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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
313
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_94
9 4
Costal Osteochondroma Causing Compression Myelopathy
94.1 Definition
Osteochondroma may be a part of hereditary multiple exostosis (HME). It is rare for
it to invade the spinal canal to cause compression myelopathy.
94.2 Incidence
Tang et al reported a case and review the cases from the literature; they found a total
fi ve cases, the male to female ratio being 2:3, and ranging in age 13–21.
94.3 Etiology
HME is a hereditary disease, which is transmitted as autosomal dominant trait.
94.4 Pathology
The tumor is benign and it consists of a bony part and a cartilaginous cap.
Osteochondromas constitute almost half of all benign tumors; the incidence of costal
osteochondroma had been estimated at 3 %; however, this fi gure may be underestimated, as costal osteochondromas are usually asymptomatic.
Abstracted from Tang et al. ( 1998 )
314
94.5 Clinical Picture
Small tumors are usually asymptomatic, bigger tumors can cause pain and swelling.
Extension into the spinal canal causes compression myelopathy, manifested by
motor and sensory compromise and sphincter disturbances.
94.6 Diagnosis
Costal osteochondromas are confi rmed by local exam of the osseous mass and the
neurological signs. Plain radiography does not show the cartilage cap to the tumor.
The intraspinous component of the tumor, the corticomedullary continuity between
the rib and the tumor, and the continuity between the intraspinous and extraspinous
components can only be detected on magnetic resonance imaging (MRI). Computed
tomography (CT) scan can show the same, however cannot reliably detect the cartilage cap if it is less than 25 mm. CT myelography will demonstrate a block at the
level of invasion, however the size of the intraspinal tumor may be underestimated.
94.7 Management
Surgery is done under general anesthesia and incision is done over the rib tumor and
the adjoining spinal cord. The rib tumor is dissected towards the spine. A laminectomy is done and may require facetectomy to give better exposure. The dura is
mobilized and the intraspinal component is traced to the intervertebral formina; the
tumor is removed piecemeal to accomplish a complete decompression of the cord.
There is no recurrence if it is completely removed.
Update
Abstract from Liu et al., 2013
We report the case of a 42-year-old man who presented with kyphosis resulting
from a giant symptomatic costal osteochondromaaround the left fourth rib. The
osteochondroma on the left side of the back was 56 cm × 47 cm × 33 cm and was
painful. The size and growth of the tumor suggested a malignant transformation of a
large costal osteochondroma. Multiple osteochondromas were also found on the
legs. The patient’s family history revealed hereditary characteristics. This patient was
clinically diagnosed as a case of multiple familial osteochondromatosis. Complete
removal of the tumor relieved the symptoms, and histopathologic examination confi rmed malignant transformation of chondrosarcoma. There was no recurrence after
16 months of follow-up.
94 Costal Osteochondroma Causing Compression Myelopathy
315
Reference
Liu W, Kong D, Tang J, Yu F. Giant costal osteochondroma in a man with multiple
exostoses. Ann Thorac Surg. 2013;96(2):675–7.
Reference
Tang WM, Luk KD, Leong JC. Costal osteochondroma. A rare cause of spinal cord compression.
Spine. 1998;23(17):1900–3.
Reference
317
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_95
9 5
Cutaneous Adenocystic Carcinoma
95.1 Definition
Adenocystic carcinoma is a malignant tumor, which arises from the major and
accessory salivary glands. Cutaneous involvement may result from direct infi ltration from salivary glands. Cutaneous adenocystic carcinoma (CACC) remote site of
the salivary glands is very rare.
95.2 Incidence
The fi rst and only case of CACC of the lumbar skin causing compression myelopathy by direct infi ltration was reported by Gelber-González et al.
95.3 Etiology
Compression myelopathy is caused by direct spread of CACC. The tumor is locally
invasive and may metastasize to the lungs, spine, and local lymph nodes.
95.4 Pathology
The tumor cells are arranged in tubular cribriform and adenoidal formation pattern, some have lumens and others are solid. The lumens are fi lled with mucinous
secretions or fi lled with debris or they may be empty. The cells are monomorphic,
with focal atypia and mitotic fi gures. The stroma is fi brous with mucinous changes.
