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Congenital teratoma of the submandibular gland
Congenital Teratoma of the Submandibular Gland By P.E. Rose and E.R. H o w a r d London, England 9 A case of benign congenital teratoma arising in the submandibular gland is presented and its relationship to the group of oronasopharyngeal and cervical teratomata is discussed. No previous examples of this condition appear to have been published in t h e American or British literature. INDEX W O R D S : Teratoma. submandibular gland
O N G E N I T A L oronasopharyngeal and cervical teratomata constitute an uncommon, but well recognized, group of tumors affecting infants and children. These tumors characteristically occur in the palate, tongue and floor of the mouth, or in the vicinity of the thyroid gland, and are usually situated on or close to the midline. This report presents what appears to be the only case of a benign congenital teratoma arising in the submandibular gland hitherto recorded in either American or British literature.
C
Microscopical examination revealed an irregular mass of central nervous tissue containing numerous fibrillary astroyctes, some oligodendroglia, occasional neurons, and a few irregular slit-like spaces lined by ependyma (Fig. 1 and 2). In some regions this tissue was degenerate and densely infiltrated by foamy macrophages. Apart from the cysts lined by ependyma, there was no evidence of histo-architectural organisation into specific cerebral or spinal structures such as cerebral or cerebellar cortex, nor even into distinct zones of grey or white matter. In the periphery of the tumor there were a number of cysts lined by stratified squamous, respiratory and gastrointestinal types of epithelium. In some of the cysts lined by a columnar or cuboidal epithelium, the epithelium assumed a papillary and hyperplastic appearance, but in no places were there dysplastic or malignant features (Fig. 3). Elsewhere, nerves, irregular masses of actively growing cancellous bone, hemopoietic tissue and adipose tissue were recognised (Fig. 4). The tumor was encircled and separated from the surrounding salivary tissue by an irregular mantle of connective tissue which was contiguous on one side with the fibrous stroma within the tumor, and on the other side with the stroma of the salivary gland.
CASE REPORT
DISCUSSION
A.R., a 3,360 g female infant, was born after 38 wk gestation and a maternal history of two previous miscarriages. A 2.0 cm diameter cystic swelling in the left submandibular region was observed on the second day and diagnosed as a lymphangioma. X-rays revealed no bony abnormality. The mass enlarged slowly during the subsequent three months and excision was therefore advised. At operation a cystic mass was found attached to the left submandibular gland and extending deeply towards the base of the tongue. The cysts contained opalescent fluid. The lesion was completely excised in continuity with the submandibular gland and with preservation of the lingual and hypoglossal nerves. Eighteen months after excision there is no evidence of recurrence.
T h e c e r v i c a l , o r o n a s o p h a r y n g e a l a n d basic r a n i a l r e g i o n s c o n s t i t u t e t h e sites o f o c c u r r e n c e of a small proportion of the teratomata of infancy a n d c h i l d h o o d . In t h e series o f 91 c a s e s o f i n f a n tile o r c h i l d h o o d t e r a t o m a t a r e v i e w e d by B e r r y et al., 1 t h r e e t u m o r s o c c u r r e d in t h e v i c i n i t y o f t h e t h y r o i d g l a n d a n d t w o t u m o r s in t h e p a l a t e . T h i s i n c i d e n c e is a p p r o x i m a t e l y c o m p a r a b l e to t h a t r e p o r t e d by M a h o u r et al. 2 w h o r e c o r d o n e c a s e o f p a l a t a l t e r a t o m a in t h e i r series o f 85 cases, a n d C a r n e y et al. 3 w h o r e c o r d o n e c a s e o f o r a l t e r a t o m a in a series o f 58 cases, N e i t h e r o f t h e l a t t e r t w o series i n c l u d e d a n y c a s e s o f c e r v i c a l t e r a t o m a . I n a d d i t i o n to t h e s e m a j o r series o f cases, s e v e r a l r e v i e w s , r e p o r t s o f series o f cases, a n d s i n g l e c a s e s o f t e r a t o m a t a in t h e n e c k , 4-~7 t o n g u e '8'~9 a n d n a s o p h a r y n x 2~ h a v e b e e n p u b -
Pathology The specimen consisted of an irregular piece of soft tissue measuring approximately 3 • 2 • 2 cm. The external surface was greyish-brown, being partly composed of muscle. The bulk of the specimen consisted of a soft, slightly variegated pale yellowish-grey tissue which contained a few cysts measuring between 0.2 cms and 0.5 cm across.
