Mediastinal lymphangioma presenting as an acute epidural hematoma

Lymphangiomas are benign collections of blind-ended lymphatic and vascular channels. Lesions typically occur in the soft tissues of the head and neck, although any region of the body can be affected. Involvement of the spine is very rare. A complete resection is generally curative. On rare occasions, these tumors are complicated by infection or hemorrhage. The authors present an unusual case of a hemorrhagic lymphangioma in a 1-year-old male child. The lesion originated in the mediastinum and extended into the cervicothoracic epidural space via a neural foramen. This resulted in an acute epidural hematoma and quadriparesis. Emergency decompression resulted in full neurological recovery. This may be the first report of a lymphangioma resulting in an acute epidural hematoma and quadriparesis.     

Infantile myofibromatosis in a newborn: a case report

Infantile myofibromatosis presents as a firm, nodular mass in soft tissues, muscles, or visceras which can be solitary or multicentric, and it may regress spontaneously. We present a one-day-old boy who was admitted to the hospital for two masses, with one below the umbilicus that looked like a hemangiomatous structure and the other in the abdominal skin as a subcutaneous nodule. There was no intraabdominal involvement, and both of the masses were resected at 10 days of life. The one-year follow-up was uneventful.     

Heel pain due to retrocalcaneal bursitis-radiographic diagnosis (with an historical footnote on Sever's disease)

Retrocalcaneal bursitis is a distinct condition causing posterior heel pain in active, healthy children. It appears to result from post-traumatic inflammation of the soft tissues of the posterior heel, and is unrelated to avascular necrosis of the calcaneal apophysis. The diagnosis may be confirmed radiographically by the loss of the lucent retrocalcaneal recess, with a normal Achilles tendon and superficial soft tissue contour, and intact cortex of the underlying os calcis.     

Air collection in the retropharyngeal soft tissues observed in lateral expiratory films of the neck in 9 infants

This is the report of 9 infants in whom a lateral roentgenogram of the neck exposed in expiration showed an increase in the thickness of the retropharyngeal soft tissues with a small collection of air within these soft tissues simulating a retropharyngeal abscess. Both findings disappeared in inspiration consistent with normal variants.     

Prospects of the use of modifiers in children's oncologic radiology]

Problems of radiotherapy of malignant tumors in children are discussed. The treatment included the use of different radiation modifiers with the aid of which radiosensitivity of normal and tumorous tissues may be selectively altered, thereby reducing radiation load of normal tissues and organs adjacent to the tumor. The data are given on the treatment of 45 children with soft tissue sarcomas. In these children, radiotherapy was carried out in combination with local microwave hyperthermia.     

Spondylocarpotarsal synostosis syndrome: MRI evaluation of vertebral and disk malformation

Spondylocarpotarsal synostosis syndrome (SSS) is a rare autosomal recessive condition characterized primarily by vertebral malsegmentation, carpal/tarsal coalition, and a dysmorphic appearance. Differentiating SSS from other congenital scoliosis syndromes requires evaluation of the vertebrae, ribs, soft tissues, and spinal cord. The enhanced resolution over plain radiographs seen with MRI allows more detailed assessment of vertebral malformation and surrounding anatomy. Diagnosis of the underlying cause of congenital scoliosis might be enhanced using this technology. We report on a 12-year-old girl of unaffected parents with SSS who was evaluated with MRI sequences of the spine to show various types of malsegmentation. Additionally, there is the new finding of fusion of teeth, with developmental failure of a canine incisor.     

Splenopancreatic Field Abnormality Is Not Unique to Trisomy 13

Splenopancreatic fusion is an uncommon finding, usually only seen as part of the splenopancreatic field abnormality associated with trisomy 13. It may present itself either as ectopic splenic tissue in the cauda pancreatis, as ectopic pancreatic tissue in the spleen or accessory spleen, or as fusion of the cauda pancreatis and splenic hilum. In this study, we report four unrelated congenital anomaly cases presenting trisomy 21, osteocraniostenosis syndrome, isolated congenital heart defect, and oligohydramnios sequence due to prune belly syndrome, in which fusion was observed. This demonstrates that, although it may be more common in trisomy 13, this phenomenon should not be interpreted as pathognomonic to that syndrome.     

Long‐term outcome for kaposiform hemangioendothelioma: A report of two cases

Kaposiform hemangioendothelioma (KHE) is a rare aggressive vascular tumor of skin and deep soft tissues that typically presents in infancy and may be associated with a potentially life‐threatening coagulopathy known as Kasabach–Merrit phenomenon (KMP). Recent advances in medical therapy have successfully treated many patients. However, our knowledge regarding the natural history of these lesions and optimum surveillance strategies remains rudimentary. We report two young women who had KHE with KMP treated in infancy and presented in adolescence with comorbidities related to their KHE tumor. This presentation supports the need for long‐term surveillance in these patients.     

