Thoracoabdominal peripheral primitive neuroectodermal tumors in childhood: radiological features.

Peripheral primitive neuroectodermal tumors (PNET) are extremely uncommon, malignant neoplasms affecting mostly children and young adults. We retrospectively reviewed the clinical data and radiological studies of four such cases. All cases were pathologically proven. Plain films, US, and CT scans were used. The youngest child had a huge pelvic tumor and two adolescents each had a chest wall (Askin) tumor. The fourth patient had a most unusual location of the PNET in the anterior mediastinum. The CT findings are emphasized. We emphasize that the markedly abnormal CT findings are not specific for PNET.

Post-traumatic recto-spinal fistula.

Acquired recto-spinal fistula has been described elsewhere as a rare complication of colorectal malignancy and Crohn's enterocolitis. We treated a young man who developed a recto-spinal fistula as a result of a high fall injury. The patient presented with meningeal signs, sepsis and perianal laceration. Computerized axial tomography revealed air in the supersellar cistern. Gastrografin enema showed that contrast material was leaking from the rectum into the spinal canal. Surgical management included a diverting sigmoid colostomy, sacral bone curettage and wide presacral drainage. To the best of our knowledge, rectospinal fistula of traumatic origin has not been previously reported in the English literature.

Intrathoracic rib demonstrated by helical CT with three-dimensional reconstruction.

Intrathoracic rib is a rare congenital anomaly. An unusual location with atypical pleural tenting is reported. Helical CT with three-dimensional reconstruction seems the best modality for demonstrating the origin and location of these abnormal ribs. The importance of the diagnosis of intrathoracic rib is to rule out pulmonary lesions and prevent unnecessary investigations.

Chronic intussusception in childhood.

A rare case of chronic intussusception is reported. Radiological investigation including ultrasound, CT and barium enema provided the correct diagnosis. Ultrasound revealed a solid mass near the transverse colon in the right lower abdomen. CT demonstrated the real nature of the solid mass as being the classical 'coiled spring' of intussusception. The barium enema was unsuccessful as an attempt at hydrostatic reduction, but confirmed the diagnosis by a 'crescent-shaped' filling defect in the ascending colon. The patient was discharged after an uneventful surgery and recovery. No organic lesion that precipitated the chronic intussusception was discovered at operation. Radiological findings, the typical clinical picture and adequate treatment are discussed.

CT findings in neonatal hypothermia.

BACKGROUND: Newborn infants are particularly prone to hypothermia, a condition with a high mortality. OBJECTIVE: To study the CT brain patterns in infants with hypothermia and neurological symptoms. MATERIALS AND METHODS: We reviewed the brain CT of nine infants with neonatal hypothermia, multiple organ failure, seizures and coma. RESULTS: Two infants had normal CT scans, acutely and at follow-up, and were clinically normal at follow-up. In seven infants, CT showed diffuse cerebral oedema, with reversal of the normal density relationship between grey and white matter and a relative increased density of the thalami, brainstem and cerebellum - the 'reversal sign'. In six surviving infants with severe developmental delay, follow-up CT revealed cerebral atrophy with multicystic encephalomalacia. CONCLUSIONS: The 'reversal sign' has been described in the abused child, birth asphyxia and anoxia due to drowning. Neonatal hypothermia is offered as a further cause.

Ganglioneuroma: an 'incidentaloma' of childhood.

In adults clinically silent adrenal masses can be discovered incidentally in imaging studies. Most of these 'incidentalomas' are benign, non-functioning adenomas. In contradistinction, in infancy and childhood the most common adrenal mass is the neuroblastoma, a malignant neural crest tumour. Four children are described, each with a benign neural crest tumour - ganglioneuroma - incidentally discovered by conventional radiography or sonographic examination. Complete surgical excision resulted in total recovery of all the children.

CT and US features of cervical lymphadenopathy due to kikuchi's disease.

Kikuchi's disease or histiocytic necrotising lymphadenitis is a distinct benign clinicopathologic entity which is to be distinguished from malignant conditions, especially lymphoma.We report a young woman with Kikuchi's disease presenting with cervical lymphadenopathy and mild hepatomegaly. CT and US findings are presented as well as a review of the pertinent literature.

Pelvic granulocytic sarcoma.

Granulocytic sarcoma is an uncommon extraskeletal tumor most frequently associated with leukemia. We present a case of bone location with unusual pattern in a patient with no evidence of myeloproliferative disorder at presentation or follow-up.

