Sarcoid Reaction in Primary Neuroblastoma: Case Report

We present a unique case of a 9-month-old infant with a left adrenal neuroblastoma with sarcoid reaction, detected by mass screening. There was no clinical evidence indicating systemic sarcoidosis or pulmonary mycobacterial infection. Histological examination of the resected adrenal tumor revealed many noncaseating epithelioid granulomas with lymphocytic infiltrate, composed of epithelioid cells and few giant cells, arising in tumor parenchyma and fibrovascular stroma. Most of the lymphocytes in the granulomas were CD3- or CD45RO-positive T cells, with fewer being CD20-positive B cells. The lymphocytes in the epithelioid granulomas expressed CD4 or CD8, but not CD56 and CD57. CD4-positive cells were observed more within the granulomas (internal area) than in the surrounding area (external area) of the same granulomas, while most of the CD8-positive cells were seen consistently at the outer margin of the granulomas (marginal zone). CD45RA-positive T cells were observed predominantly in the external area. The results of immunostaining demonstrated that lymphocytes in granulomas of this case showed the same distribution pattern as that seen in systemic sarcoidosis. Although the sarcoid reaction is a phenomenon known to be associated with the region of cancer, granuloma within the primary neuroblastoma is extremely rare. The sarcoid reaction in the present case of neuroblastoma may be associated with a delayed-type hypersensitivity reaction, and its significance and relevance still remain obscure. Received September 9, 1998; accepted October 21, 1999.     

原发性干燥综合征1例

患儿,女,13岁。近1年余反复5次腮腺大在我院门诊就诊。近3个月感口干,吞咽干性食物时需用水帮助,眼干,反复咳嗽。入院体检:体温38℃,脉搏92次/min,呼吸26次/min,发育正常,双腮腺大,不硬,无压痛及波动感,表面光滑,泪腺大,有白色分泌物。心音有力,律齐,双肺呼吸音低,未闻及音。     

Renin Levels in Nephroblastoma (Wilms' Tumour): Report of a Renin Secreting Tumour

The case history is reported of a child aged 1 year 10 months with malignant hypertension in association with Wilms'' tumour. In addition there was a marked electrolyte disturbance, with hyponatraemia and hypokalaemia, and a blood picture of microangiopathic haemolytic anaemia. All these features resolved after removal of the kidney and tumour. Pre-operative plasma renin concentration was extremely high and had returned to normal 3 months after operation. The renal tumour showed a marked degree of differentiation, with glomerulus-like structures attached to tubules, and significant quantities of renin were found on assay. No renin was detected in the renal cortex of the affected kidney. In two other normotensive patients with Wilms'' tumour no renin could be detected in the tumour tissue. We consider that the evidence suggests that this patient had a renin-secreting nephroblastoma.     

Third-Degree Heart Block in Thalassemia major: A Case Report

Background

First and second-degree heart blocks are partly common rhythm disorders in thalassemic patients but complete heart block is a very rare complication of iron overload cardiomyopathy.

Case Presentation

This 15-year-old boy, a known case of major β-thalassemia was admitted to our emergency unit with dyspnea and cough because of decompensated heart failure. The electrocardiogram showed complete heart block with junctional escape rhythm. Interestingly, his previous electrocardiogram taken 2 months earlier, had some PVC and second degree, Mobitz type 1 (Wenckebach) heart block. After improvement of dyspnea and control of blood pressure in normal range, the patient was referred to ER. A dual-chamber permanent pacemaker was implanted and his symptoms improved, but he died 24 days after discharge from hospital.

Conclusion

We present a rare case of complete heart block after a second-degree (Mobitz 1) heart block that was due to severe iron overload cardiomyopathy.     

骨原发性恶性淋巴瘤1例

患儿,男,12岁,3个月前不慎摔伤右手,当即感右肘部疼痛、迅速肿胀,活动受限,诊断为右肱骨骨折。行手法复位后,石膏夹板固定,1个月后自行拆夹板,约20 d后骨折处局部肿胀并渐加重,10 d前肘部开始疼痛并渐加重。故来我院就诊。门诊以右肱骨远端恶性肿瘤收住院。查体:体温36℃,脉搏7     

Primary Burkitt-like Lymphoma Presenting as a Solitary Rectal Polyp in a Child: Case Report

Primary rectal lymphoma in childhood is rare. We report a case in a 10-year-old boy who presented with rectal bleeding and a single rectal polyp. Histologic examination, immunophenotyping and molecular genetic study of the polyp showed a diffuse B-cell lymphoma, Burkitt-like type. The literature on this topic is reviewed and pathologic examination of childhood rectal polyps is emphasized.     

