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Sacrococcygeal teratomas in infants
Sacrococcygeal
Teratomas
in Infants
JULIUS L. LEVY, JR., M.D. AND LAWRENCE H. LINDER, M.D., New Orleans, Louisiana
From the Tulane Pediatric Surgical Service at the Charity Hospital of Louisiana and the Department of Surgery, T&me Unietersity School of Medicine, New Orleans, Louisiana.
months. A physician had incised and drained the area on two occasions. When the infant was examined at Charity Hospital two months later, there was a huge ulcerated mass with inguinal node and pulmonary metastases. Biopsy showed a highly anaplastic embryonal adenocarcinoma. This child died soon afterward. Radiologic examination of the mass was performed in all cases. Calcification was present in about half. Spina bifida, when present, suggested the likelihood of a tumor of meningeal origin. Follow-up studies showed no late recurrence in any of the patients with benign disease, even in two in whom the lesion was thought to have been incompletely excised.
0ccuRRENcE of teratomas in neonates T HE and infants has been recognized for many centuries. One of the most common locations involved is the area posterior to the lower sacrum and coccyx. The earliest record of a sacrococcygeal teratoma was made on a cuneiform tablet of Babylonian origin circa 2000 B.C. [I]. There have now been about 500 cases reported in the literature. During the sixteen year period ending February 28, 1968, twelve cases of sacrococcygeal teratoma were studied by the Tulane Pediatric Surgery Service at Charity Hospital in New Orleans. In addition, another case from our private practice is included. All patients have been followed up until the present time. A brief discussion of the problems involved forms the basis for this report.
COMMENTS
It is believed that sacrococcygeal teratomas result from an abortive attempt toward the formation of conjoined twins [3]. This seems likely since the sacrococcygeal area is a common site of bonding in conjoined twins. In one patient (case 6) the teratomatous mass represented 30 per cent of the infant’s total weight and was referred to by the attending pediatrician as a “twin.” In further support of this theory is the fact that teratomas are known to arise from primordial cells. One of the greatest concentrations of primordial cells in a neonate occurs at the “primitive knot” near the coccyx. This is the vestigial “tail” of the human specie. These tumors may be present in many different fashions. They may be well encapsulated or ulcerative and solid or cystic. They may grow quite slowly initially and then burst forth as wildly malignant lesions. They may be marblesized or huge. Although none was seen in our series, tumors which dissect anterior to the sacrum and present in the retroperitoneal area have been described. Pathologically, the size of the lesion seems to bear no relationship to the cell type and to whether the tumor is benign or malignant. Two or three germ cell layers may be present.
CLINICAL DATA A summary of the age incidence, sex incidence, and over-all results can be found in Table I. Ten of thirteen cases (76 per cent) occurred in females. The predominance of females with this condition is common and has been previously described [2]. In all but two cases the mass was present at birth; in these two it was first noted at the age of eleven months. Both were malignant and terminated fatally. The delay prior to surgery varied greatly and in almost every case was due to an original mistaken diagnosis of meningocele or meningomyelocele. In one instance (case 9) the tumor was ruptured at birth, and emergency surgery was necessary to control bleeding. There were three deaths in the series. The only nonmalignant fatality occurred in a 2,090 gm. infant (case 6) who had a 620 gm. tumor. She died two days postoperatively of pneumonitis following vomiting and aspiration after the administration of anesthesia. Another fatality occurred in a child (case 1) whose mass first appeared as a cystic growth at age eleven 822
The American Journal of Surgery
Sacrococcygeal TABLE I PATIENTS WITH SACROCOCCYGEAL TERATOMASTREATED ON THE TULANE PEDIATRIC SURGICAL SERVICE AT CHARITY
1
3
4 5 6 i 8 Q 10 I1 1” 13*
Onset
Nature of the Tumor
Result
11 *no. Birth Birth Birth Birth Birth 11 mo. Birth Birth Birth Birth Birth Birth
Malignant Benign Benign Benign Benign Benign Malignant Benign Benign Benign Benign Benign Benign
Died Living Living Living Living Died Died Living Living Living Living Living Living
Age of
Case No.
