Europenn Jourml qf Radiology, 14 (1992) 204-206 G, 1YY2 Elsevier Science Publishers B.V. All rights reserved. 0720-048X/92/$05.00
204
EURRAD
00245
Radiologic features of rhabdoid tumor of the kidney M. Panuel’,
B. Bourliere-Najean
‘, C. Scheiner2, J.-C. Gentet 3, F. Ternier ‘, C. Le Bail l, F. Fame ’ and P. Devred ’
Departments of ‘Pediatric Radiology, ‘Pathology and ‘Pediatric Oncology, CHV Timone Marseille. France (Received 23 July 1991; accepted
Key words: Kidney neoplasm;
Kidney neoplasm,
17 October
CT; Kidney neoplasm,
Introduction Rhabdoid tumor of the kidney (RTK), originally described as a sarcomatous variant of Wilms’ tumor, is now known as an entity with characteristic clinical and pathological features [l-3]. RTK is one of the most aggressive of the childhood solid tumors. The term ‘rhabdoid’ was given because, on light microscopic examination, the tumor cells look like rhabdomyoblasts. Imaging of RTK has only been described in few patients : a review of the literature found only five cases [ 1,4-61. The aim of this paper, by reporting three new cases observed in our institution and reviewed by the same pathologist, is to determine whether some radiographic signs can be retained as specific or highly suggestive of RTK. Case reports Case 1 A 3+year-old female developed a right-upperquadrant abdominal mass and was admitted in 1979 to an Algerian hospital. On IVP, the right kidney appeared ‘mute’. Laparotomy was performed but did not lead to the diagnosis, and the child was referred to our institution 10 months after the onset of the clinical signs. Here, the renal origin of the mass was confirmed. The mass was partially calcified (Fig. 1). There were no lung metastases. A preoperative chemotherapy regimen (actinomycin D and vincristine) and radiation treatment
US study; Computed
tomography,
kidney
(20 Gy) were given without effect on the tumor volume. The child died during surgery 12 months after the onset of the clinical signs. On gross examination, the tumor was partially necrotic, the renal cortex and the medulla were markedly infiltrated by the tumor. Microscopic examination concluded it to be a stage III Wilms’ tumor, but a new study, several years later, led to the diagnosis of RTK. Case
2 A previously healthy Smonth-old female infant developed fever and was referred to our institution in 1980. Chest radiographs demonstrated several parenchymal nodules and bilateral pleural fluid collection. Abdominal sonography (Fig. 2) and CT revealed a central soft-tissue mass of the right kidney with subcapsular fluid collection. There was thrombosis of the inferior vena cava and the collecting system was not opacified during CT. A chemotherapy regimen (actinomycin D and vincristine) was given but stopped because of poor clinical tolerance. A right radical nephrectomy with lymph-node dissection was performed. On cut section, the sinus and the lower pole of the kidney were found to be invaded by the tumor. Microscopic studies showed the rhabdoid nature of this aggressive tumor. Despite postoperative chemotherapy (vincristine), lung metastases did not regress, and bone and brain metastases appeared. The infant died 2 months after the onset of the clinical symptoms. Case
Address for reprints: M. Panuel, M.D., Dpt of Pediatric Radiology, CHU Timone 13385, Marseille Cedex 5, France.
1991)
3 A 5-month-old female infant was referred to our institution in 1988 for macroscopic hematuria. Abdominal sonography and CT showed a right kidney mass
Fig. 2. Longitudinal
sonogram demonstrates
an inhomogeneous
subcapsular fluid collection (Fig. 3a and b). Chest radiograph and bone scintigraphy were normal. A right nephrectomy was performed. On cut section, the tumor had developed in the central part of the kidney (Fig. 3c) and invaded the collecting system. There was no lymphnode involvement. The pathologic diagnosis was RTK. A SIOP 9 protocol (International Society of Pediatric Oncology) with vincristine, epirubicine and actinomycin D, was started, but the infant died 5 months after diagnosis with progressive disease.
with
Discussion RTK is a highly aggressive tumor accounting for 2% of the malignant renal neoplasms entered into the
a
b
mass of the sinus of the right kidney and a pleural fluid collection.
