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Year : 2017  |  Volume : 11  |  Issue : 1  |  Page : 251-253  

Anaesthetic management of a neuroectodermal tumor of infancy: A rare case report

Department of Anaesthesiology, TNMC and BYL Nair Hospital, Mumbai, Maharashtra, India

Date of Web Publication16-Feb-2017

Correspondence Address:
Dr. Pravin Ubale
Anand Bhavan, B–16, TNMC and BYL Nair Hospital, Mumbai Central, Mumbai - 400 011, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0259-1162.186863

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Melanotic neuroectodermal tumor of infancy is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of infants. We report a 5-month-old male child who presented with a swelling in the right upper tooth region of upper jaw in which we face difficult mask ventilation as well as difficult intubation. Wide surgical excision was performed under general anesthesia. The uneventful course of anesthesia in the presented case was due to the thorough systemic evaluation and careful anesthetic strategy. Patients of congenital epulis continue to pose challenge to anesthesiologist as a consequence of the potential difficult mask ventilation and intubation. We hereby present a case of congenital epulis repair using diode laser under general anesthesia.

Keywords: Difficult intubation, difficult mask ventilation, melanotic neuroectodermal tumor of infancy

How to cite this article:
Ubale P, Baldwa N, Gujjar P. Anaesthetic management of a neuroectodermal tumor of infancy: A rare case report. Anesth Essays Res 2017;11:251-3

How to cite this URL:
Ubale P, Baldwa N, Gujjar P. Anaesthetic management of a neuroectodermal tumor of infancy: A rare case report. Anesth Essays Res [serial online] 2017 [cited 2021 Oct 28];11:251-3. Available from:

   Introduction Top

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign pigmented tumor that most commonly presents in the 1st year of life. MNTI has a slight male predilection, with a male-to-female ratio of 1.48:1.[1] Most patients, by some estimates >90%, present with the tumor in the 1st year of life, usually from age 1 to 6 months, with a peak between the 2nd and 6th month of life. A few cases of MNTI have been reported in adults, notably, a 23-year-old man, a 39-year-old woman, and a 67-year-old woman.[2],[3],[4] However, only 9% of cases are seen in patients older than 12 months.[1] Although MNTI is classified as a benign lesion, it is often clinically worrisome because of its rapid onset and alarming local growth rate. Often, sucking and feeding are impaired secondary to the swelling. Patients of MNTI continue to pose a challenge to anesthesiologist as a consequence of the potential difficult mask ventilation and intubation. In the presented case, successful intubation was achieved by using gaseous induction using sevoflurane with a large face mask and displacement of the tumor to the other side using tongue depressor.

   Case Report Top

A 5-month-old, 10 kg male child was referred to our institution with a swelling in the oral cavity. The swelling was observed by the parents for the first time when the child was 3 months old. Main concerns of the parents were swelling of increasing size which was causing disfigurement of face, feeding problem, and also the swelling bled on touch. On palpation, the swelling was hard in consistency also the mucosa over the swelling was ulcerated. The decision was taken by the surgeons to excise the mass on an emergency basis. All biochemical investigations were within normal limits. Computed tomography scan revealed well defined heterogeneously enhancing lytic expansile mass lesion of size 36 mm × 31 mm (Trans × AP) in the right maxilla (alveolar process). On the day of surgery, high-risk consent was obtained from the parents. After taking the patient on the operation table, baseline blood pressure (114/68 mm of Hg), pulse rate (110/min), and oxygen saturation (98%) were noted. Electrocardiogram, noninvasive blood pressure, precordial stethoscope, and pulse oximeter were monitored. Peripheral venous access was achieved with a 22-gauge catheter. Patient was given premedication with injection glycopyrrolate 0.04 mg/kg, injection fentanyl 2 µg/kg, midazolam 0.03 mg/kg, and injection ondansetron 0.08 mg/kg intravenously. The patient was taken under sevoflurane anesthesia. The patient was not able to ventilate due mass in the oral cavity. Oropharyngeal airway was inserted from the lateral side of the mass in the oral cavity. Once the child was ventilated with the insertion of oropharyngeal airway, injection succinylcholine 1 mg/kg was given intravenously. During insertion of laryngoscope blade was very difficult so one assistant had retracted tumor on one side by tongue depressor while on the other side laryngoscope blade was inserted. On direct laryngoscopy Cormack–Lehane grade of 3 was observed. Endotracheal intubation was carried out with portex uncuffed tracheal tube of size 4.5 mm with help of external laryngeal manipulation and with the help of stylet. Throat packing was done in view of oral surgery. Anesthesia was maintained with O2, air and sevoflurane with intermittent doses of injection atracurium with Jackson Rees circuit. Duration of surgery went for 2 h. Wide surgical excision was done [Figure 1] and [Figure 2]. The sample was sent for histopathology examination which revealed MNTI. Blood loss was 100 ml and was replaced. At the end of the surgery, throat pack was removed, and patient was reversed with glycopyrrolate and neostigmine. Child was extubated after the child attains good muscle power tone and respiratory movements. The child was shifted to postanesthesia care unit for postoperative monitoring. Postoperative recovery was uneventful.
Figure 1: Preexcision photograph of child.

