Melanotic neuroectodermal

tumor of infancy

Peter L. Judd, DDS, Dip Pedod, MSC,~ Kim Harrop, DMD,’ Jerome Becker, DDS, MSC,~ Toronto, Ontario, Canada THE

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The case of a P-month-old male infant treated for melanotic neuroectodermal tumor of infancy is presented to demonstrate the importance of early treatment in the containment of the growth of such lesions. Although the lesion itself is rare, the posterior maxillary location and involvement of the optic nerve in this patient’s lesion made his case even less typical of those commonly documented in the literature. Complete surgical excision of the lesion was not possible in this patient because of the gross mutilation it would have caused. There was no evidence of the tumor recurring in 18 months of follow-up examinations. These results support the current theories regarding the debulking effect in conjunction with bodily defenses on residual tumor cells and the effect of the removal of stimulatory cells on invading peripheral cells. This case points out the importance of an early and rapid investigation of a mass that initially occurs on the alveolar ridge of an infant. (ORAL

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lassically, melanotic neuroectodermal tumor of infancy (MNTI) has been described as a rare benign pigmented lesion that usually occurs in the anterior region of the maxilla. Approximately 93% of the lesions reported have occurred in the region of the head and neck; the most common sites are the maxilla (68.8%), skull (10.8%), mandible (5.8%), and brain (4.3%).’ The most common site of occurrence is the maxillary incisal region.2 The lesion occurs predominantly and equally in children of both sexes who are less than 1 year of age. Because the tumor is considered benign, conservative excision is used in its treatment; the rate of local recurrence after excision is 15%.3%4 There is, however, a malignancy rate of approximately 2%.’ Furthermore, this benign tumor can lead to death if it invades local vital tissues. Since this tumor was first reported by Krompecher in 1918, the subject of its origin has been controversial,s resulting in a variety of names for the

“StaK Pediatric Dentist, The Hospital for Sick Children, Toronto, Ontario. Canada. bDental Resident at The Hospital for Sick Children, Toronto. Ontario, Canada. at the time of this case: now in private practice in Toronto. ‘Staff Oral and Maxillofacial Surgeon, The Hospital for Sick Children, Toronto, Ontario, Canada. Produced on a Macintosh SE with Microsoft Word 3.02: 3.5” disc is available on request in Macintosh or MSDOS format.

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tumor that include pigmented ameloblastoma,6 melanotic progonoma,7 pigmented congenital epulis.s and melanotic epithelial odontome.9 However, bio-

chemical, histochemical, and ultrastructural studies now support a neural-crest origin.’ Although conservative treatment of this benign tumor is the accepted practice, the following case report illustrates the importance of instituting treatment as soon as possible to minimize tissue destruction caused by tumor growth. CASE

REPORT

A 2-month-old male infant had an enlarged left posterior maxillary alveolar ridge. The parents first noticed the enlargement about a week before the child’s dental appointment and became concerned by its rapid growth. An intraoral examination revealed a nonulcerated swelling of the left alveolar ridge in the molar area. The mass was normal in color with a small central area of pigmentation that resembled an eruption hematoma. The mass was generally firm to palpation except for the area of pigmentation. Fluctuation in this area suggested the presence of a cyst. A periapical radiograph showed a slight radiopaque, bone-destructive lesion with vaguely demarcated borders. The primary molars in the area had been displaced from their normal developmental site and appeared to be floating within the lesion. The patient was originally scheduled for an excisional biopsy: however, surgical exposure of the lesion revealed that it was more extensive than originally suspected. Surgical exploration of the left posterior maxillary region revealed an 723

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Fig. 1. Photomicrograph of specimen showing compressed nests and cords of cells with hyperchromatic nuclei with many containing melanin pigmentation within cytoplasm. A second population of larger cuboidal cells and a moderate cellular stroma are also evident. (Hematoxylin and eosin stain. Original magnification, X400.)

