Treatment of neuroblastoma with [131I]metaiodobenzylguanidine: long-term results in 25 patients.

From 1984 to 1990 we have treated altogether 25 children with [131I]metaiodobenzylguanidine (131I-MIBG) for a refractory, relapsed or metastasized neuroblastoma. Three children had stage III and 22 children had stage IV of the disease; at diagnosis their ages were between 4 months and 10 years. Children with stage III disease had at diagnosis a median age of 3.0 years and at treatment 3.8 years. After first-line chemotherapy 2 children had achieved a complete remission (CR), while in 1 child the tumor did not respond (NR) to the initial treatment. At the time of 131I-MIBG treatment 2 children had relapsed and in the other one no further response was achievable. The children were treated by a 13.5 +/- 12.9 mCi/kg BW per course with a mean total dose of 280.7 +/- 243.9 mCi. One child achieved CR by 131I-MIBG alone, while in 2 cases no measurable success was observed. All 3 children were treated additionally by surgery, chemotherapy and bone marrow transplantation (BMT). Two children have died but one is alive and in CR. The 22 children with stage IV disease were treated in two different study groups. In group A, 14 children were studied for side-effects and response to 131I-MIBG. All children were pretreated with standard chemotherapy. Five were treated in relapse, 5 in progression and 3 at a refractory state of the disease; only 1 child was in complete remission when being treated with 131I-MIBG. Group A patients were treated with a mean of 2.4 courses, with 10.3 mCi/kg BW for each course.(ABSTRACT TRUNCATED AT 250 WORDS)     

Schistosoma mansoni Infections in Young Children: When Are Schistosome Antigens in Urine,Eggs in Stool and Antibodies to Eggs First Detectable?

Background

In Uganda, control of intestinal schistosomiasis with preventive chemotherapy is typically focused towards treatment of school-aged children; the needs of younger children are presently being investigated as in lakeshore communities very young children can be infected. In the context of future epidemiological monitoring, we sought to compare the detection thresholds of available diagnostic tools for Schistosoma mansoni and estimate a likely age of first infection for these children.

Methods and Findings

A total of 242 infants and preschool children (134 boys and 108 girls, mean age 2.9 years, minimum 5 months and maximum 5 years) were examined from Bugoigo, a well-known disease endemic village on Lake Albert. Schistosome antigens in urine, eggs in stool and host antibodies to eggs were inspected to reveal a general prevalence of 47.5% (CI₉₅ 41.1–54.0%), as ascertained by a positive criterion from at least one diagnostic method. Although children as young as 6 months old could be found infected, the average age of infected children was between 3¼–3¾ years, when diagnostic techniques became broadly congruent.

Conclusion

Whilst different assays have particular (dis)advantages, direct detection of eggs in stool was least sensitive having a temporal lag behind antigen and antibody methods. Setting precisely a general age of first infection is problematic but if present Ugandan policies continue, a large proportion of infected children could wait up to 3–4 years before receiving first medication. To better tailor treatment needs for this younger ageclass, we suggest that the circulating cathodic antigen urine dipstick method to be used as an epidemiological indicator.     

131I]metaiodobenzylguanidine and high-dose chemotherapy with bone marrow rescue in advanced neuroblastoma.

High-dose chemotherapy (HDT) and autologous bone marrow rescue (ABMR) is routinely used as consolidation treatment in advanced neuroblastoma. This study is presently examining the efficacy and toxicity of combined [131I]metaiodobenzylguanidine (131I-MIBG) therapy with HDT and ABMR. Five children (4 male, 1 female), median age of 8 years (range 4-11 years) were treated, 3 at relapse and 2 after initial chemotherapy. A single infusion of 131I-MIBG (median activity 11.1 GBq, range 7.4-11.2 GBq) was followed by HDT and ABMR 14-32 days later. High-dose chemotherapy consisted of carboplatin and melphalan in 4 patients, and vincristine, etoposide, carboplatin and melphalan in 1. One patient developed a septicaemia and died, and another failed to engraft; both had extensive bone marrow infiltration at the time of 131I-MIBG therapy. The combined therapy was well tolerated by the three other patients. Two children have relapsed and died (including one who failed to engraft), and 2 are alive 17 and 41 months after ABMR. In the absence of extensive bone marrow metastases, combined therapy offers potential as a means of consolidating treatment in advanced neuroblastoma.     

