European Journal of Radiology, 10 (1990) 140-142 Elsevier
140
EURRAD
00023
Multilocular renal cyst associated with transposition of the inferior vena cava and malrotation of the bowel H.J.Ph. Vogel ‘, T.H.M. Falke ‘, J.H.M. Wondergem ‘, H. Beerman 2 and F. Eulderink2 Departments of ‘DiagnosticRadiology and 2Pathology,UniversityMedical Center, Leiden. The Netherlands (Received 29 August 1989; revised version received 10 October
Key words: Renal cyst, multilocular;
Intestine,
malrotation;
1989; accepted 23 October
1989)
Inferior vena cava, transposition
Abstract
A case is presented of an adult male patient with a heavily calcified multilocular renal cyst (Perlmann’s tumor), transposition of the inferior vena cava and reversed rotation (malrotation) of the bowel. Because of the synchronous embryological development of the forementioned structures, a possible congenital etiology of the multilocular renal cyst is suggested.
Introduction Multilocular renal cyst has variably been designated as a malformation (segmental unilateral dysplasia) or a true neoplasm [ l-41. The main cause of the uncertainty about the true histogenesis of multilocular renal cysts
Fig. 1. CT demonstrates a heavily calcified multilocular lesion (6 x 6 x 7 cm) of the left kidney (large arrow) and transposition of the vena cava inferior (small arrow). Address for reprints: H.J.Ph. Vogel, Lauwerbes 3,2318 AT Leiden, The Netherlands. 0720-048X/90/$03.50
0 1990 Elsevier Science Publishers
are the microscopical findings that may vary from region to region within the same lesion or between lesions [5]. The lack of reported cases in the years between childhood and adulthood is an argument in favour of an acquired condition in adult cases. In this case report we will describe an adult patient with a unilateral heavily calcified multilocular cyst of the left kidney, transposition of the inferior vena cava and
Fig. 2. CT slice at the level of the position of the small bowel in the left and the colon on the right. Note the inferior (small
B.V. (Biomedical
Division)
umbilicus demonstrates malperitoneal cavity (large arrow) left position of the vena cava arrow).
141
Fig. 3. Bisected kidney with multilocular
cyst.
reversed rotation (malrotation) of the bowel [ 121.These multiple developmental errors may have a common embryological denominator suggesting that at least some of the adult multiloculated renal cysts represent congenital malformations. Case report
A healthy 57-year-old Caucasian male was admitted because of difficulty and increased frequency of micturation. He did not report incontinence or symptoms of urinary infection. Physical examination revealed no abnormalities and the laboratory tests were within normal limits. Excretory urography showed (in addition to an enlarged prostate) a heavily (curvilinear) calcified mass in the interpolar area of the left kidney. Abdominal gray
Fig. 4. Septum
with calcified plate (arrows). Haematoxylin eosin, original magnification x 80.
and
scale ultrasound showed the mass to be partially cystic. Subsequently, the patient was examined on a Philips TomoscanTM 350 with lo-mm-thick adjacent slices. Prior to the study 8 cc TClCbrix350@ (meglumine ioxitalamate containing 17.5 g iodine/50 ml) diluted in 500 cc water was administered orally to opacify stomach and small bowel. The CT examination was repeated after intravenous injection of 100 cc TClebrix 350@. The CT examination revealed a 6 x 6 x 7 cm nonenhancing calcified mass in the interpolar area of the left kidney, transposition of the vena cava inferior and malrotation of the bowel (Figs. 1 and 2). A left sided nephrectomy was performed. The resected specimen contained a kidney (11 x 5 x 4 cm) from the lateral surface of which a greyish-white, focally yellow multicystic mass of 7 x 7 x 5 cm protruded (Fig. 3). The kidney and this mass could easily be removed from the surrounding perinephric fat. The cysts contained clear, yellow, watery fluid. The septa between the cysts were fibrous and showed plates of calcification (Fig. 4); they were up to 2 mm thick. Only focally, a flat epithelial lining was found. There was a small area of large, clear epithelial cells with round nuclei and prominent nucleoli (Fig. 5). These cells showed some anisonucleosis and were lying in nests; no tubules were formed. This epithelium reminded of a cortical adenoma; the size of the above-mentioned area was too small (about 5 mm) to suggest renal cell carcinoma [6]. A diagnosis of multilocular renal cyst with calcification of septa and a small area with abnormal epithelium was made. Discussion
Our patient had a multiloculated cyst with heavily calcified septa which is most consistent with the original
Fig. 5. Focus
of clear epithelial cells. Haematoxylin magnification X 200.
