Large Intrathoracic Tumors Research Articles

A rare case of myxoid pleomorphic liposarcoma in an infant: A report.

Introduction and importanceMPL (myxoid pleomorphic liposarcoma) is an uncommon type of liposarcoma that affects mostly children and infants. Its aggressive behavior and tendency to recur warrant complete excision despite the challenges of troublesome locations.Case presentationA 12-month-old infant presented with an insidious onset of noisy breathing and respiratory distress not relieved by supplemental oxygen via face mask. Examination revealed dullness and decreased air entry on the left chest. Computed Tomographic (CT) scan showed a large solid mass occupying the left hemithorax and displacing the mediastinum to the right. Intraoperatively, a large solid mass arising from the left chest wall and attached to the fifth rib was seen. Histopathology of the resected mass showed myxoid pleomorphic liposarcoma which is non-reactive for MDM2 immunostain.Clinical discussionUnlike other liposarcomas, myxoid pleomorphic liposarcoma occurs in children, commonly in the chest. CT scan is the preferred imaging modality. Treatment is by complete excision where possible. Molecular studies like Fluorescent in-situ Hybridization (FISH) and Immunohistochemistry (IHC) is used for confirmation. It has a high propensity to metastasize and recurrence is expected. Chemotherapy and irradiation following complete resection decrease the disease recurrence.ConclusionSoft tissue malignancy must be considered in the differential diagnosis of a large intrathoracic tumor in an infant. FISH and IHC are essential for confirmation.

Large intra-thoracic desmoid tumor with airway compression: A case report and review of the literature

Intra-thoracic desmoid tumors are exceedingly rare tumors in the pediatric population, and can present with compression of cardiopulmonary structures and respiratory compromise. Surgical and anesthetic management of these tumors are challenging. We report the case of a 14-year-old male who presented with dyspnea, found to have a large intra-thoracic mass that was critically compressing his trachea. We provide a review of the literature of desmoid tumors and discuss the management of intra-thoracic masses.

Robot-Assisted Resection of a Large Posterior Mediastinal Mass

Introduction: Ganglioneuromas are rare, typically benign, tumors that arise from tissues that have a neural crest cell origin. They typically occur in patients ranging in age from 10 to 40 years and are classically found in the adrenal glands. Ganglioneuromas are frequently asymptomatic and discovered incidentally while another condition is being investigated. The video demonstrates a difficult dissection that offers technical advantages over traditional thoracoscopy instruments by the articulating instruments of the robot with run time of 4 minutes 31 seconds. Background: An 18-year-old woman who was being followed for scoliosis was found to have a large left chest mass on a chest x-ray. A chest CT scan revealed a 10×7.5×6.5-cm mass in the left upper chest, consistent with a bronchogenic cyst. She had no reports of fever, shortness of breath, or chest pain, although she did endorse frequent feelings of left chest/shoulder tightness. In an attempt to avoid a large thoracotomy or sternotomy, we proceeded with a robot-assisted resection of the large mass. Methods: The patient was laid supine with the left chest slightly elevated and her left arm extended above her head. She was intubated with a Carlens tube for single lung ventilation. With the left lung deflated, an 8-mm robotic trocar was placed in the left midaxillary line (sixth intercostal space), an 8-mm robotic trocar was placed in left anterior axillary line (fifth intercostal space), and an 8-mm trocar was placed posterior to scapula (fourth intercostal space). An additional 5-mm step trocar was placed as an assistant port, lower left midchest in the posterior axillary line. Using hook cautery and graspers with bipolar cautery, the tumor was dissected free from the surrounding tissues and the blood vessels cauterized. The mass seemed to arise from the left sympathetic ganglia chain, which had to be sacrificed for tumor removal. The tumor extended into the apex of the left chest and into the lower part of the left neck just behind the head of the clavicle. The tumor was resected free and placed into an endocatch bag, where it was cut to facilitate its removal. The anterior axillary line trocar was removed and widened to ∼5 cm to allow specimen removal. A 28F chest tube was used because the larger tube was less likely to clog, and the chest hole was already large enough to accommodate it. The operative time was 5.5 hours, with 5 to 10 minutes of setup for the robot. Final pathology revealed a ganglioneuroma. Results: She was admitted to the step down unit postoperatively with patient-controlled anesthesia pain control. She was transitioned to oral pain medicine with a general diet on postoperative day 1. Her chest tube was removed and she was discharged on postoperative day 2. She was seen in clinic the following month with some left arm numbness and slight symptoms of Horner's syndrome (left eye ptosis and miosis when tired). Twelve months later, she reports being asymptomatic from her Horner's syndrome. Conclusions: Robot-assisted thoracic surgery provides great 3D visualization and articulating instruments that can be used to dissect large intrathoracic tumors and possibly avoid a large thoracotomy or sternotomy. No competing financial interests exist. Runtime of video: 4 mins 31 secs This work was presented as a poster at the 2014 IPEG conference in Scotland.