Non-cytotoxic asialo-GM1-positive cells exert antimetastatic activity

Summary We examined the results of discontinuing therapy in Japanese children with acute lymphoblastic leukemia. Of the 209 patients in the chemoimmunotherapy study, 120 (57.4%) had all chemotherapy stopped after 3 years of complete remission, and 72 (34.4%) reached the point of discontinuing immunotherapy after 5 years of complete remission. Of the 120 children removed from chemotherapy, 14 (11.7%) have relapsed, mainly in the extramedullary sites (5: testis, 5: bone marrow, 3: central nervous system, 1: bone); relapses occurred 1–23 months after cessation of chemotherapy (median 11 months). Boys had higher post-chemotherapy relapse rate than girls (0.21 versus 0.08, P<0.05). None of the 72 children removed from immunotherapy have yet relapsed. Long-term remission and possibly cure can be expected in approximately one-half of newly diagnosed Japanese patients. Although the active immunotherapy had no beneficial effect on the overall outcome for leukemic children, it could be of benefit to the elimination of bone marrow relapses after cessation of chemotherapy.     

Long-term follow-up after relapses in children with Hodgkin's disease

In the years 1969-1980, 68 children with Hodgkin's disease were subjected to a combined MVPP and radiotherapy. Remissions were obtained in 64 patients, and relapses occurred in 11 children. The treatment of relapse consisted in administration of B-DOPA alone or alternatively with MVPP combined with radiotherapy (in 7 out of 11 patients). The patients were recycled every 2-3 weeks which, with other modifications of chemotherapy, allowed for the completion of the six first cycles within the period of 4,5 to 7 months. A relapse caused death of one child, and two others demonstrated further relapses. At present eight children have been showing disease-free survival following a relapse for the period of 29+ to 152+ (median, 94.5 months). The authors concluded that in patients with relapses of Hodgkin's disease the decisive role rests upon aggressive chemotherapy (high frequency of cycles).     

TP化疗后奥拉帕利维持治疗对晚期卵巢癌患者近远期疗效、安全性和血清肿瘤标志物的影响

摘要 目的：探讨紫杉醇+顺铂方案（TP）化疗后应用奥拉帕利维持治疗对晚期卵巢癌患者近远期疗效、安全性和血清肿瘤标志物水平的影响。方法：选择2019年6月至2020年12月期间我院收治的78例晚期卵巢癌患者作为研究对象，分为对照组和观察组，41例和37例。所有患者均行TP化疗，观察组在化疗后接受奥拉帕利维持治疗。比较两组化疗后6个月时的临床疗效和癌症患者生活质量核心问卷（EORTCQLQ-C30）评分、化疗后2年内的无进展生存期和总生存期以及治疗过程中的不良反应发生情况。比较两组患者化疗前和化疗后6个月时血清癌抗原125（CA125）、特异性组织多肽抗原（TPS）、癌抗原199（CA199）、人附睾蛋白4（HE4）水平。结果：（1）观察组患者ORR（64.86% vs. 41.46%）和DCR（83.78% vs. 60.98%）均显著高于对照组（P<0.05）；（2）观察组化疗后6个月时的自EORTCQLQ-C30评分明显高于对照组（P<0.05）；（3）观察组化疗后6个月时血清CA125、TPS、CA199和HE水平均显著低于对照组（P<0.05）；（4）观察组中位PFS和OS分别为8.000（95%CI：7.151~8.849）和15.000（95%CI：13.505~16.495），均显著大于对照组（P<0.05）；（5）两组骨髓抑制、胃肠道反应、肝功能损伤、肾功能损伤和心脏毒性等毒副反应发生率比较均无显著差异（P＞0.05）。结论：TP化疗后应用奥拉帕利能够提高晚期卵巢癌患者近期疗效，延长生存时间，改善生存质量，安全性高。     

A possible genetic factor influencing protection from infection with Ascaris lumbricoides in Nigerian children.

