Diagnostic Radiology
Angiographic Demonstration of a Calcified Splenic Hamartoma"
CASE REPORT A 56-year-old white man was admitted complaining of increasing fatigue of several years duration. Physical examination revealed a large, firm, smooth-surfaced mass in the left upper quadrant of the abdomen, extending 6 cm below the costal margin. The liver was not palpable. Laboratory findings were not remarkable. Plain films of the abdomen, upper gastrointestinal and gallbladder series, barium enema studies, and excretory urography indicated a large mass containing innumerable spotted calcifications in the left upper quadrant (Fig. 1). A 99mTc-sulfur colloid study of the liver and spleen showed no radioactivity within the mass. An ultrasound study indicated that the mass was solid. Celiac angiography showed a large, avascular mass within the splenic capsule with normal vascularity only at the upper pole (Figs. 2 and 3). Because of the numerous calcifications within the mass, hemangioma was considered. At surgery, a large, firm mass was palpable within the spleen, and total splenectomy was performed. The spleen was adherent to the left hemidia-
Senichiro Komaki, M.D.,2 and Otto F. Gombas, M.D. A case of hamartoma of the spleen is reported. Radiographic examination revealed a mass containing innumerable calcifications in the left upper quadrant. Angiography demonstrated an avascular mass. The authors believe that such calcification and avascularity are late manifestations of a slowly-growing splenic tumor which may well be hypervascular if examined earlier. INDEX TERMS: Hamartoma. Spleen, (Spleen, hamartoma, 7[751.314)
calcification. Spleen,
neoplasms
Radiology 121:77-78, October 1976
of the spleen, particularly benign tumors, are rare (1). We recently observed a case of hamartoma of the spleen with calcification. Celiac angiography revealed an avascular mass. To our knowledge, only 3 such cases with angiographic demonstration have been reported previously; the present case involves a particularly unusual angiographic manifestation.
N
EOPLASMS
Fig. 1. Excretory urogram demonstrates a large mass displacing the left kidney caudally. Note the multiple punctate calcifications within the mass. Fig. 2. Arterial phase of celiac angiography reveals stretching of the intrasplenic branches without tumor vessels or pooling of contrast material. Fig. 3. Venous phase of celiac angiography shows no tumor stain. The upper pole represents normal residual splenic tissue. Note the venous return from the upper pole (arrow). Fig. 4. Section of the fibrosed and calcified tumor tissue, showing distended and engorged sinus-like vascular channels (straight arrow) and aggregated lymphocytes (curved arrow), most likely remnants of lymph follicles. Calcifications (arrowheads) are seen as dark substances. (H & E X 150) 1 From the Departments of Radiology (S.K.) and Pathology (O.F.G.), Medical College of Wisconsin, and Veterans Administration Center, Wood (Milwaukee), Wise. 53193. Accepted for publication in November 1975. 2 Present address: Radiology Department, Kyushu University Hospital, Maidashi, Higashiku, Fukuoka City, Japan. sjh
77
78
SENICHIRO KOMAKI AND OTTO
F. GOMBAS
October 1976
phragm, colon, and left kidney. The remainder of the abdominal viscera appeared normal. Pathology: The spleen was globular and weighed 1,740 g; its contour was distorted by the mass. A cross section revealed a tumor measuring 14 X 10 X 9 cm occupying the greater part of the organ. The tumor was well demarcated from the surrounding splenic parenchyma but without a capsule and was dark red and markedly hemorrhagic in appearance, mottled with numerous yellowish-gray focal areas with pale yellow regions of fibrosis. Microscopic study (Fig. 4) revealed numerous dilated vascular spaces lined by a single layer of endothelium with considerable interspaced fibrosis. The fibrous structure showed a marked deposit of mineral salts, frequently surrounded by a number of histiocytes and occasional multinucleated giant cells. Bundles of smooth muscle were seen within the fibrous tissue, representing obliterated blood vessels. The surrounding parenchyma appeared normal, with a trabecular pattern and sinusoid spaces. Based on these microscopic findings, a diagnosis of hamartoma of the spleen was made.
fications in splenic hamartoma except for the microscopic calcifications reported by Graham et al. (4). We believe that these calcifications are due to remote hemorrhage within the slowly-growing tumor secondary to ischemia, especially since no angiographically visible vessels were present in the tumor. Histologically, fibrotic scars were seen throughout the tumor and many vessels within the tumor were obliterated by fibrous scars, confirming the ischemic nature of the entity. A review of the literature on hamartomas also appears to support our opinion. Wexler and Abrams (15) demonstrated abnormal vessels in angiograms of the spleen in a 4year-old boy. Histologically, no hemorrhage or fibrosis was noted. On the other hand, the older patients (68 and 79 years of age) described by Coe and Von Drashek (3) had both hemorrhage and fibrosis within the tumor. We therefore speculate that avascularity is merely a manifestation of degeneration of the tumor, which might have been hypervascular if examined earlier. The simultaneous calcification and avascularity of the mass strongly indicate a slowly-growing mass, suggesting hamartoma as one possibility.