Fig. 2. CT scan showing lesion occupying left maxillary sinus. The lesion can be seen encroaching on the orbit and buccal sulcus. Growth of the tumor has also caused an expansion of the left palatal plate.

invasive, darkly pigmented soft tissue mass that obliterated the left maxillary sinus and extended superiorly to the infraorbital region, medially into the nasal cavity and posteriorly to the lateral pterygoid muscle. The bony margins appeared ragged with indefinite margins. Surgery was terminated because of the extent of the lesion. Arrangements were made for a computed tomographic (CT) evaluation of the remaining tumor, followed by its surgical excision. The surgical specimen was then sent to the pathology laboratory for identification, and the patient was transferred to the Department of Otolaryngology for continued care. The two pieces of the gross specimen were irregular in contour and measured 3.9 cm X 1.6 cm X 0.9 cm and 2.5 cm X 1.3 cm X 0.5 cm. They were reddish blue, but when sectioned, revealed a gray-white periphery and a blue-black uniform core. Histologic examination (Fig. 1, courtesy of Department of Pathology, Hospital For Sick Children) showed compressed nests and cords of predominantly small basophilic cells, many containing melanin pigmentation within the cytoplasm. These nests were separated by a moderately cellular stroma. A second population consisted of larger cuboida1 cells with abundant cytoplasm that were arranged in alveolar clusters. A CT scan revealed that the lesion arose from within the left maxillary sinus (Fig. 2, courtesy of Department of Radiology, Hospital For Sick Children). It extended medially into the nasal cavity, inferiorly into the alveolus, superiorly into the orbit, and posteriorly to the lateral pterygoid muscle. The lateral pterygoid muscle appeared displaced rather than infiltrated by the tumor. Neither the cranium nor the bones of the base of the skull were involved. The lesion was limited anteriorly and laterally by the anterior wall of the maxillary sinus and temporalis muscle, respectively.

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3. CT scantaken 18 monthspostoperatively. Lesionhasalmostresolvedwith the presenceof c:alcificationsin the previoustumor bed.

Fig.

During a subsequentoperation with the patient under general anesthesia,most of the remaining lesion was excised.However, surgicalexploration into the intraobital region revealed the lesion tracking down the optic nerve. The remaininglesionwasnot further excisedbecause of the gross mutilation that would be caused to the infant. The surgical site was closed and later found to heal normally.

Regular follow-up examinationsof the patient for 30 months revealed no evidence of recurrent tumor. Continued CT evaluations have shown a steady improvement and the presence of calcifications in the previous tumor bed (Fig. 3, courtesy, Department of Radiology, Hospital For Sick Children). DISCUSSION

Clinically, MNTI is a fast-growing, nonulcerative swelling in the incisal region of the maxilla. Pigmentation may or may not be seen in the overlying soft tissue. This condition is generally found in patients lessthan 1 year of age. A review of 139 positively confirmed casesshowed 43% of them occurred within the first 3 months of age.4 The typical radiographic image shows a slight radiopaque lesion with a poorly demarcated border, suggestiveof malignancy. Teeth involved in the lesion may appear to be floating within the radiolucent area of the tumor because they are displaced from their normal area of development. This radiographic appearance can understandably mislead the clinician

into giving a preliminary diagnosis of a malignancy such as osteogenic sarcoma. Histologic investigations of the lesion usually report the appearance of two-cell populations within an abundant connective tissue stroma. One cell population is composedof large cuboidal cells containing intracytoplasmic granules arranged in strands or surrounding alveolus-like spaces;the other population is represented by smaller round cells with hyperchromatic nuclei and sparse cytoplasm.10-‘3 Although our patient had most of the classic features of MNTI, the more posterior location of the tumor in the maxilla was atypical. The tumor’s typical rapid growth quickly involved deeper and more distant structures of the midface region. Thus, the usual conservative treatment of total excision of the tumor, so easily performed on a lesion in the premaxilla, was not possiblein this case.Total excision would have led to unacceptable mutilation of the midface region of the 3-month-old infant. Moreover, asCrockett and his colleagues” have pointed out, surgically clear margins may not be attainable with lesionssuch as those reported in this case when the maxilla is largely or totally involved. The required extent of the excision of the lesion is controversial. Numerous clinicians have advocated allowing residual portions of the tumor to remain when radical excision would be mutilating.4. I ‘. I4

