Pathological characteristics and clinical prognosis of nodular sclerosis grade 2 of classic Hodgkin's lymphoma / 中华肿瘤杂志

Objective: To investigate the pathological characteristics and clinical prognosis of nodular sclerosis grade 2 of classic Hodgkin's lymphoma (cHL-NS2) in our cancer center. Methods: A retrospective collection of 23 cases of cHL-NS2 admitted in Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from July 2008 to April 2019 was performed. Fifty-five cases of nodular sclerosis grade 1 of classical Hodgkin's lymphoma (cHL-NS1) during the same period were selected as control group. Survival curves were plotted using the Kaplan-Meier method, and Cox regression model was used to analyze the influencing factors for survival. Results: The median age of 23 cases of cHL-NS2 was 30 years old. Five cases had extra nodal invasion, and 19 cases were Ⅰ-Ⅱ stage based on Ann Arbor system. The pathological morphology of cHL-NS2 showed that the lymph node structure was completely destroyed and was divided into nodules by thick collagen. The tumor cells in the nodules were abundant and proliferated in sheets. The boundaries between the tumor cells were not clear. The incidence of tumor necrosis in cHL-NS2 was 43.5% (10/23), which was significantly higher than 18.2% (10/55) in cHL-NS1 (P=0.040). The 3-year progression-free survival (PFS) rate of patients in the cHL-NS2 group was 58.1%, which was significantly lower than 89.7% in the cHL-NS1 group (P=0.002). In all of 78 cases, the 3-year PFS rate of patients who did not obtain complete response (CR) was 67.1%, which was significantly lower than 92.2% in patients who achieved CR (P=0.030). Multivariate Cox regression analysis demonstrated that both cHL-NS2 and failure to obtain CR by first-line treatment were independent indicators for short PFS time (P<0.05). Conclusions: In cHL-NS2, the morphology of tumor cells are diverse, and tumor necrosis can be easily found. Under the current first-line treatments of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) or bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone (BEACOPP), cHL-NS2 is an independent indicator for worse PFS.

Evaluación del tratamiento de linfoma de Hodgkin con esquema ABVD en Chile / Treatment of Hodgkin lymphoma: analysis of 915 patients

Background: Hodgkin lymphoma has a high rate of curability, even in advanced stages. Aim: To assess the results of Hodgkin lymphoma treatment using the ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) chemotherapy regimen. Material and Methods: Analysis of a database held by the Chilean Ministry of Health, including all patients treated at accredited cancer treatment centers. Results: Data for 915 patients, median age 35 years (range 15-86 years) and followed for a median of 97 months (range 1-347 months) were analyzed. Forty-one percent had localized disease. Overall survival at five years for localized and advanced stages was 92% and 74%, respectively. The figures for progression free survival were 87% and 64%, respectively. Patients with relapse who received autologous stem cell transplantation (ASCT) had a five year overall survival of 92%, compared to 64% among those who did not undergo this procedure (p < 0.01). The Guarantees in Health Program set up by the Ministry of Health, was associated with earlier stage disease at diagnosis. Conclusions: The ABVD regimen achieves high rates of cure in localized stages of the disease but the results in advanced stages are not optimal. ASCT significantly improves survival in patients with relapse. The Guarantees in Health Program is associated with earlier diagnosis of the disease.

Infantile myofibromatosis

Abstract: Infantile myofibromatosis is a mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle and viscera. It is the most common fibrous tumor in childhood. We present a newborn with skin and bone disease without visceral involvement, who showed good response to vinblastine and methotrexate. Clinical features, etiology, diagnosis, and treatment are reviewed.

The efficacy of 2-nitrovinylfuran derivatives against Leishmania in vitro and in vivo

Despite recent advances in the treatment of some forms of leishmaniasis, the available drugs are still far from ideal due to inefficacy, parasite resistance, toxicity and cost. The wide-spectrum antimicrobial activity of 2-nitrovinylfuran compounds has been described, as has their activity against Trichomonas vaginalis and other protozoa. Thus, the aim of this study was to test the antileishmanial activities of six 2-nitrovinylfurans in vitro and in a murine model of leishmaniasis. Minimum parasiticide concentration (MPC) and 50% inhibitory concentration (IC50) values for these compounds against the promastigotes of Leishmania amazonensis, Leishmania infantum and Leishmania braziliensis were determined, as were the efficacies of two selected compounds in an experimental model of cutaneous leishmaniasis (CL) caused by L. amazonensis in BALB/c mice. All of the compounds were active against the promastigotes of the three Leishmania species tested. IC50 and MPC values were in the ranges of 0.8-4.7 µM and 1.7-32 µM, respectively. The compounds 2-bromo-5-(2-bromo-2-nitrovinyl)-furan (furvina) and 2-bromo-5-(2-methyl-2-nitrovinyl)-furan (UC245) also reduced lesion growth in vivo at a magnitude comparable to or higher than that achieved by amphotericin B treatment. The results demonstrate the potential of this class of compounds as antileishmanial agents and support the clinical testing of Dermofural(r) (a furvina-containing antifungal ointment) for the treatment of CL.

