The Surgical Management of SacrococcygealTeratomas with Intrapelvic Extension

W. HARDY HENDREN, M.D., BRUCE M. HENDERSON, M.B., CH.B.

From the Children's Service and the General Surgical Services of the MassachusettsGeneral Hospital, and The Department of Surgery of the Harvard Medical

School at the Massachusetts General Hospital, Boston, Massachusetts

THE most common site of teratoma is inthe sacrococcygeal region, arising fromcells of the primitive streak in the caudalend of the early embryo. The tumors oc-cur three times more often in females thanin males. The majority are evident at birth,presenting as an external mass whichvaries in size from a small tumor to one aslarge as the rest of the patient. In a recentreport of 32 of these neoplasms, 38 percent were histologically malignant4 whichunderscores the importance of removingthe tumors intact and completely when-ever possible to prevent recurrence andpossible death. In general, the older thepatient at the time of discovery of the tu-mor, the higher the likelihood that themass is malignant. Nevertheless, preopera-tively all of these tumors should be con-sidered malignant regardless of the age ofthe patient, lest proper surgical excision becompromised by assuming it to be benign.The majority of sacrococcygeal tera-

tomas present externally as a sizable massarising from the perineum in the newborn.A typical example is shown in Figure 1.Surgical excision is a well standardized op-eration, as described by Goss et al.5 Theinfant is positioned face down and themass is excised through an inverted V in-cision, transecting the lower sacrum, sepa-rating the mass from the rectum and glu-teal muscles, and removing it intact withthe attached coccyx.

Occasionally these tumors are locatedentirely within the pelvis, with no mass

Sipbmitted for publication April 28, 1969.

77

palpable in the buttocks. One such patientpresenting with urinary obstruction hasbeen reported previously from the Massa-chusetts General Hospital.3

In certain sacrococcygeal teratomas, how-ever, the tumor does not grow entirely out-ward, but may extend up into the pelvisand lower abdomen as well as downwardtoward the perineum. These instances re-quire a different surgical approach formanagement. This paper reports experi-ence with four such patients seen duringthe past 8 years.

Case ReportsThe patients in Cases 1, 2, and 3 were

treated at the Massachusetts General Hos-pital. Patient 4 was operated upon at theQueen Elizabeth Central Hospital, Blan-tyre, Malawi, Africa, during a visit to thatinstitution sponsored by the U. S. Depart-ment of State (WHH). Patient 1 was op-erated upon initially elsewhere. Patients 2,3, 4 were treated from the outset.

Case 1. M. C., MGH Unit #121 51 33. A 2Y-year-old boy was admitted to the MassachusettsGeneral Hospital 12/5/62 for consideration offurther operation for recurred sacrococcygeal tera-toma following four attempted excisions elsewhere.At 9 months of age a lower abdominal mass wasdiscovered on routine examination which by rectalexamination seemed to arise from the coccygealregion. It was thought to be a benign enteric cystand excision was performed from above withoutremoving the coccyx. The surgeon felt that thelesion had been entirely removed. Microscopic ex-amination diagnosis was "benign enteric cyst."The mass recurred, and at 16 months of age therewas again a palpable mass in the lower abdomen

HENDREN AND HENDERSON Annals of SurgeryJanuary 1970

Sigmoid colostomy and suprapubic cystostomywere performed. At 27 months of age a secondperineal operation was performed by another sur-

geon, incompletely removing the tumor. At 28months of age a third surgeon performed an ab-dominal-perineal resection of the mass, removingthe rectum which seemed adherent to the mass,

but not the coccyx. The pathologic report was

"malignant teratoma, primarily embryonal carci-noma." Shortly thereafter the child was referred tothe Massachusetts General Hospital. On 1/5/63the abdomen was reopened, to assess the pelvicmass and determine whether it would be feasibleto attempt another abdominal-perineal excision ofthe lower sacrum and coccyx. Unfortunately meta-static disease was found in the peritoneum, peri-aortic lymph nodes, and the liver. Biopsy of a

lymph node showed "metastatic embryonal carci-noma." No further surgical excision Mwas attempted.The child was discharged 1/14/63 and died else-where 3/1/63.

