Tumours of the Chest in Children: A Review

Tumours of the chest in children constitute an array of pathology and clinical symptomatology. These tumours can be benign or malignant; cystic or solid; primary or as a result of secondary metastases. Collectively; tumours of the chest in children are very rare; the exact incidence of which is largely unknown globally. Non neoplastic lesions of the lung including bronchogenic cysts; sequestrations; congenital pulmonary airway malformations as well as infective and inflammatory disorders are 60 times more common than neoplastic causes.1 A tumour of the chest is considerably difficult to diagnose since patients can be asymptomatic for many years before symptoms evolve. Even more so; the symptoms are non-specific and can suggest more common and less sinister pathology. Clinically patients present with a variety of symptoms that depend largely on the location of the tumour.Airway tumours can be symptomatic or can present with chronic cough; wheeze; haemoptysis; atelectasis or persistent pneumonia. Secondary malignant parenchymal tumours are likely to be symptomatic from the primary lesion. Anterior mediastinal tumours can cause compression of the large airways or superior vena caval structures.It stands to reason that the physician needs to have a very high index of suspicion when dealing with these non-specific signs and symptoms. This article provides an approach to tumours of chest and reviews the common aetiology in the different compartments of the chest. The article will focus on common tumours of the airway; lung parenchyma; mediastinum; cardiac and chest wall pathology

Management challenge in chest wall neoplasms; a 2 year experience

Introduction: Chest wall neoplasms are rare and represent only about 5% of all thoracic neoplasm. We present our 2 years analysis of the clinical features, presentation, diagnosis and treatment of chest wall neoplasms

Study design: Case series study

Place and duration of study: Ojha Institute of Chest Diseases, Dow University of Health Sciences, Karachi, Pakistan from Nov 2012- Oct 2014

Methodology: Between 2012 and 2014, 39 patients with solid chest wall masses were enrolled in the study. Tumors were categorized as benign and malignant, including primary and secondary, after histopathological diagnosis with tissue biopsy. Data on patients' characteristics, symptoms, tumor type and management was recorded and analysed

Results: The study included 39 patients [20 males and 19 females] with age range 18-71years [mean 36.3]. 21[53.8%] patients had benign chest wall tumors while 18[46.1%] patients had malignant tumors. Among malignant tumors, 14[77%] patients had primary malignancy where as 4[22%] patients had chest wall tumor secondary to primary tumor elsewhere. Among these 4, the primary tumor remained unknown in 1 patient. The most common benign solid lesion was chest wal lesion lipoma in 8/21 patients [38%]. Among malignant tumors, chondrosarcoma [4/14, 29%] was the most common

Conclusion: Preoperative needs careful assessment of the patient, radiological imaging and histopathological examination for diagnosis of the tumor in the chest wall. Using a multidisciplinary team approach, excellent results can be available with complete surgical resection, reconstruction of the chest wall and appropriate or neo adjuvant treatment where necessary

Thoracic neuroblastoma presenting as recurrent empyema.

Neuroblastoma is the most common intra-abdominal and extracranial solid tumour in children, accounting for 7%–8% of all childhood cancers. It is a malignant tumour of the autonomic nervous system derived from the neural crest. Most children with neuroblastoma have distant metastatic disease at the time of diagnosis. Pulmonary metastasis at the time of diagnosis is rare, and rarer is the presence of associated pleural effusion. We present the case of a child with recurrent empyema, who was diagnosed to have a thoracic neuroblastoma.

Intrathoracic hibernoma: an uncommon tumour / Hibernoma intratorácico: un tumor poco frecuente

Hibernomas are uncommon benign tumours that arise from the remnants of fetal brown adipose tissue. They are usually asymptomatic and have a slow growth pattern. Intrathoracic and pleural locations are exceptional for localization of hibernoma. A review of the English language medical literature revealed more than 110 cases, 20 of which were intrathoracic. In the article below, we discuss a 40-year old male patient who had pleural involvement and was treated by surgical resection. Following resection, the patient has remained problem-free for nine years.

