RETROPERITONEAL
MAJ. M. SHELDON
TERATOMA”
POLSKY,
(MC) USA
MAJ. GARY D. SHACKELFORD, COL. CARL H. WEBER,
(MC) USAF
JR., (MC) USAF
COL. THOMAS P. BALL, JR., (MC) USAF From the Urology Service, Department of Surgery, and Department of Radiology, Wilford Hall USAF Medical Center, Lackland AFB, Texas
ABSTRACT - Retroperitoneal teratomu is the third most common primary retroperitoneal neoplasm of childhood ranking behind neuroblastoma and nephroblastoma. Ten per cent of these tumors are malignant. Two cases of benign retroperitoneal teratoma are reported, and the first description of the angiographic appearance of such a tumor is presented.
A teratoma is a true tumor or neoplasm composed of multiple tissues foreign to the part in which it arises.’ It may be benign or malignant; it can be further classified as cystic or solid, and also as monodermal, bidermal, or tridermal according to the number of germ layers present. The retroperitoneal space has been shown to rank fourth in frequency of origin of teratoma behind ovary, testis, and anterior mediastinum.2 In infancy and childhood only neuroblastoma and nephroblastoma are more frequently encountered retroperitoneal tumors.3 We herein report 2 new cases of retroperitoneal teratoma and review the literature to date. Case Reports Case 1 A fifteen-year-old Caucasian female was seen in consultation because of hematuria. She had been admitted the previous day for a closed head injury incurred in an automobile accident. She was responsive only to deep pain and had an otherwise normal physical examination. A cystogram showed an intact bladder, and excretory urogram demonstrated a left suprarenal soft tis*The views expressed herein are those of the authors and do not constitute a statement of official Army or Air Force policy.
618
sue mass, with the left kidney displaced inferiorly. The upper pole appeared indented by the mass which contained two calcific densities. Cystoscopic examination of the bladder showed clear efflux bilaterally, with evidence of hemorrhagic cystitis secondary to the indwelling Foley catheter. Because of the patient’s neurologic status, a transfemoral carotid arteriogram was obtained which demonstrated no gross cerebral lesions or displacement. Aortography confirmed the presence of a 10 by 10 cm. avascular suprarenal mass, which displaced the spleen superiorly and the left kidney inferiorly (Fig. 1). The patient was treated conservatively and showed gradual neurologic improvement. She was discharged four weeks later able to carry on her normal activities. She was electively readmitted to the urology service the following month. Adrenal function as determined by serum electrolytes, urinary vanilmandelic acid, 17-OH-corticosteroids, and 17ketosteroids were all within normal limits. A glucagon stimulation test failed to elevate the blood pressure significantly. The patient was explored through an eleventh interspace incision, and a large suprarenal, multiloculated, cystic, and solid mass was completely resected. No obvious adrenal tissue was identified. She had a benign postoperative course
UROLOGY
/
DECEMBER
1976
/
VOLUME
VIII, NUMBER 6
FIGURE 1. (A) Aortogram demonstrating suprarenal mass displacing adjacent organs. (B) Left renal arteriogram demonstrating extrarenal mass; faint calcijications resembling teeth noted between eleventh and twelfth ribs.
FIGURE 2. (A) Gross specimen demonstrating bronchuslike structure in solid portion of mass. (B) Microscopic section demonstrating two epithelial types and cartilage.
and left the hospital on the tenth postsurgical day. On gross dissection of the pathologic specimen, the cysts were noted to contain thick, brown, sebaceous-like material within which hair was present. One area of the mass, however, was solid and on sectioning revealed fat and a bronchus-like structure (Fig. ZA). This was confirmed by microscopic sections and led to the diagnosis of benign retroperitoneal teratoma (Fig. 2B). Case 2 A two-year-old black female was admitted to the pediatric service for evaluation of a mass in
UROLOGY
/ DECEMBER1976
/ VOLUME
VIII,NUMBER6
the right upper abdominal quadrant. Growth and development had been normal, and the patient was asymptomatic. Physical examination disclosed a firm, smooth, right upper quadrant abdominal mass which extended 8 cm. below the right costal margin. The clinical impression was that of an enlarged liver. Laboratory studies including liver function tests were within normal limits. A liver-spleen scan showed superior displacement of the liver by a large mass which contained no activity and was felt to be extrahepatic in origin. Plain roentgenograms of the abdomen revealed a large right abdominal mass containing fat, bone, and probable teeth. Intravenous pyelogram disclosed
619
TABLE Author
Age
Sex
Malik, Malik, and Diesh5
6 yr.