FIG. 1. Large sacrococcygeal teratoma of themost common type in an infant, with no intra-pelvic extension, which can be removed com-pletely from below. a) Position on the operatingtable, face down, for excision. b) After transec-tion of the lower sacrum, and separation of thetumor from the underlying gluteus muscles andrectum. c) Closure after excision of appropriateamount of redundant skin.

and on rectal examination. A second surgical ap-

proach was made, through the perineum, andagain the surgeon felt the mass to be completelyexcised, although the coccyx was not removed.Again, the pathologic diagnosis was "benign en-

teric cyst." At 26 months of age the mass re-

curred and obstructed both bladder and rectum.

FIG. 2. Case 2. A small sacrococcygeal tera-toma presenting as a cystic mass in a newborn.a) Outline of mass palpable in the left buttocks,referred as a possible "lipoma." It could be feltextending up into the lower abdomen on rectalexamination and its removal entailed an abdomi-nal followed by sacral exposure and resection. b)Delivery of the bilobed mass intact from below.

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SURGICAL MANAGEMENT OF SACROCOCCYGEAL TERATOMAS

i.&;YYir SN. ...F% I M...X

FIG. 3. Low power photomicrograph of specimen from Case 2, showing benign elements whichincluded respiratory-like epithelium, cartilage, and other connective tissue elements.

Case 2. R. L., MGH Unit #123 29 96. A 1-week-old boy was referred with a cystic mass

presenting in the left buttocks (Fig. 2). On rectalexamination an extension of the mass was palpableabove the sacral promentory, and could be felt inthe lower abdomen just above the pubic symphy-sis. Intravenous pyelogram showed that the ureterswere not obstructed, but the bladder was dis-placed upward and anteriorly. On 4/24/63 opera-

tion was performed. The upper aspect of the tu-mor was approached first through a transverselower abdominal laparotomy. The mass was dis-sected from the ureters, bladder, and rectum, andits arterial blood supply, the middle sacral ves-

sels were ligated. The abdomen was then closedand the child was repositioned face downward.The perineal excision was performed through an

inverted V incision over the lower sacrum andbuttocks. The sacrum was transected 1.5 cm. fromthe tip of the coccyx, leaving it attached to thespecimen, and the entire mass was deliveredeasily from below (Fig. 2B). Microscopic exami-nation disclosed a benign tumor containing pan-

creas, respiratory-like epithelium, and neural ele-

ments (Fig. 3). On follow-up examination therehas been no recurrence, and the child has hadnormal urinary and bowel function.

Case 3. S. F., MGH Unit #150 46 49. A 3-year-old girl was referred to the MassachusettsGeneral Hospital for evaluation of a large mass,

11 cm. in diameter, arising in the sacrococcygealregion. The mass extended into both buttocks andthe skin was stretched tightly over it, suggesting a

highly malignant tumor with invasion of overlyingskin. The patient had complained of pain in thisregion for 2 months and had recently begun tolimp. Her anal sphincter was atonic. For 2 weeksshe had been partially incontinent of urine andstool, although previously trained. On rectal ex-

amination the mass could be felt to extend up-

ward to the level of the sacral promentory. Intra-venous pyelogram showed displacement of thebladder forward and upward, but renal functionwas good. Operation was performed 5/15/68.First, ureteral catheters were inserted into bothureters to facilitate identification during the pelvicdissection. The abdominal component of the op-

eration was performed first, mobilizing the ureters,

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HENDREN AND HENDERSON

ArIF ,.&as,,IFIG. 4. High power photomicrograph of specimen from Case 3, showing extremely differentiated

embryonal carcinoma.

