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An Alternate Method of Fabrication of an Obturator in a Patient Diagnosed with Melanotic Neuroectodermal Tumor of Infancy

CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2019; 40(S 01): S148-S151

DOI: DOI: 10.4103/ijmpo.ijmpo_256_17

Author : Dholam, Kanchan P; Singh, Gurkaran Preet; Gurav, Sandeep V; Shinde, Arati A

Abstract

The quality of treatment and rehabilitation for the pediatric head and neck cancer patient has greatly improved due to interdisciplinary management. Surgical resection of maxilla leads to functional impairment which can be adequately restored with an obturator. However, its fabrication can be challenging in a pediatric patient. A simple, practical, and time-saving procedural modification in the fabrication of obturator in a patient diagnosed with rare melanotic neuroectodermal tumor of infancy has been presented.

Keywords

Interdisciplinary care - melanotic neuroectodermal tumor of infancy - modification - obturator - time-saving


Publication History

Article published online:
24 May 2021

© 2019. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

Abstract

The quality of treatment and rehabilitation for the pediatric head and neck cancer patient has greatly improved due to interdisciplinary management. Surgical resection of maxilla leads to functional impairment which can be adequately restored with an obturator. However, its fabrication can be challenging in a pediatric patient. A simple, practical, and time-saving procedural modification in the fabrication of obturator in a patient diagnosed with rare melanotic neuroectodermal tumor of infancy has been presented.


Keywords

Interdisciplinary care - melanotic neuroectodermal tumor of infancy - modification - obturator - time-saving

Introduction

Melanotic neuroectodermal tumor of infancy (MNTI) has been categorized as “rare clinical entity” by “Office of Rare Diseases” of National Institute of Health. It is a melanin-producing neoplasm which primarily affects maxilla during 1st year of life.[1]

Although benign, MNTI has been documented to be locally aggressive, resulting in tooth displacement, adjacent bone invasion, and impairment of oral functions such as feeding and sucking.[2] Multidisciplinary management includes surgical removal of the tumor mass followed by restoration of oral function with an obturator.

The current report is documentation of novel, simple, and practical approach in the fabrication of an obturator in a growing child.


Case Report

Previous medical history

A 2½-month-old infant presented with sudden onset and progressive left facial swelling. A 3 cm × 3 cm well-circumscribed, nonfluctuant mass was seen in the left maxilla, protruding into oral cavity. Computerized tomographic scan revealed an expansive lytic lesion involving left maxilla associated with significant peripheral sclerosis and displacement of deciduous teeth.

Incisional biopsy was indicative of MNTI. Immunohistochemical (IHC) analysis was positive for HMB-45 and cytokeratin and equivocal for S-100.

In view of vast tumor spread and focal areas of ulceration, radical surgery was planned. Left total maxillectomy was carried out, and surgical defect was lined with partial thickness skin graft. On sufficient healing, an interim maxillary obturator was given to the patient.

At present, the patient reported to the department at an age of three, with his parents with a complaint of nasal seepage of liquids due to lack of retention of the obturator.


Examination

Intraoral examination revealed well-healed, skin graft-lined total maxillectomy defect on left side [Figure 1]. Deciduous canine and molars had erupted with evidence of erupting deciduous right central incisor. The existing obturator had become loose owing to infantile growth spurt. Eruption of deciduous teeth had rendered an incomplete seating of the obturator.


| Figure. 1  Intraoral view of well‑healed maxillectomy defect with red circle showing erupting deciduous incisor


Procedure of fabrication of an interim obturator

Existing obturator was duplicated in form and used as a custom tray [Figure 2]. Height of the bulb was reduced, and tray was relieved all around by 1–1.5 mm. A handle was attached to enable accurate and convenient seating in the mouth.

| Figure. 2  Custom tray obtained by duplicating the existing prosthesis


The tray was verified for border extensions, and impression of maxillectomy defect was made with polyvinyl siloxane (PVS) impression material (Aquasil Soft Putty™ and Aquasil LV™, Dentsply International, Pennsylvania, United States). Putty material was molded and placed on the bulb and light body PVS loaded onto remaining portion of the tray. A thin layer of light body material was applied over putty to record fine details of the defect. The child was stabilized and tray carefully placed in the mouth. On reaching final set of the material, the impression was removed from the mouth and inspected for errors and inaccuracies [Figure 3]. It was poured in Type III dental stone (Ultrastone™, Kalabhai Pvt. Ltd., Mumbai, India) to obtain master cast [Figure 4]. The defect area was outlined on the cast and medial undercut was blocked. Subsequently, an interim maxillary obturator was fabricated in polymethyl-methacrylate resin (DPI Heat Cure, Dental Products of India Pvt Ltd., Mumbai, India). A relief was designed in the obturator to accommodate erupting deciduous right central incisor [Figure 5]. The obturator was finished, polished, and delivered to the patient [Figure 6]. Parents were instructed about maintenance of the prosthesis and importance of regular follow-up visits to facilitate frequent adjustments.

