Gastric Leiomyoblastoma (Epithelioid Leiomyoma) Occurring in a Child: a Case Report

Leiomyoblastomas are rare smooth muscle tumors occurring most commonly in the gastrointestinal tract of adults. We report the unusual occurrence of a gastric leiomyoblastoma in a child who presented with refractory iron deficiency anemia. The tumor had a superficial erosion which was apparently the source of intermittent hemorrhage resulting in anemia. Epithelioid cells, spindled cells, and cells arranged in a perithelial pattern were seen by microscopy, characteristic of a leiomyoblstoma. Immunohistochemical staining demonstrated muscle actin, vimentin, desmin, and alpha-1-antichymotrypsin, and electron microscopy showed focal densities alternating with thin filaments. All of these features help to differentiate the tumor from an inflammatory pseudotumor. Leiomyoblastomas are associated with paragangliomas and pulmonary chondromas in children, but these were not present in this child. The prognosis of leiomyoblastomas may be related to tumor size, mitotic rate, and cellular characteristics.     

Castleman's disease of the mesentery in a child: A case of seven years' duration without typical x-ray findings

This report describes a 9-year-old boy with intermediate variant type of giant lymph node hyperplasia or Castleman's disease (CD) originating from the mesentery. He had symptoms and signs related to the disease for seven years before the final diagnosis. The patient's general condition remained good, except for periods of fever and abdominal pain. Pallor and slow growth were the only abnormal findings on physical examination during the follow-up. Laboratory measurements showed worsening microcylic anemia, low serum iron level, and low iron stores in bone marrow samples. The erythrocyte sedimentation rate (ESR) increased to 110 mm/h, and the serum levels of C-reactive protein varied between 80 and 120 mg/l. The level of serum albumin was low, 25–28 g/l, and serum immunoglobulin G was somewhat elevated, varying between 17–13 g/l. The radiologic examination of intestine gave pathological results suggesting a small bowel disease, but no tumor was detected. The abnormal laboratory values and symptoms of the patient resolved completely after surgical removal of the mass. Med. Pediatr. Oncol. 28:362–365, 1997. © 1997 Wiley-Liss, Inc.     

Primary lymphoma of the pericardium: Report on a „Cured”︁ case and review of the literature

This report describes a 62-year-old man with a primary diffuse, immunohistochemically proven B-cell lymphoma of large non-cleaved cell (centroblastic) type of the pericardium. The patient responded completely to systemic chemotherapy and remains free of disease 30 months after diagnosis. The use of non-cardiotoxic drugs in divided doses as initial treatment is emphasized. In addition, the authors reviewed the literature of the last decade regarding the management and outcome of patients with primary cardiac lymphomas. © 1994 Wiley-Liss, Inc.     

Pulmonary capillary haemangiomatosis in children and adolescents: report of a new case and a review of the literature

Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontrolled proliferation of pulmonary microvessels which may invade pulmonary, bronchial and vascular structures, resulting in diffuse alveolar haemorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmonary hypertension (PH). A 14-year-old boy with some particular features (pericardial effusion and thrombocytopenia) is presented and 14 paediatric/adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and histopathological studies, with the aim of providing a clear-cut distinction of PCH from other conditions such as primary PH (PPH). The literature data can be regarded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of PCH and other (vascular) disorders and associations, involving focal or diffuse, disseminated forms is summarised briefly. Conclusion:The diagnosis of this progressive disorder may lead to effective therapy. Treatment possibilities include the rapidly evolving field of anti-angiogenic therapy, but at present lung transplantation is universally accepted as the final definitive treatment for pulmonary capillary haemangiomatosis.     

A case of Epstein–Barr virus–associated pulmonary leiomyosarcoma arising five yr after a pediatric renal transplant

Suzuki K, Urushihara N, Fukumoto K, Watanabe K, Wada N, Takaba E. A case of Epstein–Barr virus–associated pulmonary leiomyosarcoma arising five yr after a pediatric renal transplant. Pediatr Transplantation 2011: 15: E145–E148. © 2010 John Wiley & Sons A/S. Abstract: EBV‐associated SMTs in immuno‐compromised patients have recently been reported. We report on a case of EBV‐associated pulmonary leiomyosarcoma arising five yr after renal transplantation. The patient was an eight‐yr‐old girl, who received a living related kidney transplant from her mother. She had had bilateral giant Wilm’s tumors as an infant and underwent bilateral nephrectomy at one and two yr of age. At the age of seven, she suffered from bronchitis several times, and a year later, two nodules were detected in her left lung by X‐ray and computed tomography. We suspected a recurrence of Wilm’s tumor and performed surgical resection. The pathological finding was SMT with moderate mitosis and no evidence of Wilm’s tumor. The fact that the tumors were positive for EBER suggested an association with the EBV. Six months later, there was a recurrence in her left lung. Surgical resection was performed, and immunosuppressive agents were reduced. Two yr after the second operation, she is well with no recurrence. We report the first case of EBV‐associated pulmonary leiomyosarcoma in a pediatric patient after renal transplantation owing to a malignant tumor.     