From the Department of Morbid Anatomy and The Department of Surgery, King's College Hospital, London, England. Address reprint requests to E.R. Howard, M.S., F.R.C.S~, Consultant Surgeon, King's College Hospital, Denmark Hill, London SE5 9RS. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/1704-0019501.00/0 414
lished. In t h e oral, o r o n a s o p h a r y n g e a l and b a s i c r a n i a l r e g i o n s t e r a t o m a t a a r e c h a r a c t e r i s t i c a l l y situa t e d on or close to t h e m i d l i n e . O n t h e o t h e r hand, cervical teratomata frequently assume r e l a t i v e l y e n o r m o u s sizes w i t h c o n s i d e r a b l e late r a l e x t e n s i o n , a n d t h e site o f o r i g i n is o f t e n difficult to i d e n t i f y . H o w e v e r , B a l e 4 r e v i e w e d 56 c a s e s o f t e r a t o m a in t h e v i c i n i t y o f t h e t h y r o i d a n d f o u n d t h a t in 44 c a s e s ( 7 8 . 6 % ) t h e t u m o r was Journal of Pediatric Surgery, Vol. 17, No. 4 (August), 1982
TERATOMA OF SUBMANDIBULAR GLAND
415
Fig. 1. Field demonstrating neural tissues including ependymal spaces. Haematoxylin and eosin; • 125.
either partially or completely surrounded by and replacing part of the thyroid, or derived its blood supply from one or more of the thyroid arteries. In eleven of the other twelve cases (19.6%) in Bale's series there was no specific reference to the thyroid, and only in the remaining case were the tumour and thyroid noted to be separate. Other authors 5-j7 report a lower incidence of definite involvement of the thyroid by cervical teratomata than Bale, although thyroid involvement is present in certain cases of Daniels, 5 Hinds et al, 7 Keynes, 9 McGoon, l~ Otken, ~l Perkins and Pautler, ~2and Retief. ~3Thus, although a prominent lateral component is frequently encountered in cervical teratomata, a large proportion appear to arise paramedially, usually
Fig. 2. Area of neuroglial tissue illustrating isolated neurones, astrocytes and oligodendroglia. Haematoxylin and eosin; • 450.
Fig. 3. Field composed mainly of epithelial elements some of which exhibit a degree of hyperplasis. There'is, however, no evidence of dyspiasia or malignancy. Haematoxylin and eosin; • 140.
within or adjacent to the thyroid gland. Our review of the literature suggests that teratomata of extreme lateral structures of the oronasopharynx, basicranium and neck are rare, and we have been unable to discover any reports of teratomata in the salivary glands of infants or children. It is interesting to note, however, that Shadid et al. 21 reported a case of well-differentiated teratoma involving the right parotid gland of a 2 4 - y e a r old woman. These authors also note that no cases of teratoma of the parotid gland had been recorded amongst more than 11,000 cases of teratoma in the files of the Armed Forces Institute of Pathology. In addition to its apparently unique site, the tumour in our case is clinically unusual on account of its relatively small size. In this respect our case is similar to the rare group of oral and oronasopharyngeal teratomata in which the mean diameter is approximately 2 cm, and seldom exceeds 4 cm. In contrast, we found that the much more frequently encountered cervical teratomata have a mean diameter of 7 cm, and in approximately 30% of the cases the diameter exceeds 10 cm. The only example of a cervical teratoma of approximately the same size as ours which we have been able to discover is that of Irvine. 8 Histologically, teratomata in the cervical and oronasopharyngeal regions may contain tissues derived from all three embryonic layers, but in more than 70% of cases reviewed by Bale, 4 Hajdu et al, 6 Rosedale, 14 and Saphir ~5 the greater part
416
ROSE AND HOWARD
mal elements including bone, bone m a r r o w a n d adipose tissue. A l t h o u g h in some of the cysts the epithelium was thrown into mildly hyperplastic papillary folds, no dysplastic features were detected. REFERENCES
Fig. 4. Part of teratoma composed of bone and bone marrow, demonstrating considerable evidence of bone remodelling and extremely cellular bone marrow. Haematoxylin and eosin; x 68.
