SCHWANNOMAS OF ADRENAL GLAND AND POSTERIOR MEDIASTINUM

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SCHWANNOMAS OF ADRENAL GLAND AND POSTERIOR
MEDIASTINUM

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GENERAL DATA

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• A 30-year-old Taiwanese woman
• Unmarried
• Occupation insurance employee
• Hobby fitness
• 1st time Hospitalization date from 95-5-31 to
  95-6-12
• 2nd time Hospitalization date from 95-6-19 to
  95-7-17

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Chief complaint

• left anterior chest discomfort (sternal area) and
  left flank pain for one week.

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Present illness

• 1. Denied any systemic illness before.
• 2. Visit exercise center frequently
• 3. Felt anterior chest wall pain (over sternal
  area) and left flank pain in recent days
• 4. Visit CM OPD on 95-5-23, CXR revealed masses
  over mediastinum and superimposing left hilum.
• 5.Arranged admission on 95-5-31

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Past and personal history

• Denied of any systemic illness before
• No major operation
• No smoking
• No alcoholic drinking

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Hematology
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Biochemistry data
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Stool routine

• Occult -
• Parasite Ova-Direct -
• Microscopic WBC -
• Microscopic-Ep. Cell -
• Appearance soft
• Color Brown

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Urine routine

• Appearance Clear
• Color Yellow
• PH 6.5
• Protein -
• Glucose -
• Ketone body -
• Urobilinogen 0.1
• Bilirubin -
• Occult -
• Specific gravity 1.025
• WBC (-)
• RBC (-)
• Bacilli ()
• Cast (-)
• Crystal (-)

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Chest X-ray

• showed left paraspinal lobulated soft tissue
  tumor.

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Chest computed tomography (CT)

• revealed several well-marginated heterogeneously
  enhancing masses with central low density along
  left paraspinal region and a heterogenous
  enhancing mass over left adrenal region. R/O MEN
  type II, Pheochromocytoma with paragangliomas.

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Endocrine consultation

• CM doctor consult endocrine doctor on 6/2
• Endocrine section take over the case on 6/3
• Arranged Endocrine study MRI of adrenal gland

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Endocrinological studies (1)

• Serum calcitonin lt14.0 pg/ml (lt42)
• Cortisol (Random) 10.85 ug/dl
• ACTH 29.4 pg/ml (9-52)
• Aldosterone 117.2 pg/ml
• Renin 1.95 ng/ml

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Endocrinological studies (2)

• 24 hr urine 2000cc
• free cortisol 5.1 ug/day (lt60)
• VMA 3.7 mg/day (1.0-7.5)
• 17-KS9.12 mg/day (6-14)
• 17-OHCS 9.68 mg/day (2-8)
• Adrenaline 4.5 ug/day (lt22.4)
• Noradrenaline 33.3 ug/day (11.1-85)
• Dopamine 264 ug/day (50-450)

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Magnetic resonance imaging (MRI) of adrenal gland

• revealed a 6.3 cm left adrenal tumor, retrocrual
  and thoracic paraspinal tumors.

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Consult CS GU section

• CS doctor take over the case arrnage OP
• Due to personal problem, patient discharge on
  95-6-13
• Readmission on 95-6-18 received CS OP on 95-6-19

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Operation

• 95-6-19 Patient received tumor removal of
  posterior mediastinum
• Admitted at ICU from 96-6-19 to 96-6-29
• 95-7-3 GU take over the case
• 95-7-6 GU performed left laparoscopic
  adrenalectomy.

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Pathological finding

• Pathological examination revealed ovoid to
  spindle schwann cells arranged in fascicles with
  stromal myxoid changes.
• Immunohistochemical examination revealed tumor
  cells positive for S-100 protein but not CD34,
  diagnostic for schwannoma.

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Postoperative course

• The postoperative course was smooth. After one
  month hospitalization, the patient was discharged
  in good condition on 96-7-18.

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Final diagnosis

• Schwannomas of adrenal gland and posterior
  mediastinum

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Discussion
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Adrenal incidentalomas

• A heterogenous group of pathological entities,
  including benign or malignant adrenocortical or
  medullary tumors, hormonally active or inactive
  lesions, which are identified incidentally during
  the examination of nonadrenal-related abdominal
  complaints
• Pheochromocytomas 1.5-23
• Nonpheochromocytoma ganglioneuroma,
  gnaglioneuroblastoma, neuroblastoma, and
  malignant or benign schwannoma

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Schwann cells

• The Schwann cells are the cells that make the
  myelin in the peripheral nervous system (PNS).
• In contrast to the oligodendrocytes of the
  central nervous system, each Schwann cell
  myelinates a single axon.
• Also, Schwann cells lay down a basement membrane
  around themselves, unlike oligodendrocytes.
• Schwann cells are very important in regeneration
  a damaged peripheral nerve.
• Schwann cells can also form tumors, called
  schwannomas.