伴甲状旁腺功能低下的原发性免疫缺陷病1例

患儿，男，13岁。以反复抽搐5d为主诉入院。患儿5d前无诱因抽搐，为大发作形式，持续1min自行缓解。此后同种性质抽搐发作3次。入院前4d发热，咳嗽，自服退热药2d热退。入院前1d左下肢频繁无规律抽动，无力，行走困难。患儿3．5岁患重症肺炎住院2次，6岁因化脓性心包炎住院治愈。8岁因特发性关节炎住院。9岁因左膝关节滑膜炎院住骨科抽液。否认肝炎结核病史。否认输血史。家族史：有一兄因肺炎发展为败血症3岁死亡，     

腺样体肥大并右心衰竭1例

患儿,男,2岁6个月,张口呼吸1年余,咳嗽、气喘20d。1年来出现张口呼吸,睡眠时打鼾,夜间有憋醒,近20d出现阵发性咳嗽,气喘较明显。既往有反复呼吸道感染和双下肢水肿史。查体:体温38℃,脉搏158次/min,呼吸45次/min,体质量13kg,血压100/70mm Hg。神清,精神差,发育、营养稍差。腺样     

Presacral Mesenchymoma: a Case Report

A 4-year-old girl with a presacral benign mesenchymoma is reported. The nature of the tumor was not recognized preoperatively or intraoperatively. There was evidence of pericapsular infiltration of the soft tissues of the pelvis but not signs of malignancy. A follow-up examination after 3 months indicates that the patient has no clinical evidence of recurrence. Review of the English literature shows the rarity of benign mesenchymoma in childhood and the lack of reports of its occurrence in the pelvis.     

Primary Malignant Fibrous Histiocytoma of the Lung in a Child: A Case Report and Review of Literature

Malignant fibrous histiocytoma (MFH), an aggressive high-grade soft tissue sarcoma, usually occurs in the elderly during the fifth to seventh decade of life. It commonly arises in the retroperitoneum, extremities, and head and neck region. Primary pulmonary MFH is extremely rare and is frequently fatal. We present the youngest known case, a 9-year-old boy with a primary left lung grade II inflammatory MFH, stage II. He underwent a left upper lobectomy for tumor resection. After completing radiation therapy, he was started on vincristine, actino-mycin D, and cyclophosphamide alternating with vincristine, doxorubicin, and cyclophosphamide every 3 weeks. After five such cycles, he had a histologically proven local recurrence. He then received chemotherapy consisting of ifosfamide (2 g/m²) and etoposide (VP-16) (100 mg/m²) given daily for 3 days every 3 weeks. The patient attained complete remission (CR) after five such cycles and completed treatment without any major complications. He received a total of 16 courses and is continuing in CR 36 months off treatment. Ifosfamide and etoposide (VP-16), known for their usefulness in treatment of adult soft tissue sarcomas, can be used as salvage chemotherapy for patients with MFH who fail the front-line conventional chemotherapy.     

Congenital Hemangiopericytoma: Report of a Case

A congenital hemangiopericytoma of the lower lip, first detected by prenatal ultrasound, was only partially resected and involuted over the next 20 months.     

Accelerated Ventricular Rhythm in Children: A Review and Report of a Case with Congenital Heart Disease

We report a child with accelerated ventricular rhythm (AVR) and congenital heart disease. Three children with congenital heart defect associated with AVR were previously reported, but in each AVR occurred only postoperatively. Because our patient's 24-hour electrocardiograph recording showed AVR rates, and differences between sinus and AVR rates, exceeding published childhood limits, we reviewed the topic. On the basis of our review, we suggest guidelines for diagnosing AVR and differentiating it from ventricular tachycardia.     

Intraoperative Transesophageal Echocardiogram Using an Intracardiac Ultrasound Catheter in a Congenital Heart Surgery: A Case Report

Transesophageal echocardiography (TEE) during cardiac surgery is a routine procedure. The use of pediatric TEE probes is limited in small infants weighing less than 5 kg. Recent reports have shown the safety of monoplane intravascular ultrasound catheters in transesophageal echocardiograms. This report describes the case of a newborn with total anomalous pulmonary venous return who underwent cardiac surgery. A pre- and postbypass TEE examination was performed, with successful visualization of the cardiac anatomy and function and no complications.     

原发性草酸盐肾病并慢性肾功能不全1例

患儿，男，6个月，因进行性面色苍白及反复呕吐4个月、反应低下1周、抽搐2d入院。既往曾因反复呕吐、面色苍白辗转就诊于各大医院，1个月前因呕吐及贫血住院治疗，作全胃肠钡餐检查未见异常，经过对症治疗3d后好转自动出院。个人史及家族史无殊。体格检查：体温不升，神志不清，呼吸节律齐，呼吸稍促，面色苍白，对针刺无反应，穿刺部位出血不止，     

Congenital Posthemorrhagic Hydrocephalus: Report of a Case

A newborn infant with hydrocephalus was found to have residua of subependymal-intraventricular hemorrhage and associated obliterative arachnoiditis. This process is well known in the postnatal period but not as an in utero event. The present article documents antenatal intraventricular hemorrhage as a cause of hydrocephalus.