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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
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104.3 Etiology
The cause of carcinoma is not known, it is theorized that it may be develop after
irradiation
104.4 Pathology
The tumor is usually ulcerated and nodular arising from the epidermis and growing
into the dermis. It is usually composed of anastomosing trabeculae of epithelial
cells embedded in glycosaminoglycan-rich matrix. In many areas the cells show
ducts containing periodic acid-Schiff-positive material strongly suggestive of
eccrine nature. This histiopathology is seen in both the skin tumor and its metastasis
in the vertebral column, namely malignant eccrine poroma. The tumor shows up in
patients around 60 years of age, only fi ve cases below forty. Ten percent metastasized to the lymph nodes. It usually presents as localized nodule, a plaque, a verucous ulcer. The skin lesions frequently arise from the limbs, head, and neck, with
itching, bleeding, and pain.
104.5 Clinical Picture
From Magdum et al., case Patient was a 57-year-old male, who had a history of
chronic back pain and urinary hesitancy. Patient had an episode of psychosis and
was subsequently diagnosed with hydrocephalus, which a ventriculoperitoneal
shunt was made. The skin showed an ulcer over the left mandible, which bled on
touch. Cerebral spinal fl uid (CSF) showed malignant cells, he then developed weakness of the lower limbs. Magnetic resonance imaging (MRI) showed metastasis in
T4 and L3 and compression. Decompression laminectomy was performed with a
pedicular fi xation. The bone biopsy and skin tumor which was of the same nature,
i.e. that of malignant eccrine poroma. Patient developed pulmonary infection and
died 2 months later. No autopsy was performed. The tumor was not radiosensitive.
A new therapy is suggested isotretinoin and α-interferon for the treatment of the
metastatic lesions.
Conclusions
Patient had malignant eccrine poroma in the neck that metastasized, leading to
malignant meningitis, resulting in obstructive hydrocephalus and causing com-
pression myelopathy. This suggests that malignant skin lesions should be dealt
with as soon as possible, before metastasis to the central nervous system, which
can produce a fatal outcome.
104 Malingnant Eccrine Poroma
351
Reference
Magdum S, O’Brien D, O’Reilly G, Crooks D. Malignant eccrine poroma with spinal and cerebral-
spinal fl uid matastases: case report. Neurosurgery. 2001;49(4):1004–7.
Reference
353
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_105
105
Melanoma
105.1 Definition
Melanomas are tumors that arise in the organs or structures that have melanin producing cells.
105.2 Incidence
To date there is less than 60 cases of primary malignant melanoma of the spinal
cord. Before 1968, 26 cases of primary malignant melanomas. An average of one
case every 2 years. The mean age in the fi rst twenty cases before 1960 was 50 years
(range 15–80 years). Later, the mean age was 46 years (25–71 years. it involves
more females than males, male to female ratio: 1:1.36. Thoracic is commonest, followed by cervical.
105.3 Etiology
Melanomas can arise in any organ which contains melanin-producing cells, e.g.
skin, chromaffi n tissue, leptomeninges, etc. Melanocytes are cells that arise from
the neural crest during embryogenesis, and migrate to the skin, mucosa, and
CNS. The exact etiology is not clear, theories include:
• Primary melanoma of the CNS arises from melanoblasts accompanying the pia
sheets or vascular bundles
Abstracted from Yu et al. ( 2012 )
354
• Melanoma may arise from the neuroectodermal cells during embryogenesis.
More authors are in favor of this theory, since it suggests genetic origin and may
suggest a genetic therapy, but this is only hypothetical.
105.4 Pathology
From the case reported:
The mass was subdural, measuring 5.3 × 1.8 × 1.1 cm with some adherence to the
pia mater, the mass was black with intact capsule. Microscopically, fusiform and
epithelial cells with deposition of melanin granules, growing around vessels. After
depigmentation, vesicular nuclei and red staining nucleoli were seen. The cells
stained with S100 protein and malignant melanoma monoclonal antibody HMB45.
105.5 Clinical Picture
The case presented by the authors:
A 48-year-old Chinese man presented with progressive numbness of the upper
and lower extremities for 6 months. He had neck and shoulder pain for 1 month.
Physical exam was normal except for hypothesia C3-T1 bilaterally. MRI of the
cervical spine showed a space occupying lesion, C2-C6 with hyperintense lesion on
T1 weighted image and hypointense signal on T2 weighted image. After gadolinium
injection, hyperintense lesion T1 weighted images and showed cord compression.
