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Background: Linear nevus sebaceous syndrome (LNSS) is a rare genetic disease characterized by large linear sebaceous nevus typically on the face, scalp, or neck. LNSS could be accompanied by multisystem disorders including the central nervous system. Herein, we report gene mutational profile via whole exome sequencing of both lesional and non-lesional skin samples in a LNSS patient., Case Presentation: A 17-year-old girl presented with multisystem abnormalities, including large skin lesions, ocular disorders, abnormal bone development and neurological symptoms. A diagnosis of LNSS was established based on clinical manifestations, histopathological and imaging findings. The skin lesions were resected and no recurrence was noted at the time of drafting this report. Whole exome sequencing of genomic DNA revealed the following 3 mutations in the lesions of the index patient: KRAS (c.35G > A, p.G12D), PRKRIR (c.A1674T, p.R558S), and RRP7A (c. C670T, p.R224W), but no mutation was found in the healthy skin and peripheral blood sample of the index patient, or in the blood samples of her parents and sibling. PCR-mediated Sanger sequencing of DNA derived from lesional skin sample of the index patient verified KRAS mutation, but not PRKRIR (c.A1674T, p.R558S) and RRP7A (c. C670T, p.R224W). None of the 3 mutations was found in Sanger sequencing in skin lesions of 60 other cases of nevus sebaceous patients., Conclusions: Our findings show the relevance of KRAS mutation to LNSS, providing new clues in understanding related genetic heterogeneity which could aid genetic counselling for LNSS patients.

Background: 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., Materials and Methods: 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., Results: 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., Conclusions: Given that there is much controversy on the management and accurate classification of CN, our findings may provide useful information., (© 2018 European Academy of Dermatology and Venereology.)

Congenital melanocytic nevi (CMN) are nevi that are present at birth or arise within the first few weeks of life. They are often found on the trunk, head and neck and extremities. We report herein an unusual presentation of a CMN as a cerebriform tumour presenting as secondary cutis verticis gyrata on the scalp of an 18-year-old Filipino male., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Infantile haemangiomas are the most common soft tissue tumours of infancy and childhood, with an incidence rate of 4-10 %. These lesions commonly present between 2 and 10 weeks after birth and characteristically involute around 8-10 years of age. Most lesions occurring in the scalp are managed conservatively, with surgery reserved for those with complications., Case Description: A 5-year-old girl presented with a swelling over the left occiput noticed since birth, with a gradual increase in size and rapid progression since the last 1 year. She had undergone six sessions of intralesional steroids over the past 3 years. However, recent MRI and CT scans showed an increase in the swelling size with full-thickness erosion of the underlying calvarium without neuroparenchymal involvement. The patient underwent surgical excision of the lesion with an excellent outcome. Our literature review revealed only a single case of haemangioma which arose from the scalp and caused significant underlying calvarial erosion and intracranial extension., Conclusion: We present a case of an atypical infantile haemangioma arising in the subgaleal plane causing full-thickness erosion of the underlying calvarium.

Neurocristic hamartoma (NH) is a rare dermal melanocytic lesion that is formed due to the aberrant development of neural crest-derived melanocytes during their course of migration through the dermis at the time of embryogenesis. Here, we describe a case of NH in a 6-year-old boy who clinically presented with diffuse plaque-type blue nevus on his scalp with a contiguous extension into the cervical region and lymph node involvement. A subcutaneous nodule displaying a marked histological heterogeneity with lymph node involvement is a very unusual and diagnostically challenging presentation of NH. The importance of an accurate diagnosis of NH lies in the fact that malignant transformation can rarely occur within these lesions over an unpredictable time course and remain undetected, rendering clinical management difficult. Although our child had a benign course after a follow-up of 5 years despite lymph node involvement, the possible risk of development of malignant melanoma in such a lesion warrants long-term surveillance. This case report highlights the unusual clinical presentation and histopathological features of this rare entity along with a relevant review of the literature. The present case also underscores the concept that sentinel lymph node involvement in certain melanocytic lesions in children must not be mistaken for malignant melanoma.

