Skeletal Radiology
1 Skeletal Radio1 (1990) 19:527-530
Case report 617 Henry H. Jones, M.D. 1, Bruce R. Parker, M.D. 1' 3, Cristina G. Ballerio, M.D. 1, Frederic N. Silverman, M.D. 1, 3, and Richard L. Kempson, M.D. 2 Departments of 1 Diagnostic Radiology and Nuclear Medicine, and 2 Pathology, Stanford University, Stanford, California, and 3 Children's Hospital at Stanford, Palo Alto, California, U S A
Radiological studies
Fig. 1. Age 8 weeks. Digitally manipulated tangential image of the skull shows coarse bony spicules (arrow) extending into the soft tissue mass which is displacing the pinna downward and forward Fig. 2. Age 121/2 weeks. Tangential view of the skull shows bony spicules (arrows) projecting both outward and inward Fig. 3. Age 13 weeks. CT shows an extraaxial mass displacing the meninges inward
Clinical information This healthy, well-developed black girl, is the second child of an 18-year-old mother. A small lump behind her right ear was noted at 3 weeks of age, but may have been present since birth. At 4 weeks of age, the mass measured 11/2 x 11/2 cm. The mass grew and radiographs were obtained at age 8 weeks (Fig. 1). The patient was referred to us 4 weeks later when the mass was 4 x 4 cm in size. It was firm nontender, and
Address reprint requests to: Henry H. Jones, M.D., Department of Diagnostic Radiology and Nuclear Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA
fixed to the underlying skull. It had been reported by one examiner as initially moveable, later becoming fixed. The skin over it was freely moveable and did not transilluminate. The physical examination was otherwise normal. She was afebrile and CBC was normal. She was placed on Keflex 1 for 2 weeks with no response. The mass continued to increase in size. Radiographs at age 121/2 weeks (Fig. 2) showed a prominent soft tissue mass displacing the right ear forward. The mass involved the temporal bone and prominent spicules of bone radiated 1 Keflex is a semisynthetic cephalosporin antibiotic
from the skull into the soft tissue mass. CT examination demonstrated inward displacement of the meninges (Fig. 3). The ear was displaced forward and the external auditory canal compressed. A TC 99 diphosphonate bone scan was highly positive in the region of the tumor, but otherwise normal. Extensive laboratory work at this time was normal. Vanilmandelic acid assay was not done. Abdominal CT was normal, specifically with no evidence of an adrenal mass. A chest roentgenogram was normal. The child was taken to surgery at age 13 weeks for removal of the tumor which was adherent to the dura in the region of the lateral and sigmoid sinuses. 9 1990 International Skeletal Society
528
Diagnosis: Melanotic neuroectodermal tumor of infancy (melanotic progonoma 2) involving the calvaria 3 The t u m o r was firm in consistency and cut with some grittiness owing to the intrusive b o n y spicules (Fig. 4). N u m e r e o u s d a r k streaks and spots were present. Histologically noted were alveolar-like clumps a n d strands o f small blue staining cells with scanty c y t o p l a s m (Fig. 5). A r o u n d the periphery o f the clumps were epithelial-like cells containing b r o w n granules which stained positively for melanin (Fig. 5). A fibrous s t r o m a was present a n d b o n y spicules intruded into the t u m o r mass. This appearance is considered characteristic for melanotic neuroectodermal t u m o r o f infancy ( M N T I ) . T h e small d a r k cells raise the question o f n e u r o b l a s t o m a or r h a b d o m y o s a r c o m a , b u t neither o f these tum o r s contains pigmented cells. Melan o m a does n o t occur in this age group. T h e postoperative course was uneventful and the child is well 2x/2 years later.