Studies of Molecular Mechanisms Associated With Increased Deiodinase 3 Expression in a Case of Consumptive Hypothyroidism

Consumptive hypothyroidism (CH) is a rare form of hypothyroidism due to increased catabolic activity of type 3 iodothyronine deiodinase (DIO3) that can occur in large tumors. PATIENTs with CH typically present with markedly increased requirements for exogenous thyroid hormone and resolution after removal of the source of ectopic DIO3. DIO3 is encoded by DIO3, an imprinted gene expressed on the paternal allele that is located in a DIO3/delta-like 1 homolog (DLK1) gene locus regulated by a common control region, intergenic differentially methylated region (IGDMR). Because DIO3 is an imprinted gene, loss of imprinting at the IGDMR is thought to play a role in its increased expression; however, the molecular mechanism for DIO3 in CH currently is not known. The aim of the study was to determine the molecular mechanism for CH in an adult patient. The study was conducted in the Department of Endocrinology of a tertiary care center in Singapore. We report the case of an adult Asian female patient with a large intrathoracic fibrous tumor and severe hypothyroidism that resolved after tumor resection. The patient's tumor expressed increased levels of DIO3 and DLK1 mRNA and protein levels. Methylation-specific PCR of the IGDMR showed similar hypomethylation in placenta, thyroid, leukocytes, and tumor. Western blotting showed activation of sonic hedgehog (SHH) and MAPK signaling pathways that can increase DIO3 and DLK1 expression. Loss of imprinting did not account for overexpression of DIO3 in the patient's tumor. Instead SHH and MAPK/ERK pathway activation was associated with systemic thyroid hormone catabolism and growth of the tumor. These findings raise the possibility that other tumors that have increased SHH and MAPK/ERK signaling also may have intratumor or systemic effects on thyroid hormone function.

Thoracopleuropneumonectomy with Riblike Reconstruction for Recurrent Thoracic Sarcomas

Large intrathoracic tumors may occasionally present with massive infiltration of the lung and chest wall that would require pneumonectomy and total removal of the rib cage to obtain radical surgical excision, but this operation carries a prohibitive risk of death for cardiopulmonary failure in the absence of adequate chest wall reconstruction. We report here four consecutive cases of thoracopleuropneumonectomy (TPP) with en-bloc resection of the entire lung, chest wall, and diaphragm and immediate riblike reconstruction for recurrent thoracic sarcomas. Patients had undergone the initial thoracic surgical resection with curative intent 2-14 years before TPP. There was no postoperative mortality, and all patients were alive and free of disease 8-21 months after TPP. To our knowledge, this is the first report in the medical literature of such an extensive operation, demonstrating technical feasibility, tolerability, and efficacy of one-stage resection and reconstruction by a semirigid three-dimensional riblike prosthesis modeled on a human-derived aluminum cast. Selected patients with advanced low-intermediate thoracic sarcomas are the ideal candidates for this extreme procedure, to maximize the chance of long-term tumor control and possibly cure.

Morcellation in Semi-Exteriorized Pouch in Thoracoscopic Surgery

Extraction of small specimens such as excised blebs through trocar site is a simple procedure. However, working thoracotomy is mandatory for removal of large solid intrathoracic tumors. We have used an instrument to morcellate a specimen in semi-exteriorized pouch during video-assisted thoracoscopic surgery. Morcellation has provided the solution for removing large solid benign tumors without a working thoracotomy.

