Ganglioneuromas (GNs) are benign tumors composed of ganglion cells in a Schwannian stroma. They are derived from neural crest cells that give rise to the sympathetic nervous system. Hence, GNs can be found anywhere a sympathetic ganglion is present. Most commonly, GNs are found in the posterior mediastinum and abdominal cavity. Within the abdominal cavity, they are most likely to be found in the retroperitoneal space or adrenal glands. Cutaneous involvement is uncommon and rarely reported in literature. We report an interesting case of a cutaneous ganglioneuroma on the abdomen of an 83-year-old male.
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http://dx.doi.org/10.1111/cup.13419 | DOI Listing |
BMC Med Genomics
July 2023
Imagerie Moléculaire Et StratégiesThéranostiques, Université Clermont Auvergne, INSERM, U1240, 63000, Clermont Ferrand, France.
Background: PTEN hamartoma syndrome (PHTS) is an autosomal dominant disorder characterized by pathogenic variants in the tumor suppressor gene phosphatase and tensin homolog (PTEN). It is associated with an increased risk of muco-cutaneous features, hamartomatous tumors, and cancers. Mosaicism has been found in a few cases of patients with de novo PHTS, identified from blood samples.
View Article and Find Full Text PDFInt J Surg Pathol
February 2024
College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
A congenital melanocytic nevus is a benign melanocyte proliferation, that may be complicated by malignant transformation. We are reporting a three-year-old girl, who had a giant congenital melanocytic nevus on her back, that was treated by serial surgical excisions with tissue expander insertion. Histopathological examination confirmed the diagnosis of congenital melanocytic nevus with ganglioneuroma.
View Article and Find Full Text PDFAm J Dermatopathol
November 2022
Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA.
Cutaneous ganglioneuromas (GNs) are exceptionally uncommon tumors, and many reported cases describe association with overlying epidermal hyperplasia that may be interpreted as seborrheic keratosis (SK) or SK-like proliferation. We report 5 cases of cutaneous GN in adult patients; all of which were discovered incidentally in the immediate vicinity of epidermal hyperplasia. A review of the literature demonstrates the current-although likely imperfect-understanding of the etiopathogenesis of both SK and GN in the skin.
View Article and Find Full Text PDFCase Rep Surg
July 2022
Shiraz Geriatric Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Ganglioneuroma is a benign tumor, originating from sympathetic nervous system. Intradural and dumbbell shape spinal ganglioneuroma has been reported in the literature. In this study, we intend to present our case, a 43-year-old man with multiple cutaneous dimples-probably a Neurofibromatosis type-1 (NF-1) case-and subacute myelopathy, who presented with bilateral symmetric dumbbell shape C2/C3 and C4/C5 intradural extramedullary tumors.
View Article and Find Full Text PDFIndian J Ophthalmol
July 2022
Department of Pediatric Ophthalmology and Pediatric Pathology, SRCC Children's Hospital Managed by NH- Mumbai, Maharashtra, India.
Neurofibromtosis-1 (NF-1) is the commonest oculo-neuro-cutaneous syndrome with multiple ocular manifestations. Reporting three children who presented with unilateral glaucoma (buphthalmos), ipsilateral facial hemihypertrophy, and eyelid plexiform neurofibroma: completing the triad of François syndrome, a rare NF1 variant. Two presented with leukocoria and were referred to as retinoblastoma suspects.
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