Discussion This tumor, n o w officially called melanotic n e u r o e c t o d e r m a l t u m o r o f infancy or melanotic p r o g o n o m a , was first described in 1918 by K r o m pecher w h o n a m e d it " c o n g e n i t a l m e l a n o c a r c i n o m a " [24]. Subsequent cases have been described u n d e r a variety o f names, reflecting the site o f origin or suspected histogenesis. Approximately 200 cases have been described. The majority (70%) occur in the maxilla, with 13% in the calvaria, a n d 6 % in the mandible. Occasional cases have h a d their origins elsewhere. Twenty-seven cases have oc2 Progonoma Dorland's Medical Dictionary, 25th edn): " a tumor due to misplacement of tissue as a result of fetal atavism to a stage which does not occur in the life history of the species, but which does occur in ancestral forms of the species." This term was first used by Stowens [34] to indicate his hypothesis as to the origin of these lesions 3 We are using this term to include the frontal, parietal, occipital, temporal, and sphenoid bones
H.H, Jones et al. : Case report 617 Table l. Location of calvarial tumors (n = 27) 13 5 3 5
diagnosis if one is dealing with a rapidly growing, n o n t e n d e r mass fixed to the skull, with skin m o v e a b l e over it a n d b o n y spicules extending into it, in an otherwise healthy infant u n d e r 6 m o n t h s o f age. However, because o f its rarity, very u n c o m m o n presentations o f relatively m u c h c o m m o n e r conditions m u s t be given consideration (Table 3). O f clinical i m p o r t a n c e is the absence o f any clearly m a l i g n a n t case in the g r o u p involving primarily the calvaria. Six patients have died at operation or in the immediate postoperative period. It is n o t e w o r t h y t h a t 75% o f the patients w h o were alive and well following surgical t r e a t m e n t h a d t u m o r s which were smaller t h a n these six w h o died following surgery. O p e r a t i o n w h e n the lesion is small and s o o n after detection appears to be a d v a n t a g e o u s (Fig. 6). The single
Anterior fontanel Temporoparietal Frontotemporosphenoid Other One patient had three tumors: 2 parietal, I orbitosphenoid
curred in the calvaria. Tables 1 and 2 summarize their attributes. In 1966, u r i n a r y vanilmandelic acid was reported as being elevated in a patient with M N T I [8, 14]. Since then, a few additional instances have been recorded. The value was n o r m a l in the f o u r calvarial cases in which the test was performed. T h e m o s t difficult aspect o f the differential diagnosis o f this very unc o m m o n n e o p l a s m is to think o f it. M N T I is the m o s t likely prebiopsy
Table 2. Summary of findings in 27 cases of MNTI involving the calvaria Age first detected
Age when treated
Race
Birth to under 3 months 3-6 months
Under 3 months
4
White
6
Male
16
17
Black
9
Female
11
16 9
3-6 months
Over 6 months 1 Not specified 1
over 6 months Not specified
Size when first seen
Size when operated
Tender?
Under 1 cm 1-3 cm Over 3 cm Not specified
Under 3 cm 3-6 cm Over 6 cm Not specified
Yes
5 4 3 15
6 0
Sex
1 17 7 2
Other Not specified
2 10 Consistency 0
No Not specified
11 16
Firm Not specified
18 9
Adherent to skin
Adherent to skull
Adherent to dura
Calcification
Yes No Not specified
Yes No Not specified
Yes No Not specified
In 19 cases, caMfication mentioned; in 13 of these spiculation specified or illustrated
0 14 13
Treatment Surgery Radiation Chemotherapy Not specified
20 ~ t 6
15 3 9
Results of treatment 24 1 i 1
Up to 2 years follow-up Over 2 years follow up Died in post-op period Died later Not specified
12 7 6 Ib 1
Alive and well
3 cases listed as not adherent to skull when first seen. Later adherent b 1 case treated with chemotherapy only, died age 3 months of pneumonia
529
H.H. Jones et al. : Case report 617
Pathological studies
Fig. 4. Photograph of gross specimen, Arrow points to bony spicules. The black melanin pigment characteristic of this tumor is clearly visible Fig. 5. Histologic section shows alveolar clumps and strands of small blue-staining cells with scanty cytoplasm. Arrow points to peripherally arranged epithelial-like cells that contain granules which stained positively for melanin
14i
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3
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0
2
.
,
4
9
.
,
6
.
.
.
,
8
9
9
,
10
.
.
,
9
12
Largest recorded dimension (cm)
patient with recurrence was alive and w e l l 4 y e a r s a f t e r r e s e c t i o n o f t h e recurrence. Rare metastases have been r e p o r t e d [14, 20] in m a x i l l a r y o r mandibular lesions and occasionally f r o m o t h e r sites, b u t n o t f r o m t h e calvaria. An exception to the generally f a v o r a b l e p r o g n o s i s is p r o v i d e d b y nonosseous intracranial lesions which are nearly always fatal because of local or widespread involvement or multifocal lesions within the cen-
.