Primary Pleural Rhabdomyosarcoma: Plain Film, CT and MRI Findings of This Extremely Rare Intrathoracic Tumor

Primary pleural rhabdomyosarcoma is an extremely rare intrathoracic malignancy. We present a case of a previously healthy 2-year-old male complaining of cough and shortness of breath. The plain film, CT and MRI descriptions of this pleural tumor are presented. This is a fast growing tumor that is indistinguishable radiographically from other large intrathoracic tumors such as pleuropulmonary blastoma.

Technique of Thoracoscopic Resection of Posterior Mediastinal Tumors

Tumors of the posterior mediastinum, located in the paravertebral sulcus, account for about 25% of all mediastinal tumors. They are typically related to the sympathetic chain or the rami of intercostal nerves. Mediastinal neurogenic tumors are among the more frequent mediastinal masses seen in adults. Although they are often malignant in children, over 90% are benign in the adult population. Surgical resection is usually indicated. Posterior mediastinal tumors are frequently nerve-related neoplasms. Neurogenic tumors originate from embryonal neural crest cells, which constitute the ganglia, paraganglionic, and parasympathetic systems. Nerve sheath tumors are the most common posterior, paravertebral tumors, accounting for 40 to 60% of all neurogenic tumors. Approximately 10% have spinal canal involvement (“dumbbell” or “hourglass” tumors), thus impacting the approach to resection. Nerve sheath tumors commonly originate from Schwann cells of intercostal nerves. Although almost always benign, malignant degeneration may rarely occur. Neurofibromas also arise from peripheral nerves, with 30 to 40% of neurofibromas occurring in the setting of neurofibromatosis. Multiple tumors may occur in these individuals. The patients with von Recklinghausen’s disease frequently present at a younger age and have a higher risk of malignancy. Ganglioneuromas and ganglioneuroblastomas arise from sympathetic ganglion nerve cells. Although ganglioneuromas are benign, they tend to adhere to adjacent structures, making resection more challenging than with schwannomas. This is the most frequent benign neurogenic tumor in children. In contradistinction to ganglioneuromas, neuroblastomas frequently metastasize. Most posterior mediastinal neurogenic tumors are benign, slow growing, and asymptomatic. They may be present for a long period of time before diagnosis. However, with growth, they can produce symptoms by local compression of adjacent tissue, bone erosion, and spinal canal involvement. Large intrathoracic tumors may produce dyspnea, pain, and cough. Neurologic deficits may also occur. Spinal cord compression from dumbbell tumors may cause abnormal gait, urinary and fecal incontinence, and loss of sensation below the lesion. Radicular pain at the level of the tumor may present with a dermatomal distribution. Occasionally a high thoracic tumor may result in Horner’s syndrome. Currently, most posterior mediastinal tumors are identified incidentally, often on plain chest radiography obtained for other, unrelated indications. Chest computed tomography is indicated to effectively demonstrate size, location, and relationship to adjacent structures. Tumors of nerve sheath origin are typically smooth, spherical, solitary, and discrete. They usually abut vertebral bodies. In the setting of neurofibromatosis, they may be multifocal and appear lobulated. In contrast, tumors of autonomic ganglion (nerve cell) origin may be less circumscribed and often are oblong. Bony changes related to pressure or erosion may be present. Occasionally magnetic resonance imaging (MRI) may be useful for evaluating proximity to the neural foramen (Fig. 1) and for determining spinal cord involvement with dumbbell tumors. Percutaneous biopsy is not required for most posterior mediastinal neurogenic tumors because radiographic diagnosis is sufficient to mandate resection. Observation of posterior mediastinal tumors is rarely appropriate, unless the patient is at prohibitively high risk for thoracoscopic surgery due to significant medical comorbidities. Surgical resection simultaneously provides both diagnosis and therapy. Moreover, permitting growth may result in expansion of the tumor into the neural foramen, requiring a more complex multistage operation involving both posterior and anterior approaches.

Malignant triton tumor in the thoracic spine

We present a 15-year-old patient diagnosed with peripheral neurofibromatosis (NF-1), who was admitted with paraparesis caused by a large intrathoracic tumor with an intracanalicular component that affected the spinal cord. After surgery his condition improved, but a year later he suffered a relapse and died. Histologically the tumor was diagnosed as malignant with neurogenic and myogenic differentiation ("malignant triton tumor"). Malignant triton tumors (MTT) are infrequent; those found in the head and neck and the upper or lower extremities have a better prognosis than those in the retroperitoneum, buttock, or trunk. It is not clear whether this variation is due to a difference in tumor grade, stage, or resectability, or whether it is a consequence of therapy.

