Cutaneous Ganglioneuroma Associated With Overlying Hyperkeratotic Epidermal Changes: A Report of 2 Cases

Abstract

The occurrence of primary cutaneous ganglioneuroma is rare. We report 2 separate cases of primary cutaneous ganglioneuroma, both of which are associated with prominent overlying hyperkeratosis. The first case was in a 38-year-old woman with overlying verrucous keratosis. The second case was in a 93-year-old man with epidermal changes reminiscent of a seborrheic keratosis. Histologically, both lesions were composed of a proliferation of hyperplastic nerve fibers with spindled Schwann cells and axons with intermingled ganglion cells. Immunohistochemistry for neurofilament highlighted nerve fascicles; S100 protein displayed the associated Schwann cells, and neuron-specific enolase stained the interspersed ganglion cells. Variation in immunohistochemical staining was present between the 2 cases. A review of the literature demonstrates variable immunohistochemical staining of ganglion and Schwann cells in prior cases. Familiarity with these findings is important in establishing a diagnosis. The significance of the associated hyperkeratosis remains speculative.

Ganglioneuroma of the uterine cervix—a case report

“…Although ganglioneuroma is mostly a well-circumscribed mass, our case was not a circumscribed mass. Some ganglioneuroma in skin and colon were not a circumscribed mass [4,9]. It is possible that ganglioneuroma, especially in the choristomatous lesion, cannot form a circumscribed mass.…”

“…Similarly, ganglion cells are not normal components of the skin, but cutaneous ganglioneuroma may arise from immature precursors derived from neural crest, or they may be choristomas [9]. Glial polyps of the cervix or glial tissues in the cervix can exist [10,11] as a polypoid lesion composed of finely eosinophilic tissue with fully differentiated astrocytic glial tissue.…”

Primary cutaneous ganglioneuroma: anatomico‐clinical study of 4 cases with focus on Merkel cells

“…Adrenal GN have also been reported in the context of MEN 2A and MEN 2B, in addition to rare reports of MEN 2 in association with composite pheochromocytoma/GN . Primary cutaneous GN are extremely rare, as only 18 cases have been reported in the English literature to date, 16 of which are clear‐cut GN (Table ) …”

“…Primary GN is exceptional. Since the first observation in 1972 by Collins et al, only 16 cases have been reported in the English literature to date . Most of these cases show epidermal changes above the lesion.…”

Ganglioneuroma coincidente con una extrusión discal lumbar

“…En ocasiones pueden secretar polipéptidos intestinalmente vasoactivos, induciendo entonces un síndrome caracterizado por diarrea, hipopotasemia y aclorhidria 28 , o testosterona, lo que conlleva virilizaciones 7 . Se han descrito ubicaciones en cualquier zona del organismo: supratentoriales 3 , cerebelosas 15 , raquídeas 4,8,10,12,13,24 , mediastínicas 27 , retroperitoneales 22 , intestinales 11 o cutáneas 6 . Cuando su localización es cercana a la medula espinal es frecuente la compresión de la misma o la tracción radicular, pudiendo causar dolor, pér-dida de fuerza o trastornos sensoriales en dependencia del nivel mielo-radicular afecto.…”