Bone involvement has been described in tumors with melanocytic differentiation such as melanotic neuroectodermal tumor of infancy, and very rarely in cellular blue nevi and neurocristic cutaneous hamartoma. We present an unusual case of facial congenital melanocytic tumor that involved the underlying bones and maxillary sinus and led to unilateral blindness. A newborn with a large red bluish patch with peripheral brown and black macules overlying marked swelling on the left side of his face was presented. The tumor was shown by magnetic resonance imaging, scintigraphy, and histopathology to invade the underlying bones and maxillary sinus and to compress the left eyeball resulting in blindness. Histopathology, immunohistochemistry, morphometric computerized microscopy, molecular genetic mutation analysis, and fluorescent in situ hybridization studies were more congruent with a melanocytic nevus. An 8.5-year follow-up was uneventful, with spontaneous partial shrinkage of the tumor.

Unusual congenital or early-onset skin lesions on the scalp often pose a diagnostic challenge particularly as the clinical evaluation may be hampered by dense hair growth. Thus, this paper provides a concise review on developmental abnormalities and nevi with exclusive or predominant scalp localization. Aplasia cutis congenita occurs as an isolated finding, in association with genetic syndromes, nevi and anomalies or as a consequence of intrauterine trauma and teratogens. A hairless area with a narrow surrounding rim of hypertrichosis (hair collar sign) may point to occult cranial dysraphism, especially if accompanied by further suggestive signs as port-wine stains, large hemangiomas, dimples, congenital dermoid cysts, and sinuses. Many diverse entities may hide behind cutis verticis gyrata with the primary essential form being rare and representing a diagnosis of exclusion. In contrast to former belief, benign adnexal tumors arise in a nevus sebaceus considerably more often than basal cell carcinomas and other malignant epithelial tumors. Provided that tumor development is not suspected, excision of a nevus sebaceus nevus is indicated primarily for aesthetic-psychosocial reasons. However, surgical treatment is considerably easier in small children. Nevus sebaceus may be a cutaneous marker for several complex syndromes whereas nevus psiloliparus presents almost always in connection with encephalocraniocutaneous lipomatosis. Congenital melanocytic nevi of the scalp tend toward clinical regression, so that surgical intervention in large lesions should be carefully considered. In contrast, the threshold for excision of blue nevi and other conspicuous melanocytic nevi on the scalp should be low, especially since they are difficult to monitor.

Congenital hemangiomas (CH) are benign vascular neoplasms that proliferate in utero and have completed development by birth. Two subtypes of CH are recognized: rapidly involuting congenital hemangiomas (RICH) and non-involuting congenital hemangiomas (NICH). Involution of the RICH subtype often begins in the first weeks of life. NICH does not involute, allowing the distinction between RICH and NICH. We report a case of an infant with RICH occurring on the scalp, examined at birth and followed for 26 weeks.

Congenital melanoma is extraordinarily rare, and 3 types have been described: transplacental metastases from the mother, de novo congenital melanoma, and melanoma occurring in association with a congenital melanocytic nevus. We describe 2 reports of array comparative genomic hybridization analysis of de novo congenital melanoma. The first patient was male, and the second was female; both had a scalp lesion present at birth, which grew quickly. The scalp mass from patient 1 showed a heterogeneous, anaplastic melanocytic neoplasm with large size and depth, high mitotic rate, ulceration, and necrosis. The scalp mass from patient 2 showed a broad melanocytic neoplasm with single cell and junctional nested proliferation at the dermal-epidermal junction and cellular, confluent aggregates of highly atypical melanocytes in the dermis with high mitotic rate. Patient 1 had lung and liver metastases detected by radiologic imaging and was treated with cisplatin, vinblastine, and dacarbazine but expired at the age of 5 months. Patient 2 developed a metastasis to the right neck with similar histologic features, and pulmonary metastases were also detected by imaging. Patient 2 is currently alive at the age of 4 years. Array comparative genomic hybridization analysis of the first case revealed loss of chromosomes 3p26.3-p21.31, 5p15.33-q23.1, 11q15.5-q13.2, 14 (complete deletion), and 15q11.1-q22.31. The second case displayed gains in chromosomes 1q21.1-q44, 2p25.3-p11.1, 2q11.1-q37.3, 6p25.3-p11.1, 7p22.3-p11.2, 7q11.1-q36.3, 8p23.3-p11.1, 8q11.1-q24.3, 9p24.3-p11.2, 9q12-q34.3, 11q13.2-q13.4, 13q11-q34, 18p11.32-p11.21, 19p13.3-p11, 19q11-q13.43, 20p13-p11.1, and 20q11.21-q13.33. In both cases, the presence of multiple chromosomal aberrations corroborated the diagnosis of melanoma.

