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Teratoid tumors of the nasopharynx in children
Teratoid Tumors of the Nasopharynx in Children By C. R. BOECKMAN
I
N EVALUATING RESPIRATORY OBSTRUCTION in infants and children the differential diagnosis rarely includes nasopharyngeal tumors. Teratoid tumors may occur anywhere in the body but are usually situated in the gonads, in the midline, or along lines of fusion. Those originating in the pharyngeal area may arise anywhere around the nasopharynx, pharynx, uvula, or soft palate and are usually symptomatic shortly after birth. Ewing* described three classes of developmental anomalies appearing in the form of polypoid growths of the nasopharynx: (1) dermoids (hairy polyps), (2) teratomas, and (3) epignathi. Dermoids are composed of tissue derived from ectoderm and mesoderm, whereas teratomas and epignathi are derived from all three germ layers. In addition, epignathi consist of well-formed organs and limbs of a parasitic fetus. In general, teratoma and epignathi are found in deformed stillborn fetuses while teratoid tumors occur in otherwise healthy infants and may go unrecognized. This report is concerned with those tumors classified as dermoids or hairy POlYPs. CASE
REPORTS
Case 1: A female infant of 12 hours was noted to choke, become cyanotic, and stop breathing when placed on her back. There was a mass in the oropharynx and the baby was transferred to the Akron Children’s Hospital in acute respiratory distress. An endotracheal tube was inserted which relieved the airway obstruction. Subsequently, x-rays showed a calcified mass in the nasopharynx, best seen in the lateral view. Tracheostomy was performed, but 72 hours later the infant was found dead in the incubator. At autopsy a polypoid mass was found attached to the posterior pharyngeal wall by a 1 cm. stalk. Death was caused by a mucous plug at the tracheal bifurcation. Case 3: A female infant born at 8 months gestation had a large mass in the oropharynx at birth. A tracheostomy was performed in the delivery room and the baby transferred to our hospital. On admission the patient’s temperature was unrecordable, she was deeply cyanotic, and died shortly after admission. At autopsy a 3 X 3.5 cm. mass attached to the right lateral pharyngeal wall by a 6 mm. stalk was found. Microscopically the tumor onsisted of fat, muscle, cartilage, and brain tissue. Case 3: A full-term female infant had two bouts of cyanosis and respiratory difficulty during the first 48 hours of life and had an abnormal amount of mucus from the nose and mouth. A catheter was passed through both nostrils without obstruction. A more careful examination of the oropharynx revealed a mass just visible below the soft palate, which could also be seen on lateral films of the head (Fig. 1A). The patient was promptly operated upon and at the time of surgery the mass could be delivered into the mouth (Fig. 1B). It measured 3 cm. in greatest dimension and was attached to the left lateral pharyngeal wall by a narrow stalk. It was removed with the electrocautery. The tumor was covered by From the Division
of Pediatric
Surgery
Akron Children’s
Hospital, Akron, Ohio.
JOURNAL OF PEDUTFUC SURGERY,VOL. 3, NO. 6 @ECEMBER), 1968
735
736
C. R. BOECKM AN
Fig. 1.-A, Lateral film of neck, Case 3. Tumor is situated behind soft 1Ialatte. Tumor delivered into mouth at time of surgery.
B,
TERATOID
TUMORS
Fig. 1 .-C, represented.
OF THE
NASOPHARYNX
Gross specimen.
D, Cross section of tumor.
737
Two germ layers are
epidermis (Fig. IC), and microscopically consisted of fat, blood vessels, and cartilage (Fig. 1D). Case 4: A full-term female infant developed intercostal retraction and cyanosis while On x-ray feeding. Attempts at passin g a catheter through the nose were unsuccessful. examination of the neck a tumor mass was seen in the pharynx. The mass was attached to the posterior tonsillar pillar and could be delivered into the mouth. It measured 2 cm. in greatest diameter and consisted of fat and cartilage covered by stratifiecl squamous epithelium containing abortive hair follicles and sebaceous glands.
C.
738
R. BOECKMAN
Case 5: A 13-month-old female was found to have a pharyngeal tumor on a routine physical examination for an upper respiratory infection. The only pertinent history was difficulty in swallowing since birth. A 2 cm. long tumor containing fibroadipose tissue covered by squamous epithelium containing hair follicles and sebaceous glands was successfully removed.
