Cutaneous Ganglioneuroma Research Articles

Ganglioneuroma Arising in Congenital Melanocytic Nevus in a Patient with Cardiac Anomalies: A Case Report.

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. Out of approximately 250 case reports on congenital melanocytic nevus, we identified only two reports of medium/large congenital melanocytic nevus with cutaneous ganglioneuroma. Due to the potential malignant transformation of congenital melanocytic nevus, reporting the features and characteristics of such rare findings may help in further understanding congenital melanocytic nevus, its associations, and prognosis.

Ganglion cell like cells, diagnostic dilemma

We report a case of cutaneous swelling found on the left anterior axillary fold of a 41-year-old man. Gross examination of specimen excised from the dermis showed a well-circumscribed nodule histologically composed of spindle cells with interspersed ganglion cell like cells. On hematoxylin and eosine (H and E) staining it was diagnosed as ganglioneuroma. Ganglioneuromas are rare, benign, fully differentiated tumors that contain mature schwann cells, ganglion cells, fibrous tissue, and nerve fibers. They are commonly found along the paravertebral sympathetic ganglia and sometimes in the adrenal medulla. However primary cutaneous ganglioneuroma is an extremely rare tumor. Immunohistochemical workup revealed a fibroblastic origin and hence the case was diagnosed as fibromatosis with ganglion cell like fibroblasts. This case report suggests that the features considered diagnostic of ganglioneuromas can occur in other cutaneous lesions and, therefore, this diagnosis cannot be offered only on the basis of H and E.

Acquired Primary Cutaneous Ganglioneuroma With Adipocytic Metaplasia: “An End Point of Melanocytic ‘Maturation’?” A Case Report and Meta-Analysis With Comparison to a Cross-Sectional Study of Neurotized Melanocytic Nevi

Adipocytic metaplasia is frequently exhibited by intradermal melanocytic nevi, often in conjunction with melanocytic neurotization, a process termed maturation where melanocytes are assumed to transform to a peripheral nerve phenotype. To present the characteristics of a case of primary cutaneous ganglioneuroma (CGN) with adipocytic metaplasia and compare reported cases of primary acquired CGN with a cohort of neurotized melanocytic nevi (NMN). We report a case of primary acquired CGN that presented as an asymptomatic, 1-cm, flesh-colored papule on the thigh of a healthy 75-year-old woman. An excisional specimen revealed an intradermal tumor with a dome-shaped profile formed by an intermixed proliferation of ganglion cells, Schwann cells, and numerous adipocytes. Schwann and ganglion cells expressed S100 protein, S100A6, and glial fibrillary acidic protein. Ganglion cells and axonal elements expressed c-kit, synaptophysin, neurofilament, CD56, and neuron-specific enolase. Rare small tumor cells with scant cytoplasm weakly expressed microphthalmia transcription factor protein (Mitf). Similar to NMN, CGN affected the same age group, commonly occurred on the trunk, showed neuromatous differentiation, and in a minority, exhibited adipocytic metaplasia. In contrast, CGNs were significantly larger tumors with more frequent coexisting epidermal changes or desmoplasia. No cases of NMN had authentic ganglion cells, but a minority had ganglion-like cells, which weakly expressed the neural tissue markers c-kit, neuron-specific enolase, CD56, and glial fibrillary acidic protein. A few small Mitf+ cells were found in neuromatous areas and nevic corpuscles of NMN. CGN and NMN are neural crest stem cell-derived tumors that exhibit overlapping and unique phenotypic traits. Adipocytic and neuromatous metaplasia in melanocytic nevi is considered as a consequence of "maturation." Although transformation of an intradermal melanocytic nevus to CGN is a theoretical possibility, the multiple coexisting phenotypes they display most likely arose ab initio in the dermis, mirroring the multiple pathways of differentiation possible for neural crest stem cells. The stage of differentiation of the precursor (stem) cell and interaction with environmental influences most likely predict the final phenotype(s), a pathogenic scheme that better explains the phenomenon of NMN rather than transformation of differentiated melanocyte into a peripheral nerve sheath cell.