Adams and FreundG
Retroperitoneal
I.
teratomas
reported
since 1967
Signs and Symptoms
X-ray Studies*
Treatment
Pathology
F
Weight
IVP: mass, calcification; nonfunction kidney
Excision; nephrectomy
Benign
17 yr.
M
UGI: retroperitoneal mass
Biopsy mass
Malignant
Died
Snarr and MacPherson’
2 mo.
M
Benign
Alive (10 yr.)
18 yr.
M
IVP; mass, calcification, and incomplete visualization upper tracts IVP: ureteral displacement
Preoperative irradiation; excision
Mamakosa
Alive (5 yr.)
8 mo.
F
Biopsy and secondary excision Excision
Benign
Masuda, Ueda, and Minamis Masuda, Ueda, and Minami8 Keramidas and Voyatzis’a
Abdominal, back, and testicular pain, vomiting, weight loss; mass Mass, umbilical and scrotal hernias, and prominent superficial abdominal veins Abdominal cramping, anorexia; abdominal distention Mass
Benign
Alive
3 mo.
F
Mass
Excision
Benign
Alive
2 yr.
M
Intestinal
Biopsy and secondary excision
Benign
Alive (9 mo.)
Banerjee, Sircar, and Gidwani” Salleh12
5 mo.
Fever;
IVP: normal
Incomplete excision
Benign
?
I2 da.
Benign
Alive
1 yr.
KUB: mass and calcification IVP: mass
Excision
Talib et al. I3
None
Benign
Mahour
et al. I4
8 mo.
Abdominal enlargement Loose stools, fever; mass Discovered at autopsy
et al. I4
report
2 mo. 6 mo. 15 yr. 15 yr.
Mass Mass Weight loss; mass Asymptomatic
report
2 yr.
Mahour Mahour Mahour Current
Current
et al. r4 et al. I*
*IVP = intravenous
F
pyelogram;
loss; mass
.
obstruction
mass
Mass
UGI = upper
,
gastrointestinal
620
Excision Excision Excision Excision
Benign Benign Benign Benign
Excision
Benign
Alive
series; KUB = kidney,
inferior displacement of the right kidney, but no intrinsic renal abnormality (Fig. 3). On upper gastrointestinal series the distal stomach and duodenum were displaced anteriorly and to the left. At laparotomy a large retroperitoneal mass was found arising superior to the right kidney. Adjacent organs were displaced, and no right adrenal gland could be identified. The mass was completely excised, and on sectioning was shown to consist of a well-encapsulated, multiloculated, cystic, and solid mass measuring 15 by 12 cm. Microscopic examination disclosed a variety of mature benign tissues representative of all three
?
Died (bronchopneumonia) Died (hydrocephalus) Alive Alive Alive Alive (1 yr.)
Benign
IVP: mass, calcification, renal displacement, and compression Arteriogram: avascular mass IVP: mass, calcification, renal displacement UGI: bowel displacement
Results
ureters,
and urinary
bladder
x-ray film.
germ layers. The pathologic diagnosis nign retroperitoneal teratoma.
