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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
373
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_111
111
Myelopathy in Neurocutatneous Melanosis
111.1 Definition
The neural crest is the common embryonic origin of both the melanotic cells of the
skin and the letomeninges. According to these authors neurocutaneous melanosis
(NCM) is a congenital syndrome characterized by the development of congenital
melanocytic naevi and benign or malignant melanotic tumors of the CNS. NCM is
considered a rare complication of giant congenital meano-cytic-naevus (GCMN).
111.2 Incidence
The risk of developing begnin or malignant melanotic tumors in carries of GCMN
is unknown; just over 100 cases were reported (2001). Involvement of the brain will
not be discussed. Myelopathy is the subject of this presentation.
111.3 Etiology
NCM is thought to be due to an error in the embryogenesis of the embryological
neuroectoderm.
111.4 Pathology
There is melanotic pigmentation of the spinal arachnoid and adjacent leptomeninges. Cord compression is usually due to development of melanotic arachnoid cyst or
to direct infi ltration or syringomyelia. The chance of malignant progression and
Abstracted from Holmes et al. ( 2001 )
374
prognosis are unknown. Twenty percent of cases show or display widespread pigmentation and thickening of the full length of the spinal cord meninges. Malignant
transformation in the CNS is estimated to be up to 50 %.
111.5 Clinical Picture
Patients with GCMN on the extremities never develop NCM. Posterior axial nevi
are more subject to NCM. CNS involvement excluding intracranial lesions may be
asymptomatic. Cord compression or infi ltration will give the picture of myelopathy.
Radicular syndrome is also common.
111.6 Diagnosis
Diagnosis is based on:
1. Congenital nevi
2. Neurological fi ndings
3. MRI: for symptomatic patients and all asymptomatic patients with large multiple
lesions of the head and neck or lesions of the posterior midline.
111.7 Management
Close observation of these lesions regularly by the dermatologist. There is no therapy for leptomeningial melanotic changes. Space occupying lesions may be
approached neurosurgically.
Reference
Holmes G, Wines N, Ryman W. Giant congential melanocytic naevus and symptomatic thoracic
arachnoid cyst. Australas J Dermatol. 2001;42(2):124–8.
111 Myelopathy in Neurocutatneous Melanosis
375
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_112
112
Anaplastic Oligodendroglioma
112.1 Definition
This is an anaplastic dendro-glimoa of the spinal cord secondary to primary lesion
of the brain.
112.2 Incidence
According to Mcbryde et al. less than ten cases have been reported in world literature. Oligodendroglimoa accout for 5 % of all primary brain tumors and 30 % for all
intracranial glioma
112.3 Etiology
The causative factor(s) of this tumor are not known.
112.4 Pathology
The primary lesion brain lesion shows:
• Macroscopically, hemorrhages cystic formation and calcifi cation
• Microscopically, tumor cells are homogeneous and have a characteristic “fried
egg” appearance, showing a clear perinuclear halo surrounding a central nucleus
as a result of fi xation artifact.
Abstracted from Mcbryde et al. ( 2003 )
376
This typical appearance is less common in anaplastic oligodendroglioma. The
neoplastic cells may be in lobules, separated by branching vessels, forming a
“chicken wire” pattern, with mucinous accumulation may be noted. The anaplastic
form shows mitosis, endothelial proliferation, mitotic activity and necrosis.
Metastases of oligodendrogliomas are rare due to:
• Blood brain barrier
• Absence of lymphatics in the brain
• Short survival of the patients
Immunohistochemistry is positive for glial fi brillary acidic protein and S100.
Details of immunohistochemistry can be seen in Choon and Raepke. Only one case
had intramedullary metastasis and the rest had meningeal involvement. It is believed
that tumor cells reach the spinal cord via the CSF by the ependymal epithemlium or
the pia mater.
112.5 Clinical Picture
A previous history of brain oligodendroglioma operated upon and metastasis to the
spinal cord may be due to CSF leakage during surgery. Spinal metastasis in the
cases recurrence occurred from 3 months to 6 years after initial brain surgery.
Tumors were grade 2 or 3. The clinical picture is that of paraparesis.
112.6 Diagnosis
Diagnosis based on:
1. History of brain surgery and pathological report
MRI, CT scan, CSF cytology are all performed
112.7 Management
Surgery consists of:
1. Debulking or excision
The patient received radiotherapy; the same treatment is applied to epidural anaplastic oligodendroglioma.
