Primary malignant peripheral nerve sheath tumor of the cauda equina with metastasis to the brain in a child: case report and literature review

Background contextPrimary intradural malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare; only 23 cases have been reported in the English-language literature till now. No gold standard for treating primary intradural MPNSTs has yet been established.PurposeTo report a rare case of primary intradural MPNSTs in a child and review the literature pertaining to this rare disease.Study design/settingCase report and literature review.MethodsWe report our experience with one new case. An 8-year-old boy diagnosed with primary intradural MPNSTs underwent three surgical excisions and two rounds of radiotherapy; however, metastasis to the brain was found, and the boy died 16 months after the first surgery. We also review the literature pertaining to both MPNSTs in general and primary intradural MPNSTs.ResultsSurgery is currently the mainstay of MPNST treatment. Radiotherapy and chemotherapy are of limited value in these tumors. Based on the review of the 24 cases described in the literature, including the present case, primary intradural MPNST is a very aggressive tumor with a very high recurrence rate even after gross total resection and with significant potential for leptomeningeal and systemic metastasis. The overall prognosis is very poor and seems to be worse than that of MPNSTs in general.ConclusionsPrimary intradural MPNST is a very rare entity with a poor prognosis. Surgical tumor removal combined with postoperative high-dose radiation may be recommended. Chemotherapy is usually reserved for patients with disseminated metastases or tumors that are unresectable at the time of diagnosis.     

Intraspinal clear cell meningioma: diagnosis and management: report of two cases

OBJECTIVE AND IMPORTANCE: Intraspinal clear cell meningioma is a rare morphological variant of meningioma. Only 13 case reports are found in the literature; therefore, no management strategy has been defined for this tumor type. This article describes two patients, reviews the literature, and proposes a treatment plan for clear cell meningioma. CLINICAL PRESENTATION: Two female patients, 22 months and 8 years of age, respectively, presented with localized neck and leg pain that limited their ability to walk. Magnetic resonance imaging revealed intradural tumors, a cervical intramedullary neoplasm in the younger patient, and a cauda equina tumor in the older child. INTERVENTION: Both patients underwent radical resection of their intradural tumor. Both tumors, however, recurred shortly (5 and 6 mo) after the initial operation. During the second operation, a radical removal was performed on each patient. Both patients received adjuvant radiotherapy. In addition, the younger patient developed posterior fossa metastasis 20 months after intraspinal surgery. CONCLUSION: Intraspinal clear cell meningiomas are very uncommon tumors. The clinical course in our two patients supports the reported 40% recurrence rate within 15 months. These tumors also can disseminate within the central nervous system. We recommend serial imaging studies every 3 months. For recurrent tumors, we recommend localized radiation therapy after reoperation.     

Primary extraosseous intradural spinal Ewing’s sarcoma: report of two cases

Two cases of primary extraosseous intradural spinal Ewing’s sarcoma are reported with a review of the current literature. This rare neoplasm shares features with cerebral primitive neuroectodermal tumors, complicating a correct diagnosis. Gross total resection seems to be the main treatment, although adjuvant therapies could improve the prognosis. In case 1, a 56-year-old man presented with cauda equina syndrome. MRI showed an intradural tumor from L4 to S2. An emergency laminectomy was performed with gross total resection of a hemorrhagic tumor, followed by adjuvant treatment. In the second case, a 25-year-old female developed leg and lumbar pain. MRI study identified a homogeneously enhancing intradural mass at the L2-L3 level. A laminoplasty was performed, followed by tumor resection; no adjuvant treatment was administered afterwards. Immunohistochemical workup confirmed the diagnosis of Ewing’s sarcoma in both cases.     

Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review

BACKGROUND: This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck. METHODS: From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST. We retrospectively reviewed presenting symptoms, radiological findings, surgical management, and follow-up status and performed a literature review. RESULTS: Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation. Two lesions were growing within the sinonasal tract. The most common presenting symptom was a rapidly enlarging cervical mass. Seventy percent of the tumors could be resected completely. Long-term follow-up showed a 2-year disease-specific survival rate of 50% and 5-year survival rate of 20%. Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease. Postoperative adjuvant radiotherapy was found to make no difference in outcome. CONCLUSIONS: Although rare, MPNST is one of the most aggressive tumors in the head and neck area. Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST. Adjuvant radiotherapy should be used to assist surgical excision in local control. The role of adjuvant chemotherapy remains controversial.     

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.     

Unusual primary secreting germ cell tumor of the spine. Case report

The authors describe a young man with a rare primary spinal germ cell tumor that secreted beta-human chorionic gonadotropin. The tumor was resected, and six courses of adjuvant chemotherapy consisting of cisplatin, bleomycin and etoposide were administered together with irradiation to the craniospinal area. An additional dose of radiation was delivered to the tumor site after the first four chemotherapy sessions. The patient was well without any neurological deficit or iatrogenic sequela 33 months after diagnosis. The occurrence of this rare tumor located primarily in the spine warrants attention in pathological studies of spinal tumors in young patients.     

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST) complicated with von Recklinghausen's disease: a case report

A retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with von Recklinghausen's disease is reported. A 55-year-old woman was admitted with a left side abdominal mass. Physical examination showed numerous cafe-au-lait-spots, subcutaneous masses, scoliosis, and a baby's head-sized fixed mass in the left abdomen. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 9 x 9 cm retroperitoneal mass. Two other tumors were also found. One on the left side of the T2-T3 thoracic spine, and the other posterior to the right hip joint. The retroperitoneal tumor was resected en bloc. The tumor was a solid yellow mass. Macroscopically it has a pseudocapsule of fibrous tissue, weighed 1,120 g and measured 9 x 9 x 15 cm. The histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST). Since the responsiveness of these tumors to chemotherapy and radiation therapy is poor, we did not administer adjuvant therapy. The patient is alive with no evidence of recurrence more than 6 months after surgey.     

Drop metastasis from sinonasal undifferentiated carcinoma: clinical implications.

STUDY DESIGN: The first reported case of multiple intradural, extramedullary spinal metastasis from sinonasal undifferentiated carcinoma is presented. OBJECTIVES: To elucidate the mechanisms by which metastatic disease invades the spinal axis, and to discuss the possibility of spinal drop metastasis from head and neck tumors that invade the dura. SUMMARY OF BACKGROUND DATA: Sinonasal undifferentiated carcinoma is a rare yet aggressive neoplasm of the upper airways and anterior skull base. This neoplasm is known to invade the cranial vault and brain locally. However, it has not previously been reported to seed the cerebrospinal fluid or result in drop metastasis. Such drop metastasis may result in significant neurologic deficit if not diagnosed and treated in a timely manner. METHODS: This report is based on a single patient treated by a multidisciplinary team from the departments of neurosurgery, otolaryngology, and radiation oncology at the University of Southern California School of Medicine. RESULTS: This patient initially underwent resection and local radiation therapy for sinonasal undifferentiated carcinoma of the anterior skull base. At the time of surgery, the tumor was noted to violate the dura and arachnoid along the subfrontal plane. At 11/2 years after the initial treatment, a bandlike distribution developed at T2 as well as paresthesias and numbness below that level. Imaging of the spine showed an intradural, extramedullary tumor at T2 consistent with a schwannoma or meningioma. The patient underwent a laminectomy and tumor resection, which showed poorly differentiated sinonasal carcinoma. Local radiation therapy was administered, and the patient experienced complete recovery of neurologic function. Bilateral leg pain and weakness developed 14 months later. Magnetic resonance imaging of the spine showed a new intradural, extramedullary lesion at T12, remote from the first lesion. This second metastasis was managed with surgical resection and adjuvant radiation therapy. CONCLUSIONS: This is the first reported case of a sinonasal carcinoma leading to intradural extramedullary metastasis. The primary tumor likely seeded the cerebrospinal fluid, thus resulting in drop metastasis. Patients with sinonasal undifferentiated carcinoma that invades the dura should be monitored closely for evidence of metastasis before symptoms develop.     

