Eiji Oguma

Spontaneous regression of localized neuroblastoma detected by mass screening.

PURPOSE To clarify whether and when neuroblastomas identified through screening do regress, and to ascertain how to treat them appropriately, we observed screened patients who had localized tumors, without any therapeutic intervention. PATIENTS AND METHODS The criteria for the observation program were as follows: disease stage I or II; tumor less than 5 cm in diameter; no invasion to the intraspinal canal or growth to the great vessels; urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) less than 50 microg/mg creatinine; and informed consent. Of 25 patients identified through screening for 6-month-old infants in Saitama Prefecture, Japan between April 1994 and March 1996, 11 patients who met the criteria and one other patient with stage III tumor were enrolled onto the program. They were examined by abdominal ultrasonography (US) and their urinary VMA and HVA levels were assessed approximately once per month. The observation periods ranged from 4 to 27 months. RESULTS The 11 tumors decreased in size, although one of these 11 tumors initially enlarged until the patient was 12 months of age and decreased in size thereafter. One other tumor slightly increased in size. Urinary VMA levels decreased in all patients. None of the tumors had completely disappeared by the last observation day. CONCLUSION Our results suggest that regression of screened neuroblastoma is not a rare phenomenon. At present, it seems reasonable to adopt a wait-and-see strategy, with careful observation, for selected stage I or II tumors identified in infants screened at 6 months of age.

Kasabach-Merritt phenomenon: a report of 11 cases from a single institution.

Background: Kasabach-Merritt phenomenon (KMP) is a rare condition and optimal treatments have not yet been established, especially for cases that are unresponsive to first-line therapy. We retrospectively reviewed 11 KMP cases treated over the past 13 years in our institute. Observations: With the exception of 1 case, steroids were administered as the first-line therapy. Eight cases required second-line or third-line therapy. The effective salvage therapies include interferon (n=1), radiotherapy (n=1), and chemotherapy (n=5). One case continues to depend upon chemotherapy. Three refractory cases were therapy dependent over 1 year of age, whereas 8 were treated effectively by 6 months of age. Conclusions: Chemotherapy seems to be the most effective therapy for steroid-resistant KMP cases.

Case series of pediatric acute leukemia without a peripheral blood abnormality, detected by magnetic resonance imaging

Although abnormal peripheral blood counts are a key diagnostic finding for acute leukemia in children, between 2003 and 2010 we observed seven pediatric cases without peripheral blood abnormalities and showing abnormal signals in the bone marrow by magnetic resonance imaging (MRI). The common chief complaint in these patients was bone pain and fever. Bone marrow tests revealed six out of the seven cases to be acute leukemia, whereas one patient was diagnosed with juvenile idiopathic arthritis (JIA). There was no evident difference in MRI findings between leukemia patients and JIA patient. In three cases of leukemia, initial bone marrow aspiration failed to show the presence of leukemic cells, and diagnosis was only made by repeated bone marrow examination. Our findings indicate that in some cases MRI detects leukemia at an earlier phase than does bone marrow aspiration, suggesting that MRI is useful for the diagnosis of acute leukemia.

Malignant rhabdoid tumor of the liver: a case report with US and CT manifestation

A case of malignant rhabdoid tumor (MRT) of the liver in a 4-month-old boy with hepatomegaly was described with the help of US and CT findings. The boy had multiple liver tumors, and the liver parenchyma showed a diffuse heterogeneous appearance. Lymph node swelling was noted, although the alpha-fetoprotein concentration was not elevated. A diagnosis of MRT was confirmed pathologically. Twenty-seven cases of MRT of the liver with solitary tumors, multiple tumors, or diffuse infiltration have been reported in the English-language literature. Diagnostic imaging plays an important role in detecting metastasis because of the high frequency of distant metastasis in MRT of the liver.

Spectrum of Epstein-Barr virus infection in Japanese children: a pictorial essay.

Epstein-Barr virus (EBV) infection has been associated with infectious mononucleosis, EBV-associated hemophagocytic syndrome (EBV-AHS), chronic active EBV infection (CAEBV), lymphomas, inflammatory pseudotumor, lymphomatoid granulomatosis, and nasopharyngeal carcinoma. EBV-AHS and CAEBV are more lethal than infectious mononucleosis with imaging findings of gallbladder wall thickening, pleural effusion, cardiomegaly, and hepatomegaly. EBV infection is also associated with benign and malignant tumors.

