Opportunistic Invasive Fungal Infections Mimicking Progression of Non–Small-Cell Lung Cancer

BackgroundMany studies have shown that invasive pulmonary aspergillosis, cryptococcosis, and mucormycosis can mimic radiographic and clinical features of primary lung cancer. However, more research surveying the incidence and outcomes of these fungal infections among patients with a history of lung cancer is needed. The aim of this study was to describe the occurrence and clinical outcomes of opportunistic invasive fungal infections that can mimic tumors in non–small-cell lung cancer patients.Patients and MethodsPatients seen at Stanford University Medical Center from January 1, 2007, to May 1, 2020, with pulmonary aspergillosis, cryptococcosis, or mucormycosis after non–small-cell lung cancer (NSCLC) diagnosis were reviewed. The European Organization for Research and Treatment of Cancer National Institute of Allergy and Infectious Diseases Mycoses Study Group criteria was used to classify patients with evidence of proven or probable invasive fungal infection within our cohort.ResultsA total of 12 patients with proven or probable invasive mold infection (including 8 cases of aspergillosis) and 1 patient with proven cryptococcosis were identified, without any cases of mucormycosis. Of this cohort, 6 patients (46%) showed radiographic findings that were found to be most consistent with lung cancer by radiologists. Eight cases (62%) were suspected of cancer recurrence or progression by the treatment team on the basis of additional considerations of medical history and clinical symptoms. Most patients had active NSCLC or had a history of recurrence without active NSCLC at the time of fungal discovery (11 patients; 85%). Most patients died without full recovery (7 patients; 54%).ConclusionsInvasive pulmonary aspergillosis and cryptococcosis can often be mistaken as cancer recurrence or progression in patients with a history of NSCLC because of mimicking radiographic and clinical characteristics.     

Immune reconstitution inflammatory syndrome in cancer patients with pulmonary aspergillosis recovering from neutropenia: Proof of principle, description, and clinical and research implications

BACKGROUND: Assessing the outcome of patients with invasive pulmonary aspergillosis by using conventional criteria is difficult, particularly when clinical and radiologic worsening coincides with neutrophil recovery. Usually, it is assumed that this deterioration is related to progressive aspergillosis, prompting changes in patient management. However, its temporal relation with neutrophil recovery suggests that it may be caused by an immune reconstitution syndrome (IRIS). Galactomannan is an Aspergillus-specific polysaccharide that is released during aspergillosis and is detected by the serum galactomannan test, which has been approved by the United States Food and Drug Administration for the diagnosis of invasive aspergillosis. In this study, the authors used sequential galactomannan testing to distinguish IRIS responses from progressive aspergillosis. METHODS: From April 2001 to December 2006, patients with hematologic malignancies underwent galactomannan screening during periods when they were at risk. The clinical and laboratory findings from patients who had >or=2 consecutive positive galactomannan assays (optical density, >or=0.5) were reviewed. RESULTS: Nineteen neutropenic patients with aspergillosis developed clinical and radiologic pulmonary deterioration during neutrophil recovery. Deterioration coincided with microbiologic response, as documented by rapid normalization of serum galactomannan, and, in 16 patients, was followed by complete clinical response and survival at 3 months, although there were no changes in antifungal therapy. The 3 patients who died during the first month had no evidence of aspergillosis at autopsy examination. CONCLUSIONS: The authors propose that IRIS was responsible for the current findings and provide a definition for the syndrome. They also recommend serial galactomannan testing to guide aspergillosis management. Declining galactomannan values imply IRIS with an aspergillus response and obviate the need for invasive procedures and alternative antifungal therapies, whereas persistent galactomannan elevation indicates progressive aspergillosis and requires prompt treatment modification.     

