Spleen Imaging Enlargement of the Spleen

David Groshar, Ora Israel, and Dov Front

A 19-year-old man was admitted for the acute onset of abdominal pain. He had experi-

enced right abdominal pain for a month before admission but had no other symptoms. Physical examination revealed a hard, somewhat tender, midabdominal mass.

Ultrasound and computed tomography (CT) showed a solid vascular mass in the midabdomen. No spleen was demonstrated. The nature of the mass was not clear and a neoplasm was sus- pected.

On liver-spleen scintigram, uptake of colloid was seen in the large mass (Fig. 1). The spleen was not visualized in the left upper quadrant and the mass was considered to be a markedly enlarged and displaced spleen. At surgery a huge congestive spleen, weighing 1190 g, was found. No adhesions or indications of infarction were noted. The pedicle of the spleen was elongated and twisted. Pathologic examination only showed congestion of the spleen.

"Ectopic," "aberrant," "ptotic," "floating," and "wandering" spleen are some of the names attached to a rare condition, in which the spleen is found in locations other than the left upper quadrant. Elongation of the splenic pedicle and malformation of the dorsal mesogastrium with laxity, poor development, or even absence of the lienogastric and lienorenal ligaments cause this condition) '2'3 It often occurs in females in child- bearing age, and it must be differentiated from a tumor or an ovarian cyst. 3"4'5 A sudden torsion of the pedicle may cause acute venous occlusion which sometimes leads to infarctions. When the torsion is gradual the spleen may reach huge dimensions, as shown in the present case. The ability to take up colloid is apparently preserved.

Diagnosis may be difficult. When the enlarged spleen is not located in the left upper quadrant it may not be recognized and mistaken for a neo- plasm. Plain x-rays, excretory urography, bar- ium enema, upper GI series, ultrasound, and angiography have been suggested, but preopera- tive diagnosis is rarely made. 3'6 Scintigraphy may help in establishing the nature of the mass. Causes of spleen enlargement follow.

COM MON

Cirrhosis of liver 7'8 Extrahepatic portal venous obstruction 7'9 Infiltrative disease of spleen (Gaucher's Nie-

mann-Pick, amyloidosis, sarcoidosis, oth- ers) 8,1o

Inflammatory disease (acute and chronic bacte- rial, viral, parasitic infection) 7'11"~2

Tropical splenomegaly syndrome (endemic) ~3'~4 Myelofibrosis s:~ Polycythemia vera 15 Hemolytic anemia 1~ Leukemia 1~ Malignant lymphoma (Hodgkin's and non-

Hodgkin's) TM

UNCOMMON

Contiguous invasion of tumor 7 Unsuspected subcapsular hematoma H Undiagnosed splenomegaly (needing sur-

gery) 17'is

Narcotics addicts 19 Serum sickness and drug reactions 1~ Rheumatoid arthritis :~ Systemic lupus erythematosis ~~ Hairy-cell leukemia 7 Multiple myeloma '~ Waldenstrom's macroglobulinemia 2~ Thrombocytopenic purpura 2~ Hereditary spherocytosis ~6 Myelophtisic anemia ~~

RARE

Benign tumors ~~ True cysts 7'22'23 Primary sarcoma 7 Metastatic carcinoma of spleen TM

Strangulation by twisting of the pedicle 1'2'3'6

From the Department of Nuclear Medicine, Rambam Medical Center, and the Faculty of Medicine, Technion- Israel Institute of Technology, Haifa, Israel.

Address reprint requests to Dov Front, M.D., Ph.D., Department of Nuclear Medicine. Rambam Medical Center, Haifa 35254, Israel.

�9 1983 by Grune & Stratton, Inc, 0001-2298/83/1303-0011 $01.00/0

Seminars in Nuclear Medicine, Vol, XIII, No. 3 (July), 1983 295

296 GROSHAR, ISRAEL, AND FRONT

RARE (continued) Advanced cardiac failure 1~ Idiopathic nontropical splenomegaly as'26 Hemodialysis patients 1~ Osteopetrosis (marble-bones) 21 Hyperthyroidism 1~ G a m m a heavy chain disease 1~ Behcet's disease 28 Felty's syndrome 29

Fig. 1. Liver-spleen scin- tigraphy in the anterior, right anterior oblique, right lateral, and posterior views. A huge spleen with somewhat de- creased activity as compared to the liver is shown in the midabdomen. The liver is nor- mal.

