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Recurrent melanotic neuroectodermal tumor of infancy
Recurrent Melanotic Neuroectodermal Tumor of Infancy Wayne T. Shaia, MD,* Laurence J. DiNardo, MD, FACS,* Thomas E. Underhill, MD, DDS,† and Christine E. Cesca, MD‡ (Editorial Comment: I view that adjuvant therapy may be necessary to control residual or recurrent disease in this unusual tumor.)
Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon neoplasm found primarily in children less than one year of age. First described in the early 1900s by Krompecher as a congenital melanocarcinoma,1 the nomenclature of this neoplasm has undergone multiple changes that have been based on histologic and biochemical studies. Since Borello and Gorlin’s discovery of the associated high urinary excretion of vanillylmandelic acid (VMA) in the late 1960s,2 MNTI has been thought to be a derivative of neural crest cells. More recently, Hoshino et al have demonstrated high levels of urine catecholamines in a case of MNTI,3 which further supports the neural origin of this rare neoplasm. In a review of the world literature, approximately 227 cases of MNTI have been reported, most of which (93%) involve the head and neck region. The maxilla (69%) is the most commonly affected site, followed by the skull (11%), the mandible (6%), and less commonly, brain, mediastinum, thigh, epididymis, foot, and shoulder.4 This tumor is reported to be malignant in only 4% of the cases5 but is considered to be locally aggressive. MNTI is uncommon, and consequently oto-
From the Departments of *Otolaryngology Head and Neck Surgery, †Radiology, and ‡Pathology, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond, VA. Address correspondence to Laurence J. DiNardo, MD, Department of Otolaryngology Head and Neck Surgery, Medical College of Virginia, Virginia Commonwealth University, PO Box 980146, Richmond, VA 23298-0709. E-mail: [email protected]. Copyright 2002, Elsevier Science (USA). All rights reserved. 0196-0709/02/2304-0001$35.00/0 doi:10.1053/ajot.2002.123463
laryngologists/head and neck surgeons have limited experience managing this entity. Currently, the treatment of MNTI consists of a limited en bloc excision that leaves vital structures and organs intact. The role of adjuvant therapy such as radiation and chemotherapy has been discussed only briefly and 6,24 seems to be limited. We present a case of MNTI that was referred to our service after a failed primary excision. The lesion involved the left maxilla with extension through the left orbital floor and encompassing the left optic nerve and infraorbital fissure. A discussion of preoperative and postoperative management and a review of the literature are presented. Special emphasis is placed on persistent and recurrent disease. CASE REPORT A one-week-old male infant was unsuccessfully treated for left maxillary swelling at a local hospital with a several-day course of intravenous antibiotics. A subsequent computed tomography (CT) scan demonstrated an expansile mass with bone destruction in the left maxilla. Physical examination revealed a soft tissue mass extending from the left maxilla into the oral cavity (Fig 1). At 7 weeks of age the child was referred for oral and maxillofacial evaluation. An intraoral biopsy was carried out, and the diagnosis of MNTI was rendered. Partial resection of the left maxilla was carried out. By 9 weeks of age, the child manifested left proptosis as well as an enlargement of the left maxilla. Magnetic resonance (MR) and a repeat CT scan indicated evidence of a tumor involving the maxilla and extending through the left orbital floor and encompassing the left optic nerve (Fig 2). The child underwent a left total maxillectomy with resection of the inferior periorbita and removal of tumor from the infraorbital fissure and from around the optic nerve. An Allo-
American Journal of Otolaryngology, Vol 23, No 4 (July-August), 2002: pp 249-252
249
250
Fig 1. Patient at 7 weeks old is shown with extension of the mass from the left maxilla into the oral cavity.