Fatal haemoptysis associated with invasive pulmonary aspergillosis treated with high-dose amphotericin B and granulocyte-macrophage colony-stimulating factor (GM-CSF)

Opportunistic pulmonary infections are a leading cause of morbidity and mortality in patients with chemotherapeutically treated neoplasias. With increasingly aggressive cytotoxic regimens causing prolonged neutropenia, the risk of systemic mycoses and in particular of invasive pulmonary aspergillosis has increased. We review the case of a 10-year-old child suffering from relapsed lymphoblastic leukaemia and from high-dose amphotericin B-treated invasive pulmonary aspergillosis acquired during long-standing neutropenia in the initial phase of remission induction chemotherapy. The patient died in remission after GM-CSF-induced bone marrow recovery and clinical and radiological improvement with stable plasmatic coagulation and normal thrombocyte count. Peracute massive pulmonary bleeding caused by the simultaneous arrosion of a greater pulmonary artery and a lobar bronchus by a liquefactive fungal focus was responsible. In patients with chemotherapeutically induced neutropenia and invasive aspergillosis, bone marrow recovery may lead to the liquefaction of pulmonary foci, and, in view of the well-known vasotropic nature of the infection, to a potentially lethal arrosion bleeding. With the emerging use of colony-stimulating factors for shortening and overcoming neutropenia, this so far rare complication may become of increasing importance.     

Invasive Aspergillosis in Neutropenic Patients with Hematological Disorders

Between 1983-1988, 72 patients with acute leukemia and 4 with aplastic anemia were treated in the Hematology Unit of The Chaim Sheba Medical Center. Ten patients with acute leukemia developed invasive pulmonary aspergillosis and 2 with aplastic anemia developed invasive aspergillosis of the nose and paranasal sinuses. These infections were diagnosed during a period of profound neutropenia while these patients were receiving broad spectrum antibiotics. The diagnosis of pulmonary aspergillosis was based on positive sputum cultures in 4 cases and on the appearance of typical clinical and radiologic features in six. In 2 culture-positive and in one culture-negative patient, the diagnosis was confirmed at autopsy. Thus, the diagnosis was definitive in 5 patients and probable in the remaining five patients. The 5 patients who achieved remission responded to antifungal treatment and recovered, while of the 5 who eventually died from the fungal infection, 4 did not achieve remission, and one died while in complete remission. In the 2 patients with aplastic anemia, aspergillosis was detected in cultures from necrotic nasal tissue. Both patients remained neutropenic, failed to respond to antifungal treatment and died within a short time after diagnosis. From this experience it appears that invasive aspergillosis in neutropenic patients is potentially curable if treated early by amphotericin B, provided that the neutrophil count recovers.     

Invasive pulmonary aspergillosis: A rare complication after microwave ablation

Three cases are reported of invasive pulmonary aspergillosis (IPA) occurring after microwave ablation (MWA) for lung tumours. This is a rare complication that has not previously been described in the literature. The diagnosis of IPA was based on the following factors: host factors, clinical manifestations and mycological findings. The first case was a 63-year-old man treated for primary lung squamous carcinoma. Significant tumour regression was achieved by 18 days after MWA, medical treatment with itraconazole for 6 weeks, and postural drainage. The second case, a 65-year-old man, was confirmed with primary lung squamous cell carcinoma. Voriconazole administration using intravenous infusion combined with intracavitary lavage was therapeutically effective after MWA at 1 year follow-up. The third case was a 61-year-old woman with primary lung adenocarcinoma. Delayed pneumothorax and bronchopleural fistula secondary to IPA persisted. The patient died from secondary multiple organ function failure. Despite its very low incidence, the significance of early diagnosis and early administration of antifungal therapy should be highlighted because of the relentless severity of IPA in patients undergoing MWA.     