National Wilms’ Tumor Study [ 21. RTK is the least common of the three entities (with anaplastic Wilms’ tumor and clear cell sarcoma of the kidney) considered in the unfavorable histology group of the National Wilms’ Tumor Study [2,3,6]. On the report by Weeks et al. [ 31 of 111 pathological cases, the age at presentation ranges from 0 to 106 months, the mean age is 17 months and the median 11 months. Neonatal cases have been reported [ 3,4]. There is an approximately 1.5 : 1 male/female ratio. Pathological aspects of RTK are distinct [2,3,7,8]. On gross examination, RTK is seen to infiltrate the renal parenchyma without well-defined boundaries, and frequently invades the collecting system. Microscopic studies lead to the diagnosis, showing filamentous cyto-
C
Fig. 3. (a) Longitudinal sonogram shows an echogenic mass occupying the middle part of the right kidney. A subcapsular fluid collection is clearly visible. (b) CT scan after contrast material administration demonstrates a large, poorly enhanced soft-tissue mass compressing anteriorly and posteriorly the renal parenchyma. (c) On longitudinal cut section of the piece of nephrectomy, the mass arises from the middle part of the kidney, invades the sinus and reaches the renal capsule.
206
plasmic inclusions and large nucleoli. Metastases or extrarenal extension are often present at the time of diagnosis. The metastatic routes are hematogeneous or lymphatic to the lung, retroperitoneal lymph nodes, liver, brain, bone and the heart [2,3]. An associated primitive neuroectodermal tumor of the posterior fossa. has been described in approximately 10% of the cases [ 3,4,9]. The intracranial tumor may appear earlier than the renal tumor. Extrarenal rhabdoid tumors have been reported: soft tissues of the limbs, neck, thymus, heart, liver and bladder [7,10-121. Clinical presentation is usually an abdominal mass with, sometimes, gross hematuria. Hypercalcemia related to an inappropriate release of parathormone also has been reported [ 51. Aniridia, Beckwith-Wiedemann syndrome, hemihypertrophy and other conditions associated with increased risk for Wilms’ tumor have not been described with RTK. Among the imaging findings of RTK, heterogeneous pattern is a constant one on sonography, CT or MRI [4,5]. This is related to necrotic and/or hemorrhagic areas within the tumor. At the time of diagnosis, the tumor appears bulky (diameter more than 4 cm). Its rapid growth has been emphasized [4]. The development of RTK in the middle part of the kidney has been noted in three of the five cases found in the literature and in our three cases. A mass arising from the upper or the lower pole has been recorded twice [ 1,5]. Two of our cases had a ‘mute’ kidney, this IVP finding has not yet been reported; that may be related to the invasion of the collecting system and/or the hilar vessels by the tumor. Sisler and Siegel [5] reported two cases with a subcapsular fluid collection as we saw in two cases. These authors also noted an irregular thickening of the renal capsule. Calcifications of RTK and bilateral lesions are possible [5]. The radiographic appearance of RTK may be, however, identical to a typical Wilms’ tumor or another malignant kidney neoplasm such as clear cell sarcoma
