Melanotic Neuroectodermal Tumor of Infancy Presenting with Fast Growing Scrotal Swelling: A Case Report and Literature Review

Abstract

Testicular melanotic neuroectodermal tumor of infancy (MNTI) is extremely rare, with 2 cases reported in the literature. Its rarity, rapid and infiltrative growth pattern pose a diagnostic challenge. A previously healthy 3-month old male, presented with a history of worsening left hemiscrotal swelling for one week. An outside ultrasound was suggestive of testicular torsion. Left orchiectomy demonstrated a mass occupying almost entire testicle with a variegated cut surface, with areas of pigmentation, necrosis and hemorrhage. Histological examination confirmed MNTI of the testis and epididymis. MNTI should be included in differential diagnosis in infants presenting with fast growing scrotal swelling.

Melanotic Neuroectodermal Tumor of Infancy with a Negative VMA: A Case Report and Review of the Presentation, Etiology, and Management

“…7 Around 90% of cases occur in the head and neck region, while rare instances have been reported in other areas, such as the mediastinum, long bones of the extremities, and genital region. 2 There are different opinions regarding the origin of this rare tumor. However, the consensus favors a neuroectodermal origin as the root cause.…”

“…The tumor typically involves the maxilla, however, other sites, such as the skull, mandible, cranial vault, and the extracranial of the mediastinum, long bones of the extremities, and, rarely, the genital organs, have been reported. 2,3 Most cases occur in the first year of life and presents as a solitary, rapidly growing, expansile mass. 4 Like the neuroblastoma, it is suspected that MNTI has a neural crest cell origin.…”

Sex cord stromal tumors and tumors of the paratestis: new and old entities in a landscape of rare tumors

“…Difficulties for differential diagnosis are also due to nonspecific patient age. In fact, paratesticular tumors arise mainly in adult age, with the exception of melanotic neuroectodermal tumor which characteristically occurs in the first month of life and up the age of 8 years [19,20].…”

Atypical scrotal masses: Surgical surprise

“…2 Follow-up is recommended in view of chances of potential recurrence and rare malignant transformation. 3 Recurrence is reported from 10 days to 2 years. 4 Scrotal lymphangioma is very rare and is mostly misdiagnosed as a hydrocele, haematocele, or spermatocele.…”