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337
infarction the patient was put on Simvastatin® and aspirin. Patient refused surgical removal of the fi broelastoma. She was put on standard antiocoagulation medication. In 3 weeks the motor defi cit was not corrected. There was no followup.

Reference

Pello SJ, Ashkenazi A. Spinal cord infarction in a woman with cardiac fi broelas­toma. Neurologist. 2011;17(1):47–8.
Reference
Friedman GR, Pappas TW, Colangelo R, et al. Unusually located left atrial papillary fi broelastoma
with probable embolization to the spinal cord. Echocardiography. 2001;18(6):523–6.
Reference
339
© Springer International Publishing Switzerland 2016 I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord, DOI 10.1007/978-3-319-45147-3_101
101

Gliofibroma

101.1 Definition

Gliofi broma is a neoplasm of mixed mesenchymal and glial components, which develops in the CNS.

101.2 Incidence

22 cases of gliofi broma have been reported in the CNS, 15 cases in the brain and seven in the spinal cord: three cervical, three thoracic, and one thoracic to conus. The male to female ratio is 3:4 and commonly occurs in the fi rst two decades of life.

101.3 Etiology

The origin of the tumor is not clearly known there are theories about the origin:
• prenatal: as demonstrated by Iglagias 11 day old boy and by Windisch et al of an
intramedullary tumor in a 5 month old boy.
• Transformation of preexisting hamartoma, suggested by Reinhardt and Nahser,
however late onset tumors have the same biological features of neonatal
tumors.
• Iglasia-Rozas proposed and aberrant metaplasia in glial cells resulting into mes-
enchymal cells.
• Cerda-Nicholas and Kepes postulate that the neoplastic astrocytes produce
the connective tissue fi bers similar to the prescence of glial and sarcomatous
Abstracted from Matsumura et al. ( 2002 )
340
elements of gliosarcoma having the same genetic alteration of monosomy in
chromosome 10 and 17.
More details of molecular genetic analysis can be seen in the work of Louis et al.

101.4 Pathology

In the spinal cord the tumor is intramedullary well demarcated, most soft yellowish, grayish causing syrinx rostral and caudal. The tumor usually has no extradural extension or new adhesion to the dura. Microscopically, the tumor consists of dense mixture of astrocytic and fi broblastic elements of the mature composition. The nuclei of the tumor cells are round or oval with particular chromatin network. In the care of the tumor there is deposition of retinbin-positive collagenous matrix. Between the gliomatous and fi brous elements the reticulin-positive fi bromatous component is clearly separated from the gliomatous component. Histochemical studies can be seen in the reference paper by Matsumura et al. The tumor is usually a benign astroglial variant.

101.5 Clinical Picture

The tumor is usually encountered in the young. In the spinal cord it behaves like other intramedullary tumors. There is sensory loss of pain and temperature, motor weakness and paresis.

101.6 Diagnosis

Diagnosis is based on:
1. Clinical history
2. Neurological exam
3. MRI of spinal cord: The glial are fi brous elements can be seen as hypo- or iso-
intense mass on T1 enhanced images. The center part of the tumor has hypoin-
tensity on plain images and markedly enhanced with gadolinium. The glial
portion at the periphery shows hypointensity on plain images and slight enhance-
ment with gadolinium. Other routine tests are done especially MRI of the brain.
101 Gliofi broma
341

101.7 Management

The tumor is removed through laminectomy, posterior myelotomy and microdissec­tion. In cases of recurrence or malignant transformation, radiotherapy is to be done. If pathology shows malignant cells radiation is recommended.

Reference

Matsumura A, Takano S, Nagata M, Anno I, Nose T. Cervical intramedullary gliofi broma in a
child: a case report and review of the literature. Pediatr Neurosurg. 2002;36(2):105–10.
Reference
343
© Springer International Publishing Switzerland 2016 I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord, DOI 10.1007/978-3-319-45147-3_102
102

Glioneuronal Tumor

102.1 Definition

This is a tumor composed of two distinct histologic components: one with uniform neurocytes forming rosettes and/or perivascular pseudorosettes, the other compo­nent is astrocytic and resembles pilocytic astrocytoma.

102.2 Incidence

There are 19 cases reported in the fourth ventricle, whereas Anan et al. ( 2009 ) reported the fi rst case in the spinal cord.

102.3 Etiology

Unknown etiology: the tumor may be derived from the gray commissure or the middle motor nuclei in the middle or lower thoracic cord.

102.4 Pathology

The tumor has two components—neurocytic and astrocytic. The neurocytic compo­nent has moderate cellularity. The neurocytes formed neurocytic rosettes; the nuclei are round and small without atypica or mitosis. The cytoplasm has bipolar processes and sometimes joined neighboring rosettes together. At the center of the rosette there is an eosinophilic cell with absence of vessels. The astrocytic component
Abstracted from Anan et al. ( 2009 )
344
shows low to moderate cellularity. The nuclei are round or oval, without atypia or mitosis. The tumor cells have bipolar and spindle processes. No foci or necrosis. For more details about histochemistry see Anan et al. ( 2009 ), where the authors discuss and analyze the fourth ventricle cases.

102.5 Clinical Picture

The cases described was a 44-year-old female presenting disassociate sensory changes of the lower limbs with gradually extending to the upper limbs with weak­ness in all the extremities over a 15 year period. She had two shunts for cervicotho­racic syringomyelia. Neurological fi ndings showed spastic tetraparesis below C7, dysthesia of all senses, and a neurogenic bladder.

