Diagnostic Radiology
Splenic Cystic Lymphangiomatosis: An Unusual Cause of Massive Splenomegaly 1 Robert J. Tuttle, M.D., F.R.C.P.(C), and John A. Minielly, M.D., M.Sc., F.R.C.P.(C) Two unusual cases of massively enlarged spleens occupied by numerous lymphangiomatous cysts are described. Both angiography and pathology confirm the pathognomonic appearance. INDEX TERMS:
Spleen, neoplasms. (Spleen, other cyst, 7[75].3129)
Radiology 126:47-48, January 1978
been advocated as the most informative and reliable means of discovering suspected mass lesions (2); a recent article (3) described an unusual study of a massively en-
are rare lesions seldom diagnosed prior to surgery. They have been classified into two large groups-true and false cysts (1). Angiography has
S
PLENIC CYSTS
Fig. 1. Fig. 2.
Splenic angiogram (arterial phase) demonstrating marked stretching of intrasplenic vasculature. Splenic angiogram (tissue phase) demonstrating "swiss cheese" appearance reminiscent of adult polycystic kidney.
1 From the Departments of Radiology (R.J.T.) and Pathology (J.A.M.), S1. Joseph's Hospital, Hamilton, Ontario. Accepted for publication in July 1977. wjw
47
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ROBERT J. TUTTLE AND JOHN
larged spleen felt to be pathognomonic of splenic Iymphangiomatosis. The following two cases are presented as confirmation. CASE REPORTS CASE I: A 24-year-old woman was found to have splenomegaly on a routine visit to her family physician; he had seen her at six-month intervals in the previous six years for contraceptive advice and had not noted any splenic enlargement. A functional enquiry and physical examination were entirely normal, as were the routine laboratory studies, including hemoglobin, white cell count, differential, and sedimentation rate. No further investigation or therapy was initiated at that time. Over the next two years, increasing splenic enlargement was noted and the patient began to complain of a "scraping" discomfort in the left-upper quadrant and an increase in girth. These symptoms prompted referral to our hospital. Physical examination was again normal except for the massively enlarged spleen, which extended to the iliac crest and across the midline. Laboratory studies, including complete blood count, sedimentation rate, SMA 12 evaluation, LE preparation, antinuclear factor, coagulation studies, acid phosphatase levels, and infectious mononucleosis spot test, were all normal. Angiography confirmed massive splenomegaly with stretched intrasplenic arteries and myriads of lucent spheroid defects varying from pinhead size to 1 cm in diameter (Figs. 1 and 2), simulating the angiographic appearance of advanced renal polycystic disease. A diagnosis of advanced multi cystic disease of the spleen was suggested and surgical exploration arranged; the spleen was removed and the patient recovered.
A. MINIELLY
January 1978
CASE II: One of the authors was sent a slide of an unusual spleen that had been removed from a 59-year-old woman with a history of splenic enlargement dating back to 1956. She had had several episodes of gastrointestinal bleeding during that period, some of them massive. The enlarged nodular spleen was removed, as well as a small hemangioma of the stomach. No other abnormalities were noted. Pathology: Pathologically, both spleens showed virtually no significant gross or microscopic differences. In the first case (Fig. 3) it weighed 1,500 g and was almost completely occupied by multiple thin-walled cysts (up to 2 cm in diameter) containing a clear watery fluid. The cysts were lined by flattened endothelial cells and filled with a proteinaceous material common to multiple lymphangiomata. In the second case the spleen weighed 1,600 g; in the gastric submucosa, there was a 2-cm ulcerated vascular malformation containing both hemangiomatous and lymphangiomatous elements.
DISCUSSION
Lymphangiomatous cysts of the spleen are usually few in number, in contrast to these two cases consisting of almost complete replacement of the spleen by numerous cysts varying in size from micro- to macroscopic. The absence of major lymphatic abnormalities elsewhere, with the exception of the small hemangioma of the stomach, indicates that this is an unusual cystic lymphangiomatous process localized to the spleen and should be considered in the differential diagnosis of massive splenomegaly; the angiographic pattern is characteristic and identical to the case reported by Shanser et al. (3) and should allow the correct preoperative diagnosis. REFERENCES 1. McClure RD, Altemeier WA: Cysts of the spleen. Ann Surg 116:98-102, Jul 1942 2. Rosch J: Tumors of the spleen: the value of selective arteriography. Clin RadloI17:183-190, Apr 1966 3. Shanser JD, Moss AA, Clark RE, et al: Angiographic evaluation of cystic lesions of the spleen. Am J RoentgenoI119:166-174, Sep 1973
Fig. 3.
Cut surface of massively enlarged "polycystic" spleen.
