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Spinal cord ependymoma associated with neurofibromatosis 1 : case report and review of the literature.

Cheng H, Shan M, Feng C, Wang X - J Korean Neurosurg Soc (2014)

Bottom Line: Magnetic resonance imaging revealed a relative-demarcated, heterogeneously enhanced mass lesion accompanied by perifocal edema in C5-7 level, a left-sided T11 spinous process heterogeneously enhanced mass in soft tissue, intervertebral disk hernia in L2-5 level, and widespread punctum enhancing lesion in her scalp and in T11-L5 level.Therefore, no adjuvant therapy was applied.Following the operation, the patient showed an uneventful clinical recovery with no evidence of tumor recurrence after one year of follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, The First Affiliated Hospital of Anhui Medical University, Anhui, China.

ABSTRACT
Patients with neurofibromatosis 1 (NF1) are predisposed to develop central nervous system tumors, due to the loss of neurofibromin, an inactivator of proto-oncogene Ras. However, to our knowledge, only three cases of ependymomas with NF1 have been reported in the literature. The authors present a case of NF1 patient with a spinal cord ependymoma. She was referred for about half a year history of increasing numbness that progressed from her fingers to her entire body above the bellybutton. Magnetic resonance imaging revealed a relative-demarcated, heterogeneously enhanced mass lesion accompanied by perifocal edema in C5-7 level, a left-sided T11 spinous process heterogeneously enhanced mass in soft tissue, intervertebral disk hernia in L2-5 level, and widespread punctum enhancing lesion in her scalp and in T11-L5 level. The patient underwent C5-7 laminectomies and total excision of the tumor under operative microscope, and intraoperative ultrasonography and physiological monitoring were used during the surgery. Histopathologically, her tumor was found to be a ependymoma without malignant features (grade II in the World Health Organization classification). Therefore, no adjuvant therapy was applied. Following the operation, the patient showed an uneventful clinical recovery with no evidence of tumor recurrence after one year of follow-up.

No MeSH data available.


Related in: MedlinePlus

A : Operative scar on her back and café-au-lait spot (arrow). B : Operative scar on her left thigh and widespread café-au-lait spots (arrow). C : Widespread frecklings axillary. D : Cutaneous neurofibromas and plexiform neurofibroma (arrow). E : Patient's pedigree. The circles represent females and squares represent males. Black represents affected individuals. Oblique line represents dead individuals. The patient is signed in arrow, her mother and maternal grandmother have similar manifestations of neurofibromatosis 1.
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Figure 1: A : Operative scar on her back and café-au-lait spot (arrow). B : Operative scar on her left thigh and widespread café-au-lait spots (arrow). C : Widespread frecklings axillary. D : Cutaneous neurofibromas and plexiform neurofibroma (arrow). E : Patient's pedigree. The circles represent females and squares represent males. Black represents affected individuals. Oblique line represents dead individuals. The patient is signed in arrow, her mother and maternal grandmother have similar manifestations of neurofibromatosis 1.

Mentions: A 49-year-old female patient was admitted to our department because of numbness in her fingers that progressed to her entire body above the bellybutton for half a year. In his past medical history there were expeditiously increscent cutaneous neurofibromas respectively on her back and left thigh for five years, and a total resection for tumors had been performed in a local hospital. Histological examination revealed both of them were neurofibromas with malignant features. On physical examination, widespread café-au-lait spots, axillary and groin frecklings, cutaneous neurofibromas, plexiform neurofibromas and operative scar on her back and left thigh were present, and a sensory deficit was present between the C4 level and bellybutton. No iris hamartomas had been found and mammary gland were normal. Among the family members her mother and maternal grandmother had similar manifestations of NF1, but her daughter and sons had no clinical evidence of NF1 (Fig. 1).


Spinal cord ependymoma associated with neurofibromatosis 1 : case report and review of the literature.

Cheng H, Shan M, Feng C, Wang X - J Korean Neurosurg Soc (2014)

A : Operative scar on her back and café-au-lait spot (arrow). B : Operative scar on her left thigh and widespread café-au-lait spots (arrow). C : Widespread frecklings axillary. D : Cutaneous neurofibromas and plexiform neurofibroma (arrow). E : Patient's pedigree. The circles represent females and squares represent males. Black represents affected individuals. Oblique line represents dead individuals. The patient is signed in arrow, her mother and maternal grandmother have similar manifestations of neurofibromatosis 1.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3928348&req=5

Figure 1: A : Operative scar on her back and café-au-lait spot (arrow). B : Operative scar on her left thigh and widespread café-au-lait spots (arrow). C : Widespread frecklings axillary. D : Cutaneous neurofibromas and plexiform neurofibroma (arrow). E : Patient's pedigree. The circles represent females and squares represent males. Black represents affected individuals. Oblique line represents dead individuals. The patient is signed in arrow, her mother and maternal grandmother have similar manifestations of neurofibromatosis 1.
Mentions: A 49-year-old female patient was admitted to our department because of numbness in her fingers that progressed to her entire body above the bellybutton for half a year. In his past medical history there were expeditiously increscent cutaneous neurofibromas respectively on her back and left thigh for five years, and a total resection for tumors had been performed in a local hospital. Histological examination revealed both of them were neurofibromas with malignant features. On physical examination, widespread café-au-lait spots, axillary and groin frecklings, cutaneous neurofibromas, plexiform neurofibromas and operative scar on her back and left thigh were present, and a sensory deficit was present between the C4 level and bellybutton. No iris hamartomas had been found and mammary gland were normal. Among the family members her mother and maternal grandmother had similar manifestations of NF1, but her daughter and sons had no clinical evidence of NF1 (Fig. 1).

Bottom Line: Magnetic resonance imaging revealed a relative-demarcated, heterogeneously enhanced mass lesion accompanied by perifocal edema in C5-7 level, a left-sided T11 spinous process heterogeneously enhanced mass in soft tissue, intervertebral disk hernia in L2-5 level, and widespread punctum enhancing lesion in her scalp and in T11-L5 level.Therefore, no adjuvant therapy was applied.Following the operation, the patient showed an uneventful clinical recovery with no evidence of tumor recurrence after one year of follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, The First Affiliated Hospital of Anhui Medical University, Anhui, China.

ABSTRACT
Patients with neurofibromatosis 1 (NF1) are predisposed to develop central nervous system tumors, due to the loss of neurofibromin, an inactivator of proto-oncogene Ras. However, to our knowledge, only three cases of ependymomas with NF1 have been reported in the literature. The authors present a case of NF1 patient with a spinal cord ependymoma. She was referred for about half a year history of increasing numbness that progressed from her fingers to her entire body above the bellybutton. Magnetic resonance imaging revealed a relative-demarcated, heterogeneously enhanced mass lesion accompanied by perifocal edema in C5-7 level, a left-sided T11 spinous process heterogeneously enhanced mass in soft tissue, intervertebral disk hernia in L2-5 level, and widespread punctum enhancing lesion in her scalp and in T11-L5 level. The patient underwent C5-7 laminectomies and total excision of the tumor under operative microscope, and intraoperative ultrasonography and physiological monitoring were used during the surgery. Histopathologically, her tumor was found to be a ependymoma without malignant features (grade II in the World Health Organization classification). Therefore, no adjuvant therapy was applied. Following the operation, the patient showed an uneventful clinical recovery with no evidence of tumor recurrence after one year of follow-up.

No MeSH data available.


Related in: MedlinePlus