was be-
Comment In 1949 Palumbo et ~1.~ reviewed 57 cases of retroperitoneal teratoma from the literature, and added 1 case of their own. Sixty-two per cent were patients in the pediatric age group. Presenting symptoms were vague and frequently secondary to the pressure effects of the tumor growth on neighboring organs, including abdominal or back pain, nausea, vomiting, constipation, and urinary tract symptoms. Abdominal en-
UROLOGY
/
DECEMBER
1976
/
VOLUME
VIII,
NUMBER
6
abdominal mass or enlargement. Findings on intravenous pyelogram when recorded demonstrated, in the majority of cases, a perirenal soft tissue mass, often containing areas of calcification. Urinary tract displacement and obstruction were also noted. Bowel studies appeared to help in localizing the mass to the retroperitoneal space. Case 1 in this report is the first reported case of a retroperitoneal teratoma demonstrated arteriographically, illustrating the avascular appearance with marked displacement of neighboring organs. Arteriography would appear useful in differentiating benign teratoma from malignant tumors such as neuroblastoma, hepatoma, and nephroblastoma which might also demonstrate calcifications in abdominal masses. Urology Service Lackland AFB, Texas (DR. WEBER) References 1. WILLIS, R. A. :
FIGURE 3. Excretory urogram demonstrating placement of right kidney by mass containing cijcation consistent with bone and teeth.
discal-
largement and a palpable mass were the most common physical findings. Ten per cent of the tumors demonstrated malignant changes. Engle, Elkins, and Fletcher2 reviewed another 30 cases in 1968. Fifty-three per cent were fifteen years of age or younger, and symptoms and signs were similar to the Palumbo et ~1.~ series. Ten per cent were malignant, and all of these patients died within a year of diagnosis. In their personal case report an absence of identifiable adrenal tissue at surgical exploration was noted, similar to the findings of our 2 patients. Whether this represents pressure atrophy, tumor replacement, or maldevelopment of the adrenal gland during embryonic growth remains speculative; certainly presentation of normal adrenal glands adjacent to retroperitoneal teratomas has also been reported.3 A summary of an additional 14 cases from the recent literature is reviewed in Table I.5-14 Once again most patients were in the pediatric age group (88 per cent). The presenting symptoms and signs included abdominal, back, and scrotal pain, and weight loss, fever, loose stools, and
UROLOGY
/ DECEMBER1976
/ VOLUMEVIII, NUMBER6
Atlas of Tumor Pathology: Teratomas, Washington, D.C., Armed Forces Institute of Pathology, 1951, sect. III, fast. 9, p, 9. 2. ENGEL, R. M., ELKINS, R. C., and FLETCHER, B. D. : Retroperitoneal teratoma: review of the literature and presentation of an unusual case, Cancer 22: 1068 (1968).
3. ARNHEIM, E. E.:
Retroperitoneal teratomas in infancy and childhood, Pediatrics 8: 309 (1951). 4. PALUMBO, L. T., CROSS, K. R., SMITH, A. N., and BARONAS, A. A.: Primary teratomas of the lateral retroperitoneal spaces, Surgery 26: 149 (1949). 5. MALIK, T. K., MALIK, G. B., and DIESH, G.: Retroperitoneal teratoma with nephroblastic tissue as the main component, Internat. Surg. 47: 246 (1967). 6. ADAMS, G., and FREUND, E.: Ueber einen retroperitonealen dysontogenetischen malignen Tumor, Zentralbl. Chir. 94: 373 (1969). 7. SNARR, J. W., and MACPHERSON, R. 1.: An unusual abdominal mass in an infant - a case for diagnosis, J. Can. Assoc. Radiol. 21:53 (1970). 8. MAMAKOS, M. S. : Retroperitoneal teratoma. Five year survival in adolescence, N.Y. State J. \4ed. 71:
1838(1971). 9. MASUDA, F., UEDA, M., and MINAMI, T.:
Retroperitoneal teratoma in infants, Jap. J. Urol. 63: 175 (1971). 10.KERAMIDAS, D. C., and VOYATZIS, N. G.: Retroperitoneal teratoma, J. Pediatr. Surg. 7: 434 (1972). 11. BANERJEE, A. K., SIRCAR, P. K., and GIDWANI, C. R.: Retroperitoneal teratoma, Indian J. Pediatr. 40: 193 (1973). 12. SALLEH, H. M.: Retroperitoneal teratoma, Med. J. Malaysia 28: 43 (1973). 13.TALIB, V. H., et al. : Benign retroperitoneal teratoma, Indian J. Pediatr. 41:58 (1974). 14. MAHOUR, G. H., WOOLEY, M. >I., TRIVEDI, S. N., and LANDING, B. H.: Teratomas in infancy and childhood: experience with 81 cases, Surgery 76: 309 (1974).