bladder, and rectum, and ligating the large bloodvessels which entered the upper aspect of the tu-mor from the region of the sacral promentory.There was no evidence of metastatic disease inthe periaortic lymph nodes, liver, etc. After clo-sure of the abdominal incision the child was

turned face down for the perineal part of thecombined procedure. A large area of involvedskin overlying the tumor was sacrificed, leavingit attached to the mass which was then mobilizedlaterally on both sides, and separated from thegluteus muscles. A finger within the pelvis pal-pated an attachment to the lower sacrum, whichwas transected 4 cm. above the coccyx, leavingthe coccyx and lower sacrum attached to the tu-mor. It was then possible to deliver the entiremass intact. After removal of the specimen an en-

larged lymph node on the right lateral pelvic wall,near the sciatic notch, was removed. Histologicexamination of the specimen and this lymph nodeshowed "embryonal carcinoma." A representativesection is shown in Figure 4. There was no grosstumor extending into the spinal canal, however,some fat in the medullary canal was curetted and

contained malignant cells on microscopic exami-nation. Although the specimen had been removedintact, because of lymph node and medullarycanal involvement, the patient was given chemo-therapy for 5 days, including Actinomycin-D,Methotrexate, and Cytoxan. These agents causeda temporary reduction in leukocytes and causedher hair to fall out, but she was discharged insatisfactory condition 6/16/68, one month post-

operatively. In September 1968 she was given an

additional course of Actinomycin-D, in combina-tion with 4,000 R of radiation to the lower sacrum

and lower spinal region. No recurrence was evi-dent at this time but it was considered justifiedto give this additional treatment in view of thehighly malignant nature of the neoplasm, positivelymph node, and microscopic extension of tumor

into the medullary canal. On most recent exami-nation, 4/1/69, 11 months postoperatively, therewas no evidence of recurrence. She remains in-continent of urine and stool, and if she survivesfor a long-term, urinary diversion will probablybe required,

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SURGICAL MANAGEMENT OF SACROCOCCYGEAL TERATOMAS

FIG. 5. Case 4 show-ing large sacrococcygealteratoma with intrapelvicand lower abdominal ex-tension. a) Preoperativeview. Note abdominalmass. b) View of upperpart of mass dissectedfrom pelvic viscera atpreliminary laparotomy.Arrow points to mass. c)Removed specimen, show-ing its dumbbell shapewith attached sacrococ-cygeal segments.

Case 4. B. L. A 2-week-old girl was admittedto the Queen Elizabeth Central Hospital, Blantyre,Malawi, with a large tumor protruding from thesacrococcygeal region (Fig. 5). Part of the mass

was solid and part cystic. Fluid had been leakingfrom the mass for several days. On rectal exami-nation the mass could be felt upward behind therectum, and its upper border could be palpatedbelow the umbilicus on examination of the abdo-men. It was believed that this was a typical cysticand solid sacrococcygeal teratoma extending bothupward and downward. On 5/23/68 through a

transverse lower abdominal incision the upperpart of the tumor was mobilized, separating itfrom the ureters, the rectum, uterus, and vagina(Fig. 5B). The middle sacral arterial blood sup-

ply was ligated. Because the mass was wedgedtightly in the pelvis, making mobilization diffi-cult, and because it was principally cystic, it was

aspirated to reduce its size. This facilitated thepelvic dissection. Care was exercised not to spillany fluid; the needle hole through which the fluidhad been aspirated was tightly sutured. After clo-sure of the laparotomy incision the child was

FIG. 6. Steps in theoperative removal ofsaccrococcygeal tera-toma growing upwardinto the pelvis and lowerabdomen, as well asdownward in the moreusual fashion. la) Initialincision. b) Mobilizationof mass from pelvic vis-cera. \ YI KL

turned face downward, and the lower dissectionwas performed through an inverted V incision,transecting the lower sacrum, leaving it attachedto the specimen, and removing the mass intact(Fig. 5C). Microscopic examination by the De-partment of Pathology at the Royal College ofSurgeons, London, England, revealed "benignteratoma." The infant recovered and was dis-charged 2 weeks later. Further follow-up has notbeen possible, but it is unlikely that there will bedifficulty because there was no evidence of ma-

lignancy in this specimen.