DISCUSSION Teratoid tumors have been of interest to teratologists for the past one hundred years and clinical papers by Ehrich,2 Walker,3 Foxwell and Cadman5 have contributed to their understanding. Older views have interpreted these tumors as masses or fragments of viscera belonging to a suppressed fetus. G More recent views would favor a disturbance during the fusion of embryonic development. During the fusion stages, in the branchiogenic area, there may be incomplete, inadequate, or improper fusion at any one point. This may cause a cell or group of cells to be separated and attempt differentiation into a second embryo. The disorganized pattern of these tumors would suggest that the necessary environmental conditions were lacking and as a result true organization fails to occur. Diagnosis can usually be made on examination suggested by respiratory symptoms. Respiratory distress in the absence of obvious causes calls for thorough examination of the nasopharynx. The value of the lateral x-ray of the nasopharynx in diagnosing tumors not visible through the mouth has been evident in our material. More than half of these tumors are diagnosed during the first year of life and occur primarily in the female in the ratio of 6:l. All of our patients were females. Our experience with this small group of patients would suggest that tracheostomy is rarely necessary. These tumors should be removed promptly once the diagnosis is established. This can best be done under endotracheal anesthesia with the use of electrocautery to control bleeding. SUMMARY Benign nasopharyngeal teratomas are rare lesions that can give rise to sudden severe respiratory distress in the neonatal period. Symptoms at birth, however, may be mild and consist of excessive nasal mucus and intermittent cyanosis, especially when feeding or in the supine position. Experience with five such tumors indicates that the diagnosis can usually be made by careful examination of the pharyngeal area. These tumors are frequently mobile and pedunculated and should be promptly removed to prevent sudden death from airway obstruction. SUMMARIO IN INTERLINGUA Un causa
rar de obstruction
nasopharyngee. duraate Le
Minor
le period0 diagnose
roentgenographia
es
de1 vias aeree in neonatos
symptomas
de excessive
neonatal
deberea
establite
per
lateral
de1 cello.
un Le
suggerer
muco
e juvene infantes
nasal
le suspicion
meticulose condition
examine es plus
e de cyanosis de teratoma de1
area
commun
es teratoma intermittente
nasopharyngee.
pharyngee
in femininas
e que
per in
739
TERATOID TUMORS OF THE NASOPHARYNX
masculos
con un proportion
pro prevenir
de 4:l.
un subite obstruction
Le tractamento
con&e
de1 prompte
excision
chirurgic
de1 vias aeree. REFERENCES
1. Ewing, Philadelphia,
J.: Neoplastic Diseases, W. B. Saunders, 1941.
ed. 4.
2. Ehrich, W. E.: Teratoid parasites of the mouth. Amer. J. Oral Surg. 31:650, 1945. .3. Walker, E. A., Rigual, J. R., and Hough. J. V. D.: Teratomas of the pharynx. .%mer. Surg. 29:219, 1963. 4. Foxwell, P. B., and Kelham, B. H.: Teratoid tumors of the nasopharynx. J.
Laryngol.
Otol. 72:647,
1958.
5. Cadman, T. A., and Kintzen, W.: Nasopharyngeal teratoma. Canad. Med. Ass. J. 88666, 1963. 6. Bland-Sutton, J.: Tumors: Innocent and Malignant, Their Characters and Appropriate Treatment, ed. 7. London, Cassell & Co., 1922.
I
N EVALUATING RESPIRATORY OBSTRUCTION in infants and children the differential diagnosis rarely includes nasopharyngeal tumors. Teratoid tumors may occur anywhere in the body but are usually situated in the gonads, in the midline, or along lines of fusion. Those originating in the pharyngeal area may arise anywhere around the nasopharynx, pharynx, uvula, or soft palate and are usually symptomatic shortly after birth. Ewing* described three classes of developmental anomalies appearing in the form of polypoid growths of the nasopharynx: (1) dermoids (hairy polyps), (2) teratomas, and (3) epignathi. Dermoids are composed of tissue derived from ectoderm and mesoderm, whereas teratomas and epignathi are derived from all three germ layers. In addition, epignathi consist of well-formed organs and limbs of a parasitic fetus. In general, teratoma and epignathi are found in deformed stillborn fetuses while teratoid tumors occur in otherwise healthy infants and may go unrecognized. This report is concerned with those tumors classified as dermoids or hairy POlYPs. CASE
REPORTS
Case 1: A female infant of 12 hours was noted to choke, become cyanotic, and stop breathing when placed on her back. There was a mass in the oropharynx and the baby was transferred to the Akron Children’s Hospital in acute respiratory distress. An endotracheal tube was inserted which relieved the airway obstruction. Subsequently, x-rays showed a calcified mass in the nasopharynx, best seen in the lateral view. Tracheostomy was performed, but 72 hours later the infant was found dead in the incubator. At autopsy a polypoid mass was found attached to the posterior pharyngeal wall by a 1 cm. stalk. Death was caused by a mucous plug at the tracheal bifurcation. Case 3: A female infant born at 8 months gestation had a large mass in the oropharynx at birth. A tracheostomy was performed in the delivery room and the baby transferred to our hospital. On admission the patient’s temperature was unrecordable, she was deeply cyanotic, and died shortly after admission. At autopsy a 3 X 3.5 cm. mass attached to the right lateral pharyngeal wall by a 6 mm. stalk was found. Microscopically the tumor onsisted of fat, muscle, cartilage, and brain tissue. Case 3: A full-term female infant had two bouts of cyanosis and respiratory difficulty during the first 48 hours of life and had an abnormal amount of mucus from the nose and mouth. A catheter was passed through both nostrils without obstruction. A more careful examination of the oropharynx revealed a mass just visible below the soft palate, which could also be seen on lateral films of the head (Fig. 1A). The patient was promptly operated upon and at the time of surgery the mass could be delivered into the mouth (Fig. 1B). It measured 3 cm. in greatest dimension and was attached to the left lateral pharyngeal wall by a narrow stalk. It was removed with the electrocautery. The tumor was covered by From the Division
of Pediatric
Surgery
Akron Children’s
Hospital, Akron, Ohio.