of the t u m o r was composed of n e u r o e c t o d e r m a l elements including central nervous tissues. S u c h tissue usually exhibits a high degree of m a t u r i t y , being almost i n v a r i a b l y composed of w e l l formed astrocytes, oligodendroglia, neurons a n d ependyma, although regions resembling embryonic neural tissues are occasionally noted, as in one case m e n t i o n e d by Berry et al. ~ G e n e r a l l y there is no evidence of c y t o - a r c h i t e c t o n i c differentiation in the central nervous c o m p o n e n t of a teratoma. O f the other types of tissue which occur in these tumours, epidermis sometimes with adnexal structures, cysts lined by respiratory or gastrointestinal epithelium, bone, bone marrow, a n d smooth muscle are frequently encountered. In the majority of cases these tissues are fully differentiated a n d exhibit no i m m a t u r e , dysplastic, or m a l i g n a n t features. However, of the five cases occurring in the thyroid and palatal regions reported by Berry 1 one t u m o r contained areas of e m b r y o n i c neuroectodermal tissue a n d another contained some embryonic epithelium. O u r case conforms to the u s u a l histological pattern, with a p r e d o m i n a n c e of central nervous tissue, spaces lined by stratified squamous, cuboidal a n d c o l u m n a r epithelia, a n d mesenchy-
1. Berry CL, Keeling J, et al: Teratomata of infancy and childhood: A review of 91 cases. J Pathol 98:241-252, 1969 2. Mahour GH, WoolleyMM, et al: Teratomas in infancy and childhood: Experiencewith 81 cases. Surgery 76(2):309318, 1974 3. Carney JA, Thompson DP, et al: Teratomas in children: Clinical and pathological aspects. J Pediatr Surg 7(3):271282, 1972 4. Bale GF: Teratoma of the neck in the region of the thyroid gland. Am J Pathol 26(2):565-579, 1950 5. Daniels DW: A congenital tumor of the neck. Br J Surg 15:523-525, 1927 6. Hajdu SI, Faruque AA, et al: Teratoma of the neck in infants. Am J Dis Child 111:412-416, 1966 7. Hinds EC, Seybold WD, et al: Teratoma of the neck-Report of a case. Tex Rep Biol Med 12:86-93, 1954 8. Irvine DW: Cervical teratoma in the newborn. Arch Otolaryngol 82:546-549, 1965 9. Keynes WM: Teratoma of the neck in relation to the thyroid gland. Br J Surg 46:466-472, 1958 10. McGoonDC: Teratomas of the neck. Surg Clin North Am 32:1389-1395, 1952 11. Otken LB: Teratoma of the thyroid gland. Am J Surg 85:240-242, 1953 12. Perkins RB, Pautler EE: Teratoma of the neck apparently arising in the thyroid. Surgery 33:742-747, 1953 13. Reteif PJ: Cervical teratoma: Is it rare? S Afr J Surg 4(4):181-185, 1966 14. Rosedale RA: Neck teratomas. Arch Otolaryngol 82:535-542, 1965 15. Saphir O: Teratoma of the neck. Am J Pathol 5:313321, 1929 16. Silberman R, Mendelson IR: Teratoma of the neck. Arch Dis Child 35:159-170, 1960 17. White RR and Gosselin RS: Teratoma of the neck in a newborn infant. Pediatrics 9:565-571, 1952 18. Bras G, Butts D, et al: Gliomatous Teratoma of the Tongue. Cancer 24:1045-1050, 1969 19. Grier EA, MacNerland RH: Benign teratoma of the tongue. Ill Med J 132:43-45, 1967 20. Crook JP: Nasopharyngeal teratoma. J Term Med Assoc 58:372-373, 1965 21. Shadid EA, Engeron O'N, et al: Benign teratoid tumour of the parotid. Plast Reconstr Surg 55(3):363-365, 1975
O N G E N I T A L oronasopharyngeal and cervical teratomata constitute an uncommon, but well recognized, group of tumors affecting infants and children. These tumors characteristically occur in the palate, tongue and floor of the mouth, or in the vicinity of the thyroid gland, and are usually situated on or close to the midline. This report presents what appears to be the only case of a benign congenital teratoma arising in the submandibular gland hitherto recorded in either American or British literature.
