Lipoblastoma with unique localization requiring tracheal and esophageal resection

Lipoblastoma and lipoblastomatosis are rare tumors of infancy. They originate from embryonic fat and localize in soft tissues. We present the case of a lipoblastoma of the neck with localization in tracheal and esophageal walls that required an extended laryngotracheal and esophageal resection. To our knowledge, this is the first report of such localization of this tumor.     

Nonenhancing tumors of the spinal cord

OBJECT: Enhancement of pathological entities in the central nervous system is a common finding when the blood-brain barrier has been compromised. In the brain, the presence or absence of gadolinium enhancement is often an indicator of tumor invasiveness and/or grade. In the spinal cord, however, contrast enhancement has been shown in all tumor types, regardless of grade. In this study the authors explore the incidence of nonenhancing tumors of the spinal cord and the clinical course of patients with these lesions. METHODS: A retrospective analysis was conducted in which investigators examined the patterns of enhancement of histologically proven intramedullary spinal cord tumors that had been evaluated at the Mayo Clinic between 1998 and 2002. The tumors that did not enhance were the subject of this report. RESULTS: A total of 130 patients with intramedullary tumors were evaluated. Of those, 11 patients (9%) had tumors that did not enhance. Histologically, a majority of tumors were astrocytomas (eight low-grade and two high-grade lesions); one tumor was a subependymoma. Morphologically, most of the tumors were diffuse and none had associated cysts. Tumors spanned from two to seven levels and were located throughout the spinal cord (four cervical, three cervicothoracic, one thoracic, and three thoracolumbar). Biopsy procedures were performed in eight patients, subtotal resection was performed in two, and gross-total resection in one. After a mean follow-up period of 19 months, tumors remained stable in eight patients but progressed in three, two of whom died. CONCLUSIONS: A number of intramedullary spinal cord tumors will not enhance after addition of contrast agents. The absence of enhancement does not imply the absence of tumor.     

Lipoblastoma in a 14-year-old boy: Report of a case

Lipoblastoma is a rare benign neoplasm of embryonal fat which occurs primarily in infants and young children. Since 1958, lipoblastoma has been recognized as an entity distinct from other benign or malignant tumors of the adipose tissue. This tumor presents in two forms: as a localized, well-circumscribed lesion known as lipoblastoma, or as a multicentric type of lesion known as lipoblastomatosis. The oldest patient documented in the English literature was 8 years of age; however, we report herein the case of a 14-year-old boy in whom a lesion excised from the left thigh was histopathologically confirmed to be a lipoblastoma.     

Head and neck lipoblastomas: Report of 3 cases and review of the literature

IntroductionLipoblastoma is a rare benign tumor arising from embryonic white fat which occurs in the early childhood. It usually arises on the extremities and considered as a rare cause of a pediatric head and neck masses. The aim of this study is to shed light on lipoblastomas as a differential diagnosis of rapidly growing soft fatty masses of children in neck and head area.Patients and methodsA retrospective review of 3 patients with lipoblastoma, underwent Surgical resection (case 1 and 2) by cervical approach. The third patient with a facial lipoblastoma was not operated due to the high risk of facial paralysis. Review of literature, diagnostic methods and genetics of lipomatous tumors are discussed.ResultsComplete surgical excision via a cervical approach demonstrated irregular lobules of immature fat cells separated by a loose, myxoid connective tissue. Histology analysis confirmed the diagnosis of lipoblastoma.DiscussionLipoblastoma is a rare childhood tumor, even rarer in head and neck area. The pathogenesis is unknown, though it is believed to arise from altered embryogenesis of human white fat and genetic predisposition, as chromosome 8 abnormalities may be implicated in the development of lipoblastoma. The presumptive diagnosis is performed by imaging. The most important differential diagnosis of lipoblastoma is myxoid liposarcoma. The mainstay of treatment is complete non-mutilating resection of the tumor to avoid recurrence.ConclusionLipoblastoma should be suspected in case of heterogeneous fatty tumor in head and neck area, and included as a differential diagnosis of cervical masses in children younger than 3 years. The mainstay of treatment is complete surgical excision with a good prognosis.     