Medical management of recurrent aggressive angiomyxoma with gonadotropin-releasing hormone agonist

Patients with aggressive angiomyxoma may experience local recurrences. We report a case of recurrent aggressive angiomyxoma medically treated successfully with a gonadotropin-releasing hormone agonist. A 34-year-old woman with a huge perineal tumor underwent an extensive resection of the abdominoperineal tumor combined with total pelvic exenteration. Histology showed aggressive angiomyxoma and the tumor cells were immunoreactive for estrogen and progesterone receptors. Although the patient had experienced no local recurrence for 12 months under adjuvant therapy with a gonadotropin-releasing hormone agonist, a recurrence occurred 3 months after the completion of adjuvant therapy. The patient underwent medical treatment with a gonadotropin-releasing hormone agonist and had a complete resolution of the recurrent tumor again. Hormonal treatment with a gonadotropin-releasing hormone agonist can be applied for small primary aggressive angiomyxomas in addition to adjuvant therapy for residual tumors.     

椎管内髓外硬膜下多发性肿瘤的诊断与手术治疗

目的探讨椎管内多发性肿瘤的诊断及治疗方法。方法本组男11例,女4例。年龄16～81岁,（51.0±21.2）岁。病程2～27月,（11.0±7.6）月。均无皮肤咖啡斑及体表神经纤维瘤,头颅MR未见异常。增强MR示椎管内髓外硬膜下肿瘤46个,其中≥0.5cm肿瘤35个,肿瘤累及颈段1例,累及胸段5例,累及腰骶段12例。手术方法：采用后正中入路椎板切除术切除肿瘤,〈1cm者切除半椎板,在显微镜下操作,力争保留重要的载瘤神经而将肿瘤自神经上剔除,如肿瘤与神经无法分离,尽可能游离神经根在切除肿瘤后行神经根吻合。术后常规应用抗生素及激素治疗,术后戴颈围领、围腰保护2个月,同时训练腰背部肌肉。结果15例手术肿瘤全切12例,其中1例行神经根吻合;未全切的3例中2例术前诊断为多发神经纤维瘤或神经鞘瘤,因部位散在且直径〈0.5cm未考虑手术切除,另1例术中见肿瘤与多根神经根粘连,病理为转移瘤,仅行部分切除减压术。手术共切除肿瘤33个。病理：神经鞘瘤8例,神经纤维瘤1例,室管膜瘤2例,黑色素瘤1例,腺癌转移1例,脊膜瘤2例。黑色素瘤患者于术后22个月死亡,转移瘤患者于术后1年死亡,余13例随访4～72个月,平均30.1月,肿瘤无复发增长,其中2例多发神经鞘瘤未手术全切除者分别随访12、43个月,肿瘤无明显增长。13例存活者术后KPS评分较术前均有改善（术前75.4±13.3,术后最后一次随访时97.7±6.0,t=-7.366,P=0.000）。结论椎管内髓外硬膜下多发肿瘤以良性居多,中老年多见,半椎板及次全椎板入路显微手术切除是有效的治疗方法,〈0.5cm的无症状肿瘤可严密随访。     

Resection of a large spinal intradural ependymoma using a limited unilateral laminectomy approach in the lumbosacral region