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Figure 2: Postexcision photograph of child.

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   Discussion Top

MNTI is a rare condition that occurs normally in the anterior maxilla of infants aged <1 year. MNTI is generally a benign lesion, but it requires aggressive surgical treatment because it behaves in a locally aggressive fashion and has a high rate of recurrence, i.e., from 15% to 45%.[5],[6] More than 90% of cases of MNTI occur in the head and neck region, with a striking predilection for the anterior portion of the maxilla. In fact, the maxillary bone is the location of 61% of reported cases.[1] Other less frequently affected sites include the skull (16%), the epididymis and testis (9%), the mandible (6%), and the brain (6%).[1] A few cases have been reported in unusual locations such as the subcutaneous soft tissue of the thigh, the femur, the mediastinum, the shoulder, and the uterus.

The differential diagnosis of MNTI is quite broad, but it must be separated from other pediatric “small round cell” neoplasms, such as neuroblastoma, Ewing's sarcoma, peripheral neuroepithelioma, rhabdomyosarcoma, peripheral primitive neuroectodermal tumor, desmoplastic small round cell tumor, malignant melanoma, and lymphoma. However, immunohistochemistry, electron microscopy, histochemistry, and the detection of melanotransferrin mRNA transcripts now have confirmed that MNTIs are derived from neural crest cells, derived in turn from neuroectoderm.[1],[7] Microscopically, MNTIs are biphasic tumors characterized by larger polygonal epithelioid cells, which resemble melanocytes and contain variable deposits of melanin and smaller neuroblastoma-like round cells.

MNTI is frequently associated with elevated urinary excretion of vanillylmandelic acid (VMA), a metabolite of epinephrine and norepinephrine.[8] Although an increase in VMA is helpful, this symptom alone is not diagnostic of MNTI.[8] The treatment of choice consists of complete surgical excision, conservative surgical treatment, wide local excision, and chemotherapy.

The main anesthesia concerns in our case were (1) small child with large mass (2) difficult mask ventilation (3) difficult ventilation: In our case, the child was induced under sevoflurane anesthesia. For difficult mask ventilation, larger size face mask was used so as to include tumor mass in it and an oral airway was inserted with great difficulty from the lateral side of the tumor. For oral intubation, it was difficult to put laryngoscope blade as there was very little space. Hence, one assistant had pushed tumor mass to other side with the help of tongue depressor, and laryngoscope blade was introduced from other side. With external laryngeal manipulation, it was possible to do endotracheal intubation.

   Conclusion Top

MNTI is very rare tumor and presents a real challenge to anesthesiologist in view of difficult mask ventilation and intubation. Hence, proper airway assessment and planning is mandatory for better airway control and safe endotracheal intubation.

The main concern in anesthetic management was mask ventilation and intubation as protruding mass may interfere with mask ventilation and thereby may create difficulty in passing the endotracheal tube.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Kruse-Lösler B, Gaertner C, Bürger H, Seper L, Joos U, Kleinheinz J. Melanotic neuroectodermal tumor of infancy: Systematic review of the literature and presentation of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;102:204-16.  Back to cited text no. 1
Gorhan C, Soto-Ares G, Ruchoux MM, Blond S, Pruvo JP. Melanotic neuroectodermal tumour of the pineal region. Neuroradiology 2001;43:944-7.  Back to cited text no. 2
Vajtai I, Suták I, Varga Z. Melanotic progonoma as a component of ovarian teratoma. Histopathology 2000;36:283-5.  Back to cited text no. 3
Schulz DM. A malignant, melanotic neoplasm of the uterus, resembling the retinal anlage tumors; report of a case. Am J Clin Pathol 1957;28:524-32.  Back to cited text no. 4
Judd PL, Harrop K, Becker J. Melanotic neuroectodermal tumor of infancy. Oral Surg Oral Med Oral Pathol 1990;69:723-6.  Back to cited text no. 5
Kapadia SB, Frisman DM, Hitchcock CL, Ellis GL, Popek EJ. Melanotic neuroectodermal tumor of infancy. Clinicopathological, immunohistochemical, and flow cytometric study. Am J Surg Pathol 1993;17:566-73.  Back to cited text no. 6
Matsumoto M, Sakuma J, Suzuki K, Kawakami M, Sasaki T, Kodama N. Melanotic neuroectodermal tumor of infancy in the skull: Case report and review of the literature. Surg Neurol 2005;63:275-80.  Back to cited text no. 7
Selim H, Shaheen S, Barakat K, Selim AA. Melanotic neuroectodermal tumor of infancy: review of literature and case report. J Pediatr Surg 2008;43:E25-9.  Back to cited text no. 8


  [Figure 1], [Figure 2]

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