An epidemiological study of Ascaris lumbricoides infections was carried out in primary school children aged 5-16 yr from Ile-Ife, Nigeria. Intensity of infection was assessed directly by means of counting worms passed during a 48-hr period after chemotherapy. Reinfection patterns of A. lumbricoides were assessed at 2 6-mo intervals and statistical evidence of predisposition to infection status was obtained. An investigation of 3 groups of children who were judged to be predisposed not to be infected, to be lightly infected, and to be heavily infected was undertaken. Assignment to the groups was based upon the mean worm burden plus 1 SD above the mean, measured at 2 6-mo intervals. The distribution of class I human leucocyte antigens among the 3 groups of children was described. None of the children who were predisposed to remain uninfected was found to possess the A30/31 antigens in contrast to those children who remained infected.     

Short course chemotherapy for tuberculous lymphadenitis in children.

OBJECTIVE--To assess the efficacy of a short course chemotherapy regimen for treating tuberculosis of the lymph nodes in children. DESIGN--Open, collaborative, outpatient clinical trial. SETTING--Outpatient department of the Tuberculosis Research Centre, paediatric surgery departments of the Institute of Child Health and Hospital for Children and the Government Stanley Hospital, Madras, South India. PATIENTS--Children aged 1-12 years with extensive, multiple site, superficial tuberculous lymphadenitis confirmed by biopsy (histopathology or culture). INTERVENTIONS--Patients were treated with a fully supervised intermittent chemotherapy regimen consisting of streptomycin, rifampicin, isoniazid, and pyrazinamide three times a week for two months followed by streptomycin and isoniazid twice a week for four months on an outpatient basis. Surgery was limited to biopsy of nodes for diagnosis and assessment. MAIN OUTCOME MEASURES--Response to chemotherapy was assessed by regression of lymph nodes and healing of sinuses and abscesses during treatment and follow up. Compliance with treatment and frequency of adverse reactions were also estimated. RESULTS--197 Patients were admitted to the study and 168 into the analysis. The regimen was well tolerated and compliance was good with 101 (60%) patients receiving the prescribed chemotherapy within 15 days of the stipulated period of six months. Those whose chemotherapy extended beyond that period received the same total number of doses. Clinical response was favourable in most patients at the end of treatment. Sinuses and abscesses healed rapidly. Residual lymphadenopathy (exceeding 10 mm diameter) was present in 50 (30%) patients at the end of treatment; these nodes were biopsied. Fresh nodes, increase in size of nodes, and sinuses and abscesses occurred both during treatment and follow up. After 36 months of follow up after treatment only 5 (3%) patients required retreatment for tuberculosis. CONCLUSION--Tuberculous lymphadenitis in children can be successfully treated with a short course chemotherapy regimen of six months.     

The premature removal of tissue expanders in breast reconstruction.

The role of tissue expanders in breast reconstruction is well established. Little information exists, however, regarding the incidence and etiology of premature removal of the tissue expander before planned exchange to a permanent breast implant. The purpose of this study was to review our 10-year experience with tissue expander breast reconstruction and identify factors relating to the premature removal of the tissue expander. This study is a retrospective review of 770 consecutive patients who underwent breast reconstruction with tissue expanders over the past 10 years. Breast reconstruction was immediate in 90 percent of patients. Patients were expanded weekly, and adjuvant chemotherapy was begun during the expansion process when required. Factors potentially affecting premature expander removal (chemotherapy, diabetes, obesity, radiation therapy, and smoking) were evaluated. Fourteen patients (1.8 percent) with a mean age of 47 years (range, 38 to 62 years) required premature removal of their tissue expander. Expanders were removed a mean of 3.2 months (0.1 to 8 months) after insertion. Causes for premature removal of the tissue expander included infection (7 patients), exposure (2), skin necrosis (2), patient dissatisfaction (2), and persistent breast cancer (1). Positive wound cultures were obtained in four of the seven infected patients (57 percent), requiring expander removal for infection. Tissue expanders were removed in 11 patients for complications directly related to the expander. Among these, six (55 percent) were receiving adjuvant chemotherapy, and one was a smoker. Diabetes, obesity, other concomitant medical illnesses, and prior mantle irradiation were not associated with expander removal. Premature removal of the tissue expander was required in only 1.8 percent of the patients in this series. Infection was the most common complication necessitating an unplanned surgical procedure to remove the expander. This study demonstrates that the use of tissue expanders in breast reconstruction is reliable, with the vast majority of patients completing the expansion process.     