Vertebral pneumatocyst. A case report.

STUDY DESIGN: This study illustrates intraosseous pneumatocyst of the vertebral body, a benign lesion. OBJECTIVES: To review the incidence and location of this benign lesion during a 1-year period. SUMMARY OF BACKGROUND DATA: Intraosseous pneumatocyst is a rare benign condition, commonly seen in iliac bone or sacrum. The etiology of this entity is unclear. Other locations of these lesions are very rare, and only a few isolated cases are reported in the literature. METHODS: In the last year (1994-1995), vertebral pneumatocyst was incidentally found in four patients who underwent computed tomography examination for presumptive discal lesion. Axial computed tomography with 2- and 4-mm slice thickness was performed. RESULTS: The typical computed tomography patterns of intraosseous pneumatocyst involving the cervical, dorsal, or lumbar spine were found. The bony structure and joints were normal. To the best of our knowledge, intraosseous pneumatocyst located in the spinal process has not been reported. CONCLUSIONS: Intraosseous pneumatocyst is a benign lesion. Biopsy and follow-up are unnecessary.

Ovarian fibroma in a prepubertal girl.

Ovarian fibroma rarely occurs in prepubertal girls. However, we found such an asymptomatic, large, uncalcified pelvic mass in an otherwise healthy 12-year-old girl. The plain films (radiographs), US and CT findings are presented.

A severe form of Sjogren's syndrome.

Sjogren's syndrome is an autoimmune disease characterized by exocrine gland destruction and manifested by parotid, submandibular and lacrimal gland infection. We report a case with recurrent severe parotid gland infections. The sialographic and CT findings are presented.

[Plasma cell granuloma of the chest and lung in childhood].

Plasma cell granuloma is a benign, non-neoplastic lesion rarely found in children. It occurs mainly in the chest and lungs, the right lung mostly. Most cases are asymptomatic and are discovered incidentally on routine chest X-ray, although there may have been atypical upper respiratory symptoms. The X-ray findings, as well as those of other imaging modalities, are nonspecific, making the exact localization and diagnosis of the lesion difficult, as demonstrated in the cases of 2 boys aged 8 and 9 years, respectively. Locating the right diaphragm and its relation to the large lesion by various imaging modalities was unreliable in 1 of the cases. The large, dense, firm, adherent lesions were carefully and completely resected in both cases. In 1 case it was located in the right lung, pulmonary hilum and mediastinum. In the other, in the right pleural space in the supradiaphragmatic region, adherent to the posterolateral aspect of the lower ribs. Biopsies for frozen section should always be taken before deciding on the extent of surgery. Radical resection of normal surrounding tissue should be avoided. Our cases have been followed for 2 and 3 years respectively, with no evidence of recurrence. Prognosis is excellent when lesions are completely removed.

Sturge-Weber syndrome associated with a large left hemispheric arteriovenous malformation.

A patient with the characteristic changes of Sturge-Weber syndrome on the right side was found to have a large arteriovenous malformation on the left side which involved the superior ophthalmic vein. This association, although previously reported, is extremely rare.

Calcification in an untreated case of Burkitt's lymphoma: radiographic, ultrasound and CT diagnosis.

Calcification in untreated lymphoma is exceedingly rare. We report an unusual case of Burkitt's lymphoma, detected on plain radiographs and by ultrasonographic and CT examination.

Pulmonary plasma-cell granuloma.

A large pulmonary plasma-cell granuloma (PCG) mimicking a mediastinal germinal-cell tumor in a 9-year-old boy is presented. The nonspecificity of the tumoral calcifications and of the radiographic and CT findings in pulmonary PCG is demonstrated.

Ultraviolet-B (290-320 nm)-irradiation inhibits epidermal growth-factor binding to mammalian cells.

Mitogens, such as polypeptide growth factors and phorbol ester tumor promoters, act by binding to specific receptors and inducing a pleiotropic response in cultured mammalian cells, which results in the induction of cellular proliferation. An early effect of such agents is the inhibition of binding of epidermal growth factor (EGF) to its receptor. Ultraviolet radiation has also been shown to induce a proliferative response in vivo and in vitro and to act as a tumor promoter in animal skin. We, therefore, examined the effect of ultraviolet radiation (UVB - 290-320 nm) on EGF binding to cells in culture. We found that UVB (100-300 J/m2) induced a rapid, dose-dependent inhibition of EGF binding in a mouse fibroblast cell line, which resulted from a decrease in both number and affinity of binding sites. Phosphorylation of the EGF receptor by protein kinase C (PKC) is not likely to be the mechanism for inhibition, since UVB treatment did not result in PKC activation or modulation of phorbol diester binding.

