Solitary By Wuu-Tay
Chang,
Spinal Cord Metastasis Hou-Chuan
Chen, Hai-Chyi
Peng, Dar-Yu Yang, and Cecil Guang-Shiung
Taichung, 0 Wilms’ tumor metastasis to the spinal cord is very rare at any age. We recently encountered a case of a 4-year-old girl, who had a solitary lumbar spinal cord metastasis about 1 year following the initial nephrectomy and an incomplete course of chemotherapy for a stage I Wilms’ tumor. The patient recovered uneventfully after decompressive laminectomy. removal of the extradural spinal cord tumor, adjuvant chemotherapy, and radiotherapy according to the National Wilms’ Tumor Study (NWTSI-IV protocol. @ 1990 by W.B. Saunders Company. INDEX WORDS: Wilms’ tumor; spinal cord metastasis.
W
ILMS’ TUMOR is an embryonal renal neoplasm that may spread to the lymph nodes, lung, liver, and bone’ with an incidence of metastasis around 1O%.2Spinal cord metastasis is rarely reported in the literature.3” Epidural involvement usually occurs with other evidence of spreading.3 Solitary spinal cord metastasis without concomitant involvement to other organs is extremely rare.4 We report a case of solitary spinal cord metastasis without involvement of the lung, liver, bone, or other organs. CASE
REPORT
In November 1986, this 4-year-old girl was referred to Veterans General Hospital-Taichung for evaluation of a progressively eniarging abdominal mass. Physical examination showed a 8 x 10 cm well-defined mass over the left upper quadrant of the abdomen. Plain film of the abdomen showed a big soft tissue mass on the left side. Sonogram of the abdomen showed multiple cystic components in the solid tumor, and the outline of the left kidney was indistinct. Intravenous pyelogram disclosed that the tumor arose from the left kidney, causing distortion and dilatation of the left collecting system. The right kidney had normal function and size. Laboratory data were within normal limits. Explorations with left nephrectomy and periaortic lymph node dissection were performed on November 28, 1986. A huge solitary, homogeneous, well-capsulated mass with central necrosis, about 16 x 11 x 7 cm in size and 650 g in weight, was removed. A tumor thrombus, 1 x 1 x 2 cm in size, within the left renal vein near the hilum was extracted. The pathological finding was nephroblastoma (Wilms’ tumor) with a tumor thrombus in the renal vein. There was no renal capsule invasion; lymph nodes, ureter, and renal vein were also free of tumor tissue. Chemotherapy with vincristine and actinomycin-D was instituted for this stage I Wilms’ tumor accord-
of Wilms’ Tumor Chang
Taiwan
ing to the National Wilms’ Tumor Study (NWTS)-III protocol. Unfortunately, follow-up of the patient was lost after two courses of actinomycin-D every 6 weeks and eight doses of vincristine weekly for 8 weeks. Nearly 1 year after primary surgery, she suffered from migrating pain over both legs, and was admitted again under the impression of tumor recurrence. Physical examination showed a soft and flat abdomen without palpable mass. Both lower extremities were free movable without local inflammation. Laboratory data showed lactic dehydrogenase 273 U/L, alkaline phosphatase 302 U/L, and cholesterol 253 mg/dL. Urinalysis was normal. The chest x-ray showed no metastatic nodule. The whole body bone scan also showed no evidence of metastasis. Chemotherapy with actinomycin-D and vincrinstine, according to NWTS-III protocol, was started after admission. The leg pain subsided gradually. However, she developed acute urinary retention on the fifth hospital day, followed by weakness of both lower legs and obstipation. Neurological examination demonstrated absence of deep tendon reflex in the right leg. A lumbar puncture was performed and the spinal fluid showed normal biochemical reactions and cellularity. Filling defect between the 2nd and 3rd lumbar spine was discolosed by myelogram. Concomitant computed tomography (CT) scan of the L-spine also showed a soft tissue mass in the spinal canal (Figs 1 and 2). Decompressive laminectomy on Ll-3 and removal of the tumor were performed on December 2, 1987. A tumor about 2.4 x 2.2 x 0.6 cm was found in the extradural space posteriorly, which caused external compression of the spinal cord posteriorly and laterally. The pathological report was metastatic nephroblastoma, with a histology similar to that of the primary tumor (Figs 3 and 4). The postoperative course was rather smooth. Short-term steroid therapy (betamethasone 2 mg every 12 hours for 3 days) was given. Chemotherapy according to the NWTS-IV protocol and radiotherapy of 3,000 cGy to the tumor bed and L-spine were instituted. The muscle power of both legs was gradually restored, the gait improved, and the bladder and bowel function were recovered.
