3,920 results match your criteria: "Congenital Nevi"

Inferior vena cava aneurysms (IVCAs) are rare yet potentially lethal, especially if they are symptomatic or complicated. Among the IVCAs reported in the literature, only a few are associated with congenital vascular anomalies, including congenital IVC obstruction, tetralogy of Fallot, left-sided IVC, duplicated IVC, Ehlers-Danlos syndrome, blue rubber bleb nevus syndrome, and Klipper-Trenaunay syndrome. We present the case of an 8-cm symptomatic saccular IVCA in a patient with tetralogy of Fallot, treated successfully with surgical repair.

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Blue rubber blister nevus syndrome: A case report.

World J Gastrointest Surg

November 2024

Department of Critical Care Medicine, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou 450003, Henan Province, China.

Background: Blue rubber blister nevus syndrome (BRBNS) is a congenital, rare disease characterized by venous malformations of the skin and internal organs, affecting all systems throughout the body. The pathogenesis is unknown. There is no consensus on the treatment of BRBNS.

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BACKGROUND Optic disc melanocytoma (magnocellular nevus of the optic disc) is a benign congenital pigmented tumor, usually unilateral, asymptomatic, and mostly discovered in routine examinations in adult patients; however, it is associated with choroidal neovascularization. It can be difficult to differentiate it from juxta-papillary choroidal melanoma. Aflibercept is a monoclonal antibody targeting vascular endothelial growth factor A (anti-VEGF-A).

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[Transformation of a giant congenital nevus in an 87-year-old female patient].

Dermatologie (Heidelb)

December 2024

Department of Dermatology, Venereology and Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Deutschland.

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Therapeutic Strategies in Neurocutaneous Melanocytosis.

Anticancer Res

December 2024

Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, U.S.A.

Article Synopsis
  • * The condition is usually fatal if symptoms appear, with a high chance of the brain lesions becoming malignant, especially in those with larger skin nevi.
  • * Current treatments focus on palliative care and aim to manage symptoms, as there is no cure, highlighting the need for ongoing research to develop effective therapies and address this critical medical gap.
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Article Synopsis
  • * Misdiagnosis (both under and over) of melanomas occurs frequently because benign lesions can mimic melanoma characteristics.
  • * Accurate diagnosis relies on strong clinico-pathological correlation, detailed cytological analysis, and appropriate use of immunohistochemistry to prevent harmful misdiagnoses.
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[Spontaneous onset of a white papule on a congenital nevus cell nevus].

Dermatologie (Heidelb)

November 2024

Abteilung für Dermatologie, Wiener Gesundheitsverbund Klinik Hietzing, Wolkersbergenstr. 1, 1130, Wien, Österreich.

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Bean-Syndrome in Maxillofacial District.

Indian J Otolaryngol Head Neck Surg

December 2024

Department of Biomedical and Dental Sciences and of Morphological and Functional Images, University of Messina, Messina, Italy.

Bean syndrome (BS) is a rare congenital angiomatosis affecting multiple organ systems. The exact etiology is still not well-known. About 200 cases with heterogeneous clinical presentations have been reported worldwide.

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The Pseudoinflammatory Pattern Revisited.

J Cutan Pathol

November 2024

Section of Dermatology, The University of Chicago Medicine, Chicago, Illinois, USA.

In 1973, Dr. Martin C. Mihm, Jr.

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Article Synopsis
  • Giant congenital melanocytic nevi (GCMN) are larger than 40 cm and can lead to serious health issues, including risks of melanoma and neurocutaneous melanocytosis (NCM), which can affect the central nervous system (CNS).
  • MRI is advised for GCMN patients, especially those with multiple nevi or neurological symptoms, to check for CNS involvement and potential complications.
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  • Cutaneous neurocristic hamartoma (CNH) is a rare skin lesion linked to unusual neural crest cell development, often mistaken for other conditions like giant congenital nevus.
  • The study investigates gene expression differences between CNH and proliferative nodules (PNs) derived from it, comparing these with giant congenital nevus and malignant melanoma.
  • Key findings indicate that lower levels of the tumor suppressor gene WIF1 in PNs might contribute to the growth of these nodules, while higher expression of IGF2 and H19 in CNH and PNs points to potential abnormal gene activity involved in CNH’s development.
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Giant congenital pigmented nevi constitute an extremely diverse group of skin lesions with varying morphologies. These nevi are often associated with many clinical implications, such as increased risk of melanoma and the presence of neurocutaneous melanosis, with melanoma being the primary concern. We present a rare case of a 62-year-old patient with a giant congenital birthmark who reported to the oncology department due to a tumor in the lower abdomen detected during an ultrasound examination.

