International Journal of Pediatric Otorhinolaryngology, 18 (1989) 59-66 Elsevier 59 PEDOT 00596 Melanotic neuroectodermal tumor of infancy: report of two cases ’ Andrew Turner, ’ Jacob Friedberg, 3 Kent Mancer and ’ Jerome Becker Departments of’ Otolaryngology, 2 Dentistry and 3 Pathology, Hospital for Sick Children, and Universiry of Toronto, Toronto, Ontario (Canada) Key words: Rare neoplasm; Local excision; Child Abstract Melanotic neuroectodermal tumor of childhood is a rare lesion, but its presenta- tion appears to be quite characteristic and a clinical diagnosis may be made with some confidence. Although this is an extremely rapidly growing, aggressive tumor, local excision appears to be adequate for cure. Introduction Melanotic neuroectodermal tumor of infancy is a rare neoplasm first described by Krompecher in 1918 [6]. Numerous case reports have since appeared in both the otolaryngology and dental literature. Hupp et al. [5] reported an extensive literature review in 1981, siting 139 cases which recognized diagnostic criteria. This paper does not seek to replicate the efforts of these previous authors, but will highlight the salient features of this lesion and add two cases seen at the Hospital for Sick Children in Toronto. Case reports Case I This 2.5month-old girl was noted by her mother to develop progressive swelling in the left maxillary gingivo-buccal sulcus over a period of several weeks. The child was otherwise well, and had no feeding difficulty. She was referred by her family Correspondence: J. Friedberg, E.N.T. Associates, 99 Avenue Road, Suite 207, Toronto, Ontario, Canada M5R 2G5. 0165-5876/89/$03.50 0 1989 Elsevier Science Publishers B.V.
Transcript
International Journal of Pediatric Otorhinolaryngology, 18 (1989) 59-66
Elsevier
59
PEDOT 00596
Melanotic neuroectodermal tumor of infancy: report of two cases
’ Andrew Turner, ’ Jacob Friedberg, 3 Kent Mancer and ’ Jerome Becker
Departments of’ Otolaryngology, 2 Dentistry and 3 Pathology, Hospital for Sick Children,
and Universiry of Toronto, Toronto, Ontario (Canada)
Key words: Rare neoplasm; Local excision; Child
Abstract
Melanotic neuroectodermal tumor of childhood is a rare lesion, but its presenta- tion appears to be quite characteristic and a clinical diagnosis may be made with some confidence. Although this is an extremely rapidly growing, aggressive tumor, local excision appears to be adequate for cure.
Introduction
Melanotic neuroectodermal tumor of infancy is a rare neoplasm first described by Krompecher in 1918 [6]. Numerous case reports have since appeared in both the otolaryngology and dental literature. Hupp et al. [5] reported an extensive literature review in 1981, siting 139 cases which recognized diagnostic criteria.
This paper does not seek to replicate the efforts of these previous authors, but will highlight the salient features of this lesion and add two cases seen at the Hospital for Sick Children in Toronto.
Case reports
Case I This 2.5month-old girl was noted by her mother to develop progressive swelling
in the left maxillary gingivo-buccal sulcus over a period of several weeks. The child was otherwise well, and had no feeding difficulty. She was referred by her family
Correspondence: J. Friedberg, E.N.T. Associates, 99 Avenue Road, Suite 207, Toronto, Ontario, Canada
doctor to an oral surgeon. Examination disclosed a firm expansion of the left maxillary alveolar ridge in the premolar region.
Radiographs revealed a radiolucency involving a developing deciduous molar (premolar). Exploration of the region disclosed the developing tooth to be sur- rounded by a darkly pigmented friable soft tissue lesion which extended into, and obliterated the left maxillary sinus. Darkly pigmented friable material was curretted from the mass within the sulcus and adjacent alveolar ridge. Complete excision was not attempted at that time. Histological examination was characteristic of a melanotic neuroectodermal tumor of infancy. She was subsequently referred for otolaryngo- logic assessment and definitive management.