DISCUSSION
With the modern therapy of multimodality, Wilms’ tumor is potentially curable in most patients.’ However, Lemerle et al2 reported a recurrent rate about 54% with metastastatic rate of 10%. The possibility of tumor recurrence must be always kept in mind, especially when unusual symptoms and signs present or in high-risk patients with unfavorable histology, positive lymph nodes, tumor weight over 250 g, or aged over 2 276
From the Division of Pediatric Surgery, Department of Surgery, Veterans General Hospital, Taichung, Taiwan, Republic of China. Address reprint requests to Wuu-Tay Chang. MD, Division of Pediatric Surgery, Department of Surgery, Veterans General Hospital, Taichung, 40705 Taiwan, Republic of China. 8 1990 by W.B. Saunders Company. OOZZ-3468/90/250S-0021$03.00/0
The clinical symptoms and signs of spinal cord compression are variable. In general, back pain or radicular pain is probably the earliest and most common sign, followed by motor weakness, sensory loss, and anatomic dysfunction.’ In this patient, radicular pain was noted initially, followed by motor weakness
550
Jour& of
Pediatric Surgery,
Vol 25, No 5 (May), 1990: pp 550-552
SPINAL CORD METASTASIS OF WILMS’ TUMOR
Fig 3. The histology from left Wilma’ tumor showed blastema and atroma component compressing the normal ~renal structure. W, Wilms’ tumor; R, renal structure. &l&E, original magnification X63.1
and bladder and bowel dysfunction, but without sensory impairment. Several different mechanisms are implicated in the pathogenesis of spinal cord metastasis, including direct extension from a paravertebral tumor mass, hematogenous spreading through collateral circulation of the paravertebral venous plexus, lymphatic spreading
through the intervertebral foramen, or spreading along the perineurium of the spinal nerves.8 However, the mode of spreading cannot be confirmed in this case. The diagnostic investigations include plain spinal roentgenograms, myelograms, CT scanning, and magnetic resonate imaging (MRI). MRI is especially useful for early detection of spreading epidural metastasis before the bone scan obtains a positive result.’ As to the treatment of metastatic spinal cord Wilms’ tumor, decompressive laminectomy, adjuvant radiotherapy (2,000 to 3,000 rads over the involved spine for possible residual tumor cells in the tumor bed) and the three-drug regimen (actinomycin-D, vimcristine, and adriamycin and/or cyclophosphamide) according to the NWTS-III protocol are recommended.4
Fig 2. The computed tomography scan of the lumbar spine showed a soft tissue mass (arrow) compressing the spinal cord (arrow head) posteriorly.
Fig 4. The histology from metaatatic aplnel cord tumor ahowed abundant stroms component with uniformad tumor cell end multiple mitosis. (H&E. original magnification x4oy).)
Fig 1. The lumbar my&gram ahowed filling defect (arrow) between the 2nd and 3rd lumbar spine.
552
CHANG ET AL
The presence of a short disease-free interval after initial nephrectomy followed by metastasis to unusual sites usually indicates an aggressive tumor with poor prognosis.5 Thereafter, early detection and treatment for relieving the symptoms, preserving the neurological
function, and offering the possibility of cure are important5 However, the outcome for solitary metastatic spinal cord tumors in children remains unknown, and further investigation is required for early diagnosis and adequate management.
REFERENCES 1. Green DM: The diagnosis and management of Wilms’ tumor. Pediatr Clin North Am 32:735-754, 1985 2. Lemerle J, Tournade MF, Marchant RG, et al: Wilms’ tumor: Natural history and prognostic factors. Cancer 37:2557-2566, 1976 3. Bever CT, Koenigsberger R Antunes JL, et al: Epidural metastasis by Wilms’ tumor. Am J Dis Child 135:644-646, 1981 4. Sumner TE, Crowe JE, Parker MD, et al: Solitary spinal metastasis from Wilms’ tumor. Pediatr Radio1 9:175-176, 1980 5. Kim TH, Zaatari GS, Baum ES, et al: Recurrence of Wilms’ tumor after apparent cure. J Pediatr 107:44-49, 1985 6. Williams JA, Douglass EC, Hammond E, et al: Relapse
Wilms’ tumor, factors affecting survival and cure. Am J Clin Oncol 8:324-328, 1985 7. Tintinalli JE: Acute urinary retention as a presenting sign of spinal cord compression. Ann Emerg Med 15:1235-1237, 1986 8. Sundaresan N, Galicin JH, Lane JM, et al: Treatment of neoplastic epidural cord compression by vertebral body resection and stabilization. J Neurosurg 63:676-684.1985 9. Sarpel S, Sarpel G, Yu E, et al: Early diagnosis of spinalepidural metastasis by magnetic resonance imaging. Cancer 59: 11 I21116,1987
Chang,
Spinal Cord Metastasis Hou-Chuan
Chen, Hai-Chyi
Peng, Dar-Yu Yang, and Cecil Guang-Shiung
Taichung, 0 Wilms’ tumor metastasis to the spinal cord is very rare at any age. We recently encountered a case of a 4-year-old girl, who had a solitary lumbar spinal cord metastasis about 1 year following the initial nephrectomy and an incomplete course of chemotherapy for a stage I Wilms’ tumor. The patient recovered uneventfully after decompressive laminectomy. removal of the extradural spinal cord tumor, adjuvant chemotherapy, and radiotherapy according to the National Wilms’ Tumor Study (NWTSI-IV protocol. @ 1990 by W.B. Saunders Company. INDEX WORDS: Wilms’ tumor; spinal cord metastasis.