Concurrent Langerhans Cell Histiocytosis and B-Lineage Lymphoid Proliferation in the Bone Marrow / 대한진단검사의학회지

We present three cases of concurrent Langerhans cell histiocytosis (LCH) and B-lineage lymphoid cell infiltrations and/or nodules in the bone marrow. The first patient was a 25-month-old boy who presented with LCH on the right shoulder and multiple osteolytic lesions. Bone marrow biopsy showed the presence of LCH and two large lymphoid nodules of B-lineage, which were located in the paratrabecular region. Both LCH and the lymphoid nodules resolved after treatment with prednisone, vinblastine, methotrexate, and cyclophosphamide. The second patient was a 7-month-old girl who presented with LCH in the scalp and bone marrow. In spite of the treatment, a follow-up bone marrow analysis performed after 16 months showed LCH and increased B-lineage lymphoid cells in the interstitial area. The third patient was a 26-month-old girl, and imaging studies revealed reddish skin lesions and multiple osteolytic lesions. Skin biopsy and bone marrow biopsy did not show the presence of LCH; however, we initiated the treatment on the basis of the results of imaging studies. The follow-up study after 6 months showed the presence of LCH and large, patchy infiltration of B-lymphoid cells. We report three rare cases of concurrent bone marrow involvement of LCH and B-lineage lymphoid proliferation, which strongly suggest lymphoid malignancy. Further, clonal changes should be studied to elucidate the common pathogenic mechanism between the two diseases.

Diffuse Neonatal Hemangiomatosis: report of 5 cases.

Diffuse Neonatal Hemangiomatosis (DNH) is a rare, life-threatening condition associated with a few to hundreds of small, cutaneous and visceral hemangiomas. The authors reported 5 cases of DNH in which hepatic hemangioma were the most common visceral involvement. Response to prednisolone in these cases was not good, one died and four required second line therapy. Of these four cases, one case with embolisation; one with interferon and two with vinblastine. Response to vinblastine was good, but long-term follow-up of the side effects are needed.

Primitive neuroectodermal tumor (PNET) as second malignancy after treatment of Hodgkin's disease.

Hodgkin's disease survivors are at an increased risk of developing second malignant neoplasms including secondary bone tumors. Common secondary bone tumors are osteogenic sarcoma and fibrosarcoma. Secondary primitive neuroectodermal tumor is extremely rare in this group. We present below, a rare case of secondary PNET in an 8-year-old child with Hodgkin's disease which developed unusually early outside the radiation portal and discuss potential factors responsible for its causation.

Etoposide, vinblastine, adriamycin and prednisolone (EVAP) combination chemotherapy as first-line treatment for Hodgkin's disease.

BACKGROUND: Mechlorethamine, vincristine, procarbazine, prednisolone (MOPP) and doxorubicin, bleomycin, vinblastine, dacarbazine (ABVD) are well established first-line chemotherapy protocols for the treatment of Hodgkin's disease. The aim of this study was to try a new combination of drugs that individually have a proven efficacy in Hodgkin's disease but have less incidence of severe nausea, vomiting, pulmonary toxicity and sterility. METHODS: This prospective, single-arm study enrolled 66 newly diagnosed, previously untreated patients of Hodgkin's disease with stages IA (bulky)-IVB disease. They were given 6-8 courses of etoposide, vinblastine, doxorubicin and prednisolone (EVAP) as first-line chemotherapy between January 1992 and December 1997. Radiotherapy (RT) was given to the involved fields of those patients who had bulky (> or = 10 cm) stages I or II disease at presentation. The end-points were (i) complete and overall response; (ii) disease-free and overall survival; and (iii) toxicity. RESULTS: Complete response was seen in 78.8% and partial response in 12.2% of patients; the overall response rate was 91%. The median follow up was 48 months. The 5-year overall and disease-free survivals were 72% and 62%, respectively. There were 3 episodes of grade IV neutropenia requiring hospitalization. One patient developed avascular necrosis of the femur. There were 2 deaths during treatment, one due to chemotoxicity, and another due to progressive disease. CONCLUSION: The overall and complete responses were fractionally inferior to the recently published hybrid MOPP/ ABV combination and that of ABVD chemotherapy. The advantages of the EVAP combination are absence of pulmonary toxicity, markedly lower incidence of sterility and nausea and vomiting. EVAP is an attractive option and a randomized trial is warranted to assess its efficacy against established protocols.

