Giant benign cystosarcoma phylloides in the adolescent female

Cystosarcoma phylloides is an uncommon breast tumor in children. About 30 cases have been reported in girls under the age of 20 years. A case of giant benign cystosarcoma phylloides in a 15-year-old girl is reported. Offprint requests to: N. Gürses     

VP-16 + adriamycin vs. Adriamycin alone in advanced adenocarcinoma of the breast,phase II,a randomized trial: A southwest oncology group study

One hundred twenty-two patients with advanced adenocarcinoma of the breast were randomized to receive adriamycin (AD) alone or a combination of VP-16 plus lower dose adriamycin (VAD). The patients were stratified to good and poor risk. The starting dose (day 1) of AD was 60 mg/m² for good risk and 45 mg/m² for poor risk. The starting dose of the VAD combination for the good-risk patient was VP-16, 75 mg/m² daily × 5 plus adriamycin 35 mg/m². The poor-risk dose for VAD was VP-16, 50 mg/m² daily × 5 plus adriamycin, 30 mg/m² on day 1. The total dose of AD was 450 mg/m² on both arms. The patients who were on the VAD arm continued on VP-16 maintenance. Both arms were repeated every 21 days. There were 54 evaluable patients on the adriamycinarm and 52 evaluable patients on the VAD arm. Both arms were similar with regard to age, menopausal status, performance status, and prior hormonal therapy. More hematologic toxicity was seen in the adriamycin arm. Complete responses were observed on both arms, three (5%) with adriamycin and three (5%) with combination. Eleven (19%) and ten (18%) partial responses were observed with the adriamycin and VP-16 plus adriamycin, respectively. AD produced more stable disease than VAD (41% vs. 29%). Complete responses were seen only in the good-risk patients. Time to progression was delayed on the combination arm (P = 0.02). The survival in both arms was similar. The addition of VP-16 to adriamycin does not offer an important clinical advantage.     

A neonatal presentation of factor V deficiency: A case report

Background  

Factor V deficiency is a rare autosomal recessive coagulation disorder. Awareness of presenting features and management is important to avoid bleeding complications associated with mortality and neurodisability.     

Asymptomatic intracardiac metastasis from osteosarcoma: A case report with literature review

Osteosarcoma very rarely metastasizes to the heart. Thirteen cases have been reported in the literature so far. Diagnosis in some of these cases was made during investigation for severe cardiac failure and in most of them at autopsy. Our patient, a 13-year-old girl, showed right pulmonary metastases on chest x-ray 1 year after above knee amputation for osteosarcoma of the distal femur. Routine preoperative computerized axial tomography (CT Scan) revealed a calcified lesion in the heart in addition to the pulmonary metastases. She was very active and completely asymptomatic. Two-dimensional echocardiography, angiography, and right and left heart catheterizations were done. This revealed a large mobile metastatic lesion in the right ventricle. The intraventricular tumor was successfully removed, and 12 days later she had a second thoracotomy for removal of pulmonary metastases. Nine months after her intraventricular metastasis was removed she developed a solitary right pulmonary metastasis. This was successfully resected. Now, 10 months later, she is disease free and completely asymptomatic.     

Three dose regimens of adriamycin for induction of remission in acute leukemia in children: A Southwest oncology group study

Three therapeutic regimens (6-hr intervals for 6 doses daily for 3 days, and a single infusion) utilizing the same total dose of adriamycin for the induction of remission in children with late-stage acute leukemia were evaluated in 150 patients. Complete remission rates were 15%, 28%, and 25%, respectively. Twenty-five children who achieved complete bone marrow remissions with adriamycin therapy were treated a second time after subsequent relapses. Of these children 20% achieved second bone marrow remissions.     

Tuberculosis of the breast in an adolescent girl: a rare presentation

Tuberculosis (TB) of the breast is a rare condition and usually affects women in the reproductive age group. Its further rarity in children, even in India, where TB is rampant, poses challenges in its diagnosis. Histopathology plays an important role in the diagnosis of this condition. We report a case of TB of the breast in a 15-year-old girl proven by histopathological study.     

Castleman disease: a case with atypical presentation

Castleman disease is a benign lymphoproliferative disorder characterized by enlarged lymph nodes. In children the disease is rare, usually localized, and asymptomatic. Resection of the node is almost always curative. A case is reported that was diagnosed as hyaline vascular-type Castleman disease at 1 year of age. The disease recurred from infraclavicular region in addition to primary site, even though total excision was performed. Although the disease is mullticentric after recurrence, the patient has no systemic symptoms.     