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However, others have recommended a more aggressive treatment of total resection and even radiotherapy.12 Since the lesion is generally benign and local excision is such a successful form of treatment, this aggressive approach to treatment has been questioned by Hupp and his colleagues.4 They attribute the success of conservative excision of the MNTI lesion, in cases where remnants of the tumor remain, to the debulking effect and initiation of bodily defenses that result in destruction of any residual cells of the tumor.15 It has also been proposed that the peripheral cells are dependent on a group of stimulatory cells in the center of the tumor mass; thus, the removal of these stimulatory cells leads to the death of the invading peripheral cells. l6 The 1%month follow-up of our case appears to support these theories: the remnants of tumor seem to be disappearing. Normally, when MNTI is treated conservatively with surgical excision, a low rate of recurrence of the tumor can be expected. However, the speed of the growth of this lesion may change what is initially a simple localized tumor into a more invasive tumor that can cause extensive destruction of healthy tissue. This is not, therefore, a lesion to be observed and reassessed at some later date. Since the dentist is likely to be one of the first health professionals the parents approach about a growth on their infant’s gum, the dentist must act immediately to ensure early excision of this rapid-growing and potentially fatal tumor. In the case reported here, the procurement of a CT scan before the initial surgical biopsy would have been advisable to assess the extent of disease from the onset. This article was prepared with the assistance of the Medical Publications Department, The Hospital for Sick Children, Toronto, Ontario, Canada. REFERENCES 1. Cutler dermal review,

LS, Chaudhry AP, Topazian R. Melanotic neuroectotumor of infancy: an ultrastructure study, literature and reevaluation. Cancer 1981;48:257-70.

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LO, Thilander H. Melanoameloblastoma. Report of 2. Dahlback a case and a critical review of the literature. Odont Tids 1964;?2:44-62. 3. Block JC, Waite DE, Dehner LP, Leonard AS, Ogle RG, Gatto DJ. Pigmented neuroectodermal tumor of infancy. ORAL SURG ORAL MED ORAL PATHOL 1980;49:279-85. 4. Hupp JR, Topazian RG, Krutchkoff DJ. The melanotic neuroectodermal tumor of infancy. Int J Oral Surg 198 1;10:43246. E. Zur Histogenese und Morphologic der Ada5. Krompecher mantinome und sonstiger Kiefergeschwulste. Beitr Path01 Anat 1918;64:165-97. JL. Melanotic ameloblastoma. ORAL 6. Tiecke RW, Bernier SURG ORAL MED ORAL PATHOL 1956;9: 1197-209. tumor of infancy: the melanotic I. Stowens D. A pigmented mogonoma. J Pathol Bacterial 1957;73:43-5 1. congenital epu8. ‘Henry TC, Bodian ML. A case of pigmented lis. Br J Surg 1960;47:574-6. 9. Mummery JH, Pitts AT. A melanotic epithelial odontome in a child. Br Dent J 1926;47:121-30. 10. Carpenter BF, Jimenez C, Rob IA. Melanotic neuroectoderma1 tumor of infancy. Pediatr Path01 1985;3:227-44. 11. Crockett DM, McGill TJ, Healy GB, Friedman EM. Melanotic neuroectodermal tumor of infancy. Otolaryngol Head Neck Surg 1987;96:194-7. 12. Hall WC, O’Day DM, Glick AD. Melanotic neuroectodermal tumor of infancy. Arch Ophthalmol 1979;97:922-5. 13. Ramon Y, Oberman M, Horowitz I, Freedman A. Melanotic

neuroectodermal tumor of infancy (pigmented melanoameloblastoma). Int J Oral Surg 1979;8:312-7. 14. Thoma GW. Ameloblastic tumors. Cancer Bulletin (Houston) 1972;24:98-9. 15. Hellstrom KE, Hellstrom J. Immunity to neuroblastoma and melanomas. Ann Rev Med 1972;23:19-38. NB, Padgett EC. Two unusual melanomas of the 16. Soderberg alveolus and maxilla. Am J Orthod 1941;27:270-4. Reprint requests to: Dr. Peter L. Judd Department of Dentistry The Hospital for Sick Children 555 Uniiersity Ave. Toronto. Ontario. Canada M5G 1x8

Melanotic neuroectodermal tumor of infancy.

The case of a 2-month-old male infant treated for melanotic neuroectodermal tumor of infancy is presented to demonstrate the importance of early treat...
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