102.6 Diagnosis

Diagnosis is based on the following criteria:
1. MRI of the spinal cord: syrinx between C2 and C4. A solid tumor showed low
signal intensity on T1- weighted images and high signal in T2-weighted images
between C2 and T4. A ring enhanced lesion with gadolinium at T4 intradural
level. No calcifi cation or lesion in the fourth ventricle.

102.7 Management

C4-T4 laminectomy was performed; the tumor was removed all intramedullary, except a small portion at T4 was extramedullary. Follow-up showed slight neuro­logical deterioration, without recurrence at 14 months.

Reference

Anan M, Inoue R, Ishii K, et al. A rosette-forming glioneuronal tumor of the spinal cord: the fi rst
case of a rosette-forming glioneuronal tumor originating from the spinal cord. Hum Pathol.
2009;40(6):898–901.
102 Glioneuronal Tumor
345
© Springer International Publishing Switzerland 2016 I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord, DOI 10.1007/978-3-319-45147-3_103
103

Myelopathy in Primary Spinal Epidural Mantle Cell Lymphoma

103.1 Definition

Mantle cell lymphoma was regarded as a variant of intermediate lymphocytic lym­phoma that proliferated as wide mantles around non-neoplastic appearing germinal cen­ters. It is a non-Hodgkin’s lymphoma can affect the central nervous system (CNS) either primarily or with late stage when the disease behavior is aggressive and widespread.

103.2 Incidence

Spinal cord compression occurs in 10.2 % of cases of non-Hodgkin’s lymphomas, which are aggressive. Primary spinal epidural malignant lymphoma is rare and accounts for 3.3 % of all lymphomas and 9 % of all spinal epidural tumors. Primary mantle cell spinal lymphoma is extremely rare, three cases have been reported. Mantle cell tumors account for 2.5–4 % of all non-Hodgkin’s lymphomas. Men are more affected than women 4:1. The median age is 60.

103.3 Etiology

The disease is of uncertain etiology.

103.4 Pathology

The tumor occupies the spinal epidural space with cord compression. Microscopically, it is made of malignant lymphoid tissue containing “naked” germinal centers sur­rounded by diffuse proliferation of neoplastic lymphocytes, small or medium with
Abstracted and reported by Barnard et al. ( 2000 )
346
irregular indented nuclei containing dense chromatin and occasional nucleoli and frequent mitoses. Immunohistochemically expressed B-cell markers CD19 and CD20 and expression of T-cell marker CD5. More details can be found in the article by Barnard et al. In the mantle cell tumor, small lymphoid cells that are atypical grow in the mantle zone in a nodular or diffuse form. The lymphoma cells surround benign or germinal centers, with the normal lymphocyte cuffs. It is thought that the tumor arises in epidural lymphoid rests, but it is not proven.

103.5 Clinical Picture

In the secondary epidural lymphoma there is the full blown picture of lymphoma: lymphadenopathy, splenomegaly, and bone marrow infi ltration, etc. In the primary lymphoma it is manifested by cord compression: sensory and motor loss, spasticity, hyporefl exia or arefl exia, and sphincter dysfunction. However, after biopsy or exci­sion of the tumor recurrence and full picture will be manifested. The prognosis is poor in either case.

103.6 Diagnosis

Diagnosis is based on the following criteria:
Confi rmation is made with tissue biopsy and by other sites of lymphomatous depos-
its. If the lesion is primary as it is entitled it is a challenge for diagnosis until
tissue examination. MRI shows epidural mass usually lumbosacral, the vertebra
may be involved, so also paravertebral soft tissues. The mass described in this
third reported case was of homogeneous signal intensity and exhibited mild dif-
fused enhancement with gadolinium. After incomplete resection there was recur-
rence and open picture of mantle cell lymphoma.
Neuroradiologically the tumor may simulate metastasis, plasmacytoma, epidural schwannoma and other tumors; therefore, thorough differential diagnosis needs to be performed.

103.7 Management

• Surgery: Laminectomy and resection for histopathology
• Radiation therapy
• Chemotherapy using cyclophosphomide, vincristine, and prednisone.
This tumor has aggressive behavior and poor response to therapy and therefore poor outcome.
103 Myelopathy in Primary Spinal Epidural Mantle Cell Lymphoma
347

Reference

Barnard M, Perez-Ordoñez B, Rowed DW, Ang LC. Primary spinal epidural mantle cell lym-
phoma: case report. Neurosurgery. 2000;47(5):1239–41.
Reference
349
© Springer International Publishing Switzerland 2016 I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord, DOI 10.1007/978-3-319-45147-3_104
104

Malingnant Eccrine Poroma

104.1 Definition

Malignant sweat gland tumors are extremely rare, representing less than 0.01 % of all primary skin lesions. Malignant eccrine neoplasms can be divided into two main categories:
• Eccrine adenocarcinoma developing de novo in normal skin
• Eccrine adenocarcinoma arising in pre-existing sweat gland benign tumor (less
common)
The fi rst group is subdivided into fi ve categories as follows:
1. malignant eccrine porocarcinoma
2. syringoid (tubular) eccrine carcinoma
3. mucinous eccrine carcinoma
4. clear cell carcinoma
5. microcytic eccrine carcinoma
Eccrine porocarcinoma is the most common constituting for approximately half of all eccrine adenocarcinoma.

104.2 Incidence

Magdum et al., reported the fi rst case of myelopathy secondary to malignant eccrine poroma.
Abstracted from Magdum et al. ( 2001 )