Metastasis of carcinoid to the arch of the axis in a multiple endocrine neoplasia patient: a case report

BACKGROUND CONTEXT: Carcinoid tumors eventually metastasize to the spine, and epidural spinal cord compression is a relatively frequent neurologic complication of carcinoid. However, a case of multiple endocrine neoplasia type 1 (MEN1) presenting with spinal cord compression as a result of a metastatic carcinoid tumor has not been reported previously. PURPOSE: To report an extremely rare case of MEN1 presenting with spinal cord compression by metastatic carcinoid tumor. STUDY DESIGN: Case report. METHODS: A 51-year-old man, with a past history of thymoma, insulinoma, and gastric carcinoid presented with neck pain. Neuroradiological examination revealed that a tumor around the arch of the axis compressed the spinal cord with osteoblastic changes. RESULTS: After hemilaminectomy of the axis and removal of the tumor followed by irradiation, the patient returned to his previous job. Histological examination confirmed metastatic carcinoid tumor. CONCLUSIONS: Spinal metastasis of carcinoid tumor occurred in a multiple endocrine neoplasia patient, and it is significant to note that carcinoid metastasis is one of differential diagnoses for osteoblastic lesions.     

Spinal cord mapping as an adjunct for resection of intramedullary tumors: surgical technique with case illustrations

OBJECTIVE: Resection of intramedullary spinal cord tumors may result in transient or permanent neurological deficits. Intraoperative somatosensory evoked potentials (SSEPs) and motor evoked potentials are commonly used to limit complications. We used both antidromically elicited SSEPs for planning the myelotomy site and direct mapping of spinal cord tracts during tumor resection to reduce the risk of neurological deficits and increase the extent of tumor resection. METHODS: In two patients, 3 and 12 years of age, with tumors of the thoracic and cervical spinal cord, respectively, antidromically elicited SSEPs were evoked by stimulation of the dorsal columns and were recorded with subdermal electrodes placed at the medial malleoli bilaterally. Intramedullary spinal cord mapping was performed by stimulating the resection cavity with a handheld Ojemann stimulator (Radionics, Burlington, MA). In addition to visual observation, subdermal needle electrodes inserted into the abductor pollicis brevis-flexor digiti minimi manus, tibialis anterior-gastrocnemius, and abductor halluces-abductor digiti minimi pedis muscles bilaterally recorded responses that identified motor pathways. RESULTS: The midline of the spinal cord was anatomically identified by visualizing branches of the dorsal medullary vein penetrating the median sulcus. Antidromic responses were obtained by stimulation at 1-mm intervals on either side of the midline, and the region where no response was elicited was selected for the myelotomy. The anatomic and electrical midlines did not precisely overlap. Stimulation of abnormal tissue within the tumor did not elicit electromyographic activity. Approaching the periphery of the tumor, stimulation at 1 mA elicited an electromyographic response before normal spinal cord was visualized. Restimulation at lower currents by use of 0.25-mA increments identified the descending motor tracts adjacent to the tumor. After tumor resection, the tracts were restimulated to confirm functional integrity. Both patients were discharged within 2 weeks of surgery with minimal neurological deficits. CONCLUSION: Antidromically elicited SSEPs were important in determining the midline of a distorted cord for placement of the myelotomy incision. Mapping spinal cord motor tracts with direct spinal cord stimulation and electromyographic recording facilitated the extent of surgical resection.     

颈胸段脊柱肿瘤的外科治疗

目的 观察比较不同手术入路方法治疗颈胸段脊柱肿瘤的疗效。方法 本组９例,共１０例次。对其中４例肿瘤同时累及椎体及椎体后部结构的患者,选择前后路同期手术,经前路切除肿瘤、椎管减压及钢筋骨水泥或前路钢板固定,并同期行后路肿瘤切除术,其中１例行Ｌａｕｑｕｅ棒固定;对４例肿瘤仅累及椎体者,选择经前路切除肿瘤、椎管减压、钢筋骨水泥固定;另２例肿瘤单纯累及椎体后部结构者,经后路行肿瘤切除和椎管减压术。术后４例     

Intradural extramedullary capillary hemangioma of lower thoracic spinal cord

Capillary hemangiomas are benign tumors and tumor like conditions commonly involving skin and mucus membrane of head and neck region. They are extremely rare in the spinal cord. We report a 35-year-old male presenting with gradual progressive paraparesis over a period of 4 months. Magnetic resonance imaging showed a hypo- to isointense intradural mass at the level of D12 vertebral body on T1-weighted images and homogenous enhancement on gadolinium contrast. Complete surgical resection revealed intradural extramedullary tumor, which on histopathologic examination showed characteristics of capillary hemangioma. At 1.5 years followup patient was asymptomatic.     