Derm sling (LifeCell, Branchburg, NJ) was used to reconstruct the orbital floor. Palatal mucosa and periosteum were spared and reapproximated, thereby avoiding the need for a palatal obturator. All vital structures including the globe, optic nerve, and extraocular muscles were spared. The margins of the resection revealed evidence of microscopic disease. Approximately 3 weeks postoperatively a neuroblastoma chemotherapy protocol consisting of carboplatin, cyclophosphamide, doxorubicin, and etoposide was continued for 8 cycles over 168 days. The child is now over 1 year old with no evidence of recurrence on physical examination or repeat MR scan. The child has normal vision as
Fig 2. A. Coronal magnetic resonance image at 9 weeks showing the recurrent mass. The infraorbital extent of the mass compressing the left orbital contents can be seen. B. Axial magnetic resonance image at 9 weeks demonstrating invasion of the tumor into the left orbit and causing left proptosis.
SHAIA ET AL
Fig 3. Our patient 3 weeks postoperatively. The left maxillary incision has healed well, and proptosis has resolved.
well as a functionally and cosmetically acceptable result (Fig 3). DISCUSSION The presentation of MNTI is usually within the first year of life. Surgical removal of the mass with efforts to preserve vital structures has been well-documented as the best form of treatment.7 Successful primary excision has been reported to occur in 85-90% of all cases.4 As is with the case presented, recurrences have been reported to occur within the first several weeks postoperatively and require immediate attention. In a careful review of the literature since MNTI was first described,1 an overall recurrence rate of 10-15% has been noted.4,8 For the past 20 years the reported recurrence rate stands at 25% (22 of 89 cases). Several reasons may contribute to the finding of an apparent increase in the recurrence rate. First, there might be better follow-up and more frequent reporting of this entity since the early 1900s. Second, changes in nomenclature and the recent acceptance of a neural crest histogenesis for MNTI have likely led to its more uniform reporting. Third, with the advent of improved imaging techniques in the last 20 years, it is possible for evidence of recurrence to be found within weeks of the initial surgical intervention. Recurrence is also skewed towards younger patients. The literature reveals that 64% of all recurrences appeared in children who were
RECURRENT MELANOTIC NEUROECTODERMAL TUMOR
diagnosed at 12 weeks of age or younger (Table 1). Conversely, only 2 cases of patients diagnosed with MNTI at age 6 months or older that demonstrated evidence of recurrence (9%) have been reported since 1980. Children who present at an earlier age may have a more aggressive form of MNTI. For this reason we believe that children less than 3 months of age who have undergone excision of an MNTI require weekly examinations as well as frequent imaging studies to verify successful resection. Postoperatively, the patient was treated with a neuroblastoma chemotherapy protocol. This was based on three findings. First, the histopathogenesis of MNTI indicated neuroblastoma-like cells (Fig 4). Second, positive margins were found on the resection specimen. Third, this was a recurrent MNTI with a high mitotic rate. The utility of chemotherapy and radiation therapy in the treatment of MNTI is unclear. In 2000, Kaya et al examined 2 cases of MNTI and found that adjuvant therapy such as chemotherapy and radiation should be reserved for cases where resection with clear margins is impossible.24 Cohen et
TABLE 1. Reported Recurrences of MNTI (22 Cases), 1980 to Present
First Author
Year
Age (Months)
Location of Recurrence
Blank7 Ohne8 Gotcher9 Jimenez10 Jerrell11 Johnson5 Johnson5 Johnson5 Nagase12 Lamping13 Crockett14 Cohen6 Steinberg15 Claros16 Atkinson17 Mosby18 Pierre-Kahn19 Demas20 Patankart21 Dashiti22 Hoshina23 Shaia
1980 1980 1980 1981 1982 1983 1983 1983 1983 1985 1987 1988 1988 1989 1989 1992 1992 1992 1998 1999 2000 2002
Newborn 4 3 6 2 3 3 2 4 3 2 4 2 2 2 4 12 1 9 2 1 1
Maxilla Maxilla Maxilla Face Mandible Maxilla Maxilla Maxilla Maxilla Skull Maxilla Pineal Maxilla Maxilla Fontanel Maxilla Brain Mandible Occipital Mastoid Mandible Maxilla
Abbreviation: MNTI, melanotic neuroectodermal tumor of infancy.
