J Oral Maxillofac 50366-694,
Surg
1992
Melanotic Neuroectodermal Tumor of Infancy: Review of the Literature and Report of a Case EDWARD L. MOSBY, DDS, FACD,* MICHAEL W. LOWE, DDS,t CHARLES M. COBB, DDS, PHD,$ AND ROBERT L. ENNIS, DOSS
The melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor. We were able to find only 195 cases reported in the world literature through 19891-46 (Table 1). Carpente?’ and Parizek” proposed several hypotheses for the origin of MNTI; these included congenital melanocarcinoma of the alveolar procesq4’ odontogenic epithelium,48q49and a phylogenetic origin related to the median or pineal eye of particular lower vertebrates.50%51Another phylogenetic theory is concerned with displaced neuroectodermal tissue from the vestigial organ of Jacobson, ie, vomeronasal organ.52 Retinal tissue has also been suggested as a source of MNTI.S3,54However, the neural crest is the most commonly accepted tissue of origin for MNTI.55-58 MNTI primarily occurs mainly in the maxilla, but can occur in other areas of the skull, the mandible, brain, and epididymis, as well as other anatomic locations59-62(Table 2). It classically appears as a painless, darkened, expansion of the maxillary alveolar region, with a history of accelerated growth within the preceding few weeks to months potentially interfering with nursing. It is usually benign; only 11 cases were reported as being malignant.‘7,25,28,4’.58,63 Conventional radiographs of intrabony lesions usually show a radiolucency with or without irregular
margins. Computed tomography (CT) typically reveals hyperdense masses, but hypodense growths also have been reported.32245Magnetic resonance imaging (MRI) shows a hypointense mass, with focal areas of hyperintensity in T,-weighted images and an isointense mass on T2-weighted images.42 Histologically, MNTI consists of a biphasic population of epithelioid melanin-containing cells and neuroblastlike cells within a prominent stroma.37s43,64The melanin-containing cells can be cuboidal and arranged in alveolar, pseudoalveolar, or glandlike structures.38 The treatment for MNTI has typically been surgical and includes enucleation and/or curettage with limited, wide, or en bloc excision. Cases of surgically unmanageable MNTI have received chemotherapy,2q42 chemotherapy with radiation therapy,28.65 and radiation therapy.5 Debulking procedures also have been used.66 The recurrence rate of MNTI remains approximately 15%. Report of a Case The patient, a 4%month-old white girl, was referred to the oral and maxillofacial surgery service at Truman Medical
Center by one of the authors (R.E.) for diagnosis and treatment. The parents had noted swelling in the mouth and along the left side of the child’s nose approximately 6 weeks before seeking consultation, at which time the child was about 2 months old. They noted that the swelling was episodic, but their concern increased over the past 2 weeks as the swelling progressively enlarged and caused noticeable distortion of the left side of the face. They also reported that their daughter was having increasing difficulty in taking oral feedings. The child’s past medical history was totally unremarkable except for the chief complaint. She was taking no medications other than vitamins. The parents stated that the child had not had a fever during the period of the sporadic swelling. No allergies were reported. Physical examination did not show any abnormalities other than those related to the chief complaint. There was enlargement of the left face that caused superomedial displacement of the left alar base. No abnormalities were noted on the right side. Intraorally, there was expansion of the left maxilla from
Received from the Department of Hospital Dentistry, Oral and Maxillofacial Surgery Graduate Training Program, University of Missouri-Kansas City Schools of Dentistry and Medicine, Truman Medical Center, Kansas City, MO. * Professor, Oral and Maxillofacial Surgery; Associate Director Oral and Maxillofacial Surgery Residency. t Resident. $ Professor, Periodontology and Oral Biology. 9 Clinical Associate Professor; in private practice, Oral and Maxillofacial Surgery, Independence, MO. Address correspondence and reprint requests to Dr Mosby: Department of Hospital Dentistry, University of Missouri-Kansas City Schools of Dentistry and Medicine, Truman Medical Center, 2301 Holmes St, Kansas City, MO 64108. 0 1992 American
Association
of Oral and Maxillofacial
Surgeons
0278-2391/92/5008-0016$3.00/O
886
Table 1.
Renortr of MNTl from 1980 to Present Recurrence/ Malignant
Location
Sex
Age
Mosby et al. 1992 Prasad et al, 198940 Ogata et al. 19894’ Atkinson et al 198942 Turner et al, 1$89” Turner et al, 198944 Claros et al, 198945 Murayma et al. 198946 Scheck et al, I 98943
F M F F F M M M F
4.5 mo 6 mo 4 yr 2 mo 2.5 mo 3 mo 2 mo 10 mo 6 mo
L maxilla L orbital wall Pineal Anterior fontanel L maxilla Premaxilla L maxilla L testicle Soft tissue L thigb
t Recurrence Malignant + Recurrence + Recurrence
Steinberg et al, 198836 Melissari et al, l98837 Stirling et al. 198864 Stirling et al. 1988@ Stirling et al. 1988M Cohen et al, 198865 Marrakchi et al, 198838 Handley et al, 198839 Dale & Harrop, 1987” Crockett et al, 198733 Russegger et al. 198735 Dourov et al. l98734
M M F M M ? ? F F F F F
2.5 mo 15 mo 6 wk 7 mo 6 mo 4.5 mo ? 4mo 2 mo 2mo
t Recurrence Unknown Unknown Unknown tRecurrence
7 mo
L maxilla L mandible Forehead Maxilla Paratesticular Pineal Unknown L maxilla L maxilla R maxilla Intracranial R premaxilla
Lee et al, 198626 Parizek et al, 1986” Denadai et al, 19%? Shokry et al, 1986** Ramadas et al, 1986 Lamping et al, l98523 Lamping et al, 198523 Lamping et al, 1985*’ Lamping et al, 198523 Lamping et al. 198523 Lamping et al, 198523 Carpenter et al, 1985*’ Young & Gonzalez-Crussi, 198524 Ricketts & Majmudarr, 1985** Nagase et al, 1983” Denneny et al, 1983@ Das et al, 198316 Johnson et al, 1983” Johnson et al, 1983” Johnson et al, 1983” Johnson et al, 198317 Johnson et al, 1983” Johnson et al, 1983” Johnson et al, 1983” Walsh & Strand, 198213
F F F F F M M F ? M F M M M M M M F M M M F M F M
3 mo 33 mo 8mo 2mo 5 mo 4mo 3 mo 2 mo 2 mo 6 mo 2.5 mo 4 mo 20 mo 3mo 4mo 1.5 mo 5 mo 18 mo 5 mo 5.5 mo 3 mo 3.5 mo 5mo 2.25 mo 3mo
R maxilla Skull episubdural R testicular R maxilla Premaxilla Skull Skull L maxilla L maxilla Premaxilla R maxilla Paraepididymal R metatarasal R epidiymis L maxilla L maxilla L maxilla L femur R temporal R epididymis R maxilla L maxilla L maxilla L maxilla R skull
Malik et al, 1982” Malik et al, 1982’* Jerrell & Hill, 1982 Jimenez et al, 198 I ”
F M F M
2mo 7mo 2 mo 6 mo