Benznidazole Therapy Modulates Interferon-�� and M2 Muscarinic Receptor Autoantibody Responses in Trypanosoma cruzi-Infected Children

Objective

The presence of autoantibodies with adrenergic and cholinergic activity, capable of triggering neurotransmitter receptor-mediated effects, has been associated with pathogenesis in T. cruzi-infected hosts. The goal of this study was to investigate the production of anti-M2 muscarinic receptor autoantibodies (Anti-M2R AAbs) as well as the IFN-γ profile in children at the early stage of Chagas disease, and to examine whether trypanocidal chemotherapy with benznidazole (BZ) could modify both response patterns.

Methods

This study comprised 30 T. cruzi-infected children (mean age: 13.8 years) and 19 uninfected controls (mean age: 12.7 years). Infected patients were treated with BZ and followed-up. Blood samples collected at diagnosis-T0, end of treatment-T1, and six months later-T2 were analysed by ELISA for detection of Anti-M2R AAbs and circulating levels of IFN-γ.

Results

At T0, anti-M2R AAbs were demonstrated in 56.7% of T. cruzi-infected patients, whereas uninfected controls were 100% negative. The average age of Anti-M2R AAbs⁺ patients was higher than that from negative population. Infected children also displayed significantly stronger serum IFN-γ responses than controls. Upon BZ treatment, a significant linear decreasing trend in Anti-M2R AAb reactivity was recorded throughout the follow-up, with 29.7–88.1% decrease at T2. IFN-γ circulating levels also declined by T2.

Conclusion

Anti-M2R AAbs and IFN-γ raise early during chagasic infection in children and are downmodulated by BZ therapy. These findings reinforce the usefulness of early BZ treatment not only to eliminate the parasite but also to reduce potentially pathogenic immune responses.     

Plasma carnitine status - a prognostic factor in children with dilated cardiomyopathy

Summary Objective - Dilated cardiomyopathy is a rare disorder in childhood that results in a high mortality. The aim of our study was to evaluate the prognostic relevance of the individual plasma carnitine status in children with dilated cardiomyopathy. Methods - In 26 patients plasma carnitine concentrations were determined before and after 6 and 12 months of L-carnitine treatment. According to the plasma short chain acyl-carnitine/free carnitine ratio (AC/FC) at the first presentation children were divided into two groups. Results - In group 1 (AC/FC < 0.4) the median time from diagnosis until death was 35.8 months, the cumulative survival rate was 84% after 2 years. In group 2 (AC/FC > 0.4) median time from diagnosis until death was 8 months, the cumulative survival rate was 50% at 2 years (p < 0.05).Dividing both groups into survivors and nonsurvivors in group 2 a significantly higher AC/FC ratio in the nonsurvivors could be found (survivors 0.78 v 1.3 in nonsurvivors). A significant improvement of left ventricular function 6 and 12 months after presentation and after starting L-carnitine treatment could only be documented in the surviving patients of group 2. Conclusion - The individual plasma carnitine status in children with dilated cardiomyopathy may serve as a risk factor for survival.     

Reproductive toxicity with and without LHRHA administration during adjuvant chemotherapy in patients with germ cell tumors.