Abstracted from Gelabert-González et al. ( 1999 )
318
The other sites from which this type of tumor may arise e.g., lachrymal glands, the
maxillary and sphenoid sinuses, the breast, the intestinal and genital tracts, and
the bronchus.
95.5 Clinical Picture
The tumor usually arises in the elderly forming a cutaneous mass. In this reported
case it was in the lumbar region invaded the spinal cord and caused lumbar and
radicular pain for 5 year duration. Eventually the patient developed paraparesis with
spasticity and sphincter dysfunction. The mass about two inches and was fi xed in
situ.
95.6 Diagnosis
Diagnosis is based on the following criteria:
• Clinical fi ndings include: mass infi ltration
• Neurological defi cit
• Axial CT: L1 and L2 showed a voluminous isodense mass that extended subcu-
taneously to the spinal canal, occupying it and destroying the laminae and one
pedicle. Severe ventral compression of the conus was noted.
• MRI: A subcutaneous isosignal intensive mass extending to the lumbar epidural
space and compressing the conus.
• Tissue biopsy
95.7 Management
• Complete excision of the tumor down to the dura
• Post-operative radiation therapy: prevent recurrence
• Post-operative chemotherapy: not established however tumors consisting of tubu-
lar pattern of cells respond better than those made of sheets or solid pattern.
Reference
Gelabert-González M, Febles-Pérez C, Martínez-Rumbo R. Spinal cord compression caused by
adjacent adenocystic carcinoma of the skin. Br J Neurosurg. 1999;13(6):601–3.
95 Cutaneous Adenocystic Carcinoma
319
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_96
9 6
Myelopathy due to Intraspinal Desmoid Tumor
96.1 Definition
Desmoid tumor (sporadic aggressive fi bromatosis) is a fi broproliferative tumor that
has benign histologic features and does not metastasize. However, the tumor is
locally invasive, has aggressive clinical behavior and often recurs after excision.
96.2 Incidence
The tumor is rare, estimated incidence is 2–4 per million population per year.
Approximately 50 % occur in abdominal wall, mostly in women at third and forth
decades of life, possibly related to hormone during child bearing years. Thirty-fi ve
percent occur in the limbs, head, and neck. At age less than 15 years, most of the
tumors are extra-abdominal. There is only one case causing myelopathy recorded
by Shindle et al.
96.3 Etiology
No know causative factors. Hormonal relationship is not defi nitive. Female to male
ratio is 5:2; usually during or after pregnancy and normally in the third to fi fth
decade. The tumor arises from the connective tissues of the muscle and overlying
fascia or aponeurosis.
Abstracted from Shindle et al. ( 2002 )
320
96.4 Pathology
The tumor is locally aggressive and does not metastasize. It consists of spindle cells in
loose collagenous matrix, having nuclei. Immunohistochemical stain for the S100 protein is negative and there is no mitosis. It tends to recur locally. In the case of myelopathy, the desmoid is paraspinal and causes scoliosis, and had intraspinous extension
causing compression myelopathy resulting in paraplegia in 12-year-old girl
96.5 Clinical Picture
This is a unique case from which clinical data are abbreviated:
A 12-year-old girl premenarchal, who had back pain and spasms of the legs fol-
lowed by numbness and weakness followed by paresis and sensory level at T1
and sacral sparing. The refl exes were exaggerated and Babinski’s sign was posi-
tive. Radiography of the spine showed scoliosis 70° thoracolumbar region. CT
with IV contrast showed a mass 11 cm in diameter in the left hemothorax extend-
ing from the cranium to the left kidney and was invading the neighboring verte-
bra. MRI showed the mass invading the spinal canal and compressing the spinal
cord. MRI is very important to defi ne tumor border. Surgery was done post-
decompression intralesional anterior excision and anterior fusion. In a second
stage T9-T10 laminectomy was done and intraspinal part of the tumor was deb-
ulked down to the dura. The wound was closed. The patient was put in right lat-
eral decubitus and through a T7 thoracotomy a tumor 5 × 10 cm, was found
subpleurally and wrapping around the ventral body. The tumor was completely
removed and anterior fusion conducted. The pathology showed typical desmoids.
Two weeks later, a spinal fusion was done for the scoliosis, using rods and bone
graph. Neurologic recovery progressed; she had radiotherapy 50.4 Gy. Nine
years later she was free from recurrence.
