Ectopic thymic hyperplasia in a patient treated for Burkitt lymphoma

Thymic hyperplasia results from thymic regrowth after atrophy during stressful conditions such as burns, surgery, infection, and chemotherapy. Although thymic lesions are relatively common causes of anterior mediastinal masses, they also can develop in other mediastinal compartments on rare occasions. It is well known that thymic tissue can develop in ectopic intrathoracic lesions. Few cases of ectopic thymus associated with disturbance during thymus embryogenesis have been reported as incidental findings. We report the case of a 4-year-old boy with true thymic hyperplasia from an ectopic thymus after successful treatment for Burkitt lymphoma. This is a rare finding in the differential diagnosis of a middle mediastinal mass in a child following chemotherapy for lymphoma. The diagnosis of thymic hyperplasia from an ectopic thymus can be confirmed only histologically.     

Rebound thymic hyperplasia after liver transplantation for a child with biliary atresia; a case report

The thymus gland possesses the ability to regrow in children leading to a newly developed anterior mediastinal mass. This condition may represent a rebound phenomenon during recovery from a stressful event such as post‐chemotherapy and hence was described as RTH. RTH after LT has not been well documented. We are reporting an infant with BA who underwent LT and presented with a symptomless anterior mediastinal mass, detected on follow‐up imaging 6 months thereafter. Surgical partial excision was performed to rule out other differential diagnoses of a solid mass in the anterior mediastinum of an infant particularly lymphoma—that may arise as post‐transplant lymphoproliferative disorder—and teratoma, as well as the other aggressive lesions such as thymoma and thymic carcinoma. The final pathological analysis revealed true thymic hyperplasia, consistent with RTH. The diagnosis of RTH should be considered for a child presenting by anterior mediastinal mass after LT.     

Thymic and adenotonsillar enlargement after successful treatment of malignancies

Anterior mediastinal and adenoid masses in children after cessation of chemotherapy for malignant disease often cause a diagnostic problem. Differential diagnosis of thymic enlargement and adenoid hyperplasia from recurrence frequently poses a challenge both for the radiologist and the physician. In this study the authors evaluated 491 patients with different malignant tumors for thymic and adenoid hyperplasia. Thymic hyperplasia was seen in 18 patients (5 Hodgkin disease (HD), 5 non-Hodgkin lymphoma (NHL), 4 Wilms tumor, 2 germ cell tumor, 1 Ewing sarcoma, and 1 neuroblastoma), only adenotonsillar hyperplasia was seen in 6 patients, all with NHL, and both thymic and adenotonsillar hyperplasia were seen in 3 patients (1 HD, 2 NHL). In 5 patients, adenoid hyperplasia was proven by biopsy; 1 patient underwent to adenoidectomy. Their histopathologic investigation showed polyclonal follicular hyperplasia. The authors recommend that patients with thymic and/or adenotonsillar enlargement after successful treatment of their primary malignancy should be evaluated cautiously before an invasive procedure is planned.     

Response‐dependent and reduced treatment in lower risk Hodgkin lymphoma in children and adolescents,results of P9426: A report from the Children's Oncology Group

Background

Hodgkin lymphoma is highly curable but associated with significant late effects. Reduction of total treatment would be anticipated to reduce late effects. This aim of this study was to demonstrate that a reduction in treatment was possible without compromising survival outcomes.

Methods

Protocol P9426, a response‐dependent and reduced treatment for low risk Hodgkin lymphoma (stages I, IIA, and IIIA₁) was designed in 1994 based on a previous pilot project. Patients were enrolled from October 15, 1996 to September 19, 2000. Patients were randomized to receive or not receive dexrazoxane and received two cycles of chemotherapy consisting of doxorubicin, bleomycin, vincristine, and etoposide. After two cycles, patients were evaluated for response. Those in complete response (CR) received 2,550 cGy of involved field radiation therapy (IFRT). Patient with partial response or stable disease, received two more cycles of chemotherapy and IFRT at 2,550 cGy.