Carcinoid syndrome in the absence of liver metastasis: A case report and review of literature

A patient with carcinoid tumor of the head of pancreas and carcinoid syndrome presented without liver metastasis. The patient had retroperitoneal lymphadenopathy. He had symptoms of flushing, diarrhea and abdominal pain. 5-Hydroxyindoleacetic acid (5-HIAA) was elevated. Absence of liver metastasis was documented not only by the negative computed tomography (CT) scan and liver/spleen scan, but also by autopsy. Except for carcinoid arising from ovary, testis, or bronchi, the other carcinoid tumors rarely cause carcinoid syndrome without liver metastasis. The literature was reviewed, and the findings are presented. © 1992 Wiley-Liss, Inc.     

Hepatocellular carcinoma with central nervous system metastasis: A case report and literature review

In the United States and western Europe, hepatocellular carcinoma is not generally regarded as a distantly metastasizing tumor. The ability of hepatocellular carcinoma to metastasize to the brain is illustrated by the example of a 65-year-old white American man with no identifiable risk factors for primary liver cancer. Though hepatocellular carcinoma rarely metastasizes to the central nervous sytem, such metastases are reported to have led to tumor diagnosis or signaled tumor relapse.     

Secretory breast cancer in childhood and adolescence: Report of a case and review of the literature

A 17-year-old boy underwent a wide partial mastectomy with axillary dissection for secretory breast cancer, followed by low grade irradiation (30 Gy). There were no axillary lymph node metastases. Hormone receptor assay of the tumor was positive for progesterone and negative for estrogen. There was no local recurrence or sign of dissemination after nearly 5 years. Literature search revealed 21 cases of secretory breast cancer in childhood and adolescence; in only 2 cases was the hormonal status studied. We report an additional case and review the literature. © 1992 Wiley-Liss, Inc.     

Epidural metastasis in Wilms' tumor: A case report and review of the literature

Spinal canal involvement is not a common pattern of metastasis in Wilms' tumor. Although early detection and treatment can achieve improvement of neurological deficit, mortality remains high. We present a 5-year-old girl who had an epidural metastasis while she was receiving chemotherapy for stage IV Wilms' tumor. Within 2 months following laminectomy, total removal of tumor, radiotherapy, and adjuvant chemotherapy some of the neurological signs improved. © 1994 Wiley-Liss, Inc.     

Pediatric liver tumors: Initial presentation, image finding and outcome

BACKGROUND: Few reports have been carried out on the characteristics of pediatric liver tumors. METHODS: A retrospective study of 57 patients diagnosed with liver tumors from 1989 through 2004 was conducted. They were classified into groups; 10 benign, 33 primary malignant and 14 metastatic liver tumors. Their demographics, initial presentations, laboratory data, image findings and outcomes were investigated and compared. RESULTS: Hepatocellular carcinoma (HCC) with 91% hepatitis B virus-related, constituted 23 of 33 primary malignant liver tumors and had the poorest survival rate. Initially, 70% of patients with primary malignant liver tumors were at disseminated stages. All of HCC and 88% of hepatoblastoma had elevated serum levels of aphal-fetoprotein. However, abnormal liver function tests as alanine aminotransferase, total bilirubin, albumin and alkaline phosphatase were uncommon in patients with pediatric liver tumors. Metastatic liver tumors compared with primary malignant liver tumors showed hypo-echogenicity in abdominal ultrasound (US) exam and a lesser presence of vessel invasion and contrast enhancement in computed tomography studies (P < 0.01). CONCLUSIONS: It is important to diagnose primary malignant liver tumors before their clinical symptoms and signs develop. Children with chronic hepatitis B virus infection must be followed every 6 months by serum aphal-fetoprotein and abdominal US even when their liver function tests are normal. Image studies with abdominal US and computed tomography scan can differentiate between primary and metastatic liver tumors.     

Postvaricella acute transverse myelitis: A case presentation and review of the literature.

Although the complications and sequelae of varicella are rare, they can cause significant mortality and morbidity in both the normal and compromised patient. Eight cases of "pure" acute transverse myelitis (ATM) following varicella have previously been reported in the literature. We report the ninth case of postvaricella ATM in a 9-year-old girl. Her course was characterized by paraplegia, sensory loss, hyperesthesia, and incontinence of urine and feces. All patients, except ours, recovered fully from ATM.     