Operative ultrasonography of the brain and spinal cord pathology

B-mode real-time ultrasound using 5 or 7.5 MHz transducer has been employed during 21 operations for brain pathology and spinal cord lesions. Ultrasonic scanning was performed at the following operations: 10 brain tumors (4 glioblastomas multiforme, 2 astrocytomas, 1 medulloblastoma, 2 metastatic tumors), 2 brain cysts (arachnoid, epidermoid), 1 tuberculous abscess, 3 cerebral hematomas: 2 spinal cord tumors (malignant melanoma, glioma), 2 syringomyelias, 1 posterior longitudinal ligament thickening. Operative ultrasound was useful prior to dural incisions and particularly for subcortical lesions. In addition, ultrasound provided assistance at spinal cord surgery. Our experience has been reviewed and summarized in this report in terms of specific usefulness of assistance of this method which has proven helpful to the neurosurgeons. The types of assistance provided by operative ultrasonography include: Location of dural incision. Localization of brain and spinal cord lesions prior to biopsy. Diagnosis which has not been made preoperatively (e.g. necrosis or cystic area in tumor). Consistency of each lesion (e.g. solid or cystic, necrosis, loculation). Size, extent and depth of brain tumor, cyst, abscess and hematoma. Presence and extent of spinal cord syrinx. Relation of tumor to spinal cord and dura. Access route for biopsy and drainage (avoiding critical areas such as motor strip). Exclusion of bleeding or hematoma following biopsy. Confirmation of the effectiveness of drainage or resection of lesions. Relationship between pathology and surrounding anatomic structures. A number of important assistance by the utilization of ultrasound during neurological surgery have been identified.(ABSTRACT TRUNCATED AT 250 WORDS)     

Primary holocord ganglioneuroblastoma: case report

The authors present a case of extensive primary intramedullary spinal CNS ganglioneuroblastoma (GNB) in a 23-year-old man. Central nervous system GNB is a poorly differentiated neuroepithelial tumor composed of neuroblasts and differentiated ganglion cells, and these lesions are extremely uncommon. Most previously reported primary intraaxial neuroblastic tumors were described in the brain. There has been only one other report of primary spinal cord CNS GNB published to date; the clinical course and prognosis for primary spinal cord tumors of this type are unknown. Similar tumor types demonstrate poor prognoses. This 23-year-old man presented after 9 months of progressive myelopathy. Admission MR imaging showed an intraaxial enhancing mass extending from C-3 to the conus medullaris, with a holocord appearance in several areas. Due to the tumor size, operative intervention was initially limited to biopsy sampling. Chemotherapy resulted in histological maturation, but initial tumor regression was temporary. The patient suffered progressive quadriparesis, and neuroimaging demonstrated slow enlargement of the tumor and an associated syrinx. Nineteen months after diagnosis, the tumor was excised to gross-total resection in a 2-stage operation. One year following resection, the patient had no radiographic recurrence and was functional in a wheelchair with minimal paresis in the upper extremities. This case represents the most extensive example of primary spinal intramedullary CNS GNB reported to date. Holocord tumors present a significant challenge to the neurosurgeon, and resection bears substantial risk of morbidity. In spinal cord CNS GNB, chemotherapy followed by complete resection may be the most effective means of tumor control.     