儿童白血病化疗后合并播散性曲霉菌病三例并文献复习

目的 探讨儿童白血病化疗后合并播散性曲霉菌病的临床特征、诊断、治疗及预后.方法 回顾性分析3例白血病化疗后合并播散性曲霉菌病患儿的临床特征及诊治过程.结果3例患儿的基础疾病均为白血病,存在多个侵袭性真菌病的高危因素,临床特征包括:反复发热而抗生素治疗无效,皮下硬性结节,肺部CT提示结节影、空洞,磁共振成像提示肝、脾、肾散在低密度灶(T2加权),血清半乳甘露聚糖(GM)试验阳性,均通过病理组织学检查确诊.确诊后,2例予两性霉素B脂质体抗真菌治疗超过4周,并在临床症状好转、中性粒细胞恢复及影像学提示病灶好转或无进展情况下恢复化疗,同时予口服伏立康唑维持治疗,随访6~12个月,播散性曲霉菌病达治愈标准.1例白血病持续完全缓解,截至随访结束仍生存;1例因家属要求暂停化疗,白血病复发死亡;1例因治疗无效或严重药物不良反应,多次调整抗真菌治疗方案后感染仍无法控制,且因长时间暂停化疗,白血病复发,最终死亡.结论 白血病患儿化疗后反复发热,并有多发皮下硬性结节、肺部结节或空洞、肝脾肾散发性低密度灶、GM试验阳性,应考虑合并播散性曲霉菌病.有效且足疗程的抗真菌治疗,并在适当时机兼顾化疗,有望改善患者预后.     

Fatal airway obstruction caused by invasive aspergillosis of the thyroid gland

Invasive aspergillosis is a common form of fungal infection in patients with hematological malignancies. Because Aspergillus species have angioinvasive properties, they frequently disseminate from the lung to a variety of organs via hematogenous spread. Extra-pulmonary involvement occurs at an advanced stage of invasive aspergillosis, and represents an ominous sign. However, few reports have been published on extra-pulmonary involvement in cases of aspergillosis. Its clinical features have not been fully clarified. We experienced a patient who developed thyrotoxicosis and fatal airway obstruction caused by invasive aspergillosis of the thyroid. A 26-year-old man was admitted to our hospital for the treatment of non-Hodgkin's lymphoma. During myelosuppression following the chemotherapy, he developed cervical swelling and hyperthyroidism. We suspected lymphoma infiltration to the thyroid, and irradiated it with a total of 26 Gy. However, the cervical lesion enlarged rapidly, and he complained of wheezing and dyspnea. We underwent immediate tracheostomy to secure the airway, but he died. Autopsy findings were striking. Extensive necrosis with diffuse infiltration of Aspergillus hyphae was observed in the thyroid gland. Necrotic tissues of the thyroid protruded into the tracheal lumen, causing airway obstruction. This case demonstrated that invasive aspergillosis of the thyroid can lead to medical emergency.     

Oesophageal aspergillosis in a case of acute lymphoblastic leukaemia successfully treated with caspofungin alone due to liposomal amphotericin B induced severe hepatotoxicity

Aspergillosis is one of the most common invasive fungal infections in immunocompromised patients. Sinonasal region and upper respiratory tract are commonly involved regions whereas oesophagus is seldom involved. We present an 18-year-old male with acute lymphoblastic leukaemia with aspergillosis of oesophagus which is a rare region of involvement. The diagnosis was confirmed by the examination of the cultures of endoscopic biopsy material. The patient was already receiving empirical liposomal amphotericin B, due to severe hepatotoxicity the therapy was switched to another antifungal (caspofungin). Here we report a case of successful treatment of invasive oesophageal aspergillosis by caspofungin.     

Current issues in the clinical management of invasive aspergillosis--the AGIHO, DMykG, ÖGMM and PEG web-based survey and expert consensus conference 2009

The objectives of this study were to identify unsolved issues in the management of invasive aspergillosis, identify controversies and achieve consensus. The German Speaking Mycological Society (Deutschsprachige Mykologische Gesellschaft, DMykG) invited other German infectious diseases (ID) and mycological societies to submit unsolved issues concerning the diagnosis and treatment of invasive aspergillosis. Based on these contributions, a digital web-based questionnaire of 12 questions on Aspergillus spp. was designed to be completed by experts of the participating societies. Controversial results were identified by a mathematical model and were discussed at a consensus conference during the 43rd Annual Meeting of the DMykG in Cologne, Germany. Forty-two individuals completed the questionnaire. Analysis showed a strong consensus on effective preventive measures, choice of antifungal agents for pre-emptive, empiric and targeted treatment, as well as the evaluation of early chest CT control scans as a measure of treatment response assessment. Opinions on the indication for a pulmonary biopsy of a halo sign in high-risk neutropenic patients and on the role of Aspergillus spp. PCR as well as galactomannan from serum in the assessment of treatment duration diverged in spite of discussion such that a consensus could not be reached. Using a recently published two-step approach - web-based survey plus classical panel discussion - expert consensus was achieved on 10 of 12 questions concerning the diagnosis and treatment of invasive aspergillosis.     