621
MAJ. M. SHELDON
TERATOMA”
POLSKY,
(MC) USA
MAJ. GARY D. SHACKELFORD, COL. CARL H. WEBER,
(MC) USAF
JR., (MC) USAF
COL. THOMAS P. BALL, JR., (MC) USAF From the Urology Service, Department of Surgery, and Department of Radiology, Wilford Hall USAF Medical Center, Lackland AFB, Texas
ABSTRACT - Retroperitoneal teratomu is the third most common primary retroperitoneal neoplasm of childhood ranking behind neuroblastoma and nephroblastoma. Ten per cent of these tumors are malignant. Two cases of benign retroperitoneal teratoma are reported, and the first description of the angiographic appearance of such a tumor is presented.
A teratoma is a true tumor or neoplasm composed of multiple tissues foreign to the part in which it arises.’ It may be benign or malignant; it can be further classified as cystic or solid, and also as monodermal, bidermal, or tridermal according to the number of germ layers present. The retroperitoneal space has been shown to rank fourth in frequency of origin of teratoma behind ovary, testis, and anterior mediastinum.2 In infancy and childhood only neuroblastoma and nephroblastoma are more frequently encountered retroperitoneal tumors.3 We herein report 2 new cases of retroperitoneal teratoma and review the literature to date. Case Reports Case 1 A fifteen-year-old Caucasian female was seen in consultation because of hematuria. She had been admitted the previous day for a closed head injury incurred in an automobile accident. She was responsive only to deep pain and had an otherwise normal physical examination. A cystogram showed an intact bladder, and excretory urogram demonstrated a left suprarenal soft tis*The views expressed herein are those of the authors and do not constitute a statement of official Army or Air Force policy.
618
sue mass, with the left kidney displaced inferiorly. The upper pole appeared indented by the mass which contained two calcific densities. Cystoscopic examination of the bladder showed clear efflux bilaterally, with evidence of hemorrhagic cystitis secondary to the indwelling Foley catheter. Because of the patient’s neurologic status, a transfemoral carotid arteriogram was obtained which demonstrated no gross cerebral lesions or displacement. Aortography confirmed the presence of a 10 by 10 cm. avascular suprarenal mass, which displaced the spleen superiorly and the left kidney inferiorly (Fig. 1). The patient was treated conservatively and showed gradual neurologic improvement. She was discharged four weeks later able to carry on her normal activities. She was electively readmitted to the urology service the following month. Adrenal function as determined by serum electrolytes, urinary vanilmandelic acid, 17-OH-corticosteroids, and 17ketosteroids were all within normal limits. A glucagon stimulation test failed to elevate the blood pressure significantly. The patient was explored through an eleventh interspace incision, and a large suprarenal, multiloculated, cystic, and solid mass was completely resected. No obvious adrenal tissue was identified. She had a benign postoperative course
UROLOGY
/
DECEMBER
1976
/
VOLUME
VIII, NUMBER 6
FIGURE 1. (A) Aortogram demonstrating suprarenal mass displacing adjacent organs. (B) Left renal arteriogram demonstrating extrarenal mass; faint calcijications resembling teeth noted between eleventh and twelfth ribs.
FIGURE 2. (A) Gross specimen demonstrating bronchuslike structure in solid portion of mass. (B) Microscopic section demonstrating two epithelial types and cartilage.
and left the hospital on the tenth postsurgical day. On gross dissection of the pathologic specimen, the cysts were noted to contain thick, brown, sebaceous-like material within which hair was present. One area of the mass, however, was solid and on sectioning revealed fat and a bronchus-like structure (Fig. ZA). This was confirmed by microscopic sections and led to the diagnosis of benign retroperitoneal teratoma (Fig. 2B). Case 2 A two-year-old black female was admitted to the pediatric service for evaluation of a mass in
UROLOGY
/ DECEMBER1976
/ VOLUME
VIII,NUMBER6
the right upper abdominal quadrant. Growth and development had been normal, and the patient was asymptomatic. Physical examination disclosed a firm, smooth, right upper quadrant abdominal mass which extended 8 cm. below the right costal margin. The clinical impression was that of an enlarged liver. Laboratory studies including liver function tests were within normal limits. A liver-spleen scan showed superior displacement of the liver by a large mass which contained no activity and was felt to be extrahepatic in origin. Plain roentgenograms of the abdomen revealed a large right abdominal mass containing fat, bone, and probable teeth. Intravenous pyelogram disclosed
619
TABLE Author
Age
Sex
Malik, Malik, and Diesh5
6 yr.