| Figure. 3  Definitive elastomeric impression

| Figure. 4  Master cast

| Figure. 5  Final prosthesis with red circle showing provision to accommodate erupting deciduous incisor

| Figure. 6  Interim obturator in situ


Discussion

MNTI is a rare clinical entity and usually affects infants in first 6 months of life with a mean age of presentation of 4.3 months.[3] [4] It has no gender predilection though Kruse–Losler reported a male-to-female ratio of 1.48.[5] It is a rapidly-growing, nonulcerative enlarging mass that usually affects anterior maxilla. It has also been reported in other sites of head and neck region such as skull, mandible, and brain.[4]

Many investigators have proposed numerous theories regarding tumor histogenesis which has resulted in its varied nomenclature.[6] [7] [8] [9] [10] Current nomenclature of MNTI which reflects its neural crest cell origin is widely accepted and recognized by WHO.[11]

Radiologically, the tumor is osteolytic in appearance with adjoining deciduous teeth appearing to be “floating” within the lesion.[12]

Histologically, it shows dual cellular pattern with large melanin-containing polygonal cells arranged in alveolar or tubular structures and small neuroblast-like cells with hyperchromatic nucleus and scanty neoplasm in dense fibrous stroma. IHC markers such as HMB-45, neuron specific enolase, CD 56, synaptophysin, and chromogranin have been proposed to have a positive association with MNTI.[6]

Despite aggressive clinical presentation, the prognosis is good and curative surgery is treatment of choice. The probability of recurrence is maximum in first 6–18 months; hence, periodic follow-up is highly recommended.[1] The presented case has been on regular follow-up since 3 years and shown excellent locoregional control.

Multidisciplinary management of MNTI is essential as radical surgery impairs essential oral functions such as feeding, mastication, and speech. Interdisciplinary care reduces posttreatment morbidity by providing efficient rehabilitation during critical growth period of the pediatric patient.[13]

Maxillary obturator helps in restoring palatal contours and sealing margins of maxillary defect.[13] The exercise of recording maxillary defect during dual impression-making can be quite tedious and traumatic for a pediatric patient. In this report, existing obturator was duplicated and sufficiently relieved to act as a custom tray. This was time-saving and allowed us to forego the procedure of making preliminary impression. In addition, it enabled the construction of a well-fitted custom tray which was well tolerated by the child. Such procedural modification greatly improved patient compliance and facilitated prosthesis delivery within a day. An opening was created in the prosthesis corresponding to erupting deciduous incisor to allow unimpeded eruption.


Conclusion

The current report throws light on the significance of interdisciplinary management of a rare pediatric solid tumor such as MNTI. A simple, practical, and time-saving procedural modification in the fabrication of obturator for a pediatric patient has been presented. In addition, it stresses on the need to accommodate erupting teeth by necessary adjustment of the prosthesis which is critical in a growing child.

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.


Conflict of Interest

There are no conflicts of interest.