251
Fig 4. Microscopic view of MNTI (hematoxylin and eosin, 1 m thick; original magnification ⴛ100). The larger melanin-containing cells surround smaller nests of neuroblastic cells in a fibrous stroma.
al6 in 1988 described a case of pineal involvement of MNTI that was treated with a neuroblastoma chemotherapy protocol. The tumor size remained stable until 9 months after chemotherapy was halted. Radiation therapy was initiated and growth was controlled. The child was alive at 30 months of age and meeting developmental milestones. CONCLUSION MNTI of the head and neck is a rare entity that is usually cured with surgical resection. A 25% recurrence rate has been demonstrated in the last 2 decades with 64% of these recurrences found in infants who present earlier than 12 weeks of age. Postoperative management of this subset should include weekly examinations as well as frequent imaging studies to adequately assess cure. Surgical resection should preserve the function of vital structures and organs. The difficulty in achieving adequate surgical margins has led some to advocate adjuvant therapy such as radiation or chemotherapy. These treatments are controversial and should only be considered for metastatic disease, cases of recurrence after a second surgical resection, or when complete removal of recurrent tumor cannot be achieved.
252
REFERENCES 1. Krompecher E: Zur Histogenese und Morphologie der Adamantinome und sonstiger Kiefergeschwulste. Beitr Pathol Anat 64:165-197, 1918 2. Borello ED, Gorlin RJ: Melanotic neuroectodermal tumor of infancy—A neoplasm of neural crest origin. Cancer 19:196-206, 1966 3. Hoshino S, Takahashi H, Shimura T, et al: Melanotic Neuroectodermal tumor of infancy in the skull associated with high levels of seum catecholamines. J Neurosurg 80:919-924, 1994 4. Cutler LS, Chaudhry AP, Topazian R: Melanotic neuroectodermal tumor of infancy: An ultrastructural study, literature review and reevaluation. Cancer 48:257270, 1981 5. Johnson RE, Scheithauer BW, Dahlin DC: Melanotic neuroectodermal tumor of infancy: A review of seven cases. Cancer. 52:661-666, 1983 6. Cohen BH, Handler MS, DeVivo DC, et al: Central nervous system melanotic neuroectodermal tumor of infancy: Value of chemotherapy management. Neurology 38:163-164, 1988 7. Blank E, Runckel DN: Case Report 119. Skeletal Radiol 5:179-182, 1980 8. Ohne H, Tokunaga S, Sumiya N: A case of melanomeloblastoma found in the anterior maxilla of an infant. J Plast Reconstr Surg 23:283-285, 1980 9. Gotcher JE, Jaffrey BJ, Hudson JW, et al: Recurrent melanotic neuroectodermal tumor of infancy: Report of case and tumor heterotransplantation studies. J Oral Surg 38:702-706, 1980 10. Jimenez JF, Brown RE, Seibert RW, et al: Melanotic neuroectodermal tumor of infancy and fetal hydantoin syndrome. Am J Pediatr Hematol Oncol 3:9-15, 1981 11. Jerrell RG, Hill CJ: Melanotic neuroectodermal tumor of infancy and postsurgical dental complications: Report of case. J Dent Child 49:371-373, 1982 12. Nagase M, Udea K, Fukushima M, et al: Recurrent melanotic neuroectodermal tumor of infancy: Case report
SHAIA ET AL
