominis musculocutaneous flap has offered a possible avenue for larger lesions (shoulder and upper extremity to below the elbow), and pedicle flaps from the flank (both expanded and nonexpanded) have offered ways of improving the long-term contour in the forearm. Expanded and nonexpanded full-thickness skin grafts were chosen for reconstruction of the hand and the fingers. The authors describe in detail the surgical strategies and the techniques for reconstruction of each region of the upper extremity and then bring these ideas together in an algorithm for assessment and treatment of these challenging lesions.

025 Clinical Application of Autologous Cultured Dermal Substitutes for Giant Congenital Pigmented Nevus

This study was designed to try the application of autologous cultured dermal substitute (CDS) in conjunction with patient's own epidermis. The autologous CDS was prepared by seeding cultured autolougus fibroblasts on the spongy matrix of hyaluronic acid and atelo‐collagen. A 9‐year‐old man with a giant congenital pigmented nevus (intradermal type) was included in this clinical study. A part of nevus (30 cm × 10 cm) was excised superficially at a thickness of 20/1000 inches, and followed by second excision to remove nevus at a level of full‐thickness skin. The autologous CDS was applied to the debrided wound surface. The split‐thickenss skin obtained by first excision was preserved at 4 °C. After 1 week, the epidermis was obtained from the preserved split‐thickness skin using dispase, and followed by grafting on the wound bed, which was prepared by applying autologous CDS. This patient's own epidermis was found to take permanently, achieving an excellent clinical results.

Congenital giant pigmented nevi: Clinical features and risk of malignancy

There is general agreement that congenital giant pigmented nevi (CGPN) are precursors to malignant melanoma; however, the magnitude of the risk of malignant transformation is the subject of wide controversy. The goal of this study was to present the authors' experience with CGPN and more specifically their experience with the risk of malignancy. To identify the general features of CGPN a detailed retrospective chart review was performed at The Hospital for Sick Children in Toronto (1979 to 1994, n=84). There were 39 boys and 45 girls. The average size at presentation was 5.24% of the body surface area, and 36.9% of the nevus were located on the head and neck. An important finding was the high percentage of associated extra cutaneous disorders in patients with CGPN (23%) including a case of leptomeningeal melanocytosis. Tissue expansion was the most commonly used treatment modality. One case of malignant melanoma arising from CGPN was identified. To ensure that all cases of malignant melanoma were identified in this cohort, a questionnaire was sent to all plastic surgeons in Ontario (n=118), and data from the Ontario Cancer Registry were reviewed using the diagnostic codes for malignant melanoma and for pigmented nevus. One case of malignant melanoma was identified in all records. Thus CGPN poses a significant management challenge to the plastic surgeon and the risk of malignancy is low.

Congenital giant pigmented nevi: Clinical features and risk of malignancy

Congenital giant pigmented nevi: Clinical features and risk of malignancy

小児の悪性黒色腫

Malignant melanoma in children occurs quite rarely, but it seems to have different characteristics from those in adults. For 33 years from 1962 to 1994, totally 405 cases of malignant melanoma visited Dermatology Division of National Cancer Center Hospital. Out of them, 15 cases (3.7%) were children of less than 18 years old. The disease type was NM in 9 cases, SSM in 4 cases, ALM in 1 case and unknown in 1 case. Compared with adult cases, NM is more and ALM is less. The site of occurrence was the head in 1 case, neck in 1 case, trunk in 3 cases, forearm in 1 case, palm in 1 case, thigh in 4 cases, lower leg in 1 case, sole in 2 cases and unknown in 1 case. The distribution was quite different from adult cases. Precursor lesions of congenital giant pigmented nevus existed in 4 out of 15 cases (26.4%), but not definitely in other 11 cases. In the former group of 4 cases, the disease type was NM in 3 and unknown in 1. Two of 3 NM cases died and the prognosis was poor. In the latter group of 11 cases, NM occurred in 6, SSM in 4 and ALM in 1. Although 2 cases died, the prognosis was relatively good.

Giant Congenital Pigmented Nevi of the Face: Operative Management and Risk of Malignancy

Giant congenital pigmented nevi of the face are rare but of major consequence. Complete early excision of these lesions is recommended not only because of the cosmetic deformity they impose but also because of their life threatening potential for malignant transformation. At the Hospital for Sick Children in Toronto 19 patients with giant congenital nevi of the face were reviewed. The anatomical location of the lesions, their morphological and histological characteristics, and the authors’ experience with surgical management of these lesions were evaluated. The risk of malignancy arising from these lesions was also evaluated. In this study, one patient died of leptomeningeal melanocytosis associated with her giant congenital nevus of the face. Although an accurate incidence of malignant transformation has not been determined, the risk of malignancy in congenital giant facial nevi must be considered.

Chromosomal findings in cultured melanocytes from a giant congenital nevus

A giant congenital pigmented nevus was observed in a newborn male. A melanocyte culture was established from nevus fragments removed at age 6 days. Analysis of 136 metaphases harvested from the primary culture showed 74% normal mitoses, 22% polyploids, and 4% mitoses with chromosome rearrangements involving in particular 1p, 12q, and 19p. In addition, the culture showed a high level of HLA-DR expression. Although histology showed no sign of malignancy, these findings may illustrate one of the first events which might eventually progress toward malignancy.

Congenital malignant melanoma: a case report

Herein reported is a case of congenital malignant melanoma in a premature male baby from a 25-year-old healthy mother who was found to have hydramnios at the 29th week of gestation. The pregnancy was interrupted because of a large posterior neck mass detected by ultrasonography. The large neck mass of the baby was a malignant melanoma involving deep dermis and subcutaneous tissue. The skin over the mass showed a large area of pigmentation with hairs and the pigmentation involved the occipital scalp and posterior neck. Microscopically, the tumor cells were monotonous and showed polygonal and epithelioid appearance with prominent nucleoli indicative of malignant melanoma of a minimal deviation variety. Neither junctional components nor benign dermal nevus cells were noted. There were no distant metastasis or underlying leptomeningeal melanosis. This tumor is presumed to have developed from either preexisted congenital giant pigmented nevus with loss of benign components or de novo origin.

Congenital giant pigmented nevus and intracranial arteriovenous malformation

We describe a case with congenital giant pigmented nevus and intracranial arteriovenous malformation. This association should be included in the spectrum of neurocutaneous syndromes related to congenital giant pigmented nevus.