Operative TechnicFigure 6 shows the operative steps in re-

moval of these teratomas in Cases 2, 3, and4. Laparotomy affords access to separatethe tumor from pelvic viscera which couldbe injured if this were attempted from be-low. For the second stage the child is po-sitioned face downward, a V shaped in-cision is made over the upper buttocks, the

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82 HENDREN AND HENDERSON

lower sacrum is divided, leaving the coccyxattached to the specimen, and the previ-ously mobilized intrapelvic mass is deliv-ered. The lower part of the mass is thenseparated from the rectum, the gluteusmuscles, and finally the skin is incised pos-teriorly, just behind the anus at an appro-priate point, completing removal of thespecimen intact. The wound is then closedtransversely in layers, first bringing thelevator ani muscles upward. A drain isplaced in the presacral space, and any ex-cess skin is trimmed.

Annals of SurgeryJanuary 1970

FIG. 6-2) Prone po-sition, with cutdown inarm, and inverted V in-cision over lower sacrumand buttocks. b) Tran-section of lower sacrumand further mobilizationof pelvic mass up topoint of previous dissec-tion from above.

Discussion

The surgical approach to sacrococcygealteratomas must be individualized to thesite of the mass. Rectal examination shouldassess whether there is extension upwardinto the pelvis. If the tumor does not ex-tend upward, a posterior approach in theprone position is adequate. This is inade-quate, however, if the tumor extends intothe pelvis and lower abdomen. In all casesthe coccyx and lower sacrum should beexcised, for the tumors arise from this bonyorigin. Case 1 demonstrates this point.

FIG. 6 -3) Deliveryof pelvic component ofmass, and separation oflower part of mass fromthe rectum anteriorly, thegluteus muscles, and thelevator ani muscles. Thisdissection is facilitated ifa vaseline pack is withinthe rectum to distend it.

SURGICAL MANAGEMENT OF SACROCOCCYGEAL TERATOMAS

Priebe" reported a case of sacrococcy-

geal teratoma with extension upward inwhich the abdominal mass was extremelylarge. The upper dissection was done first;the tumor was too large to deliver throughthe pelvis, so the mass was transected, andits lower half was excised from below.Arnheim reported staged excision in

one case, removing first the inferior partof the mass, transecting its pelvic compo-

nent, and 3 weeks later removing the ab-dominal part at laparotomy.Smith et al.8 demonstrated angiographi-

cally that these teratomas derive bloodsupply almost entirely from the middlesacral vessels. They recommended earlyligation of these vessels in removal of suchtumors. With pelvic extension they favoredlaparotomy first with mobilization of theupper aspect of the mass and division ofits blood supply, followed by an immedi-ate removal of the rest of the tumor frombelow.Waldhausen et al.9 reported 23 cases of

sacrococcygeal teratoma, of which five hadposterior masses with intrapelvic exten-sions. They performed laparotomy first fol-lowed by posterior excision.

In 40 cases reported by Gross et al.there were two with pelvic extensions of

Sutues in Pr

FiG. 6-5) Final clo-sure. a) Elevating theperineum to the lowersacrum and gluteus mus- -

cles, leaving a drain inthe presacral space. b) &c) Final stages of clo-sure.

Buttocksmass

Limit of3nterior incision , Incise posterior skin flop

on the left superior to anus

FIG. 6-4) Final separation of tumor by incis-ing skin at an appropriate point adjacent to theanus.

tumor. In each the specimen was dividedand its upper part was excised at a secondoperation from above.Brown and Brown 2 reported a sacrococ-

cygeal teratoma with a small lower portionand a large intrapelvic mass for which theydid the lower dissection first, and thenturned the child for an immediate comple-tion of the dissection from above.

Ravitch and Smith 7 mentioned that a

combined abdominal and perineal ap-

proach might be required if the tumorshould be too large or too extensive for

Deep sutures closedbringing onus up toi normol position

k. Closure ofterIt, excising excess skinq.;

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HENDREN AND HENDERSON Annals of Surgery84

simple posterior excision, but they did notreport such an instance.

