Incidence, characteristics, management and outcome of patients with follicular lymphoma with tumor epidural compression, a study on 22 cases.

PURPOSE: Follicular lymphoma (FL) is one of the most common lymphoma. Occasionally, FL is associated with tumoral epidural compression and management of these patients remain poorly codified. This study aims to report incidence, clinical characteristics, management and outcomes of patients with FL and tumoral epidural compression. MATERIAL AND METHODS: Observational, retrospective cohort study of adult patients with FL and epidural tumor compression, treated in a French Institute over the last 20 years (2000-2021). RESULTS: Between 2000 and 2021, 1382 patients with FL were followed by the haematological department. Of them, 22 (1.6%) patients (16 men and 6 women) had follicular lymphoma with epidural tumor compression. At epidural tumor compression occurrence, 8/22 (36%) patients had a neurological clinical deficit (motor, sensory or sphincter function) and 14/22 (64%) had tumor pain. All patients were treated with immuno-chemotherapy; the main regimen being used was R-CHOP plus high dose IV methotrexate in 16/22 (73%) patients. Radiotherapy for tumor epidural compression was performed in 19/22 (86%) patients. With a median follow-up of 60 months (range=[1-216]), 5 year local tumor relapse free survival was achieved in 65% (95% CI 47-90%) of patients. The median PFS was of 36 months (95% CI 24-NA) and 5 years OS estimate was 79% (95% CI 62-100%). Two patients developed a relapse at a second epidural site. CONCLUSION: FL with tumoral epidural compression reached 1.6% of all FL patients. Management based on immuno-chemotherapy with radiotherapy appeared to produce comparable outcomes with the general FL population.

Primary Paraspinal and Spinal Epidural Non-Hodgkin Lymphoma in Childhood.

Primary paraspinal/spinal epidural lymphoma (PPSEL) is rare in childhood. Here, we retrospectively evaluated patients with PPSEL treated in our department. We also reviewed the cases reported in the literature. Fifteen of 1354 non-Hodgkin lymphoma cases diagnosed over a 38-year period were PPSEPL. There were 11 male individuals and 4 female individuals with a median age of 13 years. Most common symptoms were pain and limb weakness. Physical examination revealed spinal cord compression in 80% of patients. The most common tumor location was the lumbar region. Histopathologic subtypes were lymphoblastic lymphoma in 6 and Burkitt lymphom in 5 patients. Subtotal or near-total excision of the tumor with laminectomy was performed in 6 patients. Thirteen and 9 patients received chemotherapy and radiotherapy, respectively. Neurologic recovery was observed in 70% of patients. Seven patients were alive without disease at a median of 88 months. Overall and event-free survival rates were 61.7% and 50.1%, respectively. We reviewed clinical features, treatment, and outcome of 69 PPSEL cases reported in the literature. Neurologic recovery and long-term survival was achieved in 66.7% of them. Heterogeneity in diagnostic methods and treatment have made it difficult to establish the prognostic indicators for neurologic outcome and survival. Multicenter prospective studies with more cases are necessary to determine the prognostic factors.

Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a clonal disease with focal or disseminated lesions that may compress the surrounding tissues, including the spinal cord. Because few reports have described the spinal symptoms as the first manifestation of pediatric LCH, the long-term neurological outcomes remain unclear. CASE REPORT AND LITERATURE REVIEW: We report a 21-month-old boy who presented with sudden-onset paraplegia. Imaging analyses revealed that osteolytic lesions and epidural tumors compressing the spinal cord at the T7-9 vertebrae. Twelve days after he developed leg weakness, emergency radiotherapy was started after a tumor biopsy. During the course of radiotherapy, paralysis steadily ameliorated. After we excluded infections and determined the pathological diagnosis of LCH, multi-drug chemotherapy was started. Apparent improvement in his complete paraplegia was observed after a total 15 Gy of radiotherapy and subsequent chemotherapy, leaving no neurological sequelae at 4 years of age. Through a literature search of studies published from 1980 to 2017, we found that children with LCH showed a generally favorable recovery from neurological dysfunction after the acute phase of spinal symptoms. CONCLUSION: This report underscores the utility of emergency radiotherapy for the neurological recovery of spinal LCH in infants. Our long-term observation further denotes the value of this treatment in terms of the intact survival with preserved motor functions and physical growth.

Primary lumbar extradural hemangiosarcoma in a dog.