In order to evaluate the protective efficacy of an agonist of luteinizing hormone releasing hormone (LHRHA) on spermatogenic stem cells, we undertook a prospective study in patients with germ cell tumors. Following orchiectomy and unilateral lymph node dissection all patients received adjuvant chemotherapy consisting of 2 courses of PVB regimen (cisplatin, vinblastine and bleomycin). Six men were treated with LHRHA (d-Ser-(TBU)6 LHRH ethylamide) before, during and after PVB chemotherapy. Eight patients without LHRHA protection served as controls, receiving the identical chemotherapy. Follicle-stimulating hormone (FSH), luteinizing hormone (LH), and testosterone were within normal limits before therapy in all patients. In 6/6 protected patients, serum levels of FSH, LH and testosterone were effectively suppressed during pre-chemotherapeutic LHRHA administration. All protected patients showed elevated serum FSH levels and azoospermia after cessation of chemotherapy and LHRHA treatment due to germ and stem cell loss. Median FSH level and sperm density of the protected group normalized within 24 months after chemotherapy. In all unprotected patients elevated FSH values and azoospermia also occurred after chemotherapy. Likewise, median FSH level and sperm density normalized spontaneously in this group within 24 months after chemotherapy. Our results suggest completely reversible reproductive toxicity two years after 2 courses of adjuvant chemotherapy in all patients. Administration of LHRHA during chemotherapy seems to have no protective effects on germ cells since both groups developed reproductive toxicity. Furthermore, recovery time was identical in the protected and unprotected patients. FSH and LH could be used as diagnostic markers to assess the degree and duration of reproductive and endocrine gonadal toxicity after chemotherapy.     

Risk factors for ocular infection with Chlamydia trachomatis in children 6 months following mass treatment in Tanzania

Background

Trachoma is the leading infectious cause of blindness in the world, and for endemic communities, mass treatment with azithromycin reduces the pool of infection. High coverage is essential, especially in children as they are the infectious reservoir. However, infection remains post-mass treatment. We sought to determine risk factors for infection in children post-mass treatment.

Methodology

All children under 9 years in 4 villages in Tanzania were followed from baseline pre-mass treatment to six months post treatment. 1,991 children under nine years were enrolled in the longitudinal study and data on individual and household characteristics was collected at baseline. Clinical trachoma was determined by an ocular exam and infection detected by PCR of an eyelid swab. Azithromycin was offered and infection was reassessed at 6 months. A multilevel logistic regression model was used, accounting for household clustering of children for analysis.

Principal Findings

Baseline infection was 23.7% and at 6 months was 10.4%, despite 95% coverage. Infection at baseline was positively associated with infection at 6 months (OR = 3.31, 95%CI 2.40–4.56) and treatment had a protective effect (OR = 0.45, 95%CI 0.25–0.80). The age group 2–4 years had an increased risk of infection at 6 months. The household characteristics predictive of infection at 6 months were increasing number of children infected in the household at baseline and increasing number of untreated children in the household.

Conclusions

While one round of mass treatment with high coverage did decrease infection by over 50%, it appears that it is not sufficient to eliminate infection. Findings that young children (ages 2–4 years) and households with increasing numbers of infected and untreated children have a positive association with infection at 6 months suggest that such households could be targeted for more intensive follow up.     

Impact of schistosome infection on Plasmodium falciparum Malariometric indices and immune correlates in school age children in Burma Valley, Zimbabwe

A group of children aged 6–17 years was recruited and followed up for 12 months to study the impact of schistosome infection on malaria parasite prevalence, density, distribution and anemia. Levels of cytokines, malaria specific antibodies in plasma and parasite growth inhibition capacities were assessed. Baseline results suggested an increased prevalence of malaria parasites in children co-infected with schistosomiasis (31%) compared to children infected with malaria only (25%) (p = 0.064). Moreover, children co-infected with schistosomes and malaria had higher sexual stage geometric mean malaria parasite density (189 gametocytes/µl) than children infected with malaria only (73/µl gametocytes) (p = 0.043). In addition, a larger percentage of co-infected children (57%) had gametocytes as observed by microscopy compared to the malaria only infected children (36%) (p = 0.06). There was no difference between the two groups in terms of the prevalence of anemia, which was approximately 64% in both groups (p = 0.9). Plasma from malaria-infected children exhibited higher malaria antibody activity compared to the controls (p = 0.001) but was not different between malaria and schistosome plus malaria infected groups (p = 0.44) and malaria parasite growth inhibition activity at baseline was higher in the malaria-only infected group of children than in the co-infected group though not reaching statistical significance (p = 0.5). Higher prevalence and higher mean gametocyte density in the peripheral blood may have implications in malaria transmission dynamics during co-infection with helminths.     