Results

There were 294 patients enrolled, with 255 eligible for analysis. The 8‐year event free survival (EFS) between the dexrazoxane randomized groups did not differ (EFS 86.8 ± 3.1% with DRZ, and 85.7 ± 3.3% without DRZ (P = 0.70). Forty‐five percent of patients demonstrated CR after two cycles of chemotherapy. There was no difference in EFS by histology, rapidity of response, or number of cycles of chemotherapy. Six of the eight secondary malignancies in this study have been previously reported.

Conclusions

Despite reduced therapy and exclusion of most patients with lymphocyte predominant histology, EFS and overall survival are similar to other reported studies. The protocol documents that it is safe and effective to reduce therapy in low‐risk Hodgkin lymphoma based on early response to chemotherapy with rapid responding patients having the same outcome as slower‐responding patients when given 50% of the chemotherapy. Pediatr Blood Cancer 2012; 59: 1259–1265. © 2012 Wiley Periodicals, Inc.     

Dynamic contrast-enhanced MRI improves accuracy for detecting focal splenic involvement in children and adolescents with Hodgkin disease

Background

Accurate assessment of splenic disease is important for staging Hodgkin lymphoma.

Objective

The purpose of this study was to assess T2-weighted imaging with and without dynamic contrast-enhanced (DCE) MRI for evaluation of splenic Hodgkin disease.

Materials and methods

Thirty-one children with Hodgkin lymphoma underwent whole-body T2-weighted MRI with supplementary DCE splenic imaging, and whole-body PET-CT before and following chemotherapy. Two experienced nuclear medicine physicians derived a PET-CT reference standard for splenic disease, augmented by follow-up imaging. Unaware of the PET-CT, two experienced radiologists independently evaluated MRI exercising a locked sequential read paradigm (T2-weighted then DCE review) and recorded the presence/absence of splenic disease at each stage. Performance of each radiologist was determined prior to and following review of DCE-MRI. Incorrect MRI findings were ascribed to reader (lesion present on MRI but missed by reader) or technical (lesion not present on MRI) error.

Results

Seven children had splenic disease. Sensitivity/specificity of both radiologists for the detection of splenic involvement using T2-weighted images alone was 57%/100% and increased to 100%/100% with DCE-MRI. There were three instances of technical error on T2-weighted imaging; all lesions were visible on DCE-MRI.

Conclusions

T2-weighted imaging when complemented by DCE-MRI imaging may improve evaluation of Hodgkin disease splenic involvement.     

Diagnostic and therapeutic problems posed by malignant non Hodgkin lymphoma of renal origin in children. Apropos of 7 cases]

BACKGROUND. The kidneys of non Hodgkin lymphoma patients frequently contain lymphoma cells, but these tumors rarely arise in the renal tissue and are rarely located there. PATIENTS. A diagnosis of non Hodgkin lymphoma of renal origin or predominantly located in kidneys was made in 7 patients aged 2-14 years old. These patients formed part of a total of 450 patients with non Hodgkin lymphomas seen from 1974 to 1987. The first manifestation in these 7 children was an abdominal mass associated with hypertension in 3 cases; 2 of whom presented with acute kidney failure. Ultrasonography showed hypoechogenous masses in one (2 cases) or both kidneys (5 cases). The diagnosis of malignant lymphoma was made directly in only one patient who also presented with mediastinal and abdominal lymph node enlargement. In the others, the first diagnosis was Wilms tumor (5 cases) and polycystic disease of the kidney (1 case); the correct diagnosis of malignant lymphoma was later made from biopsies of renal (4 patients) or extrarenal involved tissue (2 patients). Six of the 7 cases were Burkitt lymphomas, and all 7 were in stage III (3 patients) and IV (4 patients). 6 patients (3 stage III and 3 stage IV) were successfully treated by chemotherapy with a median follow-up of 9 years. CONCLUSIONS. Differential diagnosis between non Hodgkin lymphomas of renal origin and a Wilms tumor may be difficult even though kidney failure is more frequent in lymphoma. Sonography is the best method for diagnosis showing typical hypoechogenous masses and diffuse homogeneous infiltration of the kidneys. In the absence of extrarenal (meningeal, testis, bone marrow) dissemination, definite evidence of lymphoma depends on the histological examination of tumoral tissue obtained by surgical biopsy.     