Adams and FreundG
Retroperitoneal
I.
teratomas
reported
since 1967
Signs and Symptoms
X-ray Studies*
Treatment
Pathology
F
Weight
IVP: mass, calcification; nonfunction kidney
Excision; nephrectomy
Benign
17 yr.
M
UGI: retroperitoneal mass
Biopsy mass
Malignant
Died
Snarr and MacPherson’
2 mo.
M
Benign
Alive (10 yr.)
18 yr.
M
IVP; mass, calcification, and incomplete visualization upper tracts IVP: ureteral displacement
Preoperative irradiation; excision
Mamakosa
Alive (5 yr.)
8 mo.
F
Biopsy and secondary excision Excision
Benign
Masuda, Ueda, and Minamis Masuda, Ueda, and Minami8 Keramidas and Voyatzis’a
Abdominal, back, and testicular pain, vomiting, weight loss; mass Mass, umbilical and scrotal hernias, and prominent superficial abdominal veins Abdominal cramping, anorexia; abdominal distention Mass
Benign
Alive
3 mo.
F
Mass
Excision
Benign
Alive
2 yr.
M
Intestinal
Biopsy and secondary excision
Benign
Alive (9 mo.)
Banerjee, Sircar, and Gidwani” Salleh12
5 mo.
Fever;
IVP: normal
Incomplete excision
Benign
?
I2 da.
Benign
Alive
1 yr.
KUB: mass and calcification IVP: mass
Excision
Talib et al. I3
None
Benign
Mahour
et al. I4
8 mo.
Abdominal enlargement Loose stools, fever; mass Discovered at autopsy
et al. I4
report
2 mo. 6 mo. 15 yr. 15 yr.
Mass Mass Weight loss; mass Asymptomatic
report
2 yr.
Mahour Mahour Mahour Current
Current
et al. r4 et al. I*
*IVP = intravenous
F
pyelogram;
loss; mass
.
obstruction
mass
Mass
UGI = upper
,
gastrointestinal
620
Excision Excision Excision Excision
Benign Benign Benign Benign
Excision
Benign
Alive
series; KUB = kidney,
inferior displacement of the right kidney, but no intrinsic renal abnormality (Fig. 3). On upper gastrointestinal series the distal stomach and duodenum were displaced anteriorly and to the left. At laparotomy a large retroperitoneal mass was found arising superior to the right kidney. Adjacent organs were displaced, and no right adrenal gland could be identified. The mass was completely excised, and on sectioning was shown to consist of a well-encapsulated, multiloculated, cystic, and solid mass measuring 15 by 12 cm. Microscopic examination disclosed a variety of mature benign tissues representative of all three
?
Died (bronchopneumonia) Died (hydrocephalus) Alive Alive Alive Alive (1 yr.)
Benign
IVP: mass, calcification, renal displacement, and compression Arteriogram: avascular mass IVP: mass, calcification, renal displacement UGI: bowel displacement
Results
ureters,
and urinary
bladder
x-ray film.
germ layers. The pathologic diagnosis nign retroperitoneal teratoma.