An unusual presentation of an intra-parenchymatous frontal yolk sac tumour : case report.

Yolk sac tumours are rare conditions among the germ cell tumours. Intracerebral germ cell tumours are exceedingly rare. A 15 year old girl presenting with a one week history of raised intracranial pressure is described. She had bilateral papilloedema and a right 6th nerve palsy. CT scan showed an intra-parenchymatous right frontal ring enhancing lesion of 2 cms diameter. The patient underwent microsurgical total excision of the tumour, followed by chemotherapy. She was asymptomatic at three years following surgery.

Comparative study of bulky stage IB and IA cervical cancer patients treated by radical hysterectomy with and without neoadjuvant chemotherapy: long-term follow-up.

One hundred and ninety patients with bulky (> 3 cm) stage IB and IIA cervical cancer who underwent radical hysterectomy between 1991 and 1994 at Maharaj Nakorn Chiang Mai Hospital were reviewed to determine whether neoadjuvant chemotherapy (NAC) with MVAC (Methotrexate, Vinblastine, Adriamycin, Cisplatin) improved survival. There were 42 patients treated with pre-operataive NAC (MVAC 1-3 courses) and 148 patients treated by primary surgery (PS). In the NAC group, the overall response rate from MVAC was 88.1 per cent with 31.0 per cent having complete clinical response and 7.1 per cent with complete pathological response. Pelvic lymph node metastasis was not significantly different between the NAC group (16.7%) and the PS group (18.2%). At a median follow-up of 64.5 months, 19.0 per cent in the NAC group and 18.2 per cent in the PS group had tumor recurrence. The 5-year progression free and overall survival was 80.8 per cent and 92.0 per cent respectively for the NAC group which was not significantly different from 80.2 per cent and 92.9 per cent respectively in the PS group. In conclusion, although NAC can decrease the tumor size and produce a high response rate, it does not improve survival in bulky stage IB and IIA cervical cancer patients.

Funçäo gonadal de sobreviventes de doença de hodgkin tratados na infância e adolescência com quimioterapia / Gonadal function of survivor of Hodgkin's disease treated in infancy and adolescence with chemotherapy

Avaliamos a funçäo gonadal de 21 pacientes do sexo masculino com doença de Hodgkin (grupo A), que receberam quimioterapia durante a infância e adolescência, e comparamos com 20 indivíduos adultos jovens sadios (grupo B). A mediana da idade dos pacientes no momento do estudo foi de 18 anos (17-23 anos), e do início da quimioterapia 10 anos (6-19 anos). Na época do tratamento , 14 pacientes eram impúberes e 7 já estavam na puberdade. No momento da investigaçäo todos se encontravam no estágio puberal V de Tanner e tinham completado quimioterapia entre 3 e 11 anos previamente. A mediana do volume testicular foi menor no grupo A do que no B, p = 0,001. Näo houve diferenças significativas da TT, SHBG, PRL e LH entre os grupos. A mediana dos níveis basais do FSH do grupo A foi maior do que no B, p = 0,001. Houve significativa diferença entre as medianas do pico máximo do FSH e do LH após estímulo com GnRH entre os grupos, p = 0,002 e o p = 0,0002 respectivamente. encontramos uma correlaçäo positiva entre a idade do paciente na época do tratamento e o valor máximo do LH ao estímulo com GnRH (r + 0,4; p = 0,03) e uma correlaçäo negativa com o tempo decorrido entre o término do tratamento e o estudo (r = -0,5; p = 0,008). Onze pacientes apresentavam azoospermia, 4 oligospermia e 3 pacientes apresentavam espermograma normal. Um paciente recuperou a fertilidade, com normalizaçäo do espermograma, 11 anos após o término do tratamento. Concluímos que pacientes tratados na infância e adolescência com quimioterapia apresentam importante dano no epitélio germinativo, mantendo níveis normais de testosterona às custas do aumento da secreçäo de LH. A presença de reduçäo do volume testicular nestes pacientes é sugestiva de dano no epitélio germinativo, sendo necessário um longo período de acompanhamento para avaliar possível recuperaçäo da funçäo gonadal.

Langerhans cell histiocytosis in children less than 2 years of age.

OBJECTIVE: To study the clinical profile and outcome of langerhans cell histiocytosis in children upto 2 years of life. DESIGN: Retrospective analysis. METHODS: Medical records of Children upto 2 years of age with a diagnosis of langerhans cell histiocytosis (LCH) were analyzed. Their clinical pattern, treatment modalities and outcome were studied. The patients Were categorized into 2 groups according to their clinical presentation: (i) Subject without organ dysfunction; and (ii) cases with organ dysfunction. Treatment considered of surgical intervention, radiotherapy, chemotherapy or combination of all these modalities depending upto the extent of disease. RESULTS:There were 20 children upto 2 years of age with histiocytosis during the 12 year period (January 1983 - December 1994). The median age at diagnosis was 18 months (range 52 days - 24 months). Of the twenty patients,13 patients didn't have organ dysfunction and 7 had organ dysfunction. Out of the 13 children without organ dysfunction eleven patients received treatment and all of them are alive free of disease with a median follow up of 62 months. But all children with organ dysfunction succumbed to disease within a few weeks. CONCLUSION: Children under 2 years of age with localised and or multifocal LCH without organ dysfunction have a good prognosis and they should not be exposed to aggressive form of treatment. All children with organ dysfunction require multi-agent chemotherapy.

