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.     

A chemosensitive pediatric extraosseous osteosarcoma: case report and review of the literature

Osteosarcoma arising in soft tissues is exceedingly rare in children. The tumor most often affects older adults, involves the lower extremity, responds poorly to chemotherapy, and carries a grave prognosis. The authors describe a 12-year-old girl with an extraosseous osteosarcoma of the left sternocleidomastoid muscle with pulmonary metastases. The patient responded well to neoadjuvant chemotherapy and remains disease-free nearly 3 years after her initial diagnosis. The authors review available information about this disease in children and adults. Children with extraosseous osteosarcoma may have a more favorable response to treatment than adults; thus, a curative approach using combined modality therapy appears warranted.     

MASSIVE CARDIAC CHONDROMA PRESENTING WITH HEART FAILURE AND SUPERIOR VENA CAVA OBSTRUCTION IN A TEENAGE BOY

Histologically benign soft-tissue chondromas have been reported at many anatomical sites but are an uncommon cause of soft tissue mass lesions in childhood, accounting for less than 1% of cases. The most frequent sites for extraosseous soft-tissue chondromas are the hands and feet. For the extremely rare visceral chondromas, the site can be lung, where they may represent a component of Carney's syndrome of extra-adrenal paraganglioma, pulmonary chondroma, and epithelioid leiomyosarcoma of the gastrointestinal tract. Primary cardiac chondromas are exceptionally rare in patients of any age although cardiac chondrosarcoma, both primary and metastatic, is well reported. We present a case of a teenage boy with a fatal cardiac chondroma     

Massive cardiac chondroma presenting with heart failure and superior vena cava obstruction in a teenage boy

Histologically benign soft-tissue chondromas have been reported at many anatomical sites but are an uncommon cause of soft tissue mass lesions in childhood, accounting for less than 1% of cases. The most frequent sites for extraosseous soft-tissue chondromas are the hands and feet. For the extremely rare visceral chondromas, the site can be lung, where they may represent a component of Carney's syndrome of extra-adrenal paraganglioma, pulmonary chondroma, and epithelioid leiomyosarcoma of the gastrointestinal tract. Primary cardiac chondromas are exceptionally rare in patients of any age although cardiac chondrosarcoma, both primary and metastatic, is well reported. We present a case of a teenage boy with a fatal cardiac chondroma     

Gallium citrate Ga 67 scintigraphic detection of chronic osteomyelitis in children with leukemia

Chronic osteomyelitis producing unexplained fevers without localizing symptomatology may present difficulty in detection and differentiation in patients with leukemia. Gallium citrate Ga 67 scintigraphy offers a method of detection and localization of both the osseous and extraosseous manifestations of such a process. Once identified, further evaluation by conventional radiographic or sectional imaging techniques may further define the process. We describe three patients with leukemia and unexplained fever in whom occult chronic osteomyelitis was diagnosed by 67Ga scintigraphy, which offers an ideal screening procedure and should be utilized in this clinical setting.     

CHILDHOOD LIPOSARCOMA: A Single-Institutional Twenty-Year Experience

Allogeneic bone marrow transplantation has proved to be a radical form of cure in patients with beta-thalassemia major who have a human leukocyte antigen identical donor. Although malignant neoplasms are serious late complications of bone marrow transplantation, very few reports describing the development of malignant tumors after allografting for thalassemia appeared in the literature. A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed for beta-thalassemia major. The phenotypic features of the patient's family fullfill the criteria for Li-Fraumeni syndrome. The patient was treated with chemotherapy and radiotherapy and died with recurrent disease. To the authors' knowledge, this is the first case of extraosseous Ewing's sarcoma after bone marrow transplantation for thalassemia. The possible contribution of transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are discussed.     

A case with extraosseous Ewing's sarcoma: a late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome?

Allogeneic bone marrow transplantation has proved to be a radical form of cure in patients with beta-thalassemia major who have a human leukocyte antigen identical donor. Although malignant neoplasms are serious late complications of bone marrow transplantation, very few reports describing the development of malignant tumors after allografting for thalassemia appeared in the literature. A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed for beta-thalassemia major. The phenotypic features of the patient's family fullfill the criteria for Li-Fraumeni syndrome. The patient was treated with chemotherapy and radiotherapy and died with recurrent disease. To the authors' knowledge, this is the first case of extraosseous Ewing's sarcoma after bone marrow transplantation for thalassemia. The possible contribution of transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are discussed.     