  • References

  1. Rodriguez-Galindo C, Furman WL, Pappo AS. Rare pediatric tumors. In: Pizzo PA, Poplack DG. editors Principles and Practice of Pediatric Oncology. 7th ed. Philadelphia: Wolters Kluwer; 2016: 946-7
  2. Reddy ER, Kumar MS, Aduri R, Sreelakshmi N. Melanotic neuroectodermal tumor of infancy: A rare case report. Contemp Clin Dent 2013; 4: 559-62
  3. Neven J, Hulsbergen-van der Kaa C, Groot-Loonen J, de Wilde PC, Merkx MA. Recurrent melanotic neuroectodermal tumor of infancy: A proposal for treatment protocol with surgery and adjuvant chemotherapy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 106: 493-6
  4. Rajendran R. Benign and malignant tumors of oral cavity. In: Rajendran R, Sivapathasundhram B. editors Shafer’s Textbook of Oral Pathology. 5th ed. New Delhi: Elsevier; 2006: 283-5
  5. Kruse-Lösler B, Gaertner C, Bürger H, Seper L, Joos U, Kleinheinz J. et al. 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
  6. Pattanayak S, Ray JG, Mukherjee RS, Mandal C, Chaudhuri K. Melanotic neuroectodermal tumour of infancy. BMJ Case Rep 2010;2010. pii:bcr0120102645.
  7. Stowens D. A pigmented tumour of infancy – The melanotic progonoma. J Patol Bact 1957; 73: 43
  8. Bernier JL, Tiecke RW. Melanotic ameloblastoma. Oral Surg Oral Med Oral Pathol 1956; 9: 1197-209
  9. Henry TC, Bodian M. A case of pigmented congenital epulis. Br J Surg 1960; 47: 574-6
  10. Halpert B, Patzer R. Maxillary tumor of retinal anlage. Surgery 1947; 22: 837-41
  11. Madrid C, Aziza J, Hlali A, Bouferrache K, Abarca M. Melanotic neuroectodermal tumour of infancy: A case report and review of the aetiopathogenic hypotheses. Med Oral Patol Oral Cir Bucal 2010; 15: e739-42
  12. Batsakis JG. Pathology consultation. Melanotic neuroectodermal tumor of infancy. Ann Otol Rhinol Laryngol 1987; 96: 128-9
  13. Martin JW, Chambers MS, Lemon JC, Toth BB, Helfrick JF. Prosthodontic and surgical considerations for pediatric patients requiring maxillectomy. Pediatr Dent 1995; 17: 116-21

Address for correspondence

Dr. Gurkaran Preet Singh
Dental and Prosthetic Services, Tata Memorial Centre- Advanced Centre for Treatment, Research and Education in Cancer (TMC-ACTREC)
Kharghar, Navi Mumbai - 410 210, Maharashtra
India   


Publication History

Article published online:
24 May 2021

© 2019. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

  • References

  1. Rodriguez-Galindo C, Furman WL, Pappo AS. Rare pediatric tumors. In: Pizzo PA, Poplack DG. editors Principles and Practice of Pediatric Oncology. 7th ed. Philadelphia: Wolters Kluwer; 2016: 946-7
  2. Reddy ER, Kumar MS, Aduri R, Sreelakshmi N. Melanotic neuroectodermal tumor of infancy: A rare case report. Contemp Clin Dent 2013; 4: 559-62
  3. Neven J, Hulsbergen-van der Kaa C, Groot-Loonen J, de Wilde PC, Merkx MA. Recurrent melanotic neuroectodermal tumor of infancy: A proposal for treatment protocol with surgery and adjuvant chemotherapy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 106: 493-6
  4. Rajendran R. Benign and malignant tumors of oral cavity. In: Rajendran R, Sivapathasundhram B. editors Shafer’s Textbook of Oral Pathology. 5th ed. New Delhi: Elsevier; 2006: 283-5
  5. Kruse-Lösler B, Gaertner C, Bürger H, Seper L, Joos U, Kleinheinz J. et al. 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
  6. Pattanayak S, Ray JG, Mukherjee RS, Mandal C, Chaudhuri K. Melanotic neuroectodermal tumour of infancy. BMJ Case Rep 2010;2010. pii:bcr0120102645.
  7. Stowens D. A pigmented tumour of infancy – The melanotic progonoma. J Patol Bact 1957; 73: 43
  8. Bernier JL, Tiecke RW. Melanotic ameloblastoma. Oral Surg Oral Med Oral Pathol 1956; 9: 1197-209
  9. Henry TC, Bodian M. A case of pigmented congenital epulis. Br J Surg 1960; 47: 574-6
  10. Halpert B, Patzer R. Maxillary tumor of retinal anlage. Surgery 1947; 22: 837-41
  11. Madrid C, Aziza J, Hlali A, Bouferrache K, Abarca M. Melanotic neuroectodermal tumour of infancy: A case report and review of the aetiopathogenic hypotheses. Med Oral Patol Oral Cir Bucal 2010; 15: e739-42
  12. Batsakis JG. Pathology consultation. Melanotic neuroectodermal tumor of infancy. Ann Otol Rhinol Laryngol 1987; 96: 128-9
  13. Martin JW, Chambers MS, Lemon JC, Toth BB, Helfrick JF. Prosthodontic and surgical considerations for pediatric patients requiring maxillectomy. Pediatr Dent 1995; 17: 116-21

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