Craniosynostosis in hyper-IgE-syndrome

A 9-year-old boy with hyperimmunoglobulin-E-syndrome (HIE) and craniosynostosis is reported. Premature fusion of the sagittal and lambdoid suture led to scaphocephaly. A partial optic atrophy without clinical signs of raised intracranial pressure was observed. This is the fourth reported case of craniosynostosis in HIE. Bone anomalies like osteoporosis are frequent findings in HIE. Apart from their clinical impact they could be related to factors involved in the pathogenesis of HIE, such as impairment of chemotaxis in tissues or monocyte differentiation.Abbreviation HIE hyperimmunoglobulin-E-syndrome     

Two-year-old Boy with Proteus Syndrome and Fatal Pulmonary Thromboembolism

A 2-year-old boy with a relatively mild form of Proteus syndrome (PS) died suddenly during a previously uncomplicated postsurgical convalescence. Autopsy demonstrated massive acute pulmonary embolism, which has not been previously reported in PS. In addition, clinically occult mesodermal hamartomata, predominantly hemolymphangiomata, were found to be widespread in the pelvoabdominal viscera, including spleen, appendix, kidneys, adrenal gland, liver, and retroperitoneal soft tissues. Such lesions may well be common in PS patients but may not be detected by conventional radiographic imaging techniques unless they are fairly large.     

Kirner's deformity of all fingers in a 5-year-old girl: soft-tissue enhancement with normal bones on contrast-enhanced MRI

Kirner's deformity is an uncommon, but characteristic volar-radial incurvature of the distal phalanx of the little finger. We report a 5-year-old girl with dystelephalangy of all fingers. Some members of the mother's family showed dystelephalangy of the little fingers; the father's family history was unaffected. Contrast-enhanced MRI showed enhancement of the soft tissues of the distal phalanges, but no bone deformities. This leads to the assumption that the radial and volar deviation of the distal phalanges is the result of a chronic inflammatory process or a vascularisation disorder of the soft tissue.     

Pediatric diffuse lipoblastomatosis of the foot -- a case report and review of the literature.

Diffuse lipoblastomatosis is a rare lipomatous tumor of infancy that derives from fetal-embryonal fat. It usually affects infants and children and presents as a superficial mass that extends from the subcutis to the underlying muscle in the upper or lower extremities. In spite of its benign nature, the tumor may behave in a locally aggressive manner and invade the surrounding tissues. A case of isolated diffuse lipoblastomatosis of a 5-year-old boy in the plantar aspect of his right foot that has not been documented in that area before is described. The clinical and radiological presentation and histologic evaluation of diffuse lipoblastomatosis are discussed, in order to make a differential diagnosis between this rare tumor and other soft tissue tumors. In addition, the treatment principles of the tumor are described. This case demonstrates that diffuse lipoblastomatosis can be difficult to diagnose without histologic evaluation and, once diagnosed, should be treated by adequate surgical excision in order to prevent recurrence.     

Prospects for targeted therapy of synovial sarcoma

Synovial sarcoma is a distinct tumor with unique promise for targeted therapy. It has a diagnostic translocation and a potentially informative fusion protein. It has moderate chemosensitivity, with about 50% response rates to regimens containing ifosfamide and doxorubicin. Therapeutic advances are unlikely to occur by continuing to lump synovial sarcomas in trials with other soft tissue sarcomas and adjusting traditional agents; rather, attention should be turned toward prospective molecular targets and investigation or development of novel agents to exploit them. The SYT-SSX fusion protein that results from the X,18 translocation is an appealing target, as are the proteins overexpressed in synovial sarcoma: bcl-2, EGFR, and HER-2/neu.     

Nonradiopaque penetrating foreign body: “a sticky situation”

Foreign bodies within soft tissues are common in children. They may cause a chronic inflammatory reaction that can result in abnormal findings on radiographs, including lytic or blastic osseous changes. These radiographic findings can mimic both benign and malignant processes. In cases where the history is uncertain and the foreign body is not recognized, magnetic resonance (MR) imaging can make a specific diagnosis and direct appropriate therapy. Received: 27 April 1998 Accepted: 23 February 1999     

Vertebral osteoid osteoma masquerading as a malignant bone or soft-tissue tumor on MRI

Purpose. Four pediatric patients were sent to our institution with the diagnosis of soft-tissue/malignant bone tumor. In all cases an MRI was the initial study performed for neck or back pain. All were surgically proven to have an osteoid osteoma/osteoblastoma (OO) as a final diagnosis. The MRI findings are reviewed.¶Methods. Four patients, three boys and one girl, ranging in age from 5 to 17 years, presented with symptoms of neck or back pain for 2 months to 2 years. Two had neurological findings. All patients underwent MRI.¶Results. All MRIs demonstrated decreased T1 signal and increased T2 signal in the soft tissues and bone surrounding the lesions consistent with edema. Enhancement was observed in the adjacent soft tissues and in the lesion nidus retrospectively.¶Conclusion. Investigating neck or back pain with an initial MRI may lead to misleading diagnoses unless the radiologist is aware of the typical MRI appearance of vertebral osteoid osteoma.