Cutler et al, 198 1’ Cutler et al, 198 1’ Hupp et al, I98 166 Block et al, 1980’ Shuangshoti, 19806 Palacios, 1980’ Bignold & Nicks, 19804 Gotcher et al, 19803 Ohne et al, 1980g Blank & Runckel, 1980*
F M M M M M F M F F
3 mo 5 mo 5 mo 4mo 6mo 11 yr 7 mo 3mo 4mo Newborn
Premaxilla R maxilla R mandible R facial soft tissue Anterior maxilla Anterior maxilla Mandible L maxilla Midbrain R mandible Anterior fontanel L maxilla R maxilla L maxilla
Author(s)
Histologic/Laboratory
Values
+NSE. -SlOO +(L-dopa) serum
+NSE, +SlOO tNSE, tKL1. tS100, tneurofilament. tvimentin tNSE, +GFAP +NSE. tS100
Unknown +Recurrence
-
turinary (VMA) (ta-fetoprotein) serum +a-fetoprotein +GFAP
Malignant
-
urine WNL
+Recurrence tRecurrence
urine WNL t(cY-fetoprotein) serum urine WNL
-
Unknown Malignant Metastatic +Recurrence +Recurrence t Recurrence
-
Unknown tRecurrence +Recurrence
urine WNL urine WNL urine WNL
urine WNL, t (alkaline phosphate) serum
urine WNL
Malignant Died 2 mo postop Died Unknown t Recurrence +Recurrence +Recurrence
Abbreviations: GFAP, glial fibrillary acidic protein; KLl, antikeratin antibody: NSE, neuron specific enolase: SIOO, SlOO protein; WNL, within normal limits.
887
888
MELANOTIC NEUROECTODERMAL
Table 2. Anatomical Location of MNTI Reported in the Literature
Site of Origin Maxilla Skull Mandible Brain Epididymis/testes Mediastinum Oropharynx Shoulder Scapula Thigh Zygomatic bone Ovary Uterus Maxillary sinus Femur Metatarsal Intradural Soft tissue Forehead Unknown Totals
Through 1979 (S)
1980-1989 (%)
Total Cases (%)
93 (68) 15 (11) 8 (6) 6 (4) 3 (2) 2 (1)
32 (53) 7 (12) 4 (7) 4 (7) 6 (10)
125 (64) 22(11) 12 (6) 10 (5) 9 (5) 2 (1) 1 (0.5) I (0.5) I (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 2 (1) 1 (0.5) 1 (0.5) 195
l(l) J(l) I(l) l(1)
l(l) l(1) l(1) l(1) 1 (2) 1 (2) 1 (2) 2 (3)
1 (2) 1 (2) 135
60
Data from Cutler et al.’
the midline to the area of the first deciduous molar. The tissue had a dark blue color. The mucosa overlying the area was thin and mobile. The majority of the swelling was present on the lateral aspect of the ridge, with minimal enlargement
TUMOR
over the crest and virtually none on the palate (Fig 1). There were no pulsations, bruits, or palpable warmth over the area. The remainder of the physical examination were within normal limits. The patient was scheduled for radiographs and a biopsy to be done in the operating room after the administration of a general anesthetic. Same-day surgery was planned. The differential diagnosis included 1) melanotic neuroectodermal tumor of infancy; 2) benign mesenchymal lesion: 3) hemangioma; 4) malignant mesenchymal lesion. SURGICAL
PROCEDURE
General anesthesia was induced via nasoendotracheal intubation. The patient was prepared and draped in the usual fashion for an orofacial procedure. A thorough evaluation was performed and radiographs were taken using dental and occlusal films. The radiographs showed numerous tooth buds and some radiolucency, but the exact size of the lesion could not be determined (Fig 2). Aspiration with an 1g-gauge needle was nonproductive. After injection of a local anesthesia with 1:400,000 epinephrine for hemostasis, an incision was made along the alveolar ridge from just right of the midline to just posterior to the area of the left second deciduous molar; 45” releasing incisions were made at both ends of the crestal incision. Mucoperiosteal flaps were mobilized and reflected revealing the surface of the lesion (Fig 3). An incisional biopsy was sent for frozen section, which confirmed the preoperative suspicion of melanotic neuroectodermal tumor of infancy. The lesion appeared to be encapsulated and an attempt was made to remove it by blunt dissection. It extended anteriorly from the maxillary midline into the left nasal aperture and posteriorly to the area ofthe deciduous first molar tooth. Superiorly it appeared to fill the maxillary sinus without
FIGURE I. A. View of the patient showing enlargement in the left infraorbital and maxillary areas present at the time of initial presentation. 8, Intraoral view at the time of the initial surgery showing expansion of the left maxilla from the midline to the area of the first deciduous molar. The lesion was pigmented and bluish in color. The mucosa overlying the area was thin but freely moveable. No enlargement on the palate was noted.
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MOSBY ET AL
positive ceils. The final diagnosis was melanotic neuroectodermal tumor of infancy of the left maxilla. Examination of the tumor by transmission electron microscopy showed three cell types based on their general morphology and cytoplasmic contents. The first cell type, and most numerous, was the least differentiated. These cells were relatively large and polygonal in shape. The plasma membranes were poorly defined, but their nuclei were well defined and rounded. The cytoplasm was characterized by an abundance of free ribosomes and microfilaments. Cells were often associated with an incompletely formed or disrupted basal lamina. The second cell type had the features of a fibroblast with an elongated cell body and was found in sheets of cells arranged in parallel alignment. Their nuclei were also elongated and exhibited evenly dispersed clumped chromatin. The cytoplasm contained few mitochondria, slightly dilated rough endoplasmic reticulum, and only rarely were Gollgi bodies identified. This cell type did not contain pigment granules, nor was it associated with a basal lamina. Of the three cell types observed, the third cell type appeared the most differentiated. It was generally cuboidal in shape with a nucleus of irregular outline (Fig 5). The cytoplasm featured numerous mitochondria, ribosomes. rough endoplasmic reticulum, microfilaments, and collections of immature melanosomes. This melanotic cell type was usually associated with a well-defined basal lamina. FIGURE 2. Lateral radiograph taken after induction of anesthesia showing the expansiveness of the lesion and the partially developed deciduous tooth buds floating within the tumor. The lesion appears to blend with the maxilla and lacks defined borders.
causing bony expansion. The only bony expansion visible was in the area of the lateral aspect of the left maxillary alveolus extending from the lateral incisor to the deciduous first molar tooth. The lesion and a separate extension of the lesion. which occurred more palatal, were enucleated. The left anterior maxillary buccal cortex was widely expanded, with firm bony margins on all sides, and osteoplasty was required. Wound closure was accomplished using 4-O interrupted polyglycolic acid (EGA) sutures, with inversion of the knots so as not to interfere with feeding and to prevent them from being untied by the patient’s tongue. Estimated blood loss was 40 mL.