‘I
HOSPITAL
F F M F F F F F F F M M F
* Sara Mayo
Hospital.
It seems quite important that operation be performed as soon as possible after the mass appears. Undoubtedly these tumors begin as benign lesions in many cases and undergo malignant change with time. One of the chief causes of delay seems to be confusion between these tumors and meningoceles or meningomyeloceles. The latter two can be ruled out if the tumor contains only solid matter. Neurologic defects, if present, suggest a tumor of the meninges. Also a teratoma is usually located lower than a meningocele. The operative procedure has been well de-
Vol. 116, December 1968
St”3
Teratomas
scribed by others [21 and will not be repeated. Complete ablation of the tumor is necessary. To achieve this end a portion of skin over the tumor together with the entire coccyx must be removed. A small rubber drain and a compression dressing are used to prevent formation of hematoma. Either local or general anesthesia may be used. Convalescence is usually uneventful and requires a minimal amount of nursing care. SUMMARY
Sacrococcygeal teratomas are uncommon tumors which occur in neonates and infants. They are most commonly benign and occur most frequently in females. Ablative surgery soon after diagnosis is imperative and quite well tolerated. Thirteen cases are presented, all but two of which were in neonates. Only one postoperative death occurred, and this was in a small premature infant. No late recurrences of benign tumors were seen. Ten of the thirteen are living and well and presumably cured. This seems to justify an optimistic outlook for patients with this condition. REFERENCES
1. BALLANTYNE, J. W. The teratologia records of Chaldea. Teratologia, 1: 127, 1894. 2. GROSS, R. E., CLATWORTHY, H. W., JR., and MEEKER, 1. ,4., JR. Sacrococcygeal teratomas in infants and children, Surg. Gynec. Gp Obst., 92: 341, 1951. 3. EDMONDS,H. W. and HAWKINS, J. W. The relationship of twins, teratomas, and ovarian dermoids. Cancer Res., 1: 896, 1941.
Teratomas
in Infants
JULIUS L. LEVY, JR., M.D. AND LAWRENCE H. LINDER, M.D., New Orleans, Louisiana
From the Tulane Pediatric Surgical Service at the Charity Hospital of Louisiana and the Department of Surgery, T&me Unietersity School of Medicine, New Orleans, Louisiana.
months. A physician had incised and drained the area on two occasions. When the infant was examined at Charity Hospital two months later, there was a huge ulcerated mass with inguinal node and pulmonary metastases. Biopsy showed a highly anaplastic embryonal adenocarcinoma. This child died soon afterward. Radiologic examination of the mass was performed in all cases. Calcification was present in about half. Spina bifida, when present, suggested the likelihood of a tumor of meningeal origin. Follow-up studies showed no late recurrence in any of the patients with benign disease, even in two in whom the lesion was thought to have been incompletely excised.
0ccuRRENcE of teratomas in neonates T HE and infants has been recognized for many centuries. One of the most common locations involved is the area posterior to the lower sacrum and coccyx. The earliest record of a sacrococcygeal teratoma was made on a cuneiform tablet of Babylonian origin circa 2000 B.C. [I]. There have now been about 500 cases reported in the literature. During the sixteen year period ending February 28, 1968, twelve cases of sacrococcygeal teratoma were studied by the Tulane Pediatric Surgery Service at Charity Hospital in New Orleans. In addition, another case from our private practice is included. All patients have been followed up until the present time. A brief discussion of the problems involved forms the basis for this report.