Cutaneous ganglioneuroma in a cattle (case report)

Cutaneous ganglioneuroma is a rare neoplasm in the veterinary literature. To date, only one case has been reported in a dog in the veterinary literature. This article describes histopathological and immunohistochemical characteristics of cutaneous ganglioneuroma in the skin of the gluteal region in a cattle. The tumor mass was composed of mature ganglion cells interspersed between spindle-shaped cells consistent with Schwann cells. With immunohistochemistry, the tumor cells were positive for neuron-specific enolase, synaptophysin (SYP), and S100 protein. Therefore, cutaneous ganglioneuroma was diagnosed. To the best of our knowledge, this is the first report of cutaneous ganglioneuroma in cattle.

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

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.

Cutaneous ganglioneuroma

Primary cutaneous ganglioneuroma is an extremely rare tumor. We report a case of solitary cutaneous ganglioneuroma on the back of a 75-year-old woman.

Primary cutaneous ganglioneuroma arising within a seborrheic keratosis: a rare association

A 75-year-old woman presented to her general practitioner with a keratotic skin lesion on her abdomen. Microscopic examination showed an irritated seborrheic keratosis. However, further inspection revealed a well-defined dome-shaped area in the dermis showing a number of small nerve fibres admixed with relatively few mature ganglion cells. Both the spindle cell component and the ganglion cells stained positively for S-100 protein, neurofilament protein and CD56 (Leu-19, NCAM) by routine immunohistochemistry. Primary cutaneous ganglioneuroma is an exceedingly rare benign tumour of the skin with only 13 cases having been reported in English literature. We describe the second case of primary cutaneous ganglioneuroma associated with seborrheic keratosis.

Cutaneous Ganglioneuroma Within a Giant Congenital Nevus

Cutaneous ganglioneuroma has only occasionally been reported in the literature. Cutaneous ganglioneuroma is seen even more rarely in association with a giant congenital nevus. Differential diagnosis includes malignancies, especially melanoma and metastatic neuroblastoma. It is essential to rule out malignancy in a solid lesion within a congenital nevus. The present report is possibly the first relating ganglioneuroma and a congenital nevus to a cervical mass detected during routine prenatal ultrasound.

Primary congenital cutaneous ganglioneuroma

Cutaneous ganglioneuroma is rare. Only five cases have been reported, and in all patients the lesions developed after birth. We describe a congenital cutaneous ganglioneuroma. The differential diagnosis includes well-differentiated cutaneous metastases of neuroblastoma and ganglion cells entrapped by a neurofibroma in patients with neurofibromatosis.

Cutaneous ganglioneuromas: A case report and review of the literature

The occurrence of ganglioneuromas outside the sympathetic chains of the mediastinum and abdomen in the nonpediatric age group is rare. We report the case of a solitary cutaneous ganglioneuroma on the abdomen of a 52-year-old woman. The lesion, which had been present for 20 years, measured 1.2 cm in greatest dimension. Unmyelinated axons, Schwann cells, and scattered mature ganglion cells were identified by histochemistry, immunohistochemistry, and electron microscopy. Only three similar cases have been reported in the literature to date. Ganglioneuromas have also been reported as mature components of metastatic neuroblastomas and in association with plexiform neurofibromas, neither of which was present in this case. The pathogenesis of this lesion is unclear; however, aberrant migration of neural crest elements is the most likely explanation. The possible trophic role of sex steroids is also discussed. Although no follow-up series exist, local excision should be curative considering the small size and histologically benign appearance of the lesion.

Cutaneous ganglion cell choristoma

We describe the histological, immunohistochemical, and ultrastructural findings of a cutaneous tumour composed of ganglion cells, without any other proliferating component. As ganglion cells are not normal components of the skin, we propose the term "ganglion cell choristoma" for this lesion. The differential diagnosis of related lesions such as cutaneous ganglioneuroma, well-differentiated metastases from neuroblastoma, autonomic ganglia entrapped by neurofibroma, and reactive processes, and the possible histogenesis of ganglion cell choristoma are discussed.