INTRATHORACIC PLEOMORPHIC LIPOSARCOMA IN A CHILD: Study of an Unusual Case

Primary intrathoracic liposarcoma is an extremely rare neoplasm in the pediatric age group. We report an unusual case of a 3-year-old boy who presented with a large intrathoracic tumor arising from the right chest wall. Biopsy showed a pleomorphic sarcoma with histological, immunohistochemical, and ultrastructural features of pleomorphic liposarcoma. The tumor displayed an aggressive clinical behavior, and the patient continued to have recurrences despite rigorous chemotherapy and radiation treatment. He subsequently died 2 years after the initial diagnosis.

Intrathoracic Pleomorphic Liposarcoma in a Child: Study of an Unusual Case

Primary intrathoracic liposarcoma is an extremely rare neoplasm in the pediatric age group. We report an unusual case of a 3-year-old boy who presented with a large intrathoracic tumor arising from the right chest wall. Biopsy showed a pleomorphic sarcoma with histological, immunohistochemical, and ultrastructural features of pleomorphic liposarcoma. The tumor displayed an aggressive clinical behavior, and the patient continued to have recurrences despite rigorous chemotherapy and radiation treatment. He subsequently died 2 years after the initial diagnosis.

Large intrathoracic tumors in children: imaging findings.

Massive benign or malignant thoracic tumors in children may arise from the chest wall, pleura, lung parenchyma, or mediastinum and may be primary or secondary. On initial chest radiographs, these masses usually appear as an opaque hemithorax or an obvious large opaque mass. If the site of origin of the intrathoracic tumor can be determined, the differential diagnosis may be based on the location of the tumor, the age of the patient, and distinguishing imaging features. The purpose of this essay is to illustrate imaging findings of large intrathoracic masses that cause an opaque hemithorax on initial chest radiographs. Chest wall masses that do not grow primarily intrathoracically are not included in this discussion.

Resection of advanced stage neuroblastoma with the cavitron ultrasonic surgical aspirator

Current protocols for the treatment of neuroblastoma emphasize total or near total resection of tumor to improve survival. This is preferentially performed as a primary procedure, or is attempted at a second-look operation. Unfortunately, this tumor often grows to large size with invasion of the spinal canal, or encasement of major vascular or other retroperitoneal structures. A primary attempt at complete removal may result in difficult-to-control hemorrhage or injury to, or loss of, vital organs. A second-look procedure carries other intrinsic risks. It often must be performed during a period of chemotherapeutically induced hematologic and immunologic suppression. The presence of adhesions and dense scar tissue increases the complexity of the dissection. The Cavitron Ultrasonic Surgical Aspirator (CUSA) combines continuous fragmentation, irrigation, and aspiration in one instrument. Tissues high in water content are selectively fragmented and aspirated, while tissues high in collagen and elastin (such as blood vessels and pseudocapsular walls) are selectively spared. Five patients, two with large pelvic dumbell tumors, two with large intrathoracic tumors, and one with a seemingly unresectable large right adrenal tumor (crossing the midline with extensive aortocaval nodal involvement) had total or near-total resection accomplished using the CUSA. In these patients, initial resection of the relatively soft inner part of the tumor left a collapsed pseudocapsule, which was then removed under greatly improved exposure in a relatively small field. The constant aspiration virtually eliminated tumor spillage. Since most vessels were skeletonized without penetration, total blood loss was minimized. There were no intraoperative or postoperative complications.

