Partial Splenectomy ByRobert
for Symptomatic
J. Havlik, Robert J. Touloukian, New Haven,
Splenic Hamartoma
Richard I. Markowitz,
and Patrick Buckley
Connecticut
0 Lower pole splenectomy with preservation of residual splenic function was successfully performed in a 3-year-old boy with a symptomatic splenic hamartoma. Following resection, several of the patient’s constitutional symptoms resolved. This is the first reported case of a splenic hamartoma treated by partial splenectomy. @ 1990 by W. 6. Saunders Company. INDEX WORDS: hypersplenism.
Splenectomy,
partial; splenic hamartoma:
PLENIC hamartomas are rare, nonneoplastic tumors’ composed of an abberrant mixture of normal splenic tissue components forming single or multiple masses within the splenic pulp. There have been slightly more than 100 cases reported in all age groups since the lesion was first described by Rokitansky in 186 1.2-4 Most patients with splenic hamartomas are asymptomatic and few have clinical findings.293Over 75% of reported cases have been incidental findings during Iaparotomy or autopsy.* In symptomatic patients, the most common physical finding is a left upper quadrant mass, and the most common laboratory finding is hypersplenism. A few patients have been reported with splenomegaly, hypersplenism, fevers, recurrent infec-
S
Fig 2. Operative photograph illustrates the large spherical hamartoma, replacing the lower pole of the spleen.
tions, and growth retardation. A child who underwent a partial splenectomy for a splenic hamartoma is the subject of this report. CASE
REPORT
A 2-year 1l-month-old white boy presented for his 3-year checkup with a palpable mass in the left upper quadrant of the abdomen. Beginning at approximately 2 years of age, he contracted numerous episodes of “otitis” and “viral illness,” each of which took a prolonged time to resolve. In addition, the child was increasingly lethargic, sleeping 12 to 13 hours per day, had a decreased appetite, and gained only 2 pounds over the entire year prior to admission, with body weight decreasing from the 25th to the 10th percentile, and overall height from the 50th to the 25th percentile. The child also complained intermittently of abdominal pain and suffered unexplained febrile episodes. On admission, the child was afebrile, and had a normal examination except for a firm, nontender, 8- to IO-cm mass below the left costal margin. Laboratory studies showed a hemoglobin of 8.7, a hematocrit of 29.5, retie count 0.4’S, and a mean corpuscular volume (MCV) of 58. Platelet count was 107,000 and white count was 10,400. with 64 segs, two bands, 20 lymphocytes, and one eosinophil.
Fig 1. -‘“Tc sulfur-colloid liver/spleen scan confirms splenic origin of mass, which expands the lower margin of the spleen. The mass itself faintly takes up the radionuclide (arrow).
From the Sections of Pediatric Surgery, Pediatric Radiology, and Pathology, Yale University School of Medicine. New Haven, CT. Address reprint requests to Robert J. Touloukian. MD, Yale University School of Medicine. Department of Surgery, PO Box 3333,333 Cedar St, New Haven, CT 06510. 0 1990 by W.B. Saunders Company. 0022-3468/90/2512-0025$03.00/O
Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1273-1275
1273
HAVLIK ET AL
1274
Fig 3. Splenic hamartome. The resected specimen has been cut to demonstrate the interface between normal spleen on the right and the splenic hamartoma (left).
An abdominal ultrasound examination demonstrated a round, solid, discrete mass arising from the splenic tip and situated anterior and lateral to the left kidney. The intrasplenic nature of the mass was confirmed by technetium 99m sulfur colloid liver/spleen scan (Fig l), which showed normal splenic tissue surrounding the upper part of the mass (“claw” sign). The mass itself took up the radionuclide very faintly. A computed tomography scan, with and without intravenous contrast, demonstrated no calcification within the mass. The mass was inhomogeneous, with areas of lower density suggesting hemorrhage or necrosis. The adjacent splenic rim showed normal enhancement with contrast, whereas the mass showed minimal opacification, suggesting that it was not a primary vascular malformation. Resolving hematoma was also considered to be unlikely. At surgery, a 6-cm spherical mass was observed extending from the lower pole of the spleen (Fig 2), supplied by a distinct group of accessory hilar blood vessels. With the spleen fully mobilized, these vessels were ligated and divided. The splenic capsule adjacent to the mass was then sharply incised, leaving a l- to 2-cm “rim” of grossly normal spleen intact with the mass. Hemostasis of the splenic parenchyma was achieved using electrocautery, and the capsular edges were reapproximated with 3-Osilk horizontal mattress sutures. The 6-cm-diameter tumor was partially surrounded by a rim of normal spleen (Fig 3), and was composed of a mixture of friable red tissue and firmer white areas. The friable areas were slightly cystic and appeared congested with blood. Table 1. Demographic
Case NQ.