Real‐world challenges and unmet needs in the diagnosis and treatment of suspected invasive pulmonary aspergillosis in patients with haematological diseases: An illustrative case study

Recent years have seen important advances in the diagnosis of invasive pulmonary aspergillosis (IPA), complemented by the introduction of new therapies. Despite this, IPA remains a major cause of infection‐related mortality in patients with haematological diseases. There are two main reasons for this. First, diagnosis of IPA remains a challenge, since risk factors and the clinical, radiological and mycological presentations vary not only by fungal disease stage, but also by patient group (eg neutropenic vs non‐neutropenic patients). Diagnosis is particularly challenging in patients receiving mould‐active prophylactic or empirical treatment, which reduces the sensitivity of all diagnostic tests for IPA. Second, treatment of IPA is complex due to unpredictable pharmacokinetic profiles of antifungal agents, small therapeutic window in terms of exposure and adverse events, and multiple drug‐drug interactions through the CYP450 system. Here we report a case of a 23‐year‐old male with severe aplastic anaemia and subpleural nodules. Diagnostic tests for IPA obtained during ongoing mould‐active empirical treatment were negative. Intravenous voriconazole was stopped after visual disturbances and hallucinations. The patient then had an anaphylactic reaction to liposomal amphotericin B and was switched to intravenous posaconazole, which had to be discontinued due to a significant increase in transaminase levels. He was treated with oral isavuconazole with reduced dosage, triggered by increasing transaminases under the standard dosage. Even under reduced dosage, blood concentrations of isavuconazole were high and treatment was successful. The case illustrates real‐world challenges and unmet needs in the diagnosis and treatment of IPA in patients with haematological diseases.     

Invasive aspergillosis: epidemiology and environmental study in haematology patients (Sfax,Tunisia)

Invasive aspergillosis (IA) is a major opportunistic infection in haematology patients. Preventive measures are important to control IA because diagnosis is difficult and the outcome of treatment is poor. We prospectively examined the environmental contamination by Aspergillus and other fungal species and evaluated the prevalence of invasive aspergillosis in the protect unit of haematology. A three‐year prospective study (December 2004–September 2007) was carried out in the department of haematology of Hedi Chaker Hospital. Suspected invasive aspergillosis cases were reviewed and classified as proven, probable and possible invasive aspergillosis using the EORTC criteria. During the study period, we collected weekly environmental samples (patient’s rooms, tables and acclimatisers) and clinical samples from each patient (nasal, expectoration and auricular). Among 105 neutropenic patients, 16 had probable and 13 had possible IA. A total of 1680 clinical samples were collected and A. flavus was most frequently isolated (79.2%). Analysis of 690 environmental samples revealed that Penicillium (44%) was the most frequent followed by Cladosporium (20%), Aspergillus spp. (18%) and Alternaria (13%). The PCR‐sequencing of 30 A. flavus isolates detected from clinical and environmental samples confirmed the mycological identification. Our findings underline the importance of environmental surveillance and strict application of preventive measures.     

Case reports. Secondary prophylaxis with liposomal amphotericin B after invasive aspergillosis following treatment for haematological malignancy

We report our recent experience with two cases of invasive pulmonary aspergillosis in patients who were both undergoing chemotherapy, one for acute myeloid leukaemia and the other for primary amyloidosis. Both patients had bad prognostic factors and were in very poor clinical condition, but both recovered from infection after a prolonged therapy with liposomal amphotericin B (AmBisome) without signs of toxicity.     

食管癌术后重返ICU患者的病因及临床预后分析

目的:探讨食管癌患者术后再次返回ICU的原因及预后.方法:回顾性分析65例食管癌术后患者重返ICU的病因及死亡原因.结果:65例重返ICU的患者,死亡13例,其中8例为重症肺部感染、呼衰,3例为吻合口瘘,脑梗、肺栓塞各1例.结论:肺部感染、吻合口瘘、肺动脉栓塞、脑梗塞是食管癌术后患者重返ICU的常见原因.以肺部感染发生率、病死率最高,术后加强呼吸道管理、预防肺部感染尤为重要.     