112 Anaplastic Oligodendroglioma
377
Updates
Yuh et al. (2015)
Primary spinal cord oligodendrogliomas are rare tumors comprising two percent
of all spinal cord tumors. Although a treatment guideline has yet to be established,
maximal surgical resection is primary in the treatment of spinal cord oligodendrogliomas. Adjuvant radiotherapy has remained controversial, and it is unclear
whether chemotherapy adds any benefi t. In this case report, the authors present a
24-year-old male who had a 7-year history of left leg weakness and a radiating pain
in both legs. Magnetic resonance image (MRI) showed an intramedullary mass at
the T4-T8 level. He underwent subtotal removal of the tumor and pathologic diagnosis revealed a WHO grade IIoligodendroglioma. The patient was treated with
radiotherapy postoperatively and followed up with MRI annually. Clinical and
radiological status of the patient had been stationary for 4 years after the surgery.
The 5-year follow-up MRI showed an increase in the size and extent of the residual
tumor. Despite radiological progression, considering that symptoms and the performance status of the patient had remained unchanged, further treatment has not been
performed. Given the clinical outcome of this patient, close observation after subtotal removal with adjuvant radiotherapy is one of the acceptable treatment options
for WHO grade II spinal cord oligodendrogliomas.
Reference
Yuh WT, Chung CK, Park SH. Primary spinal cord oligodendroglioma with postoperative adjuvant radiotherapy: a case report. Korean J Spine. 2015;12(3):160–4.
Reference
Mcbryde CW, Hamid NA, Mitchell RD. Anaplastic oligodendroglioma with metastasis to the spi-
nal cord. Br J Neurosurg. 2003;17(4):364–6.
Reference
379
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_113
113
Oncocytoma in Melanocytoma
113.1 Definition
This is a benign neoplasm composed predominantly of monotonous sheets of plump
oncocytes. By electron microscopy, the cytoplasm of the oncocyte is packed full
with mitochondria. Focal areas of the tumor contain spindle cells with abundant
intracytoplasmic granular deposits of brown melanin pigment that contains
melanosomes.
113.2 Incidence
This is the fi rst case described by Gelmen et al. Oncocytoma is extremely rare in the
CNS. Oncocytic variant of meningioma has been described in six cases and one
malignant spinal paraganglioma.
113.3 Etiology
The majority of oncocytomas probably have an epithelial origin. Because the prevalence of oncocytoma increases with age it is widely accepted that oncocytomas are
the result of an aberrant metaplasia occurring during cell senescence.
Abstracted from Gelman et al. ( 2000 )
380
113.4 Pathology
Oncocytomas are benign neoplasms. They occur most commonly in the kidney.
They may appear in neoplasms as single cells or as a substantial portion of the
tumor or ma constitute the whole tumor. Oncocytic transformation of a tumor of
over 50 % then it is labeled as oncocytoma.
113.5 Histopathology
Sheets of plump polygonal cells containing abundant granular eosinophilic cytoplasm many areas around the blood vessels, spindle cells with intracytoplasmic
granules, which by Fontana-Masson technique proved to be melanin; more detailed
description of the histology and histochemistry can be seen in the original work of
Gelman et al. 2000 .
113.6 Clinical Picture
The patient described is a 71-year-old female who had episodes of lower back pain
for 28 years. This extended to the legs and became worse, not responding to analgesics, urinary and fecal incontinence. The back pain then radiated to the buttocks and
thighs, mild facial paresis. The refl exes were absent.
113.7 Diagnosis
Diagnosis is based on the following criteria:
1. Clinical history
2. MRI: intracanalicular (medullary) mass from L3 to S1.
113.8 Management
Laminectomy L3-L5 revealed the mass in the cord; removal by microsurgical technique en bloc . Recovery was uneventful and no recurrence 4 years later
Reference
Gelman BB, Trier TT, Chaljub G, Borokowski J, Nauta HJ. Oncocytoma in melanocytoma of the
spinal cord: case report. Neurosurgery. 2000;47(3):756–9.
113 Oncocytoma in Melanocytoma
381
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_114
114
Osteosarcoma
114.1 Definition
Osteosarcoma is the most common nonhemtalogica primary malignancy of bone
and is commonly reported in the appendicular skeleton.