POSTOPERATIVE COURSE The patient was kept in the hospital overnight and sent home the following morning. The parents were instructed on the use of a Breck feeder and the infant was noted to be feeding well. There was minimal swelling and the mucosa was intact. Routine follow-up was scheduled.
SURGICAL PATHOLOGY REPORT The specimen consisted of three portions. The first was approximately 2 cm in diameter, the second measured about 1 cm in diameter, and the third was a small tooth. All the soft tissues were firm and pigmented. Two teeth and a small fragment of bone were noted in the largest specimen. Microscopic examinations showed a neoplasm composed of two distinct tumor cell types within a fibrous stroma. The larger, cuboidal to low columnar cells typically lined irregular alveolar spaces of varying sizes. These cells often contained a brownish pigment consistent with melanin. Within these alveolar spaces, or in adjacent nests, were clusters of slightly smaller, ovoid, basophilic cells. These cells had scant cytoplasm, round nuclei with a fine chromatin pattern, and resembled neuroblasts. Mitotic figures were noticeably absent (Fig 4). Special stains for melanin were positive and immunoperoxidase staining for chromatin revealed occasional
F’IGURE 3. Intmopemtive view after the mucosa had been reflected showing the smooth, glistening, lobulated surface of the tumor. The mucosa was easily separated and reflected from the surface of the lesion.
890
MELANOTIC NEUROECTODERMAL TUMOR
FIGURE 4. A, Photomicrograph showing typical histologic features of alveolar spaces lined with cuboidal cells and often containing melanin. The more deeply basophihc neuroblastlike cells are in separate clusters (hematoxylin-eosin stain, original magnification X100). B, Higher magnification showing the distinct types of tumor cells and the surrounding loose fibrous connective tissue (hematoxylin-eosin stain, original magnification X250).
The patient did well postoperatively for approximately 6 weeks, at which time a small swelling was noted on the lateral aspect of the alveolar ridge in the area of the left first deciduous molar (Fig 6). The supposition was that this represented recurrence and a CT scan was obtained. The CT scan showed an expansile lesion of the left half of the maxilla that was composed of two portions separated by a bony septum (Fig 7). The more anterior portion was approximately 1.5 cm in diameter and the posterior portion was 2 cm in diameter. There appeared to be two incompletely formed teeth associated with the posterior component. The contents were of low density, but not fluid. There was a small aerated sinus in the right maxilla, but no sinus in the left maxilla. There did not appear to be any distortion of the floor of the orbit. The pterygoid plates were normal and there was no encroachment on the nasal cavity. There was slight
deformity of the left side of the oropharynx caused by the expanded maxilla. On the basis of the CT scan and the clinical history, it was assumed that this was recurrent MNTI.
SURGICAL PROCEDURE
The patient was taken to the operating room, a general anesthetic was administered, and she was prepared and draped in the usual fashion for an orofacial procedure. Incisions were planned that would allow for the tissue directly overlying the lesion to be removed with the lesion; vertical release incisions were made at both ends. Mucoperiosteal flaps were elevated and, after all bony margins were identified, the lesion was enucleated from the surrounding bone (Fig 8). Any bone that
FIGURE 5. Electron micrograph showing a characteristic cuboidal-shaped cell that has features of both an epithelial and melanotic cell. SmaH arrows indicate basal lamina. Cytoplasm contains numerous immature melanosomes (large arrow and inset). (Bar = 2 pm, original X5,600; inset magnification original magnification X 18,000).
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MOSBY ET AL
FIGURE 6. Computed tomography scans in the coronal (A) and axial (B) planes showing the tumor in the left maxilla. These images were taken approximately 2 months after the initial surgical procedure. The tumor appears to be bilobulated in an anteroposterior direction and to extend to the pterygoid plate. In the superior aspect, extension appears to go to the orbital floor. A developing tooth is visible within the lesion.
had unusual pigmentation, color, or texture was removed 3 mm past the involved area. The surrounding bony margins were smoothed, the surgical site debrided, and the flaps closed primarily with interrupted 4-O PGA resorbable sutures. There was an estimated blood loss of 260 mL. The hemoglobin was 9 g/dL and the hematocrit was 27% intraoperatively. The patient did well in the operating room and in the recovery room. She received intravenous fluids postoperatively to supplement for her blood loss and by the next day her hemoglobin and hematocrit were 10.2 g/dL and 30.8%, respectively. She was taking formula with a Breck feeder and a straw when discharged the following day.
SURGICAL
PATHOLGGY
Discussion The melanotic neuroectodermal tumor of infancy occurs primarily in the young child, with the majority developing in children 1 year old or younger. Recurrence rates have been reported to be as high as 10% to 15%. Clinical evaluation of the lesions shows that they are firm and well demarcated. They are reported to progress by local extension rather than metastasis, although some in both the maxilla and the femur have been reported to be malignant.14 Microscopic exami-
REPORT
Representative portions of the surgical specimen were submitted to Children’s Mercy Hospital, Kansas City; Cytogenetics Laboratory, Los Angeles; Los Angeles Children’s Hospital; The Oral Pathology Department at the University of Missouri-Kansas City. A portion was also quick-frozen and submitted to the Molecular Biology Laboratory. The histologic appearance of the tumor from the second surgery was found to be similar to that of the original lesion.
POSTSURGICAL
FOLLOW-UP
The patient’s recovery was uneventful and healing progressed satisfactorily. Monthly visits showed no further enlargement of the maxilla or change in facial contour. There also was no discoloration of the oral mucosa. A maxillary tooth was noted to be erupting just to the right ofthe midline, but no teeth were seen to the left of the midline. Six months after the second surgical procedure a CT scan was obtained that showed no evidence of residual or recurrent tumor, nor any abnormal areas of enhancement (Fig 9).
FIGURE 7. Appearance of the lesion at the second surgical procedure. The mucosa is thin and freely mobile. The coloration is similar to that at initial presentation 2 months before.