was be-
Comment In 1949 Palumbo et ~1.~ reviewed 57 cases of retroperitoneal teratoma from the literature, and added 1 case of their own. Sixty-two per cent were patients in the pediatric age group. Presenting symptoms were vague and frequently secondary to the pressure effects of the tumor growth on neighboring organs, including abdominal or back pain, nausea, vomiting, constipation, and urinary tract symptoms. Abdominal en-
UROLOGY
/
DECEMBER
1976
/
VOLUME
VIII,
NUMBER
6
abdominal mass or enlargement. Findings on intravenous pyelogram when recorded demonstrated, in the majority of cases, a perirenal soft tissue mass, often containing areas of calcification. Urinary tract displacement and obstruction were also noted. Bowel studies appeared to help in localizing the mass to the retroperitoneal space. Case 1 in this report is the first reported case of a retroperitoneal teratoma demonstrated arteriographically, illustrating the avascular appearance with marked displacement of neighboring organs. Arteriography would appear useful in differentiating benign teratoma from malignant tumors such as neuroblastoma, hepatoma, and nephroblastoma which might also demonstrate calcifications in abdominal masses. Urology Service Lackland AFB, Texas (DR. WEBER) References 1. WILLIS, R. A. :
FIGURE 3. Excretory urogram demonstrating placement of right kidney by mass containing cijcation consistent with bone and teeth.
discal-
largement and a palpable mass were the most common physical findings. Ten per cent of the tumors demonstrated malignant changes. Engle, Elkins, and Fletcher2 reviewed another 30 cases in 1968. Fifty-three per cent were fifteen years of age or younger, and symptoms and signs were similar to the Palumbo et ~1.~ series. Ten per cent were malignant, and all of these patients died within a year of diagnosis. In their personal case report an absence of identifiable adrenal tissue at surgical exploration was noted, similar to the findings of our 2 patients. Whether this represents pressure atrophy, tumor replacement, or maldevelopment of the adrenal gland during embryonic growth remains speculative; certainly presentation of normal adrenal glands adjacent to retroperitoneal teratomas has also been reported.3 A summary of an additional 14 cases from the recent literature is reviewed in Table I.5-14 Once again most patients were in the pediatric age group (88 per cent). The presenting symptoms and signs included abdominal, back, and scrotal pain, and weight loss, fever, loose stools, and
UROLOGY
/ DECEMBER1976
/ VOLUMEVIII, NUMBER6
Atlas of Tumor Pathology: Teratomas, Washington, D.C., Armed Forces Institute of Pathology, 1951, sect. III, fast. 9, p, 9. 2. ENGEL, R. M., ELKINS, R. C., and FLETCHER, B. D. : Retroperitoneal teratoma: review of the literature and presentation of an unusual case, Cancer 22: 1068 (1968).
3. ARNHEIM, E. E.:
Retroperitoneal teratomas in infancy and childhood, Pediatrics 8: 309 (1951). 4. PALUMBO, L. T., CROSS, K. R., SMITH, A. N., and BARONAS, A. A.: Primary teratomas of the lateral retroperitoneal spaces, Surgery 26: 149 (1949). 5. MALIK, T. K., MALIK, G. B., and DIESH, G.: Retroperitoneal teratoma with nephroblastic tissue as the main component, Internat. Surg. 47: 246 (1967). 6. ADAMS, G., and FREUND, E.: Ueber einen retroperitonealen dysontogenetischen malignen Tumor, Zentralbl. Chir. 94: 373 (1969). 7. SNARR, J. W., and MACPHERSON, R. 1.: An unusual abdominal mass in an infant - a case for diagnosis, J. Can. Assoc. Radiol. 21:53 (1970). 8. MAMAKOS, M. S. : Retroperitoneal teratoma. Five year survival in adolescence, N.Y. State J. \4ed. 71:
1838(1971). 9. MASUDA, F., UEDA, M., and MINAMI, T.:
Retroperitoneal teratoma in infants, Jap. J. Urol. 63: 175 (1971). 10.KERAMIDAS, D. C., and VOYATZIS, N. G.: Retroperitoneal teratoma, J. Pediatr. Surg. 7: 434 (1972). 11. BANERJEE, A. K., SIRCAR, P. K., and GIDWANI, C. R.: Retroperitoneal teratoma, Indian J. Pediatr. 40: 193 (1973). 12. SALLEH, H. M.: Retroperitoneal teratoma, Med. J. Malaysia 28: 43 (1973). 13.TALIB, V. H., et al. : Benign retroperitoneal teratoma, Indian J. Pediatr. 41:58 (1974). 14. MAHOUR, G. H., WOOLEY, M. >I., TRIVEDI, S. N., and LANDING, B. H.: Teratomas in infancy and childhood: experience with 81 cases, Surgery 76: 309 (1974).
621