Ependymomas are the most common gliomas of the lower spinal cord, conus medullaris and filum terminale. Although there are several surgical modalities indicated in the removal of spinal tumors, total laminectomy is the conventional method used. However, surgical complications, such as spinal deformities and instability are more common with this approach compared to limited unilateral hemilaminectomy. To present the clinical and radiologic results of patient with a large spinal intradural ependymoma via a limited posterior unilateral laminectomy approach in the lumbosacral region. The aim of this study was to present a modified duramater incisions and surgical positions setup, and discussed the major controversies in treatment in the literature. This article reports the surgical resection of a lumbosacrally localized ependymoma in a 25-year-old male patient using unilateral laminectomy at the L4 to S3 levels. The tumor was removed with any difficulty in reaching to contralateral side. A follow-up lumbar magnetic resonance imaging scan with and without contrast performed 18 months postoperatively showed no residual or recurrent tumor lesions. This procedure may be more difficult than the conventional total laminectomy as suturing of the duramater. However, modified duramater incisions and setup of microscope-operating table provides best facilitates to surgery manipulation. Besides, unilateral limited laminectomy protected the posterior supporting elements. In order to reduce the postoperative complications and minimize the destruction of tissue, unilateral limited hemilaminectomy is recommended for the removal of intradural tumors. This technique facilitates the return of the patient to ordinary life.     

Clear cell meningioma of the cauda equina in an adult: case report and literature review

In the pediatric population, clear cell meningiomas are more frequently intracranial than intraspinal in location. Tumors recur in up to 40% of cases within 15 postoperative months and are often managed with repeated resection with or without radiation therapy. The management strategy for adults with clear cell meningiomas involving the lumbar spinal canal (cauda equina) is less clearly defined. A 41-year-old woman presented with mild, right greater than left, lower extremity paresis. An enhanced magnetic resonance (MR) scan revealed a homogeneously enhancing intradural lesion filling the spinal canal at the L3-L4 level. Preoperative noncontrast MR studies of the brain and cervical and thoracic spine were negative. An L2-L5 laminectomy was performed for gross total excision of the intradural lesion, which was adherent to one nerve root of the cauda equina. Frozen-section diagnosis confirmed clear cell tumor. Differential diagnoses included meningioma versus renal cell carcinoma. Negative postoperative chest, abdominal, and pelvic computed tomography studies ruled out tumor of renal cell origin. Enhanced MR studies of the neuraxis proved negative. Consultations with multiple oncologists and radiation therapists recommended neither radiation nor chemotherapy following this initial surgery. She remains disease-free 1 year postoperatively. The high recurrence rate for clear cell meningiomas in children requires repeated tumor resection with or without secondary radiation therapy. Following gross total resection of lumbar tumors in adults, reserving radiation therapy for secondary recurrences provides optimal management.     

Resection of tumors in irradiated fields with subsequent immediate reconstruction

With increased use of primary radiation therapy for treatment of cancer and adjuvant radiation therapy after surgical removal of a bulk tumor, recurrence in these fields has posed significant new and increasingly technical and biologic problems. We report our experience with ten such cases in which difficult wounds were reconstructed immediately after major regional resections of advanced or recurrent tumors in fields of previous irradiation. All of these patients could undergo extirpation of their recurrent tumors in irradiated fields because of improved techniques in reconstructive flap surgery allowing large amounts of well-vascularized tissue to be transferred, sometimes over a significant distance. Follow-up of these patients has ranged from three to 18 months (median, nine months). Primary healing, decreased deformity, reduced morbidity, and prolonged disease-free intervals have been achieved with the combination of extirpative and reconstructive techniques.     

Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum

Malignant peripheral nerve sheath tumors (MPNSTs; malignant schwannomas) rarely occur in the anterior mediastinum, and their prognosis is poor. A 75-year-old man was referred to our hospital for examination of an anterior mediastinal tumor. A computed tomography-guided percutaneous needle biopsy revealed only fibrosis. The tumor was completely excised via a median sternotomy with partial resection of the pericardium and right upper lobe of the lung. Thereafter, the tumor was diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma. At 1 year after the surgery, a distant metastasis was found in the interlobular space between the right middle and lower lobes. The tumor was completely excised via a right posterolateral thoracotomy. Reexamination of the primary and secondary tumors revealed an MPNST. No recurrence was found up to 5 years after the second surgery without adjuvant chemotherapy or radiation therapy. However, he died from multiple lung metastases after 6 years.     