We present a case of congenital malignant melanoma of the scalp in a neonate. The child was born through caesarean section with a swelling, the size of a tennis ball, on the posterior scalp. At presentation to the clinic at 25 days after birth, the swelling had significantly increased in size and ulcerated. An excision was carried out but, because of extensive haemorrhage and haemodynamic instability, the procedure was limited to subtotal resection. Later on, completion of the excision and flap coverage of the wound were performed. After an initial stable course of a few months, the child came back with local recurrence. A re-excision was planned but the child developed pneumonia resulting in sepsis leading to the demise of the child. The report adds to the literature by describing a rare entity and challenges of managing large vascular scalp lesions with complete excision and defect coverage.

Congenital melanocytic nevus (CMN) is a melanocytic proliferation that has its onset at birth or shortly thereafter and shows characteristic histopathologic features including symmetric proliferation of benign melanocytes, extension of nevus cells into the deep reticular dermis and subcutis, maturation of melanocytes with descent, tracking of melanocytes around and within adnexal structures, vessels, or nerves and splaying of collagen bundles by nevus cells arranged in single rows or cords. We report the case of a 34 year old previously healthy woman who presented with a progressively enlarging soft tissue mass in the right neck and back adjacent to a medium sized CMN. Magnetic resonance imaging showed multiple lipomatous masses within the soft tissues of the posterior superficial neck. Subsequent excision of the soft tissue mass showed a well circumscribed lipomatous lesion with diffuse infiltration by benign appearing melanocytes within the fat lobules. Excision of the mass was not accompanied by overlying skin and, thus, posed a diagnostic challenge. Sudden increase in the size of a CMN is worrisome for the development of a melanoma, however, this lesion lacked significant cytologic atypia and mitotic figures, and had a low proliferative index by Ki-67 immunohistochemistry. This case serves to illustrate the initial diagnostic dilemma as well as the plasticity of the neural crest cells.

We report a case of a 20-year-old Chinese man with an alopecic congenital combined compound and blue melanocytic nevus of the scalp, associated with alopecia areata. The diagnosis of a combined melanocytic nevus was confirmed by histopathological examination and immunohistochemical stains, with exclusion of neurocristic hamartoma, which can have a similar clinical and histopathological appearance but different prognosis. In addition, we explore the association of this large melanocytic lesion with alopecia areata.

Background: Infantile hemangioma is the most common vascular tumor in newborns, with an incidence from 12 to 23% among preterm infants with low weight at birth and a female to male ratio of 3:1. The head and neck is the most frequently affected area (60%), and the scalp is a typical site for such large lesions., Objective: We describe some clinical and medical aspects in comparison with the surgical approach to giant infantile hemangioma of the scalp., Methods: The indications to treatment are discussed. An outcome basis evaluation, by reviewing some clinical cases, is provided to help readers better understand when and how to undergo surgery safely., Conclusion: Early excision of huge infantile hemangioma of the scalp is the treatment of choice if feasible within 5 months of age.

Neurocristic tumors are rare growths with a predilection for the scalp region. These lesions have a highly variable appearance and an unpredictable course. Long-term surveillance is recommended due to their potential for recurrence and possible malignant transformation into melanoma. The following is a report on congenital neurocristic tumor of the scalp and neck region. Laryngoscope, 2013., (Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.)