The safety of interferon-gamma-1b therapy for invasive fungal infections after hematopoietic stem cell transplantation

BACKGROUND: The restoration of normal immune responses, especially of the T-helper type 1 immune response, is an important predictor of fungal infection outcome in patients with malignant disease who undergo hematopoietic stem cell transplantation (HSCT). The authors sought to evaluate the safety of adjuvant recombinant interferon-gamma-1b as an immune-modulatory therapy HSCT recipients. METHODS: Thirty-two patients received interferon-gamma-1b after undergoing HSCT at the author's institution between 1998 and 2003. A retrospective analysis was undertaken after obtaining permission from the Institutional Review Board. RESULTS: Twenty-six of 32 patients (81%) received allogeneic stem cell grafts. All but 1 patient received interferon-gamma-1b and antifungals to treat infections; the other patients received interferon-gamma-1b to promote autologous graft-versus-tumor effect. Interferon-gamma-1b usually was administered at a dose of 50 mug subcutaneously every other day. The median duration (+/- standard deviation) of interferon-gamma-1b therapy was 6+/-6.5 doses (range, 1-29 doses), and the median cumulative dose was 487+/-453 mug (range, 35-2175 microg). During therapy with interferon-gamma-1b, fever was common (n=9 patients; 28%). In 1 patient (3%), new-onset lymphocytopenia occurred but resolved after cytokine therapy was discontinued; there were no interferon- gamma-1b-related episodes of neutropenia, thrombocytopenia, anemia, or liver dysfunction. Interferon-gamma-1b therapy did not precipitate or exacerbate acute or chronic graft-versus-host disease (GVHD). In fact, in 2 of 7 patients (29%) with acute GVHD and in 3 of 10 patients (30%) with chronic GVHD, significant improvements in GVHD were noted during therapy with interferon-gamma-1b. Among the 26 patients with aspergillosis, 14 patients (54%) died. However, 5 of 10 patients (50%) with presumed pulmonary aspergillosis, 3 of 9 patients (33%) with probable pulmonary aspergillosis, 1 of 2 patients (50%) with definite pulmonary aspergillosis, and 3 of 5 patients (60%) with disseminated aspergillosis responded to antifungals and adjuvant interferon-gamma-1b. CONCLUSIONS: Recombinant interferon-gamma-1b was tolerated without serious adverse reactions in HSCT recipients. A large, prospective, randomized study will be needed to evaluate the efficacy of this cytokine in high-risk HSCT recipients who have invasive mycoses.     

Chronic pulmonary aspergillosis

Chronic pulmonary aspergillosis (CPA) is a group of consuming diseases usually presenting with prolonged and relapsing cough, dyspnoea and weight loss. Acute symptoms such as haemoptysis and bronchial or pulmonary haemorrhage may occasionally occur. CPA affects patients with underlying pulmonary conditions, for example, chronic obstructive pulmonary disease or mycobacteriosis or common immunosuppressive conditions such as diabetes. Precise epidemiology is unknown, and while prevalence is considered low the chronic and relapsing nature of the disease challenges the treating physician. Diagnostics largely rely on serologic Aspergillus precipitins and findings on thoracic computed tomography. The latter are manifold comprising cavity formation, pleural involvement and sometimes aspergilloma. Other markers for aspergillosis are less helpful, in part due to the non‐ or semi‐invasive nature of these forms of Aspergillus infection. Various antifungals were shown to be effective in CPA treatment. Azoles are the most frequently applied antifungals in the outpatient setting, but are now compromised by findings of Aspergillus resistance. Long‐term prognosis is not fully elucidated and may be driven by the underlying morbidities. Prospective registry‐type studies may be suitable to systematically broaden our CPA knowledge base. This article gives an overview of the available literature and proposes a clinical working algorithm for CPA management.     