Age (vr)
Features
sex
The histology of the hamartoma contrasted markedly with that of the surrounding normal splenic tissues. No white pulp lymphoid tissue or recognizable red pulp was present in the hamartoma. The friable red areas appeared somewhat nodular and contained many dilated blood-filled spaces in a background of compacted spindleshaped cells, and a few lymphocytes and plasma cells. The firmer white areas contained cystic spaces filled with eosinophilic proteinaceous fluid. Scattered vessels were present, but numerous plump, elongated cells with large nuclei and relatively prominent nucleoli were the major cell type. These seemed to form vessel-like structures in some regions. A large number of plasma cells and scattered eosinophils were also present. The patient’s postoperative course has been uneventful. He has had no further complaints of abdominal pain or febrile episodes since discharge from the hospital. His lethargy has resolved. He has grown 2% in height, continuing along the 20th percentile growth curve. His appetite has subjectively improved; however, he has gained only 2 lb in 10 months. A blood count obtained 4 months postoperatively showed hemoglobin of 9.3, hematocrit 31.3, MCV of 57, and white count 10.2 with 32 polymorphs, 59 lymphocytes, six monocytes, and three eosinophils. Platelet count was 363,000. Hemoglobin electrophoresis was consistent with @-thalassemia trait. A follow-up liver/spleen scan shows regeneration of “functionally normal” splenic tissue at the site of the previous resection. DISCUSSION
Splenic hamartomas are rare lesions that have been referred to as splenoma, splenoadenoma, fibrosplenoma, and nodular hyperplasia of the spleen.5-7 Hamartomas are usually asymptomatic, the majority being incidental findings at laparotomy or autopsy. They occur either as solitary or multiple nodules within the splenic parenchyma. They are nonencapsulated, but often compress the surrounding parenchyma into a pseudocapsule. Tumors greater than 2 cm in diameter are rare, but represent the majority of lesions removed surgically for symptomatic disease.2 There is a small subset of these patients who have systemic manifestations of these hamartomas. These children with “symptomatic splenic hamartoma” were first described by Videback in 1953* and Iozzo et al in 1980.4A constellation of symptoms may coexist, including fever, weakness, fatigue, anemia, leukopenia, thrombocytopenia, growth failure, and a propensity to recurrent infections. Previously reported cases of
and Clinical Signs in Splenic Hamartoma
No. of Nodules in Spleen
Growth
Retardation
Frequent Infections
FWW
1
12
F
Multiple
+
+
2
9
M
Multiple
+
+
+
3
9
M
Single
n/c
n/c
n/c
4
54
F
Single
n/c
n/c
n/c
5
29
F
Multiple
n/c
+
n/c
+
6
31
F
Single
n/c
n/c
o/c
7
24
M
Single
n/c
n/c
8
3
F
Single
+
+
n/c +
Abbreviations: +, present; n/c, not commented on by author.
PARTIAL SPLENECTOMY FOR SPLENIC HAMARTOMA
1275
Table 2. Physical Findings and laboratory Case
No.
Adenooathy
Hepatosplenomegaly
Anemia
Data in Splenic Hamartoma Leukopenia
Thrombocytopenia
Reference
+ +
+ +
+ +
4
n/c
n/c
+
2
0
0
+
7
+
0
+
8
0
0
9
0
0
+
10
0
0
0
Havlik et al
n/c
4
Abbreviations: +, present; 0. absent: n/c. not commented on by author.
symptomatic splenic hamartoma and the current report are summarized on Tables 1 and 2.9310The case presented herein illustrates a patient with systemic effects of splenic hamartoma having febrile episodes to 105OF, fatigue, lethargy, and anorexia. These symptoms resolved following resection; however, the patient’s anemia and slow growth persisted and may be attributable to other systemic factors such as hereditary thalassemia. Following the original report of King and Schu-
macker” on the risk of overwhelming postsplenectomy
infection, the merits of splenic preservation in children have been clearly documented.‘2-14 The large size (6 cm) of this solitary nodule, and its peripheral location, made this tumor amenable to resection with partial splenectomy. Follow-up nuclear scan has documented regeneration of normal splenic tissue. We conclude that splenic preservation procedure for splenic hamartoma is both curative and avoids the potential risks associated with total splenectomy.