Objective: Discuss effect and dynamics of propranolol (PR) treatment in infantile haemangioma (IH) of head and neck in children during follow-up., Methods: Between 2010 and 2011, 22 children with head and neck infantile haemangioma (IH) treated by PR were recruited into the study. In a retrospective chart review clinical data were analyzed at 5 consecutive, different check-up time from 1 week to 12-14 months. Effectiveness of PR treatment was assessed by a symptom score method., Results: In the whole series a significant regression was observed in 13 patients (59%) in the first week of the therapy. Further five patients showed this time a marked, two mild improvements, and two children did not respond initially to the PR therapy. In one of them (case #8) later on a mild improvement could be seen too. At the second check-up (1 month after initiating PR therapy) 50% of children showed definitive improvement compared to the first visit. Difference between first and second check-ups was significant, and between the 4th and 5th visits the improvement showed the lowest rate. Comparison of IH regression between the 2nd and the 5th check-ups resulted in a p value a little larger than 0.05. There was not significant correlation between the initial IH severity and the treatment effectiveness at the follow-ups (p>0.05). No significant differences were found in treatment effectiveness concerning the IH localizations, too., Conclusion: PR treatment is highly effective in children with IHs. The most striking effect is seen at the first week of treatment; later improvement is much slower, sometimes with periods of stagnations. The cause of this is probably the spectacular early effect of vasoconstriction, though other impacts of PR to the individual molecular markers of IH seemed to be less impressive clinically. However, treatment should be continued for at least 6 months because early cessation can cause a relapse., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: Malignant rhabdoid tumors (MRT) are rare but aggressive tumors presenting in the pediatric population. First thought a variant of Wilms' tumor in the kidney, it is recognized as presenting at renal, central nervous system and other extra-renal primary sites. It is uniformly of very poor prognosis, however., Case Report and Discussion: We present a case of congenital MRT of the scalp, which we believe to be the first described at this site. The clinical and histopathological features of the tumor are discussed in light of the current literature on MRT at other sites. The bleak prognosis at this site appears to be no different from others - the child succumbed at 10 months old despite surgical resection and initial excellent response to chemotherapy., Conclusion: Malignant rhabdoid tumor has a very poor prognosis and needs to be considered in the differential diagnosis of similar lesions by clinicians involved in pediatric head and neck care., (Copyright © 2011 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Introduction: 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., Material and Methods: Six giant nevi (five children and one adult) were treated by skin expansion between 1993 and 2010., Results: 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., Discussion: 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., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Congenital malignant melanoma within a pre-existing large congenital melanocytic naevus (CMN) is exceedingly rare. Its incidence is difficult to determine due to the small number of reported cases and because of problems associated with diagnosis. Some benign nodular proliferations (called proliferative nodules) arising in CMN, while rare, are significantly more common and can mimic malignant melanoma clinically or histologically. There are no reported cases of congenital melanoma or benign proliferative nodules in CMN in patients who also had eruptive disseminated Spitz naevi. We describe a girl who was noted to have a dark-brown plaque with several large erythematous nodules affecting the scalp at delivery, in addition to multiple erythematous dome-shaped papules that developed in a disseminated manner over several months, beginning at 10 days of age. It was difficult, not only clinically but also histologically, to determine the benign or malignant nature of all of these lesions. As primary cutaneous melanoma, atypical proliferative nodules in CMN, bland CMN or CMN with foci of increased cellularity and Spitz naevi show clear differences in the genetic aberration patterns, comparative genomic hybridization (CGH) could be a diagnostic help in ambiguous cases such as this. CGH performed on this patient showed multiple DNA copy number changes in the most atypical nodule, but such alterations could not be found in the remainder of the lesions. CGH showed differences between the nodular lesions that occurred in the CMN and helped us in supporting the diagnosis of this unique case of benign proliferative nodules and a possible congenital melanoma arising in a large CMN, associated with multiple widespread eruptive Spitz naevi., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists.)

Unlabelled: The infantile myofibromatosis is a rare pathology usually affecting children under 2 years. More than half are congenital, located in head and neck in a 30% of the cases. Myofibroma is a benign mesenchymal tumor that appear an isolated lesion or multiple, with or without visceral involvement. Case 1: A newborn girl that presents lump in scalp suspected of encefalocele. Ultrasound and RM: solid and heterogeneous mass 4 x 2 cm with increased peripheral vascularisation. Case 2: A 7-month-old baby boy with lump in right occipital zone detected at 4 months. Ultrasound and RM: injury 1 x 1.5 cm in head esplenio muscle suggestive of hemangioma., Results: After surgical total resection, the histological study and immunohistochemical identified the lesion as myofibroma. The evaluation of disease dissemination was normal and the recovery of the patients was favorable. The differential diagnosis of myofibroma must be carried out with other mesenchymal tumors and with non neoplastic injuries characteristics of the region affects. The visceral location and multiple forms worsen the prognosis, so is important do an exhaustive study. Treatment is conservative surgery, but if there is no risk to the child, the possibility of spontaneous regression makes the abstention therapeutic a correct alternative.

Congenital cranial teratomas are usually characterized by complete loss of the normal intracranial architecture. In the majority of reports, the tumors are associated with stillbirth, perinatal death, or significant morbidity after surgical resection. The few reported attempts at total or subtotal tumor resection have had poor outcomes, although there are rare reports of prolonged survival up to 3.5 years following resection of smaller tumors. Neonatal teratomas are rarely located in the scalp. In the literature, there were only a few patients who underwent surgery during the neonatal period with a good outcome; however, all such patients survived. In this paper, we present a neonatal case of huge congenital extracranial immature teratoma on the scalp extending to the orbita, ears, and brain. Examination of the patient revealed a large craniofacial mass and head circumference that was bigger than normal; there were no other neurological deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a multiloculated, heterogeneous cystic mass that was larger than the patient's head, displacing and distorting anatomical structures. MRI showed mass with calcifications, soft tissue, fat, and fluid components. There was deformity and remodeling of the adjacent calvaria. A total surgical excision was performed and histopathological examination showed immature teratoma. The patient's early postoperative course was uneventful. Postoperative CT and MRI were normal. To date, the patient has survived for 6 months without neurological deficit. We conclude that acceptable functional outcomes in the context of massive congenital craniofacial teratomas can be achieved by early radical resection.

