Melanotic Neuroectodermal Tumor of Infancy Disseminated by a Ventriculoperitoneal Shunt and Diagnosed from the Inguinal Sac

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare congenital neoplasm that originates from the neural crest cells, which give rise to the melanocytes of the skin and leptomeninges. We report a case of MNTI with neurocutaneous melanosis of a 28-month-old girl. She was born with hydrocephalus and several large congenital giant nevi. There were no findings except for hydrocephalus, after a ventriculoperitoneal (VP) shunt operation performed when she was 6 months old. She was operated on for a growing inguinal mass at 8 months. The specimen from the inguinal sac was positive for HMB45, vimentin, chromogranin, and neuron-specific enolase. Brain magnetic resonance imaging showed an extensive enhancing extra-axial mass with high signal intensity, along the cerebral spinal fluid space. We report a rare case of MNTI, diagnosed from an inguinal hernia sac, with a disseminated clinical manifestation.

Widespread intra-abdominal carcinomatosis from a rhabdoid meningioma after placement of a ventriculoperitoneal shunt: A case report and review of the literature

“…Thirty-eight articles with a total of 43 patients were reviewed [ Table 1 ]. [ 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 ] The mean age of patients was 15 years and 3 months, with 69.8% (30 out of 43) of the patients in the pediatric age group (<18 years of age), and 67.4% male (29 out of 43). The overall predominant histological subtypes of primary CNS tumors with VPS-related metastasis were germinomas (11 out of 43, 25.6%) and medulloblastomas (5 out of 43, 11.6%).…”

Melanotic Neuroectodermal Tumor of Infancy: A Systematic Review

“…4 Although usually benign, this tumor can grow rapidly and infiltrate adjacent bone, soft tissue, and the orbit. In addition, a few cases of malignant MNTI have been reported in the literature, 8,[11][12][13][14][15][16] and the rate of metastasis is estimated to be approximately 3%. 17 Surgery has been recommended for treatment, although the necessary amount of margin clearance has not been clearly delineated.…”

“…This yielded a total of 69 publications, which included the 116 cases (Supplementary Table 1). 4,7,[12][13][14][15][16] In addition, data were collected on 356 additional cases that were reported in the literature from 1918 through 2004 from 3 major systematic reviews. 18,81,82 These cases were combined with the 116 cases published from January 2005 through December 2013 (Supplementary Table 1) to constitute a total of 472 cases for analysis.…”

Rare complication of ventriculoperitoneal shunt. Early onset of distal catheter migration into scrotum in an adult male: Case report and literature review

“…One of the rare sites of distal catheter migration is the scrotum. 32,46 Other reported sites of migration include the ventricle, 34 scalp/subgaleal space, 10 neck, 11 mouth, 30 breast, 31 breast implant, 52 thoracic cavity, 17 pulmonary artery, 35 intracardiac, 60 lungs/pleural space/trans-diaphragmatic, 7,25,50 anterior chest wall, 6 intra-abdominal wall, 39 abdominal subcutaneous fat tissue, 33 umbilicus, 23 stomach, 3 large intestine, 15 liver, 57 gall bladder, 41 bladder/urethra, 9 inguinal sac, 62 buttocks, 48 canal of Nuck, which is the female counterpart of the spermatic cord, 63 vulva/vagina, 55 rectum/anus, 54 and knee. 36 Although cases of scrotal migration of distal catheter have been reported in pediatric patients, cases in adult patients are rare due to obliterated processus vaginalis.…”