Diffuse uterine leiomyomatosis in a child

Leiomyomas are the most common benign uterine tumor; however, this entity is relatively uncommon in the pediatric population. Although leiomyomas most commonly present as solitary uterine masses, unusual patterns of growth have been described including diffuse leiomyomatosis. In this condition, the myometrium of the uterus is symmetrically expanded by innumerable confluent leiomyomas; this pattern of growth is quite uncommon and has never been reported in a pediatric patient. This case report illustrates the imaging appearance of diffuse uterine leiomyomatosis in an otherwise healthy 16-year-old girl.     

Frontal bone agenesis in a patient of spinal dysraphism

Associated cranial abnormalities with spinal dysraphism are not uncommon. We came across an unusual case of a 1-year-old male child with spinal dysraphism having lumbar meningomyelocele, who also had split cord malformation (hemicord with intervening bony spur) with lipoma of one of the hemicord and filum terminale. The patient also had communicating hydrocephalus without Chiari malformation and also near-total frontal bone agenesis. Single photon emission computed tomography scanning of brain revealed normal perfusion. In the first stage of repair, the patient had postoperative CSF leak for which ventriculo-peritoneal shunt was performed. This constitutes a rare anomaly associated with spinal dysraphism.     

Post traumatic prepubertal high-flow priapism: a rare occurrence

High-flow or arterial priapism is an uncommon condition resulting from perineal or penile trauma. The classical features are a painless erection of the cavernous bodies while the corpus spongiosum stays flaccid. This case highlights a 11-year-old boy who presented with painless semi rigid erection of 3 days duration following a blunt skateboard injury to his perineum. Following failed conservative treatment, the patient underwent bilateral internal pudendal angiography with localization and embolization. Follow up in 1 month showed patient to have normal nocturnal and reflexogenic erections. Medical and surgical treatments for high-flow priapism have been described in literature with varying degree of success and complications. The current accepted standard of care is pelvic angiography and super-selective embolization of internal pudendal artery.     

Extraosseous Ewing sarcoma of the thyroid gland

Ewing sarcoma of the bone is a highly malignant round-cell tumor that typically presents between 10 to 20 years of age and is more common in boys. It can have an extraosseous origin, although it is rare. We report a case of extraosseous Ewing sarcoma in the thyroid gland in a 9-year-old girl.     

Extraosseous tumor uptake of 99m Technetium Phosphate Compounds in children with abdominal neuroblastoma

Sixteen patients with abdominal neuroblastoma had 99m Technetium Phosphate Compounds (99m TC-PC) bone scans as a preoperative evaluation for metastatic disease. Ten patients (62%) had extraosseous tumor uptake while six patients (38%) did not. There was no difference in the incidence of tumor calcification, tumor necrosis or hydronephrosis in the two groups. However, VMA levels were significantly higher in the group with extraosseous tumor uptake. Various bone seeking radionuclides are compared to 99m TC-PC and possible mechanism for extraosseous uptake of such radionuclides are postulated. Awareness of the frequency of such uptake should reduce the possibility of errors in the interpretation of bone scans in patients with neuroblastoma.     

Tubulo-interstitial nephritis and uveitis syndrome associated with mastitis: a case report

A new case of tubulo-interstitial nephritis and uveitis (TINU) syndrome in a 14-y-old girl is described. In this patient unilateral mastitis was an associated feature. The aetiology of this rare syndrome, the prognosis for which is usually good, is still unknown. The most common theory supports a cell-mediated immune response induced by infectious/antigenic stimuli. CONCLUSION: The presence of acute unilateral mastitis in this patient may represent an additional localization in TINU syndrome.     

Primary spinal epidural Ewing sarcoma: a case report and review of the literature

Primary extraosseous Ewing sarcoma is a rare entity, especially in the spinal epidural site. Less than 20 cases have been reported in the literature. Here, we present a previously healthy 12-year-old boy who complained of low back pain, progressive gait disturbance and weakness of right lower extremity for nearly one month before admission. Magnetic resonance imaging showed one solitary posterior extradural mass, measuring 4 x 2.2 x 2.1 cm, with severe cord compression at the level from T7 to T9. The mass appeared hypo-intense on both T1-weighted and T2-weighted images and homogeneous contrast enhancement after injection of gadolinium. He underwent laminectomies of T8 and T9 and complete resection of the tumor. The pathology confirmed a diagnosis of Ewing sarcoma after immunohistochemical staining. His profound neurological deficits recovered well and no recurrence was discovered after adjuvant chemotherapy and radiotherapy. The relevant literature is reviewed and the limited cases are also analyzed.     