JOURNAL OF PEDUTFUC SURGERY,VOL. 3, NO. 6 @ECEMBER), 1968
735
736
C. R. BOECKM AN
Fig. 1.-A, Lateral film of neck, Case 3. Tumor is situated behind soft 1Ialatte. Tumor delivered into mouth at time of surgery.
B,
TERATOID
TUMORS
Fig. 1 .-C, represented.
OF THE
NASOPHARYNX
Gross specimen.
D, Cross section of tumor.
737
Two germ layers are
epidermis (Fig. IC), and microscopically consisted of fat, blood vessels, and cartilage (Fig. 1D). Case 4: A full-term female infant developed intercostal retraction and cyanosis while On x-ray feeding. Attempts at passin g a catheter through the nose were unsuccessful. examination of the neck a tumor mass was seen in the pharynx. The mass was attached to the posterior tonsillar pillar and could be delivered into the mouth. It measured 2 cm. in greatest diameter and consisted of fat and cartilage covered by stratifiecl squamous epithelium containing abortive hair follicles and sebaceous glands.
C.
738
R. BOECKMAN
Case 5: A 13-month-old female was found to have a pharyngeal tumor on a routine physical examination for an upper respiratory infection. The only pertinent history was difficulty in swallowing since birth. A 2 cm. long tumor containing fibroadipose tissue covered by squamous epithelium containing hair follicles and sebaceous glands was successfully removed.
DISCUSSION Teratoid tumors have been of interest to teratologists for the past one hundred years and clinical papers by Ehrich,2 Walker,3 Foxwell and Cadman5 have contributed to their understanding. Older views have interpreted these tumors as masses or fragments of viscera belonging to a suppressed fetus. G More recent views would favor a disturbance during the fusion of embryonic development. During the fusion stages, in the branchiogenic area, there may be incomplete, inadequate, or improper fusion at any one point. This may cause a cell or group of cells to be separated and attempt differentiation into a second embryo. The disorganized pattern of these tumors would suggest that the necessary environmental conditions were lacking and as a result true organization fails to occur. Diagnosis can usually be made on examination suggested by respiratory symptoms. Respiratory distress in the absence of obvious causes calls for thorough examination of the nasopharynx. The value of the lateral x-ray of the nasopharynx in diagnosing tumors not visible through the mouth has been evident in our material. More than half of these tumors are diagnosed during the first year of life and occur primarily in the female in the ratio of 6:l. All of our patients were females. Our experience with this small group of patients would suggest that tracheostomy is rarely necessary. These tumors should be removed promptly once the diagnosis is established. This can best be done under endotracheal anesthesia with the use of electrocautery to control bleeding. SUMMARY Benign nasopharyngeal teratomas are rare lesions that can give rise to sudden severe respiratory distress in the neonatal period. Symptoms at birth, however, may be mild and consist of excessive nasal mucus and intermittent cyanosis, especially when feeding or in the supine position. Experience with five such tumors indicates that the diagnosis can usually be made by careful examination of the pharyngeal area. These tumors are frequently mobile and pedunculated and should be promptly removed to prevent sudden death from airway obstruction. SUMMARIO IN INTERLINGUA Un causa
rar de obstruction
nasopharyngee. duraate Le
Minor
le period0 diagnose
roentgenographia
es
de1 vias aeree in neonatos
symptomas
de excessive
neonatal
deberea
establite
per
lateral
de1 cello.
un Le
suggerer
muco
e juvene infantes
nasal
le suspicion
meticulose condition
examine es plus
e de cyanosis de teratoma de1
area
commun
es teratoma intermittente
nasopharyngee.
pharyngee
in femininas
e que
per in
739
TERATOID TUMORS OF THE NASOPHARYNX
masculos
con un proportion
pro prevenir
de 4:l.
un subite obstruction
Le tractamento
con&e
de1 prompte
excision
chirurgic
de1 vias aeree. REFERENCES
1. Ewing, Philadelphia,
J.: Neoplastic Diseases, W. B. Saunders, 1941.
ed. 4.
2. Ehrich, W. E.: Teratoid parasites of the mouth. Amer. J. Oral Surg. 31:650, 1945. .3. Walker, E. A., Rigual, J. R., and Hough. J. V. D.: Teratomas of the pharynx. .%mer. Surg. 29:219, 1963. 4. Foxwell, P. B., and Kelham, B. H.: Teratoid tumors of the nasopharynx. J.
Laryngol.
Otol. 72:647,
1958.
5. Cadman, T. A., and Kintzen, W.: Nasopharyngeal teratoma. Canad. Med. Ass. J. 88666, 1963. 6. Bland-Sutton, J.: Tumors: Innocent and Malignant, Their Characters and Appropriate Treatment, ed. 7. London, Cassell & Co., 1922.