The patient was found to have a firm fullness of the left cheek. The left maxillary alveolus and gingivo-buccal mucosa were expanded by the tumor. The left eye was displaced superiorly but extraocular movement was full. The oral mucosa was intact except for the biopsy site.
Routine laboratory investigations were normal except for a slight leukocytosis. Urinary vandelmandelic acid (VMA) level was unremarkable. CT radiographs
Fig. 1. Tumor is seen to involve almost the entire left maxilla with extension into the nasal cavity
medially and the infratemporal region laterally. The orbital floor is extensively involved with tumor,
thickened, crowding the orbital contents superiorly.
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demonstrated the extensive maxillary, infratemporal and orbital floor involvement (Fig. 1).
Surgical excision was accomplished by a partial maxillectomy through a gingivo- buccal incision. The tumor was coal-black in color and multiloculated. Bleeding was minimal and there were no areas of necrosis. Several dental buds were encountered and were sacrificed. Laterally, the tumor invaded the infratemporal fossa, but was cleanly separated from the soft tissues. The orbital floor was markedly thickened to a depth of approximately 1.0 cm by the tumor mass and deeply pigmented by it. The orbital floor was curretted away until more normal looking non-pigmented bone was encountered. In so doing the apex of the orbital floor was completely removed resulting in hemiation of orbital fat into the operative field. Small areas of pigmented tumor could be seen in the retro-orbital area, and because of its proximity to the optic nerve, an intra-operative ophthalmology consultation was obtained. It was felt that further advances would carry an unacceptably high risk to the nerve and the resection in this area was therefore discontinued. A small buccal flap permitted primary surgical closure of the maxillary defect.
The patient continues to do well over 1 year postoperatively with no clinical or radiographic evidence of tumor.
Case 2 A few months after the above patient was seen, a 3-month-old male infant
presented with a 3-week history of a rapidly growing premaxillary mass (Fig. 2).
Fig. 2. This premaxillary, smooth, pigmented, well-circumscribed lesion is a most characteristic presenta-
tion of melanotic neuroectodermal tumor.
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Fig. 3. The tumor is seen to be a discrete expansile premaxillary mass and has encompassed a dental bud (arrow). This latter feature at first is suggestive of a dentigerous cyst, but age of presentation and clinical
appearance are clearly otherwise.
This was obviously pigmented, hard, smooth and lacking in mucosal involvement. With the easy confidence of hind sight, a clinical diagnosis of melanotic neuroecto- dermal tumor was made. This was supported by radiographic findings (Fig. 3) which, however, did have some features which could be confused with a dentigerous cyst. The patient was otherwise well with no physical or laboratory evidence of problems elsewhere. A prompt surgical excision was arranged. The tumor shelled out easily (Fig. 4), and was found to involve several dental buds which were sacrificed. Again, the dissection was continued until no gross evidence of pigmented tumor could be seen clinically and peripheral curettings were free of tumor. This child has also remained free of tumor at a year.
Discussion
The origin of this tumor has engendered controversy over the years. The commonly accepted theory, as proposed by Borello and Gorlin [2], is one of neural
Fig. 4. The tumor was smooth-walled and deeply pigmented with a dental bud embedded in its periphery
(arrow).
crest origin. They cite the markedly elevated VMA level in their case as support for that theory. A review of other case reports, including our own, however, suggests that that is the exception rather than the rule.
Diagnosis should not pose a problem. The tumor presents as a rapidly growing painless mass usually located in the anterior maxilla of infants. Hupp et al. [5] in reviewing 139 cases, showed 78% involved the maxilla, 9% the anterior fontanel of the skull, and 8% the mandible. There was no overall sex difference. All cases presented under 1 year of age, with 92% presenting within the first 6 months of life.
The overlying mucosa is usually intact, but may reveal the dark pigmentation of the underlying firm rubbery mass. Although this is a benign tumor, its X-ray appearance commonly suggests malignancy because of its irregular margin and occasional osteosarcoma-like ‘sun ray’ pattern.