COMMENTS
It is believed that sacrococcygeal teratomas result from an abortive attempt toward the formation of conjoined twins [3]. This seems likely since the sacrococcygeal area is a common site of bonding in conjoined twins. In one patient (case 6) the teratomatous mass represented 30 per cent of the infant’s total weight and was referred to by the attending pediatrician as a “twin.” In further support of this theory is the fact that teratomas are known to arise from primordial cells. One of the greatest concentrations of primordial cells in a neonate occurs at the “primitive knot” near the coccyx. This is the vestigial “tail” of the human specie. These tumors may be present in many different fashions. They may be well encapsulated or ulcerative and solid or cystic. They may grow quite slowly initially and then burst forth as wildly malignant lesions. They may be marblesized or huge. Although none was seen in our series, tumors which dissect anterior to the sacrum and present in the retroperitoneal area have been described. Pathologically, the size of the lesion seems to bear no relationship to the cell type and to whether the tumor is benign or malignant. Two or three germ cell layers may be present.
CLINICAL DATA A summary of the age incidence, sex incidence, and over-all results can be found in Table I. Ten of thirteen cases (76 per cent) occurred in females. The predominance of females with this condition is common and has been previously described [2]. In all but two cases the mass was present at birth; in these two it was first noted at the age of eleven months. Both were malignant and terminated fatally. The delay prior to surgery varied greatly and in almost every case was due to an original mistaken diagnosis of meningocele or meningomyelocele. In one instance (case 9) the tumor was ruptured at birth, and emergency surgery was necessary to control bleeding. There were three deaths in the series. The only nonmalignant fatality occurred in a 2,090 gm. infant (case 6) who had a 620 gm. tumor. She died two days postoperatively of pneumonitis following vomiting and aspiration after the administration of anesthesia. Another fatality occurred in a child (case 1) whose mass first appeared as a cystic growth at age eleven 822
The American Journal of Surgery
Sacrococcygeal TABLE I PATIENTS WITH SACROCOCCYGEAL TERATOMASTREATED ON THE TULANE PEDIATRIC SURGICAL SERVICE AT CHARITY
1
3
4 5 6 i 8 Q 10 I1 1” 13*
Onset
Nature of the Tumor
Result
11 *no. Birth Birth Birth Birth Birth 11 mo. Birth Birth Birth Birth Birth Birth
Malignant Benign Benign Benign Benign Benign Malignant Benign Benign Benign Benign Benign Benign
Died Living Living Living Living Died Died Living Living Living Living Living Living
Age of
Case No.
‘I
HOSPITAL
F F M F F F F F F F M M F
* Sara Mayo
Hospital.
It seems quite important that operation be performed as soon as possible after the mass appears. Undoubtedly these tumors begin as benign lesions in many cases and undergo malignant change with time. One of the chief causes of delay seems to be confusion between these tumors and meningoceles or meningomyeloceles. The latter two can be ruled out if the tumor contains only solid matter. Neurologic defects, if present, suggest a tumor of the meninges. Also a teratoma is usually located lower than a meningocele. The operative procedure has been well de-
Vol. 116, December 1968
St”3
Teratomas
scribed by others [21 and will not be repeated. Complete ablation of the tumor is necessary. To achieve this end a portion of skin over the tumor together with the entire coccyx must be removed. A small rubber drain and a compression dressing are used to prevent formation of hematoma. Either local or general anesthesia may be used. Convalescence is usually uneventful and requires a minimal amount of nursing care. SUMMARY
Sacrococcygeal teratomas are uncommon tumors which occur in neonates and infants. They are most commonly benign and occur most frequently in females. Ablative surgery soon after diagnosis is imperative and quite well tolerated. Thirteen cases are presented, all but two of which were in neonates. Only one postoperative death occurred, and this was in a small premature infant. No late recurrences of benign tumors were seen. Ten of the thirteen are living and well and presumably cured. This seems to justify an optimistic outlook for patients with this condition. REFERENCES
1. BALLANTYNE, J. W. The teratologia records of Chaldea. Teratologia, 1: 127, 1894. 2. GROSS, R. E., CLATWORTHY, H. W., JR., and MEEKER, 1. ,4., JR. Sacrococcygeal teratomas in infants and children, Surg. Gynec. Gp Obst., 92: 341, 1951. 3. EDMONDS,H. W. and HAWKINS, J. W. The relationship of twins, teratomas, and ovarian dermoids. Cancer Res., 1: 896, 1941.