Intramedullary spinal teratoma presenting with urinary retention: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Intramedullary tumors affect a small but significant segment of patients with spinal cord tumors. Intramedullary teratomas are rare entities that are usually located in the sacrococcygeal region. Although some reports claim that diagnostic studies can exactly predict the nature of intramedullary tumors, this case report demonstrates the lack of preoperative diagnostic specificity. Therefore, the aim of surgery should be radical extirpation whenever possible. CLINICAL PRESENTATION: A case report of a thoracolumbar intramedullary teratoma is presented. This young male patient displayed urinary retention and motor deficits. Computed tomographic and magnetic resonance imaging examination revealed an intramedullary tumor; however, the exact nature of the tumor could not be determined preoperatively. The literature concerning these tumors is extensively reviewed. INTERVENTION: During surgery, a multicystic tumor was found at the base of the filum terminale, which was resected as far as possible. CONCLUSION: It is concluded that surgery should be the first choice for treatment. More aggressive teratomas are found in intramedullary locations, especially in children, emphasizing the importance of radical tumor resection. The use of adjuvant chemo- or radiotherapy should be explicitly substantiated for the nonbenign teratoma group.     

Cervical sympathetic schwannoma: case report

OBJECTIVE AND IMPORTANCE: Approximately 20 to 27% of neck masses in the parapharyngeal space may be benign nerve sheath tumors. Cervical sympathetic chain schwannomas or vagal nerve schwannomas are most common. We report a patient with a cervical sympathetic chain schwannoma. CLINICAL PRESENTATION: The patient was a 47-year-old Caucasian man with a 7-month history of a painless, enlarging right neck mass. No hoarseness or Horner's syndrome was found preoperatively. Formal head and neck examination in the otolaryngology department revealed no vocal cord dysfunction. INTERVENTION: By use of a standard right carotid incision, a tumor was identified growing from the cervical sympathetic chain posterior to the carotid bifurcation. A gross total resection was performed and a section of the cervical sympathetic chain was sacrificed. CONCLUSION: Cervical sympathetic chain schwannomas are unusual tumors that require microneurosurgical resection for cure. We discuss the differential diagnosis, evaluation, surgical management, and pathological characteristics of such tumors and review the literature.     

Midline ventral intradural schwannoma of the cervical spinal cord resected via anterior corpectomy with reconstruction: technical case report and review of the literature

OBJECTIVE AND IMPORTANCE: Spinal cord schwannomas are intradural nerve sheath tumors that almost universally occupy a dorsolateral, lateral, or ventrolateral position. Therefore, resection of these lesions typically proceeds via a posterior or posterolateral approach. CLINICAL PRESENTATION: We present a case of a midline ventral intradural schwannoma of the cervical spinal cord causing myelopathy. To the best of our knowledge, no previous reports specifically discuss purely midline ventral intradural schwannomas. INTERVENTION: Resection of the tumor was performed via an anterior cervical corpectomy with spinal arthrodesis and fixation. We review possible causes for such an anomalous location for schwannoma as well as the advantages and disadvantages of various surgical strategies for removing the tumor. CONCLUSION: This case exemplifies the usefulness of anterior approaches to the cervical spine in treating unusual intradural spinal cord tumors.     

Lipoblastoma

BACKGROUND/PURPOSE: Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat that occurs almost exclusively in infants and children. This study was done to determine the clinical and pathologic characteristics of lipoblastoma. METHODS: Nine cases of pathologically proven lipoblastoma from 1979 to 1997 were reviewed. There were 6 boys and 3 girls ranging in age from 3 months to 29 months. RESULTS: A soft tissue mass was the chief complaint in 7 patients, abdominal distension in 1 patient with a retroperitoneal mass, and defecation difficulty in 1 patient with a perirectal mass. In 4 patients, tumors occurred on the back. Other tumor location includes the neck, scrotum, retroperitoneum, perirectal area, and buttock in 1 patient each. Lesions measured 2.3 to 19.5 cm. Complete excision was done in 8 patients. One perirectal tumor was removed by both the posterior sagittal approach and the intraabdominal approach but incompletely resected. Two tumors located on the back recurred with intraspinal extension 12 months and 18 months after resection. Second resection and second resection with laminectomy were done. Leg pain and urinary incontinence developed in 1 patient but improved on conservative treatment. CONCLUSIONS: Lipoblastoma is a benign neoplasm but can frequently recur (25%) in spite of complete excision. Lipoblastomas occurring on the back had a high recurrence rate (50%) and associated with intraspinal extension. J Pediatr Surg 36:905-907.     