Congenital giant melanocytic nevi of the scalp and forehead are rare lesions present at birth. These lesions are associated with risk of malignant transformation, but they primarily represent a psychological problem to both patient and parents and merit early excision and reconstruction. In this study we report our own experience: seven patients, aged 8 months to 9 years, with congenital pigmented nevi involving forehead and scalp, and a 4-year old patient with congenital pigmented nevus of periorbital region and nose were treated successfully with excision and expanded skin flap reconstructions. The mean expansion procedures were 2 (range, 1 to 3), with an average of 8,8 injections for each expansion procedure (range, 6 to 11). In only one patient simultaneous expanders were placed in the scalp and forehead. Follow-up ranged from 4 months to 15 years. We had no rupture, extrusion or infection of the skin expanders. Complications included eyebrow ptosis and asymmetry in two patients underwent correction at a final procedure. In our opinion tissue expansion is an excellent technique for the treatment of giant nevi of the scalp and forehead because it offers the best aesthetic and functional outcomes.

Being non-hair-bearing and relatively thin and having similar color and texture of the skin of the forehead provide an excellent characteristic not only for nasal reconstruction but also in other facial areas. A 28-year-old man presented in the Department of Plastic Surgery, Argerich Hospital, Buenos Aires, Argentina, with a giant congenital melanocytic nevus extended on complete left cheek, total nose, both upper and lower lids, ocular globe, and temporoparietal region. Tumor resection and reconstructive aspect were planned in 3 stages. (1) Excision of the tumor located in the cheek, nose, and both upper and lower left lids. The reconstruction of the cheek was made with a total forehead flap. The nose was resurfaced by means of a total skin graft. (2) resection of the temporoparietal nevus. (3) Treatment of the tumor involving the ocular structures. Total forehead flap was outlined, permitting carryout of skin of the total forehead area, and the blood supply was originated from the frontal and parietal branches of the temporal superficial artery and secondarily by anastomosis with the branches arising from both homolateral auricular and occipital vessels. Postoperative control 1 year after surgery showed an excellent aesthetic and functional result not only in the recipient area but also in the donor area.

Summary Introduction: Giant congenital melanocytic naevi (GCMN) are uncommon, have a significant morbidity and require extensive treatment. This paper presents results after complete excision of GCMN on the scalp, forehead or periorbita after early tissue expansion. Based on 15 years of experience, we want to show that performing tissue expansion at a young age is advisable., Patients and Methods: We included 17 consecutive patients in whom 38 tissue expanders were used. Early and late complications were noted. Patients were seen for a clinical follow up in which scars and re-pigmentation were evaluated with a validated scar scale (POSAS)., Results: All GCMN could be excised completely with early tissue expansion. The age at treatment ranged from 4 months to 2 years of age. With a mean follow-up period of 8.7 years, mild re-pigmentation was seen in only three patients and none of the patients developed a malignant melanoma. Complication rates are comparable with the literature., Conclusion: Tissue expansion is a good method for removing GCMN located at the scalp or face with good cosmetic and oncological results. Performing tissue expansion at a young age is advisable.

Malignant melanoma in children is a rare and poorly understood pathology. We report a case of nodular melanoma that developed on congenital naevus in a 6-month-old infant. The histological results revealed a nodular melanoma on a congenital naevus measuring 6.625 mm in tumour thickness according to Breslow. The infant was treated by broad resection without adjuvant treatment. Follow up is 43 months without metastasis. Malignant melanoma is a rare pathology: 1-4% of all melanomas occur before the age of 20 and 0.3-0.4% of those are before puberty. The risk of degeneration of a congenital naevus into a melanoma is approximately 0.7%. Surgical exeresis must be broad. Up to now, no complementary treatment has proven to be effective. Pre-operative examination for sentinel lymph nodes by lymphography can be of interest although such an examination is difficult in children. The prognosis would appear to be similar to that of malignant melanoma in adults with a high mortality. This is therefore an argument in favour of early treatment and prolonged follow up of children with malignant melanoma.