Presentation of M4 acute myeloid leukemia in anuric renal failure with hyperuricemia and enlarged kidneys

Extramedullary acute myeloid leukemia (AML) is not uncommon. It has been shown to involve the kidneys in most postmortem cases but is most often clinically insignificant. By contrast, acute tumor lysis syndrome is rare in AML, especially at initial diagnosis. The authors report the management of a patient with AML who had acute tumor lysis syndrome that was probably potentiated by renal leukemia and resulted in renal failure. This patient achieved remission with dose-modified induction chemotherapy administered while he was dialysis-dependent.     

Lenticular lesions: not always an epidural hematoma

Ewing's sarcoma is a tumor of the bone, which occurs most often in the diaphysis of long and flat bone. The most common sites of metastasis are the lungs and bones. Less frequently, the primary site is an intracranial or pelvic lesion (either as a soft tissue or a bone lesion). We report a case of a 16-year-old female with an extraosseous intracranial lesion, who presented with a history of minor trauma, unilateral facial swelling, and head pain. Though head computed tomography scan showed a lesion consistent with an epidural hematoma, further exploration revealed Ewing's sarcoma.     

Multiple,symmetrical non-ossifying fibromata without extraskeletal anomalies: Report of two related cases

Two patients, mother and daughter, with multiple, symmetrical, non-ossifying fibromata without extraosseous anomalies are reported. We believe that this is a separate syndrome different from multiple non-ossifying fibromata with extraskeletal anomalies (Jaffe-Campanacci syndrome).     

Streptococcus pneumoniae-associated haemolytic uremic syndrome following influenza A virus infection

Influenza virus is a seasonal cause of community-acquired pneumonia, and Streptococcus pneumoniae is one of the most common pathogens causing secondary bacterial pneumonia. S. pneumoniae-induced haemolytic uremic syndrome is an uncommon condition mainly observed in young children. We present a patient who had invasive pneumococcal disease and haemolytic uremic syndrome. Simultaneous viral cultures grew influenza A. To the best of our knowledge, this is the first such reported case.     

Corrected Transposition of the Great Arteries with Isolated Aortic Coarctation: In Utero Echocardiographic Diagnosis

Physiologically corrected transposition of the great arteries (cTGA), defined by discordant atrioventricular and ventriculoarterial connections, is an uncommon congenital cardiac malformation. It rarely exists without associated cardiac anomalies, the most common of which are ventricular septal defect, pulmonary outflow obstruction, tricuspid valve (systemic) deformity, and rhythm disturbances. Conversely, hypoplasia of the systemic ventricle and systemic inflow or outflow obstructions have seldom been reported, although their recognition may significantly influence surgical repair and the patient's prognosis. We report a case of cTGA with complete heart block, moderate hypoplasia of the systemic ventricle, and severe aortic coarctation that was echocardiographically diagnosed in utero at 30 weeks' gestation because of fetal growth retardation and persistent fetal bradycardia. After delivery the patient underwent epimyocardial pacemaker implantation and aortic coarctation repair at 2 weeks of age. Unfortunately, the patient died on the seventh postoperative day because of systemic ventricular hypertrophy. Although it is well known that fetal echocardiography may reliably diagnose uncommon congenital cardiac malformations, to the best of our knowledge, this paper represents the first reported case of antenatal diagnosis of this complex anomaly.     

Enterococcus faecalis: an unusual cause of meningitis in a child with non-Hodgkin lymphoma

Enterococcal meningitis is an uncommon disease in children, most frequently reported in infants or in children with central nervous system pathology. We report a rare case of Enterococcus faecalis meningitis in an 11-year-old child with non-Hodgkin lymphoma. The patient during the course of chemotherapy became neutropenic, febrile, agitated, and disoriented with clinical signs of meningeal irritation. Culture of cerebrospinal fluid yielded Enterococcus faecalis. The patient was successfully treated with ampicillin without any neurological defects.