Cervical lipoblastoma: case report, review of literature, and genetic analysis

BACKGROUND: Lipoblastoma is a rare, benign tumor of infants and children, usually occurring in the extremities and trunk, with only a few cases reported in the neck. METHODS: We describe the case of an infant with a rapidly enlarging, painless neck mass. MRI revealed a 4-cm-diameter mass deep to the paraspinal muscles, in close proximity to the C2 vertebral foramen. Review of literature, diagnostic methods, and genetics of lipomatous tumors are discussed. RESULTS: Complete surgical excision via a posterior cervical approach demonstrated irregular lobules of immature fat cells separated by a loose, myxoid connective tissue. Histology and genetic analysis confirmed the diagnosis of lipoblastoma. CONCLUSION: Cervical lipoblastoma is rare, and typically presents as an asymptomatic, painless mass, rarely causing airway obstruction or nerve compression. MRI can be helpful in identifying the lipomatous nature of the mass, but the findings can be inconsistent due to variable maturity of fat cells and the mesenchymal content of the tumor. Chromosomal analysis is useful in differentiating lipoblastoma from liposarcoma. Recommended treatment is complete surgical excision.     

Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von Hippel-Lindau disease

OBJECT: Von Hippel-Lindau (VHL) disease is an autosomal-dominant neoplastic syndrome with manifestations in multiple organs, which is evoked by the deletion or mutation of a tumor suppressor gene on chromosome 3p25. Spinal hemangioblastomas (40% of VHL disease-associated lesions of the central nervous system) arise predominantly in the posterior aspect of the spinal cord and are often associated with an intraspinal cyst. Rarely, the tumor develops in the anterior aspect of the spinal cord. Ventral spinal hemangioblastomas are a surgical challenge because of difficult access and because vessels feeding the tumor originate from the anterior spinal artery. The goal of this study was to clarify whether an anterior or posterior surgical approach is better for management of hemangioblastomas of the ventral spinal cord. METHOD:. The authors performed a retrospective analysis of clinical outcomes and findings on magnetic resonance (MR) imaging studies in eight patients (two women and six men with a mean age of 34 +/- 15 years) who underwent resection of ventral spinal hemangioblastomas (nine tumors: five cervical and four thoracic). Two surgical approaches were used to resect these tumors. A posterior approach was selected to treat five patients (laminectomy and posterior myelotomy in four patients and the posterolateral approach in one patient); an anterior approach (corpectomy and arthrodesis) was selected to treat the remaining three patients. Immediately after surgery, the ability to ambulate remained unchanged in patients in whom an anterior approach had been performed, but deteriorated significantly in patients in whom a posterior approach had been used, because of motor weakness (four of five patients) and/or proprioceptive sensory loss (three of five patients). This difference in ambulation, despite significant improvements over time among patients in the posterior access group, remained significant 6 months after surgery. In all cases, MR images revealed complete resection of the tumor and in five patients significant or complete resolution of the intramedullary cyst was demonstrated (present in six of eight patients). CONCLUSIONS: The outcomes of these eight patients with hemangioblastomas of the ventral spinal cord indicate that both immediate and long-term results are better when an anterior approach is selected for resection.     

Metastatic intradural extramedullary spinal cord tumor from ovarian cancer: A case report with a literature review

Context: Metastatic intradural extramedullary spinal cord tumors are extremely rare.Findings: A 76-year-old woman presented with intractable neck pain. Three years earlier, she had been treated for ovarian cancer with bilateral salpingo-oophorectomy. A year later, she underwent resection of a brain metastasis. Magnetic resonance imaging (MRI) showed an encapsulated intradural extramedullary mass at C4–C5. C4–C5 hemilaminectomy, tumor resection, and biopsy were performed. Histological examination of the resection revealed an adenocarcinoma. After surgery, her intolerable neck-shoulder pain was fully resolved, and she had no difficulties with daily living activities. However, two months later, she underwent gamma knife radiosurgery for the recurrent metastatic brain tumor, and four months later, she died from cachexia.Conclusion: Although cases of metastatic intradural extramedullary spinal tumors from ovarian cancer are extremely rare, their possibility should be considered in the differential diagnosis. A history of brain metastases and enhancement on T1-weighted MRI were helpful for making an accurate diagnosis.     