Clinically, the differential diagnosis may include osteosarcoma, giant cell granuloma, and other benign or malignant neoplasms of odontoblastic, neuroblas- tic, or osteoblastic tissue. The classic anterior maxillary location of a lesion in an infant, however, is strongly suggestive of the melanotic neuroectodermal tumor of infancy.
Its histology moreover is very characteristic (Figs. 5, 6). Cell features which help to distinguish it from neuroblastoma and lymphoma are: (i) cell cluster around cleft-like spaces; (ii) predominance of two-cell types: a larger one with abundant
Fig. 5. Nests of pigmented tumor cells (upper centre) in a dense fibrous stoma infiltrate bone. The latter, seen below, has a fibrotic marrow. Hematoxylin & Eosinx40. (Courtesy: Dr. M. Silver, Pathology
Department, Hospital for Sick Children, Toronto, Ont., Canada).
pale cytoplasm which may contain melanin pigment, and a smaller one with scant pale cytoplasm and much darker staining nuclei; (iii) presence of pigmentation; (iv) absence of cytologic atypia; (v) lack of a rosette pattern [4].
Most authors recommend that management should be restricted to conservative surgery. Thorough curretage is effective as there is little bleeding and the dark pigmentation clearly delineates the extent of the lesion. As well, small areas of remaining tumor may undergo spontaneous regression following debulking of the larger mass [5]. The tumor is reportedly radio-sensitive, but given the high level of success with conservative surgery, radiotherapy is generally not indicated in these children. There is a 15% recurrence rate possibly associated in many cases with incomplete removal [5,10]. Conservative local excision of recurrences, have been almost invariably curative. Five cases [1,3,7-91 of malignant melanotic neuroecto- dermal tumor have been reported. The final histology in 3 of the 5, however, was felt to be more suggestive of neuroblastoma and in another of malignant teratoma with melanin.
The tumor in our first case extended beyond the maxilla into the orbit, the infratemporal fossa and the nasal cavity. As may be seen on examination of an infant skull, structural relationships differ somewhat from those seen in an adult. The orbital floor slants downward placing the optic nerve in extremely close
Fig. 6. At high magnification, immature cells centrally are surrounded by heavily pigmented epithelioid cells. Hematoxytin & Rosin X 640. (Courtesy: Dr. M. Silver, Pathology Department, Hospital for Sick
Children, Toronto, Grit., Canada).
proximity to lesions involving the posterior maxilla and nasopharyngeal region. No attempt was made to completely remove tumor invading into the region of the optic nerve in this baby. The decision to avoid potential damage in that region, was made easier by the reports of spontaneous regression following debulking. Clinical and radiographic follow-up thus far suggests that our optimism was justified.
References
Block, J.C. et al., Pigmented neuroectodermal tumor of infancy: an example of rarely expressed malignant behaviour, Oral Surg., 49 (1980) 279-285. Borello, E.D. and Gorlin, R.J., Melanotic neuroectoderrnal tumor of infancy - a neoplasm of neural crest origin, Cancer, 19 (1966) 196-206. Dehner, L.P. et al., Malignant melanocytic neuroectodermal tumor of infancy. A clinical, pathologic, ultrastructural and tissue culture study, Cancer, 43 (1979) 1389-1410. Greer, R.O., Savara, B.E. an Mierau, G.W., NeuroectodermaJ tumors and associated lesions. Tumors of the Head and Neck in Children, Praeger, New York, 1983, pp. 315-322. Hupp, J.R., Topazian, R.G. and Krutchkoff, D.J., The melanotic neuroectodermal tumor of infancy, Int. J. Oral Surg., 10 (1981) 432-446. Krompecher, E., Zur Histogenese und Morphologic der Adamantinome und sonstiger Kieferge- schwulste, Beitr. Path. Anat. Allg. Path., 64 (1917/18) 165-197.