A 9 yr old castrated male golden retriever weighing 36 kg was presented for evaluation of progressive left pelvic limb paresis and fecal and urinary incontinence. MRI demonstrated an extradural, ovoid mass compressing the lumbar spinal cord. Surgical excision of the mass was performed. Histologically, the mass was consistent with hemangiosarcoma with no involvement of the adjacent vertebrae. The dog underwent a doxorubicin-based chemotherapy protocol with the addition of oral cyclophosphamide. After completion of chemotherapy, the dog was evaluated q 4 mo for restaging. Clinicopathological evidence of primary tumor recurrence or metastatic disease was not detected for 15 mo after initial diagnosis and treatment. To the authors' knowledge, this is the first report of a primary extradural hemangiosarcoma in the lumbar vertebral column in a dog. The clinical presentation, diagnosis, treatment, and outcome are also discussed.

Spinal cord compression by epidural involvement over 21 vertebral levels in acute lymphoblastic leukemia.

Spinal cord compression is a rare complication of acute lymphoblastic leukemia (ALL). We report a 13-year-old boy with B-precursor ALL, presenting with restriction of breathing and back pain. Cerebrospinal fluid examination showed extremely high protein levels. Radiologic examination indicated that leukemia extended from the thoracic to sacral epidural spaces over 21 vertebral lengths in a band-shaped form, threatening to induce compressive spinal cord neuropathy. Prompt initiation of systemic chemotherapy relieved the obstruction of cerebrospinal fluid flow without local irradiation or surgical intervention. To our knowledge, this patient has shown the most extensive epidural involvement among ALL patients previously reported.

Spinal epidural granulocytic sarcoma in a child precedent to clinical manifestation of acute myeloid lymphoma: case report.

A 13-year-old boy presented with an epidural thoracic granulocytic sarcoma manifesting as rapidly progressive paraplegia preceding clinical manifestation of acute myeloid leukemia (AML). Magnetic resonance imaging revealed a thoracic epidural tumor. He underwent emergent laminectomy and the tumor was totally resected. The initial histological diagnosis was malignant lymphoma. The correct diagnosis of epidural granulocytic sarcoma and AML was established based on cell-surface markers and a chromosomal study of the bone marrow cells. A combination of chemotherapy and bone marrow transfusion achieved complete remission of leukemia. No evidence of AML has emerged over the 18-month follow-up period. Granulocytic sarcoma should be considered in the differential diagnosis of an epidural mass in pediatric patients with or without acute leukemia. Immediate diagnosis and appropriate treatment are recommended to prevent leukemic transformation.

Primary spinal epidural extraosseous Ewing's sarcoma mimicking a spinal abscess.

Ewing's sarcoma is one of the most common malignant tumors of the skeletal system in children and young adults. It most frequently stems from the long bones of the extremities. However, though uncommon, extraosseous localization can be seen. Epidural extraosseous presentations are extremely rare. In this case, the authors report on the long-term follow-up of a patient with this well-demonstrated, but uncommon localization, and the initial unique presentation of the patient, which clinically and radiologically mimicked a cervical abscess.

Primary cervical epidural malignant lymphoma with rheumatoid arthritis: a case report.

We report a case of primary cervical epidural malignant lymphoma with rheumatoid arthritis. Because of the acute progression of paralysis in both legs, surgical decompression and stabilization of the cervical spine were performed. The resected specimen showed proliferation of lymphoblastic cells diagnosed as malignant lymphoma. Four series of chemotherapy were administered after surgery, and the patient recovered from paralysis.

[Medullary compression revealing the presence of a follicular lymphoma: a case report]. / Compression médullaire révélatrice d'un lymphome folliculaire: à propos d'un cas.

Epidural localization is a rare presenting sign of non-Hodgkin's lymphoma. These tumours are classified in the majority of cases as large B cell lymphomas. Low grade lymphomas are rarely reported. We report a 43-year-old woman admitted for a total functional disability of the two lower limbs. Magnetic resonance imaging revealed a spinal epidural mass extending from D7 to D9. A laminectomy was performed. The histopathological study revealed a follicular lymphoma. The patient underwent a spinal irradiation and chemotherapy. Follow up evaluation at 16 months demonstrated no evidence of relapse. Our purpose is to describe the clinical features, the pathologic findings, the treatment and the prognosis of non-Hodgkin's lymphoma revealed by an epidural involvement.

[A rare cause of cauda equina syndrome: primary epidural lymphoma]. / Une cause rare de syndrome de la queue de cheval: Le lymphome epidural primitif.