and survey of 16 cases. J Maxillofac Surg 11:131-136, 1983 13. Lamping KA, Albert DM, Lack E, et al: Melanotic neuroectodermal tumor of infancy (rentinal analage tumor). Ophthalmology 92:143-149, 1985 14. Crockett DM, McGill TJ, Healy GB, et al: Melanotic neuroectodermal tumor of infancy. Otolaryngol Head and Neck Surg 96:194-197, 1987 15. Steinberg B, Shuler C, Wilson S: Melanotic neuroectodermal tumor of infancy: Evidence for multicentricity. Oral Surg Oral Med Oral Pathol 66:666-668, 1988 16. Claros P, Claros A Sr, Claros A Jr, et al: Melanotic neuroectodermal tumor of infancy: A case report. Int J Pediatr Otorhinolaryngol 17:65-67, 1989 17. Atkinson GO Jr, Davis PC, Patrick LE, et al: Melanotic neuroectodermal tumor of infancy: MR findings and a review of the literature. Pediatr Radiol 20:20-22, 1989 18. Mosby EL, Lowe MW, Cobb CM, et al: Melanotic neuroectodermal tumor of infancy: Review of the literature and report of a case. J Oral Maxillofac Surg 50:886892, 1992 19. Pierre-Kahn A, Cinalli G, Lellouch-Tubiana A, et al: Melanotic neuroectodermal tumor of the skull and meninges in infancy. Pediatr Neurosurg 18:6-15, 1992 20. Demas PN, Braun TW, Naxif MM: Melanotic neuroectodermal tumor of infancy: A report of two cases. J Oral Maxillofac Surg 50:894-898, 1992 21. Patankart T, Prasad S, Goel A, et al: Malignant melanotic neuroectodermal tumour of infancy affecting the occipital squama. J Postgrad Med 44:73-75, 1998 22. Dashiti SR, Cohen ML, Cohen AR: Role of radical surgery for intracranial melanotic neuroectodermal tumor of infancy: Case report. Neurosurgery 45:175-178, 1999 23. Hoshina Y, Hamamoto Y, Suzuki I, et al: Melanotic neuroectodermal tumor of infancy in the mandible: Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 89:594-599, 2000 24. Kaya S, Omer FU, Sarp S, et al: Melanotic neuroectodermal tumor of infancy. Report of two cases and review of the literature. Int J Pediatr Otorhinolaryngol 52:169-172, 2000
Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon neoplasm found primarily in children less than one year of age. First described in the early 1900s by Krompecher as a congenital melanocarcinoma,1 the nomenclature of this neoplasm has undergone multiple changes that have been based on histologic and biochemical studies. Since Borello and Gorlin’s discovery of the associated high urinary excretion of vanillylmandelic acid (VMA) in the late 1960s,2 MNTI has been thought to be a derivative of neural crest cells. More recently, Hoshino et al have demonstrated high levels of urine catecholamines in a case of MNTI,3 which further supports the neural origin of this rare neoplasm. In a review of the world literature, approximately 227 cases of MNTI have been reported, most of which (93%) involve the head and neck region. The maxilla (69%) is the most commonly affected site, followed by the skull (11%), the mandible (6%), and less commonly, brain, mediastinum, thigh, epididymis, foot, and shoulder.4 This tumor is reported to be malignant in only 4% of the cases5 but is considered to be locally aggressive. MNTI is uncommon, and consequently oto-
From the Departments of *Otolaryngology Head and Neck Surgery, †Radiology, and ‡Pathology, School of Medicine, Medical College of Virginia, Virginia Commonwealth University, Richmond, VA. Address correspondence to Laurence J. DiNardo, MD, Department of Otolaryngology Head and Neck Surgery, Medical College of Virginia, Virginia Commonwealth University, PO Box 980146, Richmond, VA 23298-0709. E-mail: [email protected]. Copyright 2002, Elsevier Science (USA). All rights reserved. 0196-0709/02/2304-0001$35.00/0 doi:10.1053/ajot.2002.123463