MRI of the brain lesion showed multiple lesions, especially on the surface and in the
cisterna magna and the sula, hyperintensive on T1 weighted images. There was no
mass effect and edema of the brain.
105.6 Management
Surgery consists of:
1. Laminectomy of C2–C6, duratomy, black mass was identifi ed and was incom-
pletely removed. Frozen and permanent sections sowed malignant melanoma.
The case was considered terminal and the patient died 2 months later.
Updates
Hering et al. 2016
Only four cases of primary intradural extramedullary spinal cord melanoma
(PIEM) have been reported. No previous reports have described a case of PIEM
105 Melanoma
355
located in the lower thoracic spine with long-term follow-up. Demonstrating an
unusual, extremely rare case of melanoma manifestation.
The authors report a case of a 57-year-old female suffering from increasing lower
extremity pain, left-sided paresis, and paraesthesia due to spinal cord compression
caused by PIEM in the lower thoracic spine. Extensive investigation excluded other
possible primary melanoma sites and metastases. For spinal cord decompression,
the tumor at level T12 was resected, yet incompletely. Adjuvant radiotherapy was
administered 2 weeks after surgery. The patient was recurrence-free at 104 weeks
after radiotherapy but presents with unchanged neurological symptoms. Primary
intradural extramedullary melanoma (PIEM) is extremely rare and its clinical
course is unpredictable.
Reference
Hering K, Bresch A, Lobsien D, Mueller W, Kortmann RD, Seidel C. Primary
Intradural Extramedullary Spinal Melanoma in the Lower Thoracic Spine. Case
Rep Oncol Med. 2016;2016:3815280.
Reference
Yu J, Zhao DD, Chen S, Zhang JM, Xu J. Primary melanoma of the cervical spine with cerebral
metastases: case report and review of the literature. J Int Med Res. 2012;40(3):1207–15.
Reference
357
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_106
106
Intramedullary Meningioma of the Cervical Spinal Cord
106.1 Definition
This is a meningioma within the structure of the spinal cord.
106.2 Incidence
Five cases have been reported and the authors added the sixth. Of the six reported
cases fi ve were female. Salehpour stated, “Has a predilection to the female gender
during the fi fth to seventh decade of life.”
106.3 Etiology
The causative factors are not clear; however, possible risk factors include:
• Genetic mutations
• Trauma
• Viral infection
• Irradiation
106.4 Pathology
The tumor arises mostly in the cervical cord, is well defi ned with edema around it.
Histopathology shows syncytial tumor, WHO classifi cation grade I meningioma.
Histochemical studies show positive staining for epithelial membrane antigen and
Abstracted from Salehpour et al. ( 2008 )
358
S100 protein. The reported tumor were cervical proximally, one invaded the medulla
oblongata, and one started at the cervicomedullary junction. Their pathology was
diverse: one agioblastic psammomatous, papillary fi broplastic, transitional, clear
cell, and one meningiothelial (syncytial).
106.5 Clinical Picture
Symptoms varied from burning pain in the neck, in the upper limbs, dysthesia of the
upper limbs, paresthesia in the four limbs, numbness in the limbs, spastic gait, and
hemiparesis or spastic paraplegia. Sensory changes of spinothalamic or posterior
column compression and sphincter dysfunction.
106.6 Diagnosis
Diagnosis based on:
1. Clinical picture and neurological exam
2. MRI (from Salehpour et al.): a sagittal T2 and T1 weighted-images of the cervi-
cal cord revealed isointense and isointense to hyperintensive intramedullary
C1-C2 tumor related expansion of the cord and a developed syringomyelia.
Edema of the cord at the distance between the tumor and the syrinx. Sagittal T2
weighted-images at the cervicomedullary junction showed extension of the
tumor. The tumor is homogenous enhanced.
3. Electrophysiological studies
106.7 Management
Total resection of the tumor is ideal to prevent recurrence. However, its intramedullary position and extension into the medulla oblongata makes it not ideal outcome.
The case recorded was approached through combined suboccipital craniotomy and
C1–C2 laminectomy. Microsurgical techniques were utilized and the tumor was
subtotally removed by applying Cavitron ultrasound surgical aspirator. Three years
post-op there was no recurrence, the authors recommended close followup with
MRI annually after a period of 2 years with closer observation every 3–6 months.