C
Microscopical examination revealed an irregular mass of central nervous tissue containing numerous fibrillary astroyctes, some oligodendroglia, occasional neurons, and a few irregular slit-like spaces lined by ependyma (Fig. 1 and 2). In some regions this tissue was degenerate and densely infiltrated by foamy macrophages. Apart from the cysts lined by ependyma, there was no evidence of histo-architectural organisation into specific cerebral or spinal structures such as cerebral or cerebellar cortex, nor even into distinct zones of grey or white matter. In the periphery of the tumor there were a number of cysts lined by stratified squamous, respiratory and gastrointestinal types of epithelium. In some of the cysts lined by a columnar or cuboidal epithelium, the epithelium assumed a papillary and hyperplastic appearance, but in no places were there dysplastic or malignant features (Fig. 3). Elsewhere, nerves, irregular masses of actively growing cancellous bone, hemopoietic tissue and adipose tissue were recognised (Fig. 4). The tumor was encircled and separated from the surrounding salivary tissue by an irregular mantle of connective tissue which was contiguous on one side with the fibrous stroma within the tumor, and on the other side with the stroma of the salivary gland.
CASE REPORT
DISCUSSION
A.R., a 3,360 g female infant, was born after 38 wk gestation and a maternal history of two previous miscarriages. A 2.0 cm diameter cystic swelling in the left submandibular region was observed on the second day and diagnosed as a lymphangioma. X-rays revealed no bony abnormality. The mass enlarged slowly during the subsequent three months and excision was therefore advised. At operation a cystic mass was found attached to the left submandibular gland and extending deeply towards the base of the tongue. The cysts contained opalescent fluid. The lesion was completely excised in continuity with the submandibular gland and with preservation of the lingual and hypoglossal nerves. Eighteen months after excision there is no evidence of recurrence.
T h e c e r v i c a l , o r o n a s o p h a r y n g e a l a n d basic r a n i a l r e g i o n s c o n s t i t u t e t h e sites o f o c c u r r e n c e of a small proportion of the teratomata of infancy a n d c h i l d h o o d . In t h e series o f 91 c a s e s o f i n f a n tile o r c h i l d h o o d t e r a t o m a t a r e v i e w e d by B e r r y et al., 1 t h r e e t u m o r s o c c u r r e d in t h e v i c i n i t y o f t h e t h y r o i d g l a n d a n d t w o t u m o r s in t h e p a l a t e . T h i s i n c i d e n c e is a p p r o x i m a t e l y c o m p a r a b l e to t h a t r e p o r t e d by M a h o u r et al. 2 w h o r e c o r d o n e c a s e o f p a l a t a l t e r a t o m a in t h e i r series o f 85 cases, a n d C a r n e y et al. 3 w h o r e c o r d o n e c a s e o f o r a l t e r a t o m a in a series o f 58 cases, N e i t h e r o f t h e l a t t e r t w o series i n c l u d e d a n y c a s e s o f c e r v i c a l t e r a t o m a . I n a d d i t i o n to t h e s e m a j o r series o f cases, s e v e r a l r e v i e w s , r e p o r t s o f series o f cases, a n d s i n g l e c a s e s o f t e r a t o m a t a in t h e n e c k , 4-~7 t o n g u e '8'~9 a n d n a s o p h a r y n x 2~ h a v e b e e n p u b -
Pathology The specimen consisted of an irregular piece of soft tissue measuring approximately 3 • 2 • 2 cm. The external surface was greyish-brown, being partly composed of muscle. The bulk of the specimen consisted of a soft, slightly variegated pale yellowish-grey tissue which contained a few cysts measuring between 0.2 cms and 0.5 cm across.