INTRODUCTION: Primary epidural lymphoma is generally manifested by isolated epidural involvement. On rare occasions it can lead to cauda equina syndrome. CASE: A 56-year-old man was hospitalized for lumbago, weakness in the legs and sphincter problems. A dorsolumbar MRI revealed that the anterior and posterior peridural space from D11 to L3 was filled by a mass compressing the marrow and compacting the roots. The L2 to L4 laminectomy revealed an extradural tissue leading to the diagnosis of large B-cell lymphoma. Metastatic workup demonstrated unremarkable findings. DISCUSSION: Primary epidural lymphoma accounts for less than 10% of epidural tumours and less than 1% of non-Hodgkin lymphomas. Its clinical manifestations are related to the tumoral process or involvement of medullar vessels. It can be, but rarely is, manifested by cauda equina syndrome. Diagnosis is based on histological analysis of tissue taken during the decompression laminectomy. Prognosis is relatively good.

[Case of intraspinal epidural tumor developing after systemic mastocytosis with marked osteosclerosis and myelofibrosis].

A 64-year-old man was diagnosed as having urticaria pigmentosa in 1998, and treated with PUVA therapy. In January 2002, X-ray imaging revealed osteosclerosis was detected in the systemic bone and bone scintigraphy. A bone marrow aspiration sample was not obtained due to a dry tap. CT scans showed hepatosplenomegaly and mesenteric lymphadenopathy. Myelofibrosis and diffuse mast cell infiltration were revealed by a bone marrow biopsy, and a diagnosis of systemic mastocytosis with severe osteosclerosis and myelofibrosis was made. In October 2003, he was admitted to our hospital because of mid back pain. A neurological examination showed muscle weakness in the upper and lower limbs, sensory disturbance below the level of Th4 and urinary obstruction. T1 and T2 weighted images of MRI demonstrated a high intensity epidural mass lesion extending from the vertebral level of C5 to Th2 and severely compressing the spinal cord. We considered the possibility of the invasion of the spinal canal by the mastocytosis. The patient was treated with interferon alpha-2b (IFN-alpha2b) and prednisolone. Subsequently, the motor and sensory disturbances were gradually alleviated, and spinal MRI confirmed a marked reduction in the size of the epidural tumor. However, the patient became resistant to interferon, and died of multiple organ failure in spite of steroid pulse and cladribine therapies. Multiple organ infiltration by mast cells was revealed at autopsy.

Spinal epidural rhabdomyosarcoma.

We report an extremely rare case of rhabdomyosarcoma in the lower cervical and upper thoracic spine. The MR imaging appearance of the lesion was nonspecific, and different from the one previously reported. The majority of the tumor was removed surgically less than a month from the onset of symptoms. Treatment was continued with spinal irradiation and chemotherapy, however diffuse leptomeningeal metastases were found six months later.

Rapid improvement of paraplegia caused by epidural involvements of Burkitt's lymphoma with chemotherapy.

STUDY DESIGN: Case report. OBJECTIVE: The authors present a case of atypical Burkitt's lymphoma with multiple epidural involvements. SUMMARY OF BACKGROUND DATA: Spinal cord compression in children is an emergency that requires urgent attention to minimize neurologic dysfunction. Although it is not life-threatening in most patients, cord compression can cause severe neurologic morbidity. MATERIALS AND METHODS: Because the patient showed rapid neurologic deterioration, we started chemotherapy and high-dose steroids without laminectomy or radiotherapy immediately after a tumor biopsy from the left mandible. RESULT: The combined therapies were very effective and his neurologic symptoms improved immediately. The epidural involved masses disappeared in imaging studies after the first course of chemotherapy including methylprednisolone (20 mg/kg per day for 3 consecutive days and gradually tapered off over 2 weeks), vincristine (1.5 mg/m2 per day), cyclophosphamide (2 g/m2 per day for 2 days) and pirarubicin (40 mg/m2 per day). After completing seven courses of chemotherapy, the patient is now fully ambulant. CONCLUSION: Considering the severe late effects of laminectomy and radiotherapy, chemotherapy should be considered as a first choice of treatment for spinal cord compression caused by malignant lymphoma.

Primary epidural burkitt lymphoma in a child: case presentation and literature review.

Primary spinal epidural Burkitt lymphoma, presenting with signs of spinal cord compression, is very uncommon in childhood. Previously reported pediatric cases with isolated epidural Burkitt lymphoma had a high mortality, and survivors usually suffered serious neurologic sequelae. The authors present a 13-year-old female with isolated epidural Burkitt lymphoma with favorable outcome, and review the pediatric literature.

Non-Hodgkin lymphoma in a child presenting with acute paraplegia: a case report.