892
FIGURE 8. Surgical specimen from the second surgery showing the superior aspect of the tumor.
nation shows three basic cell types: melanin-producing cells of epithelial origin, undifferentiated cells of neuroblastic origin, and stromal fibroblasts. Electron microscopy shows pigmented cells that are characterized by the presence of four types of membrane-bound pigment granules corresponding to melanosomes at different stages of maturation.38 Magnetic resonance imaging and CT scans are not pathognomonic, but may be helpful in diagnosing melanotic neuroectodermal tumors when combined with a thorough clinical examination and a high urinary excretion of VMA.56 The parents of this patient understood the potentially aggressive nature of the lesion and the need for removal of primary and/or permanent tooth buds that were included in the lesion or in the area adjacent to the lesion.
MELANOTIC NEUROECTODERMAL
TUMOR
They were told that if the lesion was benign it would be removed at the initial surgery, but if frozen sections revealed a malignancy, further studies would be indicated. They gave their oral and written consent to proceed. Before the second surgical procedure, the parents were advised regarding the aggressive nature of the recurrent lesion and that the possibility of malignancy had to be considered. They were aware that the lesion had a reported 15% recurrence rate. The indications for another surgery and all possible complications were discussed and they were aware that a 3 to 5 mm margin of bone would be removed as well as all primary and permanent tooth buds associated with the lesion. They also knew that there would be a maxillary deformity and probably no primary or permanent teeth would ever erupt. The possibility of oroantral or oronasal communication was explained, as was the need for further and more extensive reconstructive surgery in the future. Both surgeries were done very carefully and the surgeons were certain that the tumor was removed completely each time. The speed with which new tumor revealed itself, however, leads us to believe that this is a case of residual tumor rather than recurrence. This lesion is believed to be benign; however, long-term follow-up will be needed to see if further tumor develops. Acknowledgment The authors wish to thank Charles Dunlap, DDS, Professor and Chairman, Department of Oral Pathology, for his assistance with the histologic evaluation. Dr Dunlap is on the faculty of the School of Dentistry at the University of Missouri-Kansas City, Kansas City, MO.
FIGURE 9. Coronal (A) and axial (B) computerized tomographic scans 6 months subsequent to the second surgical procedure showing no evidence of residual or recurrent tumor.
MOSBY ET AL
References I. Cutler LS, Chaudhrey AP, Topazian R: Melanotic neuroectodermal tumor of infancy: An ultrastructure study, literature review, and reevaluation. Cancer 48257, 198 I 2. Blank E, Runckel DN: Case report 119. Skeletal Radio1 5:179, 1980 3. Gotcher JE, Jaffrey BJ, Hudson JW, et al: Recurrent melanotic neuroectodermal tumor of infancy: Report of case and tumor heterotransplantation studies. J &al Surg 38:702. 1980 4. Bianold LP. Nicks R: Melanotic neuroectodermal tumor of infancy arising in anterior fontanelle. Pathology I2:473, 1980 5. Palacios JJN: Malignant melanotic neuroectodermal tumor: Light and electron microscopic study. Cancer 46:529, 1980 6. Shuangshoti S: Melanotic mucin-producing neuroepithelial neoplasm of mesencephalon with consideration of similar tumours in different locations. J Neurol Neurosurg Psychiatry 43:810, 1980 7. Block JC, Waite DE, Dehner LP, et al: Pigmented neuroectodermal tumor of infancy: An example of rarely expressed malignant behavior. Oral Surg Oral Med Oral Pathol 49:279, 1980 8. Mullins JD: A pigmented differentiating neuroblastoma. Cancer 46522, 1980 9. Ohne H, Tokunaga S, Sumiya N: A case of melanoameloblastoma found in the anterior maxilla of an infant (in Japanese). Jpn J Plast Reconstr Surg 23:283, 1980 10. Jimenez JF, Brown RE, Seibert RW, et al: Melanotic neuroectodermal tumor of infancy and fetal hydantoin syndrome. Am J Pediatr Hematol Oncol 3:9, 198 1 11. Tobo M, Sumiyoshi A, Yamakawa Y: Sellar teratoma with melanotic progonoma. Acta Neuropathol 55:7 I. I98 I 12. Malik GB. Mohindra S. Aaarwal S: Melanotic neuroectodermal tumor of infancy. Indian J Cancer 19:223, 1982 13. Walsh JW, Strand RD: Melanotic neuroectodermal tumor of the neurocranium in infancy. Child’s Brain 9:329, 1982 14. Johnson RE, Scheithauer BW, Dahlin DC Melanotic neuroectodermal tumor of infancy: A malignant tumor of the femur. Mayo Clin Proc 57:719, 1982 15. Curtis JL. Rubinstein LJ: Pigmented olfactory neuroblastoma: A new example of melanotic neuroepithelial neoplasm. Cancer 49:2136, 1982 16. Das AK, Mukherjee AH, Kar NK: Melanotic neuroectodermal tumor of infancy. Indian J Cancer 20:82, 1983 17. Johnson RE, Scheithauer BW, Dahlin DC: Melanotic neuroectoderrnal tumor of infancy: A review of seven cases. Cancer 52:661, 1983 18. Nagase M, Udea K. Fukushima M, et al: Recurrent melanotic neuroectodermal tumor of infancy: Case report and survey of 16 cases. J Maxillofac Surg I I : I3 I, 1983 19. Denneny III JC, Handler SD, Rorke LB: Melanotic neuroectodermal tumor of infancy. Trans Pa Acad Ophthalmol Otolaryngol36:89, 1983 20. Bourdiniere CJ, LeClech G, deBraquilanges E, et al: Le progonome melanotique. A propos de deux cas. J Fr Otorhinolaryngol32:311 I, 1983 2 1. Carpenter BF, Jimenez C: Melanotic neuroectodermal tumor of infancy. Pediatr Path01 3:227, 1985 22. Ricketts RR, Majmudarr B: Epididymal melanotic neuroectodermal tumor of infancy. Hum Pathol I6:4 16, 1985 23. Lamping KA, Albert DM, Lack E, et al: Melanotic neuroectodermal tumor of infancy. Ophthalmology 92: 143. 1985 24. Young S, Gonzalez-Crussi F: Melanotic Neuroectodermal tumor of the foot: Report of a case with multicentric origin. Am J Clin Pathol 84:371, 1985 25. King ME, Mouradian JA, Micha JP, et al: Immature teratoma of the ovary with predominant malignant retinal analage component. Am J Surg Pathol 9:221. 1985 26. Lee CH, Hong SP, Lim CY: Melanotic neuroectodermal tumor of infancy. J Korean Med Sci 1:63, 1986 27. Parizek J, Nemecek S, Cernoch Z, et al: Melanotic neuroectodermal neurocranial tumor of infancy of extra-intra-and sub-
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53. Halpert B, Patzer R: Maxillary tumor of retinal anlage. Surgery 22837, 1947 54. Caldwell JB, Ernst KF, Thompson HC: Retinal anlage tumor of the maxilla. Oral Surg 8:796, 1955 55. Misugi K: Mediastinal origin of a melanotic progonoma or retinal anlage tumor: Ultra-structural evidence for neural crest origin. Cancer 18477, 1956 56. Bore110ED, Gorlin RJ: Melanotic neuralectodermal tumor of infancy-a neoplasm of neural crest origin. Report of a case associated with high urinary excretion of vamillmandelic acid. Cancer 19:196, 1966 57. Nikai H, I_juhinN, Yamasaki A, et al: Ultrastructural evidence for neural crest origin of the melanotic neuroectodetmal tumor of infancy. J Oral Path01 6:22 I, 1977 58. Dehner LP, Sibley RK, Sank JJ, et al: Malignant melanotic neuroectodermal tumor of infancy. Cancer 43: 1389, 1979 59. Ashley OJB: Melanotic Adamantinoma of the skull. J Path01 Bacterial 87: 179. 1964
J Oral Maxillofac 50:994-998.