U p d a t e
Abstract from Perven et al. 2015
A 52-year-old man with a 7-year history of progressive weakness, gait problems, and pain in his extremities presented with subacute worsening of his
106 Intramedullary Meningioma of the Cervical Spinal Cord
359
symptoms. Examination revealed weakness in all four extremities, increased
tone, hyperrefl exia, and sensory defi cits. MRI of the cervical spine showed an
area of signal abnormality and abnormal enhancement within the cervical cord
at the C5–C6 level. The patient initially underwent biopsy followed a few days
later by a debulking surgery. Postoperatively, the patient showed improvement
in strength as well as ambulation. Intraoperatively, the lesion was confi rmed to
be intramedullary without any dural attachments. Histopathological examination revealed an extensively hyalinized tumor with sparse collections of cells
that were immunopositive for both cytokeratin and GFAP, and immunonegative
for EMA and progesterone receptor. This is an unusual pattern of expression,
with cytokeratin immunopositivity suggesting a meningioma and GFAP immunopositivity suggesting a glioma. Considering the combination of extensive
hyalinization with cytokeratin positivity the tumor was thought to be most consistent with a hyalinized meningioma with GFAP positivity. GFAP-positive
meningiomas are rare, and these include the recently described ‘whorling-sclerosing’ variant. Only three cases of this tumor have been previously reported, all
of which were intracranial. This is the fi rst reported case of an intramedullary
whorling-sclerosing meningioma.
Abstract from Piazza et al. 2016
Reference
Perven G, Entezami P, Gaudin D. A rare case of intramedullary ‘whorlingsclerosing’ variant meningioma. Springerplus. 2015;4:318.
Spinal meningiomas are typically extra-axial, slow-growing, benign tumors
that arise from the arachnoid cap cells. Intramedullary spinal meningiomas are
exceedingly rare with few cases reported in the literature. A 64-year-old man
with a history of grade I thoracic meningioma at the T4 level resected initially
in 1989 and who required reoperation in 2013 for intradural, extramedullary
recurrence of tumor presented again in 2015 with gait diffi culty. Magnetic resonance imaging revealed a soft tissue mass at the T3 to T4 levels on the left side
of the canal that was mildly enhancing on T1 contrasted sequences. The patient
was taken to the operating room, where a purely intramedullary recurrence was
discovered without extramedullary extension or a dural-based attachment. The
intramedullary tumor was completely resected, and postoperatively the patient
recovered well and was at his neurologic baseline. The patient ultimately underwent proton beam radiotherapy because this tumor, although benign, had
recurred twice. Intramedullary spinal meningiomas, particularly intramedullary
low-grade recurrence of a previously extramedullary tumor, are rare phenomena. Although the pathogenic mechanisms are not well understood, intramedullary recurrence as described in this patient may refl ect extrinsic factors related
to prior surgical resections in addition to histologic progression. When operating on recurrent extramedullary lesions, aggressive arachnoid dissection may
predispose patients to unusual patterns of recurrence.
Update
360
Reference
Piazza MA, Ramayya AG, Geiger GA, et al. Intramedullary Recurrence of a
Thoracic Meningioma-Presentation of an Unusual Case and Review of the
Literature. World Neurosurg. 2016; Feb 4. pii: S1878-8750(16)00161-3.
Reference
Salehpour F, Zeinali A, Vahedi P, Halimi M. A rare case of intramedullary cervical spinal cord
meningioma and review of the literature. Spinal Cord. 2008;46(9):648–50.
106 Intramedullary Meningioma of the Cervical Spinal Cord
361
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_107
107
Metastatic Retinoblastoma (Pediatric)
107.1 Definition
This is a condition of intraspinal metastatic retinoblastoma causing compression
myelopathy.
107.2 Incidence
Extra ocular retinoblastoma metastasis to the central nervous system (CNS) is in
general not rare. However, compression myelopathy caused by metastatic tumor is
very rare. Tubran et al., reported 207 cases treated between 1991 and 1999 that had
extra-ocular disease, but none of them involved the spinal cord. Kao et al., studied
96 retinoblastomas cases treated between 1978 and 2000 and found 27 had CNS
metastasis, but again none involved the spinal cord. In another study spinal cord
compression (SCC) from other malignancies is not rare. Chang et al., reviewed the
literature from 1966 to 2004 and found only 5 cases of SCC and added two cases of
their own, although one case was very limited.
107.3 Etiology
Retinoblastoma is a rare tumor caused by an autosomal dominant mutation (about
6 %). The gene product of the retinoblastoma gene is a tumor suppressor that interacts with transcription factors such as to block transcription of growth regulating
genes. The Rb gene plays a role in normal development, not just that of the retina.
Abstracted from Chang et al. ( 2006 )