Treatment of hot flashes with transdermal estradiol administration.

A randomized prospective double blind study was performed to assess the ability of a transdermal therapeutic system (TTS) delivering estradiol (E2) to suppress hot flashes (HFs) in symptomatic postmenopausal women. Patients were given placebo or E2 in four doses for a 20-day period, and serum gonadotropin and estrogen levels and the occurrences of HFs were measured. Administration of placebo had no measurable effect on either estrogen or gonadotropin levels or the occurrence of HFs. A dose-response relationship was found between the rate of E2 administered and the circulating level of E2, with 25, 50, 100, and 200 micrograms/24 h dosages raising the mean E2 concentrations from mean baseline levels of 5-8 pg/ml to 18, 38, 73, and 100 pg/ml, respectively. Estrone levels also increased with TTS application, but to a lesser extent than did E2 levels. Dose-response reductions of FSH and LH with increasing amounts of E2 administration occurred, but gonadotropin levels were not lowered in any of the patients into the ranges found in premenopausal women. TTS application significantly suppressed the occurrence of HFs at the 50 micrograms/24 h dosage and higher. A significant negative correlation (r = 0.6045; P less than 0.001) between E2 levels and the rates of occurrence of HFs was found during hormone administration. Based on this regression, 50% and 100% reductions of HFs should occur at 61 and 122 pg/ml E2. These data indicate that the transdermal delivery of E2 with these systems significantly reduced the occurrence of HFs and allowed definition of the therapeutic range of hormone replacement in terms of lost ovarian function, as reflected by circulating E2 levels.

Further delineation of hypothalamic dysfunction responsible for menopausal hot flashes.

An association exists between pulsatile LH release and hot flashes (HFs). To further delineate the hypothalamic mechanism(s) responsible for HF, the basal levels and pulsatile release of LH, FSH, estradiol, and estrone and the rate of occurrence of HFs (measured objectively) were evaluated in patients with a defect of GnRH secretion [isolated gonadotropin deficiency (IGD)], patients with abnormalities of afferent input to GnRH neurons [hypothalamic amenorrhea (HA)], and postmenopausal women with severe HFs. Patients with IGD had received estrogens, which were discontinued before study. Patients with HA had experienced regular menses before disease onset, which followed emotional stress or weight loss. Studies were limited to HA patients with estrogen levels in the postmenopausal range. Pulsatile LH release was absent in patients with IGD and was absent or greatly reduced in women with HA. Objectively measured and subjectively experienced HFs occurred in IGD but not in HA patients. These results suggest that HFs are not an obligatory consequence of low endogenous estrogen levels and that the absence of episodic LH and GnRH release (IGD) does not influence the occurrence of HFs. It is possible that the dysfunction of afferent input to GnRH neurons in HA somehow prevents HFs in these women with low endogenous estrogen secretion.

Estrogen replacement therapy by transdermal estradiol administration.

To determine whether the nonoral administration of estradiol (E2) might provide physiologic replacement without alteration of hepatic function, 20 postmenopausal women were studied before and after 3 weeks of treatment with either E2-containing transdermal therapeutic systems or placebo. Twenty premenopausal women were also studied. With E2-containing systems, serum E2 and estrone levels were restored to the premenopausal range. Variable responses of the different biochemical and biologic markers of the actions of E2 were observed. The most sensitive marker was vaginal cytology, with the E2 dosage reverting the maturation index to premenopausal values. Hot flashes, measured objectively, were reduced in frequency but not abolished. Serum levels of follicle-stimulating hormone and luteinizing hormone were lowered but remained higher than the premenopausal range. No significant changes were noted in urinary calcium/creatinine and hydroxyproline/creatinine ratios, which were used as markers of bone resorption. With active systems, no significant changes were noted in the concentrations of the hepatic proteins renin substrate and thyroxine-binding globulin or in the binding capacities of cortisol-binding globulin and sex hormone-binding globulin. These results indicate that transdermal E2 administration may be used to provide estrogen replacement while exerting limited effects on hepatic function.