TUMOR OF INFANCY
60. Hahn JF, Sperber EE, Netsky MG: Melanotic neuroectodermal tumors of the brain and skull. J Neuropathol Exp Neural 35: 508, 1976 6 I. McCloskey JJ, Parker PC, Brooks WH, et al: Melanin as a component of cerebral gliomas: The rnelanotic cerebral ependymoma. Cancer 37:2373, 1976 62. Dehner LP: Peripheral and central neuroectodermal tumors. Arch Path01 Lab Med 110:997, 1986 63. Schulz DH: A malignant melanotic neoplasm of the uterus resembling the retinal anlage tumors. Am J Clin Path01 28524, 1957 64. Stirling RW, Powell G, Fletcher CDM: Pigmented neuroectodermal of infancy: An immunohistochemical study. Histopathology 12:425. 1988 65. Cohen BH, Handler MS, De Viro DC, et al: Central nervous system melanotic neuroectodermal tumor of infancy: Value of chemotherapy in management. Neurology 38: 163, 1988 66. Hupp JR, Topazian RG, Krutchkoff DJ: The melanotic neuroectodermal tumor of infancy. Int J Oral Surg 10:432. 198 1
Surg
1992
Melanotic Neuroectodermal Tumor of Infancy: A Report of Two Cases PETER
N. DEMAS,
DMD, MD,* THOMAS W. BRAUN, AND M.M. NAZIF, DDS, MDS*
DMD, PHD,t
Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively rare tumor of the anterior maxilla in young infants. The lesion often has bluish areas of pigmentation and is characterized by displacement of involved tooth buds and localized aggressiveness.‘d Historically, several names for this lesion have been proposed in the literature, including pigmented congenital epulis, melanotic progonoma, retinal analage
* Associate Professor, Department of Oral/Maxillofacial Surgery, University of Pittsburgh Medical Center and Veterans Administration Medical Center, Pittsburgh, PA. t Chairman, Department Oral/Maxillofacial Surgery, University of Pittsburah Medical Center and Veterans Administration Medical Center, Pi&burgh, PA. $ Director of Dental Services, Children’s Hospital of Pittsburgh, Pittsburgh, PA. Address correspondence and reprint requests to Dr Braun: Department of Oral and Maxillofacial Surgery, University of Pittsburgh School of Dental Medicine, 350 1 Terrace St, Pittsburgh, PA 1526 1. 0 1992 American Association of Oral and Maxillofacial Surgeons 0278-2391/92/5008-0017$3.00/O
FIGURE 1. Preoperative appearance of the expanded left maxilla.
Surg
1992
Melanotic Neuroectodermal Tumor of Infancy: Review of the Literature and Report of a Case EDWARD L. MOSBY, DDS, FACD,* MICHAEL W. LOWE, DDS,t CHARLES M. COBB, DDS, PHD,$ AND ROBERT L. ENNIS, DOSS
The melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor. We were able to find only 195 cases reported in the world literature through 19891-46 (Table 1). Carpente?’ and Parizek” proposed several hypotheses for the origin of MNTI; these included congenital melanocarcinoma of the alveolar procesq4’ odontogenic epithelium,48q49and a phylogenetic origin related to the median or pineal eye of particular lower vertebrates.50%51Another phylogenetic theory is concerned with displaced neuroectodermal tissue from the vestigial organ of Jacobson, ie, vomeronasal organ.52 Retinal tissue has also been suggested as a source of MNTI.S3,54However, the neural crest is the most commonly accepted tissue of origin for MNTI.55-58 MNTI primarily occurs mainly in the maxilla, but can occur in other areas of the skull, the mandible, brain, and epididymis, as well as other anatomic locations59-62(Table 2). It classically appears as a painless, darkened, expansion of the maxillary alveolar region, with a history of accelerated growth within the preceding few weeks to months potentially interfering with nursing. It is usually benign; only 11 cases were reported as being malignant.‘7,25,28,4’.58,63 Conventional radiographs of intrabony lesions usually show a radiolucency with or without irregular
margins. Computed tomography (CT) typically reveals hyperdense masses, but hypodense growths also have been reported.32245Magnetic resonance imaging (MRI) shows a hypointense mass, with focal areas of hyperintensity in T,-weighted images and an isointense mass on T2-weighted images.42 Histologically, MNTI consists of a biphasic population of epithelioid melanin-containing cells and neuroblastlike cells within a prominent stroma.37s43,64The melanin-containing cells can be cuboidal and arranged in alveolar, pseudoalveolar, or glandlike structures.38 The treatment for MNTI has typically been surgical and includes enucleation and/or curettage with limited, wide, or en bloc excision. Cases of surgically unmanageable MNTI have received chemotherapy,2q42 chemotherapy with radiation therapy,28.65 and radiation therapy.5 Debulking procedures also have been used.66 The recurrence rate of MNTI remains approximately 15%. Report of a Case The patient, a 4%month-old white girl, was referred to the oral and maxillofacial surgery service at Truman Medical
Center by one of the authors (R.E.) for diagnosis and treatment. The parents had noted swelling in the mouth and along the left side of the child’s nose approximately 6 weeks before seeking consultation, at which time the child was about 2 months old. They noted that the swelling was episodic, but their concern increased over the past 2 weeks as the swelling progressively enlarged and caused noticeable distortion of the left side of the face. They also reported that their daughter was having increasing difficulty in taking oral feedings. The child’s past medical history was totally unremarkable except for the chief complaint. She was taking no medications other than vitamins. The parents stated that the child had not had a fever during the period of the sporadic swelling. No allergies were reported. Physical examination did not show any abnormalities other than those related to the chief complaint. There was enlargement of the left face that caused superomedial displacement of the left alar base. No abnormalities were noted on the right side. Intraorally, there was expansion of the left maxilla from
Received from the Department of Hospital Dentistry, Oral and Maxillofacial Surgery Graduate Training Program, University of Missouri-Kansas City Schools of Dentistry and Medicine, Truman Medical Center, Kansas City, MO. * Professor, Oral and Maxillofacial Surgery; Associate Director Oral and Maxillofacial Surgery Residency. t Resident. $ Professor, Periodontology and Oral Biology. 9 Clinical Associate Professor; in private practice, Oral and Maxillofacial Surgery, Independence, MO. Address correspondence and reprint requests to Dr Mosby: Department of Hospital Dentistry, University of Missouri-Kansas City Schools of Dentistry and Medicine, Truman Medical Center, 2301 Holmes St, Kansas City, MO 64108. 0 1992 American
Association
of Oral and Maxillofacial
Surgeons
0278-2391/92/5008-0016$3.00/O
886
Table 1.