Changes in the Distance Between the Distal Rectal Pouch and Perineum From the Birth Day to the Next Day in Neonates With an Imperforate Anus

To evaluate changes in the distance between the distal rectal pouch and the perineum (pouch‐perineum distance) from the birth day to the next day and to determine which day is better for diagnosis of a low‐type imperforate anus in neonates.

Five neonatal cases of pyriform sinus fistula with cervical cystic lesion: a comparison between sonography and other modalities

Pyriform sinus fistulas are an unusual cause of neck cystic lesions in neonates. A definitive diagnosis requires detection of the fistula, which originates from the pyriform sinus and extends to the cystic lesion. Sonography has been reported to be useful for detecting fistulas. However, there have been no reports of neonatal cases in which sonography could detect fistulas not detected by other modalities, such as barium esophagography, computed tomography (CT), or magnetic resonance imaging (MRI). We describe five neonatal patients with pyriform sinus fistula-appearing cervical cystic lesions. All patients were examined by sonography; of these, three patients were also examined by barium esophagography, two by CT, and one by MRI. The fistula was detected by sonography but not CT in one patient and by barium esophagography but not MRI in one. Two patients whose fistulas were detected by sonography had an oval-shaped and relatively small cystic lesion. One patient whose fistula could be detected only by barium esophagography had a relatively large cystic lesion. In two patients, whose fistulas were not detected by sonography or barium esophagography, sonography revealed air bubbles within the cystic lesions, and a pyriform sinus fistula was suspected. Without requiring ionizing radiation, sonography was thus useful in diagnosing pyriform sinus fistula-appearing cervical cystic lesions in neonatal cases. On the other hand, in two patients whose fistulas were not detected by sonography, the shape of the cystic lesion was polygonal or multicystic. In two of three patients with infectious signs, a fistula could not be detected. The shape and size of the cystic lesion and the presence of infectious signs may be important factors for detecting fistulas.

Role of Sonography for Evaluation of Gastrointestinal Foreign Bodies.

Foreign body ingestion is frequently encountered in children. The locations of the foreign bodies and the period during which they have been present in the thorax and abdomen are important for determining the method and timing of treatment. Although plain radiography and computed tomography are primarily used for assessment of foreign bodies, sonography without radiation is also useful for diagnosis of foreign bodies. This report describes 5 cases of foreign bodies in the digestive tract and the usefulness of sonography for real‐time evaluation of foreign bodies with high spatial resolution. Physicians can use sonography along with radiography and computed tomography in cases involving foreign bodies.

MR evaluation of talonavicular angle in congenital talipes equinovarus

The talonavicular relationship in 14 patients with talipes equinovarus was quantified using gradient echo sequences. The angle formed between the short axis of the navicular and the long axis of the talus was measured. There is a significant difference in the talonavicular angle between patients who had posteromedial release (PMR) and those who had posterior release (PR) or casting only (P=.0004). This method provides an objective assessment of residual deformity following surgical and conservative management of talipes equinovarus.

Clinical analysis and outcome of interstitial lung disease complicated with juvenile dermatomyositis and juvenile polymyositis.

Abstract Objectives: The aim of this study was to determine the clinical phenotype and outcome of interstitial lung disease (ILD) complicated with juvenile dermatomyositis (JDM) or juvenile polymyositis (JPM). Methods: This was a single-center retrospective study. From 1984 to 2015, we retrospectively reviewed 29 patients who were diagnosed with JDM/JPM, among whom eight cases were ILD and 21 were non-ILD. The clinical features and laboratory findings included chest computed tomography (CT) images that were compared between the patients with ILD and non-ILD. Results: Eight cases (27.6%) were complicated with ILD. The mean age was 6.3 years, and 75% of the patients were women. We found that high fever, arthralgia, muscle weakness, and high serum Krebs von den Lungen-6 (KL-6) level were significantly associated with the presence of ILD (p < 0.05). Two patients were positive for the anti-Jo-1 antibody, and two other patients were positive for the anti-MDA5 antibody. Three cases were identified as rapidly progressive (RP)-ILD. The chest CT images of the ILD patients appeared to show ground glass opacity (GGO) with a lower lobe predominance, reticulation, and traction bronchiectasis consolidation. Three patients with RP-ILD showed random subpleural GGO with/without consolidation patterns. Further, three patients with RP-ILD died of respiratory failure (p < 0.01). Conclusion: ILD is one of the most serious complications of JDM/JPM. In the early phase of ILD, high levels of serum KL-6 can be detected, regardless of the respiratory symptoms. Additionally, RP-ILD can be predicted based on the presence of anti-MDA5 antibodies and the chest CT findings, including random subpleural GGO with/without consolidation patterns.