and eosin,
142
case described by Perlmann [6,7]. From an imaging point of view, knowledge of the entity despite its variety and its morphological manifestations is important, because the findings on CT may suggest the correct pre-operative diagnosis, as in our case, thereby permitting the option of a partial rather than a total nephrectomy. Although CT cannot differentiate between a benign multilocular cystic nephroma and a multicystic carcinoma, the extensive calcifications are indicative of a multilocular renal cyst. The large variety of the microscopic findings in multilocular renal cysts has been a source of controversy on etiology and nomenclature of this entity. In the past the lesion in its different varieties has been described under the term Perlmann’s tumor, cystadenoma, cystic lymphangioma, cystic hamartoma, benign multilocular cystic nephroma, cystic Wihns tumor, segmental polycystic kidney and segmental multicystic kidney. Nowadays, the term multiloculated renal cyst is widely accepted and the above-mentioned synonyms are best deleted from the radiologic and pathologic terminology [6]. Multiloculated renal cysts are generally defined as segmental unilateral multicystic lesions which do not communicate with the renal pelvis. At a microscopical level, the abnormality is characterized by fibrous stroma, and sometimes primitive mesenchymal components. The presence of abnormal renal tissue in capsules and septa may be misinterpreted as carcinoma [8]. Focal areas with a malignant aspect, resembling nephroblastoma or renal cell carcinoma are only exceptionally reported [ 91. To the best of our knowledge, in adults the multiloculated cyst has never previously been described in association with other congenital anomalies. Our patient is unique in that the lesion was found simultaneously with transposition of the inferior vena cava and malrotation of the bowel. It has been proposed [ 31 that premature cessation of nephron-branching leads to the conversion of the ampullary portions of renal tubules into cysts and the consequent development of generalized or segmental renal multicystic dysplasia. In relation to the case presented in this paper it is striking that this nephron branching occurs in the same period of the embryogenesis (the 6-7th fetal week) as the first phase of bowel rotation in the physiological umbilical hernial sac and
the regression of the (sub)cardinal veins [ 10,111. Although the observed congenital malformations and the renal lesion may be coincidental, the findings of multiple developmental errors, that must have occurred in the same stage of development of the embryo, suggests that at least in this case the lesion could represent a congenital malformation, originally suggested by Robinson [ 131, rather than an acquired abnormality. The enlargement of this malformation since birth may be caused by enlargement of the cysts rather than by increase of their number, analogous to what has been made probable in the adult (Potter III) type of polycystic kidney [ 141.
References 1 Daughtridge TG. Segmental multicystic renal dysplasia. J Can Assoc Radio1 1975; 26: 149-153. 2 Johnson DE, Ayala AG, Medellin H, Wibur J. Multilocular renal cystic disease in children. J Urol 1973; 109: 101-103. 3 Osathanondh V, Potter EL. Pathogenesis of polycystic kidneys. Type 2 due to inhibition of ampullary activity. Arch Path01 1964; 77: 474-484. 4 Powell T, Shackman R, Johnson HD. Multilocular cysts of the kidney. Br J Urol 1951; 23: 142-152. 5 Filmer RB, Taxy JB. Cysts of the kidney, renal dysplasia, and renal hypoplasia. In: Kelalis PP, King LR, eds. Clinical pediatric urology. Philadelphia: Saunders, 1976; 695-699. 6 Banner MP, Pollack HM, Chatten J, Witzleben C. Multilocular renal cysts: radiologic-pathologic correlation. AJR 1981; 136: 239-247. 7 Perlmann S. Ueber einen Fall von Lymphangioma Cysticum der Niere. Virchows Arch 1928; 268: 524-535. 8 Dobben G. Benign adenomatous polycystic kidney tumor, (Perlmann’s tumor). Radiology 1961; 76: 100-102. 9 Gal10 GE, Penchansky L. Cystic nephroma. Cancer 1977; 39: 1322-1327. 10 Royal SA, Callen PW. CT Evaluation of anomalies of the inferior vena cava and left renal vein. AJR 1979; 132: 759-763. 11 Hamilton WJ, Boyd JD, Mossman HW. Human embryology, 4th edn. London: The Macmillan Press, 1976. 12 Estrada RL. Anomalies of intestinal rotation and fixation (including mesentericoparietal hernias). Springfield: Thomas, 1958. 13 Robinson GL. Perlmann’s tumour of the kidney. Br J Surg 1957; 44: 620-623. 14 Eulderink F, Hogewind BL. Renal cysts in premature children. Occurrence in a family with polycystic kidney disease. Arch Path01 Lab Med 1978; 102: 592-595.