Renortr of MNTl from 1980 to Present Recurrence/ Malignant
Location
Sex
Age
Mosby et al. 1992 Prasad et al, 198940 Ogata et al. 19894’ Atkinson et al 198942 Turner et al, 1$89” Turner et al, 198944 Claros et al, 198945 Murayma et al. 198946 Scheck et al, I 98943
F M F F F M M M F
4.5 mo 6 mo 4 yr 2 mo 2.5 mo 3 mo 2 mo 10 mo 6 mo
L maxilla L orbital wall Pineal Anterior fontanel L maxilla Premaxilla L maxilla L testicle Soft tissue L thigb
t Recurrence Malignant + Recurrence + Recurrence
Steinberg et al, 198836 Melissari et al, l98837 Stirling et al. 198864 Stirling et al. 1988@ Stirling et al. 1988M Cohen et al, 198865 Marrakchi et al, 198838 Handley et al, 198839 Dale & Harrop, 1987” Crockett et al, 198733 Russegger et al. 198735 Dourov et al. l98734
M M F M M ? ? F F F F F
2.5 mo 15 mo 6 wk 7 mo 6 mo 4.5 mo ? 4mo 2 mo 2mo
t Recurrence Unknown Unknown Unknown tRecurrence
7 mo
L maxilla L mandible Forehead Maxilla Paratesticular Pineal Unknown L maxilla L maxilla R maxilla Intracranial R premaxilla
Lee et al, 198626 Parizek et al, 1986” Denadai et al, 19%? Shokry et al, 1986** Ramadas et al, 1986 Lamping et al, l98523 Lamping et al, 198523 Lamping et al, 1985*’ Lamping et al, 198523 Lamping et al. 198523 Lamping et al, 198523 Carpenter et al, 1985*’ Young & Gonzalez-Crussi, 198524 Ricketts & Majmudarr, 1985** Nagase et al, 1983” Denneny et al, 1983@ Das et al, 198316 Johnson et al, 1983” Johnson et al, 1983” Johnson et al, 1983” Johnson et al, 198317 Johnson et al, 1983” Johnson et al, 1983” Johnson et al, 1983” Walsh & Strand, 198213
F F F F F M M F ? M F M M M M M M F M M M F M F M
3 mo 33 mo 8mo 2mo 5 mo 4mo 3 mo 2 mo 2 mo 6 mo 2.5 mo 4 mo 20 mo 3mo 4mo 1.5 mo 5 mo 18 mo 5 mo 5.5 mo 3 mo 3.5 mo 5mo 2.25 mo 3mo
R maxilla Skull episubdural R testicular R maxilla Premaxilla Skull Skull L maxilla L maxilla Premaxilla R maxilla Paraepididymal R metatarasal R epidiymis L maxilla L maxilla L maxilla L femur R temporal R epididymis R maxilla L maxilla L maxilla L maxilla R skull
Malik et al, 1982” Malik et al, 1982’* Jerrell & Hill, 1982 Jimenez et al, 198 I ”
F M F M
2mo 7mo 2 mo 6 mo
Cutler et al, 198 1’ Cutler et al, 198 1’ Hupp et al, I98 166 Block et al, 1980’ Shuangshoti, 19806 Palacios, 1980’ Bignold & Nicks, 19804 Gotcher et al, 19803 Ohne et al, 1980g Blank & Runckel, 1980*
F M M M M M F M F F
3 mo 5 mo 5 mo 4mo 6mo 11 yr 7 mo 3mo 4mo Newborn
Premaxilla R maxilla R mandible R facial soft tissue Anterior maxilla Anterior maxilla Mandible L maxilla Midbrain R mandible Anterior fontanel L maxilla R maxilla L maxilla
Author(s)
Histologic/Laboratory
Values
+NSE. -SlOO +(L-dopa) serum
+NSE, +SlOO tNSE, tKL1. tS100, tneurofilament. tvimentin tNSE, +GFAP +NSE. tS100
Unknown +Recurrence
-
turinary (VMA) (ta-fetoprotein) serum +a-fetoprotein +GFAP
Malignant
-
urine WNL
+Recurrence tRecurrence
urine WNL t(cY-fetoprotein) serum urine WNL
-
Unknown Malignant Metastatic +Recurrence +Recurrence t Recurrence
-
Unknown tRecurrence +Recurrence
urine WNL urine WNL urine WNL
urine WNL, t (alkaline phosphate) serum
urine WNL
Malignant Died 2 mo postop Died Unknown t Recurrence +Recurrence +Recurrence
Abbreviations: GFAP, glial fibrillary acidic protein; KLl, antikeratin antibody: NSE, neuron specific enolase: SIOO, SlOO protein; WNL, within normal limits.
887
888
MELANOTIC NEUROECTODERMAL
Table 2. Anatomical Location of MNTI Reported in the Literature
Site of Origin Maxilla Skull Mandible Brain Epididymis/testes Mediastinum Oropharynx Shoulder Scapula Thigh Zygomatic bone Ovary Uterus Maxillary sinus Femur Metatarsal Intradural Soft tissue Forehead Unknown Totals
Through 1979 (S)
1980-1989 (%)
Total Cases (%)
93 (68) 15 (11) 8 (6) 6 (4) 3 (2) 2 (1)
32 (53) 7 (12) 4 (7) 4 (7) 6 (10)
125 (64) 22(11) 12 (6) 10 (5) 9 (5) 2 (1) 1 (0.5) I (0.5) I (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 1 (0.5) 2 (1) 1 (0.5) 1 (0.5) 195
l(l) J(l) I(l) l(1)
l(l) l(1) l(1) l(1) 1 (2) 1 (2) 1 (2) 2 (3)
1 (2) 1 (2) 135
60
Data from Cutler et al.’