REFERENCES 1. Gorvin DF, King FM: Cysts and non-lymphomatous tumors of thespleen. Path01 Ann 16:61-80,198l 2. Silverman ML, Livolsi VA: Splenic hamatomas. Am J Clin Path01 70:224-229, 1978 3. Morgenstern L, McCafferty L, Rosenberg J, et al: Hamartomas of the spleen. Arch Surg 119:1291-1293. 1984 4. Iozzo RV. Maas JE, Chard RL: Symptomatic splenic hamartoma: A report of two cases and a review of the literature. Pediatrics 66x261-266, 1980 5. Coe JI, Von Drashek SC: Hamartoma of the spleen. A report of four cases. Am J Pathol28:663-668, 1952 6. Berge T: Splenoma. Acta Path01 Microbial Stand 63:333-339, 1965 7. Ross CF. Schiller KFR: Hamartoma of the spleen associated with thrombocytopenia. J Path01 105:62-64, 1971
8. Videback A: Hypersplenism associated with hamartomas of the spleen. Acta Med Stand 146:276,1953 (abstr) 9. Schrijver H, Verdonk GS: Hamartoma of the spleen with inhibition of the bone marrow. Acta Med Stand 158:235-237, 1957 10. Shalev 0, Ariel I: Hamartoma of the spleen. A case report. Isr J Med Sci 14:862-864, 1978 11. King H, Schumacker HB Jr: Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann Surg 136:239-242, 1952 12. Francke EL, New HC: Postsplenectomy infection. Surg Clin North Am 61:135-155, 1981 13. Singer DB: Postsplenectomy sepsis, in Rosenberg HS, Bolande RP (eds): Perspectives in Pediatric Pathology. Chicago, IL, Year Book, 1973, pp 285-311 14. Dickerman JD: Splenectomy and sepsis: A warning. Pediatrics 64:938-940, 1979
for Symptomatic
J. Havlik, Robert J. Touloukian, New Haven,
Splenic Hamartoma
Richard I. Markowitz,
and Patrick Buckley
Connecticut
0 Lower pole splenectomy with preservation of residual splenic function was successfully performed in a 3-year-old boy with a symptomatic splenic hamartoma. Following resection, several of the patient’s constitutional symptoms resolved. This is the first reported case of a splenic hamartoma treated by partial splenectomy. @ 1990 by W. 6. Saunders Company. INDEX WORDS: hypersplenism.
Splenectomy,
partial; splenic hamartoma:
PLENIC hamartomas are rare, nonneoplastic tumors’ composed of an abberrant mixture of normal splenic tissue components forming single or multiple masses within the splenic pulp. There have been slightly more than 100 cases reported in all age groups since the lesion was first described by Rokitansky in 186 1.2-4 Most patients with splenic hamartomas are asymptomatic and few have clinical findings.293Over 75% of reported cases have been incidental findings during Iaparotomy or autopsy.* In symptomatic patients, the most common physical finding is a left upper quadrant mass, and the most common laboratory finding is hypersplenism. A few patients have been reported with splenomegaly, hypersplenism, fevers, recurrent infec-
S
Fig 2. Operative photograph illustrates the large spherical hamartoma, replacing the lower pole of the spleen.
tions, and growth retardation. A child who underwent a partial splenectomy for a splenic hamartoma is the subject of this report. CASE
REPORT
A 2-year 1l-month-old white boy presented for his 3-year checkup with a palpable mass in the left upper quadrant of the abdomen. Beginning at approximately 2 years of age, he contracted numerous episodes of “otitis” and “viral illness,” each of which took a prolonged time to resolve. In addition, the child was increasingly lethargic, sleeping 12 to 13 hours per day, had a decreased appetite, and gained only 2 pounds over the entire year prior to admission, with body weight decreasing from the 25th to the 10th percentile, and overall height from the 50th to the 25th percentile. The child also complained intermittently of abdominal pain and suffered unexplained febrile episodes. On admission, the child was afebrile, and had a normal examination except for a firm, nontender, 8- to IO-cm mass below the left costal margin. Laboratory studies showed a hemoglobin of 8.7, a hematocrit of 29.5, retie count 0.4’S, and a mean corpuscular volume (MCV) of 58. Platelet count was 107,000 and white count was 10,400. with 64 segs, two bands, 20 lymphocytes, and one eosinophil.