Low-dose adriamycin remission maintenance for advanced acute lymphoblastic leukemia: A southwest oncology group study

A low-dose maintenance schedule of adriamycin was evaluated in children with advanced acute lymphoblastic leukemia. Thirty-six evaluable patients who achieved two or more remissions were given adriamycin, 10 mg/m²/week, as a single maintenance agent. The median duration of second remission was 17 weeks, but only 5 weeks for third and fourth remission. Although the therapy was not found effective in more advanced disease, the median duration of second remission approached that obtained from various multi-agent treatment schedules. We conclude that the therapeutic efficacy of this treatment schedule makes it appealing for incorporation into multi-drug combination regimens.     

A 16-year-old male with gynecomastia and ductal carcinoma in situ

While gynecomastia is common in adolescents, male breast cancer in the pediatric population is exceedingly rare. Occasional reports describe malignant and pre-malignant lesions in mastectomy specimens for gynecomastia in young adults. We present a 16-year-old male with unilateral gynecomastia discovered to have ductal carcinoma in situ (DCIS), treated by mastectomy with 4 year follow-up.     

Ectopic production of human chorionic gonadotropin: A case report

The ectopic production of the β-subunit of human chorionic gonadotropin (hCG) is described in a patient with an anaplastic carcinoma. After chemotherapy the marker decreased in a logarithmic fashion to undetectable levels but the neoplasm progressed and the patient died. The specificity of the β-subunit of hCG is discussed. Discordance of the marker and clinical disease is pointed out, and several possible explanations are outlined. The lack of specificity of the β-subunit of hCG and the discordance that it may exhibit means that its use in diagnosing and following disease progression may be limited.     

血清IgA、IgA/C3比值对儿童原发性IgA肾病的诊断价值和肾脏病理的相关性分析

背景：IgA肾病是亚洲人群最常见的原发性肾小球肾炎,目前肾活检是诊断金标准,寻找可用于辅助诊断原发性IgA肾病的血清标志物有重要意义。 目的：探讨血清IgA及IgA/C3比值对儿童原发IgA肾病的诊断价值及其与病理分级的关系。 设计：诊断准确性研究。 方法：以肾活检病理为金标准分为原发性IgA肾病（真阳性）和非IgA肾病的原发性肾小球肾炎病（真阴性）,以血清IgA和IgA/C3比值为待测标准,建立预测儿童原发性IgA肾病的诊断模型。截取人口学特征、血清免疫球蛋白和补体检测结果、肾活检病理检查报告和其他实验室检查结果。按年龄分为1~4岁、~7岁、~11岁和~18岁亚组;根据入院时24 h尿蛋白定量水平（mg·kg-1·d-1）分为＜50和≥50亚组。 主要结局指标：血清IgA和IgA/C3比值对原发性IgA肾病的诊断效能。 结果：原发性IgA肾病150例、非IgA肾病474例进入分析。①原发性IgA肾病患儿血清IgA和IgA/C3 比值高于非IgA肾病患儿,且在各年龄亚组血清IgA升高的比例均高于非IgA肾病患儿。②血清IgA和IgA/C3比值的AUC 分别为0.824和0.851,敏感度分别为80.0%和74.3%,特异度分别为73.1%和82.7%;IgA和IgA/C3均以1~4岁的AUC最大;IgA和IgA/C3的诊断截断值均随年龄增加而升高。③血清IgA和IgA/C3比值在24 h 尿蛋白水平＜50 mg·kg-1·d-1亚组的敏感度分别为90.3%和93.5%,在不同年龄亚组24 h 尿蛋白水平＜50 mg·kg-1·d-1的患儿中的敏感度为86.8%~100%。在不同尿蛋白水平亚组,IgA和IgA/C3 比值的诊断截断值亦随年龄增加而升高。④病理分级：无论是2016版牛津分级积分＜4分和≥4分患儿比较,还是LEE氏分级Ⅰ~Ⅱ级和Ⅲ~Ⅴ级患儿比较,性别、年龄、血清IgA、补体C3和IgA/C3比值差异均无统计学意义。 结论：血清IgA/C3比值和IgA升高对于筛查原发性IgA肾病有重要价值,尤其是24 h尿蛋白＜50 mg·kg-1·d-1患儿。