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Malignant rhabdoid tumor of the kidney and spine in an infant.

Park S, Seo JH, Park JB, Park S - J Korean Neurosurg Soc (2014)

Bottom Line: Rhabdoid tumor of the kidney (RTK) is a rare malignancy in infancy.Central nervous system involvement in RTK is already known.If there are any abnormal signs in neurologic examination, magnetic resonance images of brain and spine are recommended.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, Korea.

ABSTRACT
Rhabdoid tumor of the kidney (RTK) is a rare malignancy in infancy. Central nervous system involvement in RTK is already known. However, solitary spinal metastasis in RTK has been hardly reported. The authors report a case of metastatic RTK to spine causing paraplegia in an 8-month-old girl. Since the patient was young, the diagnosis of spine metastasis was delayed until paraplegia was seen after radical nephrectomy. Thorough neurological examination should be performed for early diagnosis of spinal metastasis in young patients with RTK. If there are any abnormal signs in neurologic examination, magnetic resonance images of brain and spine are recommended.

No MeSH data available.


Related in: MedlinePlus

Histology of spinal surgical specimen shows small round cell tumor with extensive necrosis consistent with metastatic rhabdoid tumor (hematoxylin-eosin stain, ×200).
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Figure 5: Histology of spinal surgical specimen shows small round cell tumor with extensive necrosis consistent with metastatic rhabdoid tumor (hematoxylin-eosin stain, ×200).

Mentions: One week after the operation, weakness of both lower extremities and voiding failure were noted. On neurological examination, she failed to respond to pain in both legs. Brain magnetic resonance imaging (MRI) did not show evidence of metastatic disease, but lumbar MRI revealed a 6.5×1 cm sized intradural extramedullary tubular mass with heterogeneous enhancement at L1-S1 level (Fig. 3). On the next day from onset of symptoms, laminoplastic laminotomy was performed by a neurosurgeon to remove the tumor and decompress spinal cord. Conus medullaris was displaced by the hypervascular tumor with yellowish color, however the upper margin of the tumor was clear (Fig. 4A). The tumor was densely adhered to the left side of L5 nerve root. Near total removal of the tumor was performed using cavitron ultrasonic surgical aspirator, and there was no root injury during the surgery (Fig. 4B). The pathologic findings were consistent with metastatic malignant rhabdoid tumor (Fig. 5). After the surgery, the motor power of lower extremity was returned. She received adjuvant radiotherapy for 2 weeks and 13 cycles of chemotherapy (vincristine, doxorubicin, and cyclophosphamide) for 3 months postoperatively. The patient showed general weakness, seizure and changes in mental status during the chemotherapy. Leptomeningeal metastasis with hydrocephalus was noted in brain MRI. A ventriculoperitoneal shunt was placed. However, she did not clinically improve and died 6 months after the surgery.


Malignant rhabdoid tumor of the kidney and spine in an infant.

Park S, Seo JH, Park JB, Park S - J Korean Neurosurg Soc (2014)

Histology of spinal surgical specimen shows small round cell tumor with extensive necrosis consistent with metastatic rhabdoid tumor (hematoxylin-eosin stain, ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3928353&req=5

Figure 5: Histology of spinal surgical specimen shows small round cell tumor with extensive necrosis consistent with metastatic rhabdoid tumor (hematoxylin-eosin stain, ×200).
Mentions: One week after the operation, weakness of both lower extremities and voiding failure were noted. On neurological examination, she failed to respond to pain in both legs. Brain magnetic resonance imaging (MRI) did not show evidence of metastatic disease, but lumbar MRI revealed a 6.5×1 cm sized intradural extramedullary tubular mass with heterogeneous enhancement at L1-S1 level (Fig. 3). On the next day from onset of symptoms, laminoplastic laminotomy was performed by a neurosurgeon to remove the tumor and decompress spinal cord. Conus medullaris was displaced by the hypervascular tumor with yellowish color, however the upper margin of the tumor was clear (Fig. 4A). The tumor was densely adhered to the left side of L5 nerve root. Near total removal of the tumor was performed using cavitron ultrasonic surgical aspirator, and there was no root injury during the surgery (Fig. 4B). The pathologic findings were consistent with metastatic malignant rhabdoid tumor (Fig. 5). After the surgery, the motor power of lower extremity was returned. She received adjuvant radiotherapy for 2 weeks and 13 cycles of chemotherapy (vincristine, doxorubicin, and cyclophosphamide) for 3 months postoperatively. The patient showed general weakness, seizure and changes in mental status during the chemotherapy. Leptomeningeal metastasis with hydrocephalus was noted in brain MRI. A ventriculoperitoneal shunt was placed. However, she did not clinically improve and died 6 months after the surgery.

Bottom Line: Rhabdoid tumor of the kidney (RTK) is a rare malignancy in infancy.Central nervous system involvement in RTK is already known.If there are any abnormal signs in neurologic examination, magnetic resonance images of brain and spine are recommended.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, Korea.

ABSTRACT
Rhabdoid tumor of the kidney (RTK) is a rare malignancy in infancy. Central nervous system involvement in RTK is already known. However, solitary spinal metastasis in RTK has been hardly reported. The authors report a case of metastatic RTK to spine causing paraplegia in an 8-month-old girl. Since the patient was young, the diagnosis of spine metastasis was delayed until paraplegia was seen after radical nephrectomy. Thorough neurological examination should be performed for early diagnosis of spinal metastasis in young patients with RTK. If there are any abnormal signs in neurologic examination, magnetic resonance images of brain and spine are recommended.

No MeSH data available.


Related in: MedlinePlus