W
ILMS’ TUMOR is an embryonal renal neoplasm that may spread to the lymph nodes, lung, liver, and bone’ with an incidence of metastasis around 1O%.2Spinal cord metastasis is rarely reported in the literature.3” Epidural involvement usually occurs with other evidence of spreading.3 Solitary spinal cord metastasis without concomitant involvement to other organs is extremely rare.4 We report a case of solitary spinal cord metastasis without involvement of the lung, liver, bone, or other organs. CASE
REPORT
In November 1986, this 4-year-old girl was referred to Veterans General Hospital-Taichung for evaluation of a progressively eniarging abdominal mass. Physical examination showed a 8 x 10 cm well-defined mass over the left upper quadrant of the abdomen. Plain film of the abdomen showed a big soft tissue mass on the left side. Sonogram of the abdomen showed multiple cystic components in the solid tumor, and the outline of the left kidney was indistinct. Intravenous pyelogram disclosed that the tumor arose from the left kidney, causing distortion and dilatation of the left collecting system. The right kidney had normal function and size. Laboratory data were within normal limits. Explorations with left nephrectomy and periaortic lymph node dissection were performed on November 28, 1986. A huge solitary, homogeneous, well-capsulated mass with central necrosis, about 16 x 11 x 7 cm in size and 650 g in weight, was removed. A tumor thrombus, 1 x 1 x 2 cm in size, within the left renal vein near the hilum was extracted. The pathological finding was nephroblastoma (Wilms’ tumor) with a tumor thrombus in the renal vein. There was no renal capsule invasion; lymph nodes, ureter, and renal vein were also free of tumor tissue. Chemotherapy with vincristine and actinomycin-D was instituted for this stage I Wilms’ tumor accord-
of Wilms’ Tumor Chang
Taiwan
ing to the National Wilms’ Tumor Study (NWTS)-III protocol. Unfortunately, follow-up of the patient was lost after two courses of actinomycin-D every 6 weeks and eight doses of vincristine weekly for 8 weeks. Nearly 1 year after primary surgery, she suffered from migrating pain over both legs, and was admitted again under the impression of tumor recurrence. Physical examination showed a soft and flat abdomen without palpable mass. Both lower extremities were free movable without local inflammation. Laboratory data showed lactic dehydrogenase 273 U/L, alkaline phosphatase 302 U/L, and cholesterol 253 mg/dL. Urinalysis was normal. The chest x-ray showed no metastatic nodule. The whole body bone scan also showed no evidence of metastasis. Chemotherapy with actinomycin-D and vincrinstine, according to NWTS-III protocol, was started after admission. The leg pain subsided gradually. However, she developed acute urinary retention on the fifth hospital day, followed by weakness of both lower legs and obstipation. Neurological examination demonstrated absence of deep tendon reflex in the right leg. A lumbar puncture was performed and the spinal fluid showed normal biochemical reactions and cellularity. Filling defect between the 2nd and 3rd lumbar spine was discolosed by myelogram. Concomitant computed tomography (CT) scan of the L-spine also showed a soft tissue mass in the spinal canal (Figs 1 and 2). Decompressive laminectomy on Ll-3 and removal of the tumor were performed on December 2, 1987. A tumor about 2.4 x 2.2 x 0.6 cm was found in the extradural space posteriorly, which caused external compression of the spinal cord posteriorly and laterally. The pathological report was metastatic nephroblastoma, with a histology similar to that of the primary tumor (Figs 3 and 4). The postoperative course was rather smooth. Short-term steroid therapy (betamethasone 2 mg every 12 hours for 3 days) was given. Chemotherapy according to the NWTS-IV protocol and radiotherapy of 3,000 cGy to the tumor bed and L-spine were instituted. The muscle power of both legs was gradually restored, the gait improved, and the bladder and bowel function were recovered.