DISCUSSION
ACKNOWLEDGMENTS: We wish to thank Drs. Frank M. Maddison and George F. Unger for their advice in preparing this manuscript.
According to Kirkland and McDonald (8), hamartoma of the spleen was first reported by Falkowski in 1914. Since then, fewer than 39 cases have been reported (12), none of them diagnosed preoperatively (15). In spite of the recent trend toward angiographic evaluation of an abdominal mass prior to laparotomy, only three cases of hamartoma of the spleen with angiographic demonstration have been reported (4, 15); these had different angiographic patterns from our case, however, in that numerous large tortuous vessels were seen within the mass and early venous filling was noted. Hemangioma, hamartoma, and splenoadenoma have been described as having the same angiographic appearance: vascular lakes, a well-defined tumor edge, and curvilinear displacement of adjacent vessels (10, 14). Two separate case reports of hemangioma include findings similar to those described above, namely, hypervascularity (13) and irregular vascular channels in the arterial phase (11). Neovascularity has been reported in malignant splenic tumors (9, 10). Thus all of these cases involve the finding of abnormal vessels within the mass. On the other hand, Baltaxe et al. (2) state that all splenic tumors (whether benign or malignant) are avascular and that angiography does not allow differentiation between malignant and benign processes. The case described here appears to support their opinion. The calcification within the mass is another unusual feature of this case. Plain films of the abdomen and specimen showed numerous homogeneous calcifications widely scattered within the mass. The border of the tumor and residual normal splenic tissue were free of calcification. Histologically, calcifications were present in the fibrotic scar but not in the vascular spaces, excluding the possibility of phleboliths. Circular calcifications may be seen in both true and false intrasplenic cysts (5, 7, 10, 15). Multiple intrasplenic calcifications may be due to tuberculosis, histoplasmosis (5, 16), phleboliths (5), infarction (5), hemangioma (5-7, 10), and metastatic ovarian pseudomucinous carcinoma (10). However, a search of the literature failed to disclose similar calcl-
Radiology Service/114 Veterans Administration Center Wood, Wise. 53193
REFERENCES 1. Anderson WAD: Pathology. St. Louis, Mo., Mosby, 6th Ed, 1971, pp 1317-1318 2. Baltaxe HA, Watson RC, Levin DC: The angiographic appearance of splenic masses. Angiology 23:316-328, Jun 1972 3. Coe JI, Von Drashek SC: Hamartoma of the spleen. A report of four cases. Am J Pathol 28:663-671, Jul-Aug 1952 4. Graham JC Jr, Weidner WA, Vinik M: The angiographic features of organizing splenic hematoma (?hamartoma). Am J RoentgenoI107:430-433, Oct 1969 5. Gray EF: Calcifications of the spleen. Am J Roentgenol 51:336-351, Mar 1944 6. Grieco RV, Bartone NF: Roentgen visualization of phleboIiths in hemangioma of the gastrointestinal tract. Am J Roentgenol 101:406-408, Oct 1967 7. Husni EA: The clinical course of splenic hemangioma. With emphasis on spontaneous rupture. Arch Surg 83:681-688, Nov 1961 8. Kirkland WG, McDonald JR: Hamartoma of the spleen. Report of three surgical cases. Arch PathoI45:371-379, Mar 1948 9. Morissette JJ, Viamonte M, Viamonte M Jr, et al: Primary spindle-cell sarcoma of the spleen with angiographic demonstration. Radiology 106:549-550, Mar 1973 10. Rosch J: Tumours of the spleen: the value of selective arteriography. Clin RadioI17:183-190, Apr 1966 11. Rosenthal T, Adar R, Wolfstein I, et al: Cavernous hemangioma of the spleen; angiographic observations. Angiology 24:430433, Jul-Aug 1973 12. Ross CF, Schiller KFR: Hamartoma of spleen associated with thrombocytopenia. J Pathol 105:62-64, Sep 1971 13. Tada S, Shin M, Takashima T, et al: Diffuse capillary hemangiomatosis of the spleen as a cause of portal hypertension. Radiology 104:63-64, Jul 1972 14. Wendth AJ Jr, Samson R, O'Connor S, et al: The scope of splenic arteriography. Angiology 24:480-507, Sep 1973 15. Wexler L, Abrams HL: Hamartoma of the spleen. Angiographic observations. Am J Roentgenol 92: 1150-1155, Nov 1964 16. Young JM, Bills RJ, Ulrich E: Discrete splenic calcification in necropsy material. Am J Pathol 33: 189-197, Jan-Feb 1957
Angiographic Demonstration of a Calcified Splenic Hamartoma"