the midline to the area of the first deciduous molar. The tissue had a dark blue color. The mucosa overlying the area was thin and mobile. The majority of the swelling was present on the lateral aspect of the ridge, with minimal enlargement
TUMOR
over the crest and virtually none on the palate (Fig 1). There were no pulsations, bruits, or palpable warmth over the area. The remainder of the physical examination were within normal limits. The patient was scheduled for radiographs and a biopsy to be done in the operating room after the administration of a general anesthetic. Same-day surgery was planned. The differential diagnosis included 1) melanotic neuroectodermal tumor of infancy; 2) benign mesenchymal lesion: 3) hemangioma; 4) malignant mesenchymal lesion. SURGICAL
PROCEDURE
General anesthesia was induced via nasoendotracheal intubation. The patient was prepared and draped in the usual fashion for an orofacial procedure. A thorough evaluation was performed and radiographs were taken using dental and occlusal films. The radiographs showed numerous tooth buds and some radiolucency, but the exact size of the lesion could not be determined (Fig 2). Aspiration with an 1g-gauge needle was nonproductive. After injection of a local anesthesia with 1:400,000 epinephrine for hemostasis, an incision was made along the alveolar ridge from just right of the midline to just posterior to the area of the left second deciduous molar; 45” releasing incisions were made at both ends of the crestal incision. Mucoperiosteal flaps were mobilized and reflected revealing the surface of the lesion (Fig 3). An incisional biopsy was sent for frozen section, which confirmed the preoperative suspicion of melanotic neuroectodermal tumor of infancy. The lesion appeared to be encapsulated and an attempt was made to remove it by blunt dissection. It extended anteriorly from the maxillary midline into the left nasal aperture and posteriorly to the area ofthe deciduous first molar tooth. Superiorly it appeared to fill the maxillary sinus without
FIGURE I. A. View of the patient showing enlargement in the left infraorbital and maxillary areas present at the time of initial presentation. 8, Intraoral view at the time of the initial surgery showing expansion of the left maxilla from the midline to the area of the first deciduous molar. The lesion was pigmented and bluish in color. The mucosa overlying the area was thin but freely moveable. No enlargement on the palate was noted.
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MOSBY ET AL
positive ceils. The final diagnosis was melanotic neuroectodermal tumor of infancy of the left maxilla. Examination of the tumor by transmission electron microscopy showed three cell types based on their general morphology and cytoplasmic contents. The first cell type, and most numerous, was the least differentiated. These cells were relatively large and polygonal in shape. The plasma membranes were poorly defined, but their nuclei were well defined and rounded. The cytoplasm was characterized by an abundance of free ribosomes and microfilaments. Cells were often associated with an incompletely formed or disrupted basal lamina. The second cell type had the features of a fibroblast with an elongated cell body and was found in sheets of cells arranged in parallel alignment. Their nuclei were also elongated and exhibited evenly dispersed clumped chromatin. The cytoplasm contained few mitochondria, slightly dilated rough endoplasmic reticulum, and only rarely were Gollgi bodies identified. This cell type did not contain pigment granules, nor was it associated with a basal lamina. Of the three cell types observed, the third cell type appeared the most differentiated. It was generally cuboidal in shape with a nucleus of irregular outline (Fig 5). The cytoplasm featured numerous mitochondria, ribosomes. rough endoplasmic reticulum, microfilaments, and collections of immature melanosomes. This melanotic cell type was usually associated with a well-defined basal lamina. FIGURE 2. Lateral radiograph taken after induction of anesthesia showing the expansiveness of the lesion and the partially developed deciduous tooth buds floating within the tumor. The lesion appears to blend with the maxilla and lacks defined borders.
causing bony expansion. The only bony expansion visible was in the area of the lateral aspect of the left maxillary alveolus extending from the lateral incisor to the deciduous first molar tooth. The lesion and a separate extension of the lesion. which occurred more palatal, were enucleated. The left anterior maxillary buccal cortex was widely expanded, with firm bony margins on all sides, and osteoplasty was required. Wound closure was accomplished using 4-O interrupted polyglycolic acid (EGA) sutures, with inversion of the knots so as not to interfere with feeding and to prevent them from being untied by the patient’s tongue. Estimated blood loss was 40 mL.
POSTOPERATIVE COURSE The patient was kept in the hospital overnight and sent home the following morning. The parents were instructed on the use of a Breck feeder and the infant was noted to be feeding well. There was minimal swelling and the mucosa was intact. Routine follow-up was scheduled.
SURGICAL PATHOLOGY REPORT The specimen consisted of three portions. The first was approximately 2 cm in diameter, the second measured about 1 cm in diameter, and the third was a small tooth. All the soft tissues were firm and pigmented. Two teeth and a small fragment of bone were noted in the largest specimen. Microscopic examinations showed a neoplasm composed of two distinct tumor cell types within a fibrous stroma. The larger, cuboidal to low columnar cells typically lined irregular alveolar spaces of varying sizes. These cells often contained a brownish pigment consistent with melanin. Within these alveolar spaces, or in adjacent nests, were clusters of slightly smaller, ovoid, basophilic cells. These cells had scant cytoplasm, round nuclei with a fine chromatin pattern, and resembled neuroblasts. Mitotic figures were noticeably absent (Fig 4). Special stains for melanin were positive and immunoperoxidase staining for chromatin revealed occasional
F’IGURE 3. Intmopemtive view after the mucosa had been reflected showing the smooth, glistening, lobulated surface of the tumor. The mucosa was easily separated and reflected from the surface of the lesion.
890
MELANOTIC NEUROECTODERMAL TUMOR
FIGURE 4. A, Photomicrograph showing typical histologic features of alveolar spaces lined with cuboidal cells and often containing melanin. The more deeply basophihc neuroblastlike cells are in separate clusters (hematoxylin-eosin stain, original magnification X100). B, Higher magnification showing the distinct types of tumor cells and the surrounding loose fibrous connective tissue (hematoxylin-eosin stain, original magnification X250).
The patient did well postoperatively for approximately 6 weeks, at which time a small swelling was noted on the lateral aspect of the alveolar ridge in the area of the left first deciduous molar (Fig 6). The supposition was that this represented recurrence and a CT scan was obtained. The CT scan showed an expansile lesion of the left half of the maxilla that was composed of two portions separated by a bony septum (Fig 7). The more anterior portion was approximately 1.5 cm in diameter and the posterior portion was 2 cm in diameter. There appeared to be two incompletely formed teeth associated with the posterior component. The contents were of low density, but not fluid. There was a small aerated sinus in the right maxilla, but no sinus in the left maxilla. There did not appear to be any distortion of the floor of the orbit. The pterygoid plates were normal and there was no encroachment on the nasal cavity. There was slight
deformity of the left side of the oropharynx caused by the expanded maxilla. On the basis of the CT scan and the clinical history, it was assumed that this was recurrent MNTI.