Diabetes mellitus 12 Familial eosinophilia 3~ Malignant histiocytosis 7 Iron deficiency anemia 2~ Thalassemia 16 Hemoglobin C 16 Primary splenic panhematopenia or neutrope-

nia 1~

REFERENCES

1. Smulewicz J J, Clemett AR: Torsion of the wandering spleen. Dig Dis 20:274, 1975

2. Pollak EW, Tesluk H: Volvulus of the spleen. JAMA 237:469, 1977

3. Gordon DH, Burell MI, Levin DC, et al: Wandering spleen--The radiological and clinical spectrum. Radiology 125:39, 1977

4. Schwartz SI: Editor-in-Chief, Principles of Surgery (ed 3). New York, McGraw-Hill Book Co, Inc, 1979, pp 1394

5. Sabiston DC: Textbook of Surgery. Philadelphia, WB Saunders Co, 1977, pp 1331

6. Isikoff MB, White DW, Diaconis JN: Torsion of the wandering spleen, seen as a migratory abdominal mass. Radiology 123:36, 1977

7. Case Records of the Massachusetts General Hospital: Case 15-1982. N Engl J Med 306:918, 1982

8. Spencer RP: Spleen scanning as a diagnostic tool. JAMA 237:1473, 1977

9. Webb L J, Sherlock S: The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. QJ Med 192:627, t979

10. Wintrobe MM, Lee GR, Boggs DR, et al: Clinical Hematology, ed 8. Philadelphia, Lea & Febiger, 1981, pp 1426-1446

11. Harvey AG, Bordley J, Barondess JA: Differential diagnosis (ed 3). Philadelphia, WB Saunders Co, 1979, pp 378-385

12. Schloesser LL: The diagnostic significance of Sple- nomegaly. Am J Med Sci 245:84, 1963

13. Crane GG: Anaemia in the upper Watut Valley of New Guinea. A study of the effects of altitude and splenome- galy on hemoglobin levels. Med J Aust 1:101, 1973

14. Levitt D, Desai M, Bhagwandeen SB: An investiga- tion into the causes of massive splenomegaly at the University Teaching Hospital, Lusaka, Zambia. E Afr Med J 51:928, 1974

GAMUT: ENLARGEMENT OF THE SPLEEN 297

15. Eichner ER: Splenic function: Normal, too much and too little. Am J Med 66:311, 1979

16. Breitfeld V, Lee RE: Pathology of the spleen in hematologic disease. Surg Clin North Am 55:233, 1975

17. Hermann RE, DeHaven KE, Hawk WA: Splenee- tomy for the diagnosis of splenomegaly. Ann Surg 168:896, 1968

18. Goonewaroene A, Bourke JB, Ferguson R, et al: Spleneetomy for undiagnosed splenomegaly. Br J Surg 66:62, 1979

19. Myers J, Segal R J: Weight of the spleen: II. Range in narcotics addicts. Arch Patho198:36, 1974

20. Williams W J, Beutler E, Erslev A J, el al: Hematolo- gy, ed 2. New York, McGraw-Hill Book Co, ]nc, 1977, pp 950-956

21. Isselbach KJ, Adams RD, Braunwald I, et al: Harri- son's Principles of Internal Medicine, ed 9. McGraw-Hill Book Co, Inc, Kogakusha, 1980, pp 282-283

22. Uflacker R: Cystic lymphangioma of the spleen: A cause of splenomegaly. Br J Radiol 52:148, 1979

23. MacPherson AIS: The spleen: Cysts and tumours. Br J Hosp Med 24:413, 1980

24. Hirst AE, Bullock WK: Metastatic carcinoma of the spleen. Am J Mext Sci 223:414, 1952

25. Robbins SL, Angell M, Kumar V: Basic pathology, ed 3. Philadelphia, WB Saunders Co, 1981, pp 365-366

26. Dacie JV, Galton AG, Gordon-Smith EC, et al: Non- tropical splenomegaly: A follow-up study of ten patients described in 1969. Br J Haematol 38:185, 1978

27. Higgins MR, Grace M, Ulan RA, et al: Anemia in hemodialysis patients. Arch Intern Med 137:172, 1977

28. Kierman T J, Gillan J, Murray JP, et al: Behcet's disease and splenomegaly. Br Med J 11:1341, 1978

29. Spivak JL: Felty's syndrome: An analytical review. Johns Hopkins Mcd J 141:156, 1977

30. Stefanini M, Karaca M: Familial cosinophilia and splenomegalia. Am J Mext Sci 245:91, 1963