Sacrococcygeal extraspinal ependymomas: the role of coccygectomy

BACKGROUND: Ependymomas, the common glial tumors of the spinal cord, occur occasionally outside the central nervous system and are called exstraspinal ependymomas (EEP). EEPs are found primarily in sacrococcygeal region during childhood. The pathogenesis and the treatment of the sacrococcygeal (SC) ependymomas are still controversial. Therefore, we present our case with metaanalysis of other case reports to determine the optimal treatment modality for SC EEPs. METHODS: A metaanalysis of case reports of SC EEPs, including the current case, was conducted. Also all available case reports of EEPs, without age limit, were analyzed to determine the distribution of EEPs localization. RESULTS: EEPs usually are found in teratoma localizations such as the SC area, ovary, paraovarian structures, and medastinum. The distribution of EEPs localization differs with age. Local recurrence rate of EEPs after coccyx excision is zero, however, it increases to 71% when the coccyx was left behind. CONCLUSION: The identical clinical characteristics of the SC teratomas and EEPs imply that the SC EEPs may be monophasic teratomas as their ovarian counterparts are named. Coccyx excision is an important part of the surgical treatment of these tumors, with an apparent decrease in the recurrence rate.     

Sacrococcygeal ependymoma: case report and review of the literature.

Ependymomas are uncommon glial cell tumors found within the brain, spinal cord, cauda equina, or filum terminale. Ependymomas originating in the parasacral soft tissues are exceedingly rare. The authors report the case of a 7-year-old boy with a primary ependymoma of the sacrococcygeal area and review the world literature of this tumor at this location.     

A sacrococcygeal extraspinal ependymoma in a 67-year-old man: a case report and review of the literature.

Extraspinal ependymomas are extremely uncommon tumours of glial origin. They occur predominantly in children and adolescents. We report a case of a subcutaneous extraspinal ependymoma in a 67-year-old man. This was excised, and the defect reconstructed with a V-Y advancement flap.     

Sacrococcygeal chordoma metastatic to penis

A rare case of sacrococcygeal chordoma metastatic to the penis is presented The symptoms, diagnosis, and management of sacrococcygeal chordoma are discussed.     

Sacrococcygeal chordoma: clinicoradiological study

A case of sacrococcygeal chordoma is presented. We review the literature and we discuss the problems related to the etiology, the symptoms, the diagnosis and the treatment of this rare neoplasm. We present and discuss here the importance and the different possibilities of new diagnostic techniques, such as the CT and the MRI in the diagnosis and management of sacrococcygeal chordoma.     

Sacrococcygeal teratoma

Two cases of sacrococcygeal teratomas are presented. One case illustrates the long course of urologic problems produced by a "successful" removal of the tumor. The literature is reviewed, and the characteristics of this congenital tumor are discussed.     

Sacrococcygeal ganglioneuroma

Ganglioneuromas are benign slow-growing masses that can be treated with complete surgical extirpation without any adjuvant therapy. Such lesions involving the sacrococcygeal region are exceedingly rare. The authors present the case of a 70-year-old woman with a sacrococcygeal ganglioneuroma treated by total en bloc resection. This patient also had a previous coccygeal fracture. To the authors' knowledge, there are no other reports of ganglioneuroma in association with a history of trauma.     

Neurological complications of extraspinal.

The anatomy and physiology of the blood supply to the central nervous system from the aorta is outlined. Pertinent cases are reported, to illustrate the effect of impairment of this blood supply in producing ischemia of the brain, spinal cord, and peripheral nerves. In patients with such neurologic disease, especially if sudden in onset, a search for a circulatory basis in lesions of the aorta or its branches may be rewarding and crucial. With regard to reconstructive surgery of the aorta, certain precautions may be taken: maintenance of normotension, gentle dissection, preservation of segmental arteries when possible, bypass shunting, avoidance of prolonged aortic clamping and perhaps heparinization. Unfortunately, depending upon deficiencies in collateral circulation to the spinal cord, occasional cases of postoperative paraplegia will still occur, which are both unavoidable and unpredictable.     

Epithelioid ependymoma: a new variant of ependymoma: report of three cases

OBJECTIVE: To describe the pathological features of three very similar and unusual primary central nervous system tumors that are not readily recognized as conventional ependymomas but which, by ultrastructural examination, have an ependymomatous character. METHODS: Three distinctive tumors were found in a review of our files for cases of ependymoma. In each case, hematoxylin and eosin-stained sections were reviewed, and immunostains for epithelial membrane antigen, cytokeratin, vimentin, and glial fibrillary acidic protein were performed on formalin-fixed, paraffin-embedded sections. Electron microscopy was performed in each case. RESULTS: The tumors had a diffuse myxoid background, often containing tightly clustered cells that mimicked multinucleated giant cells, but lacking perivascular pseudorosettes or central lumen rosettes. Glial fibrillary acidic protein and vimentin immunostains did not reveal perivascular processes. Epithelial membrane antigen immunostains showed a dot-like cytoplasmic immunoreactivity in some cell clusters in two of the three cases. Cytokeratin was negative in all three cases. However, ultrastructurally, the cells of each tumor had extensive surface microvilli; the giant cell-like clusters had cells with extensive close appositions, some junctions, and, in two cases, lumina with microvilli. Two of the patients were adults (both with temporal lobe tumors), and one patient was 13 years old and had a cervical spinal cord intramedullary tumor. Each tumor was sharply circumscribed from adjacent central nervous system tissue but was not encapsulated. One of the cases in an adult was mitotically highly active; this tumor recurred locally 4 years after initial gross total excision. CONCLUSION: These tumors are unusual variants of ependymoma. This pattern of ependymoma is sufficiently distinctive to be recognized in hematoxylin and eosin stains once the architecture of the epithelioid clusters is appreciated.     

Sacrococcygeal dysgenesis.

Congenital sacrococcygeal dysplasia is an uncommon though important cause of neuropathic bladder disease leading to progressive renal damage. In 2 cases described herein and featuring long-standing incontinence and recurrent infections, the concomittant musculoskeletal abnormalities had been treated for years, while the urinary tract condition had been largely neglected until urinary diversion or unilateral nephrectomy were required.