,
14
Fig. 6. Time between detection and operation or biopsy plotted against largest recorded dimension for the 24 cases for which this information was available, including all cases reported as having died. One case (symbol e) was biopsied only and treated with chemotherapy [28] and died of infection 3 months after the biopsy. Six cases (symbol x) [2, 6, I3, 18, 21, 25] died in the immediate postoperative period. The distribution of these six cases points up the importance of early detection and surgical treatment while the tumor is still of small size
Table 3. Differential features of other lesions which will usually lead to their exclusion
Ewing's sarcoma: The youngest patient ineluded in the 89t cases tabulated from the literature by Wither [35] was 7 months old. Only 2% are primary in the skull and spiculation is not a feature.
Osteosarcoma: Roentgenographic appearance can mimic MNTI. The youngest patient in the Dahlin-Unni series of 962 cases [12] was 2.9 years old.
Table 3. (continued)
Cephalohematoma: The roentgenographic appearance is different and the lack of a history of trauma and failure to increase in size should eliminate this consideration. Infection: Local tenderness and systemic manifestations such as fever and leukocytosis will usually identify infection. An indolent process such as tuberculosis would not show rapid increase in size.
Lymphoma, leukemia, and rhabdomyosarcorna: In the rare event that one of these neoplasms should present as a mass involving the calvaria in a patient under 6 months of age, manifestations elsewhere should exclude M N T I from the differential diagnosis.
Neuroblastoma: Metastasis to the calvaria may present as a soft tissue mass and may stimulate a spiculated bony reaction. However, there are usually lesions elsewhere as well as the primary tumor. Hemangioma: When involving the calvaria this lesion is usually slowly growing and is manifest at an older age. Spiculation occurs, but the usual bone pattern is a honeycomb or reticulated appearance. Fibrous dysplasia. Slowly growing. Usually is manifest at an older age. Spiculation is not a feature. Eosinophilie granuloma: A soft tissue mass involving the infant calvaria would usually be tender. Bony spiculadon is uncommon. Solitary lesion is excessively rare in this age group [31].
Meningioma:
Roentgenographic appearance can mimic MNTI. However, there appear to be but three cases in the literature occurring in infancy [21, 27].
530
tral nervous system. More extensive dicussion is available in the recent literature [1-7, 9-11, 13-21, 23-26, 2833]. In Summary, a case of melanotic neuroectodermal tumor of infancy is presented. The behavior of this case and similar ones indicates that the lesion, when involving primarily the calvaria, is benign. Efforts must be directed toward diagnosis at an early stage when an infant may present with a rapidly growing, fixed lesion of the skull. Early diagnosis should obviate the necessity for extensive, life-threatening surgery. Careful evaluation for early recurrence should permit curative reoperation.
Acknowledgements. We would
like to thank Dr. Franklin C. Buckner for referring this patient to us, Dr. Victor Fish for translation of the Russian articles, and Dr. Jaime Aguet for translation of the articles in Spanish. Dr. John Drace prepared the diagram of Fig. 6. Mark Riesenberger assisted ably with the preparation of the manuscript.