Sindrome hemofagocitica secundaria / Secondary hemophagocytic syndrome

Embora consideradas doencas raras, as sindromes hemofagociticas representam verdadeiros desafios para o diagnostico, bem como para o tratamento. Os autores relatam o caso de uma crianca de 7 anos de idade, que desenvolveu a doenca apos uma meningite por Haemophilus influenzae e discutem os aspectos fisiopatologicos, clinicos e terapeuticos das sindromes hemofagociticas

Recent advances in cancer therapy

The treatment of cancer has developed substantially from its conception in the first years of the 20th century. Since the introduction of alkylating agents during second World War, the oncology specialty has markedly grown. In the recent years, new drugs have been approved for the treatment of cancer. Such examples include the taxanes (Docetaxel and Paclitaxel), Vinorelbine, Irinotecan, Topotecan, Gemcitabine and Gliadel. We will discuss these new chemotherapuetic agents, their pharmacology, indications, toxicity and appropriate dosing. There is no doubt that further clinical research is needed to determine the optimal use of these agents

Estudo do DNA na previsäo de resposta à quimioterapia por M-VAC em câncer infiltrativo de bexiga / DNA ploidy as a possible predictor of response to M-VAC chemotherapy for locally invasive bladder cancer

Em 20 pacientes com CCTB localmente infiltrativo realizou-se pesquisa do conteúdo do DNA em amostras de tumores obtidos por ressecçäo ou biópsia endoscópica, para avaliar a possibilidade de previsäo de resposta àquimioterapia por M-VAC. Histologicamente, 30 por cento dos tumores eram diferenciados e 70 por cento pouco diferenciados. Os tumores eram diplóides em 5 por cento, tetraplóides em 5 por cento e aneuplóide em 90 por cento. Observou-se resposta completa à quimioterapia em 55 por cento e resposta negativa em 45 por cento dos casos. A avaliaçäo da haploidia do DNA näo contribuiu para a detecçäo de neoplasias responsivas ao esquema M-VAC

Post orchiectomy management in stage II testicular seminoma.

Twenty eight patients with stage II A and twenty patients with stage II B testicular seminoma were treated at this institute between January 1982 and December 1988. The three year crude survival observed in this retrospective analysis was 82% and 75% respectively. Post orchiectomy infradiaphragmatic radiotherapy was the mainstay of the treatment. In stage II A 4 patients were administered adjuvant chemotherapy as well. Prophylactic Mediastinal Irradiation (PMI) was not employed as a routine in this subgroup. Eight patients (28%) relapsed (Mediastinal Nodes--4, Pulmonary--3, Scrotal--1). In stage II B twelve patients were treated with primary abdominal radiotherapy and of them 4 were delivered PMI as well. Induction chemotherapy was administered in remaining 8 patients. Seven patients (35%) relapsed (Pulmonary-4, Mediastinal Nodes-3). Mediastinal recurrence was noted only in those who were treated with abdominal radiotherapy alone. Though salvage chemotherapy proved successful in 5 of the seven patients (70%) with nodal relapse, none of the patients with extranodal relapse responded to subsequent chemotherapy. For stage II A we recommend abdominal radiotherapy alone and for stage II B Induction chemotherapy is advised keeping radiotherapy reserved for residual mass. We do not advocate PMI as a routine in stage II testicular seminoma as no survival benefit is observed.

Sarcoma de Kaposi: a propósito de una revisión clinicopatológica / Kaposi's sarcoma: a propose of a clinic-pathological review

Desde la aparición de la enfermedad del Sida, se ha profundizado el estudio sobre sarcoma de kaposi (SK) debido a la asociación de esta neoplasia con la infección por el virus VIH. Sin embargo, debe recordarse que existen también otras formas de sarcoma de kaposi no asociadas al Sida. Ultimamente se ha investigado la patogenia del SK y se han postulado varias teorías que explicarían el origen de esta enfermedad. En el presente trabajo, se analizan las características clínicas, histológicas, junto a la etiología de esta neoplasia, así como su tratamiento; además, se incluye el análisis del diagnóstico histológico realizado de los casos biopsiados en nuestro Departamento desde 1979 a 1994