96.6 Management
• Surgery: wide resection
• Wide resection followed by radiation when the margin is not secure
• In the pediatric population, radiation is a hazard to growth centers are followed
by contractures and potentially causative of malignancy
• Chemotherapy may be useful adjuvant and is indicated in children with inoper-
able tumor and in progressive tumors is slow its growth until the child reaches
bone maturity and the risk of irradiation is decreased
• Surgery and close observation at 2–3 months
96 Myelopathy due to Intraspinal Desmoid Tumor
321
The procedure followed by Shindle et al:
• Posterior decompression
• Anterior resection
• Anterior spinal function to correct deformity
• Post-op radiation for 5 weeks
The tumor is slow growing and extensive in growth, usually painless, giving it a
chance to invade the spinal canal and causing myelopathy.
Reference
Shindle MK, Khanna AJ, Mccarthy EF, O’neill PJ, Sponseller PD. Desmoid tumor of the spinal
canal causing scoliosis and paralysis. Spine. 2002;27(12):E304–7.
Reference
323
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_97
9 7
Intramedullary Ectopic Adrenal Cortical Adenoma
97.1 Definition
Myelopathy secondary to ectopic adrenal cortical neoplasm.
97.2 Incidence
Cassarino et al. ( 2004 ), report the fi rst male case of intramedullary adenoma with
oncocytic features. Two cases of extramedullary oncocytic spinal tumors occurring
in females has been previously been reported.
97.3 Etiology
Ectopic adrenal cortical neoplasms are extremely rare and only a few cases have
involved the central nervous system (CNS). Etiology is unknown. According to
Cassarino et al., The term Oncocytoma was fi rst coined by Hamperl in 1931; onco-
cytomas have been described in the kidney, adrenals, thyroid, and salivary glands,
among other locations. Adrenal cortical oncocytomas have rarely been demonstrated outside of the adrenals, including examples in the retroperitoneum, spinal
nerve roots, and subdural space. Oncocytic tumors involving the CNS are highly
unusual, although oncocytic meningiomas and oncocytic paragangliomas of the spinal cord and cauda equina have been described.
Abstracted from Cassarino et al. ( 2004 )
324
97.4 Pathology
To the naked eye the tumor has a tan to brown color; microscopically the tumor is
composed of sheets and large nests of bland polygonal cells with abundant granular
eosinophilic to clear cytoplasm. Non-atypical cells were found, but no necrosis identifi ed. Cassarino et al., performed electron microscopy (EM) on material collected from
paraffi n-embedded tissue showed numerous mitochondria with tubular and lamelliform cristae, stacks of endoplasmic reticulum (ER), lipid vacuoles, and lipofuscin—
all consistent with adrenocortical cell origin. In the immunohistochemical (IMHC)
confi rmation, the cells were positive for inhibin, melan-A, and synaptophysin, and
negative for GFAP, EMA, cytokeratins, S-100, HMB-45, and chromogranin.
97.5 Clinical Picture
Intramedullary neoplasm manifested by pain followed by hormonal changes. The
patient presented with weakness of the lower limbs, ambulatory loss, paresthesias of
the feet, and sphincter dysfunction. There was analgesia, anesthesia, increased
refl exes and spasticity on the neurological exam.
97.6 Diagnosis
Diagnosis is based on:
• Patient history
• Clinical exam, hormonal changes.
• Immunohistochemical testing: see Pathology
• Laboratory abnormalities: CSF high cellularity, low glucose, high protien
CT was inconclusive.
97.7 Management
In the case presented blockade of the dye column at the conus medullaris level with
obliteration of sacral nerve roots on lumbosacral myelogram. Surgery was performed
and an intramedullary tumor was identifi ed and removed in pieces. The patient is currently doing well with no evidence of recurrent disease approximately 1 year later.
Reference
Cassarino DS, Santi M, Arruda A, Patrocinio R, Tsokos M, Ghatak N, Quezado M. Spinal adrenal
cortical adenoma with oncocytic features: report of the fi rst intramedullary case and review of
the literature. Int J Surg Pathol. 2004;12(3):259–64.
97 Intramedullary Ectopic Adrenal Cortical Adenoma