From the Department of Morbid Anatomy and The Department of Surgery, King's College Hospital, London, England. Address reprint requests to E.R. Howard, M.S., F.R.C.S~, Consultant Surgeon, King's College Hospital, Denmark Hill, London SE5 9RS. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/1704-0019501.00/0 414
lished. In t h e oral, o r o n a s o p h a r y n g e a l and b a s i c r a n i a l r e g i o n s t e r a t o m a t a a r e c h a r a c t e r i s t i c a l l y situa t e d on or close to t h e m i d l i n e . O n t h e o t h e r hand, cervical teratomata frequently assume r e l a t i v e l y e n o r m o u s sizes w i t h c o n s i d e r a b l e late r a l e x t e n s i o n , a n d t h e site o f o r i g i n is o f t e n difficult to i d e n t i f y . H o w e v e r , B a l e 4 r e v i e w e d 56 c a s e s o f t e r a t o m a in t h e v i c i n i t y o f t h e t h y r o i d a n d f o u n d t h a t in 44 c a s e s ( 7 8 . 6 % ) t h e t u m o r was Journal of Pediatric Surgery, Vol. 17, No. 4 (August), 1982
TERATOMA OF SUBMANDIBULAR GLAND
415
Fig. 1. Field demonstrating neural tissues including ependymal spaces. Haematoxylin and eosin; • 125.
either partially or completely surrounded by and replacing part of the thyroid, or derived its blood supply from one or more of the thyroid arteries. In eleven of the other twelve cases (19.6%) in Bale's series there was no specific reference to the thyroid, and only in the remaining case were the tumour and thyroid noted to be separate. Other authors 5-j7 report a lower incidence of definite involvement of the thyroid by cervical teratomata than Bale, although thyroid involvement is present in certain cases of Daniels, 5 Hinds et al, 7 Keynes, 9 McGoon, l~ Otken, ~l Perkins and Pautler, ~2and Retief. ~3Thus, although a prominent lateral component is frequently encountered in cervical teratomata, a large proportion appear to arise paramedially, usually
Fig. 2. Area of neuroglial tissue illustrating isolated neurones, astrocytes and oligodendroglia. Haematoxylin and eosin; • 450.
Fig. 3. Field composed mainly of epithelial elements some of which exhibit a degree of hyperplasis. There'is, however, no evidence of dyspiasia or malignancy. Haematoxylin and eosin; • 140.
within or adjacent to the thyroid gland. Our review of the literature suggests that teratomata of extreme lateral structures of the oronasopharynx, basicranium and neck are rare, and we have been unable to discover any reports of teratomata in the salivary glands of infants or children. It is interesting to note, however, that Shadid et al. 21 reported a case of well-differentiated teratoma involving the right parotid gland of a 2 4 - y e a r old woman. These authors also note that no cases of teratoma of the parotid gland had been recorded amongst more than 11,000 cases of teratoma in the files of the Armed Forces Institute of Pathology. In addition to its apparently unique site, the tumour in our case is clinically unusual on account of its relatively small size. In this respect our case is similar to the rare group of oral and oronasopharyngeal teratomata in which the mean diameter is approximately 2 cm, and seldom exceeds 4 cm. In contrast, we found that the much more frequently encountered cervical teratomata have a mean diameter of 7 cm, and in approximately 30% of the cases the diameter exceeds 10 cm. The only example of a cervical teratoma of approximately the same size as ours which we have been able to discover is that of Irvine. 8 Histologically, teratomata in the cervical and oronasopharyngeal regions may contain tissues derived from all three embryonic layers, but in more than 70% of cases reviewed by Bale, 4 Hajdu et al, 6 Rosedale, 14 and Saphir ~5 the greater part
416
ROSE AND HOWARD
mal elements including bone, bone m a r r o w a n d adipose tissue. A l t h o u g h in some of the cysts the epithelium was thrown into mildly hyperplastic papillary folds, no dysplastic features were detected. REFERENCES
Fig. 4. Part of teratoma composed of bone and bone marrow, demonstrating considerable evidence of bone remodelling and extremely cellular bone marrow. Haematoxylin and eosin; x 68.