Diagnostic work up to assess early response indicators in invasive pulmonary aspergillosis in adult patients with haematologic malignancies

In immunocompromised patients with acute leukaemia as well as in allogeneic hematopoietic stem cell transplant patients, pulmonary lesions are commonly seen. Existing guidelines provide useful algorithms for diagnostic procedures and treatment options, but they do not give recommendations on how to evaluate early success or failure and if or when it is best to change therapy. Here, we review the diagnostic techniques currently used in association with clinical findings and propose an approach using a combination of computer tomography, clinical and all available biomarkers and inflammation parameters, especially those positive at baseline, to assess early response in invasive pulmonary aspergillosis. Computed tomography scans should be carried out at regular intervals during early and long‐term follow‐up. Imaging on day seven, or even earlier in clinically unstable patients, combined with an additional testing of biomarkers and inflammatory markers in between, is needed for a reliable assessment at day 14. If no improvement is seen after 2 weeks of therapy or the clinical condition is deteriorating, a change of antifungal therapy should be considered. Alleged breakthrough infections or treatment failure should undergo early diagnostic workup, including tissue biopsies when possible, to retrieve fungal cultures for resistance testing.     

Five-year-survey of invasive aspergillosis in a paediatric cancer centre. Epidemiology, management and long-term survival.

The epidemiology, management, and long-term survival of invasive aspergillosis was assessed in a prospective, 5-year observational study in 346 unselected paediatric cancer patients receiving dose-intensive chemotherapy for newly diagnosed or recurrent malignancies. Invasive aspergillosis occurred exclusively in the context of haematological malignancies, where it accounted for an incidence of 6.8% (n = 13 of 189). The lung was the primary site in 12 cases, and dissemination was present in three of those. Prior to diagnosis, the overwhelming majority of patients had been profoundly neutropenic for at least 14 days (n = 11 of 13) and were receiving systemic antifungal agents (n = 10 of 13). Clinical signs and symptoms were nonspecific but always included fever. All 11 patients who were diagnosed and treated during lifetime for a minimum of 10 days responded to either medical or combined medical and surgical treatment, and seven were cured (64%). Nevertheless, the overall long-term survival was merely 31% after a median follow-up of 5.68 years after diagnosis. Apart from refractory or recurrent cancer, the main obstacles to successful outcome were failure to diagnose IA during lifetime and bleeding complications in patients with established diagnosis. The frequency of invasive aspergillosis of greater than 15% in paediatric patients with acute myeloblastic leukaemia and recurrent leukaemias warrants the systematic investigation of preventive strategies in these highly vulnerable subgroups.     

Invasive fungal infections in AML/MDS patients treated with azacitidine: a risk worth considering antifungal prophylaxis?

The aim of this study is to analyse the risk of invasive fungal infection (IFI) and the need for antifungal prophylaxis in patients with acute myeloid leukaemia and myelodysplastic syndromes (AML/MDS) treated with azacitidine. We retrospectively analysed the incidence of IFI according to EORTC‐MSG criteria in 121 consecutive AML/MDS patients receiving 948 azacitidine courses (median 5, range 1–43) between June 2007 and June 2015. Four cases of IFI (two possible, one probable aspergillosis and one proven candidemia) occurred in this series. The incidence rate of proven/probable IFI was 0.21% per treatment cycle and 1.6% per patient treated for the whole series, and 0.73% per treatment cycle and 4.1% per patient treated in those with severe neutropenia. Two patients died from IFI, leading to an IFI‐attributable mortality rate of 1.65% per patient and 0.21% per treatment cycle. The numbers needed to treat with prophylaxis to prevent one case of IFI are 238 azacitidine cycles or 30 patients throughout their whole treatment course, and 137 azacitidine cycles or 24 patients among those with severe neutropenia. AML/MDS patients treated with azacitidine, including those with severe prolonged neutropenia, have a very low risk of IFI which does not justify the use of antifungal prophylaxis.     