Outcome of Hodgkin Lymphoma Patients With a Posttreatment 18F-Fluoro-2-Deoxy-d-Glucose Positron Emission Tomography (FDG-PET)–Negative Residual Mass: Systematic Review and Meta-analysis

To systematically review and meta-analyze the outcome of Hodgkin lymphoma patients with a posttreatment ¹⁸F-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET)–negative residual mass. A systematic PubMed/MEDLINE database search was performed. The methodological quality of included studies was assessed. The number of patients with a posttreatment non–FDG-avid residual mass and the number of these patients who developed disease relapse during follow-up were extracted from each included study. Heterogeneity in disease relapse proportions across individual studies was assessed using the I² test, with heterogeneity defined as I² > 50%. Using a Freeman–Tukey transformation, the disease relapse proportions from each individual study were then meta-analyzed with either a fixed-effects model (if I² ≤ 50 %) or a random-effects model (if I² > 50 %). A total of 5 studies comprising a total of 727 Hodgkin lymphoma patients with an FDG-PET–negative residual mass after first-line therapy were included. The overall quality of included studies was moderate. The proportion of patients with a posttreatment non–FDG-avid residual mass who experienced disease relapse during follow-up ranged between 0% and 13.8%. There was heterogeneity in disease relapse proportions across individual studies (I² = 61.4%). Pooled disease relapse proportion (random effects) was 6.8% (95% confidence interval: 2.6%–12.5%). The disease relapse rate in Hodgkin lymphoma patients with a FDG-PET–negative residual mass after first-line therapy is approximately 6.8%. Considering the existing literature, the presence of a non–FDG-avid residual mass has not been proven yet to be associated with a worse outcome than a posttreatment FDG-PET–based complete remission status without a residual mass.     

Pediatric Hodgkin Lymphoma: CT Features at Presentation,on Treatment and its Prognostic Significance

Objective  

To evaluate the CT features of pediatric Hodgkin Lymphoma (HL) at presentation, on treatment and to identify the risk factors on CT for early relapse.     

Cervical thymic cysts

Thymic cysts are rare embryonic remnants along the course of thymic migration in the neck or the anterior mediastinum which may result in cervical masses in children, often misdiagnosed. We present the experience gained by three European tertiary care medical centers in the treatment of thymic cysts as well as the current data on the embryology, clinical presentation, diagnosis and management of thymic cysts. A retrospective study was carried out in nine patients with thymic cysts during the period 1986–2002 at the departments of Pediatric Surgery of Children’s University Hospital “Federico II” and “Santobono” Pediatric Hospital of Naples in Italy and “Aghia Sophia” Children’s Hospital of Athens in Greece. All cases were asymptomatic, appearing mainly as masses resembling branchial cyst or lymphatic malformation. Laboratory and imaging investigations were not useful for preoperative diagnosis. In one case the mass extended into the mediastinum. The histological findings of thymic tissue and Hassal’s corpuscles in the cystic wall were diagnostic. In all cases, surgery was successful and uneventful. Surgical excision was accomplished by dissection of the cystic masses from the jugular vein, carotid artery and vagus nerve and from the sternocleidomastoid muscle. The presence of a normal thymus in the mediastinum must be documented preoperatively in order to avoid the risk of total thymectomy. If a cervical thymic cyst extends into the normal thymus, attempts should be made to preserve the thymus, especially in younger patients. Thymic cysts should always be included in the differential diagnosis of lateral cervical masses, especially in children. Bruno Cigliano and Nikolaos Baltogiannis contributed equally to the work.     