Increased incidence of hepatitis B markers in children with sickle-cell anemia

Screening of 143 children 6 months to 12 years of age with sickle-cell anemia showed that 39.2% were HBsAg-positive as compared with 19.3% of the 161 control children of the same age group, who had Hb genotype AA (chi 2 = 14.7383; P less than 0.001). Fifty percent of the HbSS children under the age of 1 year were HBsAg-positive as opposed to 4.3% of the control group (chi 2 = 9.1955; P less than 0.001), while 28.6% of patients were HBsAg-positive at the age of 4 years compared with only 7.4% of the controls at the same age. The incidence of anti-HBc in both groups was similar. Markers of HBV infection (HBsAg + anti-HBc) were, however, on the whole higher in the patients with sickle-cell anemia [88/118 (74.6%)] than in the controls [54/88 (61.3%; P less than 0.005)].     

Reconstruction of late craniofacial deformities after irradiation of the head and face during childhood

Little is known about the results of surgical management of late craniofacial abnormalities arising after irradiation of the head and face for treatment of childhood cancers. The clinical records of 10 children (4 males and 6 females) who received 4500 to 6500 rads (mean 5160 rads) of craniofacial radiation between birth and 8 years of age (mean 5 years) and who subsequently had reconstructive surgery were reviewed. Six of the 10 patients received orbital radiation, 3 received maxillary-midfacial radiation, and 1 patient underwent radiation to the frontal bone. Histologic tumor types included retinoblastoma (4), rhabdomyosarcoma (3), Ewing's sarcoma (2), and neurofibrosarcoma (1). In addition to radiation, 7 of the 10 patients underwent surgical resection or debulking of their tumors and 6 received adjuvant chemotherapy. All patients presented from 4 to 20 years after treatment (mean 10 years) with varying, but severe degrees of soft-tissue and bony hypoplasia of the irradiated territories. Onlay bone grafting with soft-tissue reconstruction by a combination of local pedicle flaps and dermal-fat grafts was initially performed in 9 patients, and an occipitoparietal bone-flap switch procedure was done in 1 patient. Late follow-up ranged from 11 months to 7.5 years (mean 34 months). A total of 8 secondary procedures were necessary in 4 of the 10 patients (40 percent). Of these 4 patients, major revisions were performed in 3 and minor adjustments in 1. In addition, 2 patients in whom secondary procedures had not been done would benefit from further reconstruction. Therapy for cancer of the head and face during childhood has profound and ongoing effects on the growth of soft tissue and bone.(ABSTRACT TRUNCATED AT 250 WORDS)     

The provocation test in children with cow-milk protein and gluten intolerance: evaluation of the clinical response and lesions in the mucous membrane of the small intestine

Provocation test (re-introduction of the noxious protein) was carried out in two groups of patients: (a) with intolerance to the cow-milk proteins (41 children) treated with milk-free diet for 6-24 months, and (b) with gluten intolerance (26 children) treated with gluten-free diet for 6-36 months. The following parameters were compared: type and frequency of the clinical symptoms seen in these patients prior to the introduction of allergen-free diet. Moreover, the type of observed morphological changes in the small intestine mucosa following provocation test were analysed in the groups of 7 patients. A two-year elimination of milk from the diet produces milk tolerance in about 61% patients; clinical symptoms in the remaining children are diversified. Re-introduction of gluten with the diet (provocation test) produces recurrence of gluten intolerance in 96% of children treated with gluten-free diet for 2-3 years. Recurrence of the disease was accompanied by the atrophy of the intestinal villi.     