140
EURRAD
00023
Multilocular renal cyst associated with transposition of the inferior vena cava and malrotation of the bowel H.J.Ph. Vogel ‘, T.H.M. Falke ‘, J.H.M. Wondergem ‘, H. Beerman 2 and F. Eulderink2 Departments of ‘DiagnosticRadiology and 2Pathology,UniversityMedical Center, Leiden. The Netherlands (Received 29 August 1989; revised version received 10 October
Key words: Renal cyst, multilocular;
Intestine,
malrotation;
1989; accepted 23 October
1989)
Inferior vena cava, transposition
Abstract
A case is presented of an adult male patient with a heavily calcified multilocular renal cyst (Perlmann’s tumor), transposition of the inferior vena cava and reversed rotation (malrotation) of the bowel. Because of the synchronous embryological development of the forementioned structures, a possible congenital etiology of the multilocular renal cyst is suggested.
Introduction Multilocular renal cyst has variably been designated as a malformation (segmental unilateral dysplasia) or a true neoplasm [ l-41. The main cause of the uncertainty about the true histogenesis of multilocular renal cysts
Fig. 1. CT demonstrates a heavily calcified multilocular lesion (6 x 6 x 7 cm) of the left kidney (large arrow) and transposition of the vena cava inferior (small arrow). Address for reprints: H.J.Ph. Vogel, Lauwerbes 3,2318 AT Leiden, The Netherlands. 0720-048X/90/$03.50
0 1990 Elsevier Science Publishers
are the microscopical findings that may vary from region to region within the same lesion or between lesions [5]. The lack of reported cases in the years between childhood and adulthood is an argument in favour of an acquired condition in adult cases. In this case report we will describe an adult patient with a unilateral heavily calcified multilocular cyst of the left kidney, transposition of the inferior vena cava and
Fig. 2. CT slice at the level of the position of the small bowel in the left and the colon on the right. Note the inferior (small
B.V. (Biomedical
Division)
umbilicus demonstrates malperitoneal cavity (large arrow) left position of the vena cava arrow).
141
Fig. 3. Bisected kidney with multilocular
cyst.
reversed rotation (malrotation) of the bowel [ 121.These multiple developmental errors may have a common embryological denominator suggesting that at least some of the adult multiloculated renal cysts represent congenital malformations. Case report
A healthy 57-year-old Caucasian male was admitted because of difficulty and increased frequency of micturation. He did not report incontinence or symptoms of urinary infection. Physical examination revealed no abnormalities and the laboratory tests were within normal limits. Excretory urography showed (in addition to an enlarged prostate) a heavily (curvilinear) calcified mass in the interpolar area of the left kidney. Abdominal gray
Fig. 4. Septum
with calcified plate (arrows). Haematoxylin eosin, original magnification x 80.
and
scale ultrasound showed the mass to be partially cystic. Subsequently, the patient was examined on a Philips TomoscanTM 350 with lo-mm-thick adjacent slices. Prior to the study 8 cc TClCbrix350@ (meglumine ioxitalamate containing 17.5 g iodine/50 ml) diluted in 500 cc water was administered orally to opacify stomach and small bowel. The CT examination was repeated after intravenous injection of 100 cc TClebrix 350@. The CT examination revealed a 6 x 6 x 7 cm nonenhancing calcified mass in the interpolar area of the left kidney, transposition of the vena cava inferior and malrotation of the bowel (Figs. 1 and 2). A left sided nephrectomy was performed. The resected specimen contained a kidney (11 x 5 x 4 cm) from the lateral surface of which a greyish-white, focally yellow multicystic mass of 7 x 7 x 5 cm protruded (Fig. 3). The kidney and this mass could easily be removed from the surrounding perinephric fat. The cysts contained clear, yellow, watery fluid. The septa between the cysts were fibrous and showed plates of calcification (Fig. 4); they were up to 2 mm thick. Only focally, a flat epithelial lining was found. There was a small area of large, clear epithelial cells with round nuclei and prominent nucleoli (Fig. 5). These cells showed some anisonucleosis and were lying in nests; no tubules were formed. This epithelium reminded of a cortical adenoma; the size of the above-mentioned area was too small (about 5 mm) to suggest renal cell carcinoma [6]. A diagnosis of multilocular renal cyst with calcification of septa and a small area with abnormal epithelium was made. Discussion
Our patient had a multiloculated cyst with heavily calcified septa which is most consistent with the original
Fig. 5. Focus
of clear epithelial cells. Haematoxylin magnification X 200.