References 1. Allen MS Jr, Harrison W, Jahrsdoerfer RA (1969) "Retinal anlage" tumors. Am J Clin Pathol 51 : 309 2. Adeloye A, Morgan SP, Aghadiuno P, Bohrer S (1977) Melanotic ameloblastoma (retinal anlage tumour) of the skull in a Nigerian child. Surg Neurol 7 : 42 3. Alipcenko LA, Arbatova GV, Rostovceva TF (1976) Melauoticheskaia progonoma cerepa. Vopr Onkol 22:95 (Russ) 4. Anagnostopoulos DI, Everard GJH (1972) Melanotic progonoma of the skull. J Neurol, Neurosurg Psychiatry 35:88 5. Ashley DJB (1964) Melanotic "adamantinoma" of the skull. J Pathol Bact 87:179 6. Best PV (1972) Pigmented tumour arising in the skull of an infant. J Pathol 107 : 69 7. Bignold LP, Nicks R (1980) Melanotic neuroectodermal tumour of infancy aris-
H.H. Jones et al. : Case report 617 ing in anterior fontanelle. Pathology 12:473 8. Borello ED, Gorlin RJ (1966) Melanotic neuroectodermal tumor of infancy - a neoplasm of neural crest origin. Report of a case associated with high urinary excretion of vanilmandelic acid. Cancer 19:196 9. Caffey J (1985) Pediatric X-ray diagnosis. 8th edn. Part I : The skull. Year Book Medical Publishers, Chicago, pp 86, 124 10. Causeret M, Saint-Andre JP, CerezPham H, Rousselet MC, Guy G, Simard CP (1987) Progonome melanotique intra-cranien. Arch Anat Cyctol Path 35:45 11. Clarke BE, Parsons H (1951) An embryological tumor of retinal anlage involving the skull. Cancer 4: 78 12. Dahlin DC (1978) Bone tumors: general aspects and data on 6,221 cases. 226259, 3rd edn. Charles C Thomas, Springfield, IL 13. Davis RL (1962) Case number 11 of slide seminar session of the 38th annual meeting of the American Association of Neuropathologists, Atlantic City, N J, June Cited in [31], p 270 14. Dehner LP, Sibley RK, Sauk JJ, Vickers RA, Nesbit ME, Leonard AS, Waite DE, Neeley JE, Ophoven J (1979) Malignant melanotic neuroectodermal tumor of infancy. A clinical, pathologic, ultrastructural and tissue culture study. Cancer 43:1389 15. DePascalis C, Mastroiacovo P, Mastrangelo R (1977) A melanotic neuroectodermal tumor of infancy arising from the anterior fontanel. Tumori 63 : 373 16. Dooling EC, Chi JeG, Gilles FH (1977) Melanotic neuroectodermal tumor of infancy. Its histological similarities to fetal pineal gland. Cancer 39:1535 17. Gilmor RL, Mealey J Jr (1972) Melanotic neuroectodermal tumor involving the cranium in infancy. J Neurosurg. 36: 507 18. Grave GF, Mills AE (1972) Retinal anlage tumors. J Pediatr Surg 7 : 36 19. Jilani SB, Ladgham A, Cammoun M, Ellouz R, Bettaieb A (1982) Le progonome melanique du nourrisson ~t propos de deux observations. Tunisie Medicale 60: 75 20. Johnson RE, Scheithauer BW, Dahlin DC (1982) Melanotic neuroectodermal tumor of infancy. A malignant tumor of the femur. Mayo Clinic Proc 57:719 21. Kasumova S Ju, Komarov VI, Alipcenko LA (1978) Sluchai melanoticheskoi
progonomy svoda cerepa. Vopr Nejrochir 1 : 46 (Russ) 22. Kolluri VRS, Reddy DR, Reddy PK, Naidu MRC, Rao SBP, Sumathi C (1987) Meningiomas in childhood. Childs Nerv Syst 3:271 23. Koudstaal J, Oldhoff J, Panders AK, Hardonk MJ (1968) Melanotic neuroectodermal tumor of infancy. Cancer 22:151 24. Krompecher E (1918) Histogenese und Morphologie der Adamantinome und sonstiger Kiefergeschwulste. Beitr Pathol Anat 64:165 25. Kuhn R, Carbanne F, Garneau R (1954) Le R6tinoblastome Pigmentaire H~t6rotopique. Rapport d'un cas et ~tude g~n6ral. Arch D'Anat Pathol 30me Ann6e:A178 26. Lamping KA, Albert DM, Lack E, Dickersin GR, Chapman PH, Walton DS (1985) Melanotic neuroectodermal tumor of infancy (retinal anlage tumor). Ophthalmology 92:143 27. Mendiratta SS, Rosenblum SA, Strobos RJ (1967) Congenital meningioma. Neurology 17:914 28. Mirkin LD L(1975) Progonoma melandtico; jtumor benigno o maligno? Rev Chil Pediatr 46:125 29. Neustein HB (1967) Fine structure of a melanotic progonoma or retinal anlage tumor of the anterior fontanel. Exp Mol Pathol 6:131 30. Parizek J, Nemecek S, Cernoch Z, Heger L, Nozicka Z, Spacek J (1986) Melanotic neuroectodermal neurocranial tumor of infancy of extra-intra- and subdural right temporal location CT examination, surgical treatment, literature review. Hippokrates, Stuttgart, p 115 31. Rawlings CE III, Wilkins RH (1984) Solitary eosinophilic granuloma of the skull. Neurosurgery 15:155 32. Reyes HA, Gonzales-Angulo A, Navarrete-Reyna A (1964) ,,Retinal anlage tumor of infancy": report of a case occurring in the anterior fontanel. J Pediatr 64: 268 33. Sakamoto K, Kobayashi N, Ohtsubo H, Tanaka Y (1986) Intracranial tumors in the first year of life. Childs Nerv Syst 2:126 34. Stowens D (1957) A pigmented tumour of infancy: the melanotic progonoma. J Pathol Bact 73 : 43 35. Wilner D (1982) Radiology of bone tumors and allied disorders. WB Saunders, Philadelphia, p 2464