or a benign mass such as mesoblastic nephroma (Bolande’s tumor) which occurs during the first months of life [ 61. Despite the few published cases, it seems that a central soft-tissue mass associated with a subcapsular fluid collection may be retained as distinct imaging features of RTK [5]. With a survival rate less than 10% within 2 years of diagnosis [2], RTK has a very poor prognosis despite the use of drugs which are so effective with the other pediatric malignant tumors of the kidney [ 11. References 1 Malik R. Rhabdoid tumor of the kidney. Med Pediatr Oncol 1988; 16: 203-205. 2 Palmer NF, Sutow W. Clinical aspects of the rhabdoid tumor of the kidney: a report of the National Wilms Tumor Study Group. Med Pediatr Oncol 1983; 11: 242-245. 3 Weeks DA, Beckwith JB, Mierau GW, Luckey DW. Rhabdoid tumor of the kidney. Am J Surg Path01 1989; 13: 439-458. 4 Chung CJ, Cammoun D, Munden M. Rhabdoid tumor of the kidney presenting as an abdominal mass in a newborn. Pediatr Radio1 1990; 20: 562-563. Sisler CL, Siegel MJ. Malignant rhabdoid tumor of the kidney: radiologic features. Radiology 1989; 172: 211-212. White KS, Grossman H. Wilms’ and associated renal tumors of childhood. Pediatr Radio1 1991; 21: 81-88. Tsokos M, Kouraklis G, Chandra RS, Bhagavan BS, Triche TJ. Malignant rhabdoid tumor of the kidney and soft tissues. Arch Path01 Lab Med 1989; 113: 115-120. 8 Weeks DA, Beckwith JB, Mierau GW. Rhabdoid tumor of the kidney. Arch Path01 Lab Med 1989; 113: 113-114. 9 Bonnin JM, Rubinstein LJ, Palmer NF, Beckwith JB. The association of embryonnal tumors originating in the kidney and in the brain. Cancer 1984; 54: 2137-2146. 10 Blatt J, Russo P, Taylor S. Extrarenal rhabdoid sarcoma. Med Pediatr Oncol 1986; 14: 221-226. 11 Sotelo-Avila C, Gonzales-Crussi F, de Mello D, Vogler C, Gooch WM, Gale C, Pena R. Renal and extrarenal rhabdoid tumors in children: a clinicopathological study of 14 patients. Semin Diag Path01 1986; 3: 151-163. 12 Harris M, Eyden BP, Joglekar VM. Rhabdoid tumor of the bladder. Histopathology 1987; 11: 1083-1092.
204
EURRAD
00245
Radiologic features of rhabdoid tumor of the kidney M. Panuel’,
B. Bourliere-Najean
‘, C. Scheiner2, J.-C. Gentet 3, F. Ternier ‘, C. Le Bail l, F. Fame ’ and P. Devred ’
Departments of ‘Pediatric Radiology, ‘Pathology and ‘Pediatric Oncology, CHV Timone Marseille. France (Received 23 July 1991; accepted
Key words: Kidney neoplasm;
Kidney neoplasm,
17 October
CT; Kidney neoplasm,
Introduction Rhabdoid tumor of the kidney (RTK), originally described as a sarcomatous variant of Wilms’ tumor, is now known as an entity with characteristic clinical and pathological features [l-3]. RTK is one of the most aggressive of the childhood solid tumors. The term ‘rhabdoid’ was given because, on light microscopic examination, the tumor cells look like rhabdomyoblasts. Imaging of RTK has only been described in few patients : a review of the literature found only five cases [ 1,4-61. The aim of this paper, by reporting three new cases observed in our institution and reviewed by the same pathologist, is to determine whether some radiographic signs can be retained as specific or highly suggestive of RTK. Case reports Case 1 A 3+year-old female developed a right-upperquadrant abdominal mass and was admitted in 1979 to an Algerian hospital. On IVP, the right kidney appeared ‘mute’. Laparotomy was performed but did not lead to the diagnosis, and the child was referred to our institution 10 months after the onset of the clinical signs. Here, the renal origin of the mass was confirmed. The mass was partially calcified (Fig. 1). There were no lung metastases. A preoperative chemotherapy regimen (actinomycin D and vincristine) and radiation treatment
US study; Computed
tomography,
kidney