Pretransplant functional imaging and outcome in pediatric patients with relapsed/refractory Hodgkin lymphoma undergoing autologous transplantation

1 Background

Pretransplant functional imaging (FI), particularly a negative positron emission tomography (PET), is a strong predictor of outcome in adults with relapsed or refractory Hodgkin lymphoma (HL), but data in pediatrics are limited.

2 Methods

The medical records of 49 consecutive pediatric patients, who received autologous transplant at a single institution, were retrospectively analyzed. All patients had either gallium or PET scan before transplant and were conditioned with carmustine, etoposide, cytarabine, and melphalan (BEAM). Deauville scores were retrospectively assigned for patients with PET (score ≥ 4 positive).

3 Results

Of the 49 patients (median age, 16.2 years), 41 (84%) were pretransplant FI negative and eight (16%) were pretransplant FI positive, after first‐ to fourth‐line salvage therapy, and a median of two salvage cycles. Eighteen patients (37%) received posttransplant radiation. At a median follow up of 46 months, 45 patients (92%) were alive and disease free, and there were three nonrelapse deaths and only one relapse death (Deauville score of 5). The 4‐year progression‐free survival (PFS) for the entire cohort was 92% (95% confidence interval [CI]: 78–97), and PFS based on pretransplant disease status was 95% (95% CI: 82–99%) in the negative FI group versus 75% (95% CI: 31–93) if positive FI (P = 0.057).

4 Conclusion

Our analysis revealed outstanding outcomes for children and adolescents with relapsed/refractory HL. There were too few relapses to identify the predictive value of pretransplant metabolic status, but pediatric patients with relapsed/refractory HL and a negative pretransplant FI had excellent survival.     

Hematological alterations and thymic function in newborns of HIV-infected mothers receiving antiretroviral drugs

Objectives

To investigate the effects of antiretroviral (ARV) drugs on hematological parameters and thymic function in HIVuninfected newborns of HIV-infected mothers.

Study design

Cross sectional study.

Setting

Chiang-Mai University Hospital, Chiang-Mai, Thailand.

Participants/Patients

49 HIV-uninfected and 26 HIV-infected pregnancies.

Methods

Cord blood samples of newborns from HIV-uninfected and HIV-infected mothers were collected. Hematological parameters were measured using automatic blood cell count. T-cell receptor excision circles (TRECs) levels in cord blood mononuclear cells (CBMCs), CD4⁺ and CD8⁺ T-cells were quantified using real-time PCR.

Main Outcome Measures

Hemotological parameters and thymic function.

Results

Newborn of HIV-infected mother tended to have lower mean levels of hemoglobin than those of HIV-uninfected mother (137 ± 22 vs 146 ± 17 g/L, P = 0.05). Furthermore, mean of red blood cell (RBC) counts and hematocrit and median of TRECs in CD4+ T-cells in the newborns of the former were significantly lower than those of the latter [3.6 ± 0.7 vs 4.8 ± 0.6 × 10₁₂ cells/L, P <0.001; 0.40 ± 0.07 vs 0.46 ± 0.05 L/L, P <0.001 and 0.53 (IQR: 0.03–5.76) vs 13.20 (IQR: 2.77–27.51) × 10^?3 pg/μL, P = 0.02, respectively].

Conclusion

ARV drugs altered hematological parameters and thymic function (TRECs CD4⁺ T-cells) in HIV-uninfected newborns of HIV-infected mothers.     

Neck masses in paediatric population: An experience with children attended the Central Teaching Hospital of Pediatrics in Baghdad 2008-2009

Background:

Pediatric neck mass is a frequent cause for surgical consultation. Neck masses can be simply classified into congenital, inflammatory, and neoplastic. Although most of the cases are due to benign processes, malignant causes must not be overlooked. The aim of this study is to assess the paediatric neck masses in Iraqi patients highlighting the distribution of cases according to their demographic characteristics and etiology.

Patients and Methods:

A cross-sectional observed study is conducted in the Department of Pediatric Surgery, at the Central Teaching Hospital of Pediatrics in Baghdad from April 2008 to March 2009. Sixty four patients with neck masses aged 14 years and below were examined and managed. The underlying causes of the neck masses were addressed and categorized.