1 Skeletal Radio1 (1990) 19:527-530
Case report 617 Henry H. Jones, M.D. 1, Bruce R. Parker, M.D. 1' 3, Cristina G. Ballerio, M.D. 1, Frederic N. Silverman, M.D. 1, 3, and Richard L. Kempson, M.D. 2 Departments of 1 Diagnostic Radiology and Nuclear Medicine, and 2 Pathology, Stanford University, Stanford, California, and 3 Children's Hospital at Stanford, Palo Alto, California, U S A
Radiological studies
Fig. 1. Age 8 weeks. Digitally manipulated tangential image of the skull shows coarse bony spicules (arrow) extending into the soft tissue mass which is displacing the pinna downward and forward Fig. 2. Age 121/2 weeks. Tangential view of the skull shows bony spicules (arrows) projecting both outward and inward Fig. 3. Age 13 weeks. CT shows an extraaxial mass displacing the meninges inward
Clinical information This healthy, well-developed black girl, is the second child of an 18-year-old mother. A small lump behind her right ear was noted at 3 weeks of age, but may have been present since birth. At 4 weeks of age, the mass measured 11/2 x 11/2 cm. The mass grew and radiographs were obtained at age 8 weeks (Fig. 1). The patient was referred to us 4 weeks later when the mass was 4 x 4 cm in size. It was firm nontender, and
Address reprint requests to: Henry H. Jones, M.D., Department of Diagnostic Radiology and Nuclear Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA
fixed to the underlying skull. It had been reported by one examiner as initially moveable, later becoming fixed. The skin over it was freely moveable and did not transilluminate. The physical examination was otherwise normal. She was afebrile and CBC was normal. She was placed on Keflex 1 for 2 weeks with no response. The mass continued to increase in size. Radiographs at age 121/2 weeks (Fig. 2) showed a prominent soft tissue mass displacing the right ear forward. The mass involved the temporal bone and prominent spicules of bone radiated 1 Keflex is a semisynthetic cephalosporin antibiotic
from the skull into the soft tissue mass. CT examination demonstrated inward displacement of the meninges (Fig. 3). The ear was displaced forward and the external auditory canal compressed. A TC 99 diphosphonate bone scan was highly positive in the region of the tumor, but otherwise normal. Extensive laboratory work at this time was normal. Vanilmandelic acid assay was not done. Abdominal CT was normal, specifically with no evidence of an adrenal mass. A chest roentgenogram was normal. The child was taken to surgery at age 13 weeks for removal of the tumor which was adherent to the dura in the region of the lateral and sigmoid sinuses. 9 1990 International Skeletal Society
528
Diagnosis: Melanotic neuroectodermal tumor of infancy (melanotic progonoma 2) involving the calvaria 3 The t u m o r was firm in consistency and cut with some grittiness owing to the intrusive b o n y spicules (Fig. 4). N u m e r e o u s d a r k streaks and spots were present. Histologically noted were alveolar-like clumps a n d strands o f small blue staining cells with scanty c y t o p l a s m (Fig. 5). A r o u n d the periphery o f the clumps were epithelial-like cells containing b r o w n granules which stained positively for melanin (Fig. 5). A fibrous s t r o m a was present a n d b o n y spicules intruded into the t u m o r mass. This appearance is considered characteristic for melanotic neuroectodermal t u m o r o f infancy ( M N T I ) . T h e small d a r k cells raise the question o f n e u r o b l a s t o m a or r h a b d o m y o s a r c o m a , b u t neither o f these tum o r s contains pigmented cells. Melan o m a does n o t occur in this age group. T h e postoperative course was uneventful and the child is well 2x/2 years later.