and eosin,
142
case described by Perlmann [6,7]. From an imaging point of view, knowledge of the entity despite its variety and its morphological manifestations is important, because the findings on CT may suggest the correct pre-operative diagnosis, as in our case, thereby permitting the option of a partial rather than a total nephrectomy. Although CT cannot differentiate between a benign multilocular cystic nephroma and a multicystic carcinoma, the extensive calcifications are indicative of a multilocular renal cyst. The large variety of the microscopic findings in multilocular renal cysts has been a source of controversy on etiology and nomenclature of this entity. In the past the lesion in its different varieties has been described under the term Perlmann’s tumor, cystadenoma, cystic lymphangioma, cystic hamartoma, benign multilocular cystic nephroma, cystic Wihns tumor, segmental polycystic kidney and segmental multicystic kidney. Nowadays, the term multiloculated renal cyst is widely accepted and the above-mentioned synonyms are best deleted from the radiologic and pathologic terminology [6]. Multiloculated renal cysts are generally defined as segmental unilateral multicystic lesions which do not communicate with the renal pelvis. At a microscopical level, the abnormality is characterized by fibrous stroma, and sometimes primitive mesenchymal components. The presence of abnormal renal tissue in capsules and septa may be misinterpreted as carcinoma [8]. Focal areas with a malignant aspect, resembling nephroblastoma or renal cell carcinoma are only exceptionally reported [ 91. To the best of our knowledge, in adults the multiloculated cyst has never previously been described in association with other congenital anomalies. Our patient is unique in that the lesion was found simultaneously with transposition of the inferior vena cava and malrotation of the bowel. It has been proposed [ 31 that premature cessation of nephron-branching leads to the conversion of the ampullary portions of renal tubules into cysts and the consequent development of generalized or segmental renal multicystic dysplasia. In relation to the case presented in this paper it is striking that this nephron branching occurs in the same period of the embryogenesis (the 6-7th fetal week) as the first phase of bowel rotation in the physiological umbilical hernial sac and
the regression of the (sub)cardinal veins [ 10,111. Although the observed congenital malformations and the renal lesion may be coincidental, the findings of multiple developmental errors, that must have occurred in the same stage of development of the embryo, suggests that at least in this case the lesion could represent a congenital malformation, originally suggested by Robinson [ 131, rather than an acquired abnormality. The enlargement of this malformation since birth may be caused by enlargement of the cysts rather than by increase of their number, analogous to what has been made probable in the adult (Potter III) type of polycystic kidney [ 141.
References 1 Daughtridge TG. Segmental multicystic renal dysplasia. J Can Assoc Radio1 1975; 26: 149-153. 2 Johnson DE, Ayala AG, Medellin H, Wibur J. Multilocular renal cystic disease in children. J Urol 1973; 109: 101-103. 3 Osathanondh V, Potter EL. Pathogenesis of polycystic kidneys. Type 2 due to inhibition of ampullary activity. Arch Path01 1964; 77: 474-484. 4 Powell T, Shackman R, Johnson HD. Multilocular cysts of the kidney. Br J Urol 1951; 23: 142-152. 5 Filmer RB, Taxy JB. Cysts of the kidney, renal dysplasia, and renal hypoplasia. In: Kelalis PP, King LR, eds. Clinical pediatric urology. Philadelphia: Saunders, 1976; 695-699. 6 Banner MP, Pollack HM, Chatten J, Witzleben C. Multilocular renal cysts: radiologic-pathologic correlation. AJR 1981; 136: 239-247. 7 Perlmann S. Ueber einen Fall von Lymphangioma Cysticum der Niere. Virchows Arch 1928; 268: 524-535. 8 Dobben G. Benign adenomatous polycystic kidney tumor, (Perlmann’s tumor). Radiology 1961; 76: 100-102. 9 Gal10 GE, Penchansky L. Cystic nephroma. Cancer 1977; 39: 1322-1327. 10 Royal SA, Callen PW. CT Evaluation of anomalies of the inferior vena cava and left renal vein. AJR 1979; 132: 759-763. 11 Hamilton WJ, Boyd JD, Mossman HW. Human embryology, 4th edn. London: The Macmillan Press, 1976. 12 Estrada RL. Anomalies of intestinal rotation and fixation (including mesentericoparietal hernias). Springfield: Thomas, 1958. 13 Robinson GL. Perlmann’s tumour of the kidney. Br J Surg 1957; 44: 620-623. 14 Eulderink F, Hogewind BL. Renal cysts in premature children. Occurrence in a family with polycystic kidney disease. Arch Path01 Lab Med 1978; 102: 592-595.