Results:

Among the 64 patients, 42 (65.6%) were male. The inflammatory group represents 57% of the cases, while the malignant neoplasm accounts for approximately 10% of the conditions mainly due to lymphoma 5 (7.8%). Sixteen patients (25%) fall in the congenital group, in which the thyroglossal duct cyst was the commonest type. Wound infection developed in two patients, while one patient with cystic hygroma showed recurrence.

Conclusion:

Pediatrics neck masses are distributed in categories that similar in pattern and distribution in the world except the infectious/inflammatory category that shows variation in distribution in respect to the socioeconomic status. The surgical intervention and procedures are related to the facility as well as to the experience.Key words: Inflammatory, neoplastic, pediatrics neck masses, reactive lymphadenitis     

Cervical mediastinoscopy for paratracheal masses in pediatric patients

A cytological diagnosis is essential for a definitive diagnosis in children who have paratracheal lesions. Thirteen pediatric patients were biopsied using cervical mediastinoscopy. Age, gender, preoperative diagnosis, and postoperative biopsy results and complications were reviewed. Five patients had mediastinal lesions residuing or recurring after chemotherapy for either Hodgkin disease or non-Hodgkin lymphoma. In 2 of these patients, the diagnosis was recurrent disease. Among the 8 patients presenting with a paratracheal mass or enlarged lymph nodes, histopathologic diagnosis showed tuberculosis in 3 children, Hodgkin disease in 2 children, and histiocytosis X and non-Hodgkin lymphoma 1 patient in each. This study shows that cervical mediastinoscopy gave 100% correct diagnosis for mediastinal residual malignancies or uncommon forms of mycobacterium tuberculosis with paratracheal masses.     

Scaling up cancer care for children without medical insurance in developing countries: The case of Mexico

Background

In 2006, the Mexican government launched the Fund for Protection Against Catastrophic Expenditures (FPGC) to support financially healthcare of high cost illnesses. This study aimed at answering the question whether FPGC improved coverage for cancer care and to measure survival of FPGC affiliated children with cancer.

Procedure

A retrospective cohort study (2006–2009) was conducted in 47 public hospitals. Information of children and adolescents with cancer was analyzed. The coverage was estimated in accordance with expected number of incident cases and those registered at FPGC. The survival was analyzed by using Kaplan–Meier survival curves and Cox proportional hazards regression modeling.

Results

The study included 3,821 patients. From 2006 to 2009, coverage of new cancer cases increased from 3.3% to 55.3%. Principal diagnoses were acute lymphoblastic leukemia (ALL, 46.4%), central nervous system (CNS) tumors (8.2%), and acute myeloid leukemia (AML, 7.4%). The survival rates at 36 months were ALL (50%), AML (30.5%), Hodgkin lymphoma (74.5%), Non‐Hodgkin lymphoma (40.1%), CNS tumors (32.8%), renal tumors (58.4%), bone tumors (33.4%), retinoblastoma (59.2%), and other solid tumors (52.6%). The 3‐year overall survival rates varied among the regions; children between the east and south‐southeast had the higher risks (hazard ratio 3.0; 95% CI: 2.3–3.9) and 2.4; 95% CI: 2.0–2.8) of death from disease when compared with those from the central region.

Conclusion

FPGC has increased coverage of cancer cases. Survival rates were different throughout the country. It is necessary to evaluate the effectiveness of this policy to increase access and identify opportunities to reduce the differences in survival. Pediatr Blood Cancer 2013;60:196–203. © 2012 Wiley Periodicals, Inc.     

Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR)

Background

Outcome of relapsed disease of localized rhabdomyosarcoma remains poor. An individual treatment approach considering the initial systemic treatment and risk group was included in the Cooperative Weichteilsarkom Studiengruppe (CWS) Guidance.