Introduction: Dermatofibrosarcoma show an extremely aggressive tendency to invade surrounding tissue. It was first described in 1924. It usually occurs in young men. This type of tumor is exceptional in childhood. The authors report a case of congenital dermatofibrosarcoma diagnosed in a child., Report of Case: A two-year old female patient presented with a tumor of the vertex scalp since her birth. Biopsy revealed a dermatofibrosarcoma. The tumor was removed surgically with 3cm margins. The primary reconstruction was performed using a double temporoparietal flap (H). There was no recurrence at five years of follow-up., Discussion: Congenital dermatofibrosarcoma is very rare. Only twenty cases have been reported.

We report a rare congenital scalp tumour with histological diagnosis of a congenital apocrine adenoma with features of a tubular adenoma. Following cranial CT and MRI, the tumour was serially excised. The macroscopic and microscopic appearances and management are discussed. To our knowledge, such a case has not been previously reported.

Background: Large hemangiomas of the scalp, though uncommon, present unique challenges to the reconstructive surgeon. If not treated early, these lesions can result in large areas of alopecia, distortion of the hairline, or deformation of the ear. Given these potential complications and the relative pliability and redundancy of the infant scalp before 4 months of age, the authors propose early surgical excision., Methods: A retrospective review of the senior author's (B.M.Z.) patient records was performed; over a period of 4 years, six infants were identified who underwent resection of a large scalp hemangioma. The surgical planning and execution of each case and follow-up are detailed., Results: All six hemangiomas were excised completely. In five cases, the excisions were performed in one stage at or before 4 months of age. In a sixth case, a tissue expander was placed before excision and closure in an 18-month-old infant. In three cases, significant ear malposition was corrected by removal of the deforming mass. There were no complications., Conclusions: The authors have demonstrated that by taking advantage of the greater elasticity of the infant scalp, large hemangiomas of the scalp can be aggressively and successfully treated with surgical intervention, often in one operation. Beyond the usual indications, early surgical excision of scalp hemangiomas may be advantageous and warranted to prevent the development of large alopecic areas or the permanent distortion of the hairline and aural anatomy.

Poliosis is a localized patch of gray or white hair. Because it can be seen with a variety of disorders and drugs, a full history and exam is indicated. Additionally, it can be associated with underlying benign and malignant tumors, necessitating histological identification. We review the lesions that are reported with poliosis. In addition, we will report a case of poliosis overlying an intradermal nevus with congenital as well as blue nevus features. To the best of our knowledge, blue nevus features associated with poliosis have not been previously described.

Objectives: To describe the dermoscopic features of congenital melanocytic nevi (CMN) and assess whether predominant dermoscopic patterns present in CMN are related to an individual's age (<12 years vs >or=12 years), sex, or lesional site (head, neck, and trunk vs extremities)., Design: Nonrandomized observational study., Patients: A total of 77 consecutive patients, each with 1 CMN (n = 77 lesions), from an outpatient dermatology clinic. A diagnosis of CMN was established by (1) documentation of a melanocytic nevus during the first year of life or (2) by clinical examination and either clinical history or biopsy findings., Main Outcome Measures: Images of CMN were evaluated for specific dermoscopic structures and patterns. The distribution of patterns was assessed by age, sex, and lesional site., Results: Most of the 77 lesions exhibited 1 of the following predominant dermoscopic patterns: reticular (18 lesions [23%]), globular (14 [18%]), or reticuloglobular (12 [16%]). Globular CMN were present in 5 of the 19 individuals who were younger than 12 years (26%) but in only 9 of the 58 individuals 12 years or older (16%). Reticular CMN were seen exclusively in the individuals who were 12 years or older. Congenital melanocytic nevi exhibiting no predominant pattern were more commonly present in the individuals younger than 12 years. Globular CMN were present in 11 head, neck, and trunk lesions (30%) compared with 3 extremity lesions (8%). Conversely, reticular CMN were present in 16 extremity lesions (40%) compared with 2 head, neck, and trunk lesions (5%). The predominant dermoscopic pattern did not vary based on sex. The most commonly observed dermoscopic structures were globules (in 64 lesions [83%]), hypertrichosis (in 61 [79%]), and reticular networks (in 55 [71%])., Conclusions: Our results suggest that the predominant dermoscopic patterns of CMN vary according to age and lesional site. These differences may inform future studies on the pathogenesis of CMN.