114.2 Incidence
Primary osteosarcoma of the vertebral column is rare. Primary vertebral osteosarcoma (PVOS) accounts for 4 % of all osteosarcoma. Females outnumbered males.
The age 8–80 years (mean 34.5 years); the cervical spine is the least affected; the
site of origin arises most commonly from the posterior elements and involved the
body of the vertebra. The spinal canal is involved in 84 % of reported cases.
114.3 Pathology
The tumor shows pleomorphic cells with abundant cytoplasm and atypical nuclei
with mitosis. Osteoblastic giant cells are encountered with osteoid and fi brocollagenous tissue. The tumor may be osteoblastic or osteolytic.
114.4 Clinical Picture
The case reported was a 60-year-old female had 3 week history of the lower limbs
and sphincteric disturbances. No history of fever nor trauma. Neurological exam
showed upper limbs normal proximally but weak grip. Lower limbs were 2/5, deep
Abstracted from Makhdoomi et al. ( 2010 )
382
tendon refl ex of the lower limbs were brisk, Babinski’s was positive. Sensory level
was T2, hematology was normal.
114.5 Diagnosis
Diagnosis based on:
1. MRI shows destruction of vertebral bodies C7-T1 and cord compression with the
tumor around. The paravertebral muscles were infi ltration. The posterior ele-
ments were destroyed by the tumor. The tumor was fi rm and somewhat
vascular.
114.6 Management
Laminectomy at C7 and T1 by removing the tumor with the posterior elements and
near total decompression was achieved. Anterior extension was noticed and but
nothing was done on the vertebral bodies. There was no subdural involvement.
Post-op radio-chemotherapy was given. Confi rmed primary osteosarcoma, metastatic tumor were ruled out by immunohistochemical studies. Five months post-op
she gained motor power 4/5 and was ambulatory with one cane.
U p d a t e
Abstract from Dekutoski et al. (2016)
Primary spinal osteosarcomas are rare and aggressive neoplasms. Poor outcomes
can occur, as obtaining marginal margins is technically demanding; further
Enneking-appropriate en bloc resection can have signifi cant morbidity. The goal of
this study is to identify prognostic variables for local recurrence and mortality in
surgically treated patients diagnosed with a primary osteosarcoma of the spine.
A multicenter ambispective database of surgically treated patients with primary
spine osteosarcomas was developed by AOSpine Knowledge Forum Tumor. Patient
demographic, diagnosis, treatment, perioperative morbidity, local recurrence, and
cross-sectional survival data were collected. Tumors were classifi ed in 2 cohorts:
Enneking appropriate (EA) and Enneking inappropriate (EI), as defi ned by pathology margin matching Enneking-recommended surgical margins. Prognostic variables were analyzed in reference to local recurrence and survival. Between 1987
and 2012, 58 patients (32 female patients) underwent surgical treatment for primary
spinal osteosarcoma. Patients were followed for a mean period of 3.5 ± 3.5 years
(range 0.5 days to 14.3 years). The median survival for the entire cohort was
6.7 years postoperative. Twenty-four (41 %) patients died, and 17 (30 %) patients
suffered a local recurrence, 10 (59 %) of whom died. Twenty-nine (53 %) patients
underwent EA resection while 26 (47 %) patients underwent EI resection with a
114 Osteosarcoma
383
postoperative median survival of 6.8 and 3.7 years, respectively (p = 0.048). EI
patients had a higher rate of local recurrence than EA patients (p = 0.001). Patient
age, previous surgery, biopsy type, tumor size, spine level, and chemotherapy timing did not signifi cantly infl uence recurrence and survival. Osteosarcoma of the
spine presents a signifi cant challenge, and most patients die in spite of aggressive
surgery. There is a signifi cant decrease in recurrence and an increase in survival
with en bloc resection (EA) when compared with intralesional resection (EI). The
effect of adjuvant and neoadjuvant chemotherapeutics, as well as method of biopsy,
requires further exploration.
Reference
Dekutoski MB, Clarke MJ, Rose P, et al. Osteosarcoma of the spine: prognostic
variables for local recurrence and overall survival, a multicenter ambispective
study. J Neurosurg Spine. 2016;25(1):59–68.
Reference
Makhdoomi K, Kursheed N, Ramzan A, et al. Primary osteosarcoma of the cervical spine.
Neurosurg Q. 2010;20(4):250–2.
Reference