Hypokalaemia and failure to thrive: report of a misleading onset

Aim: We report a case of Gitelman Syndrome (GS) in a 9‐year‐old girl, previously diagnosed as a Bartter syndrome at one year of life. Methods: She had been treated with potassium, for over 8 years and was admitted because of fatigue, numbness and weakness of both legs. The patient has typical laboratory findings, including hypokalemia, metabolic alkalosis, hypomagnesemia, and hypocalciuria, thus GS was suspected. Results: Genetic analysis was performed two mutations IVS9(+1)G>T were detected in the thiazide‐sensitive Na‐Cl cotransporter (TSC) gene (SLC12A3), thus she was diagnosed as having GS. She was treated with oral potassium and magnesium supplements with resolution of the symptoms. Conclusion: This case reminded us that doctors should be alert to the initial presentation of renal tubular diseases. Detailed electrolyte analysis, hormone evaluations and clinic follow‐up are mandatory for their correct differential diagnosis.     

Osteoglophonic dysplasia: a new case

A new case of osteoglophonic dysplasia is described in a Portuguese boy. The clinical and radiographic features are similar to the five previously described cases. The boy died suddenly at 10 months of age.     

Relationship between treatment center case volume and survival for localized Ewing sarcoma: The role of radiotherapy timing

In the treatment of localized Ewing sarcoma (EWS), delays in local therapy are known to adversely impact overall survival (OS). However, the role of treatment center volume in EWS outcomes, and the interaction between center volume and local therapy timing with definitive radiotherapy, remains unknown. Using the National Cancer Database, we demonstrate that treatment at the lowest EWS volume centers is associated with reduced OS, explained partly by higher rates of delayed local therapy. Treatment at the highest volume centers results in improved OS, but appears independent of radiotherapy timing. Future efforts to improve care for EWS patients across treatment centers are imperative.     

肺脏超声联合X线在肺动脉高压诊治中的作用

目的探讨肺脏超声对继发性肺动脉高压的诊断及疗效评估。方法回顾分析1例继发性肺动脉高压患儿诊治过程中肺脏超声同胸部X线的变化。结果患儿,男,出生2天,因呼吸困难入院。入院胸片示先天性心脏病改变(肺血增多型);超声心动图示肺动脉高压、动脉导管未闭、卵圆孔未闭,肺脏超声示胸膜线异常、A线消失、肺实变及支气管充气征等呼吸窘迫综合征的表现。经过抗感染,肺表面活性物质替代等治疗,复查肺脏超声提示A线出现、实变消失。3周后患儿治愈出院。结论肺脏超声对于继发性肺动脉高压的诊治具有一定指导意义,对比胸部X线具有一定的优越性。     

Necrotizing arteritis in acute poststreptococcal glomerulonephritis: report of a recovered case.

A patient with biopsy documented acute poststreptococcal glomerulonephritis and arteritis recovered completely with supportive therapy. Illness was preceded by group A streptococcal pharyngitis. At the time of presentation, serum creatinine concentration was 11.5 mg/dl. Serum cryoglobulins containing IgG and C3 were present. The first biopsy, performed during the acute illness, contained glomeruli with typical features of acute PSGN. Medium-sized arteries had extensive necrosis and leukocytic infiltration, and contained IgG, C3, and fibrin. Glomerular filtration rate returned to normal within three weeks; proteinuria cleared by three months, and microscopic hematuria by 11 months. Renal biopsy one year later showed minimal mesangial hypercellularity and no arteritis.     

Congenital Na+ diarrhea: a new type of secretory diarrhea

We report a new type of congenital "secretory diarrhea" in a 9-year-old girl that led to contraction and severe metabolic acidosis in the first weeks of life. Her fecal Na+ concentration was high and the pH alkaline. All known causes of secretory diarrhea were excluded. Our findings indicate a defect in the handling of Na+ and H+ in the distal ileum and colon. Treatment with orally administered Na-K-citrate supplementation has normalized her fluid and electrolyte status and allowed normal growth and psychomotor development, but the diarrhea has persisted.     

Hodgkin's disease and non-Hodgkin's lymphoma in children and young adults: A clinicopathologic study of 127 cases

One hundred twenty-seven cases of non-Hodgkin's lymphoma and Hodgkin's disease in children and young adults at the University of Wisconsin Hospital between 1969 and 1980 have been reviewed. Nodular sclerosing was the most frequent histologic type in patients with Hodgkin's disease. Malignant lymphoblastic lymphoma (MLLB) was the most common type of non-Hodgkin's lymphoma. The relationship of the histological pattern to age and sex as well as clinical behavior and survival are discussed.     

Oculo-cerebro-cutaneous syndrome: description of a new case

A newborn girl is described with the following multiple congenital anomalies: an orbital cyst, cutaneous appendages, focal hypo-aplasia of the skin and multiple cerebral cysts. This case of oculo-cerebro-cutaneous syndrome is compared with four previously published cases.