Methods

Second-line chemotherapy (sCHT) ACCTTIVE based on anthracyclines (adriamycin, carboplatin, cyclophosphamide, topotecan, vincristine, etoposide) was recommended for patients with initial low- (LR), standard- (SR), and high-risk (HR) group after initial treatment without anthracyclines. TECC (topotecan, etoposide, carboplatin, cyclophosphamide) was recommended after initial anthracycline-based regimen in the very high-risk (VHR) group. Data of patients with relapse (n = 68) registered in the European Soft Tissue Sarcoma Registry SoTiSaR (2009–2018) were retrospectively analyzed.

Results

Patients of initial LR (n = 2), SR (n = 16), HR (n = 41), and VHR (n = 9) group relapsed. sCHT consisted of ACCTTIVE (n = 36), TECC (n = 12), or other (n = 15). Resection was performed in 40/68 (59%) patients and/or radiotherapy in 47/68 (69%). Initial risk stratification, pattern/time to relapse, and achievement of second complete remission were significant prognostic factors. Microscopically incomplete resection with additional radiotherapy was not inferior to microscopically complete resection (p = .17). The 5-year event-free survival (EFS) and overall survival (OS) were 26% (±12%) and 31% (±14%). The 5-year OS of patients with relapse of SR, HR, and VHR groups was 80% (±21%), 20% (±16%), and 13% (±23%, p = .008), respectively.

Conclusion

Adapted systemic treatment of relapsed disease considering the initial risk group and initial treatment is reasonable. New treatment options are needed for patients of initial HR and VHR groups.     

Bone marrow minimal disseminated disease (MDD) and minimal residual disease (MRD) in childhood T‐cell lymphoblastic lymphoma stage III,detected by flow cytometry (FC) and real‐time quantitative polymerase chain reaction (RQ‐PCR)

Background

Despite overlapping features of T‐cell lymphoblastic lymphoma (T‐LLy) and T‐cell acute lymphoblastic leukemia (T‐ALL), which respond favorably to T‐ALL treatment, clinical and biological differences exist. We retrospectively assessed the prevalence of submicroscopic bone marrow (BM) minimal disseminated disease (MDD) at diagnosis and the early response to treatment (minimal residual disease—MRD) and their prognostic significance in 17 children with stage III T‐LLy treated according to Berlin‐Frankfurt‐Munster (BFM) non‐Hodgkin lymphoma protocols.

Procedure

Four‐color flow cytometry (FC) was used for lymphoma associated immunophenotype and real‐time quantitative polymerase chain reaction (RQ‐PCR) for T‐cell receptor (TCR β/δ/γ) gene rearrangements with at least 0.01% sensitivity.

Results

Two markers per patient were identified in all cases using FC and in 80% using RQ‐PCR. BM MDD at diagnosis of ≥0.01% was detected by FC and RQ‐PCR in 88% and 80% of patients, respectively, and by at least one of the methods in all patients. A significant correlation was achieved between the methods by Pearson correlation analysis (P = 0.004). MRD levels significantly decreased to very low levels on day 33 in 9 out of 10 patients studied. The only patient that remained positive relapsed.

Conclusions

MDD was prevalent in stage III T‐LLy, for which we could not prove a prognostic significance in the context of ALL‐like treatment. This study shows that both FC and RQ‐PCR methods are efficient for MDD and MRD analyses in T‐LLy. Pediatr Blood Cancer 2009;52:20–25. © 2008 Wiley‐Liss, Inc.     

Burkitts’s lymphoma – an atypical presentation

Background

In female adolescents and young adults, malignancies of the genital tract are the most frequent type of cancer, closely followed by Hodgkin??s and non-Hodgkin??s lymphomas.

Case Presentation

We report an unusual case of sporadic Burkitt??s lymphoma (BL) presenting with massive bilateral ovarian infiltration, peritoneal carcinomatosis and diffuse nodular lesions of the stomach and the intestine mimicking Krukenberg tumor. Diagnostic biopsies were obtained by endoscopy of the upper gastrointestinal tract. With intensive chemotherapy, complete remission was rapidly achieved, without life-threatening tumor lysis syndrome.