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Spinal Neurocutaneous Melanosis.

World Neurosurg

November 2024

Complexo Hospital de Clínicas, Universidade Federal do Parana, Curitiba, State of Paraná, Brazil; Department of Pediatrics, Complexo Hospital Pequeno Principe, Curitiba, State of Paraná, Brazil.

Neurocutaneous melanosis is characterized by melanin-producing cells within the skin, leptomeninges, or brain parenchyma. It is a severe manifestation of congenital melanocytic nevus syndrome and can develop malignant melanomas. The disorder is commonly present in pediatric patients within the first two years of life and has a poor prognosis.

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Eccrine Porocarcinoma in Linear Epidermal Nevus.

Clin Cosmet Investig Dermatol

October 2024

Department of Dermatology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, People's Republic of China.

Article Synopsis
  • Linear epidermal nevus is a type of skin disorder that is typically present at birth or develops during childhood.
  • It involves abnormal skin growths that resemble moles or patches.
  • The case presented discusses a rare occurrence where this skin condition was followed by a serious skin cancer called eccrine porocarcinoma (EPC).
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Article Synopsis
  • Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare congenital condition characterized by vascular malformations in the skin and gastrointestinal tract, but it lacks extensive research and descriptive data.
  • A multicenter study in Europe analyzed 44 BRBNS patients, revealing that the syndrome is typically diagnosed around age 12, primarily through clinical symptoms, with a significant number also experiencing gastrointestinal complications.
  • The study suggests that tests for d-dimer, fibrinogen levels, and Tie2/TEK mutations should be conducted when BRBNS is suspected, as they could aid in confirming the diagnosis.
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Pediatric melanomas are rare and some of them may arise on giant congenital melanocytic nevi. The risk of developing melanoma on a medium-sized nevus is not clear but is thought to be very rare. Proliferative cellular nodules which mimic malignant melanoma may pose significant diagnostic challenges.

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Congenital melanocytic nevus syndrome: An association between congenital melanocytic nevi and neurological abnormalities.

Semin Pediatr Neurol

October 2024

Department of Pediatrics, Division of Pediatric Dermatology, Dell Medical School, University of Texas, 1301 Barbara Jordan Blvd, Suite 200A, Austin, TX 78723, United States. Electronic address:

Article Synopsis
  • Congenital melanocytic nevus syndrome is characterized by the presence of congenital melanocytic nevi (CMN) along with various neurosystem abnormalities, primarily due to mutations in the NRAS gene.
  • CMN appears as collections of melanocytes on the skin and can be classified by size and various clinical features like location and color, while neurological issues might include seizures and developmental delays.
  • Early MRI screening of the central nervous system in infants is essential for assessing potential neurological risks, and while some outcomes are favorable with normal MRI results, severe complications can arise if melanoma develops.
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Article Synopsis
  • A newborn girl was referred for assessment of a large pigmented lesion on her lower back, accompanied by several smaller lesions.
  • Two biopsies revealed characteristics of a congenital nevus, along with some unique spindled dendritic cells that did not show signs of melanocyte positivity.
  • The findings indicate the presence of a congenital nevus with features similar to a neurocristic hamartoma, emphasizing the rarity of this condition and the need for early diagnosis and monitoring.
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Although melanocytic nevi have a relatively uneventful course throughout their existence, some may develop an inflammatory reaction known as the Meyerson phenomenon. Initially, the Meyerson phenomenon has been exclusively described in melanocytic nevi. However, it has since been observed in both pigmented and non-pigmented lesions, thus expanding the description from Meyerson nevus to the phenomenon.

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