of the t u m o r was composed of n e u r o e c t o d e r m a l elements including central nervous tissues. S u c h tissue usually exhibits a high degree of m a t u r i t y , being almost i n v a r i a b l y composed of w e l l formed astrocytes, oligodendroglia, neurons a n d ependyma, although regions resembling embryonic neural tissues are occasionally noted, as in one case m e n t i o n e d by Berry et al. ~ G e n e r a l l y there is no evidence of c y t o - a r c h i t e c t o n i c differentiation in the central nervous c o m p o n e n t of a teratoma. O f the other types of tissue which occur in these tumours, epidermis sometimes with adnexal structures, cysts lined by respiratory or gastrointestinal epithelium, bone, bone marrow, a n d smooth muscle are frequently encountered. In the majority of cases these tissues are fully differentiated a n d exhibit no i m m a t u r e , dysplastic, or m a l i g n a n t features. However, of the five cases occurring in the thyroid and palatal regions reported by Berry 1 one t u m o r contained areas of e m b r y o n i c neuroectodermal tissue a n d another contained some embryonic epithelium. O u r case conforms to the u s u a l histological pattern, with a p r e d o m i n a n c e of central nervous tissue, spaces lined by stratified squamous, cuboidal a n d c o l u m n a r epithelia, a n d mesenchy-
1. Berry CL, Keeling J, et al: Teratomata of infancy and childhood: A review of 91 cases. J Pathol 98:241-252, 1969 2. Mahour GH, WoolleyMM, et al: Teratomas in infancy and childhood: Experiencewith 81 cases. Surgery 76(2):309318, 1974 3. Carney JA, Thompson DP, et al: Teratomas in children: Clinical and pathological aspects. J Pediatr Surg 7(3):271282, 1972 4. Bale GF: Teratoma of the neck in the region of the thyroid gland. Am J Pathol 26(2):565-579, 1950 5. Daniels DW: A congenital tumor of the neck. Br J Surg 15:523-525, 1927 6. Hajdu SI, Faruque AA, et al: Teratoma of the neck in infants. Am J Dis Child 111:412-416, 1966 7. Hinds EC, Seybold WD, et al: Teratoma of the neck-Report of a case. Tex Rep Biol Med 12:86-93, 1954 8. Irvine DW: Cervical teratoma in the newborn. Arch Otolaryngol 82:546-549, 1965 9. Keynes WM: Teratoma of the neck in relation to the thyroid gland. Br J Surg 46:466-472, 1958 10. McGoonDC: Teratomas of the neck. Surg Clin North Am 32:1389-1395, 1952 11. Otken LB: Teratoma of the thyroid gland. Am J Surg 85:240-242, 1953 12. Perkins RB, Pautler EE: Teratoma of the neck apparently arising in the thyroid. Surgery 33:742-747, 1953 13. Reteif PJ: Cervical teratoma: Is it rare? S Afr J Surg 4(4):181-185, 1966 14. Rosedale RA: Neck teratomas. Arch Otolaryngol 82:535-542, 1965 15. Saphir O: Teratoma of the neck. Am J Pathol 5:313321, 1929 16. Silberman R, Mendelson IR: Teratoma of the neck. Arch Dis Child 35:159-170, 1960 17. White RR and Gosselin RS: Teratoma of the neck in a newborn infant. Pediatrics 9:565-571, 1952 18. Bras G, Butts D, et al: Gliomatous Teratoma of the Tongue. Cancer 24:1045-1050, 1969 19. Grier EA, MacNerland RH: Benign teratoma of the tongue. Ill Med J 132:43-45, 1967 20. Crook JP: Nasopharyngeal teratoma. J Term Med Assoc 58:372-373, 1965 21. Shadid EA, Engeron O'N, et al: Benign teratoid tumour of the parotid. Plast Reconstr Surg 55(3):363-365, 1975