Congenital Glioblastoma multiforme and eruptive disseminated Spitz nevi

A male neonate was born at 38 weeks’ gestation by vaginal delivery with vacuum extraction. Pregnancy was uneventful. Birth weight was 3290 g. The baby at birth was pale, hypotonic, bradycardiac, without spontaneous breathing and was promptly intubated. The Apgar score was 3, 6 and 6 at 1st, 5th and 10th minute respectively. Three hours after birth the neonate was referred to a neonatal intensive care unit. Due to intrapartum asphyxia the neonate was treated with hypothermia for 72 hours. Brain ultrasound scan (day 1) showed bilateral severe intraventricular hemorrhage (grade 3), while magnetic resonance imaging (day 4) revealed hydrocephalus with an extensive haematoma in the frontal lobes. The morphology of the bleeding was atypical, therefore a brain tumor was suspected to be the source of bleeding. An increasing ventricular dilatation was observed in the following days, therefore on day 7 haematoma was drained, an intraventricular catheter with an external shunt was placed and multiple biopsies were performed. The postoperative course was uneventful with the improvement of the hydrocefalus. Two days after surgery the baby was extubated and enteral feeding was started. Basing on the diagnosis of congenital GBM, confirmed by histology, on day 16 the external shunt was replaced with a reservoir (Ommaya). After positioning a central venous catheter (Broviac type monolumen) in the right atrium, chemotherapy was started at age 18 days and the patient was treated under the two-phase AIEOP Infant High Risk-CNS tumor protocol. Doses were adjusted for weight. The four-course Phase A included: methotrexate 250 mg/kg plus vincristine 0.04 mg/kg; etoposide 80 mg/kg; cyclophosphamide 135 mg/kg plus vincristine 0.04 mg/kg; carboplatin 25 mg/kg. Peripheral blood stem cells (PBSC) were collected for rescue therapy. Phase B included two high-dose chemotherapy regimens plus PBSC support: carboplatin 50 mg/kg plus etoposide 50 mg/kg; thiotepa 10 mg/kg/day plus melphalan 4 mg/kg/day. Common toxicity criteria (CTC) were adopted. Elevated transaminase levels (grade III) were observed after high dose methotrexate. Grade III anaemia and grade IV thrombocytopenia developed after the second (etoposide) and third (cyclophosphamide and vincristine) courses. Granulocyte colony stimulating factors (G-CSF) were given from day 35 to day 38. At the end of treatment hematopoietic stem cells were collected. On day 40 the reservoir was replaced with a ventricle-peritoneal drainage. The postoperative course was uneventful. A second curse of chemotherapy was started on day 48. After completion of treatment the clinical course was uneventful. To date no neurological impairments have been observed: development status, as assessed by motor, cognitive and social adaptive skills on the Clinical, Linguistic and Auditory Milestones (CLAM) scale, showed no significant differences from the normal range. The patient was born without any nevus clinically evident. From the age of 1 year some nevi appeared, mainly on his trunk and back. Because of the progressive increase in number and dimension of the pigmented skin lesions the child was sent to our attention for a dermatologic exam at the age of 16 months. The nevi had light-brown to black colour, with variable morphology and dimension from 2 mm to 21 mm, manly located on the trunk. The dermoscopic exam of these lesions revealed multiple atypical nevi characterized by a starburst pattern, globular pattern or multicomponent pattern [3, 4] with asymmetrical hypo-pigmented area and irregular borders. These dermatoscopic features correspond to Spitz nevi [3, 4]. To confirm our diagnostic suspicious we also performed the reflectance confocal microscopy (RCM) (VivaScope 1500®: Caliber I.D., Rochester, USA), which revealed in the nevi with starburst pattern (Fig. 1) and globular pattern (Fig. 2) the typical dense regular nests at the dermoepidermal junction and in the papillary dermis, while in the epidermis showed regular cobblestone with rare spindled and atypical cells [4]. Instead, the multicomponent pattern (Fig. 3) with RCM was characterized by irregular cobblestone with spindled and atypical cells in the epidermis, nonedged papillae and plump bright cells at the dermoepidermal junction and in the papillary dermis [4]. Therefore, we made a diagnosis of EDSN. We decided to perform a strict follow-up of the numerous Spitz nevi instead of performing multiple excisions. Nevertheless, during the follow-up we removed one lesion with multicomponent pattern (Fig. 3) suspected to be malignant which resulted to be a Spitz nevus at histopathological examination. Currently the patient is 3 years old and in the follow-up was not noted any changement in pattern and all nevi on dermoscopy were stable. In the follow-up carried out until now there has been observed the onset of new Spitz nevi.

Written informed consent was obtained from the patient’s legal guardian(s) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.