儿童肺部原发性肿瘤56例临床分析

目的探讨儿童肺部原发性肿瘤的临床特征、治疗方案及预后。方法 2009—2019年间广州市妇女儿童医疗中心共收治儿童肺部原发性肿瘤患儿56例, 收集其一般资料、影像学资料、病理资料及手术记录等, 进行回顾性分析。结果 56例患儿中, 胸膜肺母细胞瘤28例, 炎性肌纤维母细胞瘤20例, 黏液表皮样癌6例, 血管瘤1例, 硬化性肺细胞瘤1例。临床表现以呼吸道症状为主, 咳嗽26例, 气促17例, 咳血3例。另外, 发热15例, 胸痛3例, 上腹部疼痛2例。肿瘤位于左肺下叶18例, 右肺下叶11例, 左肺上叶10例, 右肺上叶7例, 右肺中叶6例, 肺门4例。56例患儿均行手术治疗, 其中行开胸手术41例, 胸腔镜手术13例, 纤维支气管镜下手术2例。5例Ⅰ型胸膜肺母细胞瘤患儿术后未行化疗, 至随访结束仍存活。5例Ⅱ型胸膜肺母细胞瘤患儿中, 2例术后未化疗, 复发后死亡;3例术后化疗, 至随访结束仍存活。18例Ⅲ型胸膜肺母细胞瘤患儿术后均予IVADo方案化疗, 出现肿瘤复发6例, 远处转移3例, 肿瘤相关死亡8例。20例炎性肌纤维母细胞瘤患儿中, 行楔形切除术13例, 复发5例;行肺叶切除术6例, 复发1例;行纤维支气管镜手术1例, 术后复发。6例黏液表皮样癌患儿中, 行肺叶切除术5例, 行肺楔形切除术1例, 至随访结束均存活。1例血管瘤患儿行支气管镜下肿物切除术, 1例硬化性肺细胞瘤患儿行肺楔形切除术, 至随访结束均存活。结论儿童肺部原发性肿瘤缺乏特异性临床表现, 手术完整切除有利于取得良好预后。对于Ⅱ、Ⅲ型胸膜肺母细胞瘤, 化疗是必要的辅助治疗。     

儿童肺部原发性肿瘤56例临床分析

目的探讨儿童肺部原发性肿瘤的临床特征、治疗方案及预后。方法 2009—2019年间广州市妇女儿童医疗中心共收治儿童肺部原发性肿瘤患儿56例, 收集其一般资料、影像学资料、病理资料及手术记录等, 进行回顾性分析。结果 56例患儿中, 胸膜肺母细胞瘤28例, 炎性肌纤维母细胞瘤20例, 黏液表皮样癌6例, 血管瘤1例, 硬化性肺细胞瘤1例。临床表现以呼吸道症状为主, 咳嗽26例, 气促17例, 咳血3例。另外, 发热15例, 胸痛3例, 上腹部疼痛2例。肿瘤位于左肺下叶18例, 右肺下叶11例, 左肺上叶10例, 右肺上叶7例, 右肺中叶6例, 肺门4例。56例患儿均行手术治疗, 其中行开胸手术41例, 胸腔镜手术13例, 纤维支气管镜下手术2例。5例Ⅰ型胸膜肺母细胞瘤患儿术后未行化疗, 至随访结束仍存活。5例Ⅱ型胸膜肺母细胞瘤患儿中, 2例术后未化疗, 复发后死亡;3例术后化疗, 至随访结束仍存活。18例Ⅲ型胸膜肺母细胞瘤患儿术后均予IVADo方案化疗, 出现肿瘤复发6例, 远处转移3例, 肿瘤相关死亡8例。20例炎性肌纤维母细胞瘤患儿中, 行楔形切除术13例, 复发5例;行肺叶切除术6例, 复发1例;行纤维支气管镜手术1例, 术后复发。6例黏液表皮样癌患儿中, 行肺叶切除术5例, 行肺楔形切除术1例, 至随访结束均存活。1例血管瘤患儿行支气管镜下肿物切除术, 1例硬化性肺细胞瘤患儿行肺楔形切除术, 至随访结束均存活。结论儿童肺部原发性肿瘤缺乏特异性临床表现, 手术完整切除有利于取得良好预后。对于Ⅱ、Ⅲ型胸膜肺母细胞瘤, 化疗是必要的辅助治疗。