Background: Active nonintervention remains the mainstay of therapy for most uncomplicated infantile hemangiomas (IH) because of their expected involution. Topical imiquimod, with its ability to induce the production of interferon, tumor necrosis factor-alpha, and the antiangiogenesis factor tissue inhibitor of matrix metalloproteinase, has been recently reported to be efficacious in the treatment of IH., Objective: We sought to evaluate the efficacy of imiquimod 5% cream in the treatment of noncomplicated IH and possible side effects., Methods: A retrospective chart review analysis was performed in 18 children (16 girls and 2 boys) with a median age of 18 weeks (range: 4-256 weeks). A total of 22 hemangiomas (14 located on head, 3 on genitalia, 2 on trunk, and 3 on extremities) were treated with imiquimod 5% cream. Imiquimod was applied 3 times weekly in 10 patients and 5 times weekly in 8 patients for a mean duration of 17 weeks (7-46 weeks)., Results: All superficial IH improved, and remission was achieved in 4 hemangiomas. There was little improvement in mixed IH with no or minimal change in all deep hemangiomas. One case with ulcerated hemangioma substantially improved with accelerated ulcer healing and hemangioma size reduction. No systemic complication was observed in any of our patients, with irritation and crusting being the most common reactive effects., Limitations: The small-sample, retrospective study limits the interpretation of results., Conclusion: Imiquimod 5% cream may be most effective in superficial IH. There was no significant correlation between response and early onset of treatment for any IH in our small sample study. Pharmacokinetic analysis and placebo-controlled study should follow to ascertain the safety and efficacy of imiquimod 5% cream in the pediatric age group.

A 1-year-6-month-old girl presented with a subcutaneous tumor of the forehead, which had developed since birth. The preoperative examinations showed nasofrontal bone defect with meningocele and subcutaneous tumor with cyst. The patient underwent excision of the tumor and reconstruction of the bone defect. Histologically, the skin tumor exhibited disordered arrangement of striated muscle fibers among normal dermal components, and the cyst was lined by cornified epithelium with a few hair adnexa and contained lamellated keratin. These findings were consistent with rhabdomyomatous mesenchymal hamartoma (RMH) and dermoid cyst. This is an interesting case of RMH co-existing with nasofrontal meningocele and dermoid cyst in the same area. We suggest embryologic errors as a possible etiology, which is incomplete dysjunction of the neural ectoderm from the cutaneous ectoderm. Failure of insertion of mesoderm between the ectoderms caused the bone defect and the disordered proliferation and differentiation of mesoderm-derived tissue, leading to formation of hamartoma.

Objective: Extracranial subcutaneous masses involving the scalp and/or skull in young children are uncommon lesions that get excised by the neurosurgeon. Although the most common reported lesion is the dermoid cyst, our experience suggests that the spectrum of pathology in these lesions can present diagnostic challenges to the pathologist., Material: We reviewed 30 consecutive extracranial masses from 29 patients between July 1998 and June 2003., Method: Hematoxylin and eosin-stained sections were reviewed in all cases, and immunohistochemistry was performed in select cases., Results: Twenty-three were within the scalp, 5 involved the scalp and skull and 2 were within the limits of the inner and outer tables of the skull. There were 8 dermoid cysts, 2 epidermoid cysts, 6 post-traumatic lesions including 3 calcified cephalhematomas and 3 pseudocysts, 5 vascular lesions including 3 capillary hemangiomas, 1 venous angioma and 1 lymphangioma, 2 cases of cranial fasciitis and 1 case each of benign teratoma, deep granuloma annulare, benign fibrous histiocytoma, congenital melanocytic nevus, hamartoma with ectopic meningothelial elements, cutaneous hyalinised ectopic meningioma and a meningocele with a fibrohistiocytic reaction. No lesions have recurred or exhibited malignant features., Conclusions: Surgical pathologists and neuropathologists should be aware that the differential diagnosis of "lumps and bumps on babie's heads" is quite varied and can be histologically challenging.

The differentiation between atypical variants of Spitz nevus and melanoma is often difficult given the many clinical and histopathologic similarities between the two. We report a case of an infant with a congenital scalp lesion exhibiting clinical features of melanoma, including variegation and regression of pigmentation and a rapidly changing appearance. Histologic examination of the excised lesion revealed a benign congenital Spitz nevus. This case emphasizes the need for clinical and histologic correlation in determining the benign or malignant nature of atypical pigmented lesions in infants.