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Schwannoma

• Most schwannomas occur in the head, neck, stomach
  or limbs with a few cases occurring in the
  retroperitoneal space
• In benign schwannoma occurring in retroperitoneal
  space, tumors are most commonly located near the
  adrenal gland.

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Pathophysiology

• Schwannomas arise from the nerve sheath and
  consist of Schwann cells in a collagenous matrix.
  
• Histologically, the terms Antoni type A
  neurilemoma and type B neurilemoma are used to
  describe varying growth patterns in schwannomas.
  Type A tissue has elongated spindle cells
  arranged in irregular streams and is compact in
  nature. Type B tissue has a looser organization,
  often with cystic spaces intermixed within the
  tissue. The cystic spaces can result in high
  signal intensity on T2-weighted MRIs.
• Tumors originating in Schwann cells can be
  detected at immunohistochemical examination by
  virtue of their positive results with S-100
  antigen tests, collagen IV, laminin and absence
  of reactivity for keratin, muscle related
  antigens, and CD34.

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This is an example of a schwannoma

• It typically has dense areas called Antoni A
  (black arrow) and looser areas called Antoni B
  (blue arrows). The cells are elongated (spindle
  shaped) and the nuclei have a tendency to line up
  as you see here in the Antoni A area. Like normal
  Schwann cells, schwannoma cells are each
  surrounded by a basement membrane.

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The resected tumor was firm and had a
yellowish-white cut surface
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Schwannoma

• Schwannoma and neurofibroma are benign peripheral
  nerve neoplasms, represent the most common
  mediastinal neurogenic tumors, and rarely
  degenerate into malignant tumors of nerve sheath
  origin

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Neurofibromatosis 2 (NF2) and Schwannoma

• Neurofibromatosis 2 (NF2) is an autosomal
  dominant disorder that causes nervous system
  tumors and ocular abnormalities such as early
  onset lenticular opacities. Vestibular schwannoma
  also noted.
• CN schwannomas are usually isolated lesions,
  except when they are associated with
  neurofibromatosis type 2 (NF2), a rare autosomal
  dominant disorder occurring in approximately 1
  live birth in 50,000.
• NF2 is also called the multiple inherited
  schwannomas, meningiomas, and ependymomas (MISME)
  syndrome.
• NF2 is characterized by bilateral vestibular
  schwannomas. Schwannomas of the other CNs occur
  more frequently in NF2, and the presence of one
  of the rare CN schwannomas should suggest the
  possibility of NF2. Meningiomas and
  intramedullary ependymomas of the spinal cord
  also occur in NF2

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CNS SCHWANNOMA

• Schwannomas account for 6-8 of intracranial
  neoplasms.
• Autopsy studies have shown that the incidence
  rates of occult vestibular schwannomas are as
  high as 2.7.
• A study of patients undergoing MRI for
  indications other than the evaluation of
  schwannoma revealed an estimated prevalence of
  0.07.
• Vestibular schwannomas are the most common CN
  schwannomas, followed by trigeminal and facial
  schwannomas and then glossopharyngeal, vagus, and
  spinal accessory nerve schwannomas.
• Schwannomas involving the oculomotor, trochlear,
  abducens, and hypoglossal nerves are rare.

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Other characters of schwannoma

• Mortality/Morbidity Morbidity resulting from
  schwannomas includes nerve dysfunction and
  brainstem compression. Mortality can result from
  mass effect with brainstem compression.
• Race No racial predilection has been described
  in schwannomas.
• Sex No sex predilection has been described in
  schwannomas.

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Detection of Schwannomas

• 123I-metaiodobenzylguanidine (MIBG) scan-reliable
  morphofunctional technique to evaluate
  catecholamine turnover in adrenal tumors
• Computed tomography (CT) scan anatomy of tumor,
  cystic lesions
• Magnetic resonance imaging (MRI) MRI provides
  the highest degree of soft tissue resolution, it
  can provide images in multiple planes

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Surgical intervention

• Transabdominal approach-suitable for
  pheochromocytoma and bilateral adrenal tumor, but
  postoperative recovery was slow
• Translumbar approach-40 pleural injury
• Laparoscopic adrenalectomy is safe and feasible
  for diagnosis and treatment of benign adrenal or
  retroperitoneal schwannoma, recovery fast

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Prognosis

• Retroperitoneal schwannoma is a primary neural
  benign tumor with a good prognosis
• The management is surgical

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Conculsion

• Schwannoma of both adrenal gland and posterior
  mediastinum are extremely rare
• Although most schwannoma are benign
• Long term follow up is mandatory