Conclusion

Besides metastatic gastric adenocarcinoma, BL is an important differential diagnosis in adolescents presenting with Krukenberg tumor.     

Outcome after first relapse in children with acute lymphoblastic leukemia: a report based on the Dutch Childhood Oncology Group (DCOG) relapse all 98 protocol

Background

We report on the treatment of children and adolescents with acute lymphoblastic leukemia (ALL) in first relapse. The protocol focused on: (1) Intensive chemotherapy preceding allogeneic stem cell transplantation (SCT) in early bone marrow relapse; (2) Rotational chemotherapy in late relapse, without donor; (3) Postponement of cerebro‐spinal irradiation in late isolated CNS relapse; and (4) Treatment in very late bone marrow relapse with chemotherapy only.

Methods

From January 1999 until July 2006 all 158 Dutch pediatric patients with ALL in first relapse were recorded. Ninety‐nine patients were eligible; 54 patients with early and 45 with late relapse. Eighteen patients had an isolated extra‐medullary relapse; 69 patients had bone marrow involvement only.

Results

Five‐years EFS rates for early and late relapses were 12% and 35%, respectively. For early relapses 5 years EFSs were 25% for patients transplanted; 0% for non‐transplanted patients. For late relapses 5 years EFS was 64% for patients treated with chemotherapy only, and 16% for transplanted patients. For very late relapses EFS was 58%.

Conclusions

Our data suggest the superiority of SCT for early relapse patients. For late relapses a better outcome is achieved with chemotherapy only using the rotational chemotherapy scheme. The most important factor for survival was interval between first CR and occurrence of the first relapse. Pediatr Blood Cancer 2011; 57: 210–216. © 2011 Wiley‐Liss, Inc.

     

Risk-adapted chemotherapy without procarbazine in treatment of children with Hodgkin lymphoma

Background

Because procarbazine is not available in the mainland of China, a risk-adapted chemotherapy without the drug was adopted for children with Hodgkin lymphoma (HL) in two tertiary referral centers for childhood cancer in Shanghai. The objective of the present study was to obtain the results comparable with those of previous studies.

Methods

From January 1998 to December 2009, patients below 18 years with newly diagnosed, untreated HL were enrolled in the study. The patients were stratified into risk groups R1 (early stage), R2 (intermediate stage) and R3 (advanced stage). All the patients who had attained a complete remission were not given involved field radiotherapy.

Results

Fifty-six patients were eligible for the study. The 4-year event-free survival (EFS) rate was 100%, 80.3%±7.2%, and 62.5%±12.1% for the risk groups R1, R2, and R3, respectively. There was statistically significant difference in EFS between patients with and those without B symptoms (P<0.001). In group R2, the EFS rate was higher for patients treated with chemotherapy combined with radiation (100% vs. 75%±8.8%). But no statistical difference was observed (P=0.177). At the time of evaluation (December 31, 2010), secondary malignancy was not observed.

Conclusions

A significant fraction of children with early stage or intermediate stage HL can be cured with a chemotherapy regimen without procarbazine. Complete response to chemotherapy seems not to be a determinant to omit radiotherapy.     

An infant with self‐healing cutaneous Langerhans cell histiocytosis followed by isolated thymic relapse

Thymic involvement with Langerhans cell histiocytosis (LCH) typically occurs in children as part of multi‐system (M‐S) LCH. Patients who develop skin‐only LCH during infancy may either follow a self‐healing course with spontaneous regression or may progress to M‐S involvement. We describe a male infant who developed isolated thymic LCH after spontaneous complete regression of isolated cutaneous lesions. His erythrocyte sedimentation rate and C‐reactive protein increased temporarily during the skin‐only stage of LCH, and increased again considerably during the thymic relapse. Even for patients with skin‐only LCH, these laboratory data might indicate possible relapse or late progression of the disease. Pediatr Blood Cancer 2009;53:229–231. © 2009 Wiley‐Liss, Inc.