We report a neonate with a giant congenital naevus on the scalp, who was treated with the erbium:YAG laser when she was 9 days old. This treatment option proved to be a valuable alternative approach to this problem.

A 26 year old man presented with a giant cerebriform nevus on the occiput. Clinical appearance of the nevus, maceration within the folding and risk of malignancy prompted us to perform a complete excision. The scalp defect was closed with a rotation flap following an implantation of a tissue expander.

A variety of treatment options exists for the management of giant congenital nevi. Confusion over appropriate management is compounded because not all giant congenital nevi are pigmented, and malignant potential varies between different types. The present study sought to define factors in the presentation of giant congenital nevi that could provide an algorithm for their management, with respect to both the extent of resection and subsequent reconstructive options.A retrospective review of all patients who presented with a congenital nevus of 20 cm2 or greater since 1980 was performed, distinguishing among nevi involving the head and neck, the torso, and the extremities. Sixty-one patients with giant congenital nevi were evaluated (newborn to age 16 years), of which 60 nevi in 55 patients have been operated on. Giant congenital nevi having malignant potential were pigmented nevi (53 patients) and nevus sebaceus (four patients). Those not having malignant potential were verrucous epidermal nevi (three patients) and a woolly hair nevus (one patient). Of the 60 giant congenital nevi operated on, expanded flaps were used in 25, expanded full-thickness skin grafts were used in 10, split-thickness or nonexpanded full-thickness skin grafts were used in 13, and serial excision was used in 30. After 1989, operations tended to use multimodality treatment plans, with an increased use of expanded full-thickness grafts and immediate serial tissue expansion. The use of serial excision, particularly in the extremities, also increased after 1989. Serial excision was the treatment of choice when it could be completed in two procedures or less, which occurred in more than 80 percent of cases using serial excision alone. Expanded flaps were the most common mode of reconstruction in the head and neck region and were used in 49 percent of these procedures. Serial excision was the most common form of treatment in the extremities, used in 50 percent of procedures. Tissue expansion in the extremities was infrequently used to provide an expanded flap (8 percent of procedures), whereas it was frequently used to provide expanded full-thickness skin grafts harvested from the torso (used in 31 percent of procedures). On the basis of these data, algorithms for the extent of resection and subsequent reconstructive options for giant congenital nevi were developed. Their management should be formulated relative to pigmentation, malignant potential, and anatomic location of the respective lesions.

The authors present a cohort of 21 consecutive patients who had congenital pigmented nevi covering 15 to 65 percent of the forehead and adjacent scalp and who were treated at their institution within the last 12 years. All patients were treated with an expansion of the adjacent texture- and color-matched skin as the primary modality of treatment. The median age at presentation was approximately 1 year; mean postoperative follow-up was 4 years. Nevi were classified according to the predominant anatomic areas they occupied (temporal, hemiforehead, and midforehead/central); some of the lesions involved more than one aesthetic subunit. The authors propose the following guidelines: (1) Midforehead nevi are best treated using an expansion of bilateral normal forehead segments and advancement of the flaps medially, with scars placed along the brow and at or posterior to the hairline. (2) Hemiforehead nevi often require serial expansion of the uninvolved half of the forehead to minimize the need for a back-cut to release the advancing flap. (3) Nevi of the supraorbital and temporal forehead are preferentially treated with a transposition of a portion of the expanded normal skin medial to the nevus. (4) When the temporal scalp is minimally involved with nevus, the parietal scalp can be expanded and advanced to create the new hairline. When the temporoparietal scalp is also involved with nevus, a transposition flap (actually a combined advancement and transposition flap because the base of the pedicle moves forward as well) provides the optimal hair direction for the temporal hairline and allows significantly greater movement of the expanded flap, thereby minimizing the need for serial expansion. (5) Once the brow is significantly elevated on either the ipsilateral or contralateral side from the reconstruction, it can only be returned to the preoperative position with the interposition of additional, non-hair-bearing forehead skin. Expansion of the deficient area alone will not reliably lower the brow once a skin deficiency exists. (6) In general, one should always use the largest expander possible beneath the uninvolved forehead skin, occasionally even carrying the expander under the lesion. Expanders are often overexpanded.

A newborn girl with severe diffuse neonatal haemangiomatosis is described. She was treated with high dose systemic corticosteroids and high dose interferon-alpha-2a, but with fatal outcome. A review of the current literature is presented.