6th May 2004 Neural sheath tumor

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6th May 2004Neural sheath tumor
Dept of Medicine Pathology CWM Hospital Fiji
School of MedicineWeekly CPC Meeting

• Dr. Venkatesh Murthy Shashidhar
• Associate Professor of Pathology

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Patient details

• 21 Year Fijian female presented with Bilateral
  Lower limb spastic paralysis, numbness since few
  weeks. P/H of trauma few months back to back but
  not treated.
• On examination, cafe-au-Lait spots all over body,
  HEENT normal, No hepatosplenomegaly.
• Large mass over back T6-t8 paraspinal extending
  to back. Myelogram showed osteolytic mass eroding
  into bony spine and compressing spinal cord.

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Patient details

• Patient was put on steroids which significantly
  improved her condition.
• Surgery done 2x2cm mass excised for
  histopathology. complete mass was not removable
  due to deeper infiltration into bone.
• Patient condition improved after surgery. able to
  walk few steps without support.

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Patient details

• Presenting Problems
• Spastic paraplegia
• T 8 cord segment sensory deficit
• Cafe-au-lei spots
• Progressive paraplegia. She has had gradual
  increasing weakness for the past 6 months and has
  not been able to walk for the past month. There
  is no pain. She has preserved bowel and urinary
  function. She has not had any spinal injuries.
  She recently had a child delivery.

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Patient details

• Spastic paralysis both lower limbs. She was
  unable to walk although she barely could support
  herself when she was helped to stand.
• 2/5 weakness in both lower limbs. There was
  generalized weakness from hip, knee and ankle
  movements.
• There is increased tone with sustained clonus
  bilaterally.
• There is cord segment sensory deficit to T8
  level. There are café it-au-lei spots throughout
  her body.
• The examination of her abdomen, chest and CVS are
  all normal.

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Myelogram Obstruction at T7
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Image reconstruction - tumor
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Image reconstruction - tumor
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Biopsy of the mass

• Encapsulated expanding mass.

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Biopsy of the mass

• Cellular tumor weavy spindle cells.

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Biopsy of the mass

• Some areas show palisading bundles of these
  spindle cells

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Biopsy of the mass

• Some large hyperchromatic pleomorphic cells seen,
  (? Malignant)

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Biopsy of the mass

• Some large hyperchromatic pleomorphic cells seen,
  (? Malignant)
• Lymphocyte infiltration (suggesting inflammatory
  reaction)

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Biopsy of the mass

• Some large hyperchromatic pleomorphic cells seen,
  (? Malignant)
• Lymphocyte infiltration (suggesting inflammatory
  reaction)

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Discussion

• Dorsal nerve root neurofibromas typically occur
  in patients suffering from neurofibromatosis.
• Consists of neoplastic schwann cells.
• Unlike schwannoma, neurofibroma is not
  encapsulated.
• Neurofibromas consist of a mixture of schwann
  cells and fibroblasts forming bundles of
  elongated cells with wavy neuclei.

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Provisional diagnosis of Neurilemmoma made and
case put on Pacific Telepathology for second
opinion.
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Pacific TelepathologySecond opinion provided
byProf. Dr. Klaus D. KunzeInstitut für
Pathologie, Universitätsklinikum
DresdenFetscherstr. 74D01307 DresdenDr Prasad
CSBRAl Hakeem PolyclinicPO.BOX 34985,
KSA.11478 Riyadh, KSA.
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Pacific Telepathology MPNST

• Features supportive of MPNST in this case are,
• Gross large gt10cm, deep paraspinal location,
• Fusiform swelling, diffuse infiltrative borders
  with surrounding bone destruction invasion.
• Microscopy Spindle cell fascicular appearance,
  perivascular whorling, vascular invasion.
• wavy tapering nuclei, myxoid stroma, poorly
  defined cell border.
• Focal areas of nuclear hyperchromatism,
  pleomorphic,
• inconspicuous nucleoli with Many mitoses.
• Infrequent palisading.

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MPNST

• MPNST - Malignant Peripheral Nerve Sheath Tumor.
  (Malignant Schwannoma or Neurofibrosarcoma).
• 30-50 arise in patients with neurofibromatosis
  or family history of neurofibromatosis.
• Adults, origin from a nerve, Paraspinal, limbs or
  trunk.
• About 10 occur following radiation exposure.
• Poor prognosis 20-50 5 year survival, better in
  neurofibromatosis.
• Pulmonary metastasis is usual final end result.
• Heterologous differentiation - commonly
  rhabdomyosarcomatous component - known as Triton
  tumor - poor prognosis.

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Microscopic features of MPNST

• 1A sweeping bundles of spindle cells.
• 1B Short bundles and pleomorphic large cells.
• 1C Geographic Patchy Necrosis.
• 2 epithelioid variant.
• 3A B PGP 9.5 vity

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MPNST

• Features to support MPNST from neurofibroma..
• Definite cell crowding
• Nuclear enlargement (gt 3 times than ordinary
  neurofibroma nuclei)
• Hyperchromasia.
• Geographic Necrosis with outer palisading.
• Ref Armed Forces Institute of Pathology (AFIP)
• All these features were seen in this case except
  necrosis.