laryngologists/head and neck surgeons have limited experience managing this entity. Currently, the treatment of MNTI consists of a limited en bloc excision that leaves vital structures and organs intact. The role of adjuvant therapy such as radiation and chemotherapy has been discussed only briefly and 6,24 seems to be limited. We present a case of MNTI that was referred to our service after a failed primary excision. The lesion involved the left maxilla with extension through the left orbital floor and encompassing the left optic nerve and infraorbital fissure. A discussion of preoperative and postoperative management and a review of the literature are presented. Special emphasis is placed on persistent and recurrent disease. CASE REPORT A one-week-old male infant was unsuccessfully treated for left maxillary swelling at a local hospital with a several-day course of intravenous antibiotics. A subsequent computed tomography (CT) scan demonstrated an expansile mass with bone destruction in the left maxilla. Physical examination revealed a soft tissue mass extending from the left maxilla into the oral cavity (Fig 1). At 7 weeks of age the child was referred for oral and maxillofacial evaluation. An intraoral biopsy was carried out, and the diagnosis of MNTI was rendered. Partial resection of the left maxilla was carried out. By 9 weeks of age, the child manifested left proptosis as well as an enlargement of the left maxilla. Magnetic resonance (MR) and a repeat CT scan indicated evidence of a tumor involving the maxilla and extending through the left orbital floor and encompassing the left optic nerve (Fig 2). The child underwent a left total maxillectomy with resection of the inferior periorbita and removal of tumor from the infraorbital fissure and from around the optic nerve. An Allo-
American Journal of Otolaryngology, Vol 23, No 4 (July-August), 2002: pp 249-252
249
250
Fig 1. Patient at 7 weeks old is shown with extension of the mass from the left maxilla into the oral cavity.
Derm sling (LifeCell, Branchburg, NJ) was used to reconstruct the orbital floor. Palatal mucosa and periosteum were spared and reapproximated, thereby avoiding the need for a palatal obturator. All vital structures including the globe, optic nerve, and extraocular muscles were spared. The margins of the resection revealed evidence of microscopic disease. Approximately 3 weeks postoperatively a neuroblastoma chemotherapy protocol consisting of carboplatin, cyclophosphamide, doxorubicin, and etoposide was continued for 8 cycles over 168 days. The child is now over 1 year old with no evidence of recurrence on physical examination or repeat MR scan. The child has normal vision as
Fig 2. A. Coronal magnetic resonance image at 9 weeks showing the recurrent mass. The infraorbital extent of the mass compressing the left orbital contents can be seen. B. Axial magnetic resonance image at 9 weeks demonstrating invasion of the tumor into the left orbit and causing left proptosis.
SHAIA ET AL
Fig 3. Our patient 3 weeks postoperatively. The left maxillary incision has healed well, and proptosis has resolved.
well as a functionally and cosmetically acceptable result (Fig 3). DISCUSSION The presentation of MNTI is usually within the first year of life. Surgical removal of the mass with efforts to preserve vital structures has been well-documented as the best form of treatment.7 Successful primary excision has been reported to occur in 85-90% of all cases.4 As is with the case presented, recurrences have been reported to occur within the first several weeks postoperatively and require immediate attention. In a careful review of the literature since MNTI was first described,1 an overall recurrence rate of 10-15% has been noted.4,8 For the past 20 years the reported recurrence rate stands at 25% (22 of 89 cases). Several reasons may contribute to the finding of an apparent increase in the recurrence rate. First, there might be better follow-up and more frequent reporting of this entity since the early 1900s. Second, changes in nomenclature and the recent acceptance of a neural crest histogenesis for MNTI have likely led to its more uniform reporting. Third, with the advent of improved imaging techniques in the last 20 years, it is possible for evidence of recurrence to be found within weeks of the initial surgical intervention. Recurrence is also skewed towards younger patients. The literature reveals that 64% of all recurrences appeared in children who were