Symptomatic radiation-induced pericarditis in Hodgkin's disease

This is a retrospective review of 193 evaluable patients treated with radiation therapy to the mediastinum for Stages 1, II and III-A Hodgkin's disease. Eligible patients were those receiving 3000 rad or more to the mediastinum and no chemotherapy prior to the radiation. During the study period, 13 patients developed symptomatic pericarditis. The interval post treatment to the development of symptoms was six to 34 months. The incidence of pericarditis was studied as a function of: (1) the was studied as a function of: (1) thedose of radiation at a depth of 2 cm, 5 cm and the midplane of the mediastinum; (2) the ratio of anterior to posterior weighting of dose; (3) the presence or absence of intrathoracic tumor; (4) the size of the tumor when present; and (5) the fraction of the heart exposed to the radiation beam. There was a significant increase in the incidence of significant increase in the incidence of pericarditis with an increased dose of radiation at 2 cm, 5 cm and midplane depths and also with the presence of a large intrathoracic tumor. A reduction in mediastinal dose is recommended.

Intrathoracic Lipomas in Children

An asymptomatic 8-year-old boy had had a large intrathoracic tumor for five years. When noninvasive technics proved fruitless in establishing a diagnosis, surgical removal was elected. A large posterior mediastinal lipoma was discovered. This is the 15th intrathoracic lipoma reported in the pediatric age group.

Extradural and Intrathoracic Lipoma

To Editor:— I was interested in article by Dr. Herbert C. Maier in Aug. 18 issue ofThe Journal( 181 :610) on Extradural and Intrathoracic Lipoma Causing Spinal Cord Compression. It is perfectly correct, as stated by Dr. Maier in his first paragraph, that the onset of paraparesis in a young child with a large intrathoracic tumor might well lead to a tentative diagnosis of neuroblastoma; but neither should such a diagnosis delay surgeons from immediately surgically decompressing spinal cord. In past 6 months at Variety Children's Hospital and at Jackson Memorial Hospital, both in Miami, we have had 2 children with this syndrome with neuroblastomas, one of whom had her spinal cord lesion operated on first, then had her chest lesion removed, following both of these with x-ray treatment. The child is now walking with a practically normal neurological examination. The second

Intrathoracic Tumors

THERE are many disease conditions which may be found within the thoracic cavity and which must be considered when speaking of intrathoracic tumors. Some of these conditions are: Intrathoracic thyroid, thymus enlargement, aneurysm, carcinoma of esophagus, diverticulum of esophagus, malignant tumor of mediastinum, sarcoma and carcinoma of lung, abscess of lung, abscess of mediastinum, Hodgkin's disease, inflammatory glandular enlargement, tumor of chest wall, pleural effusion, and diaphragmatic hernia. The choice of treatment depends on the diagnosis, and, therefore, we must endeavor to be accurate. The X-ray has proven a most valuable aid in the diagnosis of all chest diseases and yet we find that it is sometimes impossible to make a positive differential diagnosis. In the recent literature we find a report of a case in which a mediastinal abscess was found where sarcoma had been diagnosed, and in another instance an aneurysm found where tumor had been diagnosed. A patient recently came under our care who had been receiving high voltage roentgen therapy over a period of many months for what was thought to be a large mediastinal tumor. This proved to be an aneurysm. Tumors of the thoracic wall have been diagnosed as syphilis when the Wassermann reaction was positive, yet the tumor was afterwards found to be a sarcoma. The patient whose history I wish to report was at first supposed by his physician to have a large left-sided pleural effusion. The roentgen examination substantiated his diagnosis. After a considerable amount of fluid had been aspirated the physical signs showed very little change and the roentgen examination showed the left side as cloudy as it had been before the aspiration. On further examination a solid mass could be felt with the aspirating needle and a large intrathoracic tumor was suspected. On subsequent roentgen examination the heart and trachea were found displaced to the right to a very marked degree, the right lung being so encroached upon that apparently only about one-half its usual space was left for it. The patient showed considerable cyanosis and was delirious at times. An attempt at removal of the tumor was decided upon and the first of a two-stage operation done under local anesthesia on November 7, 1927. The second operation, also under local, was done November 12, 1927. On opening the thoracic cavity it was found that the tumor filled the entire left thorax and even depressed the left diaphragm so that it was concave instead of convex. The tumor was delivered with some difficulty on account of its great size and also because of the presence of some adhesions to the posterior chest wall and lung. At this point a general anesthetic was given for a short time. There was a comparatively small pedicle, broad and narrow, which was clamped, ligated, and cut. The field of operation was closed with drainage. The patient made an uneventful recovery and is very well to-day, over a year after his operation.