Chest wall lipoblastoma in a seven-month-old girl: a case report

Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infanthood and early childhood. It is typically located in the extremities and the trunk and less frequently in the head-neck region, mediastinum, and retroperitoneum. However, cases of pleural, thoracic wall, and pulmonary lipoblastoma have also been reported in the literature. Lipoblastoma is a tumor with good prognosis despite its potential for local invasion and rapid growth. Our patient is a 7-month-old girl who was brought to our clinic for a rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the mass was removed by thoracic wall resection and the resultant defect covered with an absorbable polyglactine mesh. The excised mass was diagnosed pathologically as a benign lipoblastoma. In the postoperative period, the thorax wall was stable, and after 24 months of follow-up no recurrence was observed. In the literature, 4 types of thoracic wall lipoblastomas have been reported, but thoracic wall resection has not been applied in any benign lipoblastoma case. To the best of our knowledge, the only benign lipoblastoma case in which a thoracic wall resection was carried out, with its clinical and histopathologic specifications alongside that in the literature, is presented here for the first time.     

Combined laminectomy and thoracoscopic resection of dumbbell-type thoracic cord tumor

STUDY DESIGN: A study of five patients whose dumbbell or paraspinal tumors of the thoracic spine were managed by using thoracoscopic surgery is reported. OBJECTIVES: To report on the use of combined laminectomy and thoracoscopic resection for the management of dumbbell-type thoracic cord tumor. SUMMARY OF BACKGROUND DATA: Some posterior mediastinal tumors can be resected safely with video-assisted thoracic surgery. However, there are few reports on thoracoscopic resection of dumbbell and paraspinal tumors of the thoracic spine. METHODS: Five patients who received treatment for thoracic spine dumbbell tumors and paraspinal tumors were studied retrospectively. Three patients had dumbbell tumors, and two had paraspinal tumors of the thoracic spine. Preoperative evaluation of each patient included plain chest radiography, magnetic resonance imaging, and computed tomography. All patients underwent total resection by means of a combined posteroanterior approach, with thoracoscopic surgery for dumbbell tumors and thoracoscopic surgery alone for paraspinal tumors. In all patients, a gross total resection was achieved with this approach. All patients were observed for a minimum of 3 years. RESULTS: All patients regained their ability to walk 2 days after surgery, except for one patient who had a hemothorax. A gross total tumor resection, documented by magnetic resonance imaging, was performed on all patients. Follow-up imaging at 6 weeks, 1 year, 2 years, and 3 years after surgery did not show residual tumor or recurrence in any patient. To date, spinal instability has not developed in any patient. CONCLUSIONS: Combined laminectomy and thoracoscopic surgery may be a good alternative method for managing thoracic dumbbell tumors.     

Schwannoma of the upper cervical spine–a case report

Upper cervical schwannoma is rare, and belongs to benign tumors that is usually asymptomatic. It accounted for only ten percent of schwannomas cases and mostly occurs in 40e50 years old patients. Aggressive and total resection is the treatment of choice for this tumor. Preoperative diagnosis is difficult, relying on clinical suspicion, and confirmed by surgical pathology. We report a 54-year-old male patient with chief complain of progressive weakness and numbness of his right arm for four months. He had a history of lymph node tumor in 2007 and excised in 2011. Neurological decrease was found on the right arm. The radiographic examination showed lytic lesion on the second, third, and fourth cervical spine. Computed tomography scan showed destruction extending to the first cervical vertebra. Capsulated extradural and extramedullary mass and compression to the spinal cord was found from the magnetic resonance imaging. Two-stage operation was performed. The first stage was posterior decompression with occipitocervical fusion and instrumentation, while the second was anterior decompression and iliac strut graft. Both operations performed using the surgical ultrasonic dissector. During three months'' follow-up, it showed neurological improving. Neurological deficit appears when there is compression on the spinal cord. Total resection is the treatment of choice for these tumors. Preoperative imaging should be performed to exclude malignant tumor and found tumor extension.