Willox and MacKenzie10 reported a pa-tient in whom an attempt was made to re-move the tumor entirely from below, with-out success, and with great blood loss.Incomplete removal was carried out. Onemonth later the abdominal component wasperformed, and the tumor was found to bemalignant. The patient eventually died.Although there are some reports of in-

trapelvic extensions removed successfullyin two separate operations, it is desirableto remove the intact mass with the coccyxin one procedure.We have not encountered a tumor in

which transection was required for re-moval. Conceivably a dumbbell shapedmass with a small isthmus in the pelvismight require division, but this must berare. If a sacrococcygeal teratoma wereconfined to the pelvis and lower abdomen,without downward extension, it might bepreferable to perform the lower dissectionfirst, with transection of the sacrum, andseparation of the tumor from the gluteusmuscles and rectum, followed by the lapa-rotomy and extraction of the mass fromabove. This has the disadvantage, how-ever, of not securing the blood supplywhich comes from the middle sacral ves-sels early in the operation. Two alterna-tives in this situation would be: 1) a simul-taneous abdominal-perineal resection inthe supine position as used by Priebe,6 butthis is a difficult position in which to tran-sect the sacrum, or 2) mobilization fromabove, temporary closure, turning pronefor the dissection below, and then turningagain to the laparotomy to pull the speci-men upward through the pelvis.Any tumor the upper part of which is

not too large to deliver through the pelvicoutlet is preferably dissected from aboveat laparotomy with ligation of the middlesacral vessels, closure of the abdomen andthen the perineal operation. This approach

permits examination for possible spread tothe liver and lymph nodes early in the op-eration, which is an additional advantage.

Summary

Four cases of teratomas arising in thesacrococcygeal region and growing upwardand downward are reported. Two were be-nign and two malignant. These patientsare best managed by a one stage abdomi-nal-sacral operation, usually first the lapa-rotomy in the supine position, followed bya perineal operation in the prone positionin which the specimen is pulled throughintact together with its origin from thecoccyx and lower sacrum. Patients 2, 3, and4 managed in this way are presently well,although Case 4 is recently postoperative.Patient 1, not managed in this manner,succumbed, and might have been curableif this approach had been employed ini-tially.

References1. Arnheim, E. E.: Combined Pelvic and Retro-

peritoneal Teratomas in Infancy and Child-hood. Pediatrics, 10:198, 1952.

2. Brown, R. K. and Brown, E. C.: Sacrococ-cygeal Teratoma with Urinary Retention.Arch. Surg., 60:535, 1950.

3. Case Records of the Massachusetts GeneralHospital. New Eng. J. Med., 265:388, 1961.

4. Conklin, J. and Abell, M. R.: Germ Cell Neo-plasms of Sacrococcygeal Region. Cancer,20:2105, 1967.

5. Gross, R. E., Clatworthy, H. W., Jr. andMeeker, I. A., Jr.: Sacrococcygeal Tera-tomas in Infants and Children: A Report of40 Cases. Surg. Gynec. Obstet., 92:341,1951.

6. Priebe, C. J., Jr.: Sacrococcygeal Teratoma ina Newborn Girl with Massive Intra-abdomi-nal Extension: Combined AbdominosacralApproach. Surgery, 60:1090, 1966.

7. Ravitch, M. M. and Smith, E. I.: Sacrococ-cygeal Teratoma in Infants and Children.Surgery, 30:733, 1950.

8. Smith, B., Passaro, E. and Clatworthy, H. W.,Jr.: The Vascular Anatomy of Sacrococcy-geal Teratomas: Its Significance in SurgicalManagement. Surgery, 49:534, 1961.

9. Waldhausen, J. A., Kilman, J. W., Vellios, F.and Battersby, J. S.: Sacrococcygeal Tera-toma. Surgery, 54:933, 1963.

10. Willox, G. L. and MacKenzie, W. C.: Sacro-coccygeal Teratomas. Arch. Surg., 83:27,1961.