Los hibernomas son tumores benignos poco frecuentes que surgen de restos del tejido adiposo marrón fetal. Son generalmente asintomáticos y tienen un patrón de crecimiento lento. Las localizaciones intratorácicas y pleurales son excepcionales para la localización del hibernoma. Una revisión de la literatura médica en lengua inglesa reveló más de 110 casos, 20 de los cuales se trataban de hibernomas intratorácicos. En el siguiente artículo, discutimos el caso de un paciente de 40 años de edad, que tenía una afección pleural y fue tratado con una resección quirúrgica. Después de la resección, el paciente ha permanecido sin problemas durante nueve años.

Neuroblastoma torácico en la adolescencia: caso clínico / Thoracic neuroblastoma in adolescence: clinical case

Neuroblastoma is predominantly a tumor of early childhood, most cases occur in children under 5 years old. It originates in the adrenal gland and paravertebral ganglion cells (neural crest-derived), being the most common an extracranial solid tumor in children. It is characteristic a spontaneous regression, However in some cases it shows progression and dissemination to other organs. Objetive: To show a neuroblastoma in adolescence, with poor response to chemotherapy and radiotherapy, requiring surgery treatment. Clinical case: A 16 y.o. Female patient, previously asymptomatic, who after a body temperature rise up to 39 ° C, was found to have a tumor in the right hemithorax. Biopsy was compatible with neuroblastoma. Surgical removal of a large 20 x 19 cm tumor was achieved, the only complication presented 10 days postop was recurrent pneumothorax. Conclusion: Despite little or no response to standard treatment, surgical resection of this large tumor achieved complete remission for this patient.

El neuroblastoma es predominantemente un tumor de la infancia temprana que en la mayoría de los casos se presenta en menores de 5 años. Se origina en la glándula suprarrenal y células ganglionares paravertebrales (derivadas de la cresta neural), siendo el tumor sólido extracraneal más común en pediatría, presenta regresión espontánea en algunos casos y en otros progresión y diseminación a otros órganos. Objetivo: Analizar el caso de una adolescente portadora de un neuroblastoma resistente a quimioterapia y radioterapia, que requiere tratamiento quirúrgico. Caso clínico: Paciente asintomática, edad 16 años, que a raíz de alza febril se le detecta un tumor en hemitórax derecho. La biopsia fue compatible con un neuroblastoma. Se realizo la extirpación quirúrgica de un gran tumor con diámetro de 20 cm x 19 cm. A los 10 días se complica con neumotórax recidivante. Conclusión: A pesar de la poca o nula respuesta a las armas terapéuticas habituales: quimioterapia y radioterapia, la cirugía logró la resección del tumor torácico con remisión completa.

Malignant Mesothelioma Presenting as a Giant Chest, Abdominal and Pelvic Wall Mass

Malignant mesothelioma (MM) is a relatively rare carcinoma of the mesothelial cells, and it is usually located in the pleural or peritoneal cavity. Here we report on a unique case of MM that developed in the chest, abdominal and pelvic walls in a 77-year-old female patient. CT and MRI revealed mesothelioma that manifested as a giant mass in the right flank and bilateral pelvic walls. The diagnosis was confirmed by the pathology and immunohistochemistry. Though rare, accurate investigation of the radiological features of a body wall MM may help make an exact diagnosis.

Multiple myeloma presenting with multiple thoracic manifestations.

Multiple myeloma is a malignant proliferation of plasma cells that affects mainly bone marrow but may also involve other organs as well. We report thoracic involvement in the form of left-sided pleural effusion, osseous lesions, bronchial infiltration, and mediastinal lymphadenopathy in a 61-year-old woman, non-smoker presented with chest pain, dyspnoea, cough and deterioration in general health over the preceding seven months. Immunoelectrophoresis and immunofixation showed raised kappa-light chain immunoglobulin G (IgG) in serum and pleural fluid. Bronchial and pleural biopsies documented myelomatous infiltration and bone marrow aspirate revealed extensive plasma cell infiltration. At eight months, following the fourth cycle of melphalan, endoxan and prednisone based chemotherapy, the patient died.

Transthoracic cutting needle biopsy: a valuable diagnostic procedure.