(20 Gy) were given without effect on the tumor volume. The child died during surgery 12 months after the onset of the clinical signs. On gross examination, the tumor was partially necrotic, the renal cortex and the medulla were markedly infiltrated by the tumor. Microscopic examination concluded it to be a stage III Wilms’ tumor, but a new study, several years later, led to the diagnosis of RTK. Case
2 A previously healthy Smonth-old female infant developed fever and was referred to our institution in 1980. Chest radiographs demonstrated several parenchymal nodules and bilateral pleural fluid collection. Abdominal sonography (Fig. 2) and CT revealed a central soft-tissue mass of the right kidney with subcapsular fluid collection. There was thrombosis of the inferior vena cava and the collecting system was not opacified during CT. A chemotherapy regimen (actinomycin D and vincristine) was given but stopped because of poor clinical tolerance. A right radical nephrectomy with lymph-node dissection was performed. On cut section, the sinus and the lower pole of the kidney were found to be invaded by the tumor. Microscopic studies showed the rhabdoid nature of this aggressive tumor. Despite postoperative chemotherapy (vincristine), lung metastases did not regress, and bone and brain metastases appeared. The infant died 2 months after the onset of the clinical symptoms. Case
Address for reprints: M. Panuel, M.D., Dpt of Pediatric Radiology, CHU Timone 13385, Marseille Cedex 5, France.
1991)
3 A 5-month-old female infant was referred to our institution in 1988 for macroscopic hematuria. Abdominal sonography and CT showed a right kidney mass
Fig. 2. Longitudinal
sonogram demonstrates
an inhomogeneous
subcapsular fluid collection (Fig. 3a and b). Chest radiograph and bone scintigraphy were normal. A right nephrectomy was performed. On cut section, the tumor had developed in the central part of the kidney (Fig. 3c) and invaded the collecting system. There was no lymphnode involvement. The pathologic diagnosis was RTK. A SIOP 9 protocol (International Society of Pediatric Oncology) with vincristine, epirubicine and actinomycin D, was started, but the infant died 5 months after diagnosis with progressive disease.
with
Discussion RTK is a highly aggressive tumor accounting for 2% of the malignant renal neoplasms entered into the
a
b
mass of the sinus of the right kidney and a pleural fluid collection.
National Wilms’ Tumor Study [ 21. RTK is the least common of the three entities (with anaplastic Wilms’ tumor and clear cell sarcoma of the kidney) considered in the unfavorable histology group of the National Wilms’ Tumor Study [2,3,6]. On the report by Weeks et al. [ 31 of 111 pathological cases, the age at presentation ranges from 0 to 106 months, the mean age is 17 months and the median 11 months. Neonatal cases have been reported [ 3,4]. There is an approximately 1.5 : 1 male/female ratio. Pathological aspects of RTK are distinct [2,3,7,8]. On gross examination, RTK is seen to infiltrate the renal parenchyma without well-defined boundaries, and frequently invades the collecting system. Microscopic studies lead to the diagnosis, showing filamentous cyto-
C
Fig. 3. (a) Longitudinal sonogram shows an echogenic mass occupying the middle part of the right kidney. A subcapsular fluid collection is clearly visible. (b) CT scan after contrast material administration demonstrates a large, poorly enhanced soft-tissue mass compressing anteriorly and posteriorly the renal parenchyma. (c) On longitudinal cut section of the piece of nephrectomy, the mass arises from the middle part of the kidney, invades the sinus and reaches the renal capsule.