Spinal cord compression is a rare presentation of non-Hodgkin lymphoma. Extradural location at onset is a rare but devastating event in pediatric oncology. The authors describe a girl with acute spinal cord compression due to epidural non-Hodgkin lymphoma, emphasizing the encouraging perspective for a complete recovery in children with this condition. A 5-year-old girl presented with pain followed by progressive hyposthenia and paraplegia after a trauma. CT scan and MRI showed homogeneous tissue extending from T2 to L4, occupying the entire vertebral canal and extending to the para- and peri-vertebral soft parts. Emergency surgical debulking was carried out through T6-L1 laminectomy. The patient began chemotherapy (LMB 89 Protocol) and the tumor quickly disappeared. The patient is maintaining a complete remission 42 months after diagnosis. Significant results may be obtained with the chemotherapy treatment of epidural non-Hodgkin lymphoma when the disease is promptly diagnosed. Considering the effectiveness of chemotherapy, the authors believe that a neuro-surgical approach should be employed only when rapid worsening of symptoms is observed or for diagnostic purpose.

Symptomatic neurological epidural metastasis with interleukin-2 therapy in metastatic renal cell carcinoma.

Interleukin-2 has known efficacy in the treatment of metastatic renal cell carcinoma but it also has adverse effects mediated by an inflammatory mechanism and capillary leak syndrome. The authors report the case of a patient with a metastatic renal cell carcinoma who had an asymptomatic intra-epidural metastasis in the thoracic spine, which became symptomatic during IL-2 administration without any recurrence after specific treatment by surgery and radiotherapy.

Treatment of spinal epidural metastasis improves patient survival and functional state.

OBJECTIVES: To determine factors affecting the success of radiation therapy (RT) of spinal epidural metastases and patient survival after RT. DESIGN/METHODS: One hundred thirty-nine male veterans with an initial spinal epidural metastases treated with dexamethasone and RT were evaluated prospectively. Patients were followed until death. RESULTS: At presentation, 84 patients could walk. After RT, 119 patients walked. The likelihood of regaining ambulation increased if treatment began <12 hours after loss of ambulation and if patients had bladder and bowel function and sacral sensory sparing. Treatment reduced pain levels, and ambulatory patients had less pain compared with nonambulatory patients. Median length of survival was 104 weeks for ambulatory patients and 6 weeks for nonambulatory patients. Mean interval between loss of ambulation and death was 4.0 +/- 0.5 weeks. Recurrent spinal epidural metastases occurred in 8.63% of patients. CONCLUSIONS: Patients who walked after treatment lived longer, were ambulatory for most of their remaining life, had less pain, and had a lower incidence of depression.

Intraspinal primitive neuroectodermal tumour: report of two cases and review of the literature.

Two patients with primary intraspinal primitive neuroectodermal tumour are presented. In a 32-year-old man, the tumour evolved intradurally from a sacral nerve root. Despite repeated surgery and radiochemotherapy, the patient suffered multiple intraspinal tumour relapses and intracranial seedings, and died 29 months after the first diagnosis. In a 17-year-old male adolescent, the tumour was located in the lumbar epidural space, extending into the paraspinal muscles. Following resection and radiochemotherapy, the patient is free from disease 23 months after the initial presentation. The clinical, radiological, histopathological and cytogenetic findings of both patients are presented and the relevant literature is reviewed. Particular attention is given to the histogenetic relationship between peripheral primitive neuroectodermal tumour and Ewing's sarcoma.

Primary epidural non-Hodgkin lymphoma: spinal cord compression syndrome as the initial form of presentation in childhood non-Hodgkin lymphoma.

BACKGROUND: Primary epidural non-Hodgkin lymphoma (NHL) is a rare but devastating event in pediatric oncology. Spinal cord compression due to an epidural mass from NHL, although it presents as a localized problem, in fact, is a systemic disease. Over the last 3 decades, aggressive systemic treatments with central nervous system prophylaxis have been designed with improving results. PROCEDURE: We reviewed the 20-year experience of the treatment of NHL with the LSA2-L2 protocol in our institution. Among 256 patients treated, five presented with primary epidural mass. Clinical features, treatment, and outcome of those five patients are presented. RESULTS: Three patients out of five responded favorably, and after a median follow-up of 10 years, they are cured. After presenting with paraplegia, functional and neurological recoveries were excellent. Of the patients who failed treatment, relapse occurred early, either during the treatment or right after completion of therapy. The relapses were within the central nervous system as well as systemic. CONCLUSIONS: Patients with primary epidural NHL presented with long histories of persistent back pain. Plain films of the spine were useless. All patients were diagnosed when neurologic damage had already been established. However, when the epidural NHL is treated aggressively with systemic chemotherapy and vigorous rehabilitation, the prognosis for complete neurological recovery and cure of the lymphoma is encouraging.