DISCUSSION
With the modern therapy of multimodality, Wilms’ tumor is potentially curable in most patients.’ However, Lemerle et al2 reported a recurrent rate about 54% with metastastatic rate of 10%. The possibility of tumor recurrence must be always kept in mind, especially when unusual symptoms and signs present or in high-risk patients with unfavorable histology, positive lymph nodes, tumor weight over 250 g, or aged over 2 276
From the Division of Pediatric Surgery, Department of Surgery, Veterans General Hospital, Taichung, Taiwan, Republic of China. Address reprint requests to Wuu-Tay Chang. MD, Division of Pediatric Surgery, Department of Surgery, Veterans General Hospital, Taichung, 40705 Taiwan, Republic of China. 8 1990 by W.B. Saunders Company. OOZZ-3468/90/250S-0021$03.00/0
The clinical symptoms and signs of spinal cord compression are variable. In general, back pain or radicular pain is probably the earliest and most common sign, followed by motor weakness, sensory loss, and anatomic dysfunction.’ In this patient, radicular pain was noted initially, followed by motor weakness
550
Jour& of
Pediatric Surgery,
Vol 25, No 5 (May), 1990: pp 550-552
SPINAL CORD METASTASIS OF WILMS’ TUMOR
Fig 3. The histology from left Wilma’ tumor showed blastema and atroma component compressing the normal ~renal structure. W, Wilms’ tumor; R, renal structure. &l&E, original magnification X63.1
and bladder and bowel dysfunction, but without sensory impairment. Several different mechanisms are implicated in the pathogenesis of spinal cord metastasis, including direct extension from a paravertebral tumor mass, hematogenous spreading through collateral circulation of the paravertebral venous plexus, lymphatic spreading
through the intervertebral foramen, or spreading along the perineurium of the spinal nerves.8 However, the mode of spreading cannot be confirmed in this case. The diagnostic investigations include plain spinal roentgenograms, myelograms, CT scanning, and magnetic resonate imaging (MRI). MRI is especially useful for early detection of spreading epidural metastasis before the bone scan obtains a positive result.’ As to the treatment of metastatic spinal cord Wilms’ tumor, decompressive laminectomy, adjuvant radiotherapy (2,000 to 3,000 rads over the involved spine for possible residual tumor cells in the tumor bed) and the three-drug regimen (actinomycin-D, vimcristine, and adriamycin and/or cyclophosphamide) according to the NWTS-III protocol are recommended.4
Fig 2. The computed tomography scan of the lumbar spine showed a soft tissue mass (arrow) compressing the spinal cord (arrow head) posteriorly.
Fig 4. The histology from metaatatic aplnel cord tumor ahowed abundant stroms component with uniformad tumor cell end multiple mitosis. (H&E. original magnification x4oy).)
Fig 1. The lumbar my&gram ahowed filling defect (arrow) between the 2nd and 3rd lumbar spine.
552
CHANG ET AL
The presence of a short disease-free interval after initial nephrectomy followed by metastasis to unusual sites usually indicates an aggressive tumor with poor prognosis.5 Thereafter, early detection and treatment for relieving the symptoms, preserving the neurological
function, and offering the possibility of cure are important5 However, the outcome for solitary metastatic spinal cord tumors in children remains unknown, and further investigation is required for early diagnosis and adequate management.
REFERENCES 1. Green DM: The diagnosis and management of Wilms’ tumor. Pediatr Clin North Am 32:735-754, 1985 2. Lemerle J, Tournade MF, Marchant RG, et al: Wilms’ tumor: Natural history and prognostic factors. Cancer 37:2557-2566, 1976 3. Bever CT, Koenigsberger R Antunes JL, et al: Epidural metastasis by Wilms’ tumor. Am J Dis Child 135:644-646, 1981 4. Sumner TE, Crowe JE, Parker MD, et al: Solitary spinal metastasis from Wilms’ tumor. Pediatr Radio1 9:175-176, 1980 5. Kim TH, Zaatari GS, Baum ES, et al: Recurrence of Wilms’ tumor after apparent cure. J Pediatr 107:44-49, 1985 6. Williams JA, Douglass EC, Hammond E, et al: Relapse
Wilms’ tumor, factors affecting survival and cure. Am J Clin Oncol 8:324-328, 1985 7. Tintinalli JE: Acute urinary retention as a presenting sign of spinal cord compression. Ann Emerg Med 15:1235-1237, 1986 8. Sundaresan N, Galicin JH, Lane JM, et al: Treatment of neoplastic epidural cord compression by vertebral body resection and stabilization. J Neurosurg 63:676-684.1985 9. Sarpel S, Sarpel G, Yu E, et al: Early diagnosis of spinalepidural metastasis by magnetic resonance imaging. Cancer 59: 11 I21116,1987