Surgical Management of Giant Transdural Glomus Jugulare Tumors with Cerebellar and Brainstem Compression

Objective The objective of this study is to discuss the management of advanced glomus jugulare tumors (GJTs) presenting with intradural disease and concurrent brainstem compression.Study Design This is a retrospective case series.Results Over the last decade, four patients presented to our institution with large (Fisch D₂; Glasscock-Jackson 4) primary or recurrent GJTs resulting in brainstem compression of varying severities. All patients underwent surgical resection through a transtemporal, transcervical approach resulting in adequate brainstem decompression; the average operative time was 12.75 hours and the estimated blood loss was 2.7 L. All four patients received postoperative adjuvant radiotherapy in the form of intensity-modulated radiation therapy or stereotactic radiosurgery. Combined modality treatment permitted tumor control in all patients (range of follow-up 5 to 9 years).Conclusion A small subset of GJTs may present with intracranial transdural extension with aggressive brainstem compression mandating surgical intervention. Surgical resection is extremely challenging; the surgical team must be prepared for extensive operating time and the patient for prolonged aggressive rehabilitation. Newly diagnosed and recurrent large GJTs involving the brainstem may be controlled with a combination of aggressive surgical resection and postoperative radiation.     

Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature

BACKGROUND: Malignant peripheral nerve sheath tumors, infrequent sarcomas arising within a peripheral nerve, mostly metastasize to the lung at terminal stage of disease. However, metastasis to the brain without pulmonary involvement is quite unlikely to occur. CASE DESCRIPTION: A 21-year-old man was brought in the emergency department due to sudden unconsciousness, and imaging studies showed huge intracerebral hemorrhage. Surgical removal and adjuvant therapy was performed for pathologically proven MPNST. Concurrent painful chest masses were also confirmed as MPNST through surgical resection. Nine months after craniotomy, multiple masses in the lung field and axilla region were uncovered, and surgical resection followed. Despite postoperative irradiation, the patient died of the respiratory complications at 16 months after craniotomy. CONCLUSION: To our knowledge, this kind of metastatic cerebral bleeding is the first case that was ever reported. The MPNST actually can exhibit an apoplectic manifestation even without pulmonary involvement in a young adult, albeit this is quite rare. Thus, high index of suspicion should be paid to minute complaints regarding MPNST in peripheral locations so as not to overlook an advanced or metastasized disease.     

Spinal intradural capillary hemangioma

BACKGROUND: Capillary hemangiomas are typically superficial lesions found in the skin or mucosa of the head and neck, but intradural locations are rare. We report a case of the spinal intradural capillary hemangioma of the lumbar spine with a review of the pertinent literature. CASE DESCRIPTION: A 59-year-old man presented with a 3-month history of low back pain and left leg pain. On examinations, the patient was shown to have paresthesia in the left L4, L5, and S1 dermatome and a diminution of the left knee jerk. Magnetic resonance imaging revealed an approximately 2-cm intradural enhancing lesion at the level of the L1-2 disk space. Laminectomy of L1-2 was performed for tumor removal. A reddish mass was covered by matted adherent nerve roots and derived its blood supply from radicular vessels. Complete excision was accomplished. Histologic diagnosis was capillary hemangioma. After operation, the patient's symptoms were improved. CONCLUSION: We experienced a rare spinal intradural vascular tumor of the lumbar spine. Histologic diagnosis was capillary hemangioma. We report a rare case of spinal intradural capillary hemangioma of the lumbar spine.     

Solitary fibrous tumor of the thoracic spine

Intraspinal solitary fibrous tumors are rare: to our knowledge, the literature reports only 27 cases. We present a histologically and immunohistochemically confirmed solitary fibrous tumor involving the intradural extramedullary compartment of the thoracic spine. Microsurgical gross-total resection was achieved. A definitive role for adjuvant treatments in this type of tumor has not been established and therefore, they were not used. The patient was well, without clinical or radiological recurrence, 18 months after surgery.