Discussion This tumor, n o w officially called melanotic n e u r o e c t o d e r m a l t u m o r o f infancy or melanotic p r o g o n o m a , was first described in 1918 by K r o m pecher w h o n a m e d it " c o n g e n i t a l m e l a n o c a r c i n o m a " [24]. Subsequent cases have been described u n d e r a variety o f names, reflecting the site o f origin or suspected histogenesis. Approximately 200 cases have been described. The majority (70%) occur in the maxilla, with 13% in the calvaria, a n d 6 % in the mandible. Occasional cases have h a d their origins elsewhere. Twenty-seven cases have oc2 Progonoma Dorland's Medical Dictionary, 25th edn): " a tumor due to misplacement of tissue as a result of fetal atavism to a stage which does not occur in the life history of the species, but which does occur in ancestral forms of the species." This term was first used by Stowens [34] to indicate his hypothesis as to the origin of these lesions 3 We are using this term to include the frontal, parietal, occipital, temporal, and sphenoid bones
H.H, Jones et al. : Case report 617 Table l. Location of calvarial tumors (n = 27) 13 5 3 5
diagnosis if one is dealing with a rapidly growing, n o n t e n d e r mass fixed to the skull, with skin m o v e a b l e over it a n d b o n y spicules extending into it, in an otherwise healthy infant u n d e r 6 m o n t h s o f age. However, because o f its rarity, very u n c o m m o n presentations o f relatively m u c h c o m m o n e r conditions m u s t be given consideration (Table 3). O f clinical i m p o r t a n c e is the absence o f any clearly m a l i g n a n t case in the g r o u p involving primarily the calvaria. Six patients have died at operation or in the immediate postoperative period. It is n o t e w o r t h y t h a t 75% o f the patients w h o were alive and well following surgical t r e a t m e n t h a d t u m o r s which were smaller t h a n these six w h o died following surgery. O p e r a t i o n w h e n the lesion is small and s o o n after detection appears to be a d v a n t a g e o u s (Fig. 6). The single
Anterior fontanel Temporoparietal Frontotemporosphenoid Other One patient had three tumors: 2 parietal, I orbitosphenoid
curred in the calvaria. Tables 1 and 2 summarize their attributes. In 1966, u r i n a r y vanilmandelic acid was reported as being elevated in a patient with M N T I [8, 14]. Since then, a few additional instances have been recorded. The value was n o r m a l in the f o u r calvarial cases in which the test was performed. T h e m o s t difficult aspect o f the differential diagnosis o f this very unc o m m o n n e o p l a s m is to think o f it. M N T I is the m o s t likely prebiopsy
Table 2. Summary of findings in 27 cases of MNTI involving the calvaria Age first detected
Age when treated
Race
Birth to under 3 months 3-6 months
Under 3 months
4
White
6
Male
16
17
Black
9
Female
11
16 9
3-6 months
Over 6 months 1 Not specified 1
over 6 months Not specified
Size when first seen
Size when operated
Tender?
Under 1 cm 1-3 cm Over 3 cm Not specified
Under 3 cm 3-6 cm Over 6 cm Not specified
Yes
5 4 3 15
6 0
Sex
1 17 7 2
Other Not specified
2 10 Consistency 0
No Not specified
11 16
Firm Not specified
18 9
Adherent to skin
Adherent to skull
Adherent to dura
Calcification
Yes No Not specified
Yes No Not specified
Yes No Not specified
In 19 cases, caMfication mentioned; in 13 of these spiculation specified or illustrated
0 14 13
Treatment Surgery Radiation Chemotherapy Not specified
20 ~ t 6
15 3 9
Results of treatment 24 1 i 1
Up to 2 years follow-up Over 2 years follow up Died in post-op period Died later Not specified
12 7 6 Ib 1
Alive and well
3 cases listed as not adherent to skull when first seen. Later adherent b 1 case treated with chemotherapy only, died age 3 months of pneumonia
529
H.H. Jones et al. : Case report 617
Pathological studies
Fig. 4. Photograph of gross specimen, Arrow points to bony spicules. The black melanin pigment characteristic of this tumor is clearly visible Fig. 5. Histologic section shows alveolar clumps and strands of small blue-staining cells with scanty cytoplasm. Arrow points to peripherally arranged epithelial-like cells that contain granules which stained positively for melanin
14i
O
"~ I2~
glo
b=o= 4 ,
3
2
o
o
~176176 o o
0
Ox 9
6
o 9
0
2
.