RECURRENT MELANOTIC NEUROECTODERMAL TUMOR
diagnosed at 12 weeks of age or younger (Table 1). Conversely, only 2 cases of patients diagnosed with MNTI at age 6 months or older that demonstrated evidence of recurrence (9%) have been reported since 1980. Children who present at an earlier age may have a more aggressive form of MNTI. For this reason we believe that children less than 3 months of age who have undergone excision of an MNTI require weekly examinations as well as frequent imaging studies to verify successful resection. Postoperatively, the patient was treated with a neuroblastoma chemotherapy protocol. This was based on three findings. First, the histopathogenesis of MNTI indicated neuroblastoma-like cells (Fig 4). Second, positive margins were found on the resection specimen. Third, this was a recurrent MNTI with a high mitotic rate. The utility of chemotherapy and radiation therapy in the treatment of MNTI is unclear. In 2000, Kaya et al examined 2 cases of MNTI and found that adjuvant therapy such as chemotherapy and radiation should be reserved for cases where resection with clear margins is impossible.24 Cohen et
TABLE 1. Reported Recurrences of MNTI (22 Cases), 1980 to Present
First Author
Year
Age (Months)
Location of Recurrence
Blank7 Ohne8 Gotcher9 Jimenez10 Jerrell11 Johnson5 Johnson5 Johnson5 Nagase12 Lamping13 Crockett14 Cohen6 Steinberg15 Claros16 Atkinson17 Mosby18 Pierre-Kahn19 Demas20 Patankart21 Dashiti22 Hoshina23 Shaia
1980 1980 1980 1981 1982 1983 1983 1983 1983 1985 1987 1988 1988 1989 1989 1992 1992 1992 1998 1999 2000 2002
Newborn 4 3 6 2 3 3 2 4 3 2 4 2 2 2 4 12 1 9 2 1 1
Maxilla Maxilla Maxilla Face Mandible Maxilla Maxilla Maxilla Maxilla Skull Maxilla Pineal Maxilla Maxilla Fontanel Maxilla Brain Mandible Occipital Mastoid Mandible Maxilla
Abbreviation: MNTI, melanotic neuroectodermal tumor of infancy.
251
Fig 4. Microscopic view of MNTI (hematoxylin and eosin, 1 m thick; original magnification ⴛ100). The larger melanin-containing cells surround smaller nests of neuroblastic cells in a fibrous stroma.
al6 in 1988 described a case of pineal involvement of MNTI that was treated with a neuroblastoma chemotherapy protocol. The tumor size remained stable until 9 months after chemotherapy was halted. Radiation therapy was initiated and growth was controlled. The child was alive at 30 months of age and meeting developmental milestones. CONCLUSION MNTI of the head and neck is a rare entity that is usually cured with surgical resection. A 25% recurrence rate has been demonstrated in the last 2 decades with 64% of these recurrences found in infants who present earlier than 12 weeks of age. Postoperative management of this subset should include weekly examinations as well as frequent imaging studies to adequately assess cure. Surgical resection should preserve the function of vital structures and organs. The difficulty in achieving adequate surgical margins has led some to advocate adjuvant therapy such as radiation or chemotherapy. These treatments are controversial and should only be considered for metastatic disease, cases of recurrence after a second surgical resection, or when complete removal of recurrent tumor cannot be achieved.