SURGICAL PROCEDURE
The patient was taken to the operating room, a general anesthetic was administered, and she was prepared and draped in the usual fashion for an orofacial procedure. Incisions were planned that would allow for the tissue directly overlying the lesion to be removed with the lesion; vertical release incisions were made at both ends. Mucoperiosteal flaps were elevated and, after all bony margins were identified, the lesion was enucleated from the surrounding bone (Fig 8). Any bone that
FIGURE 5. Electron micrograph showing a characteristic cuboidal-shaped cell that has features of both an epithelial and melanotic cell. SmaH arrows indicate basal lamina. Cytoplasm contains numerous immature melanosomes (large arrow and inset). (Bar = 2 pm, original X5,600; inset magnification original magnification X 18,000).
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MOSBY ET AL
FIGURE 6. Computed tomography scans in the coronal (A) and axial (B) planes showing the tumor in the left maxilla. These images were taken approximately 2 months after the initial surgical procedure. The tumor appears to be bilobulated in an anteroposterior direction and to extend to the pterygoid plate. In the superior aspect, extension appears to go to the orbital floor. A developing tooth is visible within the lesion.
had unusual pigmentation, color, or texture was removed 3 mm past the involved area. The surrounding bony margins were smoothed, the surgical site debrided, and the flaps closed primarily with interrupted 4-O PGA resorbable sutures. There was an estimated blood loss of 260 mL. The hemoglobin was 9 g/dL and the hematocrit was 27% intraoperatively. The patient did well in the operating room and in the recovery room. She received intravenous fluids postoperatively to supplement for her blood loss and by the next day her hemoglobin and hematocrit were 10.2 g/dL and 30.8%, respectively. She was taking formula with a Breck feeder and a straw when discharged the following day.
SURGICAL
PATHOLGGY
Discussion The melanotic neuroectodermal tumor of infancy occurs primarily in the young child, with the majority developing in children 1 year old or younger. Recurrence rates have been reported to be as high as 10% to 15%. Clinical evaluation of the lesions shows that they are firm and well demarcated. They are reported to progress by local extension rather than metastasis, although some in both the maxilla and the femur have been reported to be malignant.14 Microscopic exami-
REPORT
Representative portions of the surgical specimen were submitted to Children’s Mercy Hospital, Kansas City; Cytogenetics Laboratory, Los Angeles; Los Angeles Children’s Hospital; The Oral Pathology Department at the University of Missouri-Kansas City. A portion was also quick-frozen and submitted to the Molecular Biology Laboratory. The histologic appearance of the tumor from the second surgery was found to be similar to that of the original lesion.
POSTSURGICAL
FOLLOW-UP
The patient’s recovery was uneventful and healing progressed satisfactorily. Monthly visits showed no further enlargement of the maxilla or change in facial contour. There also was no discoloration of the oral mucosa. A maxillary tooth was noted to be erupting just to the right ofthe midline, but no teeth were seen to the left of the midline. Six months after the second surgical procedure a CT scan was obtained that showed no evidence of residual or recurrent tumor, nor any abnormal areas of enhancement (Fig 9).
FIGURE 7. Appearance of the lesion at the second surgical procedure. The mucosa is thin and freely mobile. The coloration is similar to that at initial presentation 2 months before.
892
FIGURE 8. Surgical specimen from the second surgery showing the superior aspect of the tumor.
nation shows three basic cell types: melanin-producing cells of epithelial origin, undifferentiated cells of neuroblastic origin, and stromal fibroblasts. Electron microscopy shows pigmented cells that are characterized by the presence of four types of membrane-bound pigment granules corresponding to melanosomes at different stages of maturation.38 Magnetic resonance imaging and CT scans are not pathognomonic, but may be helpful in diagnosing melanotic neuroectodermal tumors when combined with a thorough clinical examination and a high urinary excretion of VMA.56 The parents of this patient understood the potentially aggressive nature of the lesion and the need for removal of primary and/or permanent tooth buds that were included in the lesion or in the area adjacent to the lesion.
MELANOTIC NEUROECTODERMAL
TUMOR
They were told that if the lesion was benign it would be removed at the initial surgery, but if frozen sections revealed a malignancy, further studies would be indicated. They gave their oral and written consent to proceed. Before the second surgical procedure, the parents were advised regarding the aggressive nature of the recurrent lesion and that the possibility of malignancy had to be considered. They were aware that the lesion had a reported 15% recurrence rate. The indications for another surgery and all possible complications were discussed and they were aware that a 3 to 5 mm margin of bone would be removed as well as all primary and permanent tooth buds associated with the lesion. They also knew that there would be a maxillary deformity and probably no primary or permanent teeth would ever erupt. The possibility of oroantral or oronasal communication was explained, as was the need for further and more extensive reconstructive surgery in the future. Both surgeries were done very carefully and the surgeons were certain that the tumor was removed completely each time. The speed with which new tumor revealed itself, however, leads us to believe that this is a case of residual tumor rather than recurrence. This lesion is believed to be benign; however, long-term follow-up will be needed to see if further tumor develops. Acknowledgment The authors wish to thank Charles Dunlap, DDS, Professor and Chairman, Department of Oral Pathology, for his assistance with the histologic evaluation. Dr Dunlap is on the faculty of the School of Dentistry at the University of Missouri-Kansas City, Kansas City, MO.
FIGURE 9. Coronal (A) and axial (B) computerized tomographic scans 6 months subsequent to the second surgical procedure showing no evidence of residual or recurrent tumor.
MOSBY ET AL
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Surg
1992
Melanotic Neuroectodermal Tumor of Infancy: A Report of Two Cases PETER
N. DEMAS,
DMD, MD,* THOMAS W. BRAUN, AND M.M. NAZIF, DDS, MDS*
DMD, PHD,t
Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively rare tumor of the anterior maxilla in young infants. The lesion often has bluish areas of pigmentation and is characterized by displacement of involved tooth buds and localized aggressiveness.‘d Historically, several names for this lesion have been proposed in the literature, including pigmented congenital epulis, melanotic progonoma, retinal analage
* Associate Professor, Department of Oral/Maxillofacial Surgery, University of Pittsburgh Medical Center and Veterans Administration Medical Center, Pittsburgh, PA. t Chairman, Department Oral/Maxillofacial Surgery, University of Pittsburah Medical Center and Veterans Administration Medical Center, Pi&burgh, PA. $ Director of Dental Services, Children’s Hospital of Pittsburgh, Pittsburgh, PA. Address correspondence and reprint requests to Dr Braun: Department of Oral and Maxillofacial Surgery, University of Pittsburgh School of Dental Medicine, 350 1 Terrace St, Pittsburgh, PA 1526 1. 0 1992 American Association of Oral and Maxillofacial Surgeons 0278-2391/92/5008-0017$3.00/O
FIGURE 1. Preoperative appearance of the expanded left maxilla.