,
4
9
.
,
6
.
.
.
,
8
9
9
,
10
.
.
,
9
12
Largest recorded dimension (cm)
patient with recurrence was alive and w e l l 4 y e a r s a f t e r r e s e c t i o n o f t h e recurrence. Rare metastases have been r e p o r t e d [14, 20] in m a x i l l a r y o r mandibular lesions and occasionally f r o m o t h e r sites, b u t n o t f r o m t h e calvaria. An exception to the generally f a v o r a b l e p r o g n o s i s is p r o v i d e d b y nonosseous intracranial lesions which are nearly always fatal because of local or widespread involvement or multifocal lesions within the cen-
.
,
14
Fig. 6. Time between detection and operation or biopsy plotted against largest recorded dimension for the 24 cases for which this information was available, including all cases reported as having died. One case (symbol e) was biopsied only and treated with chemotherapy [28] and died of infection 3 months after the biopsy. Six cases (symbol x) [2, 6, I3, 18, 21, 25] died in the immediate postoperative period. The distribution of these six cases points up the importance of early detection and surgical treatment while the tumor is still of small size
Table 3. Differential features of other lesions which will usually lead to their exclusion
Ewing's sarcoma: The youngest patient ineluded in the 89t cases tabulated from the literature by Wither [35] was 7 months old. Only 2% are primary in the skull and spiculation is not a feature.
Osteosarcoma: Roentgenographic appearance can mimic MNTI. The youngest patient in the Dahlin-Unni series of 962 cases [12] was 2.9 years old.
Table 3. (continued)
Cephalohematoma: The roentgenographic appearance is different and the lack of a history of trauma and failure to increase in size should eliminate this consideration. Infection: Local tenderness and systemic manifestations such as fever and leukocytosis will usually identify infection. An indolent process such as tuberculosis would not show rapid increase in size.
Lymphoma, leukemia, and rhabdomyosarcorna: In the rare event that one of these neoplasms should present as a mass involving the calvaria in a patient under 6 months of age, manifestations elsewhere should exclude M N T I from the differential diagnosis.
Neuroblastoma: Metastasis to the calvaria may present as a soft tissue mass and may stimulate a spiculated bony reaction. However, there are usually lesions elsewhere as well as the primary tumor. Hemangioma: When involving the calvaria this lesion is usually slowly growing and is manifest at an older age. Spiculation occurs, but the usual bone pattern is a honeycomb or reticulated appearance. Fibrous dysplasia. Slowly growing. Usually is manifest at an older age. Spiculation is not a feature. Eosinophilie granuloma: A soft tissue mass involving the infant calvaria would usually be tender. Bony spiculadon is uncommon. Solitary lesion is excessively rare in this age group [31].
Meningioma:
Roentgenographic appearance can mimic MNTI. However, there appear to be but three cases in the literature occurring in infancy [21, 27].
530
tral nervous system. More extensive dicussion is available in the recent literature [1-7, 9-11, 13-21, 23-26, 2833]. In Summary, a case of melanotic neuroectodermal tumor of infancy is presented. The behavior of this case and similar ones indicates that the lesion, when involving primarily the calvaria, is benign. Efforts must be directed toward diagnosis at an early stage when an infant may present with a rapidly growing, fixed lesion of the skull. Early diagnosis should obviate the necessity for extensive, life-threatening surgery. Careful evaluation for early recurrence should permit curative reoperation.
Acknowledgements. We would
like to thank Dr. Franklin C. Buckner for referring this patient to us, Dr. Victor Fish for translation of the Russian articles, and Dr. Jaime Aguet for translation of the articles in Spanish. Dr. John Drace prepared the diagram of Fig. 6. Mark Riesenberger assisted ably with the preparation of the manuscript.
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