252
REFERENCES 1. Krompecher E: Zur Histogenese und Morphologie der Adamantinome und sonstiger Kiefergeschwulste. Beitr Pathol Anat 64:165-197, 1918 2. Borello ED, Gorlin RJ: Melanotic neuroectodermal tumor of infancy—A neoplasm of neural crest origin. Cancer 19:196-206, 1966 3. Hoshino S, Takahashi H, Shimura T, et al: Melanotic Neuroectodermal tumor of infancy in the skull associated with high levels of seum catecholamines. J Neurosurg 80:919-924, 1994 4. Cutler LS, Chaudhry AP, Topazian R: Melanotic neuroectodermal tumor of infancy: An ultrastructural study, literature review and reevaluation. Cancer 48:257270, 1981 5. Johnson RE, Scheithauer BW, Dahlin DC: Melanotic neuroectodermal tumor of infancy: A review of seven cases. Cancer. 52:661-666, 1983 6. Cohen BH, Handler MS, DeVivo DC, et al: Central nervous system melanotic neuroectodermal tumor of infancy: Value of chemotherapy management. Neurology 38:163-164, 1988 7. Blank E, Runckel DN: Case Report 119. Skeletal Radiol 5:179-182, 1980 8. Ohne H, Tokunaga S, Sumiya N: A case of melanomeloblastoma found in the anterior maxilla of an infant. J Plast Reconstr Surg 23:283-285, 1980 9. Gotcher JE, Jaffrey BJ, Hudson JW, et al: Recurrent melanotic neuroectodermal tumor of infancy: Report of case and tumor heterotransplantation studies. J Oral Surg 38:702-706, 1980 10. Jimenez JF, Brown RE, Seibert RW, et al: Melanotic neuroectodermal tumor of infancy and fetal hydantoin syndrome. Am J Pediatr Hematol Oncol 3:9-15, 1981 11. Jerrell RG, Hill CJ: Melanotic neuroectodermal tumor of infancy and postsurgical dental complications: Report of case. J Dent Child 49:371-373, 1982 12. Nagase M, Udea K, Fukushima M, et al: Recurrent melanotic neuroectodermal tumor of infancy: Case report
SHAIA ET AL
and survey of 16 cases. J Maxillofac Surg 11:131-136, 1983 13. Lamping KA, Albert DM, Lack E, et al: Melanotic neuroectodermal tumor of infancy (rentinal analage tumor). Ophthalmology 92:143-149, 1985 14. Crockett DM, McGill TJ, Healy GB, et al: Melanotic neuroectodermal tumor of infancy. Otolaryngol Head and Neck Surg 96:194-197, 1987 15. Steinberg B, Shuler C, Wilson S: Melanotic neuroectodermal tumor of infancy: Evidence for multicentricity. Oral Surg Oral Med Oral Pathol 66:666-668, 1988 16. Claros P, Claros A Sr, Claros A Jr, et al: Melanotic neuroectodermal tumor of infancy: A case report. Int J Pediatr Otorhinolaryngol 17:65-67, 1989 17. Atkinson GO Jr, Davis PC, Patrick LE, et al: Melanotic neuroectodermal tumor of infancy: MR findings and a review of the literature. Pediatr Radiol 20:20-22, 1989 18. Mosby EL, Lowe MW, Cobb CM, et al: Melanotic neuroectodermal tumor of infancy: Review of the literature and report of a case. J Oral Maxillofac Surg 50:886892, 1992 19. Pierre-Kahn A, Cinalli G, Lellouch-Tubiana A, et al: Melanotic neuroectodermal tumor of the skull and meninges in infancy. Pediatr Neurosurg 18:6-15, 1992 20. Demas PN, Braun TW, Naxif MM: Melanotic neuroectodermal tumor of infancy: A report of two cases. J Oral Maxillofac Surg 50:894-898, 1992 21. Patankart T, Prasad S, Goel A, et al: Malignant melanotic neuroectodermal tumour of infancy affecting the occipital squama. J Postgrad Med 44:73-75, 1998 22. Dashiti SR, Cohen ML, Cohen AR: Role of radical surgery for intracranial melanotic neuroectodermal tumor of infancy: Case report. Neurosurgery 45:175-178, 1999 23. Hoshina Y, Hamamoto Y, Suzuki I, et al: Melanotic neuroectodermal tumor of infancy in the mandible: Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 89:594-599, 2000 24. Kaya S, Omer FU, Sarp S, et al: Melanotic neuroectodermal tumor of infancy. Report of two cases and review of the literature. Int J Pediatr Otorhinolaryngol 52:169-172, 2000