Intrathoracic mass lesions, especially peripherally situated masses, often present as a problem as tissue diagnosis is not always possible by cytopathological examination. Cutting needle biopsy may be the method of choice in selected cases for tissue diagnosis. Cutting needle biopsy is indicated for patients with peripherally situated mass lesions abutting chest wall, which are non-vascular and non-cystic in nature.

Embryonal rhabdomyosarcoma of the chest wall: a case report and review of the literature.

Embryonal rhabdomyosarcoma is a soft-tissue sarcoma which has a predilection for the head and neck area, genitourinary tract and the extremities. We report a rare case of embryonal rhabdomyosarcoma of the chest wall in an 8-year-old girl, presenting as a destructive tumor in the rib and clinically and radiologically mimicking Ewing's sarcoma. Histopathological examination showed a small round cell tumor. Immunohistochemically, the positivity for muscle markers desmin and myogenin in the tumor cells proved to be useful for making a definitive diagnosis of embryonal rhabdomyosarcoma. Cytogenetic analysis revealed a high level of aneuploidy in the tumor cells, with double-minutes and additional chromosomal structural aberrations. The patient is responding well to chemotherapy.

CT guided fine needle aspiration cytology of thoracic mass lesions: a prospective study of immediate cytological evaluation.

To evaluate the role of CT guided FNAC as diagnostic and supportive investigation for thoracic lesions, 190 patients were studied during two years period from March 2003 to February 2005 in our institution. A total number of 163 (85.8%) lung lesions, 22 (11.6%) mediastinal lesions and 05 (2.6%) pleural lesions were obtained; only neoplastic lesions were categorized as per WHO classification. The diagnostic sensitivity for malignancy was 97.71% and specificity 100%. Though our priority was cytological assessment, the cytological diagnosis was corroborated with clinico-radiological parameters and transbronchial biopsies whenever applicable.

Tumores fetales: II parte / Fetal tumors: part II

Se presenta la segunda parte de una revisión resumida de los diferentes tumores fetales, haciendo énfasis en su diagnóstico prenatal y posibles tratamientos intrauterinos. En esta segunda parte se analizan los tumores torácicos, abdominales y misceláneos.

Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I: Unusual Presentation of Intraabdominal or Intrathoracic Mass

A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.

Thoracic lymphangioma.

Thoracic lymphangioma is a benign congenital condition that may involve any organ within the chest. Here, a case of intra thoracic isolated extramediastinal lymphangioma is reported. The child had respiratory distress from neonatal period. Surgery completely cured the patient. However, it is only the 3rd reported case in English literature.

Carcinoma Originating from Aberrant Breast Tissue of the Right Upper Anterior Chest Wall : A Case Report

Aberrant breast tissue is usually found in proximity to the normal breast, that is, in the axillary, sternal or clavicular regions. Carcinoma occurs more frequently in the aberrant tissue of the axilla than the extra-axillary site though the overall incidence of tumors of aberrant breast tissue is low. To our knowledge, studies regarding the carcinoma of aberrant breast tissue of the extra-axillary site have been reported rarely. Here we report a recent case of carcinoma originating from the extra-axillary aberrant breast tissue, presenting as a subcutaneous nodule on the right upper anterior chest wall. It is suggested that subcutaneous nodules of uncertain origin around the periphery of the breast should be suspected for breast carcinoma as a differential diagnosis and treated properly.

Askin tumor: a malignant small cell tumor.

Malignant tumours of the chest wall are rare in the pediatric age group. Askin tumour, a primitive neuroectodermal tumour with aggressive nature, is extremely rare. Clinico-pathological features make it quite distinct from Ewing's sarcoma. Aggressive nature of the tumour results in short clinical presentation of the patient. Diagnosis of Askin tumour is mainly by histo-pathology. Imaging modalities play only a complimentary role. We present a case diagnosed to have this rare malignant tumour in the thoraco-pulmonary area in a child.

Metastatic adenocarcinoma rib presenting as chondrosarcoma.

A case of chondrosarcoma rib which turned out to be a case of metastatic adenocarcinoma on histopathological examination is being presented.