Fig 1. -‘“Tc sulfur-colloid liver/spleen scan confirms splenic origin of mass, which expands the lower margin of the spleen. The mass itself faintly takes up the radionuclide (arrow).
From the Sections of Pediatric Surgery, Pediatric Radiology, and Pathology, Yale University School of Medicine. New Haven, CT. Address reprint requests to Robert J. Touloukian. MD, Yale University School of Medicine. Department of Surgery, PO Box 3333,333 Cedar St, New Haven, CT 06510. 0 1990 by W.B. Saunders Company. 0022-3468/90/2512-0025$03.00/O
Journal of Pediatric Surgery, Vol 25, No 12 (December), 1990: pp 1273-1275
1273
HAVLIK ET AL
1274
Fig 3. Splenic hamartome. The resected specimen has been cut to demonstrate the interface between normal spleen on the right and the splenic hamartoma (left).
An abdominal ultrasound examination demonstrated a round, solid, discrete mass arising from the splenic tip and situated anterior and lateral to the left kidney. The intrasplenic nature of the mass was confirmed by technetium 99m sulfur colloid liver/spleen scan (Fig l), which showed normal splenic tissue surrounding the upper part of the mass (“claw” sign). The mass itself took up the radionuclide very faintly. A computed tomography scan, with and without intravenous contrast, demonstrated no calcification within the mass. The mass was inhomogeneous, with areas of lower density suggesting hemorrhage or necrosis. The adjacent splenic rim showed normal enhancement with contrast, whereas the mass showed minimal opacification, suggesting that it was not a primary vascular malformation. Resolving hematoma was also considered to be unlikely. At surgery, a 6-cm spherical mass was observed extending from the lower pole of the spleen (Fig 2), supplied by a distinct group of accessory hilar blood vessels. With the spleen fully mobilized, these vessels were ligated and divided. The splenic capsule adjacent to the mass was then sharply incised, leaving a l- to 2-cm “rim” of grossly normal spleen intact with the mass. Hemostasis of the splenic parenchyma was achieved using electrocautery, and the capsular edges were reapproximated with 3-Osilk horizontal mattress sutures. The 6-cm-diameter tumor was partially surrounded by a rim of normal spleen (Fig 3), and was composed of a mixture of friable red tissue and firmer white areas. The friable areas were slightly cystic and appeared congested with blood. Table 1. Demographic
Case NQ.
Age (vr)
Features
sex
The histology of the hamartoma contrasted markedly with that of the surrounding normal splenic tissues. No white pulp lymphoid tissue or recognizable red pulp was present in the hamartoma. The friable red areas appeared somewhat nodular and contained many dilated blood-filled spaces in a background of compacted spindleshaped cells, and a few lymphocytes and plasma cells. The firmer white areas contained cystic spaces filled with eosinophilic proteinaceous fluid. Scattered vessels were present, but numerous plump, elongated cells with large nuclei and relatively prominent nucleoli were the major cell type. These seemed to form vessel-like structures in some regions. A large number of plasma cells and scattered eosinophils were also present. The patient’s postoperative course has been uneventful. He has had no further complaints of abdominal pain or febrile episodes since discharge from the hospital. His lethargy has resolved. He has grown 2% in height, continuing along the 20th percentile growth curve. His appetite has subjectively improved; however, he has gained only 2 lb in 10 months. A blood count obtained 4 months postoperatively showed hemoglobin of 9.3, hematocrit 31.3, MCV of 57, and white count 10.2 with 32 polymorphs, 59 lymphocytes, six monocytes, and three eosinophils. Platelet count was 363,000. Hemoglobin electrophoresis was consistent with @-thalassemia trait. A follow-up liver/spleen scan shows regeneration of “functionally normal” splenic tissue at the site of the previous resection. DISCUSSION
Splenic hamartomas are rare lesions that have been referred to as splenoma, splenoadenoma, fibrosplenoma, and nodular hyperplasia of the spleen.5-7 Hamartomas are usually asymptomatic, the majority being incidental findings at laparotomy or autopsy. They occur either as solitary or multiple nodules within the splenic parenchyma. They are nonencapsulated, but often compress the surrounding parenchyma into a pseudocapsule. Tumors greater than 2 cm in diameter are rare, but represent the majority of lesions removed surgically for symptomatic disease.2 There is a small subset of these patients who have systemic manifestations of these hamartomas. These children with “symptomatic splenic hamartoma” were first described by Videback in 1953* and Iozzo et al in 1980.4A constellation of symptoms may coexist, including fever, weakness, fatigue, anemia, leukopenia, thrombocytopenia, growth failure, and a propensity to recurrent infections. Previously reported cases of
and Clinical Signs in Splenic Hamartoma
No. of Nodules in Spleen
Growth
Retardation
Frequent Infections
FWW
1
12
F
Multiple
+
+
2
9
M
Multiple
+
+
+
3
9
M
Single
n/c
n/c
n/c
4
54
F
Single
n/c
n/c
n/c
5
29
F
Multiple
n/c
+
n/c
+
6
31
F
Single
n/c
n/c
o/c
7
24
M
Single
n/c
n/c
8
3
F
Single
+
+
n/c +
Abbreviations: +, present; n/c, not commented on by author.