206
plasmic inclusions and large nucleoli. Metastases or extrarenal extension are often present at the time of diagnosis. The metastatic routes are hematogeneous or lymphatic to the lung, retroperitoneal lymph nodes, liver, brain, bone and the heart [2,3]. An associated primitive neuroectodermal tumor of the posterior fossa. has been described in approximately 10% of the cases [ 3,4,9]. The intracranial tumor may appear earlier than the renal tumor. Extrarenal rhabdoid tumors have been reported: soft tissues of the limbs, neck, thymus, heart, liver and bladder [7,10-121. Clinical presentation is usually an abdominal mass with, sometimes, gross hematuria. Hypercalcemia related to an inappropriate release of parathormone also has been reported [ 51. Aniridia, Beckwith-Wiedemann syndrome, hemihypertrophy and other conditions associated with increased risk for Wilms’ tumor have not been described with RTK. Among the imaging findings of RTK, heterogeneous pattern is a constant one on sonography, CT or MRI [4,5]. This is related to necrotic and/or hemorrhagic areas within the tumor. At the time of diagnosis, the tumor appears bulky (diameter more than 4 cm). Its rapid growth has been emphasized [4]. The development of RTK in the middle part of the kidney has been noted in three of the five cases found in the literature and in our three cases. A mass arising from the upper or the lower pole has been recorded twice [ 1,5]. Two of our cases had a ‘mute’ kidney, this IVP finding has not yet been reported; that may be related to the invasion of the collecting system and/or the hilar vessels by the tumor. Sisler and Siegel [5] reported two cases with a subcapsular fluid collection as we saw in two cases. These authors also noted an irregular thickening of the renal capsule. Calcifications of RTK and bilateral lesions are possible [5]. The radiographic appearance of RTK may be, however, identical to a typical Wilms’ tumor or another malignant kidney neoplasm such as clear cell sarcoma
or a benign mass such as mesoblastic nephroma (Bolande’s tumor) which occurs during the first months of life [ 61. Despite the few published cases, it seems that a central soft-tissue mass associated with a subcapsular fluid collection may be retained as distinct imaging features of RTK [5]. With a survival rate less than 10% within 2 years of diagnosis [2], RTK has a very poor prognosis despite the use of drugs which are so effective with the other pediatric malignant tumors of the kidney [ 11. References 1 Malik R. Rhabdoid tumor of the kidney. Med Pediatr Oncol 1988; 16: 203-205. 2 Palmer NF, Sutow W. Clinical aspects of the rhabdoid tumor of the kidney: a report of the National Wilms Tumor Study Group. Med Pediatr Oncol 1983; 11: 242-245. 3 Weeks DA, Beckwith JB, Mierau GW, Luckey DW. Rhabdoid tumor of the kidney. Am J Surg Path01 1989; 13: 439-458. 4 Chung CJ, Cammoun D, Munden M. Rhabdoid tumor of the kidney presenting as an abdominal mass in a newborn. Pediatr Radio1 1990; 20: 562-563. Sisler CL, Siegel MJ. Malignant rhabdoid tumor of the kidney: radiologic features. Radiology 1989; 172: 211-212. White KS, Grossman H. Wilms’ and associated renal tumors of childhood. Pediatr Radio1 1991; 21: 81-88. Tsokos M, Kouraklis G, Chandra RS, Bhagavan BS, Triche TJ. Malignant rhabdoid tumor of the kidney and soft tissues. Arch Path01 Lab Med 1989; 113: 115-120. 8 Weeks DA, Beckwith JB, Mierau GW. Rhabdoid tumor of the kidney. Arch Path01 Lab Med 1989; 113: 113-114. 9 Bonnin JM, Rubinstein LJ, Palmer NF, Beckwith JB. The association of embryonnal tumors originating in the kidney and in the brain. Cancer 1984; 54: 2137-2146. 10 Blatt J, Russo P, Taylor S. Extrarenal rhabdoid sarcoma. Med Pediatr Oncol 1986; 14: 221-226. 11 Sotelo-Avila C, Gonzales-Crussi F, de Mello D, Vogler C, Gooch WM, Gale C, Pena R. Renal and extrarenal rhabdoid tumors in children: a clinicopathological study of 14 patients. Semin Diag Path01 1986; 3: 151-163. 12 Harris M, Eyden BP, Joglekar VM. Rhabdoid tumor of the bladder. Histopathology 1987; 11: 1083-1092.