Long term observation of children with Giardia intestinalis invasion treated with metronidazole and furazolidone]

The results of a long-term (3-year) follow-up of children infected with G. intestinalis treated with metronidazole and furazolidone are presented. Therapy was effective in 84-95% of cases, depending of the duration or the follow-up. Repetition of therapy with tinidazole was indicated in 5% of children after 6 months and in 15% of children after 1 and 2 years of observation because of persisting symptoms of the infection or recurrence. More frequent the treatment was necessary in children under 3 years of age. Some (14%) children required milk free diet and in a few cases (underlying disease--coeliac) gluten-free diet or a diet free of main allergens.     

The role of radiotherapy in the prevention of recurrence and central nervous system metastases in childhood medulloblastoma

PURPOSE: To present the postoperative radiotherapy technique in children with medulloblastoma, and analyse the effectiveness of radiotherapy and the survival data. MATERIALS AND METHODS: 66 consecutive children (45 male and 21 female) received postoperative chemotherapy and radiotherapy between 1986 and 1998. The mean age was 8.3 years. The radiotherapy was performed with linear accelerator 9MV X-ray irradiation. The high risk patients received 36 Gy craniospinal irradiation, the low risk patients recived 30 Gy. The boost irradiation to the posterior fossa was 20 Gy in both patient groups. The patients received multi-drug chemotherapy immediately after the tumor resection. The radiotherapy started 6-8 weeks after the operation. RESULTS: All 66 patients were evaluated. The mean follow-up time was 45.4 months. The chance of cure is higher at age 8 or more, and less favorable under age 8. After 60 months 68.6% of children under age 8 and 75.9% older than 8 are alive. 20 children (64.5%) are alive after radical tumorectomy and 11 died. The 5 year overall survival was 71%. Recurrence was observed in 23/66 cases, it was the most frequent cause of death. Local failure was in posterior fossa in 15 patients (68.2%). CONCLUSION: The radicality of operation had no significant influence to the overall survival. The tumor stage, age of patients, risk group and metastases are important prognostic factors.     

Prognostic Role of Adjuvant Chemotherapy in Node-Negative (N0), Triple-Negative (TN), Medullary Breast Cancer (MBC) in the Korean Population

Background

Despite the favorable prognosis for medullary breast cancer (MBC), the guidelines for the use of adjuvant chemotherapy for MBC have not been clearly established. This study investigated the prognostic role of adjuvant chemotherapy in Korean patients with node-negative (N0), triple-negative (TN) MBC patients.

Methods

We included data from 252 patients with N0 TN MBC, obtained from the Korean Breast Cancer Registry database. Patients were categorized as those who did not undergo adjuvant chemotherapy (group I) or those who did (group II). Clinicopathological characteristics, breast cancer-specific survival (BCSS), and overall survival (OS) were compared between the groups. In addition, a subgroup analysis for survival based on tumor size was conducted.

Results

A total of 252 N0 TN MBC patients with tumor sizes >1 cm who were diagnosed between April 1997 and March 2011 were enrolled. The median age was 44.95 years (range, 25–72 years), and the median follow-up period was 93.94 months (range, 23–195 months). Overall, the BCSS and OS in group II (97.3% and 97.3%, respectively) were significantly better compared with those in group I (89.2% and 86.2%, respectively). In the subgroup analysis, in patients with tumors >2 cm in size, those in group II had significant better BCSS and OS (97.5% and 97.5%, respectively) compared with those in group I (78.3% and 73.9%, respectively). In contrast in those with tumors 1–2 cm in size, there were no significant differences in BCSS and OS between the groups (both 97.1% for group I, and 95.2% and 92.9%, respectively for group II). Multivariate analysis revealed that adjuvant chemotherapy significantly improved BCSS (P = 0.009) and OS (P = 0.007), but only for patients with larger tumors (>2 cm).

Conclusions

In patients with N0 TN MBC, adjuvant chemotherapy had a significant clinical survival benefit, but only in those with tumors >2 cm.