PARTIAL SPLENECTOMY FOR SPLENIC HAMARTOMA
1275
Table 2. Physical Findings and laboratory Case
No.
Adenooathy
Hepatosplenomegaly
Anemia
Data in Splenic Hamartoma Leukopenia
Thrombocytopenia
Reference
+ +
+ +
+ +
4
n/c
n/c
+
2
0
0
+
7
+
0
+
8
0
0
9
0
0
+
10
0
0
0
Havlik et al
n/c
4
Abbreviations: +, present; 0. absent: n/c. not commented on by author.
symptomatic splenic hamartoma and the current report are summarized on Tables 1 and 2.9310The case presented herein illustrates a patient with systemic effects of splenic hamartoma having febrile episodes to 105OF, fatigue, lethargy, and anorexia. These symptoms resolved following resection; however, the patient’s anemia and slow growth persisted and may be attributable to other systemic factors such as hereditary thalassemia. Following the original report of King and Schu-
macker” on the risk of overwhelming postsplenectomy
infection, the merits of splenic preservation in children have been clearly documented.‘2-14 The large size (6 cm) of this solitary nodule, and its peripheral location, made this tumor amenable to resection with partial splenectomy. Follow-up nuclear scan has documented regeneration of normal splenic tissue. We conclude that splenic preservation procedure for splenic hamartoma is both curative and avoids the potential risks associated with total splenectomy.
REFERENCES 1. Gorvin DF, King FM: Cysts and non-lymphomatous tumors of thespleen. Path01 Ann 16:61-80,198l 2. Silverman ML, Livolsi VA: Splenic hamatomas. Am J Clin Path01 70:224-229, 1978 3. Morgenstern L, McCafferty L, Rosenberg J, et al: Hamartomas of the spleen. Arch Surg 119:1291-1293. 1984 4. Iozzo RV. Maas JE, Chard RL: Symptomatic splenic hamartoma: A report of two cases and a review of the literature. Pediatrics 66x261-266, 1980 5. Coe JI, Von Drashek SC: Hamartoma of the spleen. A report of four cases. Am J Pathol28:663-668, 1952 6. Berge T: Splenoma. Acta Path01 Microbial Stand 63:333-339, 1965 7. Ross CF. Schiller KFR: Hamartoma of the spleen associated with thrombocytopenia. J Path01 105:62-64, 1971
8. Videback A: Hypersplenism associated with hamartomas of the spleen. Acta Med Stand 146:276,1953 (abstr) 9. Schrijver H, Verdonk GS: Hamartoma of the spleen with inhibition of the bone marrow. Acta Med Stand 158:235-237, 1957 10. Shalev 0, Ariel I: Hamartoma of the spleen. A case report. Isr J Med Sci 14:862-864, 1978 11. King H, Schumacker HB Jr: Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann Surg 136:239-242, 1952 12. Francke EL, New HC: Postsplenectomy infection. Surg Clin North Am 61:135-155, 1981 13. Singer DB: Postsplenectomy sepsis, in Rosenberg HS, Bolande RP (eds): Perspectives in Pediatric Pathology. Chicago, IL, Year Book, 1973, pp 285-311 14. Dickerman JD: Splenectomy and sepsis: A warning. Pediatrics 64:938-940, 1979