Department of Radiology S1. Joseph's Hospital Hamilton, Ontario L8NIY4 Canada
Splenic Cystic Lymphangiomatosis: An Unusual Cause of Massive Splenomegaly 1 Robert J. Tuttle, M.D., F.R.C.P.(C), and John A. Minielly, M.D., M.Sc., F.R.C.P.(C) Two unusual cases of massively enlarged spleens occupied by numerous lymphangiomatous cysts are described. Both angiography and pathology confirm the pathognomonic appearance. INDEX TERMS:
Spleen, neoplasms. (Spleen, other cyst, 7[75].3129)
Radiology 126:47-48, January 1978
been advocated as the most informative and reliable means of discovering suspected mass lesions (2); a recent article (3) described an unusual study of a massively en-
are rare lesions seldom diagnosed prior to surgery. They have been classified into two large groups-true and false cysts (1). Angiography has
S
PLENIC CYSTS
Fig. 1. Fig. 2.
Splenic angiogram (arterial phase) demonstrating marked stretching of intrasplenic vasculature. Splenic angiogram (tissue phase) demonstrating "swiss cheese" appearance reminiscent of adult polycystic kidney.
1 From the Departments of Radiology (R.J.T.) and Pathology (J.A.M.), S1. Joseph's Hospital, Hamilton, Ontario. Accepted for publication in July 1977. wjw
47
48
ROBERT J. TUTTLE AND JOHN
larged spleen felt to be pathognomonic of splenic Iymphangiomatosis. The following two cases are presented as confirmation. CASE REPORTS CASE I: A 24-year-old woman was found to have splenomegaly on a routine visit to her family physician; he had seen her at six-month intervals in the previous six years for contraceptive advice and had not noted any splenic enlargement. A functional enquiry and physical examination were entirely normal, as were the routine laboratory studies, including hemoglobin, white cell count, differential, and sedimentation rate. No further investigation or therapy was initiated at that time. Over the next two years, increasing splenic enlargement was noted and the patient began to complain of a "scraping" discomfort in the left-upper quadrant and an increase in girth. These symptoms prompted referral to our hospital. Physical examination was again normal except for the massively enlarged spleen, which extended to the iliac crest and across the midline. Laboratory studies, including complete blood count, sedimentation rate, SMA 12 evaluation, LE preparation, antinuclear factor, coagulation studies, acid phosphatase levels, and infectious mononucleosis spot test, were all normal. Angiography confirmed massive splenomegaly with stretched intrasplenic arteries and myriads of lucent spheroid defects varying from pinhead size to 1 cm in diameter (Figs. 1 and 2), simulating the angiographic appearance of advanced renal polycystic disease. A diagnosis of advanced multi cystic disease of the spleen was suggested and surgical exploration arranged; the spleen was removed and the patient recovered.
A. MINIELLY
January 1978
CASE II: One of the authors was sent a slide of an unusual spleen that had been removed from a 59-year-old woman with a history of splenic enlargement dating back to 1956. She had had several episodes of gastrointestinal bleeding during that period, some of them massive. The enlarged nodular spleen was removed, as well as a small hemangioma of the stomach. No other abnormalities were noted. Pathology: Pathologically, both spleens showed virtually no significant gross or microscopic differences. In the first case (Fig. 3) it weighed 1,500 g and was almost completely occupied by multiple thin-walled cysts (up to 2 cm in diameter) containing a clear watery fluid. The cysts were lined by flattened endothelial cells and filled with a proteinaceous material common to multiple lymphangiomata. In the second case the spleen weighed 1,600 g; in the gastric submucosa, there was a 2-cm ulcerated vascular malformation containing both hemangiomatous and lymphangiomatous elements.
DISCUSSION
Lymphangiomatous cysts of the spleen are usually few in number, in contrast to these two cases consisting of almost complete replacement of the spleen by numerous cysts varying in size from micro- to macroscopic. The absence of major lymphatic abnormalities elsewhere, with the exception of the small hemangioma of the stomach, indicates that this is an unusual cystic lymphangiomatous process localized to the spleen and should be considered in the differential diagnosis of massive splenomegaly; the angiographic pattern is characteristic and identical to the case reported by Shanser et al. (3) and should allow the correct preoperative diagnosis. REFERENCES 1. McClure RD, Altemeier WA: Cysts of the spleen. Ann Surg 116:98-102, Jul 1942 2. Rosch J: Tumors of the spleen: the value of selective arteriography. Clin RadloI17:183-190, Apr 1966 3. Shanser JD, Moss AA, Clark RE, et al: Angiographic evaluation of cystic lesions of the spleen. Am J RoentgenoI119:166-174, Sep 1973
Fig. 3.
Cut surface of massively enlarged "polycystic" spleen.
Department of Radiology S1. Joseph's Hospital Hamilton, Ontario L8NIY4 Canada