CASE REPORT A 56-year-old white man was admitted complaining of increasing fatigue of several years duration. Physical examination revealed a large, firm, smooth-surfaced mass in the left upper quadrant of the abdomen, extending 6 cm below the costal margin. The liver was not palpable. Laboratory findings were not remarkable. Plain films of the abdomen, upper gastrointestinal and gallbladder series, barium enema studies, and excretory urography indicated a large mass containing innumerable spotted calcifications in the left upper quadrant (Fig. 1). A 99mTc-sulfur colloid study of the liver and spleen showed no radioactivity within the mass. An ultrasound study indicated that the mass was solid. Celiac angiography showed a large, avascular mass within the splenic capsule with normal vascularity only at the upper pole (Figs. 2 and 3). Because of the numerous calcifications within the mass, hemangioma was considered. At surgery, a large, firm mass was palpable within the spleen, and total splenectomy was performed. The spleen was adherent to the left hemidia-
Senichiro Komaki, M.D.,2 and Otto F. Gombas, M.D. A case of hamartoma of the spleen is reported. Radiographic examination revealed a mass containing innumerable calcifications in the left upper quadrant. Angiography demonstrated an avascular mass. The authors believe that such calcification and avascularity are late manifestations of a slowly-growing splenic tumor which may well be hypervascular if examined earlier. INDEX TERMS: Hamartoma. Spleen, (Spleen, hamartoma, 7[751.314)
calcification. Spleen,
neoplasms
Radiology 121:77-78, October 1976
of the spleen, particularly benign tumors, are rare (1). We recently observed a case of hamartoma of the spleen with calcification. Celiac angiography revealed an avascular mass. To our knowledge, only 3 such cases with angiographic demonstration have been reported previously; the present case involves a particularly unusual angiographic manifestation.
N
EOPLASMS
Fig. 1. Excretory urogram demonstrates a large mass displacing the left kidney caudally. Note the multiple punctate calcifications within the mass. Fig. 2. Arterial phase of celiac angiography reveals stretching of the intrasplenic branches without tumor vessels or pooling of contrast material. Fig. 3. Venous phase of celiac angiography shows no tumor stain. The upper pole represents normal residual splenic tissue. Note the venous return from the upper pole (arrow). Fig. 4. Section of the fibrosed and calcified tumor tissue, showing distended and engorged sinus-like vascular channels (straight arrow) and aggregated lymphocytes (curved arrow), most likely remnants of lymph follicles. Calcifications (arrowheads) are seen as dark substances. (H & E X 150) 1 From the Departments of Radiology (S.K.) and Pathology (O.F.G.), Medical College of Wisconsin, and Veterans Administration Center, Wood (Milwaukee), Wise. 53193. Accepted for publication in November 1975. 2 Present address: Radiology Department, Kyushu University Hospital, Maidashi, Higashiku, Fukuoka City, Japan. sjh
77
78
SENICHIRO KOMAKI AND OTTO
F. GOMBAS
October 1976
phragm, colon, and left kidney. The remainder of the abdominal viscera appeared normal. Pathology: The spleen was globular and weighed 1,740 g; its contour was distorted by the mass. A cross section revealed a tumor measuring 14 X 10 X 9 cm occupying the greater part of the organ. The tumor was well demarcated from the surrounding splenic parenchyma but without a capsule and was dark red and markedly hemorrhagic in appearance, mottled with numerous yellowish-gray focal areas with pale yellow regions of fibrosis. Microscopic study (Fig. 4) revealed numerous dilated vascular spaces lined by a single layer of endothelium with considerable interspaced fibrosis. The fibrous structure showed a marked deposit of mineral salts, frequently surrounded by a number of histiocytes and occasional multinucleated giant cells. Bundles of smooth muscle were seen within the fibrous tissue, representing obliterated blood vessels. The surrounding parenchyma appeared normal, with a trabecular pattern and sinusoid spaces. Based on these microscopic findings, a diagnosis of hamartoma of the spleen was made.
fications in splenic hamartoma except for the microscopic calcifications reported by Graham et al. (4). We believe that these calcifications are due to remote hemorrhage within the slowly-growing tumor secondary to ischemia, especially since no angiographically visible vessels were present in the tumor. Histologically, fibrotic scars were seen throughout the tumor and many vessels within the tumor were obliterated by fibrous scars, confirming the ischemic nature of the entity. A review of the literature on hamartomas also appears to support our opinion. Wexler and Abrams (15) demonstrated abnormal vessels in angiograms of the spleen in a 4year-old boy. Histologically, no hemorrhage or fibrosis was noted. On the other hand, the older patients (68 and 79 years of age) described by Coe and Von Drashek (3) had both hemorrhage and fibrosis within the tumor. We therefore speculate that avascularity is merely a manifestation of degeneration of the tumor, which might have been hypervascular if examined earlier. The simultaneous calcification and avascularity of the mass strongly indicate a slowly-growing mass, suggesting hamartoma as one possibility.
DISCUSSION
ACKNOWLEDGMENTS: We wish to thank Drs. Frank M. Maddison and George F. Unger for their advice in preparing this manuscript.
According to Kirkland and McDonald (8), hamartoma of the spleen was first reported by Falkowski in 1914. Since then, fewer than 39 cases have been reported (12), none of them diagnosed preoperatively (15). In spite of the recent trend toward angiographic evaluation of an abdominal mass prior to laparotomy, only three cases of hamartoma of the spleen with angiographic demonstration have been reported (4, 15); these had different angiographic patterns from our case, however, in that numerous large tortuous vessels were seen within the mass and early venous filling was noted. Hemangioma, hamartoma, and splenoadenoma have been described as having the same angiographic appearance: vascular lakes, a well-defined tumor edge, and curvilinear displacement of adjacent vessels (10, 14). Two separate case reports of hemangioma include findings similar to those described above, namely, hypervascularity (13) and irregular vascular channels in the arterial phase (11). Neovascularity has been reported in malignant splenic tumors (9, 10). Thus all of these cases involve the finding of abnormal vessels within the mass. On the other hand, Baltaxe et al. (2) state that all splenic tumors (whether benign or malignant) are avascular and that angiography does not allow differentiation between malignant and benign processes. The case described here appears to support their opinion. The calcification within the mass is another unusual feature of this case. Plain films of the abdomen and specimen showed numerous homogeneous calcifications widely scattered within the mass. The border of the tumor and residual normal splenic tissue were free of calcification. Histologically, calcifications were present in the fibrotic scar but not in the vascular spaces, excluding the possibility of phleboliths. Circular calcifications may be seen in both true and false intrasplenic cysts (5, 7, 10, 15). Multiple intrasplenic calcifications may be due to tuberculosis, histoplasmosis (5, 16), phleboliths (5), infarction (5), hemangioma (5-7, 10), and metastatic ovarian pseudomucinous carcinoma (10). However, a search of the literature failed to disclose similar calcl-
Radiology Service/114 Veterans Administration Center Wood, Wise. 53193
REFERENCES 1. Anderson WAD: Pathology. St. Louis, Mo., Mosby, 6th Ed, 1971, pp 1317-1318 2. Baltaxe HA, Watson RC, Levin DC: The angiographic appearance of splenic masses. Angiology 23:316-328, Jun 1972 3. Coe JI, Von Drashek SC: Hamartoma of the spleen. A report of four cases. Am J Pathol 28:663-671, Jul-Aug 1952 4. Graham JC Jr, Weidner WA, Vinik M: The angiographic features of organizing splenic hematoma (?hamartoma). Am J RoentgenoI107:430-433, Oct 1969 5. Gray EF: Calcifications of the spleen. Am J Roentgenol 51:336-351, Mar 1944 6. Grieco RV, Bartone NF: Roentgen visualization of phleboIiths in hemangioma of the gastrointestinal tract. Am J Roentgenol 101:406-408, Oct 1967 7. Husni EA: The clinical course of splenic hemangioma. With emphasis on spontaneous rupture. Arch Surg 83:681-688, Nov 1961 8. Kirkland WG, McDonald JR: Hamartoma of the spleen. Report of three surgical cases. Arch PathoI45:371-379, Mar 1948 9. Morissette JJ, Viamonte M, Viamonte M Jr, et al: Primary spindle-cell sarcoma of the spleen with angiographic demonstration. Radiology 106:549-550, Mar 1973 10. Rosch J: Tumours of the spleen: the value of selective arteriography. Clin RadioI17:183-190, Apr 1966 11. Rosenthal T, Adar R, Wolfstein I, et al: Cavernous hemangioma of the spleen; angiographic observations. Angiology 24:430433, Jul-Aug 1973 12. Ross CF, Schiller KFR: Hamartoma of spleen associated with thrombocytopenia. J Pathol 105:62-64, Sep 1971 13. Tada S, Shin M, Takashima T, et al: Diffuse capillary hemangiomatosis of the spleen as a cause of portal hypertension. Radiology 104:63-64, Jul 1972 14. Wendth AJ Jr, Samson R, O'Connor S, et al: The scope of splenic arteriography. Angiology 24:480-507, Sep 1973 15. Wexler L, Abrams HL: Hamartoma of the spleen. Angiographic observations. Am J Roentgenol 92: 1150-1155, Nov 1964 16. Young JM, Bills RJ, Ulrich E: Discrete splenic calcification in necropsy material. Am J Pathol 33: 189-197, Jan-Feb 1957
