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.

An unusual primitive neuroectodermal tumor in the thoracic epidural space.

Primitive neuroectodermal tumor (PNET) is a generic term used to describe a group of histologically indistinguishable neoplasms, including cerebellar medulloblastomas, which are located at various sites in the central nervous system. Primary epidural PNETs are rare and few patients have been reported. We report a 15-year-old girl who presented with gradual onset, over 1 month, of upper back pain and bilateral lower leg weakness. A thoracic spine MRI showed a dumbbell-shaped epidural mass at T2-4 with right paraspinal and posterior mediastinal extension. Surgical resection of the epidural tumor for decompression was performed. The pathologic examination revealed a PNET. Primary spinal PNETs typically have a poor prognosis and optimal therapy has not yet been defined. Surgical resection, with the combination of chemo-radiotherapy or radiotherapy, leads to better outcomes. However, primary epidural PNETs may be classified as a subtype of spinal PNETs because they are free from intrathecal invasion. For these patients, surgery alone and surgery combined with radiotherapy or chemo-radiotherapy remain controversial. Our patient received surgery alone and, 1y ear later, has experienced no local recurrence within the epidural space but the mediastinal part of the tumor has enlarged.

Malignant lymphoma in an atomic-bomb survivor.

Atomic bomb survivors outside of Japan are few and often hard to follow-up. Spinal malignant lymphoma among these survivors is rare in established studies from Japan or the United States. Here, we report an 81-year-old woman, who experienced the atomic bomb explosion in Nagasaki when she was 19 years old, who presented with papillary thyroid carcinoma when she was 70 years old. Both follicular lymphoma over the right elbow region and vertebral malignant lymphoma were found when she turned 81 years old. Bone scan did not show any increased uptake of isotope. However, thoracolumbar spine magnetic resonance imaging showed multiple infiltrative soft tissue masses involving vertebral bodies at the T10-11 level. Computed tomography-guided biopsy further showed lymphocyte infiltration. Fortunately, the neurological deficit was improved after chemotherapy. The diagnosis of malignant lymphoma in atomic bomb survivors should be more careful and aggressive, even when their bone scan results show negative findings. In addition, the authors suggest that atomic bomb survivors should be followed-up carefully throughout their entire life.

Epidural granuloma arising from epidural catheter placement.

Indwelling epidural catheter placement for prolonged drug delivery or is commonplace. Epidural catheter placement leading to a subsequent epidural mass is infrequently symptomatic and thus not often sought after or uncovered. Such masses can mimic lesions causing tumor, infection, and inflammation, and when found, the etiology of granuloma formation is unclear. This article describes a case of a benign epidural granuloma masquerading as an epidural tumor thought to be caused by epidural catheter placement for postoperative total hip replacement pain control. After symptoms of mechanical and radiculopathic pain arose, the patient underwent an open biopsy, spinal decompression, and intradural exploration as an MRI evaluation identified a mass in the spinal canal. Pathologic examination identified a benign fibrous granuloma. At 36-month follow-up, there was no evidence of symptom or tumor recurrence.

[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 angiolipoma: case report and review of the literature.

Spinal angiolipomas are rare lesions usually found in the epidural space of the thoracic spine. This report presents a case of and reviews the literature on this rare entity. The etiology, clinical presentation, imaging, and treatment are discussed. In 92 reported cases of spinal angiolipoma 56 occurred in women (61%), and 36 in men (39%). Mean age of occurrence is 42.9 years (range 10 days-85 years) with most patients presenting with slowly progressive symptoms of spinal cord compression. Most cases occur in the extradural compartment, and are of the non-invasive subtype. This rare clinical entity must be considered in the differential diagnosis of spinal epidural lesions. In most cases complete removal is possible, however, prognosis is good even for infiltrating lesions. Thus, one must not risk neurological damage to attain complete resection.

[Spinal epidural angiolipomas: a rare cause of spinal cord compression. A report of 8 cases and review of the literature]. / Les angiolipomes rachidiens épiduraux: une cause rare de compression médullaire. A propos d'une série de 8 cas avec revue de la littérature.

Extradural spinal angiolipomas are rare benign tumors containing vascular and mature adipose elements. We report 8 cases of spinal epidural angiolipomas in 6 females and 2 males. In 7 cases, the clinical manifestation was a progressive spinal cord compression and one case had a regressive paraparesis mimicking a multiple sclerosis. In the 8 cases, the localization was thoracic with an extradural complete type of contrast block in 2 cases and partial in 5 cases. The myelo-CT achieved in 7 cases and the magnetic resonance imaging done at our last case revealed a fat-containing epidural tumor. The tumors were removed in all cases through a laminectomy with a successful outcome after an average of 9 years (range: 6 months-12 years). Sixty-seven similar cases in the international literature are reviewed. A total of 75 cases have been studied: 45 women and 30 men with a mean age of 46.3 years (range: 6-73), presenting a progressive spinal cord compression in 68 cases and in 7 cases a regressive paraparesis mimicking a multiple sclerosis. Myelography shows an extradural compression of the thecal sac. MRI is nowadays the imaging modality of choice for the diagnosis of these lesions. The surgical removal often easy of the epidural spinal angiolipomas permit a fast recovery